bluuebunny/crossref_metadata_2025 huggingface dataset:
A TECHNIQUE FOR THE ENZYMATIC DIAGNOSIS OF GLYCOGEN STORAGE DISEASE ON VERY SMALL TISSUE SPECIMENS
Authors: öckerman P. A.
Published: January 2008

The difficulties to obtain an exact diagnosis in cases of glycogen storage disease are mentioned. New diagnostic methods, utilizing blood cells, have not allowed settling a definite diagnosis in all cases. Analyses on liver tissue, consequently, are still needed in many cases. However, the methods up to now used for obtaining a piece of liver tissue (surgery or large‐diameter needle‐biopsy) involve dangers to the patient. Because of this, a less risky biopsy procedure, such as can be performed with a fine‐needle, was thought preferable.

To be able to utilize very small amounts of liver tissue, as obtainable with a fine‐needle, for the diagnosis of glycogen storage disease, methods were worked out, allowing the analysis of glucose‐6‐phosphatase, phosphorylase, amylo‐1,6‐glucosidase, α‐glucosidase, glycogen and protein on 0.5‐2 mg fragments of liver tissue. The metnods involved incubation of very small volumes, assay of phosphorus by a newly described, highly sensitive method and of glucose by the hexokinase‐glucose‐6‐phosphate dehydrogenase method with fluorimetric measurement of the NADPH. Protein and glycogen could be assayed in a more conventional way. The stability of the enzyme activities and the precision of the methods is discussed.

Results on liver biopsy specimens from seven controls and on biopsy and autopsy specimens from liver in eight cases of four different types of glycogen storage disease are given and compared to the results on these specimens with earlier described methods.
FATAL GRANULOMATOUS DISEASE
Authors: Andersen Vagn , Koch Christian , Vejlsgaard René , Wilken‐Jensen Knud
Published: January 2008

Two brothers with Fatal granulomatous disease of childhood (FGD) are presented. Both patients exhibited pronounced food allergy. Otherwise, the course of the disease was typical of FGD. From infancy recurrent infections, notably suppurative lymphadenitis, and a fatal outcome before puberty.

Microscopic examination of affected organs revealed granulomas with tuberculoid structure; the macrophages contained a characteristic yellow‐brown lipid pigment.

A defective function of the neutrophil granulocytes has been demonstrated in FGD. The phagocytic capacity is intact but phagocytized bacteria are not killed since phagocytosis does not induce liberation of catabolic enzymes from the neutrophil granules. In one of our patients, defective degranulation and vacuolization of the cytoplasm of neutrophils was demonstrated in vitro following phagocytosis of staphylococci. However, exposure to sodium fluoride or kleb‐siella toxin did induce degranulation and vacuolization of the defective granulocytes.
The Development of Children in a Swedish Urban Community. A Prospective Longitudinal Study
Authors: KARLBERG PETTER , KLACKENBERG GUNNAR , KLACKENBERG‐LARSSON INGRID , LICHTENSTEIN HENRIK , STENSSON JAN , SVENNBERG INGA
Published: January 2008

The first Swedish prospective, longitudinal investigation into the development of children in a Swedish urban community‐is presented. Its aims, design and recruiting procedure are described, and the representativeness of the material obtained is discussed. The investigation forms a link in an internationally coordinated study.
The Development of Children in a Swedish Urban Community. A Prospective Longitudinal Study
Authors: KLACKENBERG G. , KLACKENBERG‐LARSSON I.
Published: January 2008

Data on breast‐feeding, weaning, and the practice of giving night‐meals, collected in the prospective longitudinal growth‐study presented earlier, have been analysed for their association with various social and family factors and personality variables in the mothers. The following statistically significant relationships were found: 1. Boys are breast‐fed longer than girls. 2. Mothers in the higher age‐group (26 and over) continue breast‐feeding longer than do younger mothers (under 26). 3. Mothers of the highest social class (Graffar I+ 11) continue breast‐feeding longer than do mothers in the intermediate (Graffar III) and the lowest (Graffar IV+ V) social classes. 4. Mothers with higher education continue breast‐feeding longer than do mothers with only elementary school education. 5. In the lowest social class, mothers who live in old‐fashioned houses stop breast‐feeding earlier than do mothers living in modern houses. 6. Some personality traits of the mother are more often than others associated with long‐continued breastfeeding.

No statistical relationships were demonstrated between the duration of breast feeding and overcrowding or the child's successional number in the family.

The reasons for weaning were analysed. “No or insufficient milk” was the most common reason given by mothers who weaned their babies before 6 months. Gainful employment was of very little importance as a reason for weaning.

The mothers' attitudes towards breastfeeding in the child's first and third months of life were generally positive. The duration of breast‐feeding cannot be predicted from the attitude declared by the mothers.

The frequency of night‐feeding on the return home from the maternity hospital was 85% and at 3 months 25%. The boys received night‐meals for a significantly longer period than did the girls. The mother's age, the child's successional number in the family, social class, the duration of breast‐feeding and the mother's attitude to breast‐feeding showed no association with the continuation of night‐feeding.
LATE INFANTILE PROGRESSIVE ENCEPHALOPATHY WITH DISTURBED POLYUNSATURATED FAT METABOLISM
Authors: Hagberg Bengt , Sourander Patrick , Svennerholm Lars
Published: January 2008

A case report is presented of a 6‐year‐old girl with a severe progressive encephalopathy of late infantile onset and with changes indicating marked disturbances in the metabolism of linolenic acid.
NONSPHEROCYTIC HAEMOLYTIC ANAEMIA AND SEVERE JAUNDICE IN A NEWBORN WITH PARTIAL PYRUVATE KINASE DEFICIENCY
Authors: Volpato S. , Vigi V. , Cattarozzi G.
Published: January 2008

We present a case of familial non spherocytic haemolytic anaemia which began with a severe neonatal jaundice (I. B. 28 mg/100 ml). The propositus presented a partial PK deficiency, which was also present in the father but not in the mother.

