bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intolerance to an extensively hydrolysed formula mistaken for postoperative diarrhoea in a child with neuroblastoma
Authors: Ward E. , Picton S. 
Published: March 2003

BackgroundPersistent diarrhoea has recently been reported as a common problem following surgical resection for advanced abdominal neuroblastoma.Case studyA 10‐month‐old child, who had previously had no nutritional problems whilst undergoing chemotherapy treatment for a stage IV neuroblastoma, developed severe diarrhoea following tumour resection. He required nutritional support and was commenced on a continuous overnight nasogastric feed of a non‐milk protein hydrolysate feed (MCT Pepdite 0‐2, S.H.S. International Ltd, UK) His diarrhoea persisted and it was assumed that this was a postoperative problem which in time would regress. Two months later the child was admitted with a rectal prolapse, and a colonoscopy and biopsy suggested allergic protocolitis. The diarrhoea resolved rapidly on stopping the feed.ConclusionAlthough he was never re‐challenged with the non‐milk protein hydrolysate, it appears that he had a rare intolerance to an extensively hydrolysed protein formula which was masked by the assumption that his diarrhoea was a postoperative problem.
Self‐injurious behaviour before and after deinstitutionalization
Authors: Nøttestad J. A. , Linaker O. M. 
Published: March 2003

AbstractThe deinstitutionalization movement is presently spreading in Europe. Studies evaluating the effects of deinstitutionalization on behaviour disturbances among people with intellectual disability (ID) have been inconclusive. The present paper focuses on people without self‐injurious behaviour (SIB) who developed SIB after deinstitutionalization. The present authors studied individual and environmental characteristics before and after deinstitutionalization to look for factors associated with the development of SIB which could also be possible intervention points for preventive action. All those individuals in an institution for people with ID who did not have SIB before deinstitutionalization were included in the present study. The individuals who developed SIB after deinstitutionalization (n = 15) formed the study group (group A) and those who did not (n = 53) comprised the control group (group B). The population was examined both before and after deinstitutionalization. As far as possible, the same methods were used at both occasions. The covariates were both individual (e.g. mental health, behaviour disturbances and behaviour deficits) and environmental (e.g. caretaker education, caretaker:patient ratio, housing and leisure activities). Psychiatric disorders were identified in 1987 and 1995 with the Psychopathology Instrument for Mentally Retarded Adults, which was filled in by the caretakers. In 1987, the people in group A who acquired SIB had lower developmental quotients, used wheelchairs more often and had trouble with moving around without help. They also had a greater frequency of epileptic seizures, and hearing and communication impairment. In 1995, there were only minor environmental differences between groups A and B. There were significantly more individuals involved in the rotation period and more unskilled caretakers working with the people in group A than group B. The present authors found no differences between the two groups on variables such as global mental health and behaviour disturbances, or in the use of neuroleptics before or after deinstitutionalization. Groups A and B did not show differences in behaviour disturbances or psychiatric disorders in 1987. In both 1987 and 1995, there were no differences between groups A and B on variables such as accommodation, caretaker:patient ratio, the number of caretakers involved in direct care, the caretakers' education, or the time spent in structured activities before and after deinstitutionalization. The individual characteristics indicating that a person may acquire SIB are behaviour deficits which are suggestive of central nervous system dysfunction or damage, even if the results are inconclusive. The development of SIB may also be facilitated by communication deficits or by reinforcement of a incidentally occurring SIB if the staff includes many unskilled caretakers in the rotation period.
Psychiatric adjustment to leaving school in adolescents with intellectual disability: a pilot study
Authors: Hepper F. , Garralda M. E. 
Published: March 2003

AbstractThe negotiation of stressful life cycle transitions may contribute to the higher prevalence of psychiatric disorders amongst people with intellectual disability (ID). It is possible that leaving school at the age of 16 years might place particular psychological demands on adolescents, increasing the risk of psychiatric morbidity at a time when they are vulnerable as a result of losing the links with health services sustained through school attendance. The present pilot study was designed as a prospective cohort study to investigate whether there is an increase of psychiatric morbidity [rated with the Strengths and Difficulties Questionnaire (SDQ), and through semi‐structured interviews with parents] in adolescents with ID at the time of their transition from school to adult education and services. Although there was a high frequency (eight out of 10 subjects) of reported emotional and behavioural problems prior to transition, there was no increase or decrease in psychiatric morbidity for the group as a whole during the 6 months after leaving school. However, there were marked individual differences in scores on the SDQ, which may be worth investigating in a larger study.
Psychometric evaluation of a Swedish version of the Reiss Screen for Maladaptive Behavior
Authors: Gustafsson C. , Sonnander K. 
Published: March 2003

