bluuebunny/crossref_metadata_2025_split huggingface dataset:
DIURNAL VARIATION OF SERUM IRON IN INFANTS AND CHILDREN
Authors: Schwartz Elias , Baehner Robert L. 
Published: January 2008

SummaryThe diurnal variation of serum iron found in healthy adults was evaluated in two groups of children of different ages. No variation was found in 12 children aged 2 weeks to 20 months. In contrast, there was a significant variation of the per cent relative change of serum iron from a. m. to p. m. for a group of 12 children from 3 to 14 years of age. These results support the evidence for the late appearance of a biological “clock” in children.
A SWEDISH FOETAL HAEMOGLOBIN VARIANT
Authors: Nilsson L.‐O. , Wranne L. 
Published: January 2008

SummaryThe infant boy of a Swedish woman with the haemoglobin variant Hb Uppsala, was studied during his first six months of life. In cord blood, two major haemoglobin fractions were seen at electrophoresis. One behaved like normal foetal haemoglobin and the other migrated faster than Hb A but slower than Hb Uppsala. When the boy was three months old, this unknown fraction had disappeared and Hb Uppsala was found. Since the abnormality of Hb Uppsala is known to be located in the α‐chain, the unknown fraction was considered to be the foetal type of Hb Uppsala.
THE RELIABILITY OF THE DIAGNOSIS OF METACHROMATIC LEUCODYSTROPHY BY PERIPHERAL NERVE BIOPSY
Authors: OLSSON YNGVE , SOURANDER PATRICK 
Published: January 2008

SummaryIn order to increase the certainty of the diagnosis from peripheral nerve biopsy in cases of suspected metachromatic leucodystrophy, peripheral nerves were examined from individuals belonging to three different categories, viz. cases of metachromatic leucodystrophy (MLD) verified chemically and post‐mortem, cases of other diseases afflicting the peripheral nerves, and cases with no clinical or morphological evidence of neurological disease. The method recommended for the diagnostic establishment of sulphatides is that described by v. Hirsch & Peiffer, with cresyl violet and acetic acid applied to frozen sections. Using this method the sulphatides appear as brown metachromatic granular substances, which are completely dissolved by chloroform and methanol. The most important results of the present investigation can be summarized as follows:1. In advanced cases of MLD there is a copious accumulation of a yellow‐brown metachromatic granular substance localized partly to Schwann cells, partly to perivascular or subperineurial phagocytes. In addition, there are advanced axon and myelin lesions..2. In peripheral neuropathies due to causes other than MLD and in normal peripheral nerves, there are also varying amounts of brown metachromatic granules localized to nerve fibres. However, no massive accumulation occurs in perivascular or subperineurial phagocytes.For the present, a reliable diagnosis of MLD by peripheral nerve biopsy requires the demonstration of sulphatide accumulation in typical phagocytes. In those cases in which the brown metachromatic substance can only be traced to nerve fibres and Schwann cells, no reliable diagnosis of MLD can be made, but neither can the possibility of an early stage of MLD be excluded.
THE LONG TERM PROGNOSIS OF NON‐OBSTRUCTIVE URINARY TRACT INFECTION IN INFANCY AND CHILDHOOD AFTER THE ADVENT OF SULPHONAMIDES
Authors: LINDBLAD B. S. , EKENGREN K. 
Published: January 2008

SummaryOne hundred and seven cases of urinary tract infection in children, hospitalized during the years 1940‐1949, were reinvestigated with an observation time ranging from 15 to 24 years. Primary and late mortality was low. Non‐obstructive urinary infections in boys show a good prognosis, concerning recurrence as well as progressive renal disease.Non‐obstructive urinary tract infection in girls seems to be a potentially serious disease, where recurrence developed in half the cases in spite of the initial sulpha‐treatment given. A minimum of 19 % of the girls showed progressive renal disease according to radiological examinations. Early recurrence, according to the primary report, increased the risk of progressive renal disease from 4 to 30%. In 40% of the girls with late symptomatic recurrence, progressive renal disease could be diagnosed at about 20 years of age.The reinvestigation shows that in certain individuals the child's susceptability to recurrent urinary tract infection is retained even in the adult, in whom it manifests itself as acute pyelonephritis. It is of special interest to note that between one half to one third of those who had been pregnant had suffered from urinary tract infection of pregnancy.At about 20 years observation time negative results of single determination of blood pressure, sedimentation rate, haemoglobin, serum‐creatinine, protein or leucocytes or bacteria in the urine and maximal urinary concentration capacity does not seem to guarantee the ab‐scence of chronic or recurrent pyelonephritis. A thorough case history, including a paediatric history, is recommended in dealing with urinary tract infections in children and young adults.
ASSESSMENT FOR ADOPTION
Authors: ILLINGWORTH R. S. 
Published: January 2008

