摘要:

SummaryUltrasoundcardiography (UCG) studies have been performed in about three hundred children. The age of the children varied between 1 day and 16 years. Most of the studies have been performed without premedication and all without discomfort.The UCG‐technique has been found valuable for demonstration of dilatation of the right ventricle both in cyanotic and acyanotic congenital heart disease. In studies of lesion affecting the tricuspid valve pathological UCG‐findings have been obtained in patients with tricuspid atresia and Ebsteins anomaly.The method has also been found useful in evaluation of the state of the mitral valve. Pathological findings have been obtained in congenital mitral stenosis, mitral atresia and other forms of hypoplastic left heart syndrome, in endocardial cushion defects and in mitral regurgitation.The UCG‐technique also provides information about the outflow tract of the left ventricle. Pathological findings have been obtained in cases with membraneous subvalvular aortic stenosis and hypertrophic obstructive cardiomyopathy.Pericardial effusion and thrombosis in the right atrium may be demonstrated by this technique. No complications have been obs

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06631.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryTwo siblings have been described who developed a fatal metabolic acidosis during the first days of life. The acidosis was found to be caused by an accumulation of lactic acid in the second sibling. In the first sibling, no determination of lactic acid was performed, but the similarities in clinical, biochemical and pathological findings combine very strongly to suggest an identical condition.The children died at the age of 100 and 177 hours. The main pathological findings were confined to the brain, the thymus and the liver. Softening of the white matter was pronounced with delayed myelination. Organized bilateral paraventricular cystic spaces were found. Fatty changes were pronounced in the liver.In some respects these children differ from those previously reported with congenital lactic acidosis.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06632.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryBiochemical studiesin vivoandin vitrowere performed in a newborn child suffering from congenital lactic acidosis. A block in the oxidation of lactate as well as an abnormally high rate of glycolysis have previously been suggested to be of etiological significance. In the present studies direct evidence has been obtained for a decreased capacity for gluconeo‐genesis as the cause of the lactate accumulation. In addition, the rate of glycolysis was not increased, and no block in the oxidation of lactate was presen

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06633.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryA Danish family with five proven and three additional possible cases of the oculo‐cerebro‐renal syndrome is described. All proven carriers had cataract, but the lenticular opacities varied considerably with respect to magnitude, appearance, and location. In this family, the disease was transmitted as a sex‐linked recessive trait, the complete syndrome being seen in boys

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06634.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryMarked abnormalities of pulmonary blood flow distribution were detected in 13 out of 21 children with cystic fibrosis using lung scanning. Underperfusion of different grade was localized most frequently in the right upper area, followed by the right middle and left upper areas. Correlation between clinical condition or impairment of gas exchange and degree of perfusion disturbances was lacking. Abnormal chest roentgenograms were encountered more frequently than the definite disturbances in the distribution of perfusion. On the other hand, even profound defects of perfusion were not revealed by chest roentgenography. Repeated lung scanning might be helpful in the evaluation of the dynamics of pulmonary involvement in cystic fibrosis.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06635.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06636.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryA test‐meal containing 2 g of glucose per kg body weight (minimum of 20 g), 0.5 g of D‐xylose per kg, 8 ml of standardized cream (12% fat) per kg and 7 500 IU of vitamin A palmitate per kg was administered through a nasogastric tube. During the next 10 hours capillary blood samples were obtained and analysed for glucose, D‐xylose, triglycerides, and vitamin A using microanalytical methods.The results in 16 normal infants aged 1 to 20 months are reported. The results for all parameters determined, except D‐xylose, were comparable to those previously reported using single tolerance tests. The variability related to age was pronounced for glucose, triglycerides and vitamin A.In patients with malabsorption syndromes (cystic fibrosis, extrahepatic biliary atresia, intrahepatic cholestasis, and gluten‐induced enteropathy) the results were also comparable with those earlier observed using single tolerance tests.A combined test‐meal has certain advantages over the examinations now used in infants with symptoms suggesting a malabsorption syndrome: a series of tolerance tests is avoided, and it is therefore easier to follow the absorption of fat and carbohydrates during the course of a disease and during a therapeu

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06637.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryAn 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 dise

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06638.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryThe respiratory response to an added load, recorded as the change in amplitude of the intra‐esophageal pressure swings caused by airway occlusion has been studied in 8 babies, 30 to 42 weeks of postmenstrual age. With increasing age the response to such an occlusion was found to increase gradually and it is concluded that this is a reflection of an increasing maturation of a thoracic respiratory reflex. The results are related to similar findings in developing animals and to earlier studies on the development of the Hering‐Breuer inflation reflex in bab

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06639.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummarySix children with a sucrase‐isomaltase deficiency were subjected to an oral tolerance test with sucrose or lactose combined withd(+)‐xylose.Simultaneous determination of a xylose curve allowed a more certain interpretation of the blood glucose curve. A flat glucose curve combined with a normal xylose rise points to an impaired enzymic hydrolysis rather than to a defective absorption of its component monosaccharides. Compared with the normal range ±2 S.D. derived from 90 xylose curves of 61 “normal” children, the sucrase‐isomaltase deficients showed normal xyl

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06640.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY