bluuebunny/crossref_metadata_2025 huggingface dataset:
GHRH Treatment: Studies in an Animal Model
Authors: SHAKUTSUI S. , ABE H. , CHIHARA K.
Published: January 2008

Shakatsui, S., Abe, H. and Chihara, K. (Division of Metabolism and Nutrition, International Centre for Medical Research, and the Third Division, Department of Medicine, Kobe University School of Medicine, Kobe, Japan). GHRH treatment: studies in an animal model. Acta Paediatr Scand [Suppl] 349 101, 1989.

This study examined the effects of chronic deletion of circulating growth hormone‐releasing hormone (GHRH) and/or somatostatin (SRIF) on normal growing male rats, as well as the effects of exogenous GHRH (1–29)NH2 and/or SMS 201–995 administration on the growth of rats with hypothalamic ablation. Passive immunization with anti‐rat GHRH goat γ‐globulin (GHRH‐Ab) for 3 weeks caused a marked decrease in the levels of pituitary GH mRNA and severe growth failure. Treatment with anti‐SRIF goat γ‐globulin (SRIF‐Ab) for 3 weeks produced a more modest decrease in GH mRNA levels in the pituitary and a slight hut significant inhibition of normal somatic growth. Hypothalamic ablation produced a marked decrease in the level of mRNA in the pituitary. Chronic continuous administration of GHRH (1–29)NH2 stimulated pituitary GH synthesis, elevated serum levels of insulin‐like growth factor I and increased body weight gain in rats with hypothalamic ahlation treated with replacement doses of cortisone, testosterone and L‐thyroxine. Combined treatment with GHRH (1–29)NH2 and SMS 201–995 appeared to promote the effect of GHRH on pituitary GH release and somatic growth in these animals. The results suggest that continuous administration of GHRH will be useful in the treatment of children with growth retardation resulting from hypothalamic disorders. In children with combined GHRH and somatostatin deficiencies, the addition of somatostatin to a GHRH treatment regimen may produce better results.
Growth Hormone Response to Overnight Growth Hormone‐Releasing Hormone Infusion and Oral Pyridostigmine in Children with Short Stature
Authors: ROSS R.J.M. , SAVAGE M.O. , KIRK J.M.W. , BESSER G.M.
Published: January 2008

Ross, R.J.M., Savage, M.O., Kirk, J.M.W. and Besser, G.M. (Departments of Endocrinology and Child Health, St Bartholomew's Hospital, London, UK). Growth hormone response to overnight growth hormone‐releasing hormone infusion and oral pyridostigmine in children with short stature. Acta Paediatr Scand [Suppl] 349: 114, 1989.

The development of a long‐acting or depot preparation of growth hormone‐releasing hormone (GHRH) may have many advantages over conventional treatment (with GH) of GH‐deficient children. Pyridostigmine, an acetylcholinesterase inhibitor, has been shown to augment basal GH secretion and the GH response to GHRH in short children. It may thus provide adjuvant therapy to depot GHRH. The GH response to a nocturnal subcutaneous infusion of GHRH (1–29)NH2 in doses of 5 and 10 pg/kg/hour was investigated in five short, slowly growing children. The effect of oral pyridostigmine 60 mg on nocturnal GH secretion and the GH response to a nocturnal infusion was also examined. The subcutaneous infusion of GHRH augmented pulsatile GH release in all five children. There was a dose‐related response to subcutaneous GHRH for the GH area under the curve and mean GH pulse amplitude, but no change in the number of pulses. There was a significant rise in the mean baseline GH concentration during the GHRH infusion compared with placebo. Pyridostigmine had no effect on either basal or stimulated GH secretion.
High Total and Free 1,25‐Dihydroxyvitamin D Concentrations in Serum of Premature Infants
Authors: SCHILLING R. , HASCHKE F. , SCHATTEN C. , SCHMID M. , WOLOSZCZUK W. , STEFFAN I. , SCHUSTER E.
Published: January 2008

ABSTRACT. We investigated the relationship between serum total and free 1,25‐dihydroxyvitamin D (1,25‐OH2D) and the biochemical regulation of 1,25‐OH2D production in premature infants. We measured 1,25‐OH2D, vitamin D binding protein and related biochemical parameters and calculated the free 1,25‐OH2D index in serum of 17 premature infants (birthweight 810–1700 g; gestational age 31–36 weeks) on two different occasions defined by body weight (Study A: 1750–1850 g, Study B: 2100–2200 g). Dietary calcium (Ca) intake was 1,5 or 2,6 mmol/kg/d, phosphorus (P) intake 1,7 mmol/kg/d and vitamin D intake 1000 IU/d. Biochemical results were similar in infants with different Ca intakes and all were within reference ranges. Concentrations of vitamin D binding protein (Study A 0.15±0.03 g/1, Study B 0.14±0.03 g/1; ± SD) were lower, concentrations of 1,25(OH)2D (Study A 180±67 pmol/1, Study B 216±53 pmol/1) were higher, and consequently the free 1,25‐OH2D index (Study A 6.6±2.7, Study B 8.8±2.6) was 4 to 6 times higher than in previously studied term infants. 1,25‐OH2D and the free 1,25‐OH2D index increased significantly with age and were not correlated with serum P or parathyroid hormone. The data indicate that in premature infants with normal biochemical parameters of Ca and P metabolism elevated concentrations of 1,25‐OH2D signify an increased fraction of free 1,25‐OH2D and that increased production of 1,25‐OH2D is not due to hypophosphatemia or hyperparathyroidism.
Duplex versus Unguided Pulsed Doppler Measurements of Right Ventricular Output in Newborn Infants
Authors: WALTHER F. J. , van BEL F. , EBRAHIMI M.
Published: January 2008