SummaryA total of 156 babies were assessed for suitability for adoption in the first year, almost all of them at the age of six months, and a further 36 infants, different ones, were first assessed in their second year. They were all graded at the time of examination, and were followed up by psychologists at school age, mainly at the age of six or seven years. The I.Q. score at school was related to the grading given in infancy.The mean I.Q. score of those placed in the first year in grade 1 (Possibly above average) was 109.1; of those placed in grade 2 (Average) was 107.3; of those in grade 3 (Doubtful) was 99.8; of those placed in grade 4 (Retarded) was 84.1. Similar figures were obtained for the 36 only seen in the second year.Analysis of the figures shows that there was a tendency to underestimate the potential of the babies, but this could be partly explained by the fact that many had suffered emotional deprivation. On the other hand only two of 110 babies thought to be average or possibly better had an I.Q. at school of below 80.Twenty‐one infants of a mentally defective parent had a mean I.Q. of 100.1, indicating the importance of not rejecting such infants as being unsuitable for adoption.It is concluded that although there never can be a high correlation between tests in infancy and the subsequent career of a child, because of all the environmental factors which will influence him, nevertheless assessment in infancy is of considerable value for the purpose of assessing babies for suitability for adoption.
HEREDITARY TYROSINEMIA
Authors: BODEGÅRD GÖRAN , GENTZ JOHAN , LINDBLAD BENGT , LINDSTEDT SVEN , ZETTERSTRÖM ROLF 
Published: January 2008

SummaryThe clinical and biochemical findings in the case of an infant with hereditary tyrosinemia followed from birth have been reported. The child received a low protein diet from birth and a formula diet restricted in phenylalanine and tyrosine when the diagnosis was established at 54 days of age. There was a steady progress of the disease and the baby died from liver failure complicated with septicemia when he was 5½ months old. The clinical course and the biochemical findings as well as the morphological changes were typical of the acute type of the disease.A 6½ year old brother suffers from the same disease of the chronic type and the two types of hereditary tyrosinemia therefore seem to belong to the same genotype.The biochemical data from the patient with hereditary tyrosinemia have been compared with those in a healthy looking baby with longstanding and pronounced transient tyrosinemia of early infancy.The patterns of amino acids in blood and of phenolic acids in urine were similar in the two patients and it is concluded that an early laboratory differential diagnosis between hereditary tyrosinemia and transient tyrosinemia may only be made by observing the biochemical response to a diet restricted in tyrosine and phenylalanine in combination with the results of phenylalanine tolerance tests.The clinical features of hereditary tyrosinemia can apparently not be attributed to a high serum‐tyrosine concentration or to the overproduction of phenolic acids; the lack of effect of early restriction in the intake of phenylalanine and tyrosine indicates a more complex pathogenesis of hereditary tyrosinemia than a primary deficiency of p‐hydroxyphenylpyruvate hydroxylase.
STUDIES ON ERYTHRO‐KINETICS IN INFANCY
Authors: WRANNE LARS 
Published: January 2008

SummaryEndogenous formation of carbon monoxide was determined in nine infants with Rh‐haemolytic disease. The calculated daily haemolysis averaged 430 mg haemoglobin per kg body weight, contrasting to the normal average of 185 mg/kg. No correlation could be found between the CO‐formation and the haemoglobin concentration of the newborn. This confirms the clinical observation that the haemoglobin concentration is not always a reliable index of the rate of haemolysis. The findings also suggest that studies of the intrauterine haemolysis do not always give true indication of the degree of foetal anaemia.
ALBUMIN METABOLISM AND GASTROINTESTINAL PROTEIN LOSS IN CHILDREN WITH NEPHROTIC SYNDROME
Authors: YSSING MINNA , JENSEN HERLUF , JARNUM STIG 
Published: January 2008