AbstractBackground People with intellectual disability (ID) are afflicted by mental health problems to a greater extent than other individuals, and the coexistence of ID and mental health problems involves diagnostic as well as treatment difficulties.Methods A Swedish version of the Reiss Screen for Maladaptive Behavior (RSMB), an instrument used for identification of mental health problems in people with intellectual disability (ID) was evaluated in terms of inter‐rater agreement, internal consistency, item grouping and criterion validity based on a random sample and a clinical group of adults with ID.Results The Swedish version of the RSMB had moderate‐to‐low inter‐rater agreement on specific items and good internal consistency. The total score was considered to be a fairly reliable measure of a positive or negative result on the RSMB. A principal component analysis yielded seven interpretable components. A close resemblance in sets of items between the Swedish version and the original version of RSMB was found for three subscales: Aggressive Behaviour, Avoidance Disorder and Depression (Behavioural Signs). The outcome of the criterion validity analysis indicated a higher rate of false negatives than false positives.Conclusions The potential influence of concurrent psychopharmacological treatment is discussed. It is concluded that the Swedish version of the RSMB can be used as intended by staff as a primary screening device for the identification of mental health problems among people with ID in a Swedish setting.
Obsessional slowness in Down's syndrome
Authors: Charlot L. , Fox S. , Friedlander R. 
Published: March 2003

AbstractBackgroundObsessional slowness was originally described by S. Rachman in 1974. His patients had obsessive–compulsive disorder (OCD) and spent hours performing daily routines such as bathing, dressing and eating. Although some ritualistic behaviours were seen, slowness was the most prominent problem for these patients. Subsequently, a number of similar case reports emerged. In 1994, R. J. Pary described a small number of patients who had both obsessional slowness and Down's syndrome (DS). Apart from this, only one other report of slowness symptoms in people with developmental disabilities has been found in the literature, and this individual also had DS.MethodsIn the present exploratory case series report, 11 individuals with DS and slowness are described based on a retrospective chart review. Descriptive data and four case vignettes are presented.ResultsThe 11 individuals with DS were described as spending hours engaged in usual daily routines. Several individuals had tics, hypothyroidism and periods of freezing. Although some ritualistic behaviours were described, slowness was seen to occur in the absence of these, and often without manifest anxiety.DiscussionObsessional slowness may be a severe variant of OCD. Although it appears to occur infrequently, there may be an elevated rate in people with DS. The current report is severely limited in scope since the case descriptions were based on a retrospective review. However, because of the paucity of published information about this clinical phenomena, it was felt that the case series might serve to establish the need for further, more systematic, prospective evaluation of individuals with DS and clinically significant slowness.
Specialist health services for people with intellectual disability in Scotland
Authors: Smiley E. , Cooper S.‐A. , Miller S. M. , Robertson P. , Simpson N. 
Published: March 2003

AbstractBackground People with intellectual disability (ID) are known to have a high prevalence of health needs, and to require access to specialist health services in addition to primary care and generic secondary care health services. However, there is no national database of each locality's specialist health service provision. Such a record would highlight variation in provision and enable benchmarking.Method A 15‐item questionnaire was developed which included questions on ID health services and staffing levels. This was sent to the chief executive of each of the 15 identified National Health Service primary care trusts/health boards which provide ID services in Scotland. The same questionnaire was also sent to the lead clinician/clinical director of each service. The results were converted to per 100 000 population per trust and presented in cumulative frequency tables to allow benchmarking.Results A response rate of 100% was achieved. The results show a wide range in the type of services provided by each locality in Scotland. Only three services (21%) have completed the process of resettlement. There was a wide‐ranging variability in the number of beds/day places and professionals employed per 100 000 population per trust.Conclusions There is widespread diversity in the service provision between different parts of Scotland. Geographical distances and responsibilities for service provision to remote and rural communities did not appear to account for these differences.
From research and development to practice‐based evidence: clinical governance initiatives in a service for adults with mild intellectual disability and mental health needs
Authors: Newman D. W. , Kellett S. , Beail N. 
Published: March 2003

