bluuebunny/crossref_metadata_2025 huggingface dataset:
DICARBOXYLIC ACIDURIA DURING KETOTIC PHASES IN VARIOUS TYPES OF GLYCOGEN STORAGE DISEASE
Authors: PETTERSEN JON ELLING , WINSNES ARNT
Published: January 2008

ABSTRACT. Petterscn, J. E. and Winsnes, A. (Institute of Clinical Biochemistry and Paediatric Research Institute, Rikshospitalet, Oslo 1, Norway). Dicarboxylic aciduria during ketotic phases in various types of glycogen storage disease. Acta Paediatr Scand, 70:309, 1981.–Urine samples were collected before and after a starvation period of 14–16 h from patients with glycogen storage disease, one with type III (amylo‐l,6‐glucosidase deficiency), four with type VIII (phosphorylase‐b‐kinase deficiency), and one with an unclassified type. The excretion of adipic, suberic, and 3‐hydroxybutyric acid was measured by combined gas chromatography‐mass spectrometry. The tendency towards ketosis seemed to decline with age in the patients with type VIII. In the non‐ketotic patients no excess amounts of dicarboxylic acids were excreted. Therefore, glycogen storage disease per se seems to have no direct relationship to the excretion of adipic or suberic acid. A positive correlation was, however, found between the urinary excretion of on one side 3‐hydroxybutyric and on the other adipic (correlation coefficient (Kendall's tau) +0.64, P<0.002 (one‐sided test)) or suberic (+0.61, P <0.003) acid. The two dicarboxylic acids are most probably formed from long‐chain monocarboxylic acids by w and β‐oxidation. It is speculated that succinyl‐CoA formed by this pathway may counteract the tendency to ketosis in patients with glycogen storage disease.
ON THE RENAL TUBULAR DAMAGE IN HEREDITARY TYROSINEMIA AND ON THE FORMATION OF SUCCINYLACETOACETATE AND SUCCINYLACETONE1
Authors: FÄLLSTROM SVEN‐PETTER , LINDBLAD BENGT , STEEN GöRAN
Published: January 2008

ABSTRACT. Fällström, S.‐P., Lindblad, B. and Steen, G. (Department of Paediatrics, East Hospital, University of Gothenburg, Gothenburg, Department of Paediatrics, Molndal's Hospital, Molndal, Department of Clinical Chemistry, University of Gothenburg, Gothenburg, Sweden). On the renal tubular damage in hereditary tyrosinemia and on the formation of succinylacetoacetate and succinylacetone. Acta Paediatr Scand, 70:315, 1981.–Phenylalanine and homogentisate increase the concentration of succinylacetoacetate and succinylacetone both in serum and urine in patients with hereditary tyrosinemia and therefore increase the excretion of 5‐aminolevulinate. Both phenylalanine and homogentisate cause a tubular proteinuria which is in agreement with our hypothesis that their metabolites maleylacetoace‐tate and fumarylacetoacetate are the toxic compounds in hereditary tyrosinemia. The patient with the highest excretion of succinylacetoacetate and succinylacetone has the slightest tubular proteinuria whereas the one with the lowest excretion of these compounds has the more pronounced tubular proteinuria. It is suggested that this is caused by a difference in the ability to reduce the presumed toxic compounds fumarylacetoacetate and maleylacetoacetate, i.e. the precursors of succinylacetoacetate.
DIAZEPAM RECTAL SOLUTION FOR HOME TREATMENT OF ACUTE SEIZURES IN CHILDREN
Authors: HOPPU K. , SANTAVUORI P.
Published: January 2008

ABSTRACT. Hoppu, K. and Santavuori, P. (Children's Hospital, Helsinki University Central Hospital, Helsinki, Finland). Diazepam rectal solution for home treatment of acute seizures in children. Acta Paediatr Scand, 70:369, 1981.–To gain clinical experience of treating acute convulsions in children with diazepam rectal solution, 17 epileptic children with prolonged convulsions were treated at home. The drug was used 65 times. On 2/3 of all occasions the seizure was interrupted in less than 15 min. A starting dose of 0.5 mg/kg is recommended, with a maximum of 20 mg per single dose. One patient had respiratory difficulties, one dizziness and two skin reactions.
THE INCIDENCE OF COELIAC DISEASE IN CHILDREN IN SOUTH‐EAST SWEDEN
Authors: STENHAMMAR L. , JOHANSSON C.‐G.
Published: January 2008

ABSTRACT. Stenhammar, L. and Johansson, C.‐G. (Department of Paediatrics, Central Hospital, Norrköping and Department of Paediatrics, Central Hospital, Jönköping, Sweden). The incidence of coeliac disease in children in south‐east Sweden. Acta Paediatr Scand, 70:379, 1981.–Among children born between 1969 and 1977 in two areas of south‐east Sweden an initial flat small intestinal mucosa was found in 84 cases, 64 of whom were considered to have coeliac disease (CD) diagnosed mostly according to the criteria formulated by the European Society for Paediatric Gastroenterology and Nutrition. The incidence of CD was 1/859, which is in agreement with that of a recent report from south Sweden. This suggests that the incidence of CD is the same in the whole of Sweden. In the present study all patients with an initial flat mucosa improved clinically and histologically after 1 year or more on a gluten‐free diet. When challenged with gluten, 19% did not relapse within 2 years, thus fulfilling the criteria for so‐called transient gluten intolerance. These patients had a lower mean age of symptom debut and for first biopsy, compared with the patients with CD.
TUBULAR PROTEINURIA AFTER PERINATAL HYPOXIA
Authors: MILTÉNYI M. , POHLANDT F. , BÓKA G. , KUN E.
Published: January 2008

