bluuebunny/crossref_metadata_2025 huggingface dataset:
Incidence of Neurosecretory Dysfunction among Children Aged 6–14 Years in Rehovot, Israel
Authors: ZADIK Z. , KOWARSKI A.
Published: January 2008

Zadik, Z. and Kowarski, A. (Paediatric Endocrine Unit, Kaplan Hospital, Rehovot, Israel and the Department of Paediatric Endocrinology, University of Maryland, Baltimore, USA). Incidence of neurosecretory dysfunction among children aged 6–14 years in Rehovot, Israel. Acta Paediatr Scand [Suppl] 349: 77, 1989.

Screening of 7500 children aged between 5.8 and 14.4 years, out of a total population of 39,690 in this age group in the Rehovot region, revealed 111 children with heights 2.5 SD below the mean for their age, according to the Tanner‐Whitehouse standards. Included among these short children were eight with hypochondroplastic skeletal disease, two with Down's syndrome, four with thalassaemia, four with Turner's syndrome, three with coeliac disease, four with classical growth hormone (GH) deficiency, four with intrauterine growth retardation, four with systemic disease and 78 without obvious underlying causes. In 35 of the 78 children in the last group, the 24‐hour integrated concentration of GH was in the hypopituitary range (< 3.2 ng/ml), and GH neurosecretory dysfunction (NSD) was accordingly diagnosed. This represents an incidence of GH neurosecretory dysfunction of 45% among abnormally short children without underlying pathology, and is consistent with the authors’previous findings. The overall frequency of GH neurosecretory dysfunction in the screened population was 4/1000.
Use of Continuous Subcutaneous Growth Hormone‐Releasing Hormone (GHRH (1–29)NH2) Infusions to Augment Growth Hormone Secretion and to Promote Growth
Authors: BRAIN C. , HINDMARSH P.C. , PRINGLE P.J. , BROOK C.G.D.
Published: January 2008

Brain, C., Hindmarsh, P.C., Pringle, P.J. and Brook, C.G.D. (Endocrine Unit, the Middlesex Hospital, London, UK). Use of continuous subcutaneous growth hormone‐releasing hormone (GHRH (1–29)NH2) infusions to augment growth hormone secretion and to promote growth. Acta Paediatr Scand [Suppl] 349: 109, 1989.

It has previously been shown that an 8‐day continuous subcutaneous infusion of GHRH (1–29)NH2 in adult males augments growth hormone (GH) secretion with no evidence of desensitization of the response. The present report details the results of treatment with continuous subcutaneous infusions of CHRH (1–29)NH2 in eight children aged 6–9 years with partial GH insufficiency. All the children showed augmentation of GH secretion and an increase in growth velocity after 3 and 6 months of therapy.
Modification of 24‐Hour Growth Hormone Secretion after Continuous Subcutaneous Infusion of Growth Hormone‐Releasing Hormone (GHRH (1–29)NH2) in Short Children with Low 24‐Hour Growth Hormone Secretion
Authors: TAUBER M.T. , PIENKOWSKI C. , LANDIER F. , GUNNARSSON R. , ROCHICCIOLI P.
Published: January 2008

Tauber, M.T., Pienkowski, C., Landier, F., Gunnarsson, R. and Rochiccioli, P. (Department of Paediatric Endocrinology, CHU Rangueil, Toulouse Cedex, and Kabi Laboratory, Paris, France and Kabi Peptide Hormones, Stockholm, Sweden). Modification of 24‐hour growth hormone secretion after continuous subcutaneous infusion of growth hormone‐releasing hormone (GHRH (1–29)NH2) in short children with low 24‐hour growth hormone secretion. Acta Paediatr Scand [Suppl] 349: 117, 1989.

Six short children with low 24‐hour growth hormone (GH) secretion were treated with continuous subcutaneous infusion of GHRH (1–29)NH2 for 3 weeks using a portable infusion pump. Restoration of pulsatile GH secretion was observed in all three children treated with 40 pg/kg/day of GHRH, but in only one of the three children treated with 20 pg/kg/day. All parameters of 24‐hour GH secretion increased, but in five children the magnitude of the GH response was greater on day 1 than on day 21 of CHRH treatment. This decrease was not observed in the single child who responded to a low dose of GHRH (20 pg/kg/day); on the contrary, the response in this patient was greater after 21 days of GHRH treatment. Plasma levels of GHRH (1–29)NH2 were significantly higher on day 21 than on day 1 of treatment, suggesting altered pharmacokinetics over time. The effect of GHRH treatment on growth could not be determined because of the short duration of the study, but the data obtained on 24‐hour GH secretion and GHRH metabolism suggest that a long‐acting analogue of GHRH could be useful for the treatment of GH deficient or insufficient children.
Plasma Amino Acids in Term Neonates and Infants with Phenylketonuria before and after Institution of the Diet
Authors: GERDES A.‐M. , NIELSEN J. B. , LOU H. , GÜTTLER F.
Published: January 2008

ABSTRACT. The plasma amino acid concentrations were measured in 12 neonates with phenylketonuria (PKU). Blood samples were taken when fasting and two hours after a meal. The individual amino acid concentrations, the glycine/valine ratios, and the total amino acid concentrations were mutually compared and also compared to reference intervals. When the diet was instituted and plasma phenylalanine decreased, we observed a normalization of the postprandial response of isoleucine, leucine, tyrosine, and valine.
Fibronectin in Meningococcal Sepsis
Authors: BLANCO A. , GUISASOLA J. A. , SOLIS P. , BACHILLER R. , GONZALEZ H.
Published: January 2008

