bluuebunny/crossref_metadata_2025 huggingface dataset:
INCREASED IMMUNOREACTIVE PLASMA AND URINARY GROWTH HORMONE IN GROWTH RETARDATION WITH DEFECTIVE GENERATION OF SOMATOMEDIN A (LARON'S SYNDROME)
Authors: KASTRUP K. W. , ANDERSEN H. , HANSSEN K. F.
Published: January 2008

ABSTRACT: Kastrup, K. W., Andersen, H. and Hanssen, K. F. (Childrens Hospital, Fuglebakken, and Steno Memorial Hospital, Copenhagen, Denmark). Increased immunoreac‐tive plasma and urinary growth hormone in growth retardation with defective somatomedin A generation (Laron's syndrome). Acta Paediatr Scand 64: 613, 1975.–In a boy 4 years old with clinical hypopituitary dwarfism, high plasma and urinary levels of immunoreactive growth hormone were found. Somatomedin A levels in serum were low and failed to respond after short‐term treatment with human growth hormone. The parents were first cousins. In the arginine and insulin tolerance tests the initially high immunoreactive growth hormone levels were later followed by a decrease to high normal values. Insulinopenic response was present during the arginine and glucose tolerance tests. As a growth hormone molecule defect is not found in these patients and no growth or other metabolic response to exogenous HGH can be demonstrated, it is concluded that a defective somatomedin generation may be present, probably in conjunction with a generalized receptor defect and deficient feedback system with abnormal release of HGH. The lack of somatomedin A is responsible for the severe growth retardation and the disturbance in carbohydrate metabolism is probably caused by sustained high growth hormone levels.
SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY
Authors: GRöBE H. , BASSEWITZ D. B. v. , DOMINICK H.‐CHR. , PFEIFFER R. A.
Published: January 2008

ABSTRACT: Gröbe, H., v. Bassewitz, D. B., Dominick, H.‐Chr. and Pfeiffer, R. A. (Departments of Paediatrics and of Medical Cell Biology, University of Münster, Münster, and Institute of Human Genetics, Medical School, Lübeck, BRD). Subacute necrotizing en‐cephalomyelopathy. Clinical, ultrastructural, biochemical and therapeutic studies in an infant. Acta Paediatr Scand, 64: 755, 1975.–Subacute necrotizing encephalomyelopathy (SNE) has been observed in an infant with regressing psychomotor development. The concentrations of alanine, pyruvate and lactate were increased in the serum and blood as well as in the cerebrospinal fluid. Pyruvate carboxylase activity was reduced in the liver tissue. An inhibitor of thiamine‐pyrophosphate‐ATP‐phosphotransferase was present in the urine. Thiamine treatment was followed by a decrease of serum alanine and blood pyruvate and lactate, but there was no clinical improvement during a period of 17 months. Ultrastructural investigations revealed high glycogen levels in liver tissue and skeletal muscle. These findings contrast with decreased gluconeogenesis, which is suggested by the diminished pyruvate carboxylase activity. Therefore it is concluded that reduced hepatic pyruvate carboxylase activity is not the primary cause of SNE.
MENARCHEAL AGE IN NORTHUMBERLAND
Authors: ROBERTS D. F. , DANSKIN M. J. , CHINN S.
Published: January 2008

ABSTRACT: Roberts, D. F., Danskin, M. J. and Chinn, S. (Department of Human Genetics, University of Newcastle upon Tyne and MRC Clinical Research Centre, Harrow, England). Menarcheal age in Northumberland. Acta Paediatr Scand, 64: 845, 1975.–A second survey of age at menarche in north‐east England, how it is affected by family environment and how it affects physique, was carried out in 1969‐70 on a large sample of schoolgirls in the suburbs of Newcastle. Age at menarche shows no independent effect of social class or of position in sibship, but is strongly influenced by the size of family in which a girl grows up. Menarche is associated with pronounced increments in height and weight, in the presence of which no consistent effects of variables of the family environment on physique can be clearly identified. The results are very similar to those from the first study in South Shields, County Durham.
19. NEW APPROACHES TO THE DELIVERY OF HEALTH CARE TO ADOLESCENTS
Authors: MILLAR HILARY E. C.
Published: January 2008

ABSTRACT

Adolescents are a new population group showing certain common characteristics which transcend the confines of geography, economics, education, culture and race. Certain health problems have emerged which are closely related to the life style of teenagers. These health needs are not met by existing health care delivery systems, and future planning must take into account the morals of young people. At present there is no unified approach to the development of health care programs for adolescents, but important explorations of effective methods are taking place in a fragmentary way. The challenge is to provide the necessary technology and professional expertise in an accessible setting and then to weld these services into programs which will become cohesive and stable.

Analyses of data show that adolescents seek help more often for primary and preventive care than for serious ilnesses. Services are particularly needed for addictive problems, emotional disorders, suicidal states, and conditions related to sexual activity. Consideration of the effect of adolescent behavior on the reproductive cycle is of the utmost importance. These sequelae of conception and veneral disease can be extremely serious for the immature girl and her baby. Therefore the opportunity for birth control, health education, abortion and prenatal care for teenagers should be priority goals in any program for adolescents. The provision of services for the young mother and her baby should be included in the overall plan.

New approaches in the ambulatory care of adolescents include an age‐specific operation, satellite clinics with hospital backup, and the inclusion of young people in the planning of services. Care should be comprehensive and continuous, and a multi‐disciplinary staff team would permit a more effective approach. The involvement of other teenagers as assistants in counseling has been found an effective method of communication; this relationship may bridge the generation gap when highly personal experiences must come to the surface.