ABSTRACT. Milt7eacute;nyi, M., Pohlandt, F., Bóka, G. and Kun, E. (2nd Department of Paediatrics, Semmelweis University, Medical School, Budapest, Hungary, and the Section of Neonatology, Centre of Paediatrics, University of Ulm, Federal Republic of Germany). Tubular proteinuria after perinatal hypoxia. Acta Paediatr Scand, 70:399, 1981.–Urinary total protein (UTP) and urinary protein pattern have been studied in 23 newborn infants with Apgar scores ±S3 at one minute or acidosis (pH ±7.15) on the first day. On the first and second day UTP excretion was increased in 13 out of 18 patients. At this time the excretion of low molecular weight microproteins (T‐4 and T‐5) was elevated in 12 patients without increased plasma urea concentration in any case. The increased excretion of the smallest microproteins T‐4/T‐5 is an early sign of an impaired tubular function.
GOODPASTURE'S SYNDROME IN A CHILD: NATURAL HISTORY AND EFFECT OF TREATMENT
Authors: MARTINI A. , BINDA S. , MARIANI G. , SCOTTA M. S. , RUBERTO G.
Published: January 2008

ABSTRACT. Martini, A., Binda, S., Mariani, G., Scotta, M. S. and Ruberto, G. (Department of Pediatrics, University of Pavia, Italy). Goodpasture's syndrome in a child: natural history and effect of treatment. Acta Paediatr Scand, 70:435, 1981.–An 8‐year‐old girl with microhematuria of recent onset developed a picture of pulmonary hemosiderosis in the space of 2 months. Some months later while pulmonary involvement was improving severe extracapillary glomerulonephritis developed. Circulating anti‐glomerular basement membrane antibody was detected and linear deposition of IgG along glomerular basement membrane was observed. A diagnosis of Goodpasture's syndrome was made and treatment was started with prednisone, cyclophosphamide and periodic plasmapheresis with complete progressive disappearance of circulating anti‐glomerular basement membrane antibody. After suspension of plasmapheresis despite immunosuppressive therapy and lack of evidence for circulating anti‐glomerular basement membrane antibody, the child went into terminal renal failure. The natural history of the disease in this case and the results of treatment are discussed. To our knowledge this is the first case of Goodpasture's syndrome reported in childhood with demonstration of the presence of anti‐glomerular basement membrane antibody.
TSH AND THYROXINE IN STORED NEONATAL FILTER‐PAPER BLOOD SAMPLES FROM PATIENTS WITH CONGENITAL HYPOTHYROIDISM
Authors: LARSSON A. , LJUNGGREN J. G. , LUNDBERG K.
Published: January 2008

ABSTRACT. Filter paper blood samples collected neonatally from infants with congenital hypothyroidism were analysed retrospectively for TSH (n= 41) and thyroxine (n= 16). The patients were detected by clinical signs and symptoms and treatment was started during the first two years of life. Similar blood samples from control infants were analysed for comparison. All CH patients would have been detected neonatally if screening had been based on TSH analyses with a cut‐off limit corresponding to 50 mU of TSH/I of plasma. A screening programme involving analyses of thyroxine would require considerably higher recall frequency to yield 100% sensitivity. These results support a nationwide CH screening program based on TSH analyses of PKU filter paper blood samples using 50 mU/I of plasma as the cut‐off limit.
EFFECTS OF INJECTED LIPID EMULSION ON OXYGEN CONSUMPTION, RQ, TRIGLYCERIDE, FREE‐FATTY‐ACID AND β‐HYDROXYBUTYRATE LEVELS IN SMALL‐FOR‐GESTATIONAL‐AGE (SGA) INFANTS
Authors: SABEL KARL‐GÖRAN , OLEGÅRD RAGNAR , HILDINGSSON KARIN , KARLBERG PETTER
Published: January 2008

ABSTRACT. Ten SGA infants were studied from 4 hours after birth (day 1) and again at 28 hours (day 2) before and for 4 hours after single injections of 0.5 g of Intralipid® fat/kg b. w. (IL‐group). Eight other SGA infants were given 9–10 ml/kg of breast milk (BM‐group). After lipid injection the elimination of triglycerides (TG) from plasma was markedly delayed. On day 2 lipolysis had improved, but was still slower than in previously studied AGA infants. The initial FFA plasma level was higher on day 1 than on day 2. Oxidation of released fatty acids was confirmed by a significant increase of Vo, and a decrease of RQ on day 1 and 2. In all infants the β‐hydroxybutyrate level in plasma increased and was still elevated 4 hours after injection of fat. A negative correlation was found between β‐hydroxybutyrate levels and RQ. In the BM‐group changes in TG and β‐hydroxybutyrate levels were small and insignificant. FFA had decreased 60 min after breast milk on day 1. In conclusion: TG elimination from plasma was impaired on day 1 and had slightly improved on day 2. The fatty acids released by lipolysis were oxidized as seen by increasing Vo, falling RQ and increasing β‐hydroxybutyrate plasma levels both on day 1 and day 2.
THE HYALINE MEMBRANE DISEASE–INTRAVENTRICULAR HEMORRHAGE RELATIONSHIP IN THE VERY LOW BIRTH WEIGHT INFANT: PERINATAL ASPECTS
Authors: GARCIA‐PRATS JOSEPH A. , PROCIANOY RENATO S. , ADAMS JAMES M. , RUDOLPH ARNOLD J.
Published: January 2008