ABSTRACT. Plasma fibronectin was measured with Laurell's immunoelectroassay in 44 patients with meningococcal sepsis. The average value (15.0±7.9 mg/dl) was lower than that in normal children (27.4±8.7 mg/dl) (p<0.001). Fibronectin in patients correlated positively with antithrombin III (AT‐III) values (p<0.02), but not with protein C (0.05<p<0.1). The decrease of fibronectin had no prognostic value. The fibronectin levels were lower in patients with disseminated intravascular coagulation (DIC+), than in those without DIC (DIC−) (p<0.02), but were lower in both groups than in a normal control group. A negative correlation between fibronectin and protein C was only present in DIC− patients (r:‐0.773=p<0.01). Fibronectin varied independent of AT‐III and protein C in DIC+ patients. The study was repeated in 11 patients 24 hours after admission when fibronectin had decreased in 7/11 cases (mean decrease: ‐2.7±8.7 mg/dl). This variation correlated in a negative way with AT‐III (r:‐0.659=p<0.05). In meningococcal sepsis fibronectin decreases very early, even in DIC− patients and its relationship to AT‐III and protein C is different, depending on the presence of DIC and on the stage of evolution of the disease.
Long‐term Treatment with Glucocorticoids/ACTH in Asthmatic Children III.
Authors: OBERGER E. , ENGSTRÖM I. , KARLBERG J.
Published: January 2008

ABSTRACT. The effect on growth of long‐term treatment with prednisolone and/or ACTH1‐24 (tetracosactrin) depot preparation was studied in 40 children with severe bronchial asthma. Height velocity was subnormal before treatment. During treatment the group of 17 children primarily treated with ACTH showed a moderate increase in mean velocity. Their height was not significantly altered, and neither was the age at peak height velocity nor adult height. In the group of 23 children treated with prednisolone the mean velocity decreased, resulting in a relative decrease in height. Peak height velocity was delayed by about 2 years in the boys but occurred at the expected time in the girls, as did menarche. Mean adult height was lower than expected after adjustment for mid‐parenteral height. In 10 children ACTH was substituted for prednisolone, and their height velocity increased but not enough to affect adult height, which was just as low as in the patients treated with prednisolone only.
Child Sexual Abuse
Authors: LINDBLAD F. , CELSING M. , ERIXON M. , LUNDBÄCK E. , RYDH B. , STÅHL F.
Published: January 2008

ABSTRACT. Thirty‐eight children, 0‐17 years of age, suspected to have been sexually abused by a relative or an acquaintance were psychiatrically evaluated. The suspicions had developed in various ways: through accounts given by the child, through the child's contacts with a person suspected of having abused another child, through a witness or through conclusions drawn, for instance, from changes in behaviour or play patterns of the child. Genital fondling was the most common sexual act reported. The children usually described repeated abuse. The duration of the abuse situation was more than one year in the majority of the cases.
Retinopathy of Prematurity. Where to Set Screening Limits? Recommendations Based on Two Danish Surveys
Authors: FLEDELIUS H. C. , ROSENBERG T.
Published: January 2008

ABSTRACT. If all cases of serious ROP are to be detected, followed, and possibly treated, and the entire natural history of ROP to be recognized, screening limits should be set at no lower than gestational age 32 weeks and/or birthweight 1750 g. These recommendations are based on the experience gained from two Danish surveys: cases registered as visually impaired (compulsory), from the whole country, covering the birth years 1971‐88 (n = 142), and an epidemiological study from a Danish county comprising 411 infants considered at risk in 1982‐1987; 63 infants developed ROP, eventually resulting in blindness in 6 of them.
Greek Immigrant Children in Southern Sweden in Comparison with Greek and Swedish Children II. Meal Pattern and Food Habits
Authors: NEIDERUD J. , PHILIP I.
Published: January 2008

ABSTRACT. Greek immigrant children (GI) belonging to the second generation of immigrants in Sweden have been compared with Swedish children (S) and Greek children in Greece (G) regarding meal pattern and food habits. Interviews were performed in the homes. Meal pattern and frequency of consumption of various food items were studied by 24‐hour recalls, and the food frequency interview method. The meal pattern and the distribution of meals and snacks during the day did not show large differences between the groups. In the GI and G groups it was common to start the day with milk and sugar. The same habit was reported before going to bed. It was common in the GI and G groups to have a prepared meal (dinner) relatively late in the evening. Of the S children only 55% had dinner. GI children had a less frequent intake of vegetables compared to S and G children. GI and G children were heavy consumers of sweets and snacks. In conclusion, the Greek immigrant group had food habits similar to those of Greek rural children with few distinctive exceptions. Greek immigrant families had no difficulties in preparing Greek food.
Development of a Screening System for Cystic Fibrosis: Meconium or Blood Spot Trypsin Assay or Both?
Authors: PEDERZINI F. , FARAGUNA D. , GIGLIO L. , PEDROTTI D. , PEROBELLI L. , MASTELLA G.
Published: January 2008

ABSTRACT. High blood trypsin levels during early days of life are found in newborns subsequently diagnosed to be affected by cystic fibrosis. The authors compared the validity of the traditional meconium test with the blood immunoreactive trypsin (IRT) assay, carried out in parallel on 113302 neonates from three regions of North‐eastern Italy. The meconium test showed a sensitivity of 57.7%. The sensitivity of the IRT test was higher (96.1 %). It was possible to identify by IRT 10 out of 11 false negative CFs at the meconium test. A shortcoming of neonatal IRT, however, is its low specificity; 1.6% of the newborns had to be retested. A new screening policy was therefore proposed and carried out on 69 640 newborns: the Lactase test (LACT) on meconium was introduced as a complementary assay in IRT positive newborns. If LACT exceeded 2 U/g dry meconium, a confirmatory sweat test was immediately requested; if LACT test was negative and IRT exceeded 85 μg/I, IRT was repeated. Postneonatal retesting values above 25 μg/l required a sweat test. As a result, the estimated prevalence of CF was 1:4352, the sensitivity was 93.3%; the specificity turned out to be 99.6%, considering all false positive newborns investigated with retesting and/or direct sweat test.
The Effects of a Pectin‐Supplemented Elemental Diet in a Boy with Short Gut Syndrome
Authors: FINKEL Y. , BROWN G. , SMITH H. L. , BUCHANAN E. , BOOTH I. W.
Published: January 2008