We were able to exclude other conditions which can be responsible for haemolytic anaemia, and neonatal jaundice.

The hypothesis that also a partial PK deficiency can, in particular conditions, be responsible for a severe haemolytic process, is suggested.
ENDOGENOUS FORMATION OF CARBON MONOXIDE IN NEWBORN INFANTS:IV. On the Relation between the Blood Carboxyhaemoglobin Concentration and the Pulmonary Elimination of Carbon Monoxide
Authors: Fällström S. P.
Published: January 2008

A procedure for the determination of the pulmonary elimination of carbon monoxide in newborn infants has been described. Quantitative collection of the expired air was accomplished with the aid of an open circuit breathing system. The low CO concentrations in room air and the collected air were measured in a Hopcalite CO meter.

Simultaneous determinations of the blood carboxyhaemoglobin (COHb) concentration and the pulmonary elimination of carbon monoxide were performed on 15 full‐term newborns without blood group incompatibility and known haemolytic disease, on 10 infants with jaundice and ABO incompatibility and on 18 infants with Rh haemolytic disease. In all infants the birth weight exceeded 2500 g and none of the infants displayed any clinical signs of respiratory disease.

In all groups a significant positive correlation was found between the pulmonary CO elimination and the COHb concentration. The results indicate that in full term newborn infants, the pulmonary CO elimination is efficient from the first day of life even in the presence of haemolytic disease and anaemia. The increased COHb concentrations found in these groups of newborns can thus not be explained by CO retention but should reflect an increased haemoglobin catabolism.
Energy requirements of school children with cerebral palsy as determined from indirect calorimetry
Authors: BERG KRISTINA , OLSSON TORSTEN
Published: January 2008

Four subjects with severe cerebral palsy were studied with heart rate telemetry and intermittent sampling of expired air gas analysis, while they permed their habitual activities.

each subject, a significant relation between oxygen uptake and heart rate was found. On the basis of the heart rate recordings and the time spent on various daily activities, a mean heart rate was found each subject. The corresponding mean daily oxygen uptake was read from this line.

The four individual lines of the correlation between oxygen uptake and heart rate differed significantly from each other with respect to the slope. Three of 4 lines crossed over at a heart rate of about 75 beats per minute and oxygen uptake of 11.5 ml per minute and kg body cell mass. This was regarded as a “basal” point, representing the basal cellular need in a thermoneutral environment.

This “basal” point was used för calculation of energy expenditure in a less extensive study of 6 subjects. In these children, only one or two air analyses at a known heart rate were perförmed. This determination, or the average of two determinations, was connected with the “basal” point. The individual line was then used to determine the mean daily oxygen uptake.

The mean daily energy expenditure found the ten subjects agreed well with the energy intake from dietary recordings, on the one hand, and body fat amounts, on the other.
Transthoracic Impedance: II. Experimental Evaluation of the Method in the Cat
Authors: KJELLMER INGEMAR , OLSSON TORSTEN , VICTORIN LARS
Published: January 2008

The assumption that measurement of the transthoracic impedance can be used to assess variations of the airlblood volumes in the lungs has been tested.

In cats transthoracic impedance, lung blood volume and lung air volume were recorded separately and the blood and air volumes varied independently. The results show that the trans‐thoracic impedance sensitively detects changes in the ratio between lung air and lung blood volumes. It is further indicated that impedance measurements can be used for evaluation of the magnitude of changes in this ratio.
INTENSIVE CARE OF SMALL PREMATURE INFANTS: I. Clinical findings and results of treatment
Authors: VAPAAVUORI EERO K. , RÄIHÄ NIELS C. R.
Published: January 2008

Clinical findings and results of treatment in a series of 49 premature infants with birth weights between 850 and 1250 g are presented.

The “intensive care” included nursing in incubators with high environmental humidity maintaining skin temperature at 36.5°C, umbilical catheterisation (both arterial and venous), intravenous glucose infusions, correction of metabolic acidosis by rapid sodium bicarbonate infusions, oxygen therapy and finally endotracheal intubation and mechanical ventilation.

The series was divided into 3 categories according to the severity of respiratory symptoms. There was only one survivor out of 12 cases in the category of severe RD, whereas 14 of 21 with moderate RD and 9 of 16 with slight or no RD survived. The overall survival rate was 45%. The correlation between clinical findings, arterial blood gases and pH, chest X‐rays, response to treatment and outcome in each of the 3 categories is discussed. The preliminary data from our follow‐up studies indicate that there are only a few cases showing signs of permanent brain injury at the age of two years.
THE USE OF AZATHIOPRINE IN REFRACTORY IDIOPATHIC THROMBOCYTOPENIC PURPURA IN CHILDREN
Authors: HILGARTNER MARGARET W. , LANZKOWSKY PHILIP , SMITH CARL H.
Published: January 2008

Five children with refractory ITP were treated with azathioprine for 8‐27 months. An excellent response was obtained in 3 of the 5 patients, a good response in a fourth. In two presplenectomy cases the result was obtained using azathioprine alone and two post‐splenectomy cases required low dose steroids in addition to azathioprine. One pre‐splenectomy patient failed to respond to the drug alone or in combination with steroids but responded well to splenectomy. A review of the cases in the literature and the experience presented here suggests that azathioprine has a definite role in the therapy of refractory idiopathic thrombocytopenic purpura in childhood.
THE DECREASE IN THE SERUM BILIRUBIN LEVEL IN PREMATURE INFANTS BY OROTIC ACID
Authors: KINTZEL H.‐W. , HINKEL G. K. , SCHWARZE R.
Published: January 2008