SummaryIn order to assess the significance of gastrointestinal protein loss as a cause of plasma protein hypercatabolism in patients with nephrotic syndrome, seven nephrotic children were studied simultaneously with radioiodine labelled albumin and a test substance, 51,Fe‐labelled iron dextran, for evaluation of gastrointestinal protein loss.All the patients displayed a pronounced hypercatabolism of albumin, which could not be accounted for by urinary protein loss. A slightly increased gastrointestinal protein loss was demonstrated in 4 patients, whereas, in 3 patients, the faecal loss of macromolecules was within normal limits.The relation between protein catabolism and faecal loss of macromolecules was compared in the nephrotic children and in 20 patients with protein‐losing enteropathies. It is concluded that gastrointestinal protein loss is insignificant in the nephrotic syndrome, and unable to account for the marked endogenous hypercatabolism of albumin.Albumin synthesis was normal or somewhat increased. An abnormally high fraction of the total albumin mass was present in the intravascular space in 4 of the 7 children.
SECONDARY CYSTATHIONINURIA
Authors: STUDNITZ WILFRIED v. 
Published: January 2008

SummaryCystathioninuria was detected in 6 of 230 hospitalized children. Three had convulsions when admitted to the hospital, two severe liver malfunction of unknown cause (suspect neonatal hepatitis), and one child had severe congenital malformations and bronchopneumonia and was in a terminal stage. Cystathioninuria disappeared in 4 children (two with convulsions and two with liver malfunction) treated with pyridoxine orally. Pyridoxine treatment (2×40 mg daily) also resulted in disappearence of convulsions. The possible background to these findings is discussed.
I. The Development of the Hering‐Breuer Inflation Reflex
Authors: BODEGÄRD GÖRAN , SCHWIELER GÖSTA H. , SKOGLUND STEN , ZETTERSTRÖM ROLF 
Published: January 2008

SummaryThe Hering‐Breuer inflation reflex has been studied in babies of varying postmenstrual ages. The strength of the reflex was assessed by relating the relative increase of the length of the breathing cycle to the transpulmonary pressure when the airway was occluded.The Hering‐Breuer inflation reflex was found to be very weak at a postmenstrual age of 32 weeks. It was then found to increase to a maximum strength at a postmenstrual age of 36 to 38 weeks. Later on there was a decline of the strength of the reflex.The significance of the findings in relation to the frequent occurrence of periodic and irregular respiratory rhythm in infants of low gestational age has been discussed.
THE FOETAL DEVELOPMENT OF SERUM LEVELS OF IgG AND IgM
Authors: BERG TORSTEN , NILSSON BO A. 
Published: January 2008

SummaryThe serum concentrations of the immunoglobulins G and M were studied in non‐viable foetuses with gestational ages of 13–26 weeks, and also in premature, mature and postmature infants. Among the prematurely born infants there was an overrepresentation of infants with very low gestational ages. The serum IgG concentrations in the mothers at the time of abortion or delivery were also studied.In full‐term infants there was a systematic difference between simultaneously taken capillary and umbilical cord samples, with higher serum concentrations of IgG in the former. For this reason umbilical cord sera were used throughout in this study.The maternal serum concentration of IgG was found to be of some importance for the foetal IgG level. The foetal: maternal IgG ratio increased, as also did the foetal IgG concentration, exponentially during pregnancy, with the exception of its last weeks when the development took place relatively more slowly. IgM in serum was observed (>1 mg/100 ml) in two foetuses with gestational ages as short as 14 and 16 weeks. From the 24th week of pregnancy onwards, IgM was found in the serum in practically all cases. From about the 29th‐30th week of pregnancy, no significant increase in the serum IgM with increasing gestational age was observed in the infants studied.
A PATIENT WITH A DEFICIENCY OF SERUM‐CARNOSINASE ACTIVITY
Authors: van HEESWIJK P. J. , TRIJBELS J. M. F. , SCHRETLEN E. D. A. M. , van MUNSTER P. J. J. , MONNENS L. A. H. 
Published: January 2008

