Autoimmune hemolytic anemia and posthepatitis-C liver cirrhosis

Here we are presenting the case of a 70-years-old woman who has hepatic cirrhosis anti-HCV and insulin-dependent diabetes mellitus, without relevant epidemiologic ascendants or previous transfusions and HBV, HIV negatives. On admission to our hospital she showed signs of autoimmune hemolytic anaemia (AHA) which was confirmed by positive direct Coombs test and an improvement in blood test after corticoid treatment. Having discarded other possible causes such as drugs infectious diseases or essential mixed cryoglobulinemia (CME), we put forward the possible association between AHA and infection by HCV, where AHA was an extrahepatic immunological manifestation of HCV. This fact has never been brought to light in previous medical literature.     

Maternal nutrition and socio-economic status as determinants of birthweight in chronically malnourished African women

The birthweight is the most important determinant of mortality and morbidity in the neonatal period and may have an influence on health in adult life. The high rate of low birthweight in developing countries is therefore a major health problem. Maternal malnutrition is usually assumed to be a causal factor but other environmental factors are also involved. In this study we analysed maternal nutritional and socio-economic factors as determinants of birthweight in term infants from a rural African society characterised by a high rate of chronic malnutrition. Relations of maternal weight, gestational weight gain, parity, socio-economic status and infant sex with birthweight were analysed in 1,477 women and child pairs. The selected women were followed from early pregnancy and had an uncomplicated delivery at term of a living singleton child. The gestational weight gain was 5.6 (SD 6.0) kg and the mean birthweight 2.933 kg (SD 408). Maternal weight, representing the maternal long-term nutritional situation, was the most important independent determinant of birthweight, accounting for 13.0% of the variance in birthweight. The weight gain, representing the short-term nutritional situation, explained only 5.6% of the variance. Birthweight increased by 20 g (CI 18-23) for each kg maternal weight and by 15 g (CI 12-18) for each kg gestational weight gained. The socio-economic difference in birth-weight was 153 g (CI 109-196) 88 of which (CI 48-128) remained unexplained after adjustment for differences in maternal weight, parity and gender. Improved long-term nutritional situation and living conditions seems to be the most important prerequisites to counteract low birthweight in developing countries.     

Prenatal diagnosis of a highly undifferentiated brain tumour--a case report and review of the literature

Intracranial tumours, often presenting with progressive hydrocephalus, are rare congenital diseases accounting for 0.5-1.5 per cent of all cases of brain tumours diagnosed during childhood. The differential diagnosis includes vascular malformations, infarctions, and haemorrhages. Sonographic signs suggestive of glioblastoma, teratoma, and astrocytoma do not establish the histological diagnosis, however. We report a case of an undifferentiated fetal glioma detected at 29 weeks' gestation. The diagnosis of an undifferentiated brain tumour was suspected by sonography because of the lack of normal brain structures in conjunction with a diffuse echogenic central lesion and an external hydrocephalus. Because of the very poor prognosis, we induced labour by intravaginal and intravenous administration of prostaglandin E2 and achieved the vaginal delivery of a stillborn child whose head circumference corresponded to 38 weeks of pregnancy. Histological and immunochemical features of this undifferentiated congenital glioma (glioblastoma) are presented.     

Rectus abdominis muscle flap (RAMF) technique for the management of bladder exstrophies: late clinical outcome and urodynamic findings

OBJECTIVE: To investigate the efficacy of the rectus abdominis muscle flap (RAMF) technique for the closure and augmentation of small, non-compliant bladder exstrophies. PATIENTS AND METHODS: The RAMF technique was used in two girls and two boys (mean age at operation, 31.7 months, range 3-72) with bladder exstrophy. The clinical outcome and urodynamics were assessed during a follow-up of 29 months to 6 years (mean 49.2 months) and included imaging, cystoscopy, biochemical and microbiological studies. RESULTS: There were no urinary tract infections, metabolic problems or electrolyte disturbances and kidney function remained normal in all patients. Radiography confirmed intact function and anatomy of the urinary tract and cystoscopy showed complete coverage of the inner peritoneal layer of RAMF with uroepithelium. No stone formation or mucus production were detected. Currently, three patients void using clean intermittent catheterization through the native urethra and the fourth through an appendiceal Mitrofanoff valve. However, the bladder volume was insufficient at the late follow-up and three patients required bladder augmentation. CONCLUSIONS: The RAMF technique is a good alternative for closing bladder exstrophies and achieves an increase in bladder capacity; however, although there is a mild improvement in bladder capacity and compliance. RAMF should not be used as a bladder augmentation procedure. The technique is indicated in the closure of large bladder defects, bladder exstrophies with small, inelastic, non-compliant bladder remnants and failed primary closures.     