The influence of higher doses of orotic acid on the serum bilirubin level of premature infants was investigated following studies with a lower dose. 102 premature infants were treated with a daily dose of 300 mg of orotic acid from the 1st‐6th day after birth. An equal number of children served as a control group. The serum level for the indirect bilirubin that was analysed from the 3rd‐6th day of life could be statistical significantly decreased by the administration of orotic acid. Blood exchange transfusions were necessary only four times with the administration of orotic acid, whereas blood exchange transfusions were required in 30 premature infants of the control group. The question of eventual side effects and the supposed mode of action of the orotic acid are discussed.
ARTERIAL ANOMALIES CAUSING COMPRESSION OF THE TRACHEA AND/OR THE OESOPHAGUS
Authors: EKLÖF O. , EKSTRÖM G. , ERIKSSON B. O. , MICHAELSSON M. , STEPHENSEN O. , SÖDERLUND S. , THORÉN C. , WALLGREN G.
Published: January 2008

The experiences of diagnosis and treatment of arterial anomalies causing compression of the trachea and/or the oesophagus in 30 symptomatic cases are reported. The main diagnostic aid is the radiological examination of the chest in the anterior‐posterior and lateral projection including adequate contrast filling of the oesophagus. This investigation, using water‐soluble contrast media, should be performed in infants with stridor and other respiratory difficulties and in dysphagia. With improved surgical technique and postoperative care the operative risk of today is low and surgery should be seriously considered in symptomatic cases. Operation may be necessary as an emergency treatment.
THE SIGNIFICANCE OF COPROANTIBODIES TO COW'S MILK PROTEINS
Authors: KLETTER B. , FREIER S. , VIES A. MICHAEL DA , GERY I.
Published: January 2008

An attempt has been made to delineate further the significance of milk coproantibodies. The presence of precipitating and of high levels of hemagglutinating antibodies in feces was found only in patients with milk sensitivity or celiac disease. These cases showed also high levels of serum antibodies to milk. In mongolism and dysautonomia no abnormal titers of coproantibodies were found, in spite of strong reactions in the sera.

Antibodies to milk were found by radio‐im‐munoelectrophoresis to consist mainly of immunoglobulin A in feces and immunoglobulin G in serum. Serum milk antibodies of the IgA type were found only in patients with milk sensitivity or celiac disease.
EPIDIDYMITIS IN CHILDREN
Authors: HAAHR J. , SPARREVOHN S.
Published: January 2008

The case histories of six children with epididymitis are submitted. In 2 cases the epididymitis formed part of Schonlein‐Henoch's purpura and microscopy of biopsies from the epididymis showed changes characteristic of the latter disease. In 1 case the epididymitis was of suppurative nature and in 1 case it represented a complication following orchiopexy. In the remaining 2 cases, the aetiology of the disease remained obscure.

Infection of the urinary tract was not observed in any of the cases and the urinary tract was normal in all except one in whom double anal‐age of one of the kidneys was demonstrable. Three of the patients, including the two with Schonlein‐Henoch's purpura, had thrombocytosis. The aetiology and the differential diagnoses are briefly discussed. It is emphasized that the suspicion primarily should be focussed on torsion of the testis or incarcerated hernia whenever a boy is found to have a tender and swollen scrotum; it is emphasized also that the two latter diagnoses as a rule can be excluded by operation.
A CONTROLLED TRIAL ON THERAPY FOR NEWBORNS WEIGHING 750‐1 250 g
Authors: MENDICINI M. , SCALAMANDRÉ A. , SAVIGNONI P. G. , PICECE‐BUCCI S. , ESUPERANZI R. , BUCCI G.
Published: January 2008

A controlled therapeutic trial was performed in newborns with birthweight of 750‐1 250 g. 40 infants (the “treatment” group) received an i.v. infusion with glucose and NaHCO3 from the 1st to the 4th‐7th day of life, and increasing amounts of human milk from the 2nd day of life. They also received, during the newborn period, 3 antibiotics (sodium penicillin G 500 000 U + methicillin 100 mg + colistin sulf. 8 mg per kg/day), a gamma globulin preparation (0.5 ml/day for 3 days) and fresh human plasma (3 ml every 2nd day in the first 5 days of birth). 40 infants (the “control” group) received only oral feeding with 10% glucose and human milk, starting in the 2nd day of life, and i.m. kanamycin (20 mg/kg/day for 5 days).

No difference between treated and control babies was observed with respect to the following findings: neonatal mortality rate and survival curve on the whole series; neonatal mortality rate in babies with birthweight above or below 1.04 kg, with gestational age below 28 weeks, with above or below median respiratory rate or arterial pH on admission, with or without marked abnormality on chest film, and in males or females (but, in subjects with gestational age above 27 weeks, a significantly lower mortality rate was found in controls); postmortem findings; incidence of apnoeic spells, tremors and cloni, and pattern of muscular tonus. Bradycardia associated with 2: 1 atrioventricular block was observed in 6 controls in the 2nd day of birth. No adverse effects presumably due to the therapeutic procedures so contrasted were demonstrated.

It was concluded that in the present series of newborns with very low weight: (a) the massive anti‐infectious therapy was not superior to the administration of a single broadspectrum antibiotic; (b) the administration of fresh human plasma was not effective in preventing hemorrhage (but further evaluation is needed); (c) the routine i.v. infusion with glucose and NaHCO3 did not improve neonatal survival as compared with oral feeding.

