摘要:

Abstract.Thirty‐five infants of strictly controlled diabetic mothers (IDM), 12 infants of gestational diabetic mothers (IGDM) and 29 control infants were studied to assess the influence of maternal blood glucose level during pregnancy on infant metabolic measurements. At 2 hours after birth the disapperance ratektof intravenously injected glucose was determined. Plasma concentration of glucose, insulin, FFA, glycerol and β‐hydroxybutyrate were followed in umbilical arterial blood. The 2 hour meanktvalue of IDMs (1.27) was higher (p<0.05) than in IGDMs (1.14) and controls (0.80), but the group mean values were no different at 3 to 5 days although thektvalues were higher. In 10 IDms,ktvalues were determined both at 2 hours (1.24) and at 3 days (1.39) without significant differences. Pretest FFA but not glycerol values correlated inversely toktvalues in IDMs and IGDMs. A late insulin peak at 60 min was found in both controls and IGDMs. Insulin responses were unrelated toktvalues. No relation was found between pregnancy glucose value (5 daily determinations during 4 and 2 weeks prior to delivery in DM and GDM respectively) or maternal glucose at delivery andktvalues of IDMs and IGDMs. According to the FFA and glycerol values at 2 hours after birth, 3 sub‐groups of IDMs and IGDMs were arbitrarily formed. IDMs and IGDMs of group 1 had glucose,kts, FFA, glycerol and β‐hydroxybutyrate values no different from controls, whereas sub‐groups 2 and 3 showed increasing metabolic deviations. Clinical data and pregnancy glucose levels could not separate the 3 sub‐groups. However, the day‐to‐day variation in maternal glucose was greater in sub‐group 3. Mean values of daily maternal blood glucose differences at 10 o'clock correlated to 2‐hourkts in IDMs. We conclude that strict metabolic control during pregnancy will normalize metabolic adjustment of the infant, thus supporting the maternal hyperglycemia‐fetal

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04398.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.Glycerokinase activity was measured in brain and liver tissue of deceased low birth weight newborns. No glycerokinase activity was found in the brain. In the liver a relatively high activity of glycerokinase was ascertained even in very immature newborns.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04399.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.Red cell organic phosphates and especially 2,3‐diphosphoglycerate (2,3‐DPG), lowers the oxygen affinity of hemoglobin (Hb) and shifts the oxygen dissociation curve to the right. Because of the importance of 2,3‐DPG (as regulator of the oxygen affinity of Hb), determinations were carried out on: 45 normal children, 7 children with iron deficiency anemia and 35 children with acute lymphoblastic leukemia. In normal children with Hb of 12.69±1.60 g/100 ml, 2,3‐DPG was 14.90±0.68 μmoles/g Hb. In children with iron deficiency anemia (Hb 7.94±1.20 g/100 ml), 2,3‐DPG was 20.87±3.11 μmoles/g Hb. 2,3‐DPG was normal (14.11±0.88 μmoles/g Hb), in 16 patients with acute lymphoblastic leukemia who had never relapsed, while in 19 patients with a history of one or more relapses, 2,3‐DPG levels were increased (22.05 ±2.75 μmoles/g Hb). No good explanation may be offered for the high 2,3‐DPG levels i

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04400.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.The clinical and laboratory findings in a 2½‐year‐old boy with non‐ketotic hyperglycinaemia are reported. Except for a somewhat impaired liver function there was a picture similar to the cases previously reported in the literature. The patient deviated from the classical description, however, in the excretion of large amounts ofd‐glyceric acid in the urine. The same compound was also repeatedly found in the serum. It is suggested that the large amounts of glycine found in various body fluids are secondary to a hitherto undescribed enzymatic defect in the degradation ofd‐gly

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04401.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.Patients with hereditary hemolytic anaemias have been known to have immunoglobulin alterations related to an increased susceptibility to infection. In the present series, immunoglobulins G, A and M have been estimated in 50 thalassemic children aged 10 months to 13 years. No significant difference was found in any of the immunoglobulins between patients and age‐matched controls. No correlation could be shown between immunoglobulin levels and (a) the severity of anaemia, (b) the degree of hemosiderosis, and (c) the frequency of febrile infections per year. Against clinical impressions patients did not show any increased susceptibility to infection

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04402.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.In a retrospective study of hospital and autopsy records of 19 male subjects with the Duchenne type of progressive muscular dystrophy the incidence of cardiac involvement was found to be 84%. Patients with dystrophic involvement of the heart almost invariably develop heart failure; an early sign may be persistent tachycardia and, possibly, electrocardiographic changes, in case of which institution of digitalis treatment should be considered. Cardiac and pulmonary complications were equally frequent causes of death (42%) but, death from cardiac complication occurred only among the patients with dystrophic involvement of the myocardium. It is concluded that patients with Duchenne progressive muscular dystrophy very often develop cardiac complications, and when relating the available information on treatment to the autopsy findings it should be stressed that early and intensive therapy of the cardiac symptoms is of the greatest importance to the patient.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04403.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.A total of 104 children with allergic bronchial asthma were included in studies comparing the results of RAST and skin testing with each other and with the results of bronchial challenges using different concentrations of extracts of Cladosporium herbarum. Prick tests predicted bronchial allergy to the mould in 83–96% of the cases compared to 74–83% for RAST predicting such bronchial allergy. Bronchial reactions to top concentrations of the extracts were considered immunologically non‐specific and probably due to contents of irritants in most inst

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04404.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

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 provocati

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04405.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.Thirty‐six patients with nephroblastoma in Stages I, II and III treated at Radiumhemmet between 1966 and 1973, were analysed. The tumours were classified into three different histological types. All 10 patients in Stage III have developed metastases. All patients in Stages I and II (5 out of 26) who developed metastases had poorly differentiated (histological Type III) tumours. The indications for postoperative irradiation and chemotherapy for the different stages and histological types of the tumour are discusse

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04406.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Abstract.Three families with hereditary nephritis of Alport's type have been studied with particular emphasis on the occurrence of symptoms and signs in different sexes and age groups. Part of the material was tested for impaired hearing or eye anomalies which may be the sole manifestation of Alport's syndrome. The youngest patients were 3–4 years of age, and the proportion of diseased individuals increased with age. In two of the families about half of the investigated males or females were affected whereas in the third family all but one of 16 investigated individuals were affected. In this last family the kidney disease was exceptionally severe, but no differences in the histopathology of the kidney lesions were seen between the families. In all the families renal symptoms progressed more rapidly in males than in females. Affected as well as unaffected sons and daughters were born to healthy as well as to diseased mothers. In contrast, healthy fathers produced only unaffected children. Diseased fathers produced only daughters, two‐thirds of whom were affected. It is concluded that Alport's syndrome in these three families most probably is transmitted by autosomal dominant inheritance with variations in expression and penetra

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1976.tb04407.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY