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Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable mental retardation. The present article discusses several questions: are T4 and T3 equivalent for the developing brain?

S1575092208762399

Thyroid hormone and iodine metabolism in pregnancy. experimental reasons for maintaining adequate iodine intake in pregnancy

Key words Thyroxine Pregnancy Neurodevelopment Iodine deficiency

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable <dis>mental retardation</dis>. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable mental retardation. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy.

S1575092208762399

Thyroid hormone and iodine metabolism in pregnancy. experimental reasons for maintaining adequate iodine intake in pregnancy

Key words Thyroxine Pregnancy Neurodevelopment Iodine deficiency

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable <dis>mental retardation</dis>. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable mental retardation. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

S1575092208762399

Thyroid hormone and iodine metabolism in pregnancy. experimental reasons for maintaining adequate iodine intake in pregnancy

Key words Thyroxine Pregnancy Neurodevelopment Iodine deficiency

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable <dis>mental retardation</dis>. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

Iodine is an essential micronutrient without which the thyroid is unable to synthesize and secrete its two iodine-containing hormones, tetra-iodo- thyronine or thyroxine (T4) and 3’, 3, 5-tri-iodothyronine (T3), containing, respectively, 4 and 3 iodine atoms per molecule. Both hormones are needed throughout life, with T4 being especially important for the development of the cerebral cortex as early as during the first trimester of pregnancy. The need for adequate iodine intake is recognized among the Rights of the Child, since, after starvation, iodine deficiency is the most frequent nutritional cause worldwide of preventable mental retardation. The present article discusses several questions: are T4 and T3 equivalent for the developing brain? What happens to T4 during iodine deficiency? What changes are imposed on maternal thyroid function by the fetus? What happens when a pregnant woman is iodine deficient? What effect does breast feeding have on iodine status? The answers to the above questions explain why iodine requirements are doubled from the very onset of pregnancy. Even in conditions of mild-moderate iodine deficiency, which still prevail throughout Spain, daily supplementation of at least 200 μg iodine is required to prevent mental retardation, starting, if possible, before the onset of pregnancy and continuing until the end of breast feeding.

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, social dysfunction and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale.

S012173721730105X

Memory maintenance and conservation activities in an elders group

Keywords Cognoscitive Deterioration Elder Cognoscitive Stimulation

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, <dis>social dysfunction</dis> and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% <scp>had <neg>no</neg> <dis>cognoscitive impairment</dis></scp>, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% <scp><neg>no</neg> <dis>social dysfunction</dis></scp>, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions <scp><neg>Absence of</neg> <dis>cognoscitive deterioration</dis></scp> is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, social dysfunction and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% had no cognoscitive impairment, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% no social dysfunction, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions Absence of cognoscitive deterioration is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% had no cognoscitive impairment, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% no social dysfunction, 95.2% no depression and 76.1% reported being satisfied with their life.

S012173721730105X

Memory maintenance and conservation activities in an elders group

Keywords Cognoscitive Deterioration Elder Cognoscitive Stimulation

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, <dis>social dysfunction</dis> and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% <scp>had <neg>no</neg> <dis>cognoscitive impairment</dis></scp>, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% <scp><neg>no</neg> <dis>social dysfunction</dis></scp>, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions <scp><neg>Absence of</neg> <dis>cognoscitive deterioration</dis></scp> is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, social dysfunction and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% had no cognoscitive impairment, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% no social dysfunction, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions Absence of cognoscitive deterioration is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Conclusions Absence of cognoscitive deterioration is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