ABSTRACT. Noninvasive measurement of pulmonary artery blood flow has widespread implications for management of newborn infants requiring intensive care. Using a precordial, unguided, single Doppler technique, we evaluated right ventricular output in 26 preterm and 16 term infants without cardiopulmonary problems and compared it with right ventricular output obtained by duplex Doppler scanning and left ventricular output measured by a suprasternal single Doppler approach. Weights ranged from 1120 to 3960 g and postconceptional ages from 29 to 42 weeks. Unguided measurements of left and right ventricular output and guided and unguided measurements of right ventricular output were highly correlated (r=+0.92 and +0.95 respectively, p<0.001). The precordial single Doppler technique offers a reliable noninvasive estimate of right ventricular output in preterm and term newborn infants.
Birthweight Specific Perinatal Mortality in Greece
Authors: TZOUMAKA‐BAKOULA CHRYSSA , LEKEA‐KARANIKA VASSO , MATSANIOTIS N. S. , MCCARTHY B. J. , GOLDING JEAN
Published: January 2008

ABSTRACT. Data from the Greek Perinatal Study in April 1983 revealed an excessively high perinatal mortality rate of 21.6 per 1000 total births among singletons despite a low birthweight rate of only 4.5%. Comparison of perinatal mortality rates with Danish mortality rates in 1983, revealed the Greek rates to be three times higher than those in Denmark. When divided by time of death, the Greek stillbirth rates were two times higher and the early neonatal mortality rates were four times higher than the corresponding Danish rates. Subdivision of the Greek perinatal deaths using the Wigglesworth classification showed that the biggest group (40%) consisted of deaths associated with intrapartum asphyxia. The incidence of such deaths was 10 times higher than that found in Denmark. We conclude that in reducing the excessively high perinatal mortality rate in Greece special attention should be made to improve intrapartum and resuscitation techniques.
Somatic Growth in Infants Receiving Prolonged Caffeine Therapy
Authors: LE GUENNEC J. C. , SITRUK F. , BREAULT C. , BLACK R.
Published: January 2008

ABSTRACT. We have evaluated the longitudinal growth of 28 premature infants who had been treated by prolonged caffeine therapy (mean: 23.8 weeks). Routine follow‐up included at least 5 physical examinations with measurement of length, weight and head circumference at the beginning of caffeine therapy, at mid therapy, at the end of the therapy and at 3 and 6 months after therapy. The distribution of the infants on the growth curves according to weight, length and head circumference showed a regular increase in growth parameters. Analysis of variance was highly significant at p<0.0001. We conclude that long term caffeine treatment does not adversely influence growth parameters, at least during early infancy.
Plasma Amino Acids in Phenylketonuric Children Treated Either with Phenylalanine‐free Amino Acids or a Protein Hydrolysate
Authors: GERDES A. ‐M. , NIELSEN J. B. , LOU H. , GÜTTLER F.
Published: January 2008

ABSTRACT. The plasma amino acid concentrations were measured in 10 children with phenylketonuria (PKU) on the phenylalanine‐free amino acid mixture Phenyldon® and 9 PKU children on the phenylalanine‐free protein hydrolysate Albumaid®. The blood samples were taken fasting and two hours after the 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. No differences of the concentration of phenylalanine on the two diet formulas were observed. Children on Phenyldon® showed fasting and postprandial isoleucine higher than the reference intervals. Furthermore, a postprandial increase of several amino acids was observed on both diet formulas. This response was statistically significant in children on Phenyldon®.
Cerebral Oedema Complicating Diabetic Ketoacidosis
Authors: McALOON J. , CARSON D. , CREAN P.
Published: January 2008

ABSTRACT. Signs of raised intracranial pressure (ICP) developed during treatment of diabetic ketoacidosis in a young child. A CT scan revealed cerebral oedema and direct measurement conlirmed elevated ICP. Aggressive treatment was successful in maintaining cerebral perfusion pressure. The child survived with mild handicap in contrast to the poor outcome of previous reports.
Growth and Growth Hormone Secretion in Children Following Treatment of Brain Tumours with Radiotherapy
Authors: DARENDELILER F. , LIVESEY E. A. , HINDMARSH P. C. , BROOK C. G. D.
Published: January 2008

ABSTRACT. We have studied the growth of 144 children after treatment of brain tumours distant from the hypothalamo‐pituitary axis. All had cranial irradiation and 87 spinal irradiation. In 56 patients observed without intervention for 3 years, height SDS in the cranial (CR) group (n= 20) declined from 0.02 to ‐0.44 and in the craniospinal (CS) group (n= 36) from ‐0.28 to ‐ 1.11. Failure of spinal growth had a marked effect in the CS group. The onset of puberty was slightly but not significantly advanced; median ages at onset of puberty were 10.3 years in girls and 12.1 years in boys.

Of the total group 86.4% had clinical and biochemical evidence of growth hormone insufficiency. Fifty‐two children, 33 (28 CS; 5 CR) of whom were prepubertal, received biosynthetic human growth hormone, in a dose of 15 mU/m2/week by daily injection for a period of one year. Height velocity SDS increased significantly in both groups from ‐2.74 to + 1.90 (CS) and from ‐1.0 to +4.26 (CR). Spinal response to GH treatment was restricted in the craniospinal group.
Decreased Adherence, Chemotaxis and Phagocytic Activities of Neutrophils from Preterm Neonates
Authors: BEKTAS S. , GOETZE B. , SPEER C. P.
Published: January 2008

