bluuebunny/crossref_metadata_2025 huggingface dataset:
JUVENILE POLYPS OF THE RECTUM AND COLON
Authors: TOCCALINO H. , GUASTAVINO E. , PINNI F. DE , ODONNELL J. C. , WILLIAMS M.
Published: January 2008

Fifty rectal polyps were observed in 4 000 pediatric gastroenterological consultations (an incidence of 1/80). The age range was 21 months to 11 years with a maximal frequency from 2–6 years. No sex difference was seen. Rectal bleeding was the motive for medical consultation. The diagnosis was made by recto‐sigmoidoscopy. The usual colon X‐ray techniques were not considered useful for diagnosis of juvenile polyps.

A single polyp was observed in 46 patients, in the majority localized within 10 cm of the anus. Histology showed the characteristics of hamartoma; 15 of them also presented signs of infection.

The treatment consisted of polypectomy through the Espeche method or fulguration or through a combination of both procedures.
MALOCCLUSION AND SUCKING HABITS OF FOUR‐YEAR‐OLD CHILDREN
Authors: KÖUHLER LENNART , HOLST KERSTIN
Published: January 2008

In an unselected population of 4‐year‐old children, 66.4% were found to have mal‐occlusion, and 10.7% were considered in need of treatment by an orthodontist. Previous or present sucking habits were reported for 77.9% of the children. 10.7% still used a dummy, 30.1% were sucking on their fingers and 13.2% used both dummy and fingers.

A strong statistical connection was found between sucking habits and malocclusion, most pronounced for dummy‐sucking. No difference in mean caries index was found between children with and without malocclusion. Significantly more caries was demonstrated in dummy‐sucking children than in finger‐sucking children, but these differences could be explained by other environmental factors.
PROPERTIES OF FIVE ACID HYDROLASES IN HUMAN SKIN FIBROBLAST CULTURES Possible Use in the Diagnosis of Inborn Lysosomal Diseases
Authors: HULTBERG B. , SJBBLAD S. , BCKERMAN P. A.
Published: January 2008

Five acid hydrolases, β‐galactosidase, β‐gluco‐sidase, a‐mannosidase, N‐acetyl‐β‐glucosamini‐dase, and β‐glucuronidase, were studied in human skin fibroblast cultures. The pH‐de‐pendency and kinetic properties of the enzymes as well as results of gel filtration and isoelectric focusing indicate that these enzymes are the same in fibroblasts as in liver.

Techniques were defined with the aim of finding methods suitable for the diagnosis of inborn lysosomal diseases. It is stressed that great caution must be executed in the interpretation of results, since isolated enzyme deficiencies may appear in early, but not in later subcultures. Also, there were great variations in the activity levels between different subcultures.
EXCESS GLYCOSAMINOGLYCAN EXCRETION IN INFANCY AND CHILDHOOD
Authors: PENNOCK C. A. , WHITE FRANCES , MURPHY D. , CHARLES R. G. , KERR HELEN
Published: January 2008

Experience with a simple cetylpyridinium chloride screening test for detection of excess urinary glycosaminoglycans (GAG) is described. The causes of false positive results are listed.

A simplified approach avoiding time‐consuming laboratory procedures such as column chromatography has been used to study glycos‐aminoglycan excretion in children giving a positive screening test. GAG have been isolated in crude form using cetylpyridinium chloride and analysed for hexosamine content and resistance to hyaluronidase digestion without further separation and purification. A simple electrophoretic separation has been used as supportive evidence for any abnormality detected.

Use of this simplified approach has been of value in the diagnosis of mucopolysacchari‐doses. Children with false positive screening test can readily be shown to be normal excre‐tors. A few anomalous results have been found in mentally retarded children and patients with other bone dysplasias.

More important, the methods used highlight the problem of detecting abnormal glycos‐aminoglycan excretion in the first year of life. Our results in this age group suggest that the full picture of abnormal excretion may be a late development in the mucopolysaccharidoses and where there is a high index of suspicion that an infant may have one of these disorders, a full, detailed analysis is desirable. The simple methods used here are of value in this respect since they are less time‐consuming than other methods and are easily undertaken by routine hospital clinical chemistry departments without specialized research facilities and staff.
URINE ELIMINATION OF AN ORAL SALT AND FLUID LOAD IN HEALTHY CHILDREN
Authors: BERG U.
Published: January 2008

A method for studying renal response to an oral sodium and fluid load has been tested in a group of normal children aged 8 to 14 years. An oral sodium load of 80 mEq/1.73BSA was given after water diuresis was induced. Urine wascollectedhourlybyspontaneous voiding. At the time of the study the glomer‐ular filtration rate and clearance of PAH was determined by the single injection technique. After the salt load was given the urinary sodium excretion was increased for 2 hours. Thereafter it reached a fairly stable level which was maintained at least during the next 4 hours. This response was a concomitant finding in all the children studied. It was thereafter considered justified to estimate the renal response to the salt load as the mean urinary sodium excretion from the third to the sixth hour after sodium administration. The urinary sodium excretion was then found to be 16 ± 1.8 mEq/hr/1.73BSA. When the salt load was doubled the urinary sodium excretion was increased by a factor of 1.6. Various explanations for this deviation from complete dose‐response are discussed. It is suggested that the test is used in the evaluation in the control of sodium homeostasis in patients with renal and circulatory disturbances.
DISPLACEMENT OF ALBUMIN‐BOUND BILIRUBIN BY INJECTABLE DIAZEPAM PREPARATIONS IN VITRO
Authors: BRATLID DAG , LANGSLET ASBJØSRN
Published: January 2008