Financial and legal barriers should be removed so that care is available to all. Social systems which affect the adolescent should coordinate their activities at all levels. Examples of some innovative methods are available. The ideal approach to health care delivery for adolescents is not yet established; but it must obviously do more than merely cope with a series of crisis oriented episodes. A recognition of underground self‐help methods is needed, even if in the last analysis these methods are found to be irrelevant and are discarded. The health of teenagers automatically and immediately affects the wellbeing of the following generation. No effort to improve the situation should be spared.
MYOCARDIOPATHY IN DUCHENNE PROGRESSIVE MUSCULAR DYSTROPHY
Authors: LETH A. , WULFF K.
Published: January 2008

Abstract. In a retrospective study of hospital and autopsy records of 19 male subjects with the Duchenne type of progressive muscular dystrophy the incidence of cardiac involvement was found to be 84%. Patients with dystrophic involvement of the heart almost invariably develop heart failure; an early sign may be persistent tachycardia and, possibly, electrocardiographic changes, in case of which institution of digitalis treatment should be considered. Cardiac and pulmonary complications were equally frequent causes of death (42%) but, death from cardiac complication occurred only among the patients with dystrophic involvement of the myocardium. It is concluded that patients with Duchenne progressive muscular dystrophy very often develop cardiac complications, and when relating the available information on treatment to the autopsy findings it should be stressed that early and intensive therapy of the cardiac symptoms is of the greatest importance to the patient.
HEMOGLOBIN‐OXYGEN AFFINITY IN HYPOPHOSPHATEMIC RICKETS
Authors: MUNK PAUL , FREEDMAN MELVIN H. , GREENBERG MARK L. , LEVISON HENRY
Published: January 2008

Abstract. In 14 patients with simple X–linked hypophosphatemic rickets, 5 were below the third percentile in height and 9 were between the third and twenty‐fifth percentile. Although the mean serum inorganic phosphorus level was only 2.01±0.65 (normal range for all age groups is 3.8 to 6.0 mg/100 ml), both the mean values for red cell 2,3‐diphosphoglycerate (2,3‐DPG) and adenosine triphosphate (ATP) were normal at 4.78±1.23 and 1.02±0.17 μmol/ml of red blood cells respectively. Moreover, the mean P50 value was normal at 26.4±0.9 mmHg. These normal oxygen transport data make unlikely any proposal that short stature seen in these patients is secondary to chronic tissue hypoxia. They also indicate that the intra‐erythrocytic organic phosphate levels are maintained at normal levels despite profound chronic hypophosphatemia.
THE SYNDROME OF RENAL TUBULAR ACIDOSIS WITH NERVE DEAFNESS
Authors: DONCKERWOLCKE R. A. , VAN BIERVLIET J. P. , KOOREVAAR G. , KUIJTEN R. H. , VAN STEKELENBURG G. J.
Published: January 2008

Abstract. Two brothers with renal tubular acidosis and nerve deafness are described. Studies of the physiopathological characteristics of the renal acidification defect show that the defect is limited to the distal tubule. Renal tubular acidosis with nerve deafness is a distinct nosologie entity that is determined by an autosomal recessive trait.
PREMATURE ADRENARCHE IN IDENTICAL TWINS
Authors: BARTSOCAS CHRISTOS S. , DANON MARCO , CRAWFORD JOHN D.
Published: January 2008

Abstract. Monozygotic female twins with premature adrenarche are reported. Endocrine work‐up excluded the existence of an endocrinopathy or a tumor.
SPONTANEOUS CEREBRAL THROMBOSIS IN CHILDREN Report of Two Cases
Authors: LILLQUIST K. B. , INGSTRUP H. M.
Published: January 2008

Abstract. Cerebral arterial occlusions are rare in children, but they are demonstrated with increasing frequency as angiographic examination of children with acute hemiplegia becomes a routine procedure. Two cases of idiopathic cerebral thrombosis in boys, aged 6 ½ and 5 years, involving the basilar artery and the middle cerebral artery, respectively, are presented. The clinical findings supplemented by angiography confirmed the diagnosis.
UNSTABLE‐HAEMOGLOBIN HAEMOLYTIC ANAEMIA
Authors: BROSTRØM KARIN
Published: January 2008

Abstract. A 6‐year‐old girl suffering from chronic haemolytic anaemia with crisis induced by drugs and infections is described. The disorder was diagnosed as an unstable‐haemoglobin haemolytic anaemia. The heat stability test, simple and specific for unstable haemoglobins is mentioned, it should be done in every child suffering from unexplained chronic haemolytic anaemia. The haemoglobin variant is also known in a patient in Brazil as Hb Niteroi.
HYPEROSMOLALITY AND INTRAVENTRICULAR HAEMORRHAGE IN PREMATURE BABIES
Authors: THOMAS D. B.
Published: January 2008

Abstract. Serial investigations of electrolytes, osmolality and nutritional status were undertaken in 28 babies less than 33 weeks gestation. 6 died with intraventricular haemorrhage, 9 died without intraventricular haemorrhage, and 13 survived. Significant hyperosmolality was detected in the babies who died with intraventricular haemorrhage, whereas babies who died without intraventricular haemorrhage and survivors had values within normal limits. No relation was found between hyperosmolality and sodium concentrations. Total fluid intake and caloric intake were comparable in the three groups, but protein intake was much reduced in babies with intraventricular haemorrhage due to a lower milk intake. The hyperosmolality could have resulted from tissue breakdown following protein starvation and may be a factor in the occurrence of intraventricular haemorrhage. In premature babies an osmolality of over 320 mOsm/1 carried a grave prognostic implication.
THE EFFECT OF FEEDING ON OXYGEN CONSUMPTION, RQ AND PLASMA LEVELS OF GLUCOSE, FFA, AND D‐β‐HYDROXYBUTYRATE IN NEWBORN INFANTS OF DIABETIC MOTHERS AND SMALL FOR GESTATIONAL AGE INFANTS
Authors: GENTZ J. , KELLUM M. , PERSSON B.
Published: January 2008