ABSTRACT. The effect of a pectin‐supplemented diet in short gut syndrome was investigated in a 3‐year‐old boy. Nitrogen absorption was higher and stomach‐to‐anus transit time was prolonged during pectin supplementation of the enteral feed. Pectin supplementation had no adverse effects on electrolyte balance or glucose absorption. These data indicate that pectin supplementation of enteral feed may enhance nitrogen absorption and seems not to adversely affect absorption in short gut syndrome.
Meningo‐Encephalitis Associated with HHV‐6 Related Exanthem Subitum
Authors: ISHIGURO N. , YAMADA S. , TAKAHASHI T. , TAKAHASHI Y. , TOGASHI T. , OKUNO T. , YAMANISHI K.
Published: January 2008

ABSTRACT. We report a 10‐month‐old boy with acute meningo‐encephalitis associated with exanthem subitum. It has recently been reported that human herpesvirus‐6 is the causative agent of exanthem subitum, and to our knowledge our case is the first report of meningo‐encephalitis associated with HHV‐6 infection.
Marrow Hypoplasia Associated with Congenital Neurologic Anomalies in Two Siblings
Authors: DRACHTMAN R. , WEINBLATT M. , SITARZ A. , GOLD A. , KOCHEN J.
Published: January 2008

ABSTRACT. Two siblings with congenital neurologic structural anomalies and delayed‐onset selective bone marrow hypoplasia in a previously undescribed constellation of symptoms are presented. Differences between these cases and other well known syndromes are discussed. The importance of this association is the implication that children with congenital neurologic abnormalities may be at increased risk for the development of hypoplastic hematopoietic conditions.
Anti‐Infective Factors in Preterm Human Colostrum
Authors: MATHUR N. B. , DWARKADAS A. M. , SHARMA V. K. , SAHA K. , JAIN N.
Published: January 2008

ABSTRACT.Feeding of the infection prone preterm neonate with concentrated immunologically active ingredients in the form of colostrum may have even more significant clinical implications than in the full term infants. The scarcity of knowledge on anti‐infective factors in colostrum of mothers delivering prematurely prompted us to carry out this study. Colostrum was collected and analysed from 25 mothers delivering prematurely (Study group) and 10 delivering at term (Control group). Major anti‐infective factors namely IgA, IgG, IgM, lactoferrin and lysozyme were quantitated and total cell, macrophage, lymphocyte and neutrophil counts were performed. The mean concentrations of IgA, lysozyme and lactoferrin of preterm colostrum were significantly higher than in full term colostrum (p <0.001). IgG and IgM were found to be similar in both groups. The absolute counts of total cells, macrophages, lymphocytes and neutrophils were found to be significantly higher in the preterm colostrum as compared to the full term colostrum (p < 0.001). Though in both the groups IgA was the predominant immunoglobulin, the mean percentage of IgA in the study group was significantly higher as compared to the control group. Degree of prematurity did not have any influence on the anti‐infective protein levels in colostrum. However total cells and macrophages were significantly higher in colostrum of mothers delivering severely preterm babies.
Severe Mental Retardation in Jordanian Children
Authors: JANSON S. , JAYAKODDY A. , ABULABAN A. , GUSTAVSON K. ‐H.
Published: January 2008

ABSTRACT. The etiology of severe mental retardation has not previously been studied in Jordan. In a group of 203 mainly severely mentally retarded children born 1975–1985, we found the etiology to be related to two main factors: (1) sequele from high perinatal morbidity or meningitis in infancy leading to a combination of severe mental retardation and cerebral palsy; (2) a high degree of intermarriage and a high frequency of retarded siblings indicating that genetic causes of MR are common. The results underscore the importance of further development of antenatal and perinatal care, especially for poor people and the need of genetic councelling.
Noninvasive Estimation of Aortic Valve Areas in Children with Aortic Stenosis
Authors: WENDEL H. , TEIEN D. , HUMAN D. G. , NANTON M. A.
Published: January 2008

ABSTRACT. Fifteen children with aortic stenosis were examined with Echo‐Doppler cardiography in order to evaluate the use of the continuity equation to calculate the aortic valve area. The results were compared to invasive estimates of valve area calculated with the Gorlin's equation. A close correlation between the two methods, r=0.94, was found and it is concluded that even in children an accurate estimate of valve area can be expected with the use of the continuity equation.
Benign Myoclonic Epilepsy of Infancy
Authors: PUIG J. SALAS , RAMOS E. , MACARRON J. , LAHOZ C. H.
Published: January 2008

ABSTRACT. We present a case of benign myoclonic epilepsy of infancy and analyze the characteristic electroclinical findings. We wish to emphasize the usefulness of recognizing this entity among myoclonic epilepsies in children to enable early treatment and to obtain a good prognosis.
Head Circumference Standards for Irish Children
Authors: HOEY HILARY M. C. V. , COX LESLIE A.
Published: January 2008

Abstract. A cross‐sectional study measuring head circumference was performed on 3344 Irish Children aged from 5 to 19 years. Comparison with other countries showed a slightly larger head circumference than the 1965 British standards produced by Tanner, but significantly smaller than the Ounsted data from Oxford. There Was no significant difference between the urban and rural children, but the children of non‐manual workers had a significantly larger head than those of manual workers.
Effect of the Long‐Acting Somatostatin Analogue SMS 201‐995 on Growth Rate and Reduction of Predicted Adult Height in Ten Tall Adolescents
Authors: TAUBER M. T. , TAUBER J. P. , VIGONI F. , HARRIS A. G. , ROCHICCHIOLI P.
Published: January 2008