SummaryA patient with severe mental retardation, generalized convulsions and electro‐encephalo‐graphic abnormalities has been described. A marked carnosinuria was observed on a regular diet as well as on a diet which was essentially free of carnosine. No carnosine was found in plasma of this patient on any occasion. The serum‐carnosinase activity was extremely diminished compared to control subjects. Oral loading with L‐carnosine resulted in a much greater excretion of the administered L‐carnosine compared to a control subject. The consanguineous parents of the patient also excreted large amounts of the dipeptide and had decreased serum‐carnosinase activities. Infusion with freshly prepared donor plasma, containing active carnosinase, resulted in a temporary elevation of the enzymic activity in serum and a decrease of carnosine excretion in urine.
5‐HYDROXYINDOLEACETIC ACID IN CEREBROSPINAL FLUID OF HYDROCEPHALIC CHILDREN
Authors: ANDERSSON H. , ROOS B.‐E. 
Published: January 2008

SummaryThere was a significant increase in the level of 5‐HIAA in lumbar CSF of children with hydrocephalus compared with “non‐hydroce‐phalic” children. In “non‐hydrocephalic” children the values of 5‐HIAA were found to be highest in the newborn successively decreasing to the levels of adult persons at the age of about one year. Prematurely born children seemed to have relatively higher levels of 5‐HIAA in CSF compared with full‐term children. Adult persons were not included in the study.The ventriculo‐lumbar ratio in different types of hydrocephalus was calculated. A diminished level of 5‐HIAA was measured after surgery with a functioning shunt. All the findings support the theory that the cause of the increased level of 5‐HIAA is a decreased elimination of the acid metabolite from CSF. Determination of 5‐HIAA in CSF might be a simple and useful diagnostic complement in various neurological diseases, where the suspicion of hydrocephalus has arisen.
GLUCOSE‐GALACTOSE MALABSORPTION
Authors: MEEUWISSE G. W. , MELIN K. 
Published: January 2008

SummaryThe histories of 6 cases, 2 male and 4 female, with glucose‐galactose malabsorption (GGM) are presented. Of these, 4 have survived and are in good general health on a diet with a reduced carbohydrate content. One case died at 2 months of age with severe malnutrition due to intractible diarrhoea before a diagnosis was made. The other patient who died, was diagnosed and treated from the neonatal period, and he was progressing until infection occurred by a Candida albicans, followed by death at 3 months of age in a picture of agam‐maglobulinaemia. One patient is now a woman of 41. The main symptoms are diarrhoea from birth, dehydration and malnutrition. Glucose is found in the watery stools and there is also a slight glucosuria. Hyperosmolar serum is often seen. Two patients, not those treated at an early stage, developed renal concrements probably owing to the long lasting water loss with the stools. Treatment during the first months consisted of replacement of the ordinary dietary carbohydrates by fructose. Although the basic biochemical disorder lasts throughout life, limited amounts of starch and milk could be introduced later in infancy. Some absorption of glucose could be demonstrated, tentatively due to a second pathway for intestinal glucose absorption which is apparently not shared by galactose. Hydrolysis of disac‐charides occurred in the small intestine of GGM patients but the released glucose was absorbed no better than when administered as a monosaccharide. This implies that the site of disaccharidase activity is located more superficially at the luminal side of the epithelial cell membrane than the disturbed step of glucose and galactose transport.
GLUCOSE‐GALACTOSE MALABSORPTION
Authors: MELIN K. , MEEUWISSE G. W. 
Published: January 2008

SummaryThe pedigree of 6 cases (2 male and 4 female) of glucose‐galactose malabsorption is presented. Besides the 6 cases seen by the authors, there occurred in the same pedigree at least 4 more instances of death in early infancy due to intractable diarrhoea. It is likely that these resulted from glucose‐galactose malabsorption. The parents of the patients were healthy and had no history of chronic diarrhoea in infancy or thereafter. As judged by oral GTT's on two pairs of parents they had normal glucose absorption. No renal glucosuria, present in all the patients, was found in the parents. It was concluded that glucose‐galactose malabsorption has an autosomal recessive mode of inheritance.In 5 patients (from 4 different sibships) where the urinary excretion of amino acids was examined, 4 had increased excretion of β‐aminoisobutyric acid although they were in a good nutritional state. Of 7 parents examined, 2 excreted increased amounts of the same compound. The finding of this selective aminoaciduria is probably incidental and, at least in the children, is not believed to be genetically determined.
Methods för evaluation of the physical working capacity of school children with cerebral palsy
Authors: BERG KRISTINA , BJURE JAN 
Published: January 2008

