bluuebunny/crossref_metadata_2025 huggingface dataset:
ANTIMICROBIAL CHEMOTHERAPY IN CYSTIC FIBROSIS PATIENTS
Authors: HØIBY NIELS , FRIIS BIRGITTE , JENSEN KLAUS , KOCH CHRISTIAN , MØLLER NIELS ERIK , STØVRING SØREN , SZAFF MARTHA
Published: January 2008

Høiby, N., Friis, B., Jensen, K., Koch, C, Møller, N.E., Støvring, S. and Szaff, M. (Statens Seruminstitut, Departments of Clinical Microbiology at Rigshospitalet and Hvidovre Hospital, and Paediatric Department TG, Rigshospitalet, Copenhagen, Denmark). Antimicrobial chemotherapy in cystic fibrosis patients. Acta Paediatr Scand 1982; suppl 301: 75‐100. — Every effort should be undertaken to combat recurrent and chronic bacterial respiratory tract infections in patients with cystic fibrosis because infections are the main pathogenetic factors of lung damage in these patients. The principles and antibiotics used to treat bacterial infections in the Danish Cystic Fibrosis centre are outlined. The chemotherapy is based on microbiological diagnosis of secretions from the lower respiratory tract. S. aureus infections are efficiently treated with oral fusidic acid in combination with isoxazolyl penicillins or rifampicin in case of penicillin allergy or methicillin‐resistant S. aureus. H. influenzae infections are treated efficiently with oral pivampicillin or amoxicillin or with erythromycin sometimes in combination with rifampicin in case of penicillin allergy or ampicillin‐resistant H. influenzae. As a result of the efficient chemotherapy S. aureus and H. influenzae infections are now minor problems generally not related to prognosis of cystic fibrosis patients. Chronic P. aeruginosa infections are treated regularly four times per year with intravenous anti‐pseudomonas β‐lactam antibiotics in combination with tobramycin. P. aeruginosa is rarely permanently eradicated by this treatment, but significantly improved survival of the patients is obtained. The problems of resistant bacterial strains, allergy, and other side effects caused by the antibiotics are discussed.
CYSTIC FIBROSIS IN DENMARK IN THE PERIOD 1945‐1981 EVALUATION OF CENTRALIZED TREATMENT
Authors: NIELSEN OLE HAAGEN , SCHIØTZ PETER OLUF
Published: January 2008

Nielsen, O. H. and Schiøtz, P. O. (Paediatric Department TG, Rigshospitalet, Copenhagen, Denmark). Cystic fibrosis in Denmark in the period 1945‐1981. Acta Paediatr Scand 1982; suppl 301: 107‐119. — The present study gives a re‐evaluation of the course of the prognosis for cystic fibrosis (CF) in Denmark in the period January 1, 1945 — September 15, 1981. A total of 490 patients fulfilled the criteria of entry.

Despite a rather constant annual incidence, the number of surviving CF patients is highly increasing: thus 31 patients were alive as on December 31, 1960, 119 as on December 31, 1970, and 223 as on December 31, 1980. The survival was further evaluated using the decrement method, where the patients are entered by time of birth or time of diagnosis, and the Warwick & Monson method, where the patients are entered by time of diagnosis. It applies to both methods that the underlying patient populations did not include patients with meconium ileus and patients with a retrospective or autopsy diagnosis. The probability of reaching the age of 10 years was 52% for CF patients treated exclusively in departments other than the CF centre as compared to 84% for the CF centre patients.

We conclude that the centralized treatment should be preserved for a so relatively uncommon disease as CF, both because our survival curves demonstrated the prognosis to be by far the better in the patients treated at the national CF centre and because centralized treatment alone can offer appropriate research conditions.
HLA ANTIGENS IN INTESTINAL COW'S MILK ALLERGY
Authors: VERKASALO M. , KUITUNEN P. , TIILIKAINEN A. , SAVILAHTI E.
Published: January 2008

ABSTRACT. The histocompatibility antigens of one hundred patients with a severe form of cow's milk allergy were studied. HLA‐A and B locus antigens were identified in all patients, C locus antigens in 62 patients and DR locus antigens in 41 patients. The A, B and C locus antigen frequencies were compared with those of healthy blood donors, and DR locus antigen frequencies with those of healthy unrelated volunteers and cadaver kidney donors. The series included six patients with concomitant coeliac disease, who were treated as a separate group. No statistically significant differences between the patients and controls were observed, but suggestive differences became apparent when the patient group was divided into subgroups according to the presence or absence of certain co‐existing conditions, and the severity of the initially observed intestinal lesion. It is concluded that several factors contribute to the pathogenesis of intestinal cow's milk allergy, and that in some cases genes linked to the HLA region may play a role.
EFFECT OF PHYSICAL TRAINING ON EXERCISE‐INDUCED BRONCHOCONSTRICTION
Authors: HENRIKSEN J. M. , NIELSEN T. TOFTEGAARD
Published: January 2008

ABSTRACT. A 6‐week period of physical training of 28 asthmatic children reduced the exercise‐induced percentage fall in peak expiratory flow (from baseline) from 44±4% (mean ± SE) to 30±4%. Resting pulmonary function was unchanged. Resting and maximum heart rate (submaximal treadmill running) and postexercise plasma lactate were all lowered by training. The individual values for exercise‐induced bronchoconstriction and postexercise plasma lactate correlated positively before and after training. Furthermore, the training‐induced reduction in these parameters correlated. In 14 asthmatic children, who served as controls, exercise‐induced bronchoconstriction and physical fitness remained unchanged during a comparable period. The study demonstrates a beneficial effect of endurance training on exercise‐induced bronchoconstriction and working capacity.
IMMUNE RESPONSES IN PATIENTS WITH OBSTRUCTIVE JAUNDICE OF INFANCY
Authors: LARCHER V. F. , VEGNENTE A. , PSACHAROPOULOS H. T. , MOWAT A. P. , MACDONALD A. , EDDLESTON A. W. L. F. , WILLIAMS R. , VERGANI D.
Published: January 2008