ABSTRACT. A large retrospective clinical study is reported confirming pathologic studies upon the effect of hyaline membrane disease on the occurrence of intraventricular hemorrhage in very low birth weight infants. Two hundred and twenty infants with birth weight 1500 g and gestational age 32 weeks were studied. Infants with hyaline membrane disease (112) had 56 % incidence of intraventricular hemorrhage whereas of those without hyaline membrane disease (108) only 31% developed intraventricular hemorrhage (p < 0.001). When controlled for gestational age, the more immature infants ( 1000 g) exhibited no difference in the occurrence of intraventricular hemorrhage whether hyaline membrane disease coexisted or not. In the 1001–1500 g group, the occurrence of hyaline membrane disease with intraventricular hemorrhage was significant (p < 0.001). The association of lower Apgar scores and the influence of intermittent positive pressure ventilation in infants with intraventricular hemorrhage is discussed. Extreme immaturity negates all perinatal clinical expertise in determining neonatal outcome. Therefore, carrying pregnancies beyond 28 weeks gestation is mandatory. Beyond 28 weeks, pulmonary maturity and the influence of therapeutic modalities and maternal transport become increasingly important.
CONGENITAL LACTIC ACIDOSIS, α‐KETOGLUTARIC ACIDURIA AND VARIANT FORM OF MAPLE SYRUP URINE DISEASE DUE TO A SINGLE ENZYME DEFECT: DIHYDROLIPOYL DEHYDROGENASE DEFICIENCY
Authors: MUNNICH A. , SAUDUBRAY J. M. , TAYLOR J. , CHARPENTIER C. , MARSAC C. , ROCCHICCIOLI F. , AMEDEE‐MANESME O. , COUDE F. X. , FREZAL J. , ROBINSON B. H.
Published: January 2008

ABSTRACT. A 6 month‐old girl with vomiting, hypotonia and motor retardation was found to have elevated blood lactate, pyruvate, and branched chain amino acids essociated with ketoglutaric aciduria. The combination of a congenital lactic acidosis with a variant form of maple syrup urine disease and ketoglutaric aciduria suggested a defect of a single component, common to pyruvate dehydrogenase, to branched chain ketoacid dehydrogenase, and to α‐ketoglutarate dehydrogenase. Dihydrolipoyl dehydrogenase is the common component (E3). The three enzyme activities and the E3 component activity were found to be reduced in liver and cultured fibroblasts, thus confirming that a single defect of this component can result in a multiple deflciency involving several oxidative decarboxylation reactions.
EVALUATION OF TWO SPIROMETERS AND TWO FLOWMETERS IN ASTHMATIC CHILDREN
Authors: DALÉN G. , KJELLMAN B.
Published: January 2008

ABSTRACT. The reliability of a Monaghan (M 403) spirometer and a Vitalograph spirometer was evaluated in 46 children with “subclinical” asthma. As in previous studies of healthy children the vital capacity (VC) and the forced expiratory volume in one second (FEV1.0) were close to those of a Bernstein spirometer. The sensltivity of the spirometers and of two flowmeters (Wright's peak flow meter and Airflometer‐Glaxo) was evaluated in the asthmatic children after inhalation of salbutamol and the changes after inhalation were compared to those obtained in a previous study of healthy children. The “simple” equipment gave substantial information about the degree of bronchial obstruction. It is concluded that the additional information obtained by using simple pulmonary function tests form a good basis for aggressive therapy.
STUDIES ON PYRUVATE CARBOXYLASE, PYRUVATE DECARBOXYLASE AND LIPOAMIDE DEHYDROGENASE IN SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY
Authors: HANSEN T. L. , CHRISTENSEN E. , BRANDT N. J.
Published: January 2008

ABSTRACT. In two autopsy‐proven cases of subacute necrotizing encephalomyelopathy (SNE, Leigh's Disease) the activities of pyruvate carboxylase, pyruvate decarboxylase and lipoamide dehydrogenase were investigated in cultured fibroblasts. Normal activities of pyruvate carboxylase and lipoamide dehydrogenase were found in both cases. The activity of pyruvate decarboxylase was low in one of the cases (p<0.05), while the activity in the other was within normal limits. The concentrations of alanine, lactate and pyruvate were normal or only slightly increased. The relationship between SNE and a defect in pyruvate metabolism is under discussion, and it is concluded that the general assumption that pyruvate carboxylase deficiency is the cause of SNE is not in agreement with our results or the present literature. However, pyruvate decarboxylase deficiency may in some cases contribute to the development of SNE.
ANTIBODIES AGAINST ESCHERICHIA COLI CAPSULAR (K) ANTIGENS IN HUMAN MILK AND SERUM
Authors: CARLSSON B. , KAIJSER B. , AHLSTEDT S. , GOTHEFORS L. , HANSON L. Å.
Published: January 2008

ABSTRACT. Specimens of milk, serum and faeces were collected from 23 healthy women, as well as faeces from their newborn infants, at several occasions from one to nine weeks after delivery. The predominating faecal E. coli strains were typed with regard to five different K antigens. The most frequently occurring was K1, followed by K3 and K13. E. coli with the same K antigen was found in only five of the mother‐infant pairs, while ten pairs never had any strain with a common K antigen during the observation period. Milk and serum antibodies were regularly found against a variety of E. coli K antigens using the enzyme‐linked immunosorbent assay. In milk the dominating immunoglobulin class of anti‐K antibodies was secretory IgA, with a high ratio of milk/serum antibody, suggesting a local production in the mammary gland. There were no significant differences of K antibody levels in serum or milk, whether or not the particular K strain was present in the mother's gut flora. The presence of specific K antibodies in serum or milk of the mothers did not prevent colonization of the gut of the infants with E. coli strains carrying the corresponding K antigen.
TRANSEPIDERMAL WATER LOSS IN NEWBORN INFANTS
Authors: HAMMARLUND K. , SEDIN G. , STRÖMBERG B.
Published: January 2008

ABSTRACT. During the first weeks after birth the transepidermal water loss was determined repeatedly in 7 infants born after 25–27 weeks of gestation, in 13 infants born after 28–30 weeks and in 14 full‐term infants. All infants were appropriate for gestational age. The very pre‐term infants had high evaporative water losses from their skin on their first days after birth. A gradual decrease was then observed, but after four weeks the values were still higher than those in full‐term infants. The transepidermal water loss in full‐term infants was unchanged during the first two weeks of life, but increased during the subsequent two weeks. The relation between evaporation rate from an interscapular skin area and ambient humidity was studied repeatedly during the first week of life in two very pre‐term infants. The magnitude of evaporation decreased with increasing post‐natal age, but was still dependent on the ambient relative humidity.
BREAST FEEDING: REASONS FOR GIVING UP AND TRANSIENT LACTATIONAL CRISES
Authors: VERRONEN P.
Published: January 2008

