bluuebunny/crossref_metadata_2025_split huggingface dataset:
THE DIAGNOSIS OF TAY‐SACHS DISEASE1
Authors: OHMAN ROLF , EKELUND HANS , SVENNERHOLM LARS 
Published: January 2008

SummaryGangliosides and neutral aminoglycolipids were determined in several organs from two subjects with classical Tay‐Sachs disease. Ganglioside GM2 was stored in all organs investigated. Of the extraneural organs, the highest concentration was found in the adrenals, spleen and liver. The rectal muscular layer had a high concentration of gangliosides. Rectal biopsy might be used for studies of the nervous ganglioside pattern.The brain tissue showed a many‐fold increase of the activities of p‐nitrophenylglycosi‐dases. The activity of ganglioside sialidase in the brain was not increased in Tay‐Sachs and some other neurometabolic diseases. Subfrac‐tionation of the glycohydrolases by ultracen‐trifugation showed no hexosaminidase activity in the supernatant fraction of the brain in Tay‐Sachs disease compared with about 2/3 of the total activity in normal human brain.A simple agarose electrophoretic method was developed for the separation of the hexosaminidases into two fractions, A and B. Hexosaminidase A was virtually absent in Tay‐Sachs disease.The known forms of gangliosidoses and their biochemical characteristics are reviewed and a scheme is given for their classification and diagnosis.
CHRONIC HYPOMAGNESEMIA WITH MAGNESIUM‐DEPENDENT HYPOCALCEMIA
Authors: NORDIO S. , DONATH A. , MACAGNO F. , GATTI R. 
Published: January 2008

SummaryA study has been carried out on the relationship between M, Ca and Sr in a case of primary Mg‐malabsorption and in control children on high‐ and low‐Mg diets.Probably more than one transfer system exists for these cations in intestine and in Mg‐malabsorption the simple diffusion of both Mg and Sr is impaired.The influence of Mg on Ca‐P homeostasis is not clearly understood but a resistance to parathormone and vitamin D in Mg‐deficiency is emphasized.
RUBIDOMYCIN IN BLASTIC PHASES OF ACUTE PROMYELOCYTIC AND MYELOBLASTIC LEUKEMIA IN CHILDREN
Authors: ARMATA J. , WYSZKOWSKI J. , CYKLIS R. 
Published: January 2008

SummaryRubidomycin was administered in doses of 1 mg/kg body weight, usually in combination with prednisone. The best results were obtained in promyelocytic leukemia. Promyelocy‐tic leukemia in children seems to be characterized by sensitivity to rubidomycin even in late exacerbations.
NITROBLUE‐TETRAZOLIUM REDUCTION BY NEUTROPHILS OF NEWBORN INFANTS IN IN VITRO PHAGOCYTOSIS TEST
Authors: COCCHI P. , MORI S. , BECATTINI A. 
Published: January 2008

SummaryVenous blood from healthy premature infants, premature infants with known bacterial infections, normal term infants and adults, was incubated in vitro with Pseudomonas aeruginosa. At 30 min intervals, blood samples were tested for the nitroblue‐tetrazolium (NBT) reducing activity of neutrophils. The results of the histochemical NBT test were compared with those of the blood controls from which bacteria were omitted.The stimulation of neutrophils with Ps. aeruginosa induces a considerable increase of NBT reduction in all 4 groups of subjects examined. The maximum rate of NBT reduction was observed after 1 hour of incubation with the test organism, and reached its lowest degree after 3 hours. By contrast the NBT reduction by un‐stimulated neutrophils of the same subjects remain unchanged during a 3 hour period of incubation.
HEPATIC INVOLVEMENT IN THE COURSE OF ACQUIRED TOXOPLASMOSIS
Authors: KOUBA KAREL , JIRA JINDŘICH , ZITOVA DAISY 
Published: January 2008

SummaryThe authors report hepatosplenomegaly in 5‐year‐old girl together with pluriglandularlymphadenopathy, hepatic involvement and eosinophilia. Antibody tests for toxoplasmosis revealed high titres. The condition improved after treatment with toxoplasmacidal drugs.SummaryThe authors report hepatospienomegaly in a 5‐year‐old girl together with pluriglandular lymphadenopathy, hepatic involvement and eosinophilia. Antibody tests for toxoplasmosis revealed high titres. The condition improved after treatment with toxoplasmacidal drugs.
COMPARISON OF GLOMERULAR FILTRATION RATE AND EFFECTIVE RENAL PLASMA FLOW DETERMINATIONS OBTAINED BY A SINGLE INJECTION TECHNIQUE AND BY MEANS OF A STANDARD CLEARANCE TECHNIQUE IN CHILDREN
Authors: VÖGELI B. , RIEDWYL H. , DONATH A. , OETLIKER O. 
Published: January 2008

