bluuebunny/crossref_metadata_2025_split huggingface dataset:
THE INCIDENCE OF PKU IN FINLAND
Authors: VISAKORPI J. K. , PALO J. , RENKONEN O.‐V. 
Published: January 2008

SummaryGuthrie's bacterial inhibition test was applied to 71 135 newborn infants from different parts of Finland. No cases of PKU were diagnosed. In addition, 3 685 mentally retarded patients, mostly idiots and imbeciles, have been screened in Finland with the same method. With two cases of PKU among them, a prevalence of 0.54 per 1 000 is obtained. The incidence of the disease among the newborn is estimated to be less than one per 100 000.
A GIRL WITH RECURRENT INFECTIONS, LOW IgM AND AN ABNORMAL CHROMOSOME NUMBER 1
Authors: ØSTERGAARD FOUL AABEL 
Published: January 2008

SummaryA girl, now 3 years of age, is described, who since the age of 3 months had many, often severe, infections mainly caused by microorganisms with a polysaccharide structure. In addition she had very low IgM and no iso‐agglutinins in her serum, and furthermore we were able to show an abnormal chromosome number 1 in more than 80% of the cells investigated from peripheral blood. The possibility that antibodies to polysaccharide antigens are directed from chromosome number 1 is referred to in this paper.
EXCRETION OF BILE ACIDS IN EXTRAHEPATIC BILIARY ATRESIA AND INTRAHEPATIC CHOLESTASIS OF INFANCY
Authors: NORMAN ARNE , STRANDVIK BIRGITTA 
Published: January 2008

SummaryThis series included 24 infants, 16 boys and 8 girls, who were admitted to hospital with the diagnosis of obstructive jaundice. Five of the infants were subsequently found to have extra‐hepatic biliary atresia (BA) and the other 19 infants intrahepatic cholestasis of infancy (IHC).The infants were investigated given special attention to: the quantitative urinary excretion of cholic and chenodeoxycholic acids, the isotope excretion after intramuscular injection of cholic acid‐24–14C, the nature of labelled urinary bile acids, the half‐life and the pool size of cholic acid.At the first examination of the infants after admission the urinary excretion of cholic and chenodeoxycholic acids varied greatly between the patients. However, on comparing the values obtained in the two groups, it was found that there was virtually no difference between the mean daily values of cholic and chenodeoxycholic acids in urine, and the ratio cholic to chenodeoxycholic acid between the BA group and the IHC group. After the injection of isotopic cholic acid most of the isotope was recovered in the urine in all cases. In the infants with BA the faecal excretion of the isotope was low, being less than 3 per cent of the injected isotope. Out of the 19 infants with IHC the recovery of the injected isotope in faeces was also less than 3% in 11 infants. In 8 infants with IHC the faecal isotope excretion was significantly high to exclude extrahepatic biliary atresia.The first 24 hour urine specimen contained small amounts of unconjugated labelled cholic acid in all cases whereas in no case did the patients excrete unconjugated labelled cholic acid 48 hours after the injection of the isotope. No transformation of cholic acid was observed. There was no difference between the BA group and IHC group with regard to the percentage labelled glycine conjugates of total excreted urinary conjugates. Neither was there any difference between the two groups with regard to half‐life and pool size of cholic acid.There was no difference with respect to the bile acid metabolism between infants with congenital CMV infection, decreased serum concentrations of alfal‐antitrypsin and the other patients.
STUDIES IN CYSTIC FIBROSIS1: Urinary Excretion of Hexosamine, Sialic Acid and Fucose
Authors: KOLLBERG H. , LUNDBLAD A. , EKBOHM G. 
Published: January 2008

SummaryNormal values for the total urinary excretion of hexosamine, sialic acid and fucose and their ratios to creatinine in childhood were determined. The excretion of these substances in 25 CF children was slightly higher at all ages than in controls. The increase was more pronounced in higher ages and in more severely affected patients.No difference in the ratio of fucose to sialic acid was found between controls and CF patients, either in the unfractionated urine or in the ultrafiltrable or non‐ultrafiltrable fractions of urine.Zone electrophoretic fractionation of the non‐ultrafiltrable material disclosed a proportionately higher excretion of both carbohydrate‐ and protein‐containing material in CF patients as compared with controls.Two CF patients exhibited normal gel‐chromatographic distributions of fucose‐con‐taining low molecular weight carbohydrate material.
THE EFFECT OF LYSINE FORTIFICATION OF IRANIAN BREAD ON THE NUTRITIONAL STATUS OF SCHOOL CHILDREN
Authors: HEDAYAT H. , SHAHBAZI H. , PAYAN R. , AZAR M. , BAVENDI M. , DONOSO G. 
Published: January 2008