Three injectable diazepam preparations, Va‐lium®, Stesolid®, and Vivala® were tested for their ability to displace bilirubin from albumin in vitro. Both Valium® and Stesolid® caused marked displacement of bilirubin and this effect was potentiated by a reduction in pH. Both these diazepam preparations contain benzoate in the buffer preservative. Vivala, which does not contain benzoate in the buffer preservative, did not show any displacement at pH 7.4 and had only a slight effect at pH 6.8.
LINEAR GROWTH RATE, BONE MATURATION AND GROWTH HORMONE SECRETION IN PREPUBERTAL CHILDREN WITH CONGENITAL ADRENAL HYPERPLASIA
Authors: RAPPAPORT R. , BOUTHREUIL E. , MARTI‐HENNEBERG C. , BASMACIOGULLARI A.
Published: January 2008

This study was designed to evaluate the effect of various oral hydrocortisone dosages on linear growth and bone maturation in a group of 21 children with congenital adrenal hyper‐plasia between the age of 3 and 10 years. Treatment was monitored on urinary pregnanetriol excretion. When considering the individual annual height increment, a normal growth rate was achieved with dosages between 15 and 36 mg/per 24 hours, and a reduced growth rate with dosages between 27 and 55 mg/ per 24 hours. The same range of dosages was found when bone maturation increments were considered separately. In order to study the dose‐response relationship, the slopes of individual linear growth curves (considered for minimal periods of two years) were correlated with hydrocortisone dosages. A significant negative correlation was found which assesses the growth reducing activity of hydrocortisone for dosages up to 45 mg/per 24 hours. Linear growth of children overtreated during early infancy did not show a normal catch up rate in some cases, suggesting a permanent damage of growth. In all cases growth hormone secretion was in the normal range. In late treated children, dosages between 30 and 45 mg/ per 24 hours reduced predominantly the skeletal maturation and allowed some linear growth.
MANNOSIDOSIS: CLINICAL, FINE‐STRUCTURAL AND BIOCHEMICAL FINDINGS IN THREE CASES
Authors: AUTI SEPPO , NORDÉN NILS E. , ÖCKERMA PER‐ARNE , RIEKKINE PAAVO , RAPOL JUHANI , LOUHIM TUULA
Published: January 2008

Three boys, 4, 5, and 10 years old, with psycho‐motor retardation, slightly gargoyle‐like faces and recurrent infections were found to have vacuolized lymphocytes in the blood and bone marrow as well as diffuse skeletal alterations. Electron microscopy of liver biopsy specimens revealed vacuoles, presumably representing enlarged lysosomes engorged with storage material. The liver. a‐mannosidase activity was somewhat low but not absent, while several other acid hydrolases in the liver had very high activities. The a‐mannosidase activity was low also in white blood cells but not in plasma or urine. The liver and urine contained very high amounts of mannose‐rich compounds. It is concluded that the patients suffer from man‐nosidosis.
LOWE'S SYNDROME: Absence of Amino Acid Transport Defect in Cultured Fibroblasts
Authors: BARTSOCAS CHRISTOS S. , ERBE RICHARD W.
Published: January 2008

Transport of lysine and glycine was studied in cultured fibroblasts of a patient with Lowe's oculocerebrorenal syndrome. Although patients with this syndrome demonstrate renal and intestinal amino acid transport defects, no abnormality was found in our study.
DETERMINATION OF GLOMERULAR FILTRATION RATE IN THE NEWBORN Comparison between Results Obtained by the Single Injection Technique without Collection of Urine and the Standard Clearance Technique
Authors: BROBERGER ULF
Published: January 2008

Determination of glomerular filtration rate was performed in fifteen infants by both the single injection technique and the standard inu‐lin clearance technique. Eleven infants were fasted for four hours before the study. In those patients there was a good accordance between the two methods for determination of glomerular filtration rate. Four infants received breast milk or formula within 1 hour before the single injection procedure was started. In those infants there was a considerable over‐estimation of glomerular filtration rate with the single injection technique. The error of the method introduced by recent feeding is discussed.
TUFTSIN DEFICIENCY SYNDROME A Report of Two New Cases
Authors: CONSTANTOPOULOS ANDREAS , NAJJA VICTOR A.
Published: January 2008

Two families with deficiency in the phagocytosis stimulating peptide, tuftsin, are reported. Both cases gave a history of recurrent infections. In the first case, (D. I.) all infection involved only external surfaces. The second patient (W. M.) showed involvement of internal organs with septicimia pneumonia and liver abcess in addition to skin lesions. In both cases, Staphylococcus aureus was the infecting organism.
EFFECT OF FASTING AND OF A PROTEIN FREE DIET ON AMINO ACID RATIOS IN PLASMA AND ERYTHROCYTES IN RATS
Authors: AZAR MAHIN , TER‐SARKISSIAN NAZNIK , BAVENDI MOHSEN , HEDAYA HABIB , DONOS GONZALO
Published: January 2008

The concentration and ratio of nonessential to essential amino acids were determined by paper chromatography in serum and erythro‐cytes of fasting rats and in animals that received a non‐protein diet, and compared with rats on a stock diet.

Our results show that very dicfferent changes are brought about in the non essential to essential amino acid ratios, in the amino acid concentrations and in their relative erythro‐cyte/plasma distribution by the different dietary treatments. The changes induced by the non‐protein diet resemble those reported in clhildren suffering from protein‐calorie malnutrition. The fact that the changes are already apparent after only 3 days of dietary treatment seem to indicate that abnormal ratios reflect more the quality of the diet than the nutritional status.
THE INFLUENCE OF THE METHOD OF DELIVERY AND THE CLAMPING TECHNIQUE ON THE RED CELL VOLUME IN INFANTS OF DIABETIC AND NON‐DIABETIC MOTHERS
Authors: KLEBE JOACHIM G. , INGOMAR CARL JOH.
Published: January 2008

The red cell volume has been investigated in 35 infants of diabetic mothers and in 18 infants of non‐diabetic mothers. It is shown that not only the clamping technique but also, the method of delivery has an influence on the red cell volume of the infant. Thus the red cell volume is less in infants delivered vaginally with early clamping, compared with infants delivered by caesarean section with early clamping.