ABSTRACT. Using microanalytic assays various phagocytic functions of separated neutrophils from preterm neonates (mean birthweight 1 506 g, n = 13) were simultaneously studied. Adherence of neutrophils to nylon fibre was decreased in cells from preterm infants (77.1 ± 3.1 %) when compared with adult controls (86.9 ± 2.1 %, mean ± 1 SD, p <0.05). In addition neutrophil chemotaxis in response to zymosan activated serum was reduced in preterm neonates (131.9 ± 19.7, adults 166.6 ± 11.1, p<0.001); directed migration towards Formyl‐Methionyl‐Leucyl‐Phenylalanine was also decreased (preterm neonates 93.4 ± 15, adults 111.1 ± 16.8, p<0.05). Preterm infants had a higher percentage of slow moving neutrophils when compared with adults (p< 0.001). Phagocytosis of Candida albicans was reduced in neutrophils from preterm neonates (phagocytic index: preterm neonates 41.4 ± 12.7, adults 83 ± 7.2). Adult neutrophils ingested more Candida per cell (p <0.001). Chemiluminescence, exocytosis of elastase and lactoferrin during uptake of opsonized zymosan was also reduced in neutrophils from preterm neonates. However, random migration, phagocytosis of Staphylococcus aureus and production of O‐2, in response to Phorbol myristate acetate or opsonized zymosan were identical in cells from either source. We conclude, that these abnormalities of neutrophils could predispose the preterm infant to serious and often overwhelming bacterial and fungal infections.
Postenteritis Enteropathy in Infancy
Authors: PÆRREGAARD A. , HJELT K. , CHRISTIANSEN L. , KRASILNIKOFF P. A.
Published: January 2008

ABSTRACT. Ten patients, who developed postenteritis enteropathy with chronic diarrhoea, failure to thrive and small intestinal mucosal damage after an episode of acute gastroenteritis, were studied prospectively. All patients experienced severe growth retardation. Maximum deviation of height and weight from mean values of age‐correlated normal children were −1.7 and −3.1 SD (median values), respectively, as compared to −0.1 and 0 SD before onset of the illness. One child died after 14 months of illness. The long‐term outcome was satisfactory for the 9 surviving children. For these children, treatment with an elemental diet (in some cases supplemented initially with parenteral nutrition) and later a lactose‐free diet lead to resolution of diarrhoea within periods ranging from 0.5 to 10 months. The phase of catch‐up growth lasted from 4 to 36 months, resulting in final height and weight deviating −0.6 and −0.8 SD (median values), respectively, from normal mean values. Attempt to predict duration of diarrhoea or length of catch‐up growth phase by means of age, weight before illness, characteristics of small intestinal biopsy or maximum deviation of height or weight were unsuccessful. The incidence of postenteritis enteropathy for children of North European ethnic origin was estimated to be 7.6/1000000 children below 7 years of age/year, corresponding to 1.2/1000 children hospitalized for acute gastroenteritis.
Placebo Controlled Trial of Systemic Corticosteroids in Acute Childhood Asthma
Authors: GLEESON J. G. A. , LOFTUS B. G. , PRICE J. F.
Published: January 2008

ABSTRACT. In a randomised controlled trial 38 asthmatic children aged 2‐11 yr who had not received regular oral or inhaled steroids during the previous year, were treated with a standard regime of nebulised salbutamol and intravenous aminophylline plus either hydrocortisone and oral prednisolone for 5 days, or placebo. The children were observed throughout their hospital stay and for 3 months afterwards. There was a greater fall in heart rates in the steroid treated group on the second day of treatment (mean diff. 16 beats/min) and at discharge (mean diff. 13 beats/min); p < 0.025. Peak Expiratory Flow Rates recorded in 26 children, 13 in each group, showed more improvement on day 2 in those given steroids (mean diff 16% predicted); p < 0.05. This difference was not apparent at discharge but 9 children treated with steroids were clinically wheeze‐free when they left hospital compared with 3 in the placebo group, p < 0.05. There were no differences in respiratory rate, pulsus paradoxus and arterial oxygen saturation. Trends in duration of hospital stay and relapse rate during the succeeding 3 months favoured active treatment. These findings support the use of systemic corticosteroids in addition to high dose bronchodilators to treat ‘non steroid dependent’ children hospitalised with acute severe asthma.
Growth Hormone Secretion in Prader‐Willi Syndrome
Authors: COSTEFF H. , HOLM V. A. , RUVALCABA R. , SHAVER J.
Published: January 2008

ABSTRACT. Integrated 12‐hour growth hormone secretion studies, peak growth hormone response to clonidine provocation. Somatomedin‐C levels, T‐4 and TSH levels were studied in six growth‐retarded children with the Prader‐Willi syndrome, of whom five had a 15 q‐karyotype. Only one of the subjects was obese. All showed abnormally low growth hormone secretion. None achieved a nocturnal peak above 10 μg/l, none had a mean nocturnal level over 1.8, and none showed a level above 8 μg/l after clonidine provocation. These findings contrasted with normal TSH in all and normal T‐4 in five. These findings suggest that the poor linear growth in the Prader‐Willi syndrome is caused by a true deficiency of growth hormone secretion, and that the low growth hormone levels observed in such cases are not an artifact of obesity
Hereditary Tyrosinemia of Chronic Course without Rickets and Renal Tubular Dysfunction
Authors: SØVIK O. , KVITTINGEN E. A. , STEEN‐JOHNSEN J. , HALVORSEN S.
Published: January 2008

ABSTRACT. Three patients with hereditary tyrosinemia type 1, two brothers and one girl, studied at the age of 5, 12 and 15 years, respectively, had neither generalized hyperaminoaciduria, glucosuria nor clinical symptoms of rickets. Untreated the elder brother had only slightly elevated plasma tyrosine level (141 μmol/l, normal <80), and low excretion of p‐hydroxyphenyllactate. He presented with pronounced thrombocytopenia (3 × 109/1). At 13 years of age he contracted hepatocellular carcinoma. The younger brother presented with serum tyrosine of 318 μol/l and thrombocyte count 48 × 109/1. Succinylacetone in urine was elevated in both, 30 and 79 μmol/mmol creatinine, respectively. The female patient was investigated for hepatomegaly in infancy, atypical tyrosinemia being considered, but afterwards developed normally without diet or any other treatment until she contracted hepatoma at the age of 15 years. Her plasma tyrosine level was 600‐700 μmol/1, and she excreted large amounts of p‐hydroxyphenyllactate. Succinylacetone in urine was low but elevated (8 μmol/mmol creatinine). The fumarylacetoacetase activity in fibroblasts from the brothers and in lymphocytes from the girl was less than 5% and 10% of control levels, respectively. In conclusion, the chronic form of hereditary tyrosinemia may occur without evidence of renal tubular dysfunction.
Haemolytic Crises due to Glucose‐6‐Phosphate Dehydrogenase Deficiency in the Mid‐Southern Region of Turkey
Authors: KILINÇ Y. , KÜMI M.
Published: January 2008