SummaryThe effect of enrichment of bread with lysine was studied on a group of Iranian village children receiving free school lunch over a period of 210 days. Two groups received typical dishes, which were purposely modified to make them limited by lysine; one with enriched and the other with non‐enriched bread. The third group did not receive lunch and served as control to assess the changes in the nutritional status of the former groups. The food intake at the homes was not controlled.Markedly greater increases in weight and mid‐arm circumference were observed in groups which received the school lunch (with or without lysine) than in the control group. Lysine enrichment showed no significant effect in improving the nutritional status except that children who received the amino acid supplementation exhibited significantly higher increments in mid‐arm circumferences. An increment in height and weight was observed in children of non‐lysine group over those who received lysine which was significant in respect to height. The latter group also showed a small but significant decrease in serum albumin.
COMBINED SUPRAPUBIC ASPIRATION AND CLEAN‐VOIDED URINE EXAMINATION IN INFANTS AND CHILDREN
Authors: ARONSON A. S. , GUSTAFSON B. , SVENNINGSEN N. W. 
Published: January 2008

SummaryIn 120 infants and children a comparison was made between suprapubic aspiration urine and concomitantly obtained clean‐voided urine.The following conclusions could be made from this study:(1) By performing suprapubic aspiration of urine, true bacteriuria could be diagnosed. 31 false positive and 8 false negative clean‐voided urine samples were disclosed.(2) Furthermore, by the combined procedure of urinary collection, it was possible to elucidate the diagnostic value of accurately performed leucocyte counting in uncentrifuged urine. In infants leucocyte counts above 250/ mm3 in clean‐voided urine and above 10/mm3 in suprapubic aspiration urine always accompanied a urinary infection, whereas the degree of leucocyturia in children was inconclusive.
EVALUATION OF THE EFFECTIVENESS OF TREATMENT ON ADULT HEIGHT PROGNOSIS IN DISORDERS WITH ADVANCED AND RETARDED BONE AGE Height and Height Velocity related to Chronological or to Bone Age
Authors: BOSSI EMILIO , JOSS ETIENNE E. , ZURBRÜGG ROLF P. 
Published: January 2008

SummaryDifferent growth parameters and their graphical presentation in disorders with advanced and retarded bone age are discussed. Their usefulness for the evaluation of the effect of treatment on growth and maturation is compared. In precocious puberty, more information is obtained when height is plotted against bone age instead of chronological age. It is more reasonable from a physiological standpoint to plot height velocity against bone age rather than chronological age. For the evaluation of the efficiency of treatment on adult height prognosis in precocious puberty, two possibilities are proposed: height velocity expressed as height increment per bone age year, or the quotient height age increment/bone age increment. However, where skeletal maturation is severely retarded, as in hypopituitary growth retardation, these growth parameters should be related in the conventional way to chronological age, rather than to bone age.
CONTINUOUS POSITIVE AIRWAY PRESSURE WITH A FACE CHAMBER IN EARLY TREATMENT OF IDIOPATHIC RESPIRATORY DISTRESS SYNDROME
Authors: AHLSTRÖM HANS , JONSON BJÖRN , SVENNINGSEN NILS W. 
Published: January 2008

SummaryCPAP treatment started early with a new face chamber was found to be convenient and, without risks. Treatment of IRDS applied early with the presented technique appears to radically improve the prognosis.
HAEMORRHAGIC CYSTITIS COMPLICATING CYCLOPHOSPHAMIDE TREATMENT IN CHILDREN
Authors: HELIN INGEMAR , OKMIAN LUDVIG 
Published: January 2008