These findings support the theory that during vaginal delivery a temporary change in It is suggested that IDM during vaginal delivery with early clamping deposit more blood in the placenta than do the non‐IDM, since we found a smaller red cell volume per kg the distrfbution of. the foeto‐placental blood volume between placenta and child occurs.
THE “BLUE” METER, A PHOTOMETER DESIGNED TO MEASURE LIGHT EMISSION DURING PHOTOTHERAPY OF HYPERBILIRUBINEMIA
Authors: LUND H. T. , GUDUM J. , KOP V.
Published: January 2008

In order to measure the efficiency of fluorescent lamps used in phototherapy, a new photometer has been devised. The spectral response of the meter is matched to the action spectrum of bilirubin photo‐oxidation, corrected for skin attenuation. The theory of operation and a technical description of the instrument is given.
METABOLISM OF LITHOCHOLIC ACID‐24–14C IN EXTRAHEPATIC BILIARY ATRESIA
Authors: NORMAN ARNE , STRANDVIK BIRGITTA
Published: January 2008

Lithocholic acid‐24‐14C was given to 2 infants with extrahepatic biliary atresia. After oral administration about half of the amount of isotope was found to be adsorbed. After par‐ 9. Makino, I., Sjovall, J., Norman, A. & Strandvik, enteral administration about half of the amount of isotope was excreted in the urine for 3 days. Lithocholic acid was shown to be transformed to a small extent to more polar compounds. These metabolites were not cholic, chenodeoxycholic, α‐ or,β‐muricholic, hyocholic, hyodeoxycholic or ursodeoxycholic acids. Most of the lithocholic acid was excreted as the sulphate esters of tauro‐ and glycolithocholic acid. No sulphate ester of lithocholic or free lithocholic acid was excreted.
AN OUTBREAK OF COXSACKIE VIRUS TYPE B2 AMONG NEONATES IN AN OBSTETRICAL WARD
Authors: EILARD T. , KYLLERMAN M. , WENNERBLOM I. , EEG‐OLOFSSON O. , LYCKE E.
Published: January 2008

An outbreak of Coxsackie B2 virus infections in 12 neonates was studied. The source of the infection seemed to be the mother of the first diseased baby who later spread the infection in the ward, and although no virus carrier was found among the staff members there was serological evidence that nearly 40% of the personnel had been infected during and the month following the infection of the babies. The need for careful observation of patients and personnel presenting signs of infection especially when Coxsackie virus infections are common is stressed.
A FOLLOW‐UP STUDY OF CHILDREN WITH ASTHMATOID BRONCHITIS: II. Serum IgE and Eosinophil Counts in Relation to Clinical Course
Authors: FOUCARD TONY
Published: January 2008

After a follow‐up period for 15–40 months (mean 31 months) of 81 children, aged 2–66

A high serum IgE concentration was found initially in 44 % of first‐time‐wheezers who became asthmatics and in 7% of children regarded as “healthy at the end of the investigation period. Asthma seemed to be predicted better by a high serum IgE value than by eosinophilia (< 400/mm3) but the reverse was found when the number of blood eosinophils was high (> 700/mm3).

A variation of the serum IgE concentration with the time of the year was found in all months, with asthmatoid bronchitis 19 (23 %) were clinically considered to suffer from true asthma and 8 (10%) from allergic rhinoconjunctivitis or urticaria. These children had significantly higher serum IgE concentrations and a significantly greater number of blood eosinophils than the 35 children considered to be “healthy. Nineteen children (23 %) had still episodes of wheezy bronchitis at the end of the investigation period and these children also had significantly higher serum IgE levels and a significantly greater number of blood eosinophils than the “healthy children. Although none of the children with “asthmatoid bronchitis had clinically apparent asthma, 2 had IgE antibodies to animal danders and pollen as shown by RAST and in both bronchial provocation tests were positive. Another 4 children in this group had high IgE as well as an increased total of blood eosinophils (> 700/mm3) indicating that they probably had reaginic asthma.

groups but least pronounced in the asthma group. The lowest levels were found at the

beginning of the year and the highest values in the autumn.
HISTIDINEMIA AND “NORMOHISTIDINEMIC HISTIDINURIA”Report of Three Cases and the Effect of Different Protein Intakes on Urinary Excretion of Histidine
Authors: HOLMGREN G. , HAMBREUS L. , de CHATEAU P.
Published: January 2008

Abstract. Holmgren, G., Hamhræus, L. and de Chateau, P. (Departments of Paediatrics, University Hospitals, Umeå and Uppsala and the Department of Nutrition, University of Uppsala, Sweden). Histidinemia and “normohistidinemic histidinuria”. Acta Paediat Scand, 63: 220, 1974.–Three patients with histidinemia and one with “normohistidinemic histidinuria” are presented. The effects of varying protein intakes on urinary histidine excretion were studied in three of the patients and in 14 controls; histidine excretion in the urine was found to be parallel to an increased protein intake. In the “histidinuria” patient, the urinary histidine excretion was much lower than in the histidinemic patients, hut higher than in the controls at different levels of protein intake. The occurrence of different phenotypes of histidinemia and renal histidinuria and the value of early dietary treatment is discussed.
PHOTOSENSITIZED SHIFT IN THE O2 DISSOCIATION CURVE OF FETAL BLOOD
Authors: OSTREA ENRIQUE M. , ODELL GERARD B.
Published: January 2008

Abstract. Ostrea, E. Jr., and Odell, G. (Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA). Photosensitized shift in the O2 dissociation curve of fetal blood. Acta Paediatr Scand, 63: 341, 1974.–The exposure of fetal erythrocytes to blue light in the presence of bilirubin was associated with a significant decrease in the affinity of fetal cells for O2, (δP50) at pH 7.4=+3.17± 0.68 mmHg. This change was not observed with either adult erythrocytes or hemolysates of fetal and adult red cells. Associated with the shift in P50, there was a decrease in the Na+, K+ ‐ATP'ase of the irradiated erythrocytes and no electrophoretic evidence of alteration in the fetal hemoglobin. The results suggest that the shift in the O2 dissociation curve represents an additional manifestation of photodynamic membrane injury and the stroma of fetal erythrocytes influence their oxygen affinity.
TEN CASES OF SO‐CALLED LONG SURVIVAL IN CHlLDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA
Authors: ARMATA J. , CYKLIS R. , WYSZKOWSKI J.
Published: January 2008