AbstractBackground  Practice‐based evidence represents the contribution of practitioners who utilize research methodologies to examine the quality of their clinical practice and service provision.Methods  The present paper describes the evolution of a routine practice‐based evidence system (PBES) via four phases of research and development. The four phases are described, as is their relevance to assessment and intervention with regard to the mental health problems of people with mild intellectual disability. Phase four describes the development of a routine PBES.Results  The PBES is capable of profiling the individual mental health needs of service users and examining service effectiveness and quality at an organizational level.Conclusions  The PBES is discussed according to its current utility and possible directions for future development. The system is presented as an example of clinical governance that could be utilized by multidisciplinary teams to develop and maintain an organizational culture of quality.
Increased sensitivity of factor IX to phenprocoumon as a cause of bleeding in a patient with antiphospholipid antibody associated thrombosis
Authors: Harbrecht  , Oldenburg  , Klein  , Weber  , Rockstroh  , Hanfland  
Published: March 2003

Harbrecht U, Oldenburg J, Klein P, Weber D, Rockstroh J, Hanfland P (University of Bonn, Bonn; University of Würzburg, Würzburg; and Evangelische Diakonissen Anstalt Bremen, Bremen, Germany). Increased sensitivity of factor IX to phenprocoumon as a cause of bleeding in a patient with antiphospholipid antibody associated thrombosis. J Intern Med 1998; 243: 73–77.We report one patient who presented with a spontaneous bleeding complication under phenprocoumon therapy. Oral anticoagulation was initiated due to deep‐vein thrombosis which was attributed to an antiphospholipid antibody syndrome. Coagulation analysis revealed a strong and selective reduction of factor IX (F IX) activity to 1%, whereas the other vitamin K‐dependent factors (II, VII, X), the prothrombin time and International Normalized Ratio (INR) were within the therapeutic range. After withdrawal of phenprocoumon, all vitamin K‐dependent factors including F IX normalized. Because the patient suffered from a recurrence of thrombotic events, he was re‐exposed to phenprocoumon and the disproportionate decline of F IX was observed again. These findings indicate an increased sensitivitiy of F IX to vitamin K antagonists, representing an uncommon mechanism associated with bleeding complications under oral anticoagulant treatment.
Erythrocytic protein kinase C activity in primary hypertension
Authors: Ek  , Toomik  , Eriksson  , Frithz  , Ronquist  , Engström  
Published: March 2003

Ek P, Toomik R, Eriksson S, Frithz G, Ronquist G, Engström L (University of Uppsala and University Hospital, Uppsala; and Central Hospital, Eskilstuna, Sweden). Erythrocytic protein kinase C activity in primary hypertension. J Intern Med 1998; 243: 299–305.ObjectivesIncreased protein kinase C activity has been reported in erythrocytes from patients with primary hypertension and also from hypertensive rats. In this phenomenological study, we investigated whether a possible increased activity was the result of an augmented amount of enzyme molecules or a more active enzyme.DesignCollect blood samples, separate erythrocytes from other blood cells. After partial purification of protein kinase C in the erythrocyte lysate, assay the enzyme activity under optimal conditions using a specific peptide substrate.SettingCentral Hospital in Eskilstuna and University Hospital in Uppsala, Sweden.SubjectsHealthy individuals: 47 persons (20 women and 27 men). Ten patients with untreated primary hypertension.Main outcome measuresErythrocytes were separated from leucocytes and platelets by passing through a cellulose column followed by repeated washings. Some proteins in the erythrocyte lysate interfering with protein kinase C assay were removed by chromatography on DEAE‐cellulose.ResultsThe mean protein kinase C activity in erythrocytes from healthy individuals was 0.18 ± 0.02 pmol [32P]phosphate min−1× 106 cells, regardless of sex and age. The corresponding value for patients with primary hypertension was 0.16 ± 0.04 pmol [32P]phosphate min−1× 106 cells.ConclusionsThe amount of protein kinase C, measured as the activity at optimal assay conditions, in erythrocytes from patients with primary hypertension is not critical for the development of moderate hypertension.
Dynamic assessment of parathyroid function in acute malaria
Authors: Davis  , Singh  , Choo  , Ibrahim  , Sulaiman  , Kadir  , Ismail  
Published: March 2003