S012173721730105X

Memory maintenance and conservation activities in an elders group

Keywords Cognoscitive Deterioration Elder Cognoscitive Stimulation

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, <dis>social dysfunction</dis> and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% <scp>had <neg>no</neg> <dis>cognoscitive impairment</dis></scp>, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% <scp><neg>no</neg> <dis>social dysfunction</dis></scp>, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions <scp><neg>Absence of</neg> <dis>cognoscitive deterioration</dis></scp> is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Objective To obtain a clinical and socio-demographic profile of a group of elders of the community who participated in Memory Maintenance and Conservation activities. Materials and methods A series of 21 elders in whom cognoscitive status was determined applying the NEUROPSI Test and the presence of somatic symptoms, anxiety, social dysfunction and depression using the General Health Questionnaire (GHQ-28). Life satisfaction was assessed with the Satisfaction with Life Scale. Results Twenty-one elders, average age 73.5 years (SD: 7.3 years), range: 57-82, 90.5% female; 71.4% reported no current marital relationship, in 66.5% educational level ranged from 1 to 5 years, 42.9% assiduously attended the activities during the last 6 years, 76.2% had no cognoscitive impairment, 90.5% no somatic symptoms, 90.5% no anxiety, 95.2% no social dysfunction, 95.2% no depression and 76.1% reported being satisfied with their life. Conclusions Absence of cognoscitive deterioration is similar to that within the general population. Participants did not report depression, anxiety or somatic complaints, showed good social functioning and integration level and were satisfied with their life. Despite the limitations associated with small sample size, the results encourage us to continue this work and to engage in more rigorous research.

Abstract People with severe mental illnesses, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia.

S2173505009700317

Cardiovascular disease and diabetes in people with severe mental illness

Keywords Severe mental illness Schizophrenia Depression Bipolar disorder Physical health Weight gain Cardiovascular disease Diabetes

Abstract People with <dis>severe mental illnesses</dis>, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from <dis>severe mental illness</dis>. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from <dis>severe mental illness</dis> to screen and treat increased cardiovascular risk factors and diabetes.

Abstract People with severe mental illnesses, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from severe mental illness. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from severe mental illness to screen and treat increased cardiovascular risk factors and diabetes.

Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population.

S2173505009700317

Cardiovascular disease and diabetes in people with severe mental illness

Keywords Severe mental illness Schizophrenia Depression Bipolar disorder Physical health Weight gain Cardiovascular disease Diabetes

Abstract People with <dis>severe mental illnesses</dis>, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from <dis>severe mental illness</dis>. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from <dis>severe mental illness</dis> to screen and treat increased cardiovascular risk factors and diabetes.

Abstract People with severe mental illnesses, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from severe mental illness. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from severe mental illness to screen and treat increased cardiovascular risk factors and diabetes.

The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from severe mental illness. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from severe mental illness to screen and treat increased cardiovascular risk factors and diabetes.

S2173505009700317

Cardiovascular disease and diabetes in people with severe mental illness

Keywords Severe mental illness Schizophrenia Depression Bipolar disorder Physical health Weight gain Cardiovascular disease Diabetes

Abstract People with <dis>severe mental illnesses</dis>, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from <dis>severe mental illness</dis>. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from <dis>severe mental illness</dis> to screen and treat increased cardiovascular risk factors and diabetes.

Abstract People with severe mental illnesses, such as schizophrenia, depression or bipolar disorder, have worse physical health and reduced life expectancy compared to the general population. The excess cardiovascular mortality associated with schizophrenia and bipolar disorder is attributed to an increased risk of the modifiable coronary heart disease risk factors, obesity, smoking, diabetes, hypertension, and dyslipidaemia. Antipsychotic medication and possibly other psychotropic medication like antidepressants can induce weight gain and further increase the risk of adverse metabolic effects which may result in further increased incidence of cardiovascular disease. Patients have limited access to general healthcare with less opportunity for cardiovascular risk screening and prevention than would be expected in a non- psychiatric population. The European Psychiatric Association (EPA), supported by the European Association for the Study of Diabetes (EASD) and the European Society of Cardiology (ESC) published this statement aiming to improve the care of patients suffering from severe mental illness. The intention is to initiate co-operation and shared care between the different health care professionals and to increase the awareness of psychiatrists caring for patients suffering from severe mental illness to screen and treat increased cardiovascular risk factors and diabetes.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC.

S217358081300151X

Significance of tuber size for complications of tuberous sclerosis complex

Keywords Tuberous sclerosis complex Tubers Seizures in tuberous sclerosis complex Autism in tuberous sclerosis complex Mental retardation in tuberous sclerosis complex Magnetic resonance imaging in tuberous sclerosis complex

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, <dis>mental retardation</dis>, and <dis>autistic behaviour</dis>. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and <dis>severe mental retardation</dis>; 3 of these cases also presented with <dis>autistic behaviour</dis>, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and <dis>mental retardation</dis>. Nine patients who had experienced infantile spasms during the first year of life presented <dis>autistic behaviour</dis>. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of <dis>autism</dis>. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, <dis>mental retardation</dis> and <dis>autistic behaviour</dis>), even when the smaller tubers are quite numerous.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres.