SummaryIn children 28 values for glomerular filtration rate and 25 values for effective renal plasma flow were simultaneously obtained by means of the inulin‐ and PAH‐clearance and by means of a single injection method with 51Cr‐EDTA and 123I‐OIH. These were compared in an appropriate statistical model. The results show good agreement of the two methods. The single injection method can therefore be of value in childhood when only values for GFR and effective renal plasma flow are asked or especially if inappropriate urinary collection is expected or bladder catheterisation is contraindicated. It has no place in studies concerned with renal physiology or pathophysiology.
HIGH FREQUENCY OF SALMONELLA SPECIES AS A CAUSE OF NEONATAL MENINGITIS IN IBADAN, NIGERIA
Authors: BARCLAY NAOMI 
Published: January 2008

Summary538 cases of bacterial meningitis admitted from January 1964 to May 1969 in all age groups at the U.C.H., Ibadan, were reviewed. The proportion of enterobacterial cases of meningitis admitted to the U.C.H., Ibadan was higher than has been reported from any other centre, the maximum incidence being in the neonatal period. A very large number of these were due to various salmonella species, and 70% of these are currently resistant to the more commonly used antibiotics, including chloramphenicol. This resistance to chloramphenicol is reflected amongst all enterobacteria isolated in Ibadan and poses a considerable problem. At present, over 90% of neonates with enterobacterial meningitis die and this case fatality is maintained in the older age groups in cases where resistant strains are encountered.
ANAPHYLAXIS AND RED CELL SURVIVAL STUDIES IN A CHILD WITH INSULIN‐RESISTANT DIABETES MELLITUS
Authors: TOMSOVIC E. J. , FAULK W. PAGE , FUDENBERG H. H. 
Published: January 2008

SummaryA child with the syndrome of diabetes mellitus, insulin‐resistance, Coombs' positive autoimmune hemolytic anemia, lymphadenopathy, and hepatosplenomegaly was described. The patient's IgG anti‐insulin antibody was shown to be negative for passive cutaneous anaphylaxis (PCA). In as much as the patient was refractory to prednisone therapy and presented a negative PCA test, he was consistent with the Oakley hypothesis that insulin‐resistant prednisone‐insensitive patients will be negative for PCA. The half‐life of the patient's red blood cells was shown to vary as a function of the type of insulin used in therapy. The child was shown to have immune‐complexes in his serum of insulin‐anti‐insulin specificity, and it was suggested that these complexes are partially responsible for the microangiopathy that is so often observed in diabetes mellitus.
HOMOVANILLIC ACID AND 5‐HYDROXYINDOLEACETIC ACID IN CEREBROSPINAL FLUID OF A CHILD WITH FAMILIAL DYSAUTONOMIA
Authors: ANDERSON H. , HAGNE I. , ROOS B. E. 
Published: January 2008

SummaryA case of familial dysautonomia with a high level of HVA and a normal level of 5‐HIAA in CSF is described. After probenecid loading test the increase in HVA was only slight, while the increase in 5‐HIAA was normal. These findings suggest an explanation for the pathogenesis of the disease.
STORAGE IRON IN FOETAL LIVERS
Authors: CHANG L. L. 
Published: January 2008

SummaryStorage iron was estimated in the livers and kidneys of 12 premature and 12 mature stillborn infants. In both organs, the total storage iron content more than doubled during the last weeks of gestation. There was a significant positive correlation between the level of storage iron in these organs and the birth weight and gestational age of the infant.
DYNAMIC PRESSURE‐VOLUME RELATIONSHIP OF THE LUNG AND POSITION IN HEALTHY NEONATES
Authors: SPOELSTRA A. J. G. , SRIKASIBHANDHA S. 
Published: January 2008

SummaryThere is no significant difference in the dynamic V/P loops of the lung between supine position with the head in normal median position, and prone position, i.e. when there is no deformation of the upper airways. In both prone and supine position with flexion or rotation of the head, the slope of the V/P loops was decreased and the width of the loops in creased. In pulmonary function tests special attention should therefore be given to the position of the head in relation to the body.
THE OCCURRENCE OF TWO VASCULAR RINGS IN THE SAME INFANT
Authors: AHLSTRÖM HANS , LUNDSTRÖM NILS‐RUNE , MORTENSSON WIGHER 
Published: January 2008