ABSTRACT. A prospective study of the course of breast feeding was carried out in a group of 150 mothers. The reasons for terminating breast feeding before six months and the occurrence of transient lactational crises were recorded. 23% of mothers gave up breast feeding because of lactational difficulties. 12% stopped for external reasons and 65% carried on beyond six months. 36% of mothers experienced one or more transient lactational crises, which were successfully overcome by proper breast feeding techniques in a few days.
VITAMIN D METABOLISM IN HYPOPHOSPHATASIA
Authors: OPSHAUG O. , MAURSETH K. , HOWLID H. , AKSNES L. , AARSKOG D.
Published: January 2008

ABSTRACT. A 4‐month‐old boy with the infantile form of hypophosphatasia was followed for 9 months with measurements of serum calcium, phosphate, alkaline phosphatase and various vitamin D metabolites, together with urinary excretion of cyclic AMP. During the initial hypercalcemic stage the serum concentration of 25‐hydroxyvitamin D was normal. Urinary cyclic AMP was low and the serum concentration of the dihydroxymetabolites of vitamin D were appropriate to the high serum calcium with low 1,25(OH)2D and relatively high 24, 25(OH)2 and 25, 26(OH)2D levels. Due to restrictions of the vitamin D intake and lack of exposure to sun he developed vitamin D deficiency rickets at 9 months of age with very low serum concentration of 25‐hydroxyvitamin D and markedly increased urinary excretion of cyclic AMP. Following vitamin D treatment the serum level of 1,25(OH)2D showed a brisk rise to a considerably elevated value. Initially the serum concentration of alkaline phosphatase was well below the normal range, rase markedly during the stage of active rickets and returned to the characteristic low levels of hypophosphatasia with healing of the rickets.
DYSKINETIC CEREBRAL PALSY
Authors: KYLLERMAN M. , BAGER B. , BENSCH J. , BILLE B. , OLOW I. , VOSS H.
Published: January 2008

ABSTRACT. An unselected series of 116 dyskinetic cerebral palsy cases born 1959‐70 was delineated and subgrouped according to neurological criteria into 35 hyperkinetic (30%) and 81 dystonic (70%) cases. Of the hyperkinetics, 80% had a mild motor disability, while 90% of the dystonics had moderate or severe disability. Subordinated spastic signs were found in 9% of the hyperkinetic and in 44% of the dystonic cases. IQ was >90 in 69% of the hyperkinetics and 25% of the dystonics and <50 in 11% and 41%, respectively. Two or more additional neurological abnormalities (spasticity, mental retardation, epilepsy, anarthria, dysarthria, hearing deficit) were detected in 46% hyperkinetic and 81% dystonic cases. A heavy multihandicap complex was present predominantly in the dystonic subgroup. The 5‐19 year mortality rate was 6 % and was confined to the most severely handicapped dystonic cases. The twin rate was 5%. A total of 9% had birth weights ≤ 1500 g, and 31% had ≤ 2500 g. The weight‐ and age‐specific incidences increased rapidly with decreasing birth weights and gestational ages. The crude incidence of hyperkinetic cerebral palsy was 0.07 and dystonic cerebral palsy 0.17 per thousand live births.
HYPOCALCEMIA IN INFANTS OF DIABETIC MOTHERS
Authors: SALLE B. , DAVID L. , GLORIEUX F. , DELVIN E. E. , LOUIS J. J. , TRONCY G.
Published: January 2008

ABSTRACT. Twenty‐two infants of diabetic mothers (IDM) were studied and were divided into two groups: a first group of 14 IDM did not receive vitamin D3 and was studied at birth and at 2, 24, 48 and 120 hours; a second group was given daily dosage of 60 μg of vitamin D3 from 3 hours to 120 hours and was studied at 2 hours and 120 hours.

In the first group, serum calcium levels decreased markedly during the first 24 hours of life (mean ± SD: 1.77±0.3 mmol/1, p<0.01) and remained low at 5 days. Serum phosphorus levels remained normal but serum magnesium levels decreased significantly at 24 hours (mean ± SD: 0.64±0.108 mmol/1, p<0.01) and returned to normal at 5 days. Serum immunoreactive parathormone levels increased consistently to high levels at 24 hours and remained elevated at 120 hours (p<0.001). Serum immunoreactive calcitonin levels increased at 24 hours (p<0.001) and decreased at 120 hours to low or undetectable values in all infants.

In group II, serum 25O‐HD levels and 1.25 OH2 D levels increased significantly (p<0.001) respectively to 27.2±2.7 ng/ml and 114±20 pg/ml at 5 days. The results of this study show hypocalcemia to be a common event in IDM during the first days of life and furthermore hypophosphatemia, hypoparathyroidism, hypomagnesemia or defect of vitamine D metabolism would not seem to be the main etiological factors.
CYCLICAL VOMITING WITH PNEUMOMEDIASTINUM
Authors: BULLIMORE D. W. , COOKE D.
Published: January 2008

ABSTRACT. A case is reported of cyclical vomiting of sufficient severity to result in both dehydration and electrolyte disturbance, culminating in grand mal seizures and pneumomediastinum with subcutaneous emphysema. Similar episodes of subcutaneous emphysema over the face and neck had been noted by the patient on previous occasions.
LATE PRESENTATION OF SHWACHMAN'S SYNDROME
Authors: HISLOP W. S. , HAYES P. C. , BOYD E. J. S.
Published: January 2008

ABSTRACT. Shwachman's syndrome is a rare cause of pancreatic exocrine insufficiency in childhood. It is usually associated with poor growth, neutropaenia and dyschondroplasia of bone metaphyses. Numerous associated congenital abnormalities and hereditary conditions have been described. The vast majority of cases are diagnosed before the age of two. The case described is of a girl presenting with steatorrhoea at age fourteen. Although of small stature, she had been completely well until that time. While survival of infantile cases into adolescence is reported, presentation of the condition in later years is rare and Shwachman's syndrome should therefore be considered as a possible cause of pancreatic insufficiency in older children and adolescents.
GOLDENHAR'S SYNDROME: DISCORDANCE IN MONOZYGOTIC TWINS AND UNUSUAL ANOMALIES
Authors: EBBESEN F. , PETERSEN W.
Published: January 2008