S217358081300151X

Significance of tuber size for complications of tuberous sclerosis complex

Keywords Tuberous sclerosis complex Tubers Seizures in tuberous sclerosis complex Autism in tuberous sclerosis complex Mental retardation in tuberous sclerosis complex Magnetic resonance imaging in tuberous sclerosis complex

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, <dis>mental retardation</dis>, and <dis>autistic behaviour</dis>. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and <dis>severe mental retardation</dis>; 3 of these cases also presented with <dis>autistic behaviour</dis>, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and <dis>mental retardation</dis>. Nine patients who had experienced infantile spasms during the first year of life presented <dis>autistic behaviour</dis>. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of <dis>autism</dis>. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, <dis>mental retardation</dis> and <dis>autistic behaviour</dis>), even when the smaller tubers are quite numerous.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour.

S217358081300151X

Significance of tuber size for complications of tuberous sclerosis complex

Keywords Tuberous sclerosis complex Tubers Seizures in tuberous sclerosis complex Autism in tuberous sclerosis complex Mental retardation in tuberous sclerosis complex Magnetic resonance imaging in tuberous sclerosis complex

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, <dis>mental retardation</dis>, and <dis>autistic behaviour</dis>. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and <dis>severe mental retardation</dis>; 3 of these cases also presented with <dis>autistic behaviour</dis>, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and <dis>mental retardation</dis>. Nine patients who had experienced infantile spasms during the first year of life presented <dis>autistic behaviour</dis>. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of <dis>autism</dis>. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, <dis>mental retardation</dis> and <dis>autistic behaviour</dis>), even when the smaller tubers are quite numerous.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes.

S217358081300151X

Significance of tuber size for complications of tuberous sclerosis complex

Keywords Tuberous sclerosis complex Tubers Seizures in tuberous sclerosis complex Autism in tuberous sclerosis complex Mental retardation in tuberous sclerosis complex Magnetic resonance imaging in tuberous sclerosis complex

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, <dis>mental retardation</dis>, and <dis>autistic behaviour</dis>. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and <dis>severe mental retardation</dis>; 3 of these cases also presented with <dis>autistic behaviour</dis>, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and <dis>mental retardation</dis>. Nine patients who had experienced infantile spasms during the first year of life presented <dis>autistic behaviour</dis>. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of <dis>autism</dis>. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, <dis>mental retardation</dis> and <dis>autistic behaviour</dis>), even when the smaller tubers are quite numerous.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

S217358081300151X

Significance of tuber size for complications of tuberous sclerosis complex

Keywords Tuberous sclerosis complex Tubers Seizures in tuberous sclerosis complex Autism in tuberous sclerosis complex Mental retardation in tuberous sclerosis complex Magnetic resonance imaging in tuberous sclerosis complex

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, <dis>mental retardation</dis>, and <dis>autistic behaviour</dis>. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and <dis>severe mental retardation</dis>; 3 of these cases also presented with <dis>autistic behaviour</dis>, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and <dis>mental retardation</dis>. Nine patients who had experienced infantile spasms during the first year of life presented <dis>autistic behaviour</dis>. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of <dis>autism</dis>. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, <dis>mental retardation</dis> and <dis>autistic behaviour</dis>), even when the smaller tubers are quite numerous.

Abstract Introduction Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to- average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

Abstract We report on nine cases with jugular dehiscence in middle ear diagnosticated and followed up in our clinic from 1987 to 2000. Diagnosis was made, in some cases, when carrying out an otoscopy and others by clinical sintomatology, otomicroscopically imaging, audiometry, ct-scan and mri. All patients were affected of hearing loss. Some of them also suffered pulsatile tinnitus and even vertigo.