SummaryApplication to children with cerebral palsy of ordinary physical working capacity tests involving standardized work on bicycle ergo‐meters is not possible since the handicap of such children involves poor neuromotor control. The work load perförmed will vary since the involontary movements and tensions also are oxygen‐consuming and varying. The mechanical efficiency also changes considerably in such subjects, even at the same load in the same individual on different occasions.Thus, direct measurements of oxygen consumption during work is the only solution. However, due to mouth involvement, it is often difficult to perförm air collection, in subjects with cerebral palsy particularly at maximal work intensity.A simple rubber membrane was thereföre made which facilitated air collection in children with cerebral palsy.A graphical method which correlated oxygen uptake to heart rate at two or more submaximal levels was also developed. för a rough estimate of the maximal oxygen uptake in subjects where direct determinations were not possible, extrapolation of the line to the maximal heart rate, individually determined, was done.Training of these inactive subjects could change the original line in two ways. An improved muscular perförmance with respect to strength and technique, the “muscle” effect, seemed to increase the maximal heart rate and extended the line; a higher oxygen capacity, the “oxygen” effect, raised the original line, i.e. an increased oxygen uptake at a given heart rate was demonstrated. Either effect alone or the effects in combination increased the maximal
Effect of physical training of school children with cerebral palsy
Authors: BERG KRISTINA 
Published: January 2008

SummaryThe results of training school children with cerebral palsy are reported. Twenty‐two severely handicapped subjects, with a mean age of 14 years, were trained three times a week för 20 minutes each time and för varying periods, ranging between 1 1/2 and 16 months. The effect of training on the observed “maximal” oxygen uptake was pronounced: 8 subjects improved 10–15 per cent above the original value and 12 subjects 25 per cent and more. Several students subjectively noted improved perförmance and well‐being.It was noted that inactivity during the 31/2‐months of summer vacation significantly reduced the observed “maximal” oxygen uptake incurred after a previous training period of equally long time.A measurable training effect was sometimes not found immediately. Once an effect was noted, it seemed to continue at the same rate för several months.It can be concluded that simple training as described here probably can be fitted into any rehabilitation program, and that it is important in increasing the physical capacity of severely handicapped children with cerebral palsy
Body composition and nutrition of school children with cerebral palsy
Authors: BERG KRISTINA , ISAKSSON BJÖRN 
Published: January 2008

SummaryHeight, weight, body composition, and intake of energy and of some essential nutrients were determined on 23 full‐board subjects with cerebral palsy, aged 7–21 years, from Bräcke Öster‐gärd Pediatric Habilitation Centre.Body height was generally small för age while weight often was increased för height.The mean body cell mass was 86 per cent of the value predicted för height.The extracellular water was strikingly increased in all subjects.Body fat was increased in 7 spastics and reduced in 5 spastics and in 6 of the 7 dyskinetics.The observed energy intake was low but adequate för active metabolic mass in 11 of 23 subjects. The reduced energy requirements of most subjects resulted in an insufficient supply of several essential nutrients due to the composition of the food offered.Low physical activity and malnutrition were suggested as possible causes of the abnormal body composition.
Effect of physical activation and of improved nutrition on the body composition of school children with cerebral palsy
Authors: BERG KRISTINA 
Published: January 2008

SummaryTwenty‐two subjects with cerebral palsy and with abnormal body composition, reduced physical activity and malnutrition, were subjected to treatment with physical activation and/or improved nutrition. Nine subjects were trained för 4 months (Study A); 8 of these and 5 more subjects then received training and improved nutrition during one school‐year (Study B). Eight more subjects had only the improved nutrition för one school year (Study C).The treatment in Studies A, B, and C had no significant effect on the initially reduced body cell mass (BCM).The markedly increased extracellular water (ECW) was significantly reduced in Studies A and B, but not in C.The effect of the treatment on body fat (BF) varied. In Study A, the body fat increased significantly, in Studies B and C no significant changes were observed.The effects were interpreted as evidence of the fact that inactivity and malnutrition were relevant causes of the abnormal body composition. The observed absence of a growth spurt during the therapy indicated that the initial malnutrition could not have been too serious. A clear‐cut separation between the two therapeutic measures was not achieved, however, as an increased activity induced an increased intake of energy and of essential nutrients.