ABSTRACT. Clinical and laboratory evaluation of 60 boys with hemizygous, 12 girls with homozygous, and 11 girls with heterozygous erythrocyte glucose‐6‐phosphate dehydrogenase deficiency was made during haemolytic crisis. The main clinical symptoms were paleness, dark urine and oliguria. Only one patient needed peritoneal dialysis. Coexistence of glucose‐6‐phosphate dehyrogenase deficiency associated with haemoglobinopathy was found to be higher than expected (32 out of 83 cases). Also, the high prevalence of glucose‐6‐phosphate dehydrogenase deficiency among females with homozygous and heterozygous disease was surprising. The precipitating factors of haemolysis were variable. Rather than antimalarial and antipyretic‐analgesic drugs, infections seemed to be the main haemolytic factor.
Gamma‐Aminobutyric Acid Concentration in Lumbar Cerebrospinal Fluid from Patients with Febrile Convulsions and Controls
Authors: SCHMIEGELOW KJELD , JOHNSEN ANDERS H. , EBBESEN FINN , MORTENSEN TORBEN , BERG ANNA MOURIER , THORN INGRID , SKOV LISELOTTE , ØSTERGAARD JOHN R. , SØRENSEN OLE
Published: January 2008

ABSTRACT. The cerebrospinal fluid (CSF) concentration of the inhibitory neurotransmitter gamma‐aminobutyric acid (GABA) was analysed in 41 children with febrile convulsions (FC), 41 febrile controls of similar age (control group 1), and 59 controls, who had no fever and/or were outside the age range for FC (control group 2). A significant correlation between CSF‐GABA and age was demonstrated for controls (1 + 2) (r= 0.63, p < 0.00001), as well as for patients with FC (r= 0.42, p= 0.003). Patients with FC did not differ significantly from control group 1 in respect to CSF‐GABA. Duration of FC was related to both CSF‐GABA and age (GABA: r=−0.29, p < 0.05; age: r =−0.32, p < 0.05). For 56 controls (1 + 2) > 1 year of age, a significant negative correlation between CFC‐GABA and body temperature was found (r=−0.34, p = 0.01). The low CSF‐GABA in the FC‐labile age group, the negative correlation of CSF‐GABA to body temperature, and the negative correlation of the duration of FC to both CSF‐GABA and age, all indicate that GABA could be of importance in the pathophysiology of FC.
Public Awareness of and Attitudes toward Cerebral Palsy in a Nation‐Wide Adult Population
Authors: SILLANPÄÄ M. L.
Published: January 2008

ABSTRACT. Of 1200 people randomly sampled from the total Finnish population, cerebral palsy was known to 95.4% and its meaning to 60.7%. Nine of ten (88.4%) would let their children play with a child suffering from cerebral palsy. A total of 48% stated that they would, other circumstances being equal, hire persons with cerebral palsy. Half (47.5%) of the subjects regarded it as possible and a further 28.0% as fully possible for individuals with cerebral palsy to make and maintain a family of their own. A good knowledge of cerebral palsy was independently related to a good basic education, age of more than 24 years, female sex and Finnish as native language. A positive attitude toward people with cerebral palsy was independently related to Finnish as native language and a good basic education. A good knowledge of cerebral palsy fostered a positive attitude toward people with this disease.
Cavernous Haemangioma of the Jejunum
Authors: HANSEN U. , BOESGAARD S. , ANDERSEN J.
Published: January 2008

ABSTRACT. A 15‐year‐old boy who had been examined for almost lifelong iron deficiency anaemia caused by occult gastrointestinal bleeding was eventually, by abdominal surgery, found to have a cavernous haemangioma of the jejunum. A diagnostic approach is described and the importance of systematic evaluation and subsequent diagnosis in such patients is stressed.
Neurogenic Pulmonary Oedema
Authors: LEAR G. H.
Published: January 2008

ABSTRACT. Neurogenic pulmonary oedema followed a subarachnoid haemorrhage secondary to an arteriovenous malformation in an eight‐year‐old boy. Despite a complicated course, he made a full recovery. Neurogenic pulmonary oedema is rare in childhood. It is sudden in onset, and potentially fatal, but is amenable to prompt and vigorous treatment.
Self‐Induced Pattern‐Sensitive Epilepsy in Childhood
Authors: MATRICARDI M. , BRINCIOTTI M. , TRASATTI G. , PORRO G.
Published: January 2008

Abstract. We studied five children (1 boy and 4 girls) with self‐induced pattern‐sensitive epilepsy. All patients had refractory epilepsy with multiform, though mainly myoclonic, seizures and medium grade to severe mental retardation. Spontaneously self‐induced seizures were documented in all cases by EEG. All the patients underwent full neurophysiological assessment (baseline EEG recording, with activation: eyelid closed, hyperventilation, ILS, EEG during the randomized presentation of 3 types of spatial structured stimuli, VEP‐F and VEP‐PR). The pattern that triggered the EEG anomalies was highly specific and selective for each patient. Clinical seizures were reproduced by the same patterns as the ones used by the patients to bring on the seizures at will. Authors stress the importance of identifying such peculiar type of epilepsy and of attempting adequate treatment.
Rapp‐Hodgkin Ectodermal Dysplasia
Authors: SANTOS H. , CORDEIRO M. J. G. , VIANA I. FARO , CORDEIRO I. , COLARINHA J. , RODRIGUES M. L.
Published: January 2008