However, previous studies, as well as observations on the present series reported elsewhere (11), have shown that several biochemical abnormalities commonly seen in orally fed newborns with very low weight can be prevented or corrected, at least in part, by the early infusion with glucose and NaHCO3. Since some of these abnormalities are potentially dangerous to the central nervous system, the final evaluation of this therapy must await long‐term follow‐up studies in survivors. It is therefore suggested that, before such studies are available, the early i.v. infusion with glucose and NaHCO3 should be given routinely to newborns with very low weight.
DERMATOGLYPHICS AND THE SIMIAN CREASE IN INFANTS OF LOW BIRTH WEIGHT
Authors: DAR H. , CARNEY F. E. , WINTER S. T.
Published: January 2008

Dermatoglyphic features and simian crease incidence were studied in 73 neonates with a birth‐weight under 2 000 g. There was a higher frequency of simian crease, typical and transitional types, as compared with two control groups of 100 normal neonates and 929 school‐children. There was no significant difference in the incidence of dermatoglyphic features between the low birth weight and the control neonates.
MALNUTRITION AND SIZE OF THE CEREBRAL VENTRICLES1
Authors: VAHLQUIST B. , ENGSNER G. , SJÖGREN IRÉNE
Published: January 2008

Examination of the size of the cerebral ventricles by means of echoencephalography in Ethiopian infants and young children with severe malnutrition showed a moderate but significant increase in children with kwashiorkor, examined up to 3–4 weeks after admission, whereas children with marasmus showed no deviation from the normal.

Further studies will show whether or not this abnormality in the kwashiorkor children is a transient phenomenon. The observation gives one more reason for caution in using head circumference as a measure of brain size.
CONCENTRATION OF PURINE NUCLEOTIDES IN ERYTHROCYTES OF PATIENTS WITH THE LESCH‐NYHAN SYNDROME BEFORE AND DURING ORAL ADMINISTRATION OF ADENINE
Authors: LOMMEN E. J. P. , VOGELS G. D. , ZEE S. P. M. VAN DER , TRIJBELS J. M. F. , SCHRETLEN E. D. A. M.
Published: January 2008

This paper presents the results of an investigation on the concentration of purine nucleotides in erythrocytes of two children with the Lesch‐Nyhan syndrome before and during oral adenine administration. The nucleotides AMP, ADP, ATP and GTP could be calculated. Before administration the patients showed a decreased concentration of the three adenine nucleotides expressed as pinoles per litre of free cell water. The concentration of GTP was found to be normal. After oral adenine administration, adenine nucleotides were found to be normal and GTP showed a slight increase. Some suppositions about the mechanism of increased purine synthesis de novo in patients with the Lesch‐Nyhan syndrome have been discussed.
AN EPIDEMIOLOGICAL STUDY OF CHILD HEALTH AND NUTRITION IN A NORTHERN SWEDISH COUNTY
Authors: SAMUELSON GÖSTA
Published: January 2008

The health and nutritional status of 1 401 children aged 4, 8 and 13 years in one urban area and two rural areas of a northern Swedish county were studied.

In none of the age groups was there any undernutrition. Anthropometrical measurements reflected the good nutritional status, with height and weight generally conforming to current values of a Swedish urban population. The girls had significantly thicker skinfolds than the boys. The children in the city of Umea had thicker skinfolds than the children in the mountain foreland and were also significantly taller.

There was a rather high frequency of subclinical upper respiratory infections among the children, especially among the 4‐year‐olds.
THE INCIDENCE OF PKU IN FINLAND
Authors: VISAKORPI J. K. , PALO J. , RENKONEN O.‐V.
Published: January 2008

Guthrie's bacterial inhibition test was applied to 71 135 newborn infants from different parts of Finland. No cases of PKU were diagnosed. In addition, 3 685 mentally retarded patients, mostly idiots and imbeciles, have been screened in Finland with the same method. With two cases of PKU among them, a prevalence of 0.54 per 1 000 is obtained. The incidence of the disease among the newborn is estimated to be less than one per 100 000.
A GIRL WITH RECURRENT INFECTIONS, LOW IgM AND AN ABNORMAL CHROMOSOME NUMBER 1
Authors: ØSTERGAARD FOUL AABEL
Published: January 2008

A girl, now 3 years of age, is described, who since the age of 3 months had many, often severe, infections mainly caused by microorganisms with a polysaccharide structure. In addition she had very low IgM and no iso‐agglutinins in her serum, and furthermore we were able to show an abnormal chromosome number 1 in more than 80% of the cells investigated from peripheral blood. The possibility that antibodies to polysaccharide antigens are directed from chromosome number 1 is referred to in this paper.
EXCRETION OF BILE ACIDS IN EXTRAHEPATIC BILIARY ATRESIA AND INTRAHEPATIC CHOLESTASIS OF INFANCY
Authors: NORMAN ARNE , STRANDVIK BIRGITTA
Published: January 2008

This series included 24 infants, 16 boys and 8 girls, who were admitted to hospital with the diagnosis of obstructive jaundice. Five of the infants were subsequently found to have extra‐hepatic biliary atresia (BA) and the other 19 infants intrahepatic cholestasis of infancy (IHC).

The infants were investigated given special attention to: the quantitative urinary excretion of cholic and chenodeoxycholic acids, the isotope excretion after intramuscular injection of cholic acid‐24–14C, the nature of labelled urinary bile acids, the half‐life and the pool size of cholic acid.

At the first examination of the infants after admission the urinary excretion of cholic and chenodeoxycholic acids varied greatly between the patients. However, on comparing the values obtained in the two groups, it was found that there was virtually no difference between the mean daily values of cholic and chenodeoxycholic acids in urine, and the ratio cholic to chenodeoxycholic acid between the BA group and the IHC group. After the injection of isotopic cholic acid most of the isotope was recovered in the urine in all cases. In the infants with BA the faecal excretion of the isotope was low, being less than 3 per cent of the injected isotope. Out of the 19 infants with IHC the recovery of the injected isotope in faeces was also less than 3% in 11 infants. In 8 infants with IHC the faecal isotope excretion was significantly high to exclude extrahepatic biliary atresia.