Abstract. Eight infants of strictly controlled diabetic mothers (IDM), 8 infants of gestational diabetic mothers (IGDM) and 6 small for gestational age infants (SGA) were studied before the first feeding and during an early feeding regimen. In IDMs and IGDMs continuous monitoring from 2 hours up to 7 1/2 hours after birth before feeding revealed no consistent changes of Vo2 and RQ. The groups of infants were studied on 4 different occasions: (I) 2 to 16 hours, (II) 1 to 2 days, (III) 3 to 4 days, and (IV) 7 to 11 days. Prefeeding Vo2‐values were not significantly different between each of the groups, but mean RQ was higher in IGDMs than in IDMs. Age of the infant and prefeeding RQ were inversely correlated (r=‐0.537, p<0.02). With increasing age and milk intake Vo2 increased significantly in all groups. RQ decreased during the first 24 to 48 hours in all groups and rose thereafter with highest values at 7 to 11 days. Plasma levels of glucose, FFA, and D‐β‐hydroxybutyrate were not significantly different between each of the groups. The highest values for D‐β‐hydroxybutyrate were found at 1–2 days when the lowest RQ values were also recorded. D‐β‐hydroxybutyrate concentrations and RQ values (r= ‐0.648, p<0.001) were inversely correlated suggesting increasing oxidation of fat. Feeding resulted in a marked rise in RQ to values around unity, which preceded a distinct increase in Vo2 that reached a maximum at 1 to 1 1/2 hours after the feed, then slowly returned to pretest values. The rise in Vo2 was accompanied by an increase in rectal temperature (0.4 to 1.5°C). Vo2, RQ, and plasma levels of glucose, FFA, and D‐β‐hydroxybutyrate, were almost identical for each of the groups. We suggest: 1) That differences in feeding practice is the most likely explanation for the discrepancy between reported values for Vo2, RQ, and circulating substrates in normal and low birth weight newborns. 2) That the rise in Vo2 during the neonatal period, caused by feeding, reflects the cost of growth.
AORTIC ANEURYSM DEVELOPING AFTER UMBILICAL ARTERY CATHETERIZATION
Authors: RAJS JOVAN , FINNSTRÖM ORVAR , WESSTRÖM GÖRAN
Published: January 2008

Abstract. A variety of complications of umbilical vessel catheterization in the newborn have been reported by earlier investigators. This paper describes a case in which umbilical artery catheterization probably gave rise to an aortic aneurysm. The histological findings suggested that the hypoxic changes in the aortic wall as well as the mechanical effect of the catheter were involved in the origin of the aneurysm.
SERUM ALPHA‐FETOPROTEIN LEVELS IN EXTRAHEPATIC BILIARY ATRESIA, IDIOPATHIC NEONATAL HEPATITIS AND ALPHA‐1‐ANTITRYPSIN DEFICIENCY (PiZ)
Authors: JOHNSTON D. I. , MOWAT A. P. , ORR HILARY , KOHN J.
Published: January 2008

Abstract. Serum alpha‐fetoprotein levels were measured using a sensitive radioimmunoassay in 77 infants presenting with persistent conjugated hyperbilirubinaemia. A broad range of alpha‐fetoprotein concentrations occurred in both the 23 infants with extrahepatic biliary atresia and the 35 with idiopathic neonatal hepatitis but the 13 with alpha‐1‐antitrypsin deficiency had uniformly low levels. High alpha‐fetoprotein concentrations (above 10 000 μg/I) favoured the diagnosis of neonatal hepatitis especially in the first ten weeks of life, but the overlap between neonatal hepatitis and extrahepatic biliary atresia was large and alpha‐fetoprotein determination cannot be recommended as a reliable method for distinguishing the two conditions. Serial alpha‐fetoprotein values showed no consistent relationship with standard liver function tests and gave no guide to prognosis. There was an association between alpha‐fetoprotein production and needle biopsy evidence of hepatic giant cell transformation. The uniformly low alpha‐fetoprotein levels in alpha‐1‐antitrypsin deficient infants with neonatal hepatitis is a new observation and possible mechanisms for disordered glycoprotein release are discussed.
EPIDEMIOLOGY OF CORNELIA DE LANGE'S SYNDROME
Authors: BECK BENTE
Published: January 2008

Abstract. A prevalence investigation of Cornelia de Lange's syndrome in Denmark is presented. The patients were traced by screening all institutionalized mentally retarded patients, patients in schools and kindergartens for imbecile patients and finally by getting information on Cornelia de Lange patients known to pediatric departments. In this way 24 patients, 10 men and 14 women, were found. This amounts to a population prevalence of 0.5/100 000. Clinical data, histories of the patients and genealogical data are presented by means of tables. The eldest patient was 49 years old, but 75 % of the patients were younger than 20 years. Two of the probands were sibs. Another 2 sibs were registered as mentally retarded without specific syndromes. One case of consanguinity among parents was found. The mode of ascertainment is discussed and it is concluded that the present investigation presents a minimum prevalence figure. Four patients are presented who for various reasons were not available during the prevalence investigation proper. A prevalence figure of 0.6/100 000 is found if these 4 patients are included in the calculations. One of the last mentioned patients represented a familial case. The patient in question was a girl with a younger half‐brother, the mother in common, both children being very typical cases of Cornelia de Lange's syndrome.
GENERAL HEALTH SCREENING OF FOUR‐YEAR‐OLDS IN A SWEDISH COUNTY IV. An Analysis of the Effectiveness of the Psychological Examination Program
Authors: NILSSON C. , SUNDELIN C. , VUILLE J.‐C.
Published: January 2008

