Bone disease in primary biliary cirrhosis: Lack of association with distal renal tubular acidosis

Background and Aims: Primary biliary cirrhosis (PBC) might be complicated by osteoporosis, whose etiology remains unknown but seems to be multifactorial. Prevalence rates of 30% to 60% for distal renal tubular acidosis (DRTA) have been reported in PBC patients, generally as incomplete DRTA. Although it is undisputed that a reduced bone mineral density (BMD) is the expected outcome among patients who have been suffering from longstanding chronic metabolic acidosis, it is unclear if incomplete DRTA is also associated with metabolic bone disease in PBC patients. The present study was undertaken to compare the BMD of PBC patients with and without DRTA.
Methods: The BMD of 23 PBC patients (11 with DRTA and 12 without), all with normal clearance of creatinine, was assessed by dual energy radiograph absorptiometry. The diagnosis of DRTA was made if the urine pH was above 5.4 in all samples after the oral acid overload, showing tubular inability to acidify urine in the presence of test-induced systemic metabolic acidosis.
Results: Densitometric signs of osteoporosis were found in 82% of DRTA cases and in 83% of patients without DRTA (difference not significant). There were no significant differences in BMD measurement, T and Z scores of patients with and without DRTA.
Conclusions: The present study could not support a correlation between the presence of DRTA and the bone loss observed in PBC patients.     

Female patients complaining about hair loss: documentation of defective scalp hair dynamics with contrast-enhanced phototrichogram

BACKGROUND: The complaint of chronic hair loss frequently affects female subjects and there is little or no objective technology available in the general dermatology or even in the hair clinics to guide the observer in the management of the patient. The purpose of this report is to share the results of refined hair growth measurements that were collected in 92 female subjects complaining about hair loss. METHODS: Clinically they were classified as having a patterned hair loss according to Ludwig (L; n=50), diffuse hair loss (D; n=13) or no visible hair loss but complaining of hair shedding (N; n=29). Two scalp sites on the top of the head and one occipital site were investigated after clipping by close-up photography before and after a hair dye (contrast enhancement, CE). Forty-eight hours later a new photograph was taken after CE in view of phototrichogram analysis (CE-PTG). Finally a last hair clip was performed 30 days later and hair thickness and length determined for linear growth measurements (LHGR). RESULTS: Herein we confirm that the top of the head shows usually a higher hair density than occipital sites, a physiological observation that applies both to men and women. From the technological perspective, we also document that CE improves hair detection in all sites. Interestingly, in affected patients (L and D) the relative increase of hair counts after CE was much higher (range +22.4% to +28.3%) compared with apparently unaffected females (N; range +8.2% to +9.7%). This increase in hair counts was only due in part to the presence of less pigmented thinning hair (thickness less than 40 microm). Such thin hairs were found in statistically significantly higher proportions in younger patients with mildly severe (grade I) patterned alopecia (Ludwig: L). In other patients with hair loss and in more severe forms of patterned alopecia - especially in older patients - the thin hair is not detected in abnormal proportions. In all sites slower growth rates and decreased anagen percentages indicate a defective hair replacement programme distinguishing L patterns from diffuse hair loss and from apparently unaffected patients complaining of chronic hair loss. Globally, we also noted that increasing age is associated with significant regression of scalp hair (decreased hair counts, thinner hair and slower LHGR). CONCLUSION: On the basis of the present data together with female data from the literature and our own studies in male subjects, we suggest a three-step mechanism leading to hair loss 1.Shortening of growth phase the hair cycle with maintained thick hair, i.e. more frequent hair cycling that leads to more hair shedding. 2.Intermittent production of short thin hair, i.e. morphological evidence of miniaturisation. 3.Very occasional or almost no hair production, i.e. dormant follicles or irreversible follicular atrophy. Depending on the genetic background, hormonal microenvironment in the scalp and conditioning of individual hair follicle bio-responses, female and male patterned hair loss may end up into different phenotypes.     

Attempts to make models for Alzheimer''s disease

Profound reductions in cortical acetylcholine levels together with degeneration of cholinergic neurons in the basal forebrain have been reported in patients with Alzheimer's disease. A similar loss of the cholinergic neurons of the basal forebrain and impairment of learning and memory occur in animals injected with a nerve growth factor-diphtheria toxin conjugate, suggesting that this animal model is suitable to analyze cholinergic roles on learning and memory processes, and also the pathogenesis of Alzheimer's disease. In addition, animal models constructed by electrolytic or neurotoxic lesioning of the basal magnocellular nucleus, and models made by transgenetic technology were described.     