Abstract. Armata, J. Cyklis, R. and Wyszkowski, J. (Institute of Paediatrics, Medical Academy in Cracow, Cracow, Poland). Ten cases of so‐called long survival in children with acute lymphoblastic leukemia. Acta Paediatr Scand 63: 369, 1974.–Ten cases of acute lymphoblastic leukemia are presented, in children all of whom survived more than 4 years. WBC and blast cell counts were registrered at low levels at the onset of the disease and at subsequent bone marrow relapses. Extramedullary leukemia occurred after 2 years (average) in bone marrow remission, which did not predict early bone marrow relapse. From 1962 to 1970 three treatment regimens for acute leukemia were compared; the best results depended on the intensity of treatment during the first remission.
AMYLASE CONTENT OF MIXED SALIVA IN CHILDREN
Authors: ROSSITER MARY A. , BARROWMAN J. A. , DAND ANNE , WHARTON B. A.
Published: January 2008

Abstract. Rossiter, M. A., Barrowman, J. A., Dand, A. and Wharton, B. A. (Queen Elizabeth Hospital for Children, Hackney Road, London E.2 and the London Hospital, Whitechapel, London, E.l., England). Amylase content of mixed saliva in children. Acta Paediatr Scand, 63: 389, 1974.–Salivary amylase levels were determined in normal subjects from birth until adult life and in children with conditions sometimes associated with low pancreatic amylase such as malnutrition, coeliac disease and cystic fibrosis. Mixed saliva was collected under carefully standardised conditions and amylase was measured by the method of Dahlqvist. There was a wide scatter of values in the 84 normal subjects, but concentrations rose from very low levels at birth to reach adult levels by the age of 6 months to 1 year. Salivary amylase activity rose normally over ten weeks in one premature infant fed milk by gastrostomy. Thirteen children with coeliac disease and 9 children with cystic fibrosis mostly had normal salivary amylase concentrations. Six out of 12 malnourished children with jejunal villous atrophy of uncertain aetiology had low levels which rose to normal as recovery began.
A METHODOLOGICAL STUDY OF THE DIAGNOSIS OF CYSTIC FIBROSIS BY INSTRUMENTAL NEUTRON ACTIVATION ANALYSIS OF SODIUM IN NAIL CLIPPINGS
Authors: KOLLBERG H. , LANDSTROM O.
Published: January 2008

Abstract. Kollberg, H. and Landström, O. (Department of Paediatrics, University Hospital, Uppsala and AB Atomenergi, Studsvik, Sweden). A methodological study of the diagnosis of cystic fibrosis by instrumental neutron activation analysis of sodium in nail clippings. Acta Paediatr Scand, 63: 405, 1974.–Instrumental neutron activation analysis (INAA) of nail clippings was used in the development of a diagnostic method for cystic fibrosis (CF). From CF patients and controls more than three years old, nail clippings were sampled for tests of different errors. As a result of this study a precise collection routine was outlined. Sodium is easily washed out from nail clippings. The risk of sodium contamination of the nails seems to be small. The sodium distribution in the nail is inhomogeneous, and the sodium content varies also from nail to nail in the same person. There is good evidence that the increased sodium in the nails of CF patients comes from the sweat. There seems to be a basic “intrinsic” sodium level, which is about the same in the nails of CF patients as in controls. The precision and accuracy of INAA for determining sodium in nails is considered satisfactory. It is concluded that INAA of the sodium concentration in nail clippings is a suitable method as an aid in the diagnosis of CF if a precise collection routine is used.
MICROBIAL FLORA OF STOMACH AND SMALL INTESTINE IN INFANTILE GASTROENTERITIS
Authors: BISHOP RUTH F. , BARNES G. L. , TOWNLEY R. R. W.
Published: January 2008

Abstract. Bishop, R. F., Barnes, G. L. and Townley, R. R. W. (Department of Gastroenterology, Royal Children's Hospital, Melbourne and Department of Paediatrics, University of Melbourne, Victoria, Australia). Microbial flora of stomach and small intestine in infantile gastroenteritis. Acta Paediatr Scand, 63: 418, 1974.–Microbial flora of mouth, stomach, duodenum and faeces was examined in 39 infants with acute gastroenteritis. Only one recognised pathogen, Salmonella typhimurium, was isolated. No enteropathogenic strains of Escherichia coli were isolated. There was an increase in abundant growth of Candida albicans in stomach and small intestine of infants with gastroenteritis compared with the control group.
DIAGNOSTIC USEFULNESS OF THE NITROBLUE TETRAZOLIUM DYE REDUCTION TEST IN A CHILDREN'S HOSPITAL
Authors: MARKS MELVIN I. , DERY PIERRE
Published: January 2008

Abstract. Marks, M. I. and Dery, P. (Dept. of Pediatrics, Infectious Disease, McGill University‐Montreal Children's Hospital Research Institute, Montreal, Canada). Diagnostic usefulness of the nitroblue tetrazolium dye reduction test in a children's hospital. Acta Paediatr Scand, 63: 427, 1974.–The usefulness of the classical and stimulated nitroblue tetrazolium dye (NBT) reduction test in the diagnosis of pediatric infectious diseases was evaluated. One hundred and fifty‐two patients were studied. An overall correlation of increased phagocytosis with bacterial infections was noted, but the test was not judged useful in differentiating most infections that were difficult to diagnose by other clinical and laboratory criteria. There were 4.8% false negatives among 21 proven bacterial infections studied before treatment, and 27% false positives in 26 proven viral infections. Caution is urged in the use of the NBT test for diagnosis and treatment of the individual patient. When carefully performed and controlled, the NBT test should serve to help confirm clinical diagnoses and to screen for certain leukocyte bactericidal deficiencies.
CONGENITAL LACTOSE INTOLERANCE OF GASTROGEN ORIGIN ASSOCIATED WITH CATARACTS
Authors: RUSSO GIUSEPPE , MOLCICA FLORINDO , MAZZONE DOMENICO , SANTONOCITO BENEDETTA
Published: January 2008