ABSTRACT. To investigate possible involvement of immune responses in the pathogenesis of obstructive jaundice in infancy we measured antibody to liver specific lipoprotein (LSP) by radio immunoassay and immune complexes by their ability to bind Clq in sera from 16 patients with extrahepatic biliary atresia and 16 with neonatal hepatitis and 13 age matched controls. Anti‐LSP was present in 6 of 16 with preoperative biliary atresia and 6 of 16 with hepatitis. Mean percentage Clq bound was higher in hepatitis (22 SD 15 %) than preoperative biliary atresia (11.1 SD 2.3 %). Nine of 16 hepatitis patients had elevated Clq binding as compared with 1 of 16 with biliary atresia. The highest value for anti‐LSP and Clq binding were found in sera from patients with histologically severe hepatitis and hepotitis associated with specific viral or bacterial causes. Anti‐LSP was significantly raised 5 months post‐operatively in all of 6 patients with biliary atresia and poor biliary drainage but only 2 of 5 survivors. Elevated Clq binding was detected in 6 of 7 with poor drainage and 1 of 7 survivors at the same stage. Anti‐LSP and Clq binding fell in 4 patients with neonatal hepatitis on recovery. These findings suggest that immunological mechanisms, possibly involving antibody to hepatocyte membrane components and immune complexes, may be involved in the pathogenesis of progressive liver disease in biliary atresia.
“BASOLATERAL” AND MITOCHONDRIAL MEMBRANE TRANSPORT DEFECT IN THE HEPATOCYTES IN LYSINURIC PROTEIN INTOLERANCE
Authors: RAJANTIE JUKKA , SIMELL OLLI , PERHEENTUPA JAAKKO
Published: January 2008

ABSTRACT. In lysinuric protein intolerance, diamino acid transport is defective at the basolateral membrane of the jejunal and renotubular epithelia. The plasma has depressed concentrations of ornithine and arginine but, in contrast, supranormal levels of another urea cycle intermediate, citrulline. The patients have “ornithinopenic” postprandial hyperammonemia. The concentrations of the diamino acids and citrulline in the patients’liver were normal or elevated rather than depressed. Their extrarenal plasma clearance of citrulline and its conversion to arginine and ornithine were retarded. A hypothesis reconciles these apparently conflicting findings: in the hepatocytes the transport defect is located in the “basolateral” cell membrane and in the mitochondrial membranes. The diamino acids accumulate in the cytoplasm, because exit from the cells and transport into the mitochondria are impaired, but these acids are depleted in the mitochondria, where ornithine is needed in the urea cycle. As a result, the urea cycle is reversed at this point, producing citrulline.
RENAL FUNCTION AND BIOPSY CHANGES DURING THE COURSE OF HENOCH‐SCHÖNLEIN GLOMERULONEPHRITIS
Authors: LINNÉ TOMMY , APERIA ANITA , BROBERGER OVE , BERGSTRAND ANDERS , BOHMAN SVEN‐OLOF , WASSERMAN JERZY
Published: January 2008

ABSTRACT. Renal function studies were performed in 18 subjects in different stages of Henoch‐Schönlein glomerulonephritis (HS GN). Nine children were serially investigated, and nine adolescents or young adults, who were considered to have clinically recovered, were investigated only once, 10.5–14 years after the onset. Inulin and PAH clearance, as well as sodium excretion, were determined during hydropenia (HP) and 3% volume expansion (VE) with isotonic saline. In most patients in the former group a renal biopsy was performed during the first investigation and again one year later. The early disturbances in renal function resembled those we have found in other types of GN. The GFR was normal during HP or after VE in most cases one year after the onset. The natriuretic response to VE was decreased in most patients initially, and this was found to persist in half of the patients 2‐3 years after the onset. Pathological urinalyses then indicated disturbances in the renal handling of sodium. A reduced capacity to excrete sodium, however, did not seem to be of prognostic significance since all patients, except one who developed renal insufficiency and hypertension, had normal urinalyses and blood pressure six years after the onset. This study provides no evidence that subjects with previous HS GN will later develop impaired renal function or be predisposed to hypertension.
INITIAL SYSTOLIC TIME INTERVALS AS PREDICTORS OF THE SEVERITY OF TRANSIENT TACHYPNEA IN TERM NEONATES
Authors: HEINONEN K.
Published: January 2008

ABSTRACT. 42 term neonates with transient tachypnea (TTN) underwent echocardiography and determination of systolic time intervals before the age of 4 hours. Based on initial measurement of right ventricular systolic time intervals (RVSTIs) the patients were divided in two groups: neonates with RVSTI ratios <0.50 (Group I) (n=35) and neonates with RVSTI ratios >0.50 (Group II) (n=7). Group II neonates also had significantly more prolonged left ventricular systolic time intervals (LVSTIs) than Group I neonates. Group II neonates developed markedly more severe form of TTN than Group I neonates. Initially prolonged RVSTI was best predictor of the development of severe TTN (relative risk ratio 17.5, p<0.001): clinical characteristics and oxygen requirements at the admission had limited predictive value.
SURGICAL TREATMENT OF ULCERATIVE COLITIS IN CHILDREN
Authors: HAFFNER JON , RØ JOHS. S. , ALBRECHTSEN DAGFINN
Published: January 2008

