bluuebunny/crossref_metadata_2025_split huggingface dataset:
SIMULTANEOUS DETERMINATION OF PLASMA VOLUME AND TRANSCAPILLARY ESCAPE RATE WITH 131 I‐LABELLED ALBUMIN AND T‐1824 IN THE NEWBORN
Authors: PARVING H.‐H. , KLEBE J. G. , INGOMAR C. J. 
Published: January 2008

SummaryA new micro method for plasma volume determination with T‐1824 was investigated in 10 newborn infants. Dye concentration was measured with a two wavelength spectrophotometric method. The method was found to be easy, rapid to perform and requires only 150 μl plasma. Thus several plasma samples could be obtained and the dye loss during mixing time calculated and corrected for. This is of special importance in the newborn due to the high transcapillary albumin loss mentioned below. Plasma volume was determined with 131I human serum albumin (RIHSA) serving as reference. Identical results were obtained, mean 43 ml/kg. Consequently it is concluded that the dye method, being a non‐radioactive method, should be preferred for plasma volume determination in the newborn. Transcapillary escape rate of albumin (fraction of intra‐vascular mass of albumin escaping to the extravascular space per unit time) was determined from the disappearance of intravenously injected RIHSA and T‐1824 during the first hour after the injection. The mean transcapillary escape rate with RIHSA was: 18.4%/hour, with T‐1824: 20.7%/hour (p<0.1). These values are 3 to 4 times higher than those obtained in adults thus indicating a high capillary permeability to albumin in newborns.
ACCIDENTS AND SURGICAL EMERGENCIES IN A POPULATION OF MENTALLY RETARDED CHILDREN
Authors: BERGGREEN SHEILA MARGARET 
Published: January 2008

SummaryThe incidence of casualties in an institution for mentally retarded children is shown to be about three times as great as in the normal Danish child population. The circumstances concerned are analysed by means of a retrospective investigation to elucidate the mechanisms involved and the contributory factors. The investigation reveals that there is a definite relationship between the degree of mental handicap and the underlying disease (e.g. epilepsy and/or cerebral paresis, mon‐golism etc.) and the incidence of casualties. Other types of incapacity such as motor handicap, anaesthesia, defective sight or hearing and other physical disease (e.g. hydrocephalus) may predispose to accidents. Some children are undoubtedly more accident‐prone than others and the cause may be found in their disease (most frequently epilepsy) while, in others, purposeless motor activity and the inability to learn from experience combined with severe or profound mental retardation appear to be responsible. Phases of accident‐proneness may be associated with the acquiring of new skills.Some prophylactic measures are suggested which should be considered when new institutions are planned.A broad spectrum of somatic, developmental, handicap, environmental, emotional and mental factors interplay in the production of accidents in mentally retarded patients. Attempts should be made to analyse the mechanism of every accident so that appropriate prophylactic measures can be undertaken.
LOWES SYNDROME
Authors: CYVIN K. B. , WEIDEMANN J. , BATHEN J. 
Published: January 2008

SummaryTwo siblings, a boy and a girl, with characteristic features of the oculo‐cerebro‐renal syndrome of Lowe are described. In both cases the impaired renal function was a predominant feature of the disease, and both died at the age of 3–4 months. Whereas the classical form of the disease probably is X‐linked recessive, other clinical variants suggest different modes of inheritance, possibly autosomal recessive.
PRIMARY HYPERPARATHYROIDISM IN CHILDHOOD
Authors: LUND H. T. 
Published: January 2008

SummaryA case of primary hyperparathyroidism in a 12‐year‐old boy with renal lithiasis is reported. Laboratory investigations showed a cortisone‐resistant hypercalcemia and a low serum phosphate concentration. The renal handling of calcium and phosphate, too, was indicative of primary hyperparathyroidism. At the following operation a parathyroid adenoma was removed. The importance of estimating the serum calcium concentration in all uncertain states of disease in children is stressed, since primary hyperparathyroidism is probably un‐derdiagnosed in the pediatric age group.
JUVENILE POLYPS OF THE RECTUM AND COLON
Authors: TOCCALINO H. , GUASTAVINO E. , PINNI F. DE , ODONNELL J. C. , WILLIAMS M. 
Published: January 2008

SummaryFifty 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

SummaryIn 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

SummaryFive 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

SummaryExperience 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

SummaryA 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

SummaryThree 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

SummaryThis 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

SUMMARYThree 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

SUMMARYTransport 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

SUMMARYDetermination 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

SUMMARYTwo 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

SUMMARYThe 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

SummaryThe 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

SummaryIn 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

SummaryLithocholic 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

SummaryAn 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

SummaryAfter a follow‐up period for 15–40 months (mean 31 months) of 81 children, aged 2–66A 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 thebeginning of the year and the highest values in the autumn.
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.
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.