Abstract. Russo, G., Mollica, F., Mazzone, D. and Santonacito, B. (Paediatric Clinic, University of Catania, Catania, Italy). Congenital lactose intolerance of gastrogen origin associated with cataracts. Acta Paediatr Scand, 63: 457, 1974.–A case of congenital lactose intolerance with lactosuria associated with bilateral cataracts in a male infant is described. Lactosuria and cataracts were also present in four members of the two preceding generations.

According to per oral lactose tolerance tests evidence of transient intestinal lactose deficiency was found in the proband, and it was shown that lactosuria was related to the gastric passage of the lactose, since it disappeared when lactose was given intraduodenally.
COCKAYNE'S SYNDROME IN TWO SISTERS
Authors: SRIVASTAVA R. N. , GUPTA P. C. , MAYEKAR G. , ROY S.
Published: January 2008

Abstract. Srivastava, R. N., Gupta, P. C., Mayekar, G. and Roy, S. (Departments of Paediatrics, Neurology and Pathology, All India Institute of Medical Science, New Delhi, India). Cockayne's Syndrome in two sisters. Acta Paediatr Scand, 63 461, 1974.–Clinical and laboratory data in two sisters with Cockayne's syndrome are presented. Both had severe dwarfiim and mental retardation and the elder girl (11 years) had senile facies, optic atrophy and intracranial calcification. The serum cholesterol values were elevated. Peripheral neuropathy was found in both cases, evidenced by slow nerve conduction velocities. Examination of sural nerve biopsies showed focal demyelination with preservation of axis cylinders. These observations support the concept of Cockayne's syndrome being a leucodystrophy.
HEREDITARY RECURRENT CHOLESTASIS WITH LYMPHOEDEMA–TWO NEW FAMILIES
Authors: AAGENÆS Ø.
Published: January 2008

Abstract. Aagenres, Ø. (Paediatric Department, University Hospital, Oslo, Norway). Hereditary recurrent cholestasis with lymphoedema–two new families. Acta Paediatr Scand, 63: 465, 1974.–Hereditary, neonatal cholestasis with primary lymphoedema at birth or later have earlier heen described in two families of Norwegian origin. This report includes the clinical course and the liver histology in two new cases with this syndrome. The two patients were unrelated and unrelated to the earlier described families. Both had neonatal intrahepatic cholestasis, in one lasting many years, in the other only about 6 months. Lymphwdema was severe in the fvst mentioned and more moderate in the latter. Liver histology showed giant cell transformation in infancy and some fibrosis or cirrhosis in later childhood. In the first family 3 of 6 siblings were similarly affected at birth, and in the other family, the parents were related, supporting earlier theory of an autosomal, recessive heredity as the aetiology of this condition.
FAMILIAL ATAXIC DIPLEGIA
Authors: SUBRAHMANYAM G. , TRIPATHI A. M. , AGARWAL K. N.
Published: January 2008

Abstract. Subrahmanyam, G., Tripathi, A. M. and Agarwal, K. N. (Department of Paediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India). Familial ataxic diplegia. Acta Paediatr Scand, 63: 472, 1974.–A family with ataxic diplegia, mental and physical growth retardation in two generations is presented. The disease was limited to male members, suggesting a sex‐linked transmission.
ELEVATION OF CONGENITAL DEPRESSED FRACTURES OF THE SKULL BY THE VACUUM EXTRACTOR
Authors: TAN K. L.
Published: January 2008

ABSTRACT. Tan. K. L. (Department of Paediatrics, University of Singapore, Singapore). Elevation of congenital depressed fractures of the skull by the vacuum extractor. Ada Paediatr Scand 63: 562, 1974.—The vacuum extractor has been found to be effective in elevating depressed fractures of the skull, especially those of some duration. Two cases are reported to illustrate this procedure. No ill effects have been encountered.
THE TRANSCAPILLARY ESCAPE RATE OF T‐1824 IN NEWBORN INFANTS OF DIABETIC MOTHERS AND NEWBORN INFANTS WITH RESPIRATORY DISTRESS OR BIRTH ASPHYXIA
Authors: INGOMAR C. JOH. , KLEBE J. G.
Published: January 2008

ABSTRACT. Ingomar, C. Joh. and Klebe, J. G. (Diabetes Center of the Royal Maternity Hospital and the University Department for Newborn Infants, Rigshospitalet, Copenhagen, Denmark). The transcapillary escape rate of T‐1824 in newborn infants of diabetic mothers and newborn infants with respiratory distress or birth asphyxia. Acta Paediatr Scand, 63: 565, 1974.—The influence of certain clinical conditions (idiopathic respiratory distress, birth asphyxia and diabetic embryopathy) on the transcapillary escape rate of human albumin, was investigated in 52 newborn infants. The dyestuff T‐1824 (Evan's blue) was used for the labelling of plasma albumin in vivo, and its plasma concentration was determined spectrophotometrically using a micro‐method. From serial measurements carried out during the first hour following the injection of T‐1824, the escape rate (%/hour) was calculated. Among healthy newborn infants the escape rate was found to increase proportional to the magnitude of the placental transfusion. The same applied to infants with respiratory distress and infants of diabetic mothers, the escape rate of whom did not differ from that of healthy infants. By contrast, the escape rate of albumin was, among some cases of birth asphyxia, found to be increased out of proportion to the placental transfusion, which the infants had received. It is discussed whether the increased escape rate found in these cases is caused by an increased capillary permeability or an increased capillary surface area.
DOWN'S SYNDROME IN SWEDEN An Epidemiological Study of a Three‐year Materiral
Authors: LINDSJÖ A.
Published: January 2008