The first 24 hour urine specimen contained small amounts of unconjugated labelled cholic acid in all cases whereas in no case did the patients excrete unconjugated labelled cholic acid 48 hours after the injection of the isotope. No transformation of cholic acid was observed. There was no difference between the BA group and IHC group with regard to the percentage labelled glycine conjugates of total excreted urinary conjugates. Neither was there any difference between the two groups with regard to half‐life and pool size of cholic acid.

There was no difference with respect to the bile acid metabolism between infants with congenital CMV infection, decreased serum concentrations of alfal‐antitrypsin and the other patients.
STUDIES IN CYSTIC FIBROSIS1: Urinary Excretion of Hexosamine, Sialic Acid and Fucose
Authors: KOLLBERG H. , LUNDBLAD A. , EKBOHM G.
Published: January 2008

Normal values for the total urinary excretion of hexosamine, sialic acid and fucose and their ratios to creatinine in childhood were determined. The excretion of these substances in 25 CF children was slightly higher at all ages than in controls. The increase was more pronounced in higher ages and in more severely affected patients.

No difference in the ratio of fucose to sialic acid was found between controls and CF patients, either in the unfractionated urine or in the ultrafiltrable or non‐ultrafiltrable fractions of urine.

Zone electrophoretic fractionation of the non‐ultrafiltrable material disclosed a proportionately higher excretion of both carbohydrate‐ and protein‐containing material in CF patients as compared with controls.

Two CF patients exhibited normal gel‐chromatographic distributions of fucose‐con‐taining low molecular weight carbohydrate material.
THE EFFECT OF LYSINE FORTIFICATION OF IRANIAN BREAD ON THE NUTRITIONAL STATUS OF SCHOOL CHILDREN
Authors: HEDAYAT H. , SHAHBAZI H. , PAYAN R. , AZAR M. , BAVENDI M. , DONOSO G.
Published: January 2008

The effect of enrichment of bread with lysine was studied on a group of Iranian village children receiving free school lunch over a period of 210 days. Two groups received typical dishes, which were purposely modified to make them limited by lysine; one with enriched and the other with non‐enriched bread. The third group did not receive lunch and served as control to assess the changes in the nutritional status of the former groups. The food intake at the homes was not controlled.

Markedly greater increases in weight and mid‐arm circumference were observed in groups which received the school lunch (with or without lysine) than in the control group. Lysine enrichment showed no significant effect in improving the nutritional status except that children who received the amino acid supplementation exhibited significantly higher increments in mid‐arm circumferences. An increment in height and weight was observed in children of non‐lysine group over those who received lysine which was significant in respect to height. The latter group also showed a small but significant decrease in serum albumin.
COMBINED SUPRAPUBIC ASPIRATION AND CLEAN‐VOIDED URINE EXAMINATION IN INFANTS AND CHILDREN
Authors: ARONSON A. S. , GUSTAFSON B. , SVENNINGSEN N. W.
Published: January 2008

In 120 infants and children a comparison was made between suprapubic aspiration urine and concomitantly obtained clean‐voided urine.

The following conclusions could be made from this study:

(1) By performing suprapubic aspiration of urine, true bacteriuria could be diagnosed. 31 false positive and 8 false negative clean‐voided urine samples were disclosed.

(2) Furthermore, by the combined procedure of urinary collection, it was possible to elucidate the diagnostic value of accurately performed leucocyte counting in uncentrifuged urine. In infants leucocyte counts above 250/ mm3 in clean‐voided urine and above 10/mm3 in suprapubic aspiration urine always accompanied a urinary infection, whereas the degree of leucocyturia in children was inconclusive.
EVALUATION OF THE EFFECTIVENESS OF TREATMENT ON ADULT HEIGHT PROGNOSIS IN DISORDERS WITH ADVANCED AND RETARDED BONE AGE Height and Height Velocity related to Chronological or to Bone Age
Authors: BOSSI EMILIO , JOSS ETIENNE E. , ZURBRÜGG ROLF P.
Published: January 2008

Different growth parameters and their graphical presentation in disorders with advanced and retarded bone age are discussed. Their usefulness for the evaluation of the effect of treatment on growth and maturation is compared. In precocious puberty, more information is obtained when height is plotted against bone age instead of chronological age. It is more reasonable from a physiological standpoint to plot height velocity against bone age rather than chronological age. For the evaluation of the efficiency of treatment on adult height prognosis in precocious puberty, two possibilities are proposed: height velocity expressed as height increment per bone age year, or the quotient height age increment/bone age increment. However, where skeletal maturation is severely retarded, as in hypopituitary growth retardation, these growth parameters should be related in the conventional way to chronological age, rather than to bone age.
CONTINUOUS POSITIVE AIRWAY PRESSURE WITH A FACE CHAMBER IN EARLY TREATMENT OF IDIOPATHIC RESPIRATORY DISTRESS SYNDROME
Authors: AHLSTRÖM HANS , JONSON BJÖRN , SVENNINGSEN NILS W.
Published: January 2008

CPAP treatment started early with a new face chamber was found to be convenient and, without risks. Treatment of IRDS applied early with the presented technique appears to radically improve the prognosis.
HAEMORRHAGIC CYSTITIS COMPLICATING CYCLOPHOSPHAMIDE TREATMENT IN CHILDREN
Authors: HELIN INGEMAR , OKMIAN LUDVIG
Published: January 2008

