bluuebunny/crossref_metadata_2025_split huggingface dataset:
PYROGLUTAMIC ACIDURIA Studies in an Infant with Chronic Metabolic Acidosis
Authors: HAGENFELDT L. , LARSSON A. , ZETTERSTROM R. 
Published: January 2008

SummaryA one‐year‐old girl with chronic metabolic acidosis was found to have normal renal acidi‐fication. In the urine, however, she excreted a daily amount of 50 mmoles of L‐pyroglutamate (5‐oxo‐˜‐proline). This excretion did not increase with increasing protein intake. Studies of the turnover of L‐pyroglutamate under steady state conditions revealed that 75% of the pyroglutamate synthesized was metabolized by the patient. The level of pyroglutamate degrading enzyme (5‐0times0‐prolinase) in the patient's leucocytes was not decreased, and the enzyme appeared to have normal affinity for its substrates, i.e. pyroglutamate and ATP.
MUCOLIPIDOSIS II (I‐CELL DISEASE) A Clinical and Biochemical Study
Authors: WIESMANN ULRICH N. , VASSELLA FRANCO , HERSCHKOWITZ NORBERT N. 
Published: January 2008

SummaryThe diagnosis of mucolipidosis I1 (I‐cell disease) was made in a patient with a Hurler like appearance but only borderline muco‐polysacchariduria. The cultured fibroblasts of high doses of prednison, which resulted in a further increase in the enzyme activity. On the ultrastructural level there was the storage of polymorphous material in the liver and in the peripheral nerve. Vacuolated cells were present in the peripheral leucocytes and in the bone marrow. These findings indicate that the lysosmal enzyme depletion in the cells in the patient could be similar to that found in cultured cells. However, the clinical picture could also be caused by the excess activity of lysosomal enzymes in the extracellular fluids which are able to degrade the matrix substance of the connective tissues and could mimic a mucopolysaccharidosis.
CONVERSION OF ANGIOTENSIN I IN PULMONARY AND SYSTEMIC VASCULAR BEDS OF CHILDREN1
Authors: FRIEDLI BEAT , BIRON PIERRE , FOURON JEAN‐CLAUDE , DAVIGNON ANDRE 
Published: January 2008

SummaryThe systemic pressor response to bolus injections of Angiotensin I (A‐I) into the pulmonary artery (PA) and the aortic root (Ao) was compared in 11 children during routine left and right heart catheterization; five had a left‐to‐right shunt and 6 had no hemodynamic abnorm'alities. An additional 4 children with normal hemodynamics were tested with Angiotensin I1,(A‐II). Systemic pressor peaks to PA injections of A‐I were slightly higher than those resulting from Ao injections. A study of the time course of the pressor responses showed that the intervals from the injection to onset of response, and from injection to half peak, were significantly prolonged after injection of A‐I into the aorta, as compared with injections of A‐I1 by the same route. Such a difference was not observed when A‐I and A‐I1 were given into the PA. These results indicate that conversion occurs to a considerable degree in the peripheral circulation, with a delay that is most probably due to the process of activation. When this peripheral conversion is maximal, the systemic pressor response assay is unable to detect pulmonary conversion solely on the basis of the height of pressor peaks, because the responses are equal after PA and Ao injections. PA injections of Angiotensin I and I1 had no effect on pulmonary artery pressure. It is concluded that: (1) pulmonary conversion occurs in all children with and without shunt; (2) peripheral (systemic) conversion occurs to a considerable degree and can account for most of the overall conversion of Angiotensin I, so that the role of the lung in the renin‐angiotensin system seems unlikely.
CLINICAL APPLICATIONS OF ECHOCARDIOGRAPHY IN INFANTS AND CHILDREN I. Investigation of Infants and Caildren without Heart Disease
Authors: LUNDSTRÖM NILS‐RUNE 
Published: January 2008

SummaryEchocardiographic examinations have beenA test of the reproducibility of the echo cardiographic measurements was performed by examining eleven subjects on 2 consecutive days. Four of these 11 infants and children had no signs of heart disease while the other 7 had various forms of congenital heartdisease.The results of the investigation of infants and children without heart disease are presented as nomograms for the prediction of various echocardiographic measurements in relation to the body weight. Such nomograms are presented for the amplitude of movement of the echo from the anterior mitral leaflet, the aortic root diameter, a left atrial dimension, a left ventricular internal dimension and the distance between the anterior chest walland the right side of the interventricular septum. Normal values are also given for a right ventricular dimension and the width of the interventricular septum. performed on 64 infants and children with out heart disease, aged from 3 days to 15 years. Examinations of the following four regions were made: 1, the anterior mitral leaf let; 2, the aortic root and the left atrium; 3, the interventricular septum and the posterior left ventricular wall, and 4, the anteriortricuspid leaflet.
DEVELOPMENT OF PULMONARY FUNCTION IN LATE GESTATION: I. The Functional Residual Capacity of the Lung in Premature Children
Authors: LACOURT GERALD , POLGAR GEORGE 
Published: January 2008