ABSTRACT. Ten adolescents (four boys and six girls) aged 11.5 to 17 years, presenting with constitutionally tall stature were treated with twice daily subcutaneous injections of 250 μg of SMS 201‐995 (Sandostatin®). Results are reported after 6 and 12 months of therapy. Reduction of growth rate was obtained in 9 patients and correlated with the reduction of 24‐hour growth hormone pulsatility and with the decrease of plasma Somatomedin‐C values, Moreover, acceleration of bone maturation on SMS 201‐995 therapy occurred in seven patients, and mean bone age increased from 13.3 years to 14.5 and 15.8 years after 6 and 12 months of SMS therapy. The mean reduction of predicted adult height was 4.9 cm at the last evaluation after 6 or 12 months of therapy. The dual effect of SMS 201‐995 on growth rate and bone maturation suggests that it may be an alternative treatment to reduce adult height.
Metabolic and Cardiovascular Responses to Exogenous Triiodothyronine Favour Nontreatment of a Girl with Familial Receptor‐Positive Thyroid Hormone Resistance
Authors: HAUFFA B. P. , NAYER P. DE
Published: January 2008

ABSTRACT. A father and daughter with inappropriate TSH secretion due to generalized relative receptor‐positive thyroid hormone resistance are described. Minor variations of tissue peripheral resistance were observed in the adolescent girl. Shortened ankle reflex relaxation time and other clinical signs were consistent with a mild hyperthyroid state of the neuromuscular unit. The response of sex hormone‐binding globulin concentration, 24 hour‐ECG data and left ventricular echocardiographic parameters to increasing doses of triiodothyronine confirmed an euthyroid state of heart and liver tissue in the presence of excess thyroid hormone. Based on these findings and the benign course of the disease, treatment was withheld. Careful assessment, including echocardiography and 24 hour‐ECG, of the functional state of thyroid hormone target tissues during triiodothyronine administration is recommended in these patients. No treatment is required when the functional state of the target tissues is eumetabolic.
Diagnostic Symptoms of Severe and Moderate Haemophilia A and B A Survey of 140 Cases
Authors: LJUNG R. , PETRINI P. , NILSSON I. M.
Published: January 2008

ABSTRACT. With a view to the characterisation of presenting symtoms, a survey was made of 140 boys diagnosed as having haemophilia A or B, severe or moderate form, in Sweden during the years 1960–1987. Mean age at diagnosis was nine months for the severe cases and 22 months for the moderate cases. Although the heredity was known in 59/140 cases, 35 had had bleeding episodes before diagnosis had been established, thus emphasising the importance of genetic information and carrier identification in haemophilia families. Of the presenting symptoms, subcutaneous bleedings constituted 41% while joint and muscle bleedings were uncommon; 16% were bleedings in conjunction with puncture of vessels, injections or surgery. Fourteen percent had anaemin and received blood‐transfusion at diagnosis; 9% were diagnosed post‐neonatally but 20% had shown abnormal bleeding tendency already in the neonatal period; seven boys (5%) had intracranial haemorrhages, five of them neonatally. A thorough family history and an extensive investigation of bleedings in the neonatal period should make early diagnosis possible.
The Predictive Value of a Normal Ultrasound Scan in the Preterm Baby–a Meta‐Analysis
Authors: NG P. C. , DEAR P. R. F.
Published: January 2008

Abstract. Interpretation of the prognostic value of cranial ultrasound abnormalities in preterm infants has been hampered by the lack of a uniform approach to research, especially in the definition and classification of the abnormal appearances. Defining normallty should be less controversial, but from a selected reading of the literature it is possible to gain quite varied impressions as to the prognostic value of normal ultrasound appearances. In order to determine the predictive value for normal development of consistently normal cranial ultrasound scans in the preterm baby, we have performed a meta‐analysis on all the published reports in the English language which provide follow‐up data on such cases. The overall prevalence of major disability among 1604, mainly very low birthweight, babies was 15%. The predictive value of a normal scan for freedom from major disability was 93% and the predictive value for an entirely normal outcome was 88%. The value of this information in the counselling of parents is discussed.
Serum Haemolytic Classical and Alternative Pathways of Complement in Infancy: Age‐Related Changes
Authors: FERRIANI VIRGINIA P. L. , BARBOSA J. E. , de CARVALHO I. F.
Published: January 2008

Abstract. The haemolytic activity of complement was evaluated in the serum of healthy children from birth to 2 years of age using the kinetic method for the determination of the time needed to lyse 50 % of target red cells (t 1/2). No sex‐linked differences were observed in any of the age groups studied and the lowest lytic activity levels for both complement pathways were detected in neonates. The two pathways, however, showed different maturation patterns, i. e., lytic activity levels similar to those of adults were reached between the 1st and 3rd month of life (classical pathway) and around the 13th month (alternative pathway). In the age group of 7 to 24 months, the lytic activity of the classical pathway was higher than in adults. The present data permitted us to establish normal ranges of t 1/2 values for the classical and alternative pathways in serum of healthy neonates and children aged 1 to 24 months.
Platelet Surface‐bound IgG and Platelet‐specific IgG in Plasma in Childhood Thrombocytopenia
Authors: DONNÉR M. , BÉKÁSSY N. A. , HELDRUP J. , WIEBE T. , GARWICZ S. , HOLMBERG L.
Published: January 2008