ABSTRACT. Twenty‐four children below the age of 16 were operated on for ulcerative colitis at Rikshospitalet during the 20‐year period 1960‐79. Fifteen were treated primarily with colectomy and ileo‐rectal anastomosis (IRA), nine with proctocolectomy and ileostomy (PCI). There were twice as many boys as girls in both groups, an equal age distribution (median 13 1/2 years for IRA, 14 1/2 for PCI), and identical median duration of disease (3 1/2 years). Twenty‐three of the 24 patients were below the expected weight and/or height for their age, 6 in each group were more than 2 SD. below. Six patients with IRA had been treated with steroids, compared with 5 with PCI. Six patients with IRA had normal rectal mucosa, 2 mild, five moderate and one severe proctitis. One had a rectal stricture. Two of the patients with IRA required immediate reoperation, and one subsequently died because of leakage at the anastomosis and septicaemia. One further patient, who required immediate proctectomy because of bleeding, also died of septicaemia. Six other IRA patients have required proctectomy later because of proctitis. One has died of rectal carcinoma. Five IRA patients are alive with intact rectum, but 2 require treatment for proctitis. None of the patients with PCI are dead, one has required an external revision of the ileostomy, otherwise the primary treatment has been completely successful. None have stomal, urinary or sexual dysfunction. It is concluded that colectomy with IRA gives poorer results than proctocolectomy with ileostomy in children, just as it does in adults. The results of PCI are so good that the present paediatric prejudice against surgical treatment seems quite unjustified.
PERCEPTUAL, MOTOR AND ATTENTIONAL DEFICITS IN SEVEN‐YEAR‐OLD CHILDREN
Authors: RASMUSSEN P. , GILLBERG C.
Published: January 2008

ABSTRACT. 42 children representative of Swedish urban seven‐year‐olds with a combination of various minor neurodevelopmental deficits in the form of so‐called minimal brain dysfunction (MBD) syndromes were compared with 51 children of the same age without such problems as regards general health data from parents’interviews and questionnaires and from paediatric examinations. The findings at the physical examination revealed only few and small differences concerning minor physical anomalies that, in part, is dealt with in a separate report. Children with MBD had experienced simple febrile convulsions significantly more often than controls, and an abnormal EEG was a common finding in these cases. So‐called psychosomatic complaints were not overrepresented in the MBD group, but enuresis and encopresis was somewhat more common. The parents’answers to a set of 6 questions (concerned with late speech development, late motor development, gross motor clumsiness, fine motor clumsiness, “shuffling” and difficulties to concentrate) were found to have a high discriminating capacity detecting for MBD syndromes. The possibility of a clinical application of this finding is discussed.
TRANSIENT CONGENITAL HYPOTHYROIDISM IN AN INFANT WITH CONGENITAL NEPHROSIS OF FINNISH TYPE
Authors: TRIMARCHI F. , GEMELLI M. , BENVENGA S. , GENOVA R. , LUCA F. DE
Published: January 2008

ABSTRACT. Congenital hypothyroidism was detected on routine neonatal screening in an infant with congenital nephrosis of Finnish type and followed up for 3 years. The hypothyroidism was transient; clinical and biochemical signs and symptoms disappeared after a 20‐month period of thyroid replacement therapy and partial improvement of nephrotic symptoms.
ORAL HYDRATION OF DIARRHOEAL DEHYDRATION
Authors: SABERI MOHAMMAD S. , ASSAEE MEHDI
Published: January 2008

ABSTRACT. Two groups of infants aged 2 to 20 months with moderate to severe dehydration were randomly assigned to either sucrose high sodium (90 mEq/l) or sucrose low sodium (58 mEq/l) solution in a double blind manner. Rehydration was assessed on clinical grounds and confirmed by serial determination of body weight, hematocrit, total serum protein and blood urea nitrogen. Twenty (80%) of 25 patients on sucrose high sodium solution and 20 (77%) of 26 patients on sucrose low sodium solution were successfully hydrated. Only the assigned sucrose‐electrolyte solution was given during the average rehydration period of about 7 hours when the serum electrolytes were remeasured. Three patients on high sodium solution developed mild hypernatremia. Slight hyponatremia was encountered in 2 patients on low sodium solution. Purging rate was significantly higher in patients who failed as compared to those who succeeded. The results of this study suggest that oral sugar electrolyte solution with sodium concentration of 90 mEq/l is safe and effective in the majority of infants with diarrhoeal dehydration of diverse causes. However, intravenous fluids must be available particularly for those with a high purging rate as a significant number of them may fail.
METACHROMATIC LEUKODYSTROPHY AND PSEUDOARYLSULFATASE A DEFICIENCY IN A DANISH FAMILY
Authors: TØNNESEN T. , BRO P. V. , NIELSEN K. BRØNDUM , LYKKELUND C.
Published: January 2008

ABSTRACT. A child with a diagnosis of lateinfantile metachromatic leukodystrophy (MLD), and a normal father with low arylsulfatase A (ASA) activity in leucocytes and cultured fibroblasts is described. The child had a pathologically increased amount of sulfatides in the urine, whereas no sulfatides could be found in the father's urine. Sulfatide‐loading of the child's cultured fibroblasts showed an accumulation of sulfatides, whereas the fibroblasts from the father had a marginally decreased sulfatide turnover. It is thus possible to discriminate between these two forms of low ASA activity in this family, and to ensure a correct diagnosis should the amniotic fluid cells show a low ASA activity in future pregnancles.
EARLY CLOSURE OF PATENT DUCTUS ARTERIOSUS WITH INDOMETHACIN IN PRETERM INFANTS WITH IDIOPATHIC RESPIRATORY DISTRESS SYNDROME
Authors: KÄÄPÄ PEKKA , LANNING PETER , KOIVISTO MAILA
Published: January 2008

ABSTRACT. Thirty‐seven preterm infants with idiopathic respiratory distress syndrome were prospectively studied for the effect of the early closure of patent ductus arteriosus with indomethacin on the course of idiopathic respiratory distress syndrome. Serial retrograde aortograms were performed in all infants in order to visualize the ductus arteriosus, apart from three patients, who died early and were evaluated aortographically only once. The ductus was initially open in 27 infants and closed in 10 infants. The infants with open ductus arteriosus were randomly divided into two groups. The first group consisted of 13 infants, in whom the ductus was closed with indomethacin at a median age of 18 hours. The other 14 infants served as controls. Total time on assisted ventilation and duration of exposure to additional oxygen were significantly shorter in medicated infants than in controls. Oxygenation of infants with an initially closed ductus was better from birth and duration of their ventilatory assistance and oxygen exposure were shorter than in infants with initial ductal shunting. The data suggest that the early closure of the patent ductus arteriosus with indomethacin in distressed preterm infants has a favourable effect on the course of idiopathic respiratory distress syndrome.
SYSTOLIC TIME INTERVALS IN CHILDREN WITH HEART DISEASE
Authors: HEDVALL G.
Published: January 2008