SummaryA fatal case of an infant with a combination of two vascular rings is described. One ring consisted of a right‐sided aortic arch, aberrant left subclavian artery and persistent ductus arteriosus, the other of an anomalous left pulmonary artery branched off from the right pulmonary artery. The importance of a properly performed roentgen examination of the oesophagus is stressed.
STUDIES ON GROWTH HORMONE SECRETION IN A PATIENT WITH THE DIENCEPHALIC SYNDROME OF EMACIATION
Authors: HÄGER A. , THORELL J. I. 
Published: January 2008

SummaryA girl with a large optic glioma, and the typical features of diencephalic syndrome of emaciation (Russell) was followed up from 1/2 to 2 1/2 years of age. She had very high levels of growth hormone (GH) in the plasma, which were not influenced by hyperglycaemia, by hypoglycaemia or by dexamethasone, reserpine or chlorpromazine. Interference by the glioma with GH releasing factor or more likely with GH inhibiting factor is suggested. In spite of the elevated plasma GH, linear growth was markedly retarded. Sulfation factor activity (Somatomedin) was low with no significant response to injections of HGH. The patho‐genesis of the profound metabolic disturbances in this syndrome is not properly understood. The endogenous GH obviously exerts its full adipokinetic effect, whereas the synthesis of the sulfation factor (Somatomedin) must be defective. This case illustrates a type of retardation of linear growth, not seen in any other known syndrome.
HUMAN GROWTH HORMONE THERAPY IN HYPOPITUITARISM DUE TO TUBERCULOUS MENINGITIS
Authors: BARTSOCAS CHRISTOS S. , PANTELAKIS STEFANOS N. 
Published: January 2008

SummaryLesions due to tuberculous meningitis resulting in endocrine dysfunction are not rare. They usually cause inappropriate ADH secretion, but occasionally other endocrinopathies related to the affected hypothalamic or pituitary regions are seen.Two female patients, 13 1/2 and 16 1/2 years old, treated in early childhood for tuberculous meningitis are presented. Both were very short and had intracranial calcifications; however, the hypothalamic area was involved in only one of the patients. Both had GH and ADH deficiency, while one of them had gonado‐tropin deficiency as well. Administration of HGH resulted in an adequate growth response.
NEONATAL HYPERTHYROIDISM
Authors: ØRBECK HARALD 
Published: January 2008

SummaryNeonatal Graves disease, with frank cardiac failure and pronounced bilateral breast enlargement, is described in a premature infant female.Thyrotoxicosis, culminating rapidly in thyroid storm, was successfully treated by administration of Lugol's solution, digitalis and phenobarbital.The case is discussed in reference to the legitimacy of long‐acting thyroid stimulator (LATS) as the originator of neontal thyrotoxicosis.Although LATS could not be detected, the occurrence of gastric parietal cell antibodies in both mother and child yielded presumptive evidence for thyrotoxicosis as an autoimmune disease.
CONGENITAL ERYTHROPOIETIC PORPHYRIA WITH A HITHERTO UNDESCRIBED PORPHYRIN PATTERN
Authors: HOFSTAD F. , SEIP M. , ERIKSEN L. 
Published: January 2008

SummaryOne proven and one probable case of CEP in second cousins from Northern Norway are reported. The porphyrin pattern in Case 1, who has been studied extensively, in certain respects differs from that reported earlier in CEP, indicating that the disease may be genetically heterogeneous. The most impressive finding is the extraordinary high amounts of 7 carboxylic porphyrin in urine. Isomer analysis has shown this porphyrin to belong to the isomer III series. The presence of increased amounts of protoporphyrin in plasma and feces is also at variance with the picture seen in classical CEP.
PERINATAL ACIDOSIS AND PLACENTAL TRANSFUSION1
Authors: CHOU PAULA J. , ACKERMAN BRUCE D. 
Published: January 2008

SummaryUmbilical artery pH and residual placental blood volume (RPBV) were determined for 75 full‐term newborn infants born by vaginal delivery. For 39 infants of multiparous mothers (IMM), there was a significant relationship between fetal acidosis and reduced RPBV. For 36 infants of primiparous mothers (IPM) a significant relationship was not present. Results of an earlier study suggested that perinatal acidosis was associated with reduced RPBV. The present study confirms this association for IMM's. The reason for the lack of a clearcut effect in IPMs is unknown.
INTERVERTEBRAL DISC CALCIFICATION IN CHILDHOOD
Authors: JESPERSEN N. B. , LUND H. T. , EGEBLAD M. 
Published: January 2008