Abstract. Since 1969, 4‐year‐olds in the County of Uppsala have been offered extensive health screening at the Child Health Centres. In 1969 and 1970 a total of 3810 children underwent the screening procedure. The psychological screening instrument consisted of a questionnaire (to be answered by the parents), an interview of the parents, a psychomotor examination and an observation of the child's behaviour. 156 (4.1%) 4‐year‐olds were referred to a specialist team for investigation and decision concerning treatment. In the present study the effectiveness of the psychological screening instrument was assessed by its sensitivity, specificity and predictive value. The sensitivity was found to amount to a maximum of 0.73. The specificity was estimated to be 0.98–0.99, and the positive predictive value to 0.67. The authors consider the effectiveness to be sufficiently high to motivate continuation of the screening procedure for detection of psychological problems in pre‐school age. Efforts should be made, however, to further improve the effectiveness.
MEASLES VACCINATION
Authors: NORRBY ERLING , LAGERCRANTZ RUTGER
Published: January 2008

ABSTRACT. Children immunized with 4 doses of formalin‐inactivated vaccine and/or purified hemagglutinin prepared from Tween 80‐ether (TE) treated material were subjected to a follow‐up 8–9 years after the last dose of vaccine. 11 out of 27 children had clinical and/or serological signs of infections with wild measles virus during the 8 to 9 years post‐booster period. 10 out of the 11 children with infections had non‐hemagglutinating‐in‐hibiting (HI) hemolysis‐inhibiting (HLI) antibodies demonstrable hi their sera after removal of HI antibodies by absorption with TE antigen. In contrast 13 out of 16 vaccinees without detectable signs of infection lacked non‐HI HLI antibodies. 10 out of these 13 children were vaccinated with further attenuated live measles virus. There were no clinical reactions to vaccination. 4 vaccinees with low pre–vaccination HI antibody titers showed significant rises of antibody titers including non‐HI HLI antibodies. In the remaining children no take of the live vaccine could be demonstrated. Thus HI antibodies of a certain minimal concentration can block the replication of vaccine virus even in the absence of non‐HI HLI antibodies. However, since it will be difficult to establish these conditions by use of available inactivated vaccines it is recommended that future vaccine products should include both major virus envelope surface components, the hemagglutinin and the hemolysin.
EXPERIENCES ON THE USE OF DIPROPYLACETATE IN THE TREATMENT OF CHILDHOOD EPILEPSY
Authors: SILLANPÄÄ MATTI , DONNER MÄRTA
Published: January 2008

ABSTRACT. The antiepileptic efficiency of dipropylacetate (DPA) was studied in 80 epileptic children who suffered either from seizures resistant to previous medication, so‐called idiopathic epilepsy or progressive myoclonus epilepsy. The average peroral daily dosage of DPA was 21 mg/kg divided into 2 to 3 doses. DPA was most effective in cases with 1) so‐called idiopathic epilepsy (genuine petit mal, photogenic epilepsy, myoclonic petit mal in older children), 2) seizures beginning in later childhood years, 3) photosensitive and hyperventilation‐sensitive seizures, 4) EEG showing symmetric generalized spike‐slow wave complexes provoked by photostimulation, 5) EEG without generalized disturbance or focal findings, 6) normal neurological and mental status, 7) progressive myoclonus epilepsy. DPA may thus be recommended for the treatment of the above‐mentioned cases and, due to good treatment results, also tried in other types of epilepsy.
TISSUE TOXICITY OF INTRAVENOUS SOLUTIONS A Phlebographic and Experimental Study
Authors: ENGER E. , JACOBSSON B. , SÖRENSEN S.‐E.
Published: January 2008

ABSTRACT. A phlebographic study in 32 children has shown that i.v. infusion of invertose, glucose, Vamine and Intralipid but not saline, damages the tissues as judged from changes in the vessel walls, oedema and disturbances in the venous circulation. The addition of Heparin to the solutions markedly reduced the frequency of these untoward reactions. The toxicity of invertose, glucose, Vamine and Intralipid was studied experimentally in a biologic tissue: the cheek pouch of the hamster. All solutions particularly Vamine caused some damage to the microcirculation. In this study the factor mainly responsible for thrombophlebitis was the toxicity of the solution infused. Other factors such as surgical trauma, site of entry, local infection, temperature of solutions etc. played only a minor role.
MASSIVE CHRONIC FETO‐MATERNAL BLEEDING ASSOCIATED WITH PLACENTAL CHORIOANGIOMAS
Authors: SIMS D. G. , BARRON S. L. , WADEHRA V. , ELLIS H. A.
Published: January 2008

ABSTRACT. After pregnancy complicated by polyhydramnios and the antenatal discovery of a very large placenta a newborn infant suffered from anemia, thrombocytopenia and hypoproteinemic edema, and was successfully treated by exchange transfusion. The placenta contained two chorioangiomas and there was diffuse placental hypertrophy with edema and patchy chorioangiomatosis. There was evidence of major chronic feto‐maternal bleeding which could be the explanation for most of the hematological and biochemical problems which occurred. The child was developing normally at subsequent follow‐up aged 15 weeks.
CHRONIC ACTIVE HEPATITIS IN CHILDREN A Clinical and Immunological Long‐term Study
Authors: LIDMAN K. , BIBERFELD G. , STERNER G. , NORBERG R.
Published: January 2008

Abstract Six girls and one boy with chronic active hepatitis (CAH) of unknown etiology were between 9 and 15 years at the clinical onset of their illness. After beginning immunosuppressive therapy the course of their disease was followed from one to ten years. All had markedly increased IgG, high titres of smooth muscle antibodies (SMA) and antinuclear antibodies of IgG class in the earliest serum specimens tested. Therapy resulted in an improved sense of well‐being and a decrease in SGOT, IgG and titres of SMA. Very high titres of measles antibodies were observed in all cases. In one of the cases CAH manifested itself after measles and in another after rubella infection. The first case in our series of patients died of liver failure after 5 years of illness. The other patients have survived and are able to live a normal life. The possibility of CAH should be considered when children develop symptoms of hepatitis. Long‐term immunosuppressive treatment with regular clinical and laboratory evaluation is important. Estimation of titres of SMA is an additional parameter of value in following of the activity of CAH in these young patients.
MEDIUM CHAIN TRIGLYCERIDES FOR TREATMENT OF SPONTANEOUS, NEONATAL CHYLOTHORAX Lipid Analysis of the Chyle
Authors: PEITERSEN B. , JACOBSEN B. BROCK
Published: January 2008