ABSTRACT. Of the systolic time intervals, the preejection period is known to correlate well with invasively measured isometric contraction time, and increase of the quotient preejection period/left ventricular ejection time (PEP/LVET) is considered to be of a good indicator of left ventricular failure. The different systolic time intervals have been recorded from the carotid pulse curve from 40 normal children, 20 aged five and 20 aged ten years. Their PEP/LVET was 0.31 ± 0.04. Seventy‐eight children with different heart diseases were then investigated. In patients with congenital total heart block or extrasystoles, there was a negative correlation between PEP/LVET and the R‐R interval, in accordance with the Frank‐Starling law. In patients with ventricular septal defects PEP/LVET differentiates between small and large shunts; the increased PEP/LVET of the latter normalizes after operation. The increased PEP/RVET of children with transposition of the great arteries is an expression of the inadequacy of the right ventricle as a systemic chamber. In aortic stenosis “normalization” of a previously decreased PEP/LVET may indicate early left ventricular failure. In primary myocardial disease registration of the systolic time intervals enables us to follow the left ventricular function more closely than is possible with invasive techniques.
VITAMIN D METABOLISM IN PRETERM INFANTS
Authors: SALLE B. L. , GLORIEUX F. H. , DELVIN E. E. , DAVID L. S. , MEUNIER G.
Published: January 2008

ABSTRACT. In order to evaluate after birth the changes in circulating vitamin D metabolite levels in preterm babies supplemented with vitamin D (2100 I. U./d), the serum concentration of 25‐hydroxyvitamin D [25‐OHD] and 1 α,25‐dihydroxy vitamin D [1, 25(OH)2D] were measured in 22 infants (31 to 35 weeks of gestation) from birth up to 96 hours of age. Compared to cord blood levels, serum calcium decreased significantly during the first 24 hours of life (p<0.005) and remained low until day 4. Serum immunoreactive parathyroid hormone (iPTH) levels increased from birth to 24 hours and then plateaued. The 25‐OHD levels at birth were 27.5±2.5 nmol/l and increased to 67.5±12.5 nmol/l (p<0.005) during the four days of the study. During the same period, the 1, 25(OH)2D serum levels increased steadily from 84<7 to 343<105 pmol/l (p<0.005). At all times, there was a positive correlation between 25‐OHD levels and those of 1, 25(OH)2D. Our data demonstrate that in preterm infants after 31 weeks of gestation, absorption and activation of vitamin D is present as soon as 24 hours after birth and that early neonatal hypocalcemia is unlikely to be caused by an impairment of either PTH secretion or vitamin D activation.
MALE PSEUDOHERMAPHRODITISM DUE TO DEFICIENCY OF TESTICULAR 17‐KETOSTEROID REDUCTASE
Authors: LEINONEN P. , DUNKEL L. , PERHEENTUPA J. , VIHKO R.
Published: January 2008

ABSTRACT. A 12.9 year‐old girl, genotypically 46, XY, and considered to have a testicular feminization syndrome, developed signs of virilization and gynaecomastia. Very high androstenedione concentrations (10‐fold the mean of the reference interval in boys) in relation to low normal testosterone in peripheral serum indicated a 17‐ketosteroid reductase deficiency. In addition to androstenedione, the basal peripheral levels of 17‐hydroxyprogesterone and estrone were increased, being 5‐ and 3‐fold the mean of the reference interval, respectively, whereas pregnenolone, progesterone, dehydroepiandrosterone, 5α‐dihydrotestosterone and estradiol concentrations were within pubertal stage‐appropriate reference intervals. The total spermatic vein serum steroid concentrations were about 5‐fold the mean in old men, and androstenedione, estrone and dehydroepiandrosterone were particularly elevated, whereas estradiol was normal and testosterone subnormal by a factor of 1/8. In the testis tissue, the concentration of androstenedione was extremely high, whereas that of testosterone tended to be relatively low. Our patient was obviously producing testicular steroids at her maximal rate, because no response to hCG administration was observed. This state was assiociated with a high‐normal circulating LH concentration. The concentration of testicular LH/hCG receptors was only one‐fifth of that seen in old men, which may have resulted from receptor down‐regulation associated with a high degree of stimulation.
DEOXYCHOLIC AND SULPHOLITHOCHOLIC ACID CONCENTRATIONS IN SERUM DURING INFANCY AND CHILDHOOD
Authors: FINNI KALEVI , SIMILÄ SEPPO , KOIVISTO MAILA , KOUVALAINEN KAUKO
Published: January 2008

ABSTRACT. The concentrations of the two secondary bile acids (deoxycholic (DCA) and sulpholithocholic (SLCA) acid) were determined by radioimmunoassays in the serum of infants and children at ages ranging from 1 hour to 15 years. The same bile acids were measured also in the umbilical cord serum. The concentrations of the secondary bile acids in the serum of 1‐hour old infants corresponded to those in the umbilical cord serum. Secondary bile acid serum concentrations were after the age of 7 days and up to the age of 3 to 6 months significantly lower than those in the umbilical cord serum. After the age of 6 months a significant increase in DCA serum concentrations could be shown. During the first 6 months of life DCA concentrations were clearly lower than those of SLCA. Our observations suggest that in the perinatal period DCA is mainly of maternal origin and that an alternate hepatic pathway may exist for the synthesis of lithocholic acid in early infancy.
DEVELOPMENT OF GLOMERULAR FILTRATION RATE AND EXCRETION OF β2‐MICROGLOBULIN IN NEONATES DURING GENTAMICIN TREATMENT
Authors: ELINDER G. , APERIA A.
Published: January 2008