Abstract. Quantification of platelet‐bound immunoglobulin is widely used in the evaluation of thrombocytopenia. Several methods have been devised among which labelled ligand‐binding assays seem to be most appropriate. In series of adult patients such assays have been shown to be superior in separating immune‐thrombocytopenia from thrombocytopenia of non‐immune causes. We studied 62 children with thrombocytopenia of various causes, using radiolabelled protein A as a ligand to measure platelet‐surface bound IgG. The test was highly sensitive (93%) in detecting immune‐thrombocytopenia. The specificity, however, was only 57 %, which is less than in published studies of adults. In a number of cases presumed to be non‐immune‐thrombocytopenia, notably a few patients with leukaemia and bone marrow aplasia, we found increased amounts of platelet surface‐bound IgG. The significance of this finding is not clear. An indirect assay measuring platelet‐specific IgG in plasma was less sensitive (46 %) but highly specific for immune‐thrombocytopenia (89 %). The measurements of platelet‐surface‐bound IgG and platelet‐specific IgG in plasma are of limited diagnostic value in childhood thrombocytopenia but are useful in following the treatment in chronic ITP.
Scaly Lesions on the Feet in Children–Tinea or Eczema?
Authors: BROBERG A. , FAERGEMANN J.
Published: January 2008

Abstract. Fungal cultures were negative in 20 children aged 1 to 14 years with a clinical picture consistent with juvenile plantar dermatosis. During the same period (April 1987 to August 1988) 4 cases of tinea pedis were seen in the same age group. The important difference between juvenile plantar dermatosis (with or without associated atopic disease) and the clinical picture seen in dermatophytic infection is described.
Motilin and Infantile Colic
Authors: LOTHE L. , IVARSSON S.‐A. , EKMAN R. , LINDBERG T.
Published: January 2008

ABSTRACT. Two hundred and nineteen infants, consecutively born, took part in a prospective study of infantile colic from birth to 12 weeks of age. The prevalence of infantile colic in this group was 17.4%. S‐motilin was studied in 78 term infants (19 with and 59 without infantile colic) at birth (cord blood), at one day, and at 6 and 12 weeks of age, respectively. Basal motilin levels were raised both in cord blood (p<0.01) and in blood from neonates (p<0.001) who developed colic as well as in 6 week (p<0.05) and in 12‐week‐old infants with colic (p<0.01). Formula‐fed infants with colic had higher basal motilin levels than formula‐fed controls at 6 and 12 weeks of age (p<0.05). Breast‐fed infants with colic had higher basal motilin levels than breast‐fed controls at 12 weeks of age (p<0.05). The raised levels of motilin from the first day of life in infants who develop infantile colic might indicate that the gastrointestinal tract is affected in infants with infantile colic, before any symptoms of colic appear.
Autonomic Reflexes in Preterm Infants
Authors: LAGERCRANTZ H. , EDWARDS D. , HENDERSON‐SMART D. , HERTZBERG T. , JEFFERY H.
Published: January 2008

ABSTRACT. Some autonomic nervous reflexes often tested in adult medicine have been studied in 21 preterm infants (25‐37 gestational weeks). The aim was to develop such tests for preterm infants and see if there were any differences in babies with recurrent apnea and bradycardia and babies who had been exposed to sympathicolytic drugs before birth. To test sympathetic nervous activity the peripheral vascular resistance was measured before and during 45° of head‐up tilting. To test parasympathetic nervous activity the degree of bradycardia was measured in response to cold face test (application of an ice‐cube on the fore‐head) and laryngeal stimulation with saline. Finally the heart rate changes after a sudden noise (85 dB) were studied as an indicator of both sympathetic and vagal activity. The peripheral resistance was found to be relatively low in these preterm infants, particularly in some infants tested at the postnatal age of about two months. Heart rate and mean blood pressure did not change during tilting, while the peripheral resistance increased significantly mainly due to lowered limb blood flow. The median decrease of the heart rate during the cold face test was 20.0% and during laryngcal receptor stimulation 23.7%. The sudden noise usually caused a biphasic heart rate response. An autonomic nervous reflex score was calculated and found to be negative (parasympathetic) in infants with recurrent prolonged apnea and bradycardia and positive in infants with clinical signs of increased sympathetic nervous activity.
Effect of Oxandrolone on Growth and Final Height in Turner's Syndrome
Authors: NAERAA R. W. , NIELSEN J. , PEDERSEN I. L. , SØRENSEN K.
Published: January 2008

ABSTRACT.Thirty‐two girls with Turner's syndrome aged 11.5‐16.7 years were treated with oxandrolone (0.125 mg/kg/day). The treatment period was scheduled to 2 years. Height velocity (HV) was expressed in Standard Deviation Scores (SDS), calculated by growth standards for untreated Danish Turner‐girls. For girls with initial bone age below 13 years HV increased significantly from a mean pretreatment value of ‐0.2 SDS (3.1 cm/year) to + 3.5 SDS (5.6 cm/year) in the 1st year of treatment and + 2.1 SDS (4.1 cm/year) in the 2nd year. Mean bone age velocity during treatment was 0.9 year/year. Twenty‐two girls have reached final height. Predictions of their final height were made by three different methods and compared to observed final height. A significant (p<0.001) improvement in the order of 3‐4 cm was found for girls with initial bone age below 13 years, while girls with higher initial bone age had no height gain (p>0.3). Side effects were seen in 16% of the girls.
Growth Hormone Treatment in Non‐Growth Hormone‐Deficient Children: Effects of Stopping Treatment
Authors: ACKLAND F.M. , JONES J. , BUCKLER J.M.H. , DUNGER D.B. , RAYNER P.H.W. , PREECE M.A.
Published: January 2008