Abstract Volume and contents of lipid and protein in the pleural fluid from a three weeks old girl with spontaneous chylothorax were studied (a) during parenteral, nonfatty nutrition and later (b) during administration of a formula (Biosorbin®) containing medium chain triglycerides (MCT). The pleural fluid production could not be correlated to the treatment employed but suddenly ceased after 20 days management. Triglyceride and total esterified fatty acid concentrations in pleural fluid were high on admission when feeded with human milk, and chylomicrons and other lipoproteins were present in the chyle. During parenteral treatment a pronounced decrease in pleural fluid concentrations of triglyceride and total fatty acids occurred concomitant with a disappearance of the chylomicrons. During the following MCT diet a pronounced increase in triglyceride and total fatty acids concentrations appeared and the chylomicrons reappeared in the chyle. The cholesterol and phospholipid concentrations in the pleural fluid showed only small changes during the different treatments. No significant changes in protein and albumin concentrations of chyle were observed. It is concluded, that administration of the Biosorbin® MCT formula, containing 871/2% of the fat as MCT, seems without value in the treatment of spontaneous, neonatal chylothorax.
CORD BLOOD PORPHYRINS A Quantitative Assay in Newborn Infants
Authors: HANDA F. , SHARMA S. C.
Published: January 2008

Abstract. A quantitative assay of free erythrocyte porphyrins was done in umbilical cord blood of 30 newborn babies by a spectrophotometry method. The mean value of free erythrocyte protoporphyrin in umbilical cord blood was found to be 74.58 μg/100 ml packed erythrocytes and that of free erythrocyte coproporphyrin to be 0.77 μg/100 ml packed erythrocytes with a standard deviation of ±29.99 and ±1.48 respectively. Relationship between free erythrocyte protoporphyrin and free erythrocyte coproporphyrin and their correlations with birth weight and sex of the newborn baby were found to be statistically significant.
ENDOCRINE ASPECTS OF TRISOMY 4p
Authors: GIOVANNELLI G. , BERNASCONI S. , FORABOSCO A.
Published: January 2008

Abstract. The main endocrinological parameters were investigated in two sisters affected with trisomy 4p. Our findings rule out any impairment of the endocrine system in this rare syndrome, even in those cases in which stunting of growth is more pronounced. The marked weight deficit in one of the two patients had no relationship to the chromosomal anomaly; it was determined by the association of a deficit of immunoglobulin with a Giardia Lamblia infestation.
EFFECT OF AGE ON HUMAN ADIPOSE TISSUE METABOLISM AND HORMONAL RESPONSIVENESS
Authors: NYBERG G. , HÄGER A. , SMITH U.
Published: January 2008

Abstract. The effects of age and fat cell size on the metabolism of white human adipose tissue were analysed independently. Abdominal subcutaneous adipose tissue specimens were obtained from children varying in age from 0–15 years and from adults (mean age±S.D.; 38.2±14.1 years). The basal rates of lipolysis and glucose incorporation into lipids were considerably higher in children than in adults even when differences in fat cell size had been taken into account. Lipolysis in response to a maximal concentration of noradrenalin was higher in fat cells from children. However, on a percentage basis the responsiveness to this agent was similar in children and adults. Irrespective of age, glucagon did not elicit a lipolytic response. Thus, it does not seem that the increased lipolysis known to occur in vivo in the neonatal period is due to a direct effect of glucagon on white adipose tissue. However, the overall lipolytic capacity is increased in fat cells from children.
FACTORS RELATED TO EARLY TERMINATION OF BREAST FEEDING A Retrospective Study in Sweden
Authors: SJÖLIN S. , HOFVANDER Y. , HILLERVIK C.
Published: January 2008

Abstract. In 1972, 298 mothers representative of the city of Uppsala in general breast fed their babies for only a brief period of time despite a clear wish to continue—36% up to 8 weeks. The most common reason for terminating breast feeding was that the “milk dried up” (66%). More precise reasons commonly mentioned were anxiety of all kinds, lack of motivation, stress, tiredness and work outside the home. It was further found that mothers who enjoyed their breast feeding, were well educated, were older than 25 years and belonged to social class 1, tended to breast feed longest.
CARBAMYL‐PHOSPHATE‐SYNTHETASE DEFICIENCY WITH NEONATAL ONSET OF SYMPTOMS
Authors: FARRIAUX J. P. , PONTE CI. , POLLITT R. J. , LEQUIEN P. , FORMSTECHER P. , DHONDT J. L.
Published: January 2008

Abstract. The clinical course and biochemical findings in a case of carbamyl‐phosphate‐synthetase deficiency are described. The patient, a boy, presented 48 h after birth with rapidly developing hypotonia and hypothermia. Pulmonary haemorrhage, melaena and haematemesis ensued and despite ventilatory assistance and peritoneal dialysis the patient died on the fifth day. A virtual absence of carbamyl phosphate synthetase I (N‐acetylglutamate dependent) was proved by analysis of tissue samples removed post mortem. Other urea cycle enzymes were normal.
CELL‐MEDIATED IMMUNITY AND IMMUNOGLOBULIN LEVELS IN LIGHT‐FOR‐DATE INFANTS
Authors: BHASKARAM C. , RAGHURAMULU N. , REDDY VINODINI
Published: January 2008