ABSTRACT. Ten infants of different gestational ages (GA) and postnatal ages (PNA), treated with gentamicin, were compared with 10 control patients of similar GA and PNA to evaluate the possible nephrotoxic effects of this drug. Changes in the glomerular filtration rate (GFR) and the fractional excretion of β2‐microglobulin in urine (FEβ) were used as indicators of renal dysfunction. In the control infants there was a postnatal increase in the GFR that was higher in full‐term than in preterm infants. The FEβ decreased logarithmically as a function of both the GA and the PNA. The GFR was statistically lower in 5/10 and 6/10 of the patients on the first and the last days of gentamicin treatment (GT) respectively. Three weeks after GT, 8/10 had a normal GFR. The FEβ was statistically higher in 4/10 of the patients on their first day of GT and 7/10 on their last day of GT. Three weeks after GT, 9/10 of the patients had a normal FEβ for their postnatal and gestational ages. It is concluded that GT influences filtration and proximal reabsorption in GT infants by decreasing the GFR and increasing the FEβ. However, the observed renal dysfunction seemed to be reversible.
CORD PLASMA HYPOXANTHINE AS A MEASURE OF FOETAL ASPHYXIA
Authors: THIRINGER K.
Published: January 2008

ABSTRACT. The clinical value of cord plasma hypoxanthine (HX) determination as a measure of foetal asphyxia was investigated. HX levels found in mixed cord plasma from 50 infants with clinical signs of perinatal asphyxia were compared to the levels found in 29 infants delivered after risk pregnancies and in 62 control neonates. All infants were assessed by three clinical scores: Apgar score, a clinical asphyxia score and perinatal reduced optimality score. In half of the infants cord blood pH, lactate and base excess were also determined. The mean cord plasma HX level in asphyctic infants was significantly higher than the mean HX level found in the two non‐asphyxiated groups. The concentration of HX correlated significantly to the clinical asphyxia score, reduced optimality score and to cumulative Apgar score, but no correlation was found to 1 and 5 min Apgar score. Cord plasma HX also correlated significantly to pH and lactate in cord blood. However, the correlation of HX to these two laboratory measures of asphyxia was no longer significant, when infants with birth weights/gestational ages below 1.0 SD were considered. This was found to be due to the lack of lactate elevation in these infants during asphyxia. Two newborn infants with substantially elevated HX levels, with only minor signs of clinical asphyxia turned out on follow‐up with spastic diplegia. It is suggested that the assay of cord plasma HX may add additional information to previously used hypoxia parameters and may be a more reliable index of perinatal asphyxia in the growth‐retarded infant, than lactate and pH at birth.
PRETERM INFANTS FED HUMAN MILK ATTAIN INTRAUTERINE WEIGHT GAIN
Authors: JÄRVENPÄÄ ANNA‐LIISA , RÄIHÄ NIELS C. R. , RASSIN DAVID K. , GAULL GERALD E.
Published: January 2008

ABSTRACT. The adequacy of human milk for lowbirth‐weight infants remains controversial. In this study, 35 healthy preterm infants with gestational age from 31 to 36 weeks and a birth weight less than 2 200 g, were followed until they attained a weight of 2 400 g at about 5 to 7 weeks of age. These infants were fed pooled, expressed human milk, partly supplemented with their own mother's milk, at intakes of 185 and 200 ml/kg/day. There were no consistent differences between the feeding groups in the rate of gain in weight, length, or head circumference, in serum total protein, in acid‐base status, or in plasma amino acid concentrations. It is concluded that pooled, expressed human milk in volumes of 185 or 200 ml/kg/day produces a postnatal weight gain (196±6 or 205±7 g/week, respectively) in healthy preterm infants with a gestational age over 31 weeks which compares to intrauterine weight gain (207 g/week). This growth is achieved without apparent metabolic stress.
ESSENTIAL FATTY ACIDS IN PHOSPHOGLYCERIDES OF HUMAN MILK
Authors: CONDE C. , FRIAS E. CASADO DE , MORO M.
Published: January 2008

ABSTRACT. 35 milk samples from 19 women giving birth between 28 and 41 weeks of gestation were studied. The samples were collected on the 3rd, 10th and 21st postpartum days. The phosphatidylethanolamine and phosphatidylcholine fatty acids were analyzed. In this report the proportion of linoleic acid is shown to be significantly lower in colostrum from mothers giving birth prematurely in comparison to colostrum from mothers giving birth at term. No other differences were found in relation to post conception age.
ORGANOCHLORINE CONTAMINANTS IN SWEDISH HUMAN MILK FROM THE STOCKHOLM REGION
Authors: NORÉN K.
Published: January 2008

ABSTRACT. The levels of fat‐soluble organochlorine contaminants in human milk in the Stockholm region are decreasing. For p, p'‐DDT, p, p'‐DDE, and dieldrin a reduction has taken place during the last decade, but the levels of polychlorinated biphenyls (PCBs) started declining only from about 1977. Generally, the pooled samples from the Mothers' Milk Centre in Stockholm in 1980 contained lower levels of organochlorine contaminants than the current Swedish maximum acceptable limits for milk. In 2% of the samples the total DDT levels slightly exceeded the limit of 0.05 mg DDT+DDD+DDE/kg. Milk from three mothers was analysed during the nursing of two or three consecutive infants. In each case, the levels of organochlorine contaminants declined.
IMMUNOLOGICAL STUDIES IN TWO CHILDREN WITH RECURRENT PAROTITIS
Authors: FRIIS B. , PEDERSEN F. KARUP , SCHIØDT M. , WIIK A. , HØJ L. , ANDERSEN V.
Published: January 2008