ABSTRACT. Overnight physiological growth hormone (GH) secretion was evaluated in 95 short, prepubertal children (73 boys, 22 girls). All the children were below the 3rd centile for height and achieved CH levels greater than 15 mU/1 following pharmacological stimulation. The mean average GH level was 7.1 mU/l and the mean sum of pulse amplitudes 80.4 mU/l. No relationship was found between age, height or height velocity and any of the parameters of GH secretion. The group was randomized to receive placebo, GH or remain under observation for the first 6 months and then all patients received GH treatment for a further 6 months. Those treated with GH, 0.27 IU/kg (0.1 mg/kg) three times weekly, in the first phase. demonstrated a mean increase in height velocity SDS of 3.24. There was no difference in growth response between the placebo or observation groups. In the second 6‐month period. all children received GH according to the same dose regimen: they were then observed for a further 6 months following its discontinuation. In the 6 months following withdrawal of GH, all groups showed a significant fall in height velocity SDS, which returned to pretreatment levels, without demonstrating‘catch‐down’growth. Repeat sampling of overnight GH secretion within 3 days of discontinuing GH showed normal secretory patterns with a small reduction in mean peak amplitude. These results suggest that short children without classic GH insufficiency respond well to exogenous GH in the short term and return to pretreatment height velocities afterwards. Consequently, it may be possible to increase final adult height in such children by GH treatment.
Clinical Usefulness of Urinary Growth Hormone Measurement in Short Children
Authors: TANAKA T. , YOSHIZAWA A. , MIKI Y. , ITO J. , TANAKA M. , TANAE A. , YOKOYA S. , HIBI I.
Published: January 2008

Growth hormone GH) levels in nocturnal urine were measured in 96 short children and 73 children of normal height in order to investigate whether urinary CH levels reflect spontaneous GH secretion and whether they might be used to screen short children for GH treatment. GH levels in 24‐hour urine samples were significantly correlated with urinary albumin and β2‐microglobulin levels in normal children, demonstrating an influence of renal function on urinary GH measurements. Nocturnal urinary GH levels showed significant positive correlations with mean serum GH levels during 3 hours of sleep (r= 0.26. p < 0.05) and plasma insulin‐like growth factor 1 (IGF‐I) levels, reflecting physiological GH secretion. Urinary GH levels were significantly lower in the eight children with complete GH deficiency (3.1 ± 2.3 ng/g creatinine) than in the normal children (13.8 ± 11.2 ng/g creatinine). Urinary GH levels in three other groups of short children, partial CH deficiency (11.1 ± 16.9 ng/g creatinine), impaired GH secretion during sleep (10.4 ± 12.6 ng/g creatinine) and non‐endocrine short stature (18.8 ± 19.5 ng/g creatinine), were not significantly different from those in the normal children. However, when the cut‐off point for defining GH insufficiency was set at 5 ng/g creatinine, 87.5% (21 out of 24) of the short children with low urinary GH levels were suitable subjects for GH treatment (i.e. had complete GH deficiency, partial GH deficiency or impaired GH secretion during sleep). It is concluded that urinary GH measurement, though influenced by renal function, is potentially a simple, non‐invasive and clinically useful method for screening short children for GH insufficiency. Further refinements of the technique are required before it can be widely applied.
Effects of Insulin‐Like Growth Factor I in Man
Authors: GULER H.P. , SCHMID C. , ZAPF J. , FROESCH E.R.
Published: January 2008

ABSTRACT. A 6‐day period of subcutaneous infusion with recombinant human insulin‐like growth factor I in three healthy male volunteers resulted in an increase in the ratio of insulin to C‐peptide levels and significant decreases in triglyceride levels and the ratio of total to high density lipoprotein—cholesterol in serum. Increased renal plasma flow and glomerular filtration rates were also observed.
Assay of Growth Hormone Levels in Human Plasma using Commercial Kits: Analysis of Some Factors Influencing the Results
Authors: CHATELAIN P. , BOUILLAT B. , COHEN R. , SASSOLAS G. , SOUBERBIELLE J.C. , RUITTON A. , JOLY M.O. , JOB J.CI.
Published: January 2008

ABSTRACT. The reliability of growth hormone (GH) assays, performed in 39 different laboratories, using five different immunoassay kits was evaluated. It was found that the variability in GH levels measured by different commercial assay kits may be due to human factors, as well as differences between the kits. The influence of the variation in amount of circulating GH, during provocative testing and spontaneous secretory episodes, on the results of GH assay was also evaluated. It was found that large molecular weight forms of GH may be underestimated by some assay kits.
Assay‐Dependent Results of Immunoassayable Spontaneous 24‐Hour Growth Hormone Secretion in Short Children
Authors: GRANADA M.L. , SANMARTÍ A. , LUCAS A. , SALINAS I. , CARRASCOSA A. , FOZ M. , AUDÍ L.
Published: January 2008