ABSTRACT. Lindsjö, A. (Department of Drugs, National Board of Health and Welfare, Stnckholm, Sweden). Down's syndrome in Sweden — an epidemiological study of a three‐year material. Acta Paediatr Scand, 63: 571, 1974.—A continuous, exact monitoring of the incidence of congenital chromosomal disorders is of great interest for the study of the possible significance of exogenous agents in the origin of various Chromosome aberrations. An investigation of the incidence of Down's syndrome in Sweden has been carried out covering the years 1968–70. The total incidence (1:755) was found to he lower than in earlier Swedish investigations. This is probably due to a reduction in the mean maternal age at childbirth. The earlier, characteristic bimodal distribution of maternal age at the birth of children with Down's syndrome has now been reversed. The age‐specific incidences showed significantly higher values for the maternal age groups 2629 and more than 40 years in comparison with two earlier extensive surveys from the United Kingdom and Australia. A higher confirmation efficiency due to reports from cytogenetic laboratories may also possibly have contributed to the higher Swedish figures. From 1971 onward, Down's syndrome will be reported to the Swedish register of congenital malformations, so that changes in the incidence can be continuously observed. The investigation demonstrates the difficulty in comparing incidence figures from different time periods of a disease, the origin of which is dependent on maternal age.
LYSOZYMES IN FECES FROM INFANTS AND CHILDREN
Authors: HANEBERG BJÖRN , FINNE PER
Published: January 2008

ABSTRACT. Haneberg, B. and Finne, P. (Department of Pediatrics and the Broegelmann Research Laboratory for Microbiology, The University of Bergen, School of Medicine, Bergen, Norway). Lysozymes in feces from infants and children. Acta Paediatr Scand, 63: 588, 1974.—An agar diffusion technique seemed useful for the assay of lysozyme activity in serum, milk, and extracts of freeze‐dried feces. Normal levels of this activity were established before the method was applied to extracts of feces from premature infants, receiving either human milk, with lysoyme activity of its own, or cow's milk with or without added albumen lysozyme. Results of these investigations, in addition to immunologic studies, using antisera to human and albumen lysozymes, indicated that human milk lysozyme and albumen lysozyme in cow's milk formula may pass through the infants' intestinal tract. Albumen lysozyme, however, will most often be inactivated before being excreted with feces. Low fecal pH was found in those receiving albumen lysozyme as well as in those receiving human lysozyme. This may reflect the intestinal activity of the lysozymes. Low intestinal pH, brought about by lactulose in the cow's milk feed, did not in itself lead to increased lysozyme activity. At present, no advantage of adding albumen lysozyme to cow's milk formula has been substantiated.
ASPECTS ON REWARMING NEWBORN INFANTS WITH SEVERE ACCIDENTAL HYPOTHERMIA
Authors: TAFARI N. , GENTZ J.
Published: January 2008

ABSTRACT. Tafari, N. and Gentz, J. (Department of Paediatrics, Haile Selassie I University, Addis Ababa and the Department of Paediatrics, Karolinska Institutet, S:t Göran's Hospital for Children, Stockholm, Sweden). Aspects on rewarming infants with severe accidental hypothermia. Acta Paediatr Sad, 63: 595, 1974.—Thirty low birth weight infants with severe hypothermia corresponding to a rectal temperature of ≤32°C (range 26.0°C‐32.0°C) were studied. Fourteen infants were rewarmed according to a slow and 16 infants according to a fast procedure. The alleged disadvantages of fast warming over slow warming were not demonstrated. A significant fall in mortality was achieved in both groups following the intravenous administration of 0.15 M saline in a volume of 20 ml per kg bodyweight early in the rewarming procedure, from 9 out of 12 dead to 3 out of 18. The reason for this effect of saline infusion is not clear but extrapolating from induced hypothermia restoration of plasma volume is a possible explanation. Factors such as birthweight, degree and duration of hypothermia were also important in influencing survival. Although there was no difference between the two methods in the outcome in this material, analysis of temperatures recorded during rewarming (rectal, skin and environmental air temperature) showed that the rapidly warmed infants increased their body temperature as a result of net transfer of heat from the ambient air. In contrast, among the slowly warmed infants there was no demonstrable transfer of heat from the ambient air. It therefore appears that the rapid warming of infants with severe hypothermia may have the advantage of minimizing additional energy expenditure for heat production.
THE EFFECT OF L‐DOPA ON THE BLOOD CONCENTRATIONS OF GROWTH HORMONE, THYROTROPHIN, GONADOTROPHINS, CORTISOL AND GLUCOSE IN CHILDREN WITH SHORT STATURE
Authors: NILSSON K. O. , THORELL J. I.
Published: January 2008

ABSTRACT. Nilsson, K. O. and Thorell, J. I. (Department of Paediatrics, Endocrinology and Nuclear Medicine, University Clinics, Malmö General Hospital, Malmö, Sweden). The effect of L‐dopa on the blood concentrations of growth hormone, thyro‐trophin, gonadotrophins, cortisol and glucose in children with short stature. Acta Paediatr Scand,63: 812, 1974.—Studies were performed in nine constitutionally short children to evaluate the diagnostic usefulness of oral L‐dopa as compaired to insulin induced hypoglycemia as a provocative agent for the stimulation of growth hormone production. In addition the effect of L‐dopa on the blood concentrations of thyro‐trophin, gonadotrophins, cortisol and glucose was investigated. The mean growth hormone concentration was 4.8±1.6 (S.E.M.) ng/ml before L‐dopa and 2.9±1.0 ng/ml before insulin. The mean peak growth hormone concentration was 20.7±2.6 ng/ml after L‐dopa and 17.5±3.1 ng/ml during insulin induced hypoglycemia. AU children showed peak growth hormone concentrations at or above 8 ng/ml both after L‐dopa and insulin, although in one patient this level was reached only after priming with testosterone. No significant changes in the blood concentrations of thyrotrophin, gonadotrophins, cortisol or glucose were observed. It is concluded that L‐dopa is an effective stimulus for the release of growth hormone in young subjects.
α1,‐ANTITRYPSIN DEFICIENCY Pi Genotype ZO, SO und MO
Authors: LAURELL C.‐B. , SVEGER T. , LJUNGGREN G.‐G.
Published: January 2008