Davis TME, Singh B, Choo KE, Ibrahim J, Sulaiman SA, Kadir ZA, Ismail R (University of Western Australia, Fremantle Hospital, Fremantle, Western Australia, Australia. Universiti Sains Malaysia, Kubang Kerian, Kota Bharu; General Hospital, Kota Bharu, Kuala Krai Hospital, Kuala Krai; Kelantan, Malaysia) Dynamic assessment of parathyroid function in acute malaria. J Intern Med 1998; 243: 349–54.ObjectivesTo investigate the dynamic parathyroid response to rapidly induced, sustained hypocalcaemia in patients with acute malaria and in healthy volunteers.DesignSerum intact parathormone (PTH) concentrations were measured on samples taken before and during a variable‐rate tri‐sodium citrate infusion designed to ‘clamp’ the whole blood ionised calcium concentration 0.20 mmol L−1 below baseline for 120 min.SubjectsSix Malaysian patients aged 17–42 years with acute malaria, four of whom were restudied in convalescence, and 12 healthy controls aged 19–36 years.Main outcome measuresWhole‐blood ionised calcium and serum intact PTH concentrations.ResultsThe mean (SD baseline ionised calcium was lower in the malaria patients than in controls (1.09 ± 0.06 vs. 1.18 ± 0.03 mmol L−1, respectively; P= 0.01) but PTH concentrations were similar (3.0 ± 1.8 vs. 3.3 ± 1.3 pmol L−1; P= 0.33). Target whole‐blood ionised calcium concentrations were achieved more rapidly in the controls than the patients (within 15 vs. 30 min) despite significantly more citrate being required in the patients (area under the citrate infusion‐time curve 0.95 (0.25 vs. 0.57 ± 0.09 mmol kg−1; P < 0.01). The ratio of the change in serum PTH to that in ionised calcium (δPTH/δCa2+), calculated to adjust for differences in initial rate of fall of ionised calcium, was similar during the first 5 min of the clamp (132 ± 75 × 10−6 vs. 131 ± 43 × 10−6 in patients and controls, respectively, P > 0.05), as were steady‐state serum PTH levels during the second hour (7.0 ± 2.2 pmol L−1 in each case). Convalescent patients had normal basal ionised calcium levels but the lowest serum intact PTH levels before and during the clamp, consistent with an increase in skeletal PTH sensitivity after treatment.ConclusionsThere is a decreased ionised calcium ‘set point’ for basal PTH secretion but a normal PTH response to acute hypocalcaemia in malaria. Skeletal resistance may attenuate the effects of the PTH response but patients with malaria appear relatively resistant to the calcium chelating effects of citrated blood products.
Do genetic determinates of ischaemic heart disease imply that prevention may become more difficult?
Authors: Tonstad  
Published: March 2003

Tonstad S. (Ullevål Hospital, Oslo, Norway). Do genetic determinants of ischaemic heart disease imply that prevention may become more dificult? (Review) J Intern Med 1998; 243: 333–37.Favourable changes in environment and lifestyle in the community may lead to a situation in which genetic determinants of ischaemic heart disease assume greater importance. Does this imply that ischaemic heart disease becomes more difficult to prevent? Much of the role of the family history as a risk factor within populations may be explained by new genetic mutations. However, differences between populations are generally attributable to environmental differences. A model of gene‐environment interaction implies that individuals or populations with genetic risk markers are both more susceptible and more responsive to environmental changes, including lifestyle and drugs, and thus, may be eminently treatable.
Germline and somatic mutation of the gene for multiple endocrine neoplasia type 1 (MEN1)
Authors: Marx  , Agarwal  , Kester  , Heppner  , Kim  , Emmert‐Buck  , Debelenko  , Lubensky  , Zhuang  , Guru  , Manickam  , Olufemi  , Skarulis  , Doppman  , Alexander  , Liotta  , Collins  , Chandrasekharappa  , Spiegel  , Burns  
Published: March 2003