ABSTRACT. A rare case of ectodermal dysplasia–the Rapp‐Hodgkin syndrome–is presented. Its diagnosis is discussed and the importance of a multidisciplinary management and genetic counselling outlined.
SCSB Method Compared to EEG‐Based Polygraphy in Sleep State Scoring of Newborn Infants
Authors: ERKINJUNTTI M. , KERO P. , HALONEN J. ‐P. , MIKOLA H. , SAINIO K.
Published: January 2008

Abstract. Knowledge of the sleep state is important in physiological studies since many physiological variables show different properties in different sleep states. The recently developed static charge sensitive bed (SCSB) method allows long‐term recordings of body movements, respiration and ballistocardiogram without electrodes attached to the subject. The recordings are easy to carry out and they do not disturb the subject in any way. The recorded variables are basic characteristics of different sleep states. SCSB‐recordings and electroencephalography (EEG) based polygraphy as well as blind sleep state scoring were carried out in 8 newborn infants. The positive correlation between SCSB‐scoring and EEG‐based polygraphy scored by two clinical neurophysiologists was 68.1 and 64.1%. Only in 2 % of all epochs was active sleep scored as quiet sleep or vice versa. The results indicate the usefulness of the SCSB method in sleep state scoring of newborn infants.
Renal Consequences of Immobilisation in Children with Fractured Femurs
Authors: ANDREWS P. IAN , ROSENBERG ANDREW R.
Published: January 2008

Abstract. Parameters of renal function and calcium homeostasis were studied in 8 children, immobilised for 5‐9 weeks with fractured femurs, weekly during immobilisation and fourth weekly following mobilisation until all parameters returned to normal. During immobilisation 1 patient became hypercalcaemic, but all showed an increase in serum calcium and all developed hypercalciuria. During immobilisation all showed diminished urine osomolality after a 12‐hour fast (mean 591·133 mOsm/kg) which improved 4‐39 weeks after mobilisation (mean 973·87 mOsm/kg). Serum creatinine, urinary beta‐2‐microglobulin and renal ultrasound appearances were all normal. An inverse relationship, R= ‐0.70, was demonstrated between serum calcium and fasting urine osmolality during immobilisation. Three patients showed diminished urinary concentrating ability beyond 4 weeks after mobilisation. For 1 patient this defect persisted for 8 months and glomerular filtration rate was diminished 9 months after mobilisation, raising the possibility of long term renal damage in immobilised patients.
Electrolytes, Amino Acids and Proteins in Lumbar CSF during the Treatment of Acute Leukemia in Childhood
Authors: KORINTHENBERG R. , ULLRICH K. , RITTER J. , STEPHANI U.
Published: January 2008

Abstract. We performed analyses of electrolytes, amino acids, albumin, alpha2‐macroglobulin, gamma‐globulin and LDH in the lumbar cerebrospinal fluid of children undergoing treatment for acute lymphoblastic leukemia, non‐Hodgkin‐lymphoma or acute myeloid leukemia. At the time of diagnosis signs of a disturbance of the blood‐brain barrier were found in some patients. During induction treatment with L‐asparaginase a rise of glutamic acid and a decrease of glutamine occurred. This finding correlated with slowing of the EEG. Treatment with vincristine was associated with a slight drop of sodium and chloride concentration in serum, but not in the cerebrospinal fluid. Central nervous system prophylaxis with cranial irradiation, and to a lesser degree with intravenous medium‐dose methotrexate, gave rise to a further deterioration of the blood‐brain barrier function as indicated by an increase in albumin, alpha2‐macroglobulin and LDH levels. During radiotherapy the concentration of several amino acids rose, probably due to a disturbance of active carrier mechanisms. Patients with elevated albumin at the end of radiotherapy more often suffered an early leukemia relapse while still on treatment. No other clinical or electroencephalographic correlations of altered barrier function could be found.
Saethre‐Chotzen Syndrome Associated with Defective Neutrophil Chemotaxis
Authors: ETZIONI A. , OBEDEANU N. , BENDERLY A. , GERSHONI‐BARUCH R.
Published: January 2008

Abstract. An 11‐month‐old male infant with Saethre‐Chotzen syndrome and recurrent respiratory infections is described. Persistent extremely high leukocytosis warranted evaluation of neutrophil functions. It was found that the opsonophagocytic activity was normal, but neutrophil chemotaxis was markedly decreased. Further studies pointed to an intracellular neutrophil defect causing this motility dysfunction.
Occipital Osteodiastasis A Rare Complication in Cephalic Delivery
Authors: ROCHE M. C. , VELEZ A. , SANCHEZ P. GARCIA , CASTROVIEJO I. PASCUAL
Published: January 2008

Abstract. A case of occipital bone osteodiastasis in an infant born by cephalic vaginal delivery is presented. Cerebral echography and CT scan did not show posterior fossa haemorrhage. Neurological assessment at three years of age was completely normal.
Small Bowel Permeability to 51Cr‐EDTA in Children with Recurrent Abdominal Pain
Authors: VAN DER MEER S. B. , FORGET P. P. , HEIDENDAL G. A. K.
Published: January 2008

ABSTRACT. Small bowel permeability was investigated in 87 children with recurrent abdominal pain by measuring the 24‐h urinary excretion of orally administered 51Cr‐EDTA. The mean excretion was 3.64%± 1. 49% per 24 h. The difference between the mean urinary excretion in children with recurrent abdominal pain and control children (2.51 %± 0.70 %), was significant (p<0.01, two sample t‐test). The increased small bowel permeability in children with recurrent abdominal pain might indicate an intestinal etiology for the patients' complaints.
Insulin Sensitivity and Glucose‐Induced Insulin Response Changes during Adolescence
Authors: LINDGREN F. , DAHLQUIST G. , EFENDIĆ S. , PERSSON B. , SKOTTNER A.
Published: January 2008