ABSTRACT. Goldenhar's syndrome occurring discordant in monozygotic twins and unusual anomalies associated with the syndrome–unilateral aplasia of the trigeminal nuclei and of the facial nerve, and a lateral lingual cleft–are reported.
AUDITORY BRAINSTEM RESPONSES (ABR) IN HIGH‐RISK NEONATES
Authors: MJØEN S. , LANGSLET A. , TANGSRUD S. E. , SUNDBY A.
Published: January 2008

ABSTRACT. In the present study, auditory brainstem responses (ABR) were recorded in 60 highrisk neonates in the intensive care unit selected by the following criteria: Birth‐weight <2000 g, hyperbilirubinemia requiring phototherapy or exchange transfusion, idiopathic respiratory distress syndrome, artificial ventilation, asphyxia, sepsis or meningitis, intracranial haemorrhage, neurological symptoms and potential ototoxic medication (aminoglycoides, furosemide). The infants tested ranged in gestational age from 27‐44 weeks. The ABR testing was performed in a sound‐proof room using the Madsen (ERA‐74) equipment. Four infants did not reveal responses to 70 dB HL (“nonresponders”), and the total of 10 neonates (16.6%) had abnormal ABR‐tests, when the physiological changes related to gestational age and conceptional age (gestational age plus the age after birth) were taken into account. The 10 neonates with abnormal tests were reexamined after discharge, and in six there were no improvement of threshold sensitivity. Three of the “nonresponders” were retested several times within the two years after birth (one died at age 18 months of pertussis), and none of them revealed ABR at stimulus intensity of 70 dB HL. They all attend an audiological training program started at age of six months as a consequence of the early diagnosis of impaired auditory function. It is our opinion that a routine ABR‐evaluation should be performed on high risk neonates (criteria mentioned above) in the newborn intensive care unit. Retesting of infants with abnormal responses within three months, and several times within the next two years if abnormal responses persist, is important. Transient impairment of auditory functions is not uncommon in these infants. However, the children with persisting hearing impairment should be discovered early to attend an early audiological training program.
VASOACTIVE INTESTINAL POLYPEPTIDE (VIP) IN CHILDREN WITH NEURAL CREST TUMOURS
Authors: SCHEIBEL ELMA , RECHNITZER CATHERINE , FAHRENKRUG JAN , HERTZ HENRIK
Published: January 2008

ABSTRACT. The concentration of vasoactive intestinal polypeptide (VIP) in plasma was measured in 22 children with neural crest tumours (NCT) during a 5‐year period; the mean concentration of VIP in plasma was 22.5 pmol/l (interval 2.0‐95.0 pmol/l). To establish a reference interval the plasma concentration of VIP was measured in 41 children without tumours; the mean concentration of VIP in plasma was 6.2 pmol/l (interval 0.5‐19.0 pmol/l). Of the 22 children with NCT 16 had a plasma concentration of VIP within the normal range while 6 children (27%) had elevated plasma concentration of VIP between 28 and 95 pmol/l. Only one child, whose plasma concentration of VIP was 95 pmol/l had diarrhoea. Elevated plasma concentration of VIP in children with NCT but no diarrhoea has not previously been described. The urinary excretion of vanillylmandelic acid (VMA) was increased in 18 of the children with NCT (82%). In 2 of the children with normal excretion of VMA the concentration of VIP in plasma was elevated. Thus, the plasma concentration of VIP may be a supplement to VMA as a tumour marker in some cases of NCT.
CHILDHOOD ACROMEGALY SUCCESSFULLY TREATED WITH INTERSTITIAL IRRADIATION USING YTTRIUM‐90
Authors: ROSENSTOCK J. , DOYLE F. H. , HALL R. , MASHITER K. , JOPLIN G. F.
Published: January 2008

ABSTRACT. A child with a growth hormone producing tumour presented at the age of 4 1/2 years. The onset of the disease was at 18 months of age. Treatment was given with three doses of interstitial irradiation using yttrium‐90 implants. There were no local complications from the procedures. Now, 11 years after diagnosis, she is asymptomatic, of normal appearance, and her height and the size of the pituitary fossa are normal. Growth hormone levels are almost normal, thyroid function is intact, and she is maintained on prednisone and sex hormones.
CHERRY RED MACULAR DEGENERATION
Authors: SAKURA N. , HYODO S. , MASUDA H. , SAKANO T. , HOSOKAWA A. , FUJII H. , USUI T. , SAKODA K. , CHOSHI K.
Published: January 2008

ABSTRACT. Cherry red macular degeneration was presented in a 6‐month‐old Japanese girl suffering from classical features of the diencephalic syndrome. The association of cherry red macular degeneration with the diencephalic syndrome was previously undescribed.
GRANULOMATOUS GIANT CELL ARTERITIS IN AN INFANT
Authors: SANDSTEDT B. , NILSSON H.
Published: January 2008

ABSTRACT.The first example of giant cell arteritis–with concomitant thrombocytosis‐in infancy is described. In a 4‐month‐old girl the entire aorta, main aortic branches, systemic and pulmonary artery were affected. Secondary thrombosis of the coronary, renal and mesenteric arteries resulted in Infarction. The difficulty in characterizing the various forms of giant cell arteritis is discussed.
PREVALENCE AND TEN‐YEAR (1970‐1979) INCIDENCE OF INSULIN‐DEPENDENT DIABETES MELLITUS IN CHILDREN AND ADOLESCENTS IN FINLAND
Authors: REUNANEN ANTTI , ÅKERBLOM HANS K. , KÄÄR MARJA‐LIISA
Published: January 2008