SummaryIntervertebral disc calcification (IVDC) is a disease seldom reported in the pediatric literature. It is often associated with local symptoms from the back and signs of systemic disease. A recent case in a 6‐year‐old boy is reported.
PERCENTILES OF BIRTH WEIGHTS OF SINGLE, LIVE BIRTHS AT DIFFERENT GESTATION PERIODS: Based or. 125 485 births in Norway, 1967 and 1968
Authors: BJERKEDAL TOR , BAKKETEI LEIV , LEHMAN EGIL H. 
Published: January 2008

SummaryA total number of 125 485 single, live births with known sex and birth weight, and a gestation period estimated to be from 28.0 to 48.9 weeks, were registered in Norway in 196 and 1968. Information about these births pro vided by a national scheme of medical registration of births is used in a study of the relation between birth weight and gestation period.An almost linear relation was found to exist for gestation periods of 35 to 42 weeks. In this interval, median birth weight increased about 200 g a week. At the same gestation period male births were found to be on the average about 150 g heavier than female births. Parity also had a notable influence on birth weight. For gestation periods of 35 weeks and more, the birth weight of a first born male was found to average about 170 g less than that of a second and subsequently born. For female births the difference was 130 g.The material is suggested for use as reference for birth weight at various gestation periods and tables are included for this purpose.
PROPER LOCATION OF UMBILICAL VENOUS CATHETERS BY INTERNAL ELECTROCARDIOSCOPY
Authors: NODARI SANDRO , AGOSTINO ROCCO , BUCCI GIOVANNI 
Published: January 2008

SummaryA method is described which facilitates the location of the tip of an umbilical venous catheter in the inferior vena cava near the right atrium by means of internal e!ectrocardio‐scopy using standard umbilical catheters and cardiomonitoring equipment. Entering the atria was chiefly indicated by tall and peaked P waves. On withdrawal of the catheter, small and peaked P waves were elicited at a radiological position of the catheter tip between 0.5 cm above and 1.0 cm below the diaphragm, presumably corresponding to the inferior vena cava near the atrial inlet. The latter location was obtained in 38 (67%) out of 57 newborns undergoing the procedure. The present method is simple and accurate, and it is considered superior to methods used so far for the same purpose. The need for safety precautions against electrical hazards has been stressed.
FIBRINOLYSIS IN LYMPHANGIOMA
Authors: KORNFALT R. , NILSSON I. M. , OKMIAN L. 
Published: January 2008

SummaryTwo operations upon a giant gluteal lymph‐angioma in a boy were complicated by massive haemorrhage. The complication proved to be due to fibrinolysis stimulated by activators released by the tumour. The diagnosis was confirmed by the demonstration of high fibrino‐lytic activity and FDP in blood and discharge drained from the wound. The bleeding was effectively controlled by AMCA (tranexamic acid, Cyclokapron®). Long‐term treatment with AMCA was accompanied by further reduction in size of the lymphangioma.
THE TRANSCAPILLARY ESCAPE RATE OF T‐1824 IN HEALTHY NEWBORN INFANTS The Influence of the Placental Transfusion
Authors: INGOMAR C. JOH. , KLEBE J. G. , BÆKGAARD P. 
Published: January 2008

SUMMARYThe transcapillary escape rate of T‐1824 was measured in 12 normal newborn infants and found to be significantly higher among infants with late clamping of the umbilical cord (25.0% per hour) than in early‐clamped infants (19.1% per hour). The magnitude of the placental transfusion was assessed from the volume of the residual placental blood and it was demonstrated that the transcapillary escape rate of T‐1824 increases parallel to the placental transfusion. The disappearance rate of the early‐clamped infants was almost the same as that previously found in early‐clamped infants of diabetic mothers.
STAPHYLOCOCCI AND INFECTION IN MATERNITY WARDS IV. Studies on A Partial Rooming‐in System
Authors: LAGERCRANTZ RUTGER , NYSTRÖM BERTIL , WRETLIND BENGT 
Published: January 2008

SUMMARYInfections and colonization with Staphylococcus aureus were studied in 159 mothers and their newborns. The patients were observed when in hospital, and the mothers were given questionnaires to report symptoms of infection in themselves or their babies during the first weeks after discharge from the hospital. The infection rate measured in this way was high, 27% among the children and 16% among the mothers. Interviews with mothers and reports from child welfare centres that were attended by all children showed, however, that anxious and insecure mothers over‐reported infection symptoms. Such a re‐evaluation reduced the infection rates to 8% among the children and 7% among the mothers.No differences were observed in infection rates and in staphylococcal colonization rates between a ward with a partisal rooming‐in system and a similar ward with conventional care. A less than total rooming‐in system thus does not seem to reduce the infection rate.
THE IMP DEHYDROGENASE CATALYSED REACTION IN ERYTHROCYTES OF NORMAL INDIVIDUALS AND PATIENTS WITH HYPOXANTHINE GUANINE PHOSPHORIBOSYLTRANSFERASE DEFICIENCY
Authors: LOMMEN E. J. P. , ABREU R. A. DE , TRIJBELS J. M. F. , SCHRETLEN E. D. A. M. 
Published: January 2008

