摘要:

SummaryIntestinal biopsies from two patients with glucose‐galactose malabsorption have been subjected to biochemical and histological studies and the absorption of glucose, mannitol and sodium has been studied with a perfusion technique in one of the patients. No accumulation of glucose against a concentration gradient was found duringin vitroincubation of mucosa from the patients. Phloridzin did not further decrease the slow glucose uptake by the mucosa. L‐Alanine uptake was normal. Intestinal mucosa from both parents of a patient with glucose‐galactose malabsorption accumulated glucose. In mucosa from patients with glucose‐galactose malabsorption disaccharidase activities were normal, sodium activation of invertase could be demonstrated and ouabain‐inhibited (pump‐) ATP‐ase activity was present. The histology was essentially normal both by light microscopy and electron microscopy. The per fusion studyin vivodemonstrated not only a very slow absorption of glucose but also leakage of glucose into the lumen. Sodium absorption was not impaired. From the data obtained it is suggested that this inborn error of metabolism is localized at the level of the glucose‐galactose membrane carrier of the small in

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07291.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryTwo patients with Hunter's syndrome (mucopolysaccharidosis type II) were studied. The exact diagnosis could be settled by the finding of clinical symptoms and signs, typical for this form of mucopolysaccharidosis, and of a greatly increased excretion of glycosaminoglycans (acid mucopolysaccharides) in the urine, as well as by the fact that the patients were half‐brothers with unrelated fathers.Analyses of glycosidases in skin demonstrated the existence of low activity of β‐galactosidase in both patients. β‐acetylglucosaminidase was more active in both patients and β‐glucuronidase in one of them than in the controls.In plasma increased activities were noted for β‐glucuronidase and β‐acetylglucosaminidase in both patients and of β‐galactosidase and α‐mannosidase activities in one of them and of α‐fucosidase activity in the other.The results are very similar to those described in patients with Hurler's syndrome (muco

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07292.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryA comparison of the effect of penicillin V and ampicillin in the treatment of scarlet fever has been made in two series of 110 patients each. Clinically and bacteriologically both drugs showed a very satisfactory effect, penicillin V being superior–half the dose of this drug bringing about a rather shorter period of fever, quicker elimination of bacteria, and less streptococcal recurrence. The serological reaction, judged from the antistreptolysin titre, was the same in the two series, and likewise the incidence of recurrences of scarlet fever.As regards sife‐effects there was a considerable difference. Exanthema, sometimes accompanied by fever, occurred on the eighth to tenth day of 10 ampicillin cases (9%) but in no penicillin V case. One patient on ampicillin had a mild mucocutaneous syndrome. In 11 (10%) of the ampicillin cases and 4 of the penicillin V cases there was a clinically inexplicable febrile condition of a fairly pronounced nature beginning on the third to eighth day. The author interpret the exanthema cases as an allergic reaction of the nature of serum disease, the febrile cases on the other hand as a non‐allergic type of drug fever, perhaps being brought about by a change in the intestinal flora. A knowledge concerning the latter type of reaction is of practical signifi

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07293.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryTwo patients with Hartnup disease showed increased urinary excretion of conjugated histamine and increased faecal excretion of histamine‐like activity. After addition of L‐histidine, histamine was formed in faecal specimensin vitro. After ingestion of L‐histidine there were signs of no more than a moderate intraluminal formation of histamine in the large bowel. These findings indicate that in Hartnup disease there is no marked malabsorption of L‐histidine, a thesis that will be tested

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07294.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07295.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummarySerum immuno‐reactive insulin was measured during glucose tolerance tests carried out in 18 children aged between 5 months and 13 1/4 years. Relatively small increments in serum‐insulin were found in many of the younger child

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07296.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryTwo siblings with hypoglycemia had from early infancy retardation of growth which seemed to be due to isolated deficiency of somatotropin. The father had diabetes mellitus which may be of etiologic significance. They have been followed from birth to the ages of 6 and 7 1/2 years respectively. Experience with long‐term therapy with HGH, 2 mg three times weekly, from the age of 3 1/2 and 2 years is reported. A purified HGH preparation which is supposed to contain only the somatotropin proper, seems to be preferable. A reduction of the HGH dosage to 1 mg three times weekly resulted in a marked reduction in height increments, and almost no growth was observed in the short periods without therapy. Total growth during a 4‐year‐period was 29.5 and 32.5 cm respectively. However, the therapy was probably initiated too late and the height continues to be far below the 2.5 percentile, particularly in the older child. The importance of an adequate therapy from early infancy in the more severe cases of hypopituitary dwarfism is stressed.Attention is drawn to the hematological aspect of hypopituitary dwarfism, and the increased iron requirement during the initial period with catchup g

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07297.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryCord blood red cells from 8 full‐term infants were labelledin vitrowith di‐iso‐propylfluorophos‐phonate (DF32P) and transfused into 8 adult recipients.The disappearance of the labelled cells was slightly curvilinear with a rate of 1.0–1.5% per day during the first month after transfusion and 0.7‐1.0% per day during the following month.The data are discussed with reference to earlier studies and it is concluded that cord blood red cells from full‐term infants disappear from the circulation of adult recipients in much the same way as from the circulation of newborn infants. The finding favours the hypothesis that “intrinsic” rather than “extrinsic” factors are likely to cause the difference in life span between focta

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07298.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryEquations were developed for the relation between the production rate, the life span frequency function and the number of circulating erythrocytes and for the relation between the production rate, the life span frequency function and the relative number of circulating cells after random labelling.With the help of explicit expressions for the production rate and the life span frequency function of erythrocytes formed during the latter part of foetal life, the increase in number of circulating cells during this period and the relative decrease in circulating labelled cells in a simulated cord red blood cell survival experiment were calculated on a digital computer and compared with experimental data. Through the simulation procedure, the parameters in the life span frequency function and the production rate function were estimated.The life span frequency function of the red blood cells did not change appreciably during the last 60 days of foetal life.The mean cell life span was found to be between 45 and 70 days. The life span frequency function was skew with the majority of cells dying before the mean life span.The relative rate of production of erythrocytes during the last two months of gestation was found to be 3–5 times that of normal adult

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07299.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY

摘要:

SummaryA 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 catabolis

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1968.tb07300.x

出版商:Blackwell Publishing Ltd    

年代:1968

数据来源: WILEY