ABSTRACT. The prevalence and incidence of insulin‐dependent diabetes mellitus in Finnish children and adolescents were studied by means of a national register of drug‐treated diabetics and hospital records. At the end of 1979 the prevalence of diabetes in children aged 0‐14 was 191/100000, and in those aged 0‐19, 262/100000 respectively. During the 10‐year period from 1970 to 1979, 3916 new diabetics aged 0‐19 years were registered. The annual incidence of diabetes was 27.3/100000 in the age group 0‐19 years and 28.6/100000 in those 0‐14 years. The peak incidence of diabetes in girls occurred at 12 years of age and in boys at 14 years. There was a seasonal variation in the symptom debut, with peaks in April and in September and low rates in the summer. A regional variation in the prevalence and incidence rates was evident the rates being highest in eastern and lowest in northern Finland. The prevalence of diabetes mellitus increased clearly during the observation period, and the incidence tended to increase especially during the last years. Both prevalence and incidence rates of diabetes mellitus in Finnish children are high by international standards, possibly the highest in the world.
ACCELERATION OF DELAYED GROWTH WITH FLUOXYMESTERONE
Authors: LENKO HANNA LIISA , MÄENPÄÄ JORMA , PERHEENTUPA JAAKKO
Published: January 2008

ABSTRACT.61 boys with constitutional delay of growth and maturation, aged 9‐19 years and with a bone age (BA) lag of 1.3‐5.5 years, were administered fluoxymesterone (0.05‐0.24 mg/kg daily orally, relative dose increasing with age) to accelerate growth. The therapy was continuous and lasted 0.4‐3.6 years. The findings are compared with 37 observation periods in a similar group of untreated boys. Growth velocity increased in every treated boy during the therapy, the mean first‐year increment being 4.3±1.6 cm/year. For most boys this brought about a decrease in the height difference from peers, and so afforded the psychosocial relief that was the objective of the therapy. After therapy the velocity decreased slightly hi most boys, from a mean of 9.1±1.4 to 7.1±3.3 cm/year. The effect of the intervention on final height was assessed by three relatively independent methods of prediction. These were found to be equally valid in the 15 control boys for whom final heights are known. The effect appeared to vary individually, but on the average there appeared to be no loss of height potential. No individual boy with initial BA>10.5 years showed a substantial reduction in predicted final height.
PLASMA NORADRENALINE AND ADRENALINE IN INFANTS OF DIABETIC MOTHERS AT BIRTH AND AT TWO HOURS OF AGE
Authors: HERTEL J. , CHRISTENSEN N. J. , PEDERSEN S. A. , KÜHL C.
Published: January 2008

ABSTRACT. Plasma noradrenaline and adrenaline in the umbllical artery were very high in many infants of both diabetic mothers (IDM) and non‐diabetic mothers (controls) compared to values obtained in adults. Blood pH was slightly but significantly lower in IDM than in controls at delivery. There was a significant negative correlation between plasma noradrenaline and blood pH in IDM at birth, but no correlation between plasma noradrenaline and blood glucose, birth weight and Apgar scores. Plasma noradrenaline decreased significantly from birth to two hours of age both in IDM and controls. At two hours of age plasma noradrenaline and adrenaline were significantly greater in IDM compared to controls whereas blood glucose concentration was lower in the former group. There was a close inverse correlation between plasma noradrenaline and adrenaline, respectively, and blood glucose in IDM at two hours of age but not in controls. The elevated plasma noradrenaline at delivery in IDM may be explained by clinically undetectable acidosis whereas elevated plasma catecholamines at two hours are probably due to hypoglycaemia.
CARDIORESPIRATORY FUNCTION IN COMPETITIVE ENDURANCE RUNNERS AGED 12‐16 YEARS COMPARED WITH ORDINARY BOYS
Authors: SUNDBERG S. , ELOVAINIO R.
Published: January 2008

ABSTRACT. Thirty‐four male elite endurance runners aged 12‐16 years and 56 ordinary boys of the same age were studied in cross‐sectional age group. At the age of 12‐14 years, there were only a few differences between the runners and the controls: the runners weighed less, were leaner and had higher V02 max/kg body weight. The runners' good competitive performance could not be explained by a superior aerobic power at that age. In the age group of 16‐year‐olds, additional major differences were found: significantly higher VO2 max (4.05 I/min, 66 ml/min/kg), W170 (214 W, 3.5 W/kg), vital capacity (5,31 I), maximal expiratory volume (153 I/min), lower resting heart rate (62 beats/min) and larger heart volume (792 ml and 453 ml/m2 BSA) in the runners. In this respect our runners resembled adult endurance athletes. No differences could be observed in any age group as to height, hemoglobin concentration, blood pressure and maximal heart rate. The differences at the age of 16 years are either training effects or due to a selection of certain “endurance runner types”.
NATURAL HISTORY OF CHILDREN WITH ALPHA1‐ANTITRYPSIN DEFICIENCY AND NEONATAL CHOLESTASIS
Authors: NEMETH ANTAL , STRANDVIK BIRGITTA
Published: January 2008

ABSTRACT. Ten children with α1‐antitrypsin deficiency, phenotype PiZ, and neonatal cholestasis were followed to the ages of 4‐20 years. Standard liver function tests, urinary bile acid excretion and liver morphology were investigated within one week and the results were compared. Three patients had morphological signs of liver cirrhosis and one of these had clinical signs as well. Compared to healthy children of the same age urinary bile acid excretion was increased only in those with morphologically confirmed liver cirrhosis. Although most of the other patients had some minor histological changes in the liver biopsies, the normal urinary bile acid excretion was in accordance with their excellent general clinical state and is interpreted as a short‐term favourable prognostic sign.
PSYCHOSOCIAL AND INTELLECTUAL DEVELOPMENT IN 12 PATIENTS WITH INFANTILE NEPHROPATHIC CYSTINOSIS
Authors: WOLFF G. , EHRICH J. H. H. , OFFNER G. , BRODEHL J.
Published: January 2008