ABSTRACT. In two children with recurrent parotitis, labial salivary gland biopsies showed chronic sialoadenitis. Immunofluorescence studies disclosed deposits of immunoglobulins and complement in juxta‐acinar small vessels. Case 1 had gluten enteropathy, IgA deficiency and high titres of antinuclear antibodies (ANA), and in vivo fixation of ANA to nuclei of different cells in lip, skin and jejunum was present. Case 2 showed deposition of IgM in the dermo‐epidermal junction of the skin. These findings suggest that autoimmune reactivity and immune complexes may play a role in the pathogenesis of this disorder.
DYGGVE‐MELCHIOR‐CLAUSEN DYSPLASIA
Authors: ENGFELDT B. , BUI T. ‐H. , EKLÖF O. , HJERPE A. , REINHOLT F. P. , RITZEN E. M. , WIKSTRÖM B.
Published: January 2008

ABSTRACT. The results of light and electron microscopic examination and of biochemical proteoglycan studies of costochondral and iliac crest biopsies from a recently diagnosed case of Dyggve‐Melchior‐Clausen dysplasia are reported. At light microscopy of resting cartilage large lacunae containing clusters of five or more chondrocytes were seen in some areas. In the hyaline cartilage there were scattered fibrous foci but no mineralized areas. Electron microscopy revealed chondrocytes containing widened cisternae of rough endoplasmic reticulum and vesicles coated with a smooth single‐layered membrane. The content of the cisternae and of the vesicles was amorphous. Throughout the cartilage a considerable proportion of the chondrocytes displayed more or less pronounced necrobiotic changes. The biochemical analysis showed an increased amount of glucosaminoglycans in the cartilage and indicated that the ability of proteoglycan monomers to reaggregate to hyaluronic acid chains was decreased. Our findings support the suggestion that Dyggve‐Melchior‐Clausen dysplasia is due to a disturbance in proteoglycan metabolism.
STROKE DUE TO FIBROMUSCULAR HYPERPLASIA OF THE INTERNAL CAROTID ARTERY
Authors: LLORENS‐TEROL J. , SOLE‐LLENAS J. , TURA A.
Published: January 2008

ABSTRACT. An 11‐year‐old girl developed an ischemic stroke syndrome a few minutes after she had been swimming in a swimming pool. The motor deficit of the extremities reversed within 24 hours. The patient was discharged on the 6th hospital week with a completely recuperated neurological function. The left internal carotid arteriogram showed typical “string of beads” appearance of segmental fibromuscular hyperplasia.
THE FIRST CASE OF THE SANFILIPPO TYPE C SYNDROME IN SCANDINAVIA
Authors: ARVIDSSON J. , CHESTER M. A. , HECHT H.
Published: January 2008

ABSTRACT. A Swedish patient with typical symptoms of the Sanfilippo Syndrome (Mucopolysaccharidosis III) is described. The early onset of the disease, the presence of hepatomegaly, early dementia and the absence of umbilical bernia are consistent with the subgroup Sanfilippo A. Enzyme studies indicate the diagnosis Sanfilippo C, and thus the patient represents a more severe form of this subgroup than any of the four other patients hitherto described in detail.
A FIVE‐YEAR FOLLOW‐UP STUDY ON CARDIORESPIRATORY FUNCTION IN ADOLESCENT ELITE ENDURANCE RUNNERS
Authors: ELOVAINIO R. , SUNDBERG S.
Published: January 2008

ABSTRACT. It is not known whether the superior cardiorespiratory function in adolescent athletes is attributed to the effects of training or to genetic disposition. We re‐examined 6 elite endurance runners and 7 boys with ordinary physical activity 5–6 years after the first examination. At the age of 14 years the two groups did not differ as regards height, weight, percent body fat and lean body mass (LBM). The runners had slightly superior cardiorespiratory characteristics but, with the exception of lower resting and maximal heart rates (HR) in the runners, the differences were not statistically significant. At the age of 19–20 years the runners had significantly higher maximal oxygen uptake (VO2 max) per kg body weight (74 vs. 61 ml/min/kg), VO2 max/LBM (85 vs. 74 ml/min/kg LBM), calculated work at a heart rate of 170 beats/min (W170) per kg body weight (3.9 vs. 2.7 W/kg), W170/LBM (4.5 vs. 3.3 W/kg LBM), larger relative heart volume (HV) (472 vs. 369 ml/m2) and lower resting HR (52 vs. 68 beats/min) than the controls. The runners also displayed greater increases in the growth‐corrected percentage changes in cardiorespiratory variables, but the differences did not reach statistical significance. We conclude that the relative VO2max, W170 and HV values in the runners had increased as a result of a prolonged endurance training.
RESERVE ALBUMIN AND BILIRUBIN TOXICITY INDEX IN INFANT SERUM
Authors: CASHORE W. J. , OH W. , BRODERSEN R.
Published: January 2008

ABSTRACT. Reserve albumin concentration (the concentration of albumin available for binding of unconjugated bilirubin) was determined in 95 sera from 76 subjects by dialysis with 14C‐monoacetyl diamino diphenyl sulfone (MADDS). An index, I of bilirubin toxicity in the plasma was calculated for each subject, based on the bilirubin and reserve albumin concentrations, the affinity of bilirubin for serum albumin, and the pH‐dependent solubility of bilirubin in the plasma. The values of reserve albumin and of I varied significantly with gestational age, clinical condition (whether sick or well), and serum bilirubin level. The value of reserve albumin was decreased and I was increased in association with clinical factors (e. g., hyperbilirubinemia, hypoxia, acidosis, or sepsis) recognized as increasing the risk for bilirubin encephalopathy. The lowest values of reserve albumin and the highest values of I were found in the least mature and sickest infants.
OCCULT BLEEDING FROM GIANT SMALL‐BOWEL DIVERTICULA
Authors: HELIN I. , FLODMARK P. , LINDHAGEN T. , NETTELBLAD S. ‐C. , RAUSING A.
Published: January 2008