SummaryThis paper deals with a preliminary investigation concerning the possible mechanisms causing an aberrant purine nucleotide pattern in erythrocytes of hypoxanthine guanine phos phoribosyltransferase deficient individuals (Lesch‐Nyhan Syndrome). The AMP deaminase inhibitor 2,3 DPG has been found to be also a strong inhibitor of IMP dehydrogenase obtained from normal erythrocytes. IMP dehydrogenase from HGPRT‐ase deficient erythrocytes, however, showed a complete hensitivity for this inhibition.
INFANTILE ANEURYSM OF THE DUCTUS ARTERIOSUS: Diagnosis, Incidence, Pathogenesis, and Prognosis
Authors: HEIKKINEN ERKKI S. , Ä SEPPO SIMIL , LAITINEN JUHANI , LARMI TEUVO 
Published: January 2008

Abstract. Heikkinen, E. S., Similä, S., Laitinen, J. and Larmi, T. (Departments of Surgery, Paediatrics and Roentgenology, University of Oulu, Oulu, Finland). Infantile aneurysm of the ductus arteriosus. Acta Paediat Scand, 63:241, 1974.–A neoplastic‐like density on X‐ray is not a rare diagnostic problem in infancy. Nevertheless, knowledge about the infantile ductal aneurysm (IDA) producing a characteristic tumour‐like mediastinal shadow on plain chest film is very limited.In 1972 the authors operated two newborns because of left‐sided tumour‐like mediastinal shadows produced by IDA. A very similar radiographic mass according to location, shape and opacity could he discerned retrospectively on the chest films of 10 other neonates in a material of 1 138 newborn babies, who had been radiographed mainly for respiratory troubles. Apparently the IDA is not so rare as considered in the literature.The patients presented herein were found during their first day tb have inadequate ventilation, a low Apgar score and respiratory acidosis. Therefore postnatal hypoxaemia was considered the most probable primary factor in the pathogenesis of IDA.A follow‐up examination at the age of 6–24 months showed that the typical tumour‐like mediastinal shadow had disappeared also in the non‐operated patients. A spontaneous, uncomplicated involution seems to he the most usual outcome of IDA. HOWever, according to the literature IDA is a potentially dangerous abnormality. It calls for meticulous observation since surgical intervention is indispensable if fatal complications seem imminent.
CHEMICAL PATHOLOGY OF KRABBE'S DISEASE I. Lipid Composition and Fatty Acid Patterns of Phosphoglycerides in Brain
Authors: VANIER MARIE THERESE , SVENNERHOLM LARS 
Published: January 2008

ABSTRACT. Vanier, M.‐Th. and Svennerhohn, L. (Department of Neurochemistry, Psychiatric Research Centre, University of Göteborg, Göteborg, Sweden). Chemical pathology of Krabbe's Disease. I. Lipid composition and fatty acid patterns of phosphoglycerides in brain. Acta Paediatr Scand, 63: 494, 1974.—Lipid biochemical determinations were performed on autopsy material from 18 cases of Krabbe's disease, aged 7–23 months, collected in Sweden between 1960 and 1972.Cerebral white matter. The lipid concentration of the cerebral white matter was found substantially reduced. It was the same in 17 cases, independent of age at death, and rather similar to that of cerebral cortex. All major lipids were diminished, but the myelin lipids, cholesterol and particularly galactolipids (cerebrosides and sulfatides) were reduced more than the phospholipids. The ratio cerebroside/sulfatide was significantly increased, but this. was not a constant finding. The relative percentage of ethanolamine phosphoglycerides was decreased and that of choline phosphoglycerides increased. The fatty acid composition of the phosphoglycerides shifted towards values found in the white matter of 2 to 4 month‐old infants. In addition, a characteristic significant increase of arachidonic acid occurred.Cerebral cortex. Only minor changes were observed, but they were of the same type as some of the changes of the white matter: a decrease in the relative percentage of ethanolamine phosphoglycerides and an increase of arachidonic acid.The lipid changes in Krabbe's disease are suggested to be the result of the combined effect of a serious myelin deficiency, and the replacement of the normal brain cytoarchi‐tecture by proliferated astroglial cells, ephitheliod cells and globoid bodies.