Abstract. Cell‐mediated immune response and circulating levels of immunoglobulins were studied in 75 full‐term newborn babies. The results showed that though the immunoglobulin levels were not altered, cell‐mediated immunity was significantly depressed in infants with birth weight less than 2500 g and this may result in lowered resistance to infection.
THE LARSEN SYNDROME AND GLIAL PROLIFERATION IN THE BRAIN
Authors: HENRIKSSON PER , IVARSSON STEN , THEANDER GEORG
Published: January 2008

Abstract. A case of the Larsen syndrome is reported in which postmortem examination of the brain revealed glial proliferation resembling tuberous sclerosis. The differential diagnosis of the syndrome and the possible significance of the lesions are discussed.
ANTIBODY RESPONSE IN MARASMIC CHILDREN DURING RECOVERY
Authors: AWDEH Z. L. , KANAWATI A. K. , ALAMI S. Y.
Published: January 2008

Abstract. The I, II and III antibody responses to tetanus and diphtheria were assessed in marasmic and moderate protein‐calorie malnourished children and compared to that of controls. The results suggest that during recovery marasmic children are capable of responding adequately to tetanus and diphtheria vaccines.
URINARY HYPOXANTHINE, XANTHINE AND URIC ACID EXCRETION IN NEWBORN INFANTS WITH PERINATAL COMPLICATIONS
Authors: MANZKE H. , DÖRNER K. , GRÜNITZ J.
Published: January 2008

Abstract. The concentration of hypoxanthine, xanthine and uric acid in the first 24‐h urine of 105 newborn infants was measured densitometrically by thin‐layer chromatography. 45 of them had moderate or severe perinatal complications. Among these newborns, 26 infants with perinatal complications (58 %) and 4 infants without perinatal complications (7 %) had an elevated urinary excretion rate of hypoxanthine. Urinary xanthine was not increased, uric acid was slightly higher in the group of infants with perinatal complications. It seems that a crucial mark is involved, if the rate of hypoxanthine exceeds 15% of the total urinary oxypurine excretion, or, if related to urinary creatinine, more than 0.075 μmol hypoxanthine/μmol creatinine. Apparently, with hypoxic newborns increased values of urinary hypoxanthine excretion can be used to quantify the lack of oxygen retrospectively.
MONOZYGOTIC TWINS CONCORDANT FOR TRACHEO‐ESOPHAGEAL FISTULA AND DISCORDANT FOR THE VATER ASSOCIATION
Authors: KING SUSANNE L. , LADDA R. L. , SHOCHAT S. J.
Published: January 2008

Abstract. Monozygotic female twins were concordant for tracheo‐esophageal fistula (TEF); and one twin also manifested other anomalies of the VATER association, including agenesis of the female reproductive tract. Review of the literature discloses a familial tendency for recurrence of TEF and a high concordance rate in monozygotic twins, indicating a significant genetic influence for the isolated anomaly. In the case of the VATER association, the sporadic occurrence of affected individuals and discordance in twins implicates the effect of non‐genetic factors.
OBSERVATIONS ON INTRAUTERINE GROWTH IN URBAN ETHIOPIA
Authors: GEBRE‐MEDHIN MEHARI , STERKY GÖRAN , TAUBE ADAM
Published: January 2008

ABSTRACT. A total of 3 144 deliveries comprising a wide range of socio‐economic groups in Addis Abeba were investigated. A positive correlation was found between family income and birth weight, with a difference of nearly 500 g between the extremes of socio‐economic classes. A seasonal variation in meart birth weight was also observed. The mean length of gestation was 7.4 days shorter, and the anthropometric measures at birth were significantly lower in the Ethiopian infants as compared with the Swedish norm. The median weight and length development in relation to gestational age in the Ethiopian newborns was similar to the Swedish standard up to approximately 34–35 weeks of gestation. After that time there was very little further intrauterine growth in Ethiopian infants. The possible reasons for the observed pattern of growth are discussed.
SEASONAL VARIATIONS IN WEIGHT OF CHILDREN ATTENDING AN UNDER‐FIVES CLINIC IN LESOTHO
Authors: COHEN NICHOLAS , CLAYDEN A. D.
Published: January 2008

ABSTRACT. The incidence of weight loss between successive visits, prevalence of under centile 3 weight‐for‐age and over centile 97, showed a marked bimodal annual variation for 1243 children attending an under‐fives clinic. 9 949 weighings on 661 boys and 682 girls over a five year period, contributed to the database. Season of birth also significantly influenced the centile distributions of weight‐attained‐for‐age. Children over 1½ years old showed considerably higher rates of weight loss (about 1 in 5 children attending in their fifth year of life), than children younger than the mean age of weaning. The rates of weight loss, together with seasonal variation, was considerably higher for a group of ‘Regular Attenders’ to the clinic, than for a group of ‘Low Attenders’. Seasonal influences on growth need to be taken into accounts in evaluating the quality of care and outcome produced by under‐fives clinics.
LONG TERM PROGNOSIS OF INFANTS WITH SEVERE IDIOPATHIC RESPIRATORY DISTRESS SYNDROME
Authors: KAMPER J.
Published: January 2008

ABSTRACT. 76 out of 77 children surviving IRDS with the aid of intermittent positive pressure ventilation have been folio ed‐up by the age 2.6–7.6 years together with 68 matched controls. Moderate or severe neurological, developmental or mental abnormalities were present in 17% of all IRDS survivors. Statistical comparison of the matched pairs of IRDS survivors and controls revealed no significant differences in the prevalence of abnormalities. In the IRDS survivors the occurence of cerebral palsy related to prematurity while the development of psycho‐motor and mental retardation related to low birth weight and low milk ìntake durìng the first week suggesting that both prenatal and postnatal growth retardation may have been of importance. Statistical analysis of a number of preventilatory and ventilatory parameters did not show significant differences between these groups of IRDS survivors and the remainder. Ventilator treatment is recommended as a promising adjunct to the therapy of severe IRDS in centers where the necessary experience and equipment is at hand.
DELETION SHORT ARM 18 AND SILVER‐RUSSELL SYNDROME
Authors: CHRISTENSEN MOGENS FJORD , NIELSEN JOHANNES
Published: January 2008