ABSTRACT. The psychosocial and intellectual development of 12 children with infantile nephropathic cystinosis was investigated longitudinally by use of biographical data, long‐term behavioral observations and psychological assessment. Of the 12 patients, eleven suffered terminal renal failure and 7 of these were followed up after renal transplantation. In spite of the severe illness and the resulting unusual life conditions the patients showed normal intellectual capacity and most of them average school performance. The patients were socially adapted, their behavior was predominantly cooperative, shy and reserved, but depressive at times. After transplantation the children became more active and outgoing. However, growing discrepancies between their physical development and their emotional and social age may lead to adaptational problems at adolescence which may require psychological counselling.
AN EPIDEMIOLOGICAL STUDY OF HYPOSPADIAS IN SWEDEN
Authors: KÄLLÉN BENGT , WINBERG JAN
Published: January 2008

The prevalence at birth of hypospadias started to increase in the beginning of the 1970s, as judged from the registration in the Swedish Register of Congenital Malformations. Further studies supported the notion that the increase was real and not due to registration artifacts. A study of the geographical distribution showed an uneven distribution in the country. Three case‐control studies with different design were performed in order to find an aeti‐ological factor which could explain the observations. None was found. No support could be obtained for a role of gestagens in the aetiology of hypospadias. A hypothesis is presented based on an observation of a possibly increased infertility—possibly male—in the couples who had a boy with hypospadias. The possible implications of this finding for aetiological studies are discussed in the light of current literature. Based on a possible male subfertility, a hypothesis is presented which could explain the noticed increase in prevalence of hypospadias in the early seventies.

The hypospadiac infants were analysed from various points of view. There were signs of a generally disturbed fetal development. Thus a shorter than normal gestational age with a component of growth failure was found. There was a statistically significant association, not only with other genital malformations such as retentio testis, but also with congenital heart disease, cleft lip/palate, anal atresia, and neural tube defects. The analysis indicates that this association was not random, but possibly indicates common teratogenic pathways.
1 Epidemiological Aspects of Gonorrhoeal and Chlamydial Infection. Local Effects of Silver Nitrate and Hexarginum
Authors: WAHLBERG V. , KALLINGS I. MOBERG , WINBERG J.
Published: January 2008

Since the Credé method was introduced for prophylaxis against gonococcal ophthalmia, there have been great changes with regard to prevention, diagnosis and treatment of this potentially dangerous disease, which warrants a reconsideration of its general application. In this study we report firstly on the incidence of maternal gonorrhoeal and Chlamydial infection at the time of delivery as a basis for evaluating the risk of neonatal infection. Secondly we report on pain reactions as well as on the frequency of conjunctivitis following different kinds of prophylaxis or placebo. The investigation included more than 1 000 mother‐infant pairs delivered in 1978. Eye prophylaxis was given with 1 % silver nitrate, 10 % Hexarginum (a less irritating silver compound), or physiological saline as placebo, in a randomized double‐blind fashion. Cultures for gonorrhoea were made on 1 027 women and for Chlamydia on a sub sample of 165 women. Observations for pain reactions and for irritation/conjunctivitis were made on 627 infants. Conjunctival samples for cultures were taken from 156 infants with conjunctivitis. Conjunctival samples for Chlamydia cultures were taken from 250 healthy infants and from 15 infants with long‐lasting conjunctivitis. Maternal gonococcal infections were observed in 111 027 women and maternal Chlamydia infections in 1/165 women in the sub sample; in both instances the infections were asymptomatic. There were no cases of gonococcal ophthalmia, but among 15 infants who showed a long‐lasting conjunctival irritation, Chlamydia was isolated in two. The observation of pain reaction showed that silver nitrate induced significantly more violent reactions than Hexarginum and saline. Almost 40 % of the silver nitrate infants had a heavy purulent secretion during days 1–3 post partum, but only 2 % of the other two groups. The generally applied prophylaxis against gonococcal ophthalmia with silver nitrate does not fulfil the requirements for general prophylaxis. It is suggested that the Credé method should be abandoned but that there should be compulsory notification of diagnosed cases of gonococcal ophthalmia.
A COMPARATIVE STUDY OF VARYING PROTEIN INTAKE IN LOW BIRTHWEIGHT INFANT FEEDING
Authors: SVENNINGSEN N.W. , LINDROTH M. , LINDQUIST B.
Published: January 2008

Svenningsen, N.W., Lindroth, M. and Lindquist, B. (Department of Paediatrics, University of Lund, Sweden). A comparative study of varying protein intake in low birthweight infant feeding. Acta Paediatr Scand. Suppl. 296: 28, 1982. — In a prospective longitudinal study of 48 very low birthweight and preterm infants with mean birthweight 1 385±343 and gestational age 30.8±2.9 w an assessment was made of the impact of varying the protein intake in the postnatal period from the 3rd to 7th week of life. The infants were randomly allocated to one of three dietary groups with isocaloric energy supply but different protein content—i.e. human milk (1.6 g/100 kcal), formula 1 (2.3 g/100 kcal) and formula 2 (3.0 g/100 kcal). In the human milk group 12 of 18 infants were fed their own mother's breastmilk. During the study period the mean weight gain was slightly higher in the infants fed formula 1 and 2. There were no group differences in S‐albumin whereas B‐urea‐N and B‐base deficit were significantly increased in the formula fed infants in comparison to infants fed human milk. After the study period until around 15 weeks of age the slope in weight gain remained slightly higher for formula fed infants. However, the gain in body length and head circumference was equal in all three groups. After around 8 months of age there was no difference in any growth parameter. Neurodevelopmental examinations showed no group differences during the follow‐up period to 2 years of age.
RESERVOIR AND EMPTYING FUNCTION OF THE STOMACH OF THE PREMATURE INFANT
Authors: CAVELL B.
Published: January 2008