Marx SJ, Agarwal SK, Kester MB, Heppner C, Kim YS, Emmert‐Buck MR, Debelenko LV, Lubensky IA, Zhuang Z, Guru SC, Manickam P, Olufemi SE, Skarulis MC, Doppman JL, Alexander RH, Liotta LA, Collins FS, Chandrasekharappa SC, Spiegel AM, Burns AL (National Institutes of Health, Bethesda, USA). Germline and somatic mutation of the gene for multiple endocrine neoplasia type 1 (MEN1) (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 447–53.Dideoxyfingerprinting was used to screen for germline and somatic MEN1 mutations. This method, applied to a panel of germline DNA from 15 probands with multiple endocrine neoplasia type 1 (MEN‐1), allowed confident discovery of the MEN1 gene. Germline MEN1 mutation has been found in 47 out of 50 probands with familial MEN‐1, in 7 out of 8 cases with sporadic MEN‐1, and in 1 out of 3 cases with atypical sporadic MEN‐1. Germline MEN1 mutation was not found in any of five probands with familial hyperparathyroidism. Somatic MEN1 mutations were found in 7 out of 33 parathyroid tumours not associated with MEN‐1. Allowing for repeating mutations, a total of 47 different germline or somatic MEN1 mutations have been identified. Most predict inactivation of the encoded ‘menin’ protein, supporting expectations that MEN1 is a tumour suppressor gene. The 16 observed missense mutations were distributed across the gene, suggesting that many domains are important to its as yet unknown functions.
Comparison between subcutaneous and intravenous interleukin‐2 treatment in HIV disease
Authors: Witzke  , Winterhagen  , Reinhardt  , Heemann  , Grosse‐Wilde  , Kreuzfelder  , Roggendorf  , Philipp  
Published: March 2003

Witzke O, Winterhagen T, Reinhardt W, Heemann U, Grosse‐Wilde H, Kreuzfelder E, Roggendorf M, Philipp T (Departments of Medicine, Immunology and Virology, University of Essen, Germany). Comparison between subcutaneous and intravenous interleukin‐2 treatment in HIV disease. J Intern Med 1998; 244: 235–40.ObjectivesTherapy of HIV‐infection using intravenous interleukin‐2 (IL‐2) is known to be effective in terms of increasing CD4‐counts but is associated with significant side‐effects and hospitalization. However, the combination with protease‐inhibitor therapy has not been tested yet. The aim of the present study was to investigate the safety and efficacy of intravenous vs. subcutaneous IL‐2 regimes using 9 Mio I.U. of IL‐2, in combination with protease‐inhibitor therapy.DesignAll patients were treated with a combination of two reverse transcriptase inhibitors and a protease‐inhibitor prior to IL‐2 administration for at least 6 weeks. Ten patients were assigned to the intravenous IL‐2 group (group A), 10 to the subcutaneous group (group B).ResultsIn both treatment groups, CD4 count significantly increased shortly after the end of therapy (group A: 223% over baseline [day 7]; group B: 264% over baseline [day 7]). During the follow‐up CD4 counts slowly decreased thereafter but remained above baseline 3 months following IL‐2 treatment. The CD8 lymphocytes showed a similar but less pronounced pattern with a maximum at day 7 (group A: 116% over baseline, group B: 158% over baseline) and reached baseline earlier in the follow‐up‐period. Altogether the CD4/CD8‐ratio was elevated through long periods on follow‐up. Throughout follow‐up, there were no apparent changes in viral load during IL‐2 therapy in either groups. IL‐2 therapy was administered for a mean time of 4.2 ± 0.1 days in the intra‐venous group and of 4.8 ± 0.1 days in the subcutaneous group until therapy was terminated at day 5 or due to side‐effects. Only 1/10 patients completed the 5‐day course of intravenous therapy in contrast to 6/10 in the subcutaneous group.ConclusionsSubcutaneous interleukin‐2 using 9 Mio IU day−1 in combination with protease‐inhibitors showed equal efficacy as intravenous therapy and was associated with less side‐effects.
Lipid disorders in NIDDM: implications for treatment
Authors: Taskinen  , Smith  
Published: March 2003

Taskinen M‐R, Smith U (University of Helsinki, Helsinki, and Sahlgrenska University Hospital, Gothenburg, Sweden). Lipid disorders in NIDDM: implications for treatment. J Intern Med 1998; 244: 361–70.Diabetes conveys high risk lipid status for cardiovascular disease (CVD). To target the management of diabetic dyslipidaemia requests the understanding of metabolic basis of diabetic dyslipidaemia and its components. Recent progress has opened new information to direct future lipid lowering intervention trials in type 2 diabetic patients. To reduce substantially the risk for CVD aggressive modification of all known risk factors is warranted on the basis of existing data.