ABSTRACT. Forty‐eight children, referred for evaluation of short stature, underwent 24‐hour spontaneous growth hormone (GH) secretion studies. The GH level in pooled sera was assessed for each child, using up to 11 commercial immunoassays. In a group of 15 children, the mean GH values obtained by nine of the assays were compared with the mean value given by a polyclonal radioimmunoassay (RIA) from Sorin: four gave higher results (p < 0.0001), three gave comparable results and two gave lower results (p < 0.001). The assay yielding the highest results (Nichols: 5.9 ± 2.3 ng/ml, mean ± SD) gave values that were approximately triple those obtained by the assay yielding the lowest results (Hybritech: 1.8 ± 0.8 ng/ml; p < 0.0001); both of these are monoclonal immunoradiometric assays (IRMAs). The GH concentrations measured in 24‐hour pools from 32 children using a monoclonal IRMA from Biomerieux were similar to those obtained using a polyclonal RIA from Farmos (2.8 ± 1.1 ng/ml and 2.9 ± 1.4 ng/ml, respectively) but significantly lower than those measured by another polyclonal RIA from Sorin (3.5 ±1.5 ng/ml). Two polyclonal assays (Biomérieux and Sorin) were then used to measure the GH levels in all of the 30‐minute samples and in the day, night and 24‐hour pools from the secretion studies of 22 children. The ratio of the results of the two assays remained fairly constant for a given child (although the GH levels in different 30‐minute samples differed considerably). However, the ratios between different children showed quite wide variation (from 2.03 to 1.04). It was concluded that the GH assay must be taken into account when evaluating data from GH secretion studies, and the disparity in the GH level measured by two or more assays may differ from child to child.
Growth Response in Prepubertal Children with Idiopathic Growth Hormone Deficiency during the First Year of Treatment with Human Growth Hormone. Analysis of the Kabi International Growth Study.
Authors: RANKE M.B. , GUILBAUD O. ,   On behalf of the Executive Scientific Committee the Kabi International Growth Study
Published: January 2008

ABSTRACT. In order that children with growth hormone deficiency (GHD) reach the goal of normal adult stature, treatment modalities need to be optimized. From the large database of patients enrolled in the Kabi International Growth Study (KIGS), 257 prepubertal patients with idiopathic GHD undergoing their first year of growth hormone (GH) substitution therapy were selected. A multiple regression analysis was performed to determine both auxiological factors characterizing the patients and the factors related to the chosen treatment modalities which are of significance for the observed magnitude of the growth response. Due to the structure of the data, pretreatment height velocity and bone age‐derived auxiological data were not considered. It was observed that the magnitude of the growth response was inversely correlated with chronological age and relative height (HT SDS) at the start of GH treatment but was positively correlated with mid‐parental height. The growth response was also positively correlated with the GH dose (IU/kg/week) and the frequency of GH injections per week. A regression equation using these five parameters was derived, allowing the growth response of these patients to be predicted. The extension of this analytical approach in the future will allow the treatment of patients with GHD to be tailored to individual requirements.
Growth Response in the First Year of Growth Hormone Treatment in Prepubertal Children with Organic Growth Hormone Deficiency: a Comparison with Idiopathic Growth Hormone Deficiency
Authors: PRICE D.A. , RANKE M.B. , GUILBAUD O. ,   On behalf of the Executive Scientific Committee of the Kabi International Growth Study
Published: January 2008

ABSTRACT. Patients in the Kabi International Growth Study (KIGS) up to 1st January 1990 who had organic growth hormone deficiency (OGHD) were identified. They accounted for 21% of all patients with growth hormone deficiency (GHD). Diagnostic categories within the OGHD group included septo‐optic dysplasia, postnatal trauma, craniopharyngioma, other cranial tumours, and following acute leukaemia. Features at presentation and during the first year of hGH treatment were compared with those of children with idiopathic growth hormone deficiency (IGHD). Ninety prepubertal children with OGHD were selected for comparison of observed first‐year height velocity (HV) with predicted values based on those observed in 257 children with IGHD. Those with septo‐optic dysplasia, postnatal trauma and craniopharyngioma responded as predicted, whereas those with other cranial tumours appeared to grow less well than predicted. Glucocorticoid treatment did not affect response, but previous cranial or craniospinal irradiation was found to be associated with an observed HV which was significantly less than predicted.
Low Concentrations of Sodium and Magnesium in Erythrocytes from Cystic Fibrosis Heterozygotes
Authors: FOUCARD T. , GEBRE‐MEDHIN M. , GUSTAVSON K.‐H. , LINDH U.
Published: January 2008

ABSTRACT. The concentrations of electrolytes and trace elements in erythrocytes were determined in eight children with cystic fibrosis (CF) and the parents of four of the children and compared with age‐ and sex‐matched healthy controls. The children with CF had significantly lower median erythrocyte concentrations of sodium, magnesium and zinc and a higher median concentration of calcium than both the healthy control children and the parents of the CF children. Although the sodium and magnesium median values were higher in CF parents than in CF children they were nevertheless significantly lower than in adult controls. These data indicate a system for heterozygote detection.
Loss of Therapeutic Control in Congenital Adrenal Hyperplasia due to Interaction between Dexamethasone and Primidone
Authors: YOUNG M. C. , HUGHES I. A.
Published: January 2008

ABSTRACT. A 14‐year‐old girl with congenital adrenal hyperplasia showed rapid conversion from undertreatment to overtreatment when primidone, used to treat coexistent epilepsy, was withdrawn while on a constant steroid dose. In addition to signs of hypercortisolism and a decrease in random measurements of plasma testosterone and 170H‐progesterone concentrations, the deterioration in control was also illustrated by changes in the pattern of blood spot steroid profiles. Adequate control was achieved only after a 3‐fold reduction in steroid dose. The decrease in dose requirement was probably the result of a reversal of liver enzyme induction consequent upon the withdrawal of primidone.
Testicular Dysgenesis and Mental Retardation in Two Incompletely Masculinized XY‐Siblings
Authors: SCHIPPER J. A. , WAAL H. A. DELEMARRE‐V. D. , JANSEN M. , SPRANGERS M. A. J.
Published: January 2008

ABSTRACT. Aberrations of fetal sexual development were studied in two retarded phenotypic female 46, XY dysgonadal sisters from a consanguineous marriage. Endocrine evaluation revealed an inadequate response of plasma‐testosterone to human chorionic gonadotropin (hCG) stimulation and a normal response to adrenocorticotropic hormone (ACTH) administration. At exploratory laparotomy dysgenetic testes and remnants of the Müllerian and of the Wolffian duct were found. Loss of testicular function, resulting in male pseudohermaphroditism (MPH), can occur at different times during intrauterine development, resulting in a variety of clinical manifestations. A thorough evaluation is warranted in all patients in order to reach a correct diagnosis which is of importance for appropriate gender assignment and genetic counseling.
Cerebral Blood Flow Velocity and Cardiac Output in Infants of Insulin‐Dependent Diabetic Mothers
Authors: van BEL F. , BOR M. VAN DE , WALTHER F. J.
Published: January 2008