ABSTRACT. Laurell, C.‐B., Sveger, T. and Ljunggren, C.4. (Departments of Clinical Chemistry and Paediatrics, Malmö General Hospital, Malmö, and the Department of Paediatrics, Norrköping Central Hospital, Norrköping, Sweden). α1‐antitrypsin deficiency: Pi genotype ZO, SO and MO. Ada Paediatr Scand,63: 855, 1974—Of 88 300 babies screened for classical α1,‐antitrypsin deficiency, samples from 98 were requested for Pi typing. Among the first 67 infants studied, one was found to have a Pi type suggesting incompatibility with the mother. The serum concentration of α1,‐antitrypsin was determined with electroimmunoassay and the Pi type by acid‐crossed immunoelectro‐phoresis. The family study produced strong evidence for the occurrence of an inherited Pi0 allele appearing as Pi genotype MO, SO and ZO with increasing degree of α1‐anti‐trypsin deficiency. The phenotype Pi ZZ may cover subjects of genotype Pi ZZ and Pi ZO, which may complicate prognostic studies of classical a,‐antitrypsin deficiency. However, the Z‐allele seems to be at least 20 times more common than the 0 allele.
RENAL FUNCTION IN NEWBORN INFANTS WITH HIGH HEMATOCRIT VALUES BEFORE AND AFTER ISOVOLEMIC HAEMODILUTION
Authors: APERIA A. , BERGQVIST G. , BROBERGER O. , THODENIUS K. , ZETTERSTRÖM R.
Published: January 2008

ABSTRACT. Aperia, A., Bergqvist, G., Broberger, O., Thodenius, K. and Zetterström, R. (Department of Paediatrics, St Göran's Children's Hospital, Kardinska Institutet, Stockholm, Sweden). Renal function in newborn infants with high hematocrit values, before and after isovolemic hernodilution. Acta Paediatr Scand63: 878, 1974.—To evaluate the effect of high hematocrit (secondary polycythemia) on renal function in newborn babies, 10 infants with values above 70% have been studied before and after an isovolemic hemodilution. By replacing blood with a solution containing albumin, glucose and sodium chloride the hematocrit was reduced to about 60%. Hematocrit, blood viscosity, glomerular filtration rate (single injection technique), and the renal response to an oral sodium and fluid load were determined before and after hemodilution. The fall in hematocrit was accompanied by a concomitant fall in blood viscosity. All parameters of renal function which were studied were low before hemodilution but improved after this procedure. Water excretion increased out of proportion to glomerular filtration rate. It is suggested that the reduction in renal function as seen in newborn infants with high hematocrit are secondary to an impairment of glomerular plasma flow which in turn is the consequence of high viscosity. The depression of renal function as seen in polycythemic newborn infants may cause marked changes in the handling of certain drugs.
A RECESSIVE DISORDER WITH GROWTH AND MENTAL RETARDATION, PECULIAR FACIES, ABNORMAL PIGMENTATION, HEPATIC CIRRHOSIS AND AMINOACIDURIA
Authors: TAY C. H. , RAJAGOPALAN K. , McEVOY‐EfOWE E. , TOCK. E. P. C. , COSTA J. L. Da
Published: January 2008

ABSTRACT. Tay, C. H., Rajagopalan, K., McEvoy‐Bowe, E., Tock, E. P. C. and Da Costa, J. L. (Departments of Medicine, Biochemistry and Pathology, University of Singapore, Singapore, and the Skin Clinic, General Hospital, Johore Bahru, West Malaysia). A recessive disorder with growth and mental retardation, peculiar facies, abnormal pigmentation, hepatic cirrhosis and aminoaciduria. Acta Paediatr Scand, 63: 777, 1974.—Two Indian teenage sisters from West Malaysia were recently found to have a previously undescribed syndrome consisting of (1) growth retardation and hypogonad‐ism, (2) mental deficiency, (3) peculiar fades consisting of microcephely, triangular shaped face, prominent eyes, hypoplastic alae nasi, small ‘pinched’ nose., tiny mouth and large, pegged‐shaped incisors, (4) abnormal pigmentations such as cafäau‐lait spots, multiple lentigines, peripheral vitiligo and premature canities, (5) abnormal limbs consisting of trident hands, hypoplastic transverse palmar creases, large big toes and short, stubbed small toes, (6) liver involvement with fatty infiltration and hepatic cirrhosis with hypersplenism, (7) raised serum globulins and serum immunoglobulins, and (8) hyperaminoaciduria mainly of taurine, β‐aminoisobutyric acid and glycine. This syndrome is probably due to a recessive autosomal trait.