Treatment of hepatitis B and C after liver transplantation. Part 1, hepatitis B

Abstract The outcome of OLT for HBV-related liver disease is dependent on the prevention of allograft re-infection. Over the past decade, major advances have been made in the management of HBV transplant candidates. The advent of long-term hepatitis B immune globulin (HBIG) administration as a prophylaxis against HBV recurrence, and the introduction of new antiviral agents against HBV infection, such as lamivudine (LAM), were a major breakthrough in the management of these patients. Results of OLT for HBV infection are similar to those achieved with other indications. Pre-OLT antiviral treatment such as LAM can suppress HBV replication before OLT and thus decrease the risk of re-infection of the graft. Combination prophylaxis with LAM and HBIG after transplantation highly effectively reduces the rate of HBV re-infection, even in HBV replicative cirrhotic, patients. The optimal HBIG protocol in the LAM era is yet to be defined: dosing of HBIG, routes of administration, and possibility of stopping HBIG. Several antiviral drugs have been developed for the management of HBV infection on the graft, so outcome is currently good.     

Familial spontaneous pneumothorax in three generations and its HLA

We experienced a case of familial spontaneous pneumothorax in three generations. Six of 13 family members had episodes of spontaneous pneumothorax. It is well established that there are some diseases associated with human leukocyte antigen (HLA). We performed HLA phenotyping for HLA of A, B and C. In our study, we detected the HLA haplotype A2, B61 in three of 4 who had episodes of spontaneous pneumothorax. The HLA haplotype A2, B70 were also detected in three of 4 who had episodes. This suggests that familial spontaneous pneumothorax might have hereditary factors.     

Management of ascites in cirrhosis

Ascites is one of the earliest and most common complications of patients with cirrhosis. A typical circulatory dysfunction characterized by arterial vasodilation, high cardiac output and stimulation of vasoactive systems is commonly present in these patients and is associated with a poor prognosis. The treatment of ascites has been based on the combination of a low-sodium diet and the administration of diuretics. The reintroduction of paracentesis and the recent introduction of the transjugular intrahepatic portosystemic shunt (TIPS) are the most relevant innovations in the treatment of ascites during the past two decades, although controlled trials in large series of patients are needed to delineate whether TIPS is a safe and useful treatment for these patients.     

表皮生长因子及其受体与流产关系的探讨

采用放射免疫竞争抑制饱和分析法，检测４７例难免流产患者，６５例人工流产及２０例健康非孕妇女血清表皮生长因子（ＥＧＦ）水平；同时采用免疫组织化学法检测流产者胚胎组织中表皮生长因子受体（ＥＧＦＲ）表达程度。结果：人工流产组血清ＥＧＦ水平高于自然流产及非孕妇女，而自然流产与非孕妇女差异无显著性；胚胎组织中ＥＧＦＲ阳性表达率人工流产组明显高于自然流产组。提示：ＥＧＦ通过与其受体结合对妊娠维持、胚胎及胎儿的     

Chronic parasite infection in mice induces brain granulomas and differentially alters brain nerve growth factor levels and thermal responses in paws

Schistosoma mansoni infection, both in humans and in animal models, is known to induce granulomas in the liver and intestine. It has also been reported that in humans the eggs of this parasite can reach the brain, causing psychiatric and neuropathological disorders. Whether this also occurs in rodents is unknown. To answer this question, mice were infected with this parasite and the central nervous system (CNS) examined at various time intervals. The results show that schistosomiasis induced granulomas in several regions of the CNS and increased nerve growth factor (NGF) levels in the cortex, hypothalamus and brain stem, but not in the hippocampus. The infection also caused paw hyperalgesia, as determined by the hot-plate test, and a local increase in NGF, but not in substance P. These findings indicate that the murine model of infection can be used for studying mechanisms leading to human neuroschistosomiasis and suggest that the neuropathological disorders and the sensory deficits observed in human schistosomiasis are associated with impaired levels of NGF in the peripheral and central nervous system. Received: 18 January 1996 / Revised, accepted: 16 April 1996     

小儿氨基酸注射液治疗胎儿宫内发育迟缓的探讨

经临床鉴定静脉内注射小儿氨基酸对治疗胎儿宫内发育迟缓具有肯定效果。在Ｂ超监视下，使用小儿氨基酸的孕妇组于用药期间，胎儿生长参数ＢＰＤ和ＨＣ显著高于使用成人氨基酸的孕妇组。１０例足月娩出胎儿体重均达到甚至超过２．５ｋｇ。由此得出结论：使用小儿氨基酸注射液对于改善胎儿宫内发育迟缓优于成人氨基酸。