ABSTRACT. Two cases of occult intestinal blood loss due to giant ileal diverticula are described. In one of them, ectopic gastric mucosa was found at microscopic dissection. In the other that was probably originating from a blind loop formation at a previous operation, multiple mucosal erosions, probably due to retained bowel contents, were considered responsible for the blood loss. Though giant diverticula are rare throughout the gastrointestinal tract, and usually cause symptoms other than bleeding, the importance of a careful search for this malformation in children with occult faecal blood loss is stressed.
SERUM α‐FETOPROTEIN—A BIOCHEMICAL INDICATOR OF PRENATAL HYPOTHYROIDISM
Authors: LARSSON A. , HAGENFELDT L. , BLOM L. , MORTENSSON W.
Published: January 2008

ABSTRACT. Serum α‐fetoprotein (AFP) was analysed at 10–30 days of age in infants with positive TSH screening tests for congenital hypothyroidism. These levels were significantly higher in infants with true positive screening tests than in those with false positive tests; 27 of 43 infants with congenital hypothyroidism had serum AFP levels above the age‐related reference range. The postnatal rate of elimination of AFP from serum did not differ in hypothyroid and euthyroid infants, indicating that the difference in serum AFP was due to more extensive synthesis in hypothyroid individuals. The level of serum AFP was inversely correlated with the roentgenological skeletal maturation index. It is postulated that increased serum AFP is caused by prenatal hypothyroidism and that analysis of serum AFP is a valuable tool to identify those infants with congenital hypothyroidism who are at risk of neuropsychological sequelae.
CONGENITAL HYPOTHYROIDISM AND CHANGES IN THE ENAMEL OF DECIDUOUS TEETH
Authors: NOREN J. G. , ALM J.
Published: January 2008

ABSTRACT. Deciduous teeth from children with congenital hypothyroidism were collected and ground sections were prepared. The sections were analysed by means of polarised light and microradiography. The enamel in ground sections of deciduous teeth from children with congenital hypothyroidism showed an increase in areas with elevated pore volume distribution in both the pre‐ and postnatal enamel. The findings suggest that thyroid hormone influences enamel maturation. Further, alterations in enamel structure, presumably due to prenatal thyroid deficiency, were found to be more common among children with neurological abnormalities at the age of 7 to 9 years.
TRACKING OF BLOOD PRESSURES IN DIABETIC CHILDREN
Authors: IBSEN K. KAAS , ROTNE HELLE , HOUGAARD P.
Published: January 2008

ABSTRACT. Repeated blood pressure measurements in 127 diabetic children, obtained at intervals of 6 months, were studied. Tracking of measurements described by the correlation between repeated observations of systolic blood pressure showed highest degree of tracking between consecutive measurements (0.38–0.76), while there was a lower degree of tracking between measurements at longer time intervals, and lower degree of tracking in the observations of diastolic blood pressure than of systolic blood pressure. After adjustment for changes in height and weight, the tracking correlations are slightly smaller on the whole. The tracking correlations are generally rather small and repeated measurements of blood pressure are necessary.
THE RELATIONSHIP BETWEEN CARNITINE AND KETONE BODY LEVELS IN DIABETIC CHILDREN
Authors: SOLTÉSZ G. , MELEGH B. , SÁNDOR A.
Published: January 2008

ABSTRACT. Free carnitine was significantly (p<0.001) reduced both in the ketotic (29.7±3.4 nmol/ml) and in the ketoacidotic (24.6±1.4 nmol/ml) groups when compared to controls (50.0±2.4 nmol/ml). At the same time, acylcarnitine values in the ketotic (21.2±2.4 nmol/ml) and ketoacidotic (25.4±2.3 nmol/ml) groups were significantly above the control value (4.71±0.6 nmol/ml). There was no significant difference between the two ketotic groups in carnitine derivatives. The abnormal distribution of plasma free and acylcarnitines could be reversed by insulin treatment. There was an inverse correlation between ketone body levels and free carnitine in the ketotic (r =‐0.71, p<0.02) and ketoacidotic group (r =‐0.71, p<0.05). However, there was no correlation between ketone bodies and acylcarnitine and between free carnitine and acylcarnitines. We concluded that the increased acylation was only partly responsible for the reduction of free carnitine in diabetic ketosis.
CLINICAL APPEARANCE AND OUTCOME IN MUMPS ENCEPHALITIS IN CHILDREN
Authors: KOSKINIEMI M. , DONNER M. , PETTAY O.
Published: January 2008

ABSTRACT. Forty‐one patients with mumps encephalitis examined at the Department of Paediatrics, University of Helsinki, during the period Jan. 1, 1968, to Dec. 31, 1980, were reviewed with special reference to clinical appearance and outcome. The ratio of males to females was 4: 1 and the age range 1.2 to 13.7 years. The clinical findings were high fever ±39°C in 83.0 %), impairment of locomotion and balance (36.6%), seizures (24.4%), psychic disorders (22.0%), depressed level of consciousness (19.5 %), vertigo (12.9%) and/or gastric pains (12.9 %). One patient with congenital toxo‐plasma and cytomegalovirus infections died. On leaving the hospital eight were still ataxic, one of these was not able to speak and two had difficulties in concentration. One patient was transferred to another hospital because of psychosis. At the follow‐up examination 4 to 24 months after the onset of the disease two patients were ataxic and seven suffered from behavioural disturbances. Electroencephalography (12.2 % of all and 25 % of examined patients) showed generalized slow wave disturbance at follow‐up in six patients and borderline disturbances in eight.
LEVELS OF VITAMIN D‐METABOLITES IN A CASE OF ACRO‐OSTEOLYSIS SYNDROME
Authors: SAKANO T. , HYODO S. , NISHI Y. , KOBAYASHI M. , TANAKA T. , YAMAOKA K. , SEINO Y. , USUI T.
Published: January 2008