S0001651901782374

Jugular dehiscence in the hypotympanum: on purpose of nine cases

Key Words Jugular dehiscence Proeminent jugular Pulsatile tinnitus Hearing loss Vertigo

Abstract We report on nine cases with jugular dehiscence in middle ear diagnosticated and followed up in our clinic from 1987 to 2000. Diagnosis was made, in some cases, when carrying out an otoscopy and others by clinical sintomatology, otomicroscopically imaging, audiometry, ct-scan and mri. All patients were affected of <dis>hearing loss</dis>. Some of them also suffered pulsatile tinnitus and even vertigo. The follow up of all patients has been stable and devoid of bleeding complications

Abstract We report on nine cases with jugular dehiscence in middle ear diagnosticated and followed up in our clinic from 1987 to 2000. Diagnosis was made, in some cases, when carrying out an otoscopy and others by clinical sintomatology, otomicroscopically imaging, audiometry, ct-scan and mri. All patients were affected of hearing loss. Some of them also suffered pulsatile tinnitus and even vertigo. The follow up of all patients has been stable and devoid of bleeding complications

The follow up of all patients has been stable and devoid of bleeding complications

S0001651901782374

Jugular dehiscence in the hypotympanum: on purpose of nine cases

Key Words Jugular dehiscence Proeminent jugular Pulsatile tinnitus Hearing loss Vertigo

Abstract We report on nine cases with jugular dehiscence in middle ear diagnosticated and followed up in our clinic from 1987 to 2000. Diagnosis was made, in some cases, when carrying out an otoscopy and others by clinical sintomatology, otomicroscopically imaging, audiometry, ct-scan and mri. All patients were affected of <dis>hearing loss</dis>. Some of them also suffered pulsatile tinnitus and even vertigo. The follow up of all patients has been stable and devoid of bleeding complications

Abstract We report on nine cases with jugular dehiscence in middle ear diagnosticated and followed up in our clinic from 1987 to 2000. Diagnosis was made, in some cases, when carrying out an otoscopy and others by clinical sintomatology, otomicroscopically imaging, audiometry, ct-scan and mri. All patients were affected of hearing loss. Some of them also suffered pulsatile tinnitus and even vertigo. The follow up of all patients has been stable and devoid of bleeding complications

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area.

S2173573511000937

Hearing Loss and Airway Problems in Children With Mucopolysaccharidoses

Keywords Mucopolysaccharidoses Hearing loss Airway

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, <dis>hearing loss</dis> and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed <dis>mild bilateral sensorineural hearing loss</dis>; another case was diagnosed as <dis>mixed hearing loss</dis>. The remaining auditory pattern was <dis>moderate bilateral conductive hearing loss</dis>. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing <dis>sensorineural hearing loss</dis>. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010.

S2173573511000937

Hearing Loss and Airway Problems in Children With Mucopolysaccharidoses

Keywords Mucopolysaccharidoses Hearing loss Airway

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, <dis>hearing loss</dis> and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed <dis>mild bilateral sensorineural hearing loss</dis>; another case was diagnosed as <dis>mixed hearing loss</dis>. The remaining auditory pattern was <dis>moderate bilateral conductive hearing loss</dis>. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing <dis>sensorineural hearing loss</dis>. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss.

S2173573511000937

Hearing Loss and Airway Problems in Children With Mucopolysaccharidoses

Keywords Mucopolysaccharidoses Hearing loss Airway

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, <dis>hearing loss</dis> and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed <dis>mild bilateral sensorineural hearing loss</dis>; another case was diagnosed as <dis>mixed hearing loss</dis>. The remaining auditory pattern was <dis>moderate bilateral conductive hearing loss</dis>. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing <dis>sensorineural hearing loss</dis>. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss.

S2173573511000937

Hearing Loss and Airway Problems in Children With Mucopolysaccharidoses

Keywords Mucopolysaccharidoses Hearing loss Airway

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, <dis>hearing loss</dis> and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed <dis>mild bilateral sensorineural hearing loss</dis>; another case was diagnosed as <dis>mixed hearing loss</dis>. The remaining auditory pattern was <dis>moderate bilateral conductive hearing loss</dis>. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing <dis>sensorineural hearing loss</dis>. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

S2173573511000937

Hearing Loss and Airway Problems in Children With Mucopolysaccharidoses

Keywords Mucopolysaccharidoses Hearing loss Airway

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, <dis>hearing loss</dis> and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed <dis>mild bilateral sensorineural hearing loss</dis>; another case was diagnosed as <dis>mixed hearing loss</dis>. The remaining auditory pattern was <dis>moderate bilateral conductive hearing loss</dis>. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing <dis>sensorineural hearing loss</dis>. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Abstract Introduction Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007–2010. Results Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.