ABSTRACT. Recently we reported that insulin resistance and glucose induced insulin release are inversely correlated to age in young healthy siblings of diabetic patients. To confirm this pattern of change with age, the subjects were reexamined after two years. The study was limited to the age groups with the lowest insulin sensitivity, i. e. 14.0‐15.9 years for females and 16.0‐17.9 years for males. All five girls and four of five boys showed an increased insulin sensitivity as measured by the somatostatin‐insulin‐glucose infusion test (p=0.02). All subjects showed a decrease in fasting levels of insulin‐like growth factor 1 (IGF‐1) (p<0.01) during the observation period. All except one showed an increase in the levels of dehydroepiandrosterone sulphate (DHEA‐S) (p<0.01). These data confirm that insulin sensitivity increases in the late teenage period. The parallelism to the changes of IGF‐1 indicate that the pubertal changes in insulin sensitivity may partly be caused by growth hormone. Our data contradict the hypothesis that the low insulin sensitivity of puberty is due to the increased levels of DHEA‐S.
Microalbuminuria in Diabetic Children and Adolescents
Authors: SALARDI S. , CACCIARI E. , PASCUCCI M. G. , GIAMBIASI E. , TACCONI M. , TAZZARI R. , CICOGNANI A. , BORIANI F. , PUGLIOLI R. , MANTOVANI W. , DONATI S.
Published: January 2008

ABSTRACT. Urinary albumin excretion (UAE) was determined by radioimmunoassay in two 24 h urine collections from 125 diabetic children and adolescents and from 71 normal children matched for age and sex. Thirteen patients (10.4%) aged > 12 years had microalbuminuria, i. e. log transformed UAE levels above the upper normal range (24.5 mg/24 h). UAE values were positively correlated with age, GH secretion, but not with duration of disease, glycosylated hemoglobin, renal size or N‐acetyl‐beta‐glucosaminidase excretion. Diabetic normoalbuminuric children aged 10 years and older had significantly higher UAE than controls and than younger diabetic patients matched for duration of disease. HLA DR3/DR4 heterozygosity frequency was significantly higher (p<0.01) in the microalbuminurine group than in the normoalbuminuric. All microalbuminuric subjects (n=8) with short duration of disease (3.92 ± 3.43 yr) developed diabetes at puberty. In conclusion, our cross‐sectional study suggests: if a number of factors are combined, i. e. HLA DR3/DR4 heterozygosity, onset of disease at puberty and higher GH values, the probability of developing abnormal levels of UAE will increase.
Decreased Absence due to Infectious Diseases in Children at Two Day Care Centres over an Eight‐Year Interval
Authors: SÖDERSTRÖM M. , HOVELIUS B. , SCHALÉN C.
Published: January 2008

ABSTRACT. Causes of absence were recorded at two day care centres during a seven‐month period in 1979/80 and a corresponding period in 1987/88, for 82 and 87 children, respectively. During the eight‐year interval absence due to disease decreased from 8.2 % to 5.7 % of total day‐care days. A decrease in epidemic diseases during the eight years was evident. There were no cases of morbilli, parotitis or rubella in 1987/88, following an immunisation programme for these diseases initiated in 1982. An out‐break of varicellae occurred in 1979/80, as compared with only few cases in 1987/88. Respiratory tract infection was the most common type of illness both in 1979/80 and in 1987/88. The mean number of illness episodes of respiratory tract infections per child, aged 5‐6, was significantly higher in the earlier than in the later period, whereas no corresponding difference was evident for the younger age groups. Although, in the meantime parent benefits for home care of sick children had become more generous, attendance at the two day care centres rose from 62 % in 1979/80 to 79 % in 1987/88 of total day‐care days, suggesting a truly decreased morbidity. The carriage rates of pneumococci, Haemophilus influenzae and Branhamella catarrholis decreased with increasing age of the children, and that of beta‐haemolytic streptococci increased; however, the carriage rates during the two periods did not differ significantly. The overall isolation frequencies of these bacteria were 72 %, 43 % and 38 %, respectively, for children aged 1‐2, 3‐4, and 5‐6 years, and 5.9% for the staff.
Antidiuretic Hormone Following Surgery in Children
Authors: JUDD B. A. , HAYCOCK G. B. , DALTON R. N. , CHANTLER C.
Published: January 2008

ABSTRACT. We studied 13 children subjected to elective tonsillectomy, 6 of whom (study patients) received supplemental intravenous isotonic saline during and after operation, and 7 of whom (controls) did not. Clinical and biochemical evidence of hypovolaemia was present in the control but not in the study patients. Plasma antidiuretic hormone (ADH) and urine osmolality were higher in controls (p<0.005 and p <0.05 respectively). Plasma sodium concentration and osmolality were similar in the two groups. We conclude that hypovolaemia is the principal stimulus to ADH release following surgery and that, in addition to replacement of observed losses of blood and other fluids by fluids of appropriate composition, hypovolaemia should be prevented by the administration of maintenance quantities of isotonic fluid, rather than exacerbated by fluid restriction, in patients in whom oral fluid intake is interrupted for more than a brief period. Hypotonic and sodium free fluids should be avoided because of the risk of hyponatraemia.
Variation of Growth in Height and Weight of Children II. After Infancy
Authors: SORVA R. , LANKINEN S. , TOLPPANEN E.‐M. , PERHEENTUPA J.
Published: January 2008