SummaryHaemorrhagic cystitis complicating cyclo‐phosphamide treatment is a serious complication. The bladder irritation is considered to be a consequence of direct contact between bladder wall and urine containing metabolites of cyclo‐phosphamide. The early symptoms are vesical tenesmus, pollakisuria and haematuria. Endo‐scopy is the essential way of diagnosing the cyclophosphamide cystitis. High daily fluid regimen and frequent voiding must be recommended as the prophylaxis of choice. Moreover the drug must be administered as a single 24‐hour‐dose in the morning. Surgical techniques differ according to the severity of the bleeding. Administration of an anti‐fibrinolytic agent proved ineffective.
GASTRIC LIPOLYSIS OF HUMAN MILK LIPIDS IN INFANTS WITH PYLORIC STENOSIS
Authors: OLIVECRONA T. , BILLSTRÖM Å. , FREDRIKZON B. , JOHNSON O. , SAMUELSON G. 
Published: January 2008

SummaryIn gastric contents a potent lipase is present which hydrolyses the milk lipids to mainly diglycerides and free fatty acids. This hydrolysis hasearlier beendemonstratedinrats and now in human infants. The gastric lipo‐lysis may facilitate the further digestian of the milk lipids by transformation of the fat droplets to a substrate that is more readily attacked by the pancreatic lipases.
THE EFFECT OF MATERNAL AGE, PARITY AND SOCIAL CLASS ON THE INCIDENCE OF SMALL‐FOR‐DATES NEWBORNS
Authors: PAPAEVANGELOU G. , PAPADATOS C. , ALEXIOU D. 
Published: January 2008

SummaryThe incidence of S.F.D. newborns was studied in relation to maternal age, parity, and social class in a sample of 4 369 consecutive births at the “Alexandra” Maternity Hospital of Athens. The incidence of S.F.D. babies in the lower classes (IV and V) is substantially higher when compared with mothers belonging to social groups I, II or III. Furthermore, the birth of S.F.D. babies is encountered more frequently in very young as well as in women above the age of 30 years. Finally, the birth of a S.F.D. baby is more frequent in primiparous than multiparous women.
SUGAR ABSORPTION BY FLAT JEJUNAL MUCOSA
Authors: DESJEUX J. F. , SASSIER P. , TICHET J. , SARRUT S. , LESTRADET H. 
Published: January 2008

SummaryTo obtain further information on the role of jejunal mucosa in malabsorption, characteristic activities of jejunal epithelial cells (disaccharid‐ase activities and maltose and glucose absorption) were correlated with jejunal histology of 20 biopsy specimens with no villi (flat mucosa, F.M. (group I)) and 20 biopsy specimens with higher than 350 pm villi (control mucosa, C.M. (group 11). Except for lactase activity (F.M. = 13% of C.M.), the enzymatic and absorptive activities measured in F.M. specimens were uniformly about 30% of the corresponding activity in the C.M. tissue. An attempt was made to predict maltase and sucrase activities in F.M. specimens by extrapolating plots of C.M. activities vs. villous height. Measured enzyme activities in F.M. tissue, however, were significantly higher than the zero activity predicted by such plots. In contrast, the regression lines slopes of maltase or sucrase activities vs. total height (crypts and villi) measured in C.M. specimens were not different from the slopes obtained when data from C.M. and F.M. specimens were plotted together. These results suggest (i) the presence of absorptive cells in flat jejunal mucosa, which might be localised in so‐called crypts; (ii) that a reduction in the number of epithelial cells is not the single mechanism in the origin of flat mucosa; (iii) lactase activity behaves differently than other studied epithelial activities.
COW'S MILK ALLERGY: PREVALENCE AND MANIFESTATIONS IN AN UNSELECTED SERIES OF NEWBORNS
Authors: Gerrard J. W. , MacKenzie J. W. A. , Goluboff N. , Carson J. Z. , Maningas C. S. 
Published: January 2008