Haemorrhagic cystitis complicating cyclo‐phosphamide treatment is a serious complication. The bladder irritation is considered to be a consequence of direct contact between bladder wall and urine containing metabolites of cyclo‐phosphamide. The early symptoms are vesical tenesmus, pollakisuria and haematuria. Endo‐scopy is the essential way of diagnosing the cyclophosphamide cystitis. High daily fluid regimen and frequent voiding must be recommended as the prophylaxis of choice. Moreover the drug must be administered as a single 24‐hour‐dose in the morning. Surgical techniques differ according to the severity of the bleeding. Administration of an anti‐fibrinolytic agent proved ineffective.
GASTRIC LIPOLYSIS OF HUMAN MILK LIPIDS IN INFANTS WITH PYLORIC STENOSIS
Authors: OLIVECRONA T. , BILLSTRÖM Å. , FREDRIKZON B. , JOHNSON O. , SAMUELSON G.
Published: January 2008

In gastric contents a potent lipase is present which hydrolyses the milk lipids to mainly diglycerides and free fatty acids. This hydrolysis hasearlier beendemonstratedinrats and now in human infants. The gastric lipo‐lysis may facilitate the further digestian of the milk lipids by transformation of the fat droplets to a substrate that is more readily attacked by the pancreatic lipases.
THE EFFECT OF MATERNAL AGE, PARITY AND SOCIAL CLASS ON THE INCIDENCE OF SMALL‐FOR‐DATES NEWBORNS
Authors: PAPAEVANGELOU G. , PAPADATOS C. , ALEXIOU D.
Published: January 2008

The incidence of S.F.D. newborns was studied in relation to maternal age, parity, and social class in a sample of 4 369 consecutive births at the “Alexandra” Maternity Hospital of Athens. The incidence of S.F.D. babies in the lower classes (IV and V) is substantially higher when compared with mothers belonging to social groups I, II or III. Furthermore, the birth of S.F.D. babies is encountered more frequently in very young as well as in women above the age of 30 years. Finally, the birth of a S.F.D. baby is more frequent in primiparous than multiparous women.
SUGAR ABSORPTION BY FLAT JEJUNAL MUCOSA
Authors: DESJEUX J. F. , SASSIER P. , TICHET J. , SARRUT S. , LESTRADET H.
Published: January 2008

To obtain further information on the role of jejunal mucosa in malabsorption, characteristic activities of jejunal epithelial cells (disaccharid‐ase activities and maltose and glucose absorption) were correlated with jejunal histology of 20 biopsy specimens with no villi (flat mucosa, F.M. (group I)) and 20 biopsy specimens with higher than 350 pm villi (control mucosa, C.M. (group 11). Except for lactase activity (F.M. = 13% of C.M.), the enzymatic and absorptive activities measured in F.M. specimens were uniformly about 30% of the corresponding activity in the C.M. tissue. An attempt was made to predict maltase and sucrase activities in F.M. specimens by extrapolating plots of C.M. activities vs. villous height. Measured enzyme activities in F.M. tissue, however, were significantly higher than the zero activity predicted by such plots. In contrast, the regression lines slopes of maltase or sucrase activities vs. total height (crypts and villi) measured in C.M. specimens were not different from the slopes obtained when data from C.M. and F.M. specimens were plotted together. These results suggest (i) the presence of absorptive cells in flat jejunal mucosa, which might be localised in so‐called crypts; (ii) that a reduction in the number of epithelial cells is not the single mechanism in the origin of flat mucosa; (iii) lactase activity behaves differently than other studied epithelial activities.
COW'S MILK ALLERGY: PREVALENCE AND MANIFESTATIONS IN AN UNSELECTED SERIES OF NEWBORNS
Authors: Gerrard J. W. , MacKenzie J. W. A. , Goluboff N. , Carson J. Z. , Maningas C. S.
Published: January 2008

1. A consecutive series of 787 babies born at the University Hospital were followed for 12–36 months in order to determine the prevalence of cow's milk allergy (CMA).

2. Fifty‐nine babies (7.5%) were shown by repeated challenge to have evidence of CMA.

3. The main manifestations of CMA were eczema in 27, recurrent diarrhea in 24, recurrent rhinorrhea in 18, repeated vomiting in 12, recurrent bronchitis in 10, and asthma in 7.

4. Thirty‐four babies had two or more allergic problems, e.g., had recurrent rhinorrhea and eczema, or recurrent bronchitis and recur‐rent diarrhea, 25 babies had only one.

5. Babies allergic to cow's milk were often allergic to other foods, 20% for example were allergic to soya.

6. Twenty‐five babies developed their first symptoms within 7 days of being given their cow's milk formula.

7. Seventeen of the babies lost their sensitivity to cow's milk, 10 of these babies were less than 12 months old. Thirty‐eight babies over 12 months of age when last reviewed were still allergic to cow's milk.

8. Babies with CMA, as well as those with other allergic problems, attended their doctors on account of illness more frequently than did the non‐allergic babies.

9. Allergic disorders were commonest in the parents and siblings of babies with CMA, and least common in the parents and siblings of the non‐allergic children.
THE RE‐EMERGENCE OF EARLY CONGENITAL SYPHILIS
Authors: TAN K. L.
Published: January 2008

Ten infants with early congenital syphilis are presented. Hepatosplenomegaly is a marked feature in all the infants. The majority of them were of low birth weight. Syphilitic pemphigus was present in 5, and hydrops fe talis in 4. Five infants survived, including one witn hydrops fetalis. Early diagnosis and intensive therapy are necessary for survival. A high index of swpicion is required for early and more frequent diagnosis of this condition.
SEA BLUE HISTIOCYTE SYNDROME
Authors: ÖZSOYLU SLINASI , KOÇAK NURTEN , BERKEL A. IZZET
Published: January 2008

Three children with congenital form of sea blue histiocyte syndrome are reported. Two of them had liver involvement and the third one was mentally retarded. In all these cases, marked growth retardation was documented and the two had frequent epistaxis. The diagnosis was made by the demonstration of sea blue histiocytes in the bone marrow. These cells were also shown in the liver and spleen needle biopsy specimens, whichever was obtained.
PERIPHERAL BLOOD B LYMPHOCYTES IN INFANCY AND CHILDHOOD
Authors: UGAZIO A. G. , MARCIONI A. F. , ASTALDI A. Jr. , BURGIO G. R.
Published: January 2008