ABSTRACT. Cardiac output and cerebral blood flow velocity in the anterior cerebral and internal carotid arteries were investigated in eight large‐for‐date infants of insulin‐dependent diabetic mothers and 12 healthy term infants during the first four days of life using two‐dimensional/pulsed Doppler ultrasound. Temporal mean flow velocity was used as an indicator of changes in cerebral blood flow. Six of the eight infants of diabetic mothers had ventricular septal hypertrophy with reduced cardiac outputs and stroke volumes. Mean flow velocity in both cerebral vessels showed a comparable pattern in both groups throughout the study period and was independent of mean arterial pressure, suggesting unaltered cerebral hemodynamics in the infants of diabetic mothers.
Phenotypes of Peripheral Blood Lymphocytes during Acute Hepatitis A
Authors: MÜLLER C. , GÖDL I. , GÖTTLICHER J. , WOLF H. M. , EIBL M. M.
Published: January 2008

ABSTRACT. We investigated peripheral blood lymphocyte phenotypes of 74 patients at weekly intervals during the course of acute hepatitis A. In the second week after onset of jaundice, a significant elevation of total lymphocytes was observed (4096 × 106± 1003 × 106/l vs. controls 3038 × 106± 1208 × 106/l, p < 0.005). However, no change in the relative percentages of B‐cells (CD20+), T‐cells (CD3+ or CD2+), or T‐cell subpopulations (CD4+ helper cells and CD8+ suppressor cells) could be demonstrated during the course of the disease. Activated T‐cells (CD3+DR+) were elevated during the first week (204 × 106± 134 × 106/l vs. normal 91 × 106± 54 × 106/l, p<0.005) and during the second week (202 × 106± 82 × 106/l, p < 0.0005) after onset of disease and returned to normal values until the third week. Cells expressing phenotypes of lymphocytes capable of exerting non‐MHC‐restricted cellular cytotoxicity, i.e. Natural Killer cell activity (CD57+, CD16+, and CD56+) were significantly elevated in percentage in the first week of disease, as compared to controls (CD57: 14.5 ± 7.0% vs. 9.3 ± 5.8%, p <0.05; CD16: 13.4 ± 7.3 vs. 9.5 ± 5.1%, p < 0.05; CD56: 10.5 ± 3.5% vs. 8.0 ± 1.5%, p < 0.005). Also the absolute numbers of these lymphocyte subpopulations were found to be elevated during the first and second week. The increase in NK cells in the initial phase of acute hepatitis A suggests an important role of these cells in the first line of defence in this disease.
Effect of Metabolic Control on Serum Protein Concentrations in Diabetes
Authors: KEMP STEPHEN F. , FRINDIK J. PAUL
Published: January 2008

ABSTRACT. Serum albumin, transferrin, transthyretin (prealbumin), and retinol binding protein concentrations were determined in 74 children with insulin‐dependent diabetes mellitus before and after a 10‐day camp session during which blood glucose concentrations were controlled. Initial concentrations of albumin and transferrin in the subjects were not different from those in 21 children and adults without diabetes, and did not change during the study period. Transthyretin and retinol binding protein concentrations were lower in subjects with diabetes than in the control population, and increased from 182±49 mg/l and 42.5±13.4 mg/l to 232±71 mg/l and 47.2±13.5 mg/l, respectively. We observed correlations between the changes in transferrin, transthyretin, and retinol binding protein. Although reductions in glycated albumin and transferrin indicated improvement in blood glucose control, there was no correlation between changes in the glycated markers and the concentrations of serum transport proteins. Thus, serum protein concentrations were influenced by the metabolic control of diabetes, but did not directly reflect blood glucose.
Thrombocytopenic Purpura during the Incubation of Hepatitis B
Authors: KORMAN S. H.
Published: January 2008

ABSTRACT. A 6‐year‐old girl presented with acute thrombocytopenic purpura. Hepatitis B surface antigenaemia was present at the time of this illness, and 8 weeks later she developed acute icteric hepatitis B. Screening for hepatitis B virus should be considered in children with apparently idiopathic thrombocytopenic purpura.
Atrial Natriuretic Peptide and Renin‐Aldosterone Relationship in Healthy Newborn Infants
Authors: GEMELLI M. , MAMÌ C. , LUCA F. DE , STELITANO L. , BONACCORSI P. , MARTINO F.
Published: January 2008

ABSTRACT. Plasma atrial natriuretic peptide (ANP) and aldosterone concentrations, and plasma renin activity (PRA) were measured by radioimmunoassay concurrently in 20 healthy full term infants, in cord blood, at 24 hours after birth and on the 4th day of life. ANP and aldosterone increased significantly at 24 hours and was persistently elevated on the 4th day of life. PRA remained unchanged during the first four days of life. In cord blood, ANP concentrations were correlated with aldosterone concentrations (r= 0.49, p<0.05) and hematocrit (r= 0.58, p<0.02). At the 24 hours of life, plasma ANP concentrations were correlated with weight loss observed on the 4th day of life (r= ‐0.70, p<0.005), while the percentage changes in plasma aldosterone concentrations were correlated with percentage changes in systolic blood pressure (BP) (r= 0.49, p<0.05). These findings suggest that during the early newborn period ANP and aldosterone act as an integrated system which has a role in regulation blood pressure and intravascular volume homeostasis.