The relationship of this to other similar conditions, especially the ‘Bird‐Headed Dwarfism’ is briefly discussed.
USE OF THE NITROBLUE TETRAZOLIUM (NBT) TEST IN THE DIFFERENTIATION BETWEEN PYELONEPHRITIS AND CYSTITIS
Authors: BJÖRKSTÉN B. , de CHATEAU P.
Published: January 2008

ABSTRACT: Björkstén, B. and de Chateau, P. (Department of Paediatrics, Umeå University, Umeå, Sweden.) Use of the Nitroblue Tetrazolium (NBT) Test in the differentiation between pyelonephritis and cystitis. Acta Paediatr Scand 64:182, 1975.– NBT tests were performed on blood from 37 patients with urinary tract infections caused by conform bacteria. The level of infections was evaluated by the clinical signs, ESR, renal concentrating capacity and titre of antibodies against the strains of E. coli isolated in their urine. Elevated proportions of NBT‐positive neutrophils were found in 11 of 14 patients with pyelonephritis and in 3 of 23 patients with cystitis or asymptomatic bacteriuria (ABU). The total number of NBT‐positive neutrophils was 1000 or more per mm3 blood in 11 of 13 patients considered to have pyelonephritis, while it was 800 or less in all the patients investigated, evaluated as having cystitis or asymptomatic bacteriuria. The NBT test is recommended as an adjunct in the level diagnosis or urinary tract infections in children. The utility of the test in smouldering pyelonephritis is presently being investigated.
LEVEL DIAGNOSIS OF SYMPTOMATIC URINARY TRACT INFECTIONS IN CHILDHOOD
Authors: JODAL U. , LINDBERG U. , LINCOLN K.
Published: January 2008

ABSTRACT: Jodal, U., Lindberg, U. and Lincoln, K. (Department of Paediatrics and Institute of Medical Microbiology, University of Göteborg, Göteborg, Sweden). Level diagnosis of symptomatic urinary tract infections in childhood. Acta Paediatr Scand, 64:201, 1975.–As no method for localization of urinary tract infection has been shown to be absolutely reliable, six procedures have been carried out simultaneously in 25 girls with acute symptomatic infections and the reliability of each method assessed. While clinical diagnosis of pyelonephritis or cystitis correlated well with the overall results of the battery of tests, the reliability of individual tests varied. Highest reliability was obtained with CRP determinations followed by antibody titration, sedimentation rate, and renal concentrating capacity. In the bladder washout test only 8 of the 14 patients with pyelonephritis had findings clearly indicating high infection. Intermittent or inadequate discharge of bacteria from the renal parenchyma is suggested as the major source for this inaccuracy. In fact, half of the 42 final washout specimens from girls with acute pyelonephritis contained less than 1 000 bacteria per ml, indicating that low numbers of organisms in ureteric urine is common in childhood pyelonephritis.
BETAMETHASONE 17‐VALERATE IN CHILDHOOD ASTHMA
Authors: TAYLOR BRENT , NORMAN A. P.
Published: January 2008

ABSTRACT: Taylor, B. and Norman, A. P. (The Hospital for Sick Children, Great Ormond Street, London, England). Betamethasone 17‐valerate aerosol in childhood asthma. A double‐blind crossover trial in children not taking other corticosteroid therapy. Acta Paediatr Scand, 64:234, 1975.–A duble‐blind crossover trial of Betamethasone 17‐Valerate steroid aerosol compared with placebo was carried out on 18 children with severe intermittent or chronic asthma. Seventeen of the 18 children showed a clinically obvious improvement on the active drug. The remaining child was unable to use the inhaler properly. In 14 children the response was dramatic. Improvement was shown at home by a reduction in the number of asthmatic symptoms, improvement in twice daily peak flow rates and by a lowered intake of sympathomimetic tablets. At monthly clinic visits, improvement was shown by chest examination, weight gain and by detailed respiratory function tests. By each of these criteria for improvement the benefit of Betamethasone 17‐Valerate over the placebo was highly significant. There were no side effects attributable to the therapy and no evidence, as judged by short tetracosactrin tests, of adrenal suppression resulting from the drug. Betamethasone 17‐Valerate appears to be a safe and effective therapy in children with severe asthma.
TEMPERATURE REGULATION IN CHILDREN DURING EXERCISE
Authors: GULLESTAD R.
Published: January 2008

ABSTRACT: Gullestad, R. (Institute of Work Physiology and Institute of Zoophysiology, University of Oslo, Oslo, Norway). Temperature regulation in children during exercise. Acta Paediatr Scand, 64:257, 1975–Rectal and skin temperature and sweat rate were measured in eight 11‐year‐old boys exercising one hour on a bicycle ergometer at each of three different work loads. Rectal temperature rose according to the relative work load and reached a steady state level after a shorter time than had been previously observed in adult subjects. A good relationship was observed between the levelling off value of rectal temperature during work and the relative work load, but the value of rectal temperature at rest just before the start of work affects this steady state value. The average skin temperature was kept fairly constant during exercise except on the heaviest work load (70%), during which it rose about 1o mainly as a result of the rise in skin temperature on the arm, hand and thigh. Temperature increases on skin locations in general were minimal except on the back where the temperature decreased slightly. Sweat rate showed a close relationship to the absolute work load, and in this report the conclusion is supported that the 11‐year‐old boys regulate their body temperature during exercise at constant work load in the same way as do adult subjects.
STUDIES ON PHOTOTHERAPY IN NEWBORN INFANTS
Authors: Jr ADOLF WINDORFER , FAXELIUS GERD , BOREUS LARS O.
Published: January 2008

ABSTRACT: Windorfer, A., Faxelius, G. and Boréus, L. O. (Departments of Clinical Pharmacology and Paediatrics, Karolinska Hospital, Stockholm, Sweden). Studies on phototherapy in newborn infants: Influence on protein binding of bilirubin and salicylate and on activity of acetylsalicylic acid esterase. Acta Paediatr Scand 64:293, 1975.– Phototherapy of newborn infants with hyperbilirubinemia was shown to result in an increase in hematocrit values and in the activity of the erythrocyte enzyme acetylsalicylic acid esterase. The elevation of the enzyme activity also could be produced in light‐treated rabbits and in vitro after illumination of blood from adult volunteers. The binding of bilirubin to serum albumin and of salicylate to plasma proteins did not alter, nor did the concentrations of albumin or total proteins in plasma. It is concluded that light does not increase the unbound fraction of bilirubin in blood.