Abstract. Ugazio, A. G., Marcioni, A. F., Astaldi, A. Jr and Burgio, G. R. (Paediatric Clinic, University Medical School of Pavia, Italy). Peripheral blood B lymphocytes in infancy and childhood. Acta Paediat Scand, 63: 205, 1974.–In order to investigate B lymphocytes in the paediatric age, 63 normal children were examined for the presence of immunoglobulin‐bearing lymphocytes in their peripheral blood. Lymphocytes were stained with a fluorescein‐conjugated anti‐immunoglobulin anti‐serum and the percentage of positive cells counted under ultraviolet light. The percentage of lymphocytes staining with fluorescein‐conjugated anti‐immunoglobulin antiserum has been shown to remain constant from the first days of life to adult age. It was concluded that an homeostatic mechanism maintains B cells a constant proportion of blood lymphocytes irrespective of the well known age‐dependent changes in the numbers of blood lymphocytes per volume whole blood.
BRAIN STEM ENCEPHALITIS1
Authors: YALAZ KALBIYE , TINAZTEPE KERIMAN
Published: January 2008

Abstract. Yalaz, K. and Tinaztepe, K. (Departments of Paediatric Neurology and Paediatric Pathology, Hacettepe Children's Hospital, Ankara, Turkey). Brain stem encephalitis. Acta Paediat Scand 63: 235, 1974.–Since the clinical picture in brain stem encephalitis resembles that of various other neurological diseases and conditions, especially brain stem gliomas, and since the prognosis and treatment of these disorders differ greatly, accurate diagnosis is very important. Eight cases of brain stem encephalitis are presented; seven with signs indicating good prognosis, and the post‐mortem findings of one fatal case. Analyses of the clinical and laboratory findings of our cases allowed the following conclusions:

1. There is a prodromal period of fever and general malaise before neurological symptoms appear.

2. Dysphagia, peripheral facial paralysis and pyramidal tract signs are usually present.

3. Although the clinical condition is grave at the onset of illness, the prognosis is good; in 7–8 weeks complete recovery may occur but facial nerve paralysis may persist.

4. Lymphocytes may be present in the cerebro‐spinal fluid along with slight increase in protein.

5. Distortion of the fourth ventricle which is of extreme importance in the diagnosis of brain stem gliomas is also usually encountered in brain stem encephalitis.
REFERENCE VALUES FOR CORD BLOOD LIPID AND LIPOPROTEIN CONCENTRATIONS
Authors: DYERBERG J. , HJØRNE N. , NYMAND G. , OLSEN J. S.
Published: January 2008

Abstract. Dyerberg, J., Hjørne, N., Nymand, G. and Olsen, J. S. Department of Clinical Chemistry A, Rigshospitalet, Copenhagen, Department of Clinical Chemistry and Department of Gynaecology and Obstetrics, Aalborg Sygehus, Aalborg, Denmark. Reference value for cord blood lipid and lipoprotein concentrations. Acta Paediatr Scand, 63: 431, 1974.–Cord blood lipid and lipoprotein concentrations in 120 newborns, 60 boys and 60 girls, have been determined in order to evaluate reference values for these components. The lipoprotein analyses were performed with an electrophoretic technique, which enables correction for the difference in lipid composition of the lipoproteins in the quantitation procedure. The lipid analyses revealed a small, but statistically significant higher phospholipid concentration in girls than in boys (p<0.05). A similar difference in cholesterol concentration was not statistically significant (p >0.05). No sex difference was found in lipoprotein concentrations. Our results were generally found in accordance with the few other previous studies on lipoproteins in cord blood. The results were also directly comparable to those in a recent study on the LDL‐cholesterol concentration in cord blood (12). This supports the assumption that the lipoprotein composition in newborns is similar to that found in adults. The distribution of the cord blood lipid and lipoprotein concentrations in the sample are given, and by use of non‐parametric statistics the contidence intervals for the true percentiles in the population were calculated.
CHILD HEALTH IN SWEDEN
Authors: SJÖLIN STIG , VAHLQUIST BO
Published: January 2008

ABSTRACT. Sjölin, S. and Vahlquist, B. (Department of Paediatrics, University Hospital, Uppsala, Sweden). Child health in Sweden. Acta Paediatr Scand, 63: 485, 1974.—On the basis of relevant vital statistics, some characteristic features of the health of Swedish children during the last two centuries are presented and analysed with regard to probable causative factors. An attempt is also made to define in brief the child health problems of the future.
CYSTINE: A SEMI‐ESSENTIAL AMINO ACID IN THE NEWBORN INFANT
Authors: POHLANDT F.
Published: January 2008

ABSTRACT. Pohlandt, F. (Department of Paediatrics, Section of Neonatology, University Hospital, Ulm, Federal Republic Germany). Cystine: a semi‐essential amino acid in the newborn infant. Acta Paediatr Scand63: 801, 1974.—The enzyme activities of the transsulfuration pathway (Cystine biosynthesis) are low or not measurable in the livers of human fetuses, premature and full‐term newborns. Cystine thus may be an essential amino acid in newborn infants. To test this suggestion, we studied the plasma amino acid concentrations in 20 premature and 7 full‐term infants treated for prematurity and/or respiratory distress syndrome by ion exchange columnchromatography. The infants received infusions of a 5% glucose‐electrolyte‐solution during the first two days of life. In premature newborns the plasma cystine concentrations markedly decreased within the first 12 hours of life (median <5 μmol/l) and remained low tlfereafter. In full‐term infants the plasma cystine concentrations decreased similarly. To rule out the possibility that these low plasma cystine concentrations were the result of a reduced cystine biosynthesis due to a lack of methionine, a substrate of transsulfuration pathway, we studied premature on infusion of a mixture of synthetic l‐amino acids free from cystine. Despite elevated plasma methionine concentrations (median 113.4 μnol/l) the plasma cystine concentrations remained very low. Cystine, therefore, can be considered a semi‐essential amino acid in the newborn infant and should be supplied to newborns receiving balanced parenteral nutrition.
BRAIN GROWTH IN CHILDREN WITH KWASHIORKOR A Study using Head Circumference Measurement, Transillumination und Ultrasonic Echo Ventriculography
Authors: ENGSNER GUNNAR , HABTE DEMISSIE , SJÖGREN IRÉNE , VAHLQUIST BO
Published: January 2008