ABSTRACT. To provide for early detection of abnormal changes in growth, we propose the monitoring of all children for changes in relative height and relative weight as indirect indicators of growth velocity. To this end we analyzed the growth of 2156 children, as recorded by the child health surveillance services at ages 2 to 19 years. From their data we constructed growth standards on charts of a novel type, which allow direct reading of relative height (SD score, SDS) and relative weight (percentage deviation of weight from median weight for height and sex, % DW). Variation in height explained most (mean 60%) of the variation in weight, and age did not contribute significantly. Hence, our weight charts are height‐based. Next, we defined the variations of changes in (Δ) SDS and %DW during the different periods of growth. The group means of changes in each period were zero. Variation in ΔSDS is widest at the earliest ages, then decreases until year 9‐10 (girls) and 10‐11 (boys), and again increases. For Δ%DW the picture is similar. We present these variations as diagrams for use in growth screening.
Clinical Disease Activity and Inflammatory Activity in the Rectum in Relation to Mucosal Inflammation Assessed by Colonoscopy A Study of Children and Adolescents with Chronic Inflammatory Bowel Disease
Authors: HOLMQUIST L. , ÅHRÉN C. , FÄLLSTRÖM S. P.
Published: January 2008

ABSTRACT. The relationship between clinical disease activity, rectal inflammatory activity and mucosal inflammation established at total colonoscopy has been studied in 36 young patients with ulcerative colitis (UC) and 24 with Crohn's colitis (CC). Semiformed stools, diarrhea and frequent bowel movements seem to be better indicators of extensive mucosal inflammation of a moderate or severe degree than blood in faeces. There were, however, several patients with a discrepancy between the degree of clinical disease activity and the extent and degree of mucosal inflammation at endoscopic and histologic assessment. In about one‐third of the patients with UC and in about half of the patients with CC, who had mucosal inflammatory activity of a moderate or severe degree, the rectal inflammatory activity was of a lower degree. Total colonoscopy seems to be necessary in order to establish the extent and degree of mucosal inflammation.
Alterations of the Respiratory Burst of Polymorphonuclear Leukocytes from Diabetic Children A Chemiluminescence Study
Authors: KANTAR A. , WILKINS G. , SWOBODA B. , LITTARRU G. P. , BERTOLI E. , CATASSI C. , COPPA G. , GIORGI P. L.
Published: January 2008

ABSTRACT. The respiratory burst of polymorphonuclear leukocytes was investigated in 24 children with insulin dependent diabetes mellitus and 24 healthy controls. This oxygen dependent, membrane associated process generates a number of toxic oxygen metabolites which are implicated in the pathogenesis of endothelial damage. The activity of polymorphonuclear leukocytes was studied in terms of luminol amplified chemiluminescence. It was found that the resting luminol amplified chemiluminescence activity of isolated polymorphonuclear leukocytes from diabetic children was significantly higher than that of controls (342000±174000 cpm vs. 165000±82000 cpm, p <0.01). The addition of respiratory burst inhibitors caused a significant reduction of basal chemiluminescence (> 80%). When the ratio of phorbol myristate acetate stimulated activity to basal activity was calculated and used as an activation index, it was found to be significantly reduced in diabetics relative to controls (4.29 ± 2.46 vs. 8.34±3.21, p<0.01). These observations suggest that increased release of toxic oxygen metabolites from polymorphonuclear leukocytes in diabetic subjects may play a role in the development of diabetic angiopathies.
Response to Growth Hormone Treatment and Final Height after Cranial or Craniospinal Irradiation
Authors: SULMONT V. , BRAUNER R. , FONTOURA M. , RAPPAPORT R.
Published: January 2008

ABSTRACT. Growth hormone (GH) deficiency (GHD) induced by cranial irradiation has become a frequent indication of hGH substitutive therapy. This study analyses the growth response to hGH therapy and the factors involved in the decrease in growth velocity observed after cranial irradiation. One hundred children (61 boys and 39 girls) given cranial radiation for pathology distant from the hypothalamo‐pituitary area were studied. Fifty‐six of them received hGH therapy for GHD resulting in decreased growth velocity. The initial annual height gain in the cranial‐irradiated group was comparable to that of patients treated for idiopathic GHD; additional spinal irradiation significantly reduced the growth response. Twenty‐eight hGH‐treated patients reached final heights which were compared to those of 2 untreated irradiated groups, one with GHD (n=27) and the other with normal GH secretion (n= 17). The height SD score changes observed in hGH therapy were +0.3 in the cranial (n=10) and ‐ 1.2 SD in the craniospinal (n = 18) groups. GH deficiency had contributed to a mean height loss of 1 SD and spinal irradiation to a loss of 1.4 SD. The small effect of hGH therapy on final height is probably linked to the small bone age retardation at onset of hGH therapy and to the fact that irradiated children entered puberty at a younger age in terms of chronological age (10.6±0.3 yr in girls and 11.0± 0.3 yr in boys) and bone age (9.6 ± 0.4 yr in girls and 12.6 ± 0.3 in boys) than the idiopathic GHD patients. These data suggest that the results of hGH therapy in irradiated children might be improved with higher and more fractionated hGH doses and, in some patients, by delaying puberty using luteinizing hormone releasing hormone analogs.
Ventricular Tachycardia Associated with Coxsackie B4 Virus Infection
Authors: TUBMAN T. R. J. , CRAIG B. , MULHOLLAND H. C.
Published: January 2008

ABSTRACT. We describe a case of ventricular tachycardia in association with acute Coxsackie B4 virus infection occurring in an otherwise normal infant. The dysrhythmia responded to flecainide acetate.
Early Treatment of Idiopathic Respiratory Distress Syndrome Using Binasal Continuous Positive Airway Pressure
Authors: KAMPER J. , RINGSTED C.
Published: January 2008

ABSTRACT. During a 3‐year period (1979‐81) 85 premature infants with idiopathic respiratory distress (IRDS) were treated early with an easily applicable light‐weight CPAP‐system with a binasal tube and a gas jet. We used conservative criteria for ventilator treatment. The treatment proved sufficient in 18 out of 25 infants with a birth weight > 1500 g and in 53 out of 60 infants with a birth weight > 1500 g. Seven infants developed pneumothorax during CPAP treatment. Seventy‐four infants survived, all without bronchopulmonary dysplasia. At the age of 1.5‐4.5 years the incidence of respiratory tract infections did not differ from that in a group of siblings; and the incidence of lower respiratory tract infections was low compared to previous studies. With the criteria used, early CPAP proved effective in the majority of infants with IRDS.
Renal Biopsy and Family Studies in 65 Children with Isolated Hematuria
Authors: SCHRÖDER C. H. , BONTEMPS C. M. , ASSMANN K. J. M. , STEKHOVEN J. H. SCHUURMANS , FOIDART J. M. , MONNENS L. A. H. , VEERKAMP J. H.
Published: January 2008