SummaryFunctional residual capacity has been measured in premature and term infants soon after birth and repeatedly thereafter. Good correlation with body weight, length and gestational age have been found. However, the known relationships between the development of lung volume and attributes of body growth in older individuals are only partially valid during late gestation. Some peculiar growth patterns observed in this age could be explained by disproportionate variations in the rate of growth of body length and weight on the one hand and of lung volume on the other.By extrapolation over a very short range it was possible to verify the post mortem estimates of the gestational age at which alveolization occurs.
URINARY BILE ACID CONJUGATES IN EXTRAHEPATIC BILIARY ATRESIA
Authors: NORMAN ARNE , STRANDVIK BIRGITTA , OJAMÄE ÖIE 
Published: January 2008

SummaryThe urinary excretion of bile acid conjugates was studied after the administration of 24‐14C‐labelled chenodeoxycholic acid and cholic acid and their corresponding glycine conjugates labelled with glycine‐l‐14T in 5 infants with extrahepatic biliary atresia. Chenodeoxycholic acid‐24‐14C and chenodeoxycholyl[l‐14C]glycine were mainly excreted in the form of labelled metabolites with the TLC behaviour of monosulphates of tauro‐ and glycochenodeoxycholate and glycochenodeoxycholate, respectively. Most of the cholic acid‐24‐14C and the cholyl[l‐14C]glycine were found to be excreted in the form of glycocholate. All labelled bile acids were excreted in conjugated form.
FAILURE TO THRIVE IN LEBANON: III. Family Income, Expenditure and Possessions
Authors: KANAWATI ABDALLAH A. , DARWISH OLFAT , McLAREN DONALD S. 
Published: January 2008

SummaryInformation was obtained from 40 Thriving and 30 Failing to Thrive families, as previously defined, living in two low socio‐economic areas of Beirut concerning their income, expenditure on various items and their possessions. Both groups were unable to save money but the Failing to Thrive were usually in debt. This group spent proportionally more on food and bought less nutritious food. Families of neither group were fully able to meet their energy or protein requirements. The possessions of the Thriving group were worth, on average, about three times those of the Failing to Thrive group. The contrast between the groups was least for household goods, greater for houses, and greatest for motorcars. The Failing to Thrive group had far fewer decorations, jewelry, and toys and the latter may be an important index of the richness of the child's environment. Refrigerators, radios and heaters were especially prized.
EFFECT OF OROTIC ACID UPON SERUM BILIRUBIN IN NEWBORN INFANTS WITH ERYTHROCYTE G‐6‐PD DEFICIENCY
Authors: CUTILLO S. , MELONI T. , DORE A. 
Published: January 2008

SummaryFifty mature male newborns with erythrocyte G‐6‐PD deficiency were used for a study con cerning the effectiveness of orotic acid in preventing severe hyperbilirubinemia. Twentyfive randomly selected neonates were given orotic acid (100 mg/kg/day) orally in two daily doses from their 1st to their 5th day of life. Twenty‐five newborns were not treatedAccording to these results orotic acid does not seem to be effective in preventing severe hyperbilirubinemia, which frequently occurs in newborn babies' with erythrocyte G‐6‐PD deficiency. and served as controls. Six exchange transfusions were performed in the test group and six in the controls.
COLONIC PERFORATION SECONDARY TO THROMBO‐EMBOLUS FROM AN UMBILICAL ARTERY CATHETER
Authors: ANDERSON A. , HOFER P. Å. , HOLMLUND D. E. W. , WENDEL H. Å. 
Published: January 2008

SummaryA case of colonic perforation in a newborn infant is described. Perforation was probably secondary to an arterial thrombo‐embolus originating from a catheter placed in the aorta via an umbilical artery.
SERUM LEVELS AND RENAL EXCRETION OF DIGOXIN DURING MAINTENANCE THERAPY IN CHILDREN
Authors: HSALO E. , DAHL M. 
Published: January 2008