ABSTRACT. Engsner, G., Habte, D., Sjögren, 1. and Vahlquist, B. (Ethiopian Nutrition Institute and the Ethio‐Swedish Pediatric Clinic, Addis Ababa, Ethiopia, and the Department of Paediatrics, University Hospital, Uppsala, Sweden). Brain growth in children with kwashiorkor. A study using head circumference measurement, transillumination and ultrasonic echo ventriculography. Acta Paediatr Scand, 63, 1974.—Brain growth was studied in 53 children with kwashiorkor and marasmic kwashiorkor by the simultaneous use of head circumference measurement, transillumination and ultrasonic echo ventriculography. The results of examinations on admission showed that the head circumference was reduced but probably slightly less so than in marasmic children. The transillumination findings were distinctly abnormal: bearing the age difference in mind, the abnormality was more pronounced than in the cases of marasmus. The lateral ventricle index, calculated from echo ventriculograms, was increased, which was not the case in marasmus. A systematic follow‐up study for 6 months of 10 patients showed a gradual and complete normalization of the transillumination and echo ventriculographic findings. The interpretation of the results, taking into consideration also the possible sources of error, is discussed.
THE EFFECT OF DISACCHARIDES ON THE HYPERLACTACIDAEMIA OF GLUCOSE‐6‐PHOSPHATASE‐DEFICIENT CHILDREN
Authors: FERNANDES J.
Published: January 2008

ABSTRACT. Fernandes, J. (Department of Paediatrics, Sophia Children's Hospital and Neonatal Unit, Erasmus University, Rotterdam, The Netherlands). The effect of disaccharides on the hyperlactacidaemia of glucose‐6‐phosphatase‐deficient children. Acta Paediat Scand,63:695, 1974.—Two unrelated children with hepatic glycogenosis due to glucose‐6‐phosphatase deficiency were investigated as regards the influence of dietary disaccharides on their hyperlactacidaemia. The disaccharides were administered orally as 10% solutions hourly during 12 hours in an A‐B‐A sequence, i.e. maltose‐lactose‐maltose, or maltose‐sucrose‐maltose. Each disaccharide was given in repeated doses. Blood lactate levels increased during the lactose or sucrose feedings and decreased during the maltose feedings, while normoglycaemia was maintained throughout the experiments. It is concluded that lactose and sucrose should be restricted in the diet of children with a glucose‐6‐phosphatase deficiency.
THE ROLE OF SERUM FACTORS IN THE LYMPHOCYTE TRANSFORMATION TEST OF CHILDREN WITH ACUTE LEUKAEMIA
Authors: RÉVÉSZ T. , SZlGETI R. , SCHULER D.
Published: January 2008

ABSTRACT. Révész, T., Szigeti, R. and Schuler, D. (Second Department of Paediatrics, Semmelweis University Medical School, Budapest, Hungary). The role of serum factors in the lymphocyte transformation test of children with acute leukaemia. Acta Paediatr Scand,63:715, 1974.—PHA‐induced lymphocyte transformation tests were carried out in 14 children who were in full remission of acute lymphoblastic leukaemia and in 12 controls. Stimulation was assessed on the basis of tritiated thymidine uptake. For an adequate testing of lymphocyte reactivity, dose‐response curves were established for each child. No significant difference could be observed in either the maximum response, or the shape of the curve, between the leukaemic and the control groups. Supplementation of the culture medium with autologous plasma instead of AB serum did not result in any significant inhibition or stimulation of lymphocyte reactivity to PHA. The lymphocyte response of both leukaemic and control children, however, was greatly reduced when sera from untreated leukaemic children was used. Allogeneic sera drawn from patients in remission exerted little or no inhibitory effect, while that obtained in relapse was again more inhibitory. The observed effect was not due to cytotoxic antibodies, nor due to natural iso‐antibodies, but is thought to be a phenomenon closely connected with the active phase of malignant diseases. The exact nature of the agent is still far from being clarified at present.
ABSENCE OF INSULIN RESISTANCE IN 4 CASES OF MILD JUVENILE DIABETES
Authors: THORELL J. I. , NILSSON K. O. , HAGER A.
Published: January 2008

ABSTRACT: Thorell, J. I., Nilsson, K. O. and Häger, A. (Departments of Nuclear Medicine and Paediatrics, University Clinics, General Hospital, Malmö and the Department of Paediatrics, University Hospital, Linköping, Sweden). Absence of insulin resistance in 4 cases of mild juvenile diabetes. A preliminary report. Acta Paediatr Scand 64:248, 1975. –Four children with a mild non‐insulin‐requiring diabetes were studied. They had no insulin response at intravenous glucose tolerance test. When insulin was infused at a rate which simulated a normal early insulin response to intravenous glucose, blood glucose decreased to the same extent as it did in healthy subjects. When a normal early insulin response was simulated during the intravenous glucose tolerance test, the glucose assimilation rate was normalized. These results suggests that a peripheral resistance to insulin is unlikely in mild juvenile diabetes, and that the primary defect is a deficient release of insulin.