ABSTRACT. We have investigated 65 children with isolated hematuria persisting for at least a year. Renal biopsy specimens were studied by light microscopy, electron microscopy and immunofluorescence with antisera specific against basement membrane components. The majority of the biopsies (62/65) showed variable histologic abnormalities. Four categories could be distinguished on combined histological and clinical criteria: Alport syndrome (n=8), benign hematuria (n=33, familial in 23), IgA nephropathy (n = 16) and increase in mesangial cells and matrix (n=5). On the basis of our results, we suggest that a renal biopsy can establish diagnosis and prognosis in those children with isolated hematuria where the family history is negative. If the family has adult male individuals with isolated hematuria, a biopsy can usually be avoided, since this family history effectively excludes Alport syndrome. The use of antisera against basement membrane components did not allow a differentiation between Alport syndrome and benign hematuria. Goodpasture serum immunofluorescence was variable in the former and normally present in the latter.
Two‐Year Results of Treatment with Methionyl Human Growth Hormone in Children with Turner Syndrome
Authors: RONGEN‐WESTERLAKEN C. , FOKKER M. H. , WIT J. M. , KEIZER‐SCHRAMA S. M. P. F. DE MUINCK , OTTEN B. J. , OOSTDIJK W. , WAAL H. A. DELEMARRE‐V. D. , GONS M. H. , BOT A.
Published: January 2008

ABSTRACT.Methionyl growth hormone (somatrem) in a daily dosage of 4 IU/m2body surface area was administered to 16 girls with Turner syndrome. Low dose ethinyl estradiol (0.1 μg/kg body weight) was added in girls aged 13 years or more. Mean (SD) height velocity increased from 3.4 (0.9) to 7.2 (1.7) and 5.3 (1.3) cm/year in the first and second year, respectively. Bone age advanced 1.8 years over 2 years and predicted adult height was increased. Apart from the occurrence of anti‐GH antibodies there were no side effects. In conclusion, somatrem is an efficacious and safe therapy for short stature in Turner syndrome over a period of 2 years. Longer follow‐up is needed before conclusions about its effect on final height can be drawn.
Atypical Characteristics of a Group of Children with Intussusception
Authors: REIJNEN J. A. M. , FESTEN C. , JOOSTEN H. J. M. , WIERINGEN P. M. V. van
Published: January 2008

ABSTRACT. We describe the characteristics of a group of 140 children with intussusception. They differed strikingly from the classical picture of intussusception given in textbooks and in publications concerning large series. We found a low incidence of intussusception especially in infants and young children. There were far more children of older ages, the delay in diagnosis was significantly longer and there was a very high percentage of leading points. These factors explained our low hydrostatic reduction rate. The weight of most children was under the fiftieth percentile. The percentage of small bowel intussusceptions was somewhat higher. These differences are important in the light of early diagnosis and evaluating the results of treatment.
Clinical and Biochemical Outcome of Marrow Transplantation for Gaucher Disease of the Norrbottnian Type
Authors: ERIKSON ANDERS , GROTH CARL GUSTAV , MÅNSSON JAN‐ERIC , PERCY ALAN , RINGDÉN OLLE , SVENNERHOLM LARS
Published: January 2008

ABSTRACT. Three children, two girls and one boy, with Gaucher disease of the Norrbottnian type were treated by allogeneic bone marrow transplantation (BMT). Two of the donors were heterozygotes for Gaucher disease, and the third did not carry the gene. Engraftment was achieved in all three children but the boy became a chimera. The children have been followed from 1.5 to 6 years after transplantation. In each case the outcome was favourable, and in two children (patients 1 and 3) the BMT has been life saving. In all three cases a positive biochemical effect was apparent. Almost all biochemical parameters normalized within one year. The authors conclude that BMT is the treatment of choice in severe cases of Gaucher disease. BMT should be considered early in the course of disease and vigorous efforts made to find a suitable donor.
Magnetic Resonance Imaging of Central Nervous System Vasculitis A Case Report of Henoch‐Schönlein Purpura
Authors: ELINSON PHYLLIS , FOSTER KENDALL W. , KAUFMAN DONALD B.
Published: January 2008

ABSTRACT. A case of Henoch‐Schlönlein purpura (HSP) with central nervous system vasculitis diagnosed by MRI is discussed.
Trisomy 22 Mosaicism Limited to Skin Fibroblasts in a Mentally Retarded, Dysmorphic Girl
Authors: LUND H. T. , TRANEBÆERG L.
Published: January 2008

ABSTRACT. Less than 10 cases of trisomy 22 mosaicism have been reported. The patients have clinical features in common with both Down's and Turner's syndromes. We report on a 12‐year‐old girl, in whom the GFR‐stimulated gonadotropine response suggested ovarian dysgenesis, although ovarian structures could be demonstrated by ultrasound. The diagnosis of trisomy 22 mosaicism was established by chromosome analysis of cultured skin fibroblasts following a negative lymphocyte culture.
Suboptimal Mineral Composition of Cow's Milk Formulas: A Risk Factor for the Development of Late Metabolic Acidosis
Authors: KALHOFF H. , MANZ F. , DIEKMANN L. , STOCK G. J.
Published: January 2008

ABSTRACT. Late metabolic acidosis was observed in a term baby boy with renal tubular acidosis type 4 who received two cow's milk formulas in succession. Suboptimal mineral composition of the formulas turned out to be an important risk factor for the development of late metabolic acidosis.