Insertion/deletion polymorphism in the angiotensin-converting enzyme gene associated with macroangiopathy and blood pressure in patients with non-insulin-dependent diabetes mellitus

In the search for new risk factors for diabetic macroangiopathy the insertion/deletion (I/D) polymorphism in the angiotensin-converting enzyme gene was studied in 237 consecutive patients (125 men and 112 women) with non-insulin-dependent diabetes. The female population showed an excess of ischemic electrocardiographic changes or definite myocardial infarctions in the patients homozygous for the deletion [D/D; odds ratio (OR) 2.8; 95% confidence interval (CI) 1.4-5.3] and in the insertion/deletion heterozygotes (I/D; OR 1.8; CI 1.1-3.1) compared with the patients homozygous for the insertion (I/I). In the total series coronary heart disease, cerebrovascular disease, and claudication were more often observed in the patients with I/D (OR 1.5; CI 1.0-2.2) or the D/D genotype patients (OR 1.7; CI 1.1-2.6) than in those with the genotype I/I. The systolic blood pressure was lower in patients with genotype I/I (138 +/- 19 mmHg) than in those with the genotype I/D (149 +/- 22 mmHg) or D/D (150 +/- 21 mmHg; P < 0.02). The prevalence of hypertension and the median urinary albumin excretion rate also tended to be lowest in the I/I genotype patients. Multiple logistic analysis revealed that in women the angiotensin-converting enzyme D/D genotype is independently associated with coronary heart disease. Our findings suggest that variation at the angiotensin-converting enzyme gene locus is one of the factors involved in the predisposition of diabetic patients to the development of arterial disease and hypertension.     

The function of the reproductive system in x-ray-irradiated male rats after the transplantation of a culture of testicular cells from newborn piglets

The effect exerted by transplantation of the 5 day culture of testicles from newly born piglets on the hypogonadal state of mature X-ray irradiated (3 Gy) rats was studied. It has been found that a month after transplantation (two months after irradiation) the hypogonadal state disappeared for a while, which is confirmed by normalization of the weight of seminal vesicles, content of testosterone and biologically active lutropine, restoration of the reproductive function of rats. No significant changes in steroidogenesis activity have been found, which proves a substituent character of transplantation. The effect disappears three months later but certain restoration of the content of nucleic acids in the testicles is observed.     

Effect of cyclosporin on the activity of cytidine deaminase and adenosine deaminase in the serum and polymorphonuclear leukocytes of patients with rheumatoid arthritis

Cytidine deaminase (CDA) and adenosine deaminase (ADA) were investigated in the serum and polymorphonuclear leukocytes (PMNLs) of healthy controls and ten patients with rheumatoid arthritis before and during cyclosporin therapy. CDA was significantly raised in the serum and decreased in the cells of patients. A dramatic increase (10-fold or more) in CDA activity was observed in the cells of some patients after only one month of cyclosporin therapy. Serum CDA significantly increased after three months' therapy. While the increase in serum CDA level during therapy was transient, the enzyme level in cells remained permanently raised, as shown in two patients evaluated for sixteen months. ADA in the serum of RA patients was somewhat higher as compared with healthy controls and remained almost unchanged during cyclosporin therapy. ADA activity in the cells also increased, but compared with the increase in CDA activity this increase was lower. Cyclosporin increased both CDA and ADA activities in PMNLs of RA patients. The dramatic increase in CDA observed in PMNLs of patients could be the cause of the transient increase in CDA in the serum. Further investigations will show to what extent this property of cyclosporin can reflect the immunoregulatory effect of this drug.     

Treatment of essential arterial hypertension with hypercholesterolemia. Effects of an alpha-adrenergic blocker and an inhibitor of the angiotensin converting enzyme

BACKGROUND: Hypertension and hypercholesterolemia are frequently associated with this leading to considerable cardiovascular risk. METHODS: An open parallel randomized study was performed in which the effects of doxazosin, an alpha-adrenergic blocker and enalapril, an inhibitor of the angiotensin converting enzyme were compared in 70 patients with essential high blood pressure and plasma cholesterol levels greater than 240 mg/dl. Following 2-4 weeks of placebo administration the patients were randomly treated with one of the two drugs. When required doses were increased and hydrochlorothiazide added until blood pressure lower than 160/95 mmHg was achieved. After this period the patients were observed for a minimum of 8 weeks. The mean length of the study was of 22 weeks. RESULTS: Both drugs significantly reduced blood pressure without modifying cardiac frequency. Doxazosin tended to favorably modify the lipid profile of the plasma while enalapril significantly reduced the levels of cholesterol, lipids and high density lipoproteins (HDL). Upon termination of the study the total HDL/cholesterol index increased 8.6% in those treated with doxazosin and decreased 5.5% in those receiving enalapril (p < 0.05). CONCLUSIONS: Although doxazosin and enalapril are potent antihypertensive drugs, the effects on plasma lipid obtained with doxazosin indicate that a reduction in cardiovascular risk was achieved with this drug in the patients included in this study.