ABSTRACT. On the initial examinations of a 17‐year‐old boy with acro‐osteolysis syndrome, the levels of serum 25‐hydroxycholecalciferol (25OHD) were low, whereas serum levels of 1,25‐dihydroxycholecalciferol (1,25(OH)2D) were normal. Administration of 1α‐hydroxycholecalciferol (1OHD) failed to improve the osteoporosis, although the serum 25OHD level rose to normal. It is unlikely that impaired bone remodeling due to abnormal vitamin D‐metabolism was a primary defect in our patient.
BENIGN PREMATURE ADRENARCHE: CLINICAL FEATURES AND SERUM STEROID LEVELS
Authors: VOUTILAINEN R. , PERHEENTUPA J. , APTER D.
Published: January 2008

ABSTRACT. 18 girls with premature adrenarche were evaluated both clinically and by serum steroid measurements. Age at first appearance of the symptoms ranged from 3.0 to 7.8 years. Clinical findings included pubic or axillary hair, acne, accelerated growth, adult‐type perspiration and oily skin or hair. Bone age was 0.3–3.2 years ahead of chronological age. 15 of these 18 girls had accelerated growth and most of these already before the appearance of pubic hair. Five girls had severe acne requiring topical treatment. Serum dehydroepiandrosterone was elevated for age in all patients. Androstenedione and testosterone correlated positively with the dehydroepiandrosterone values. Dihydrotestosterone was also elevated in many girls. Administration of dexamethasone brought about a rapid normalization of the elevated steroid levels.
UMBILICAL CORD CORTISOL AND PROLACTIN LEVELS IN PRETERM INFANTS
Authors: PROCIANOY RENATO S. , CECIN SANDRA K. G. , PINHEIRO CARLOS EDUARDO A.
Published: January 2008

ABSTRACT. The influence of labor and route of delivery upon the umbilical cord serum levels of cortisol and prolactin in ninety‐nine preterm infants not exposed prenatally to corticosteroids was studied. Vaginally born infants (group A) presented a higher mean cord cortisol concentration than those delivered by cesarean section (group B); mean prolactin values, however, were not different between both groups. Although there was no difference in cortisol and prolactin levels between infants delivered by cesarean section after spontaneous onset of labor (group B‐I) and those without labor (group B‐II), the mean cortisol concentration was significantly higher in group A than in group B‐I. The mean prolactin levels did not differ among all the studied groups. It is concluded that there is no association between presence of labor or route of delivery and cord seum levels of prolactin, there is no association between spontaneous preterm labor and cord cortisol values and there is an association between vaginal delivery and high cord cortisol levels in preterm infants. It is suggested that the increase in serum cortisol levels does not precede the initiation of preterm parturition but it is secondary to the stress caused by vaginal delivery.
TRANSEPIDERMAL WATER LOSS IN NEWBORN INFANTS
Authors: STRÖMBERG B. , ÖBERG P. Å. , SEDIN G.
Published: January 2008

ABSTRACT. In order to investigate whether sweating and cutaneous vasodilatation. Induced by a warm environment, is inhibited by central cold‐stimulation, the evaporation rate and skin blood flow were studied by non‐invasive techniques before and after the feeding of cold glucose to 17 fullterm appropriate for gestational age infants, born by elective Caesarean section, on their first day after birth. In newborn infants who were visibly sweating and had an increased skin blood flow at a body temperature above 37.1°C, such central cold‐stimulation quickly inhibited sweating and decreased skin blood flow. In infants with almost the same body temperature but with no sweating, this stimulation caused no change in evaporation rate or skin blood flow. The regulation of body temperature in fullterm newborn infants can thus be disturbed by central cold‐stimulation, resulting in a decreased heat loss to the environment even though the body temperature is elevated.
OEDEMA FLUID COMPOSITION IN CHILDHOOD DISORDERS
Authors: TRIPATHI A. M. , AGRAWAL K. K. , AGARWAL K. N.
Published: January 2008

ABSTRACT. Oedema fluid was collected from the leg through a sterile 21 gauge needle inserted into the subcutaneous space in 12 patients with protein energy malnutrition, 12 with nephrosis, 5 with Indian childhood cirrhosis, 4 with acute nephritis, 4 with epidemic dropsy and 3 with congestive heart failure. The concentrations of protein, free amino acids and electrolytes were measured in plasma and oedema fluid. The plasma/oedema fluid ratios were 36: 1, 49: 1, 32: 1 and 52: 1 in protein energy malnutrition, nephrosis, Indian childhood cirrhosis and congestive heart failure. These ratios were significantly smaller in epidemic dropsy (4: 1) and acute nephritis (21: 1). The free alpha amino nitrogen concentrations in these two compartments were almost in equilibrium. This was also found for essential and non‐essential amino acid distributions in protein energy malnutrition and nephrosis, whereas differences in amino acid patterns were found in nephritis and epidemic dropsy. Sodium and potassium concentrations varied substantially between diseases where the underlying cause was gross hypoproteinemia compared to non‐hypoproteinemic conditions.
PLASMA CONCENTRATIONS OF VITAMIN D METABOLITES IN A CASE OF RICKETS OF PREMATURITY
Authors: MARKESTAD T. , AKSNES L. , FINNE P. H. , AARSKOG D.
Published: January 2008

ABSTRACT. Rickets was diagnosed in an extremely low‐birthweight infant 16 weeks after birth. She had a normal plasma concentration of 25‐hydroxyvitamin D, a relatively low level of 24,25‐dthy‐droxyvitamin D, and a markedly elevated 1,25‐dihydroxyvitamin D level compared with adult standards. The plasma concentrations of the vitamin D metabolites were, however, indistin‐guishable from those of healthy preterm infants who received a similar diet of human milk and vitamins. The results indicate that rickets was not caused by vitamin D deficiency or by abnormal vitamin D metabolism, but by calcium and/or phosphate deficiency, and that the calcium and phosphorous content of human milk may be inappropriately low for very low‐birthweight infants.