Cavell, B. (Department of Paediatrics, University of Lund, Sweden). Reservoir and emptying function of the stomach of the premature infant. Acta Paediatr Scand, Suppl. 296: 60, 1982. — Gastric emptying of meals of human milk and infant formula was studied in 11 healthy preterm (AGA) infants using a marker dilution technique. Gastric emptying rate and gastric half‐emptying time were compared with data from 17 healthy infants aged 4 weeks to 6 months. The amounts of human milk emptied from the stomach per 0.1 m2 of body surface area were 19.4±4.5 ml in preterm infants and 29.5±4.6 ml in term infants. The corresponding amounts with meals of infant formula were 13.8±2.8 and 22.7±4.2 ml, respectively. With the volumes given gastric half‐emptying times for meals of human milk and infant formula were 25.1±11.5 and 51.9±9.8 min, respectively, in preterm infants. The corresponding half‐emptying times for term infants were 48±15 and 78±14 min, respectively. The differences in gastric emptying rate as well as in gastric half‐emptying time between the two meals were significant (p<0.01) both in preterm and term infants.
FAT ABSORPTION IN LOW BIRTHWEIGHT INFANTS
Authors: REY J. , SCHMITZ J. , AMEDEE‐MANESME O.
Published: January 2008

Rey, J., Schmitz, J. and Amedee‐Manesme, O. (Division of Paediatric Gastroenterology and Nutrition, Department of Paediatrics, Hôpital des Enfants‐Malades, Paris, France). Fat absorption in low birthweight infants. Acta Paediatr Scand, Suppl. 296:81, 1982.—Studies on the incomplete fat absorption in newborns, particularly preterm ones, are reviewed. In the first week term breastfed newborns have a fat absorption coefficient of 91%. Lyophilization or pasteurigation reduces this figure in term as well as in preterm newborns. Excess Ca++ lowers fat absorption. So far, there is no evidence of immaturity of intracellular fat transport through the enterocyte. In the newborn low pancreatic lipase activity and low intraluminal bile salt concentrations appear to limit fat absorption. The most effective method to improve fat absorption in preterm babies seems to substitute part of the milk fat by medium‐chain triglycerides.
TWO EPIDEMICS OF WHOOPING COUGH IN FINLAND 1976–1977; age and vaccination of the patients, DPT vaccination rate in children in the epidemic areas, home exposure attack rates and symptomless bacteria carriers.
Authors: Riitta Huovila
Published: January 2008

During a whooping cough epidemic in the region of Alavus and Töysä in 1976–1977 Bordetella pertussis strains were isolated from 51/121 (42%) patients showing symptoms of respiratory tract infection. Of these patients, 49 were children and two were adults. Among the children 42/49 (86%) were of school‐age. Vaccination was incomplete in 46 children. Of the children in this region 2.1% were unvaccinated. Basic DPT vaccination was incomplete in 6.2% and booster vaccination was lacking in 73.7% of children±4 years of age.

In 1977, 40/71(56%) of the suspected whooping cough patients in Soukka were found to be culture‐positive. Thirty patients were children and 10 were parents of these. 20/30 (66.7%) children were incompletely and 10/30(33.3%) completely vaccinated. The children given complete basic vaccination fell ill one year, those given also booster vaccination 2–3 years after the last injection. Of the children in Soukka 0.7% were unvaccinated. Basic DPT vaccination was incomplete in 2.9% and DPT booster vaccination was lacking in 38.8% of children±3 years of age. Among those exposed in the home 16/58(28%) children and 80/102(78%) adults remained healthy. Two children and two adults were symptomless bacteria carriers.

Whooping cough in children of pre‐school age is accounted for by lack of DPT booster vaccination. In addition, the pertussis vaccine used before 1976 gave protection of short duration against whooping cough.
THE EFFECT OF EARLY ERYTHROMYCIN TREATMENT ON THE INFECTIOUSNESS OF WHOOPING COUGH PATIENTS.
Authors: Riitta Huovila
Published: January 2008

The effect of early erythromycin treatment was studied in 63 whooping cough patients. They were all culture‐positive in the catarrhal stage and/or two weeks or less after the onset of coughing. 28 patients were treated with eryhtromycin for 7–10 days: children with a dose of 40 mg/kg/day and adults with 1g/day. 35 patients received no antibiotics. 152 Bordetella pertussis cultures were obtained from the untreated patients and 70 from the patients treated with eryhtromycin.

The erythromycin‐treated patients were culture‐negative on average 1‐2 weeks earlier than the untreated patients. Of the patients treated with erythromycin, 6/28 (21%) were still culture‐positive after treatment. This study shows that erythromycin treatment may reduce the period during which patients are infectious and that the treatment should be started two weeks from the onset of coughing, or preferably earlier.
FAT ABSORPTION IN THE NEWBORN
Authors: HARRIES J. T.
Published: January 2008

Harries, J. T. (Institute of Child Health and Hospital for Sick Children, Great Ormond Street, London). Fat absorption in the newborn. Acta Paediatr Scand, Suppl. 299: 17, 1982.—Dietary fat is an important source of energy to the newborn. Pancreatic lipase and esterase activity are both low in newborns, particularly in premature ones; similarly, bile salt metabolism is markedly compromised. This could result in grossly defective intraluminal lipolysis and micellar solubilisation, leading to appreciable steatorrhea and energy loss. Lingual lipase and bile salt‐stimulated lipase (BS‐SL), however, play an important compensatory role. Lingual lipase is secreted from branching serous glands (von Ebner's glands) located on the dorsal aspect of the posterior region of the tongue, and its physicochemical properties are highly suited to passage through the stomach into the small intestine; a high fat diet and the act of suckling stimulates this enzyme's activity, and it may act in a synergistic fashion with pancreatic lipase. BS‐SL (i.e. milk lipase) also has properties which are well designed for promoting fat absorption in the newborn. For example, it has no positional specificity for the ester bonds of the triglyceride molecule, and the optimal concentration of bile salts required for activation are low.