Autoimmune reactions in patients with M-component and peripheral neuropathy.

A study of 17 patients with autoimmune axonal or demyelinating peripheral neuropathy in combination with M-component is described. The M-component was associated with MGUS (monoclonal gammopathy of undetermined significance) in 12 patients, CLL in one patient, WaldenstrÖm's disease in one patient, and myeloma in three patients. Immunohistological examination with direct and indirect fluorescence showed binding of antibodies to nerve structures of the same class and light chain as seen in the M-component. In five cases of IgM M-component, the demyelinating neuropathy was caused by binding of the IgM M-protein and complement C3b to myelin-associated glycoproteins (MAG). In 12 cases with axonal neuropathy, binding of IgG to the connective tissue of the peri- and endoneurium was found in 50% of cases, IgM in five cases, and IgD in one case. None of the patients had central nervous system (CNS) symptoms. The clinical and therapeutic difficulties are discussed; only two patients with an acute course responded to immunosuppression. A marked co-expression of other autoimmune phenomena is interpreted in the light of cross-reactions between the autoantibody and similar tissue autoantigens.     

Comparative analysis of the polycystic kidney disease 1 (PKD1) gene reveals an integral membrane glycoprotein with multiple evolutionary conserved domains

PKD1 is the major locus of the common genetic disorder autosomal dominant polycystic kidney disease (ADPKD). Analysis of the predicted protein sequence of the human PKD1 gene, polycystin, shows a large molecule with a unique arrangement of extracellular domains and multiple putative transmembrane regions. The precise function of polycystin remains unclear with a paucity of mutations to define key structural and functional domains. To refine the structure of this protein we have cloned the genomic region encoding the Fugu PKD1 gene. Fugu PKD1 spans 36 kb of genomic DNA and has greater complexity with 54 exons compared with 46 in man. Comparative analysis of the predicted protein sequences shows a lower level of homology than in similar studies with identity of 40 and 59% similarity. However key structural motifs including leucine rich repeats (LRR), a C-type lectin and LDL-A like domains and 16 PKD repeats are maintained. A region of homology with the sea urchin REJ protein was also confirmed in Fugu but found to extend over 1000 amino acids. Several highly conserved intra- and extra- cellular regions, with no known sequence homologies, that are likely to be of functional importance were detected. The likely structure of the membrane associated region has been refined with similarity to the PKD2 protein and voltage gated Ca2+ and Na+ channels highlighted over part of this area. The overall protein structure has therefore been clarified and this comparative analysis derived structure will form the basis for the functional study of polycystin and its individual domains.      

SCREENING PROCEDURES IN PEDIATRICS

Pediatric health screening procedures, both prenatal and postnatal, have a tremendous potential in improving the health status of children and in turn reducing the resource burden on the parents and the State. The existing recommendations, inherent problems and different screening procedures are discussed. The need for suitable mass screening pediatric procedures in the Indian context is stressed.KEY WORDS: Pediatric screening procedures     

术中胆道造影在腹腔镜胆囊切除术中发现胆道损伤的作用

目的：评估术中胆道造影（IOC）在早期发现腹腔镜胆囊切除术（LC）术中胆道损伤的作用。方法：回顾分析31例LC术后胆道损伤的部位，机理，诊断时间，治疗方法及结果，并对是否行IOC进行评估。结果：胆道错认引起胆道损伤共19例，其中12例IOC显示胆道错认，致胆道部分切开损伤，腹腔镜下行I期修复或开腹修复，T管引流，但无并发症。19例中3例IOC误读及4例未行IOC患者中发生迷走胆管损伤2例，胆总管完全离断1例，胆总管完全离断合并胆道缺损2例，本组有2例损伤发生于IOC后，结论：行IOC并正确阅读可及时发现腹腔镜胆囊切除术中胆道错认所致的胆道损伤，从而防止随之可能发生的严重并发症。     

OVARIAN HYPERSTIMULATION SYNDROME DURING INDUCTION OF OVULATION FOR INTRA UTERINE INSEMINATION

Ovarian Hyperstimulation Syndrome (OHSS) is a known iatrogenic complication of ovulation induction. Our experience of such complication while managing basic assisted conception cycles has been analysed in the present study. 12 such cases were identified in 976 cycles studied giving an overall incidence of 1.22%. All the cases were of mild to moderate variety and were managed conservatively. The duration of the complication ranged between 10 days to 6 weeks. Polycystic ovarian disease, LH: FSH ratio of more than 1, presence of four or more secondary follicles were found to be important predictive criteria. Identification of predictive factors of OHSS can be helpful in taking due care while using ovulation inducing drugs. Conception does worsen OHSS, but termination is usually not necessary.Key Words: OHSS, Ovulation Induction     

绝经后女性类风湿关节炎患者骨密度变化影响因素分析

目的探讨绝经后女性类风湿关节炎(RA)患者发生骨质疏松(OP)的原因。方法收集63例绝经后女性RA患者一般临床资料、疾病活动相关指标、手足X线资料,并同时测量患者7个部位的骨密度。结果23例患者(36.5%)有至少一处检测部位表现为低骨量。前臂和桡尺骨远端的低骨密度发生率(23.8%)与OP总发生率(28.6%)无显著性差异(P>0.05)。OP的发生与疾病活动指标及糖皮质激素的使用均无相关性。多元回归分析显示绝经的年限和关节腔狭窄为低骨量发生的独立危险因素(P<0.05);而雌激素替代疗法(HRT)为低骨量发生的唯一保护因素(P<0.05),小剂量糖皮质激素对本组患者的骨密度无影响。结论OP是绝经后女性RA患者的常见并发症,绝经年限长和关节破坏严重是低骨量发生的独立危险因素,HRT是防治低骨量的保护因素。