ABSTRACT. 4‐year‐old boy presented with physical signs of the Silver‐Russell syndrome. He further had low‐set, large protruding ears, ptosis, broad nasal base, thick protruding lips, pronounced caries, malalignment of teeth, micrognathia and mental retardation. Chromosome examination showed deletion of the short arms of chromosome No. 18. It is concluded that considering the previous findings of chromosome No. 18 aberrations in some patients with Silver‐Russell syndrome such patients should always have chromosome examination made.
ANTINUCLEAR ANTIBODIES IN JUVENILE CHRONIC ARTHRITIS
Authors: PERMIN HENRIK , HØRBOV STEEN , WIIK ALLAN , KNUDSEN JETTE VESTERGAARD
Published: January 2008

ABSTRACT. One hundred patients with juvenile chronic arthritis (JCA) were studied with respect to granulocyte‐specific and organ‐nonspecific antinuclear antibodies (GS‐ and ON‐ANA) in relation to clinical features of disease. Seventy‐two were girls and 28 boys. Sixty‐seven patients had IgG ANA, 31 IgM, 10 IgA, 6 IgD, 19 IgE and 35 had ANA, which fixed complement C3. Sixteen of 17 sera containing IgG GS‐ANA were from girls. The prevalence of IgG GS‐ANA increased with the number of joints affected. No patient with the acute febrile type of the disease had IgG GS‐ANA or C3 fixing ANA. The prevalence of IgG ON‐ANA did not differ significantly in the mono‐, pauci‐, polyarticular and acute febrile types of JCA. Patients showing clinical activity more frequently had IgG and IgM ANA and C3 fixing ANA. The high titers of ANA were most often seen in girls. Chronic uveitis occurred in 10 of the patients and IgG ANA were present in sera from all of these.
PAPAVERINE IN THE PROPHYLAXIS OF MIGRAINE AND OTHER VASCULAR HEADACHE IN CHILDREN
Authors: SILLANPÄÄ M. , KOPONEN M.
Published: January 2008

ABSTRACT. A Daily dose of papaverine (5–10 mg per kg body weight) was administered on a double‐blind basis for two months to 42 successive child patients suffering from migraine or other vascular headache attacks not less than twice a month. Thirty‐seven patients received the drug for the whole period of two months and 31 of these could be followed for a period of one to 10 months (mean 4.5 months) after the cessation of the treatment. Nineteen received papaverine and 18 placebo. Six out of 19 papaverine patients and none of 18 placebo patients were completely free from attacks during the treatment. Altogether 11 patients in the papaverine group and 5 in the placebo group experienced a 75–100% reduction in attack frequency which was accompanied by lower intensity and shorter duration of attacks. The results were best in cases with classical migraine. An “excellent” or “good” drug effect was experienced by 58% of papaverine patients and 17% of placebo patients.
RENAL FUNCTION IN ANOREXIA NERVOSA
Authors: APERIA A. , BROBERGER O. , FOHLIN L.
Published: January 2008

ABSTRACT. Renal function was examined in twelve patients, eight girls and four boys, with anorexia nervosa (AN) ranging in age from 12.6 to 18.2 years. The weight loss at the time of the study averaged 26%. Determinations were made of glomerular filtration rate (GFR), PAH clearence (CPAH) and urinary concentrating capacity. For references the same studies were also carried out in five healthy teenagers. Both GFR and CPAH were generally reduced in AN. The reduction of GFR was, however, out of proportion to the reduction of CPAH as shown by a significantly lower filtration fraction (FF) in AN. Indirect evidence suggests that the low FF could be attributed to reduced water permeability of the glomerular capillary. The urinary concentrating capacity following fluid deprivation was moderately depressed both before and after the administration of vasopressin. The concentrating defect in AN must therefore be primary of renal origin.
MVPP CHEMOTHERAPY COMBINED WITH RADIOTHERAPY IN THE TREATMENT OF HODGKIN'S DISEASE IN CHILDREN
Authors: ARMATA J. , STOPYROWA J. , DEPOWSKI M. , STRZESZYǸSKI J. , BORKOWSKI W. , KACZOR Z. , DEPOWSKA T.
Published: January 2008

ABSTRACT. Thirty‐four children with Hodgkin's disease were treated during the years 1969–75. After radiotherapy, 7–15 cycles of MVPP were given within 24–53 months. In order to avoid chronic leukopenia, leukocyte counts were made frequently during chemotherapy, and the drug doses adjusted accordingly. A complete remission was obtained in 32 of the 34 children. Two patients died because of progressive disease. Twelve of the 32 survivors have been followed for at least 5 years, and a further 12 for at least 3 years. Three children are still on chemotherapy, whereas the remaining 29 being followed are in continued complete remission.
RENOGRAPHY IN THE FOETAL AND NEWBORN LAMB
Authors: HIRVONEN LEO , RÄTY JUHANI , KIVINIITTY KALEVI , TIMISJÄRVI JOUNI
Published: January 2008