ABSTRACT. Iisalo, E. and Dahl, M. (Departments of Pharmacology and Paediatrics, University of Turku, Turku, Finland). Serum Levels and Renal Excretion of Digoxin during Maintenance Therapy in Children. Acta Paediatr Scand,63:699, 1974.—To exclude an increased excretion of digoxin as a possible reason for higher dose requirement in children a radioimmunoassay of digoxin in the serum and the 24 hr urine was carried out in 26 children, mainly infants, with congenital heart disease. This procedure was repeated on two consecutive days, in total 64 times during digoxin maintenance therapy.The daily digoxin dose per kilogram of body weight in these small children was twice as high as that used in adults. The steady state level of serum digoxin corresponded approximately to that of adults. A few fairly high digoxin levels were, however, measured in infants without any signs of digitalis toxicity.The daily excretion of digoxin in the urine during maintenance therapy was approximately the same in all age groups and on an average 47 per cent of the daily dose. This percentage is somewhat lower than that found in adults. The low renal excretion of digoxin in one newborn caused high serum digoxin levels.The higher dosage requirement of digoxin per kilogram of body weight in children as compared to adults cannot be explained with differences in the renal excretion of digoxin.
POLYUNSATURATED FATTY ACID LIPIDOSIS INFANTILE FORM OF SO‐CALLED NEURONAL CEROIDLIPOFUSCINOSIS: I. Clinical and Morphological Aspects
Authors: HAGBERG B. , HALTIA M. , SOURANDER P. , SVENNERHOLM L. , EEG‐OLOFSSON O. 
Published: January 2008

ABSTRACT. Hagberg, B., Haltia, M., Sourander, P., Svennerholm, L. and Eeg‐Olofsson, O. (Departments of Paediatrics II, Pathology I and Neurochemistry, University of Gothenburg, Sweden, and Pathology II, University of Helsinki, Finland). Poly‐unsaturated fatty acid lipidosb—an infantile form of so‐called neuronal ceroidlipo‐fuscinosis. Acta Paediatr Scand, 63:753, 1974.—Clinical, histological and ultrastructural findings in three children of Finnish origin and with a severe progressive encephalopathy are reported. The main symptoms were rapid developmental regression from about one year of age, loss of speech, severe visual failure and pronounced secondary microcephaly. A decerebrated state was reached within 1–3 years. Laboratory tests revealed a successively decreasing CSF τ‐fraction. The fatty acid composition of Serum lecithin showed an increased amount of arachidonic acid in the early stage of the disease, a pattern consistent with the biochemical changes in the brain.The morphological characteristics consisted of extreme cerebral and cerebellar atrophy, massive neuronal destruction associated with a pronounced macrophage and astrocytic reaction, and strongly PAS‐reacting and autofluorescent granular deposits in the cytoplasm of the remaining cells. Electron microscopy revealed cytoplasmic osmiophilic deposits in the form of aggregates of globules with a uniform and finely granular ultra‐structure.The condition was considered to be a nosological entity with a uniform clinical picture and characteristic ultrastructural changes in the brain. The evidence produced suggests that the disease described is identical with a progressive heredodegenerative disorder, known to have been diagnosed during recent years in more than 50 Finnish infants and small children and earlier described under the name of infantile type of so‐called neuronal ceroidlipifuscinosis.
27. THE ADOLESCENT WITH A LEARNING PROBLEM. EXPERIENCE AND INSIGHT WITH DELINQUENT BOYS
Authors: FAIGEL HARRIS C. 
Published: January 2008

ABSTRACTTwelve teenaged boys referred by a juvenile court clinic because of delinquent behavior and academic failure were examined by an experienced team of pediatricians, neurologists, psychologists, social workers, nurses, and occupational, physical, recreational, speech and hearing therapists. Two boys came from troubled families with long histories of legal problems, had behavior problems antedating their school difficulties and had no evidence of perceptual handicaps. The remaining ten were the family blacksheep whose academic problems began early and whose behavior decompensated later. They all had perceptual handicaps and both groups of boys had intense psychological reactions to their school difficulties. The learning disabled require a flexible team approach in school together with individual, group and family therapy in order to achieve success.
BRONCHIAL LABILITY IN CYSTIC FIBROSIS
Authors: SKORECKI K. , LEVISON H. , CROZIER D. N. 
Published: January 2008