Summary1. A consecutive series of 787 babies born at the University Hospital were followed for 12–36 months in order to determine the prevalence of cow's milk allergy (CMA).2. Fifty‐nine babies (7.5%) were shown by repeated challenge to have evidence of CMA.3. The main manifestations of CMA were eczema in 27, recurrent diarrhea in 24, recurrent rhinorrhea in 18, repeated vomiting in 12, recurrent bronchitis in 10, and asthma in 7.4. Thirty‐four babies had two or more allergic problems, e.g., had recurrent rhinorrhea and eczema, or recurrent bronchitis and recur‐rent diarrhea, 25 babies had only one.5. Babies allergic to cow's milk were often allergic to other foods, 20% for example were allergic to soya.6. Twenty‐five babies developed their first symptoms within 7 days of being given their cow's milk formula.7. Seventeen of the babies lost their sensitivity to cow's milk, 10 of these babies were less than 12 months old. Thirty‐eight babies over 12 months of age when last reviewed were still allergic to cow's milk.8. Babies with CMA, as well as those with other allergic problems, attended their doctors on account of illness more frequently than did the non‐allergic babies.9. Allergic disorders were commonest in the parents and siblings of babies with CMA, and least common in the parents and siblings of the non‐allergic children.
THE RE‐EMERGENCE OF EARLY CONGENITAL SYPHILIS
Authors: TAN K. L. 
Published: January 2008

SUMMARYTen infants with early congenital syphilis are presented. Hepatosplenomegaly is a marked feature in all the infants. The majority of them were of low birth weight. Syphilitic pemphigus was present in 5, and hydrops fe talis in 4. Five infants survived, including one witn hydrops fetalis. Early diagnosis and intensive therapy are necessary for survival. A high index of swpicion is required for early and more frequent diagnosis of this condition.
SEA BLUE HISTIOCYTE SYNDROME
Authors: ÖZSOYLU SLINASI , KOÇAK NURTEN , BERKEL A. IZZET 
Published: January 2008

SummaryThree children with congenital form of sea blue histiocyte syndrome are reported. Two of them had liver involvement and the third one was mentally retarded. In all these cases, marked growth retardation was documented and the two had frequent epistaxis. The diagnosis was made by the demonstration of sea blue histiocytes in the bone marrow. These cells were also shown in the liver and spleen needle biopsy specimens, whichever was obtained.
BRAIN STEM ENCEPHALITIS1
Authors: YALAZ KALBIYE , TINAZTEPE KERIMAN 
Published: January 2008

Abstract. Yalaz, K. and Tinaztepe, K. (Departments of Paediatric Neurology and Paediatric Pathology, Hacettepe Children's Hospital, Ankara, Turkey). Brain stem encephalitis. Acta Paediat Scand 63: 235, 1974.–Since the clinical picture in brain stem encephalitis resembles that of various other neurological diseases and conditions, especially brain stem gliomas, and since the prognosis and treatment of these disorders differ greatly, accurate diagnosis is very important. Eight cases of brain stem encephalitis are presented; seven with signs indicating good prognosis, and the post‐mortem findings of one fatal case. Analyses of the clinical and laboratory findings of our cases allowed the following conclusions:1. There is a prodromal period of fever and general malaise before neurological symptoms appear.2. Dysphagia, peripheral facial paralysis and pyramidal tract signs are usually present.3. Although the clinical condition is grave at the onset of illness, the prognosis is good; in 7–8 weeks complete recovery may occur but facial nerve paralysis may persist.4. Lymphocytes may be present in the cerebro‐spinal fluid along with slight increase in protein.5. Distortion of the fourth ventricle which is of extreme importance in the diagnosis of brain stem gliomas is also usually encountered in brain stem encephalitis.
1. PERSPECTIVES ON ADOLESCENT MEDICINE: CONCEPTS AND PROGRAM DESIGN
Authors: COHEN MICHAEL I. , LITT IRIS F. , SCHONBEKG S. KENNETH , SHEEHY AUDREY J. , DAUM FREDRIC , HEIN KAREN 
Published: January 2008

ABSTRACTThe concepts and goals of a program in adolescent medicine should include development of a capability to focus on current health needs of youth in a variety of settings; to plan clinical services to meet those needs with the flexibility necessary to respond to changing future requirements; and to deliver service within such a context while simultaneously creating a milieu conducive to education and investigation into the very process and definition of adolescence.The Division of Adolescent Medicine at Montefiore Hospital and Medical Center was designed 7 years ago to fulfill these goals and consequently may serve others as a functional model for health care delivery to teenagers. The Division is comprised of: (1) a 37 bed in‐patient unit; (2) a hospital‐based ambulatory program including general diagnostic and follow‐up services, as well as a specialty service capability in the areas of gynecology and family planning, cardiology, gastroenterology and nutrition; (3) primary care health services within teenage detention and prison facilities; (4) addictive disease diagnostic and treatment programs; (5) school health programs from intermediate school through college levels, and (6) the division also performs supportive and consultative functions for a variety of community‐based agencies. Within the programatic design approximately 70,000 adolescents have been served.The cornerstone of the educational and investigative efforts has been the concept that all of the above six functional units are clinical laboratories and classrooms so that training and research activities are integral parts of each of the service areas.This program design is continually undergoing revision and refinement so as to remain ever‐responsive to new and emerging problems to meet additional training demands and, most importantly, to permit and encourage creativity, and growth among patients and staff.
POSSIBLE EFFECTS OF KANAMYCIN AND INCUBATION IN NEWBORN CHILDREN WITH LOW BIRTH WEIGHT
Authors: WINKEL S. , BONDING P. , LARSEN P. KILDEGÅRD , ROOSEN J. 
Published: January 2008