ABSTRACT. Hippuran renography indicates kidney function as reflected in a substance handled mainly by the tubules. The working conditions of the kidneys undergo a fundamental change at birth with the cessation of placental circulation, and these organs become responsible for waste elimination. 131I‐hippuran renography was performed on foetal and newborn lambs using a gamma camera and a computer. The intervals of the maximum‐ and half‐time renal activity were determined. These decreased by a half from mean foetal value of Tm=7 min and T½=19 min in 1 to 1.5 days and reached the full‐grown level in 2 to 5 weeks. No sudden change occurred as a result of the first breath.
HOW TO APPROACH THE CHILD SUSPECTED OF MALABSORPTION Experience from a Prospective Investigation of Suspected Malabsorption in Children 1968–1976 in Malmö
Authors: BERG N. O. , BORULF S. , JAKOBSSON I. , LINDBERG T.
Published: January 2008

ABSTRACT. 180 children (mean age 20 months) suspected of malabsorption because of failure to thrive, abnormal stools more than 3 weeks, vomiting, an/or abdominal distension were investigated with peroral small intestinal biopsy at duodeno‐jejunal flexure (172 children) and/or duodenal intubation for analysis of trypsin and amylase activity in duodenal juice before and after a test meal of water (76 children). Results of xylose tolerance test, lactose tolerance test, faecal fat, B‐folate, S‐iron, and S‐albumin were related to morphology of mucosa. A normal finding of one of these tests means in 15–26% a normal mucosa (diagnostic sensitivity). An abnormal finding means in 40–85% a severely damaged mucosa and in 85–100% a slightly, moderately, or severely damaged mucosa (diagnostic specificity). Combinations of these tests increase the diagnostic sensitivity 10–15%. Faecal chymotrypsin seems to be a reliable screening test for exocrine pancreatic function. Border values or low values indicate a direct evaluation of exocrine pancreatic function. The simple test meal (water) method with determination of trypsin in duodenal juice gives, from a practical point of view, good information of the exocrine pancreatic function.

The following plan of investigation is proposed: Step 1. careful clinical history and examination; Step 2. analysis of faeces for Giardia lamblia, entero‐pathogenic microorganisms, and chymotrypsin, sweat test; Step 3. peroral small intestinal biopsy and/or duodenal juice analysis, and finally—if steps 2 and 3 give normal results; Step 4. re‐evaluation of dietary history and tests to detect any food intolerance (e.g. carbohydrate).
CONTAMINANTS IN HUMAN MILK
Authors: OLSZYNA‐MARZYS A. E.
Published: January 2008

ABSTRACT. There is a paucity of information regarding excretion of contaminants in human milk, due to experimental difficulties and until recently a general lack of interest. Because of the high fat content of milk and as its acidity is higher than that of plasma, nearly all liposoluble and basic agents consumed by the mother will be excreted in the milk. Distinction must be made between, on the one hand drugs and social toxicants such as smoking and alcohol, whose intake can be stopped or limited during pregnancy and lactation, and ecological toxicants present in a polluted environment to which the mother is exposed. Cases have occurred of heavy prenatal and postnatal intoxication of infants with hexachlorobenzene in Turkey and methylmercury in Iraq due to consumption of fungicide‐treated seed wheat by pregnant and lactating mothers. Recent attention has been concentrated on contamination of milk with organochlorine compounds such as DDT and PCB's, that are found in many parts of the world. The heaviest contamination with DDT has been found in Guatemala, resulting in suckling infants consuming many times the Acceptable Daily Intake of this compound proposed by WHO, with unknown future effects.
FOLLICULAR DEVELOPMENT IN OVARIES OF CHILDREN WITH DOWN'S SYNDROME
Authors: HØJAGER B. , PETERS H. , BYSKOV A. G. , FABER M.
Published: January 2008

ABSTRACT. Ovaries of children with Down's syndrome were examined histologically in order to determine in what way the ovarian development differed from the normal. Twenty‐six specimens obtained at autopsy at various ages between birth and 14½ years were available and compared with ovaries from normal children of similar ages. All ovaries from normal children were characterized by active follicle growth. The ovaries of the children with Down's syndrome, however, showed absence or retardation of follicle growth. Furthermore the number as well as the size of the antral follicles differed from those in the normal ovary. The decrease of the number of small follicles occurs earlier in life in the ovaries of children with Down's syndrome than in the control. The possible relationship between an abnormal ovarian growth pattern and hormonal imbalance is discussed.
DISSEMINATED HERPES SIMPLEX VIRUS INFECTION IN ATAXIA‐TELANGIECTASIA
Authors: BEN‐ZVI A. , SOFFER D. , YATZIV S.
Published: January 2008

ABSTRACT. The clinical and pathological features are described in a child with ataxia‐telangiectasia, complicated by fatal disseminated herpes simplex virus infection. Herpes simplex virus was isolated from the patient's blood, and the histopathological findings in the skin, liver and adrenals were consistent with herpes simplex virus infection. The patient had a combined immune deficiency state, as a part of the ataxia‐telangiectasia syndrome. She had impaired cellular immune response to herpes simplex virus and developed no antibodies against the virus. To our knowledge, this is the first fatal case of disseminated herpes simplex virus infection in ātaxia‐telangiectasia.
PARADOXICAL ENHANCEMENT OF TOLBUTAMIDE‐INDUCED INSULIN RELEASE BY DIAZOXIDE IN A PATIENT WITH ISLET CELL HYPERPLASIA
Authors: SCHIKMAN C. H. , CHERTOW B. S. , FARISS B. L.
Published: January 2008

ABSTRACT. A case of islet cell hyperplasia in a ten year old black male with symptomatic fasting hypoglycemia was documented histopathologically. Provocative studies with glucose, tolbutamide, glucagon, and diazoxide were performed to test the insulin response of hyperplastic islets. The islets responded to glucose, glucagon, and tolbutamide. Diazoxide potentiated the tolbutamide‐induced insulin response, and this effect of diazoxide was not blocked by propranolol. In the diagnostic work up of islet cell hyperplasia, dizoxide may paradoxically potentiate tolbutamide‐induced insulin release, a finding which may falsely suggest progression of the disease.