Abstract. We investigated the airway's response to exercise in cystic fibrosis (CF). Due to the reported high incidence of allergy in CF we tried to demonstrate the well documented post‐exercise bronchospasm of asthma in patients with CF. Subjects ran on a treadmill for 6 minutes at a speed of three miles per hour and a gradient of 15°.For the 12 controls studied, the mean % rise in peak expiratory flow rate (PEFR) during excercise was 4.7% and the mean % fall in PEFR during recovery was 0.7%. For the eight atopic asthmatics studied the mean % rise in PEFR was 12.2% and the mean % fall in PEFR was 33.7%. For the 29 patients with CF, the mean % rise in PEFR was 20.3% and the mean % fall in PEFR was 3.1%. Patients with CF showed no statistically significant post‐exercise constriction compared with the controls, while asthmatic patients showed a post‐exercise % fall in PEFR significantly greater than seen in controls (p<0.001) or in CF patients (p<0.001). No patient with CF showed post‐exercise bronchospasm, nor was there any difference between patients with CF who had positive skin tests to common allergens and those who did not in their pattern of bronchial lability. It is suggested that the general desensitization therapy should not be instituted in patients with CF merely on the basis of positive skin tests, without more stringent criteria such as characteristic seasonal variation in respiratory symptomatology, family history of atopy and positive bronchial inhalation provocation tests.
CONTINUOUS NEGATIVE CHEST‐WALL PRESSURE THERAPY FOR ASSISTING VENTILATION IN OLDER CHILDREN WITH PROGRESSIVE RESPIRATORY INSUFFICIENCY1
Authors: SANYAL SHYAMAL K. , AVERY THOMAS L. , THAPAR MOHINDER K. , HUGHES WALTER T. , HARRIS KENNETH S. 
Published: January 2008

Abstract. Continuous negative chest‐wall pressure (CNP) was used to assist ventilation in 14 children, 6 months to 14 years of age, who had progressive respiratory insufficiency caused by diffuse bilateral alveolar disease. Before the start of CNP therapy, each child had a respiratory rate>50/min, arterial oxygen tension (PaO2)<70 mmHg (FIO2≥50%), and arterial carbon dioxide tension (PaCO2)<45 mmHg. The mean intrapulmonary right‐to‐left shunt was 28.7±3.8%. Within 6 hours after therapy was started, PaO2 increased from 55.4±15.9 to 81.6±17.7 mmHg (p<0.005). This improvement was sustained and within 24 hours permitted a decrease in fractional concentration of inspired oxygen (FIO2) from 51.8±6.2 to 41.0±8.4% (p<0.001) and in respiratory rate from 78.1±23.0 to 56.4±21.3 (p<0.01). There was a concomitant decrease in intrapulmonary right‐to‐left shunt. Four of the 14 patients developed pneumothorax that was successfully decompressed. Ten patients survived.These observations establish CNP therapy as an effective means of improving arterial oxygenation in spontaneously breathing older children. Of added significance, this mode of therapy eliminates the need for endotracheal intubation and prolonged use of muscle relaxants and sedatives. It also minimizes exposure to high FIO2, thereby minimizing the hazards of pulmonary oxygen toxicity.
PATIENTS AT A PAEDIATRIC OUT‐PATIENT CLINIC A Study with Particular Reference to Psychological and Social Background Factors I. The Problem, Material, Methods and the Actual Visit
Authors: JONSELL R. 
Published: January 2008

Abstract. A large proportion of the children who attend paediatric clinics present with indefinite somatic symptoms or complaints which are clearly or possibly of a psychological nature. At the same time it is known that patients with serious psychological problems tend to seek help far too late. This study is an attempt to find out whether the patients who are liable to become problem cases in the long term can be identified by the paediatrician at an early stage and, if so, in what ways these patients differ from others in a paediatric clientele.All the patients who attended the Paediatric Out‐Patient Clinic at Umeå Hospital during the course of one year were assessed by the physicians there. It was judged that psychological factors of importance were involved in 5.2 %, that the importance was doubtful in 8.3 % and that no psychological factors were involved in 86.5%. The proportion of cases involving psychological factors of importance increased with age and amounted to 17 % among the 10 to 15‐year‐olds.
Dieting in Teenage Schoolgirls A Longitudinal Prospective Study
Authors: SCHLEIMER K. 
Published: January 2008