ABSTRACT: In an acoustico‐vestibular follow‐up investigation of 91 four‐ to six‐year‐old children with birth weight below 2 000 g, the same incidence of sensorineural hearing loss (19 %) was found in 54 children treated with kanamycin in the neonatal period as in a group of 37 infants not treated with kanamycin. When comparing a group of children treated with both kanamycin and incubator (54 children) with a group treated with incubator only (16 children), no definite signs of synergism between incubator noise and kanamycin were found. However, the 5 cases of moderate or severe hearing loss all belonged to the group treated with both incubator and kanamycin. These 5 children had more severe neonatal complications, especially apnea, cyanotic spells and hyperbilirubinemia, which may increase the severity of the hearing loss.Among 56 incubator treated children with normal hearing (ISO standards) 52% had an audiogram pattern suggesting minor noise‐provoked cochlear lesions. Among 18 non‐incubator treated children with normal hearing only one child (6%) had a similar pattern. It should be stressed, however, that these children had no clinical symptoms of hearing loss.
DISCORDANT BONE MATURATION OF THE HAND IN CHILDREN WITH PRECOCIOUS PUBERTY AND CONGENITAL ADRENAL HYPERPLASIA
Authors: VEJVODA M. , GRANT D. B. 
Published: January 2008

ABSTRACTVejvoda, M. and Grant, D. B. (The Hospital for Sick Children, London WCIN 3JH, England). Discordant bone maturation of the hand in children with precocious puberty and congenital adrenal hyperplasia. Acta Paediatr Scand, 70: 903, 1981.‐Difference between the bone maturation scores for the tubal and carpal bones of the hand and wrist were assessed by the method of Tanner et al. on X‐rays from 10 children with precocious puberty and 10 children with late‐diagnosed congenital adrenal hyperplasia, and compared with results in 20 normal children. Scores for the tubular bones were significantly more advanced than those for the carpal bones in both groups of patients, emphasizing the need for caution when using standards derived from normal children to assess bone age in children with markedly accelerated bone maturation.
Psychological Reactions in 102 Families with a Newborn Who Has a Falsely Positive Screening Test for Congenital Hypothyroidism
Authors: BODEGÅRD GÖRAN , FYRÖ KARIN , LARSSON AGNE 
Published: January 2008

The potential psychological risks of falsely positive test results in neonatal screenings have not been studied previously. 20000 newborns were screened for congenital hypothyroidism. Of the 144 positive tests, 137 were false. The families of 102 babies with false positives were explored in reference to their (1) initial parental psychic reactions (emotional reactions and abilities for coping) and (2) residual reactions 6 to 12 months later. 78 families initially exhibited strong emotional reactions. Providing information about a positive screening test is therefore an acute strain to the majority. After a period of 6 to 12 months there was in 18 families persistent insecurity regarding the baby's health. These concerns were linked by the family to the screening and the disease screened for. This may impair the parent–child relation and, thus, the child's development. Thus a false positive test appear to trigger in the majority a development of a psychic crisis. 12 families seemed however to have been totally untouched by the potential threat of the information. The crisis is effectively solved by most (consideration must be paid to the integrated psychological support given to all). It is not known whether the 18 risk families actually have been iatrogenically hurt or if their worries brought to attention in connection with the screening merely represent habitual psychic maladjustment.Preparation for parenthood and education about screening principles are beneficial and important. This study demonstrates the importance not only of asking how much worry is elicited but also how this worry is being handled (coping ability) in order to provide a fair estimate as to which families are at risk.