ABSTRACTThe aim of the present study was to demonstrate what far‐reaching consequences dieting may produce in teenage girls and to determine whether there is a direct relation between dieting and the development of anorexia nervosa. The study took the form of a longitudinal prospective investigation of dieting teenage schoolgirls over a 10‐year observation period, particularly as regards physical and mental state of health and general psychosocial development.The questionnaire study underlying the present work—entitled “The feeling of being fat and dieting in a school population”—was published in 1971 by Ingvar Nylander. With the aim of determining how often teenagers feel overweight, how often they diet and how often they develop disorders from dieting, Nylander interviewed 2370 students (1129 boys and 1241 girls). His study was published in Acta Socio‐Medica Scand 1971, 1.In the present work a primary material of 130 girls was selected from the dieting schoolgirls and 111 of these were examined by the author. The methods included a personal interview, examination of physical and mental health, an EEG examination and an intelligence test. Moreover, information was obtained concerning the subjects from their parents and from school health records and hospital case records. A follow‐up study was done 10 years later, in which 97 of the subjects were again examined by the author while 12 subjects merely answered a questionnaire. The methods used were very similar to the foregoing ones. The subjects’dieting behaviour was evaluated on the basis of an anorectic behaviour scale. Further information was obtained from hospital case records.The overall situation of the subjects was evaluated on the basis of case histories and of data from case records concerning heredity, environment, psychosocial adjustment, state of health and findings on examination.Some of the subjects had had a dieting problem for a long time; they had generally dieted for short but repeated periods, but, in most cases, with little effect on their weight. Some patients had developed anorectic behaviour, or even AN, others did not. The former group had shown such behaviour at an early stage—or other signs of mental insufficiency—while the latter group was healthy and during dieting periods developed neither anorectic behaviour nor anorexia nervosa.Thus no direct causal relation between dieting and anorexia nervosa could be demonstrated. If dieting is caused by mental insufficiency, this may lead to the development of anorectic behaviour and/or anorexia nervosa. However, it appears that dieting in a physically and mentally healthy teenage girl can hardly have such effects.A prevalence evaluation of anorexia nervosa in this material showed higher values than those in previous studies.
Atherosclerosis precursors in Finnish children and adolescents. XII. Smoking behaviour and its determinants in 12–18‐year‐old subjects
Authors: BYCKLING T. , SAURI T. 
Published: January 2008

ABSTRACT. The cross‐sectional study of 1980 for atherosclerosis precursors in Finnish children and adolescents aimed at a wide coverage of the relevant relationships of the smoking behaviour to Socioeconomic and psychoSocial factors. The initiation and establishment of the smoking habits were primarily viewed as a function of the youngsters’main Socializing agents: the peer group and the family.A subpopulation comprising 1,790 children and adolescents aged 12, 15 and 18 years of the total sample was included in the study on smoking behaviour. The information on smoking habits was collected in connection with the medical examination in a solitary room where the youngsters could respond undisturbed. Data on the children's families were obtained by means of a general questionnaire filled out by the parents.The prevalence of daily smoking was 1 % in the 12‐year‐old, 10 % in the 15‐year‐old, and 30 % in the 18‐year‐old subjects. The best friend's behaviour was the best predictor of an adolescent's smoking behaviour, although the family had retained its role as an important model as regards the learning of smoking behaviour. Quitting school turned out to be a major event leading to an increased risk of becoming a habitual smoker. No clear associations between Socioeconomic status of the family and daily smoking were found, except that farmers’children had generally lower rates of daily smoking than children from other Socio‐economic groups. First contacts with tobacco formed part of a normal behaviour pattern at a certain age, and this experimentation was unrelated to a later regular habit. Smoking seemed to become part of a more general health behavioural pattern after the age of 15 in a young person's life.
Bone Mineral Content in Preterm Infants at Age 4 to 16
Authors: HELIN INGEMAR , LANDIN LENNART A. , NILSSON BO E. 
Published: January 2008

ABSTRACTUsing photon absorptiometry the forearm bone mineral content (BMC) was determined in 75 children aged 4 to 16, who all had a low birth weight. Forty‐five of them were born preterm AGA (27 boys, 18 girls, mean weight 1580 g; range 920‐2060 g) and 30 preterm SGA (17 boys, 13 girls, mean weight 1510; range 940‐2130 g). The results were compared with a control group of children of the same age, and analyses of covariance with age, height and weight as the covariant factors were performed. The BMC, weight and height did not differ between the children born AGA or SGA. Irrespective of AGA or SGA, the BMC was significantly decreased in boys but the difference was less pronounced and less significant when height and weight were used as covariant factors. Boys who had been born preterm had a less BMC than the control boys for their age but the were also somewhat shorter and lighter than expected with regard to their age.