触须样免疫性肾小球病1例报告并文献复习

触须样免疫肾小球病（immunotactoidg lomerulopathy，IT）是指肾小球内存在呈中空的微管样结构的纤维样物质，但对淀粉样蛋白特殊染色阴性，一般不伴有系统性疾病的一类肾小球疾病，临床少见。我科曾收治1例，现结合文献报告如下。     

纤维样肾小球病1例报道

纤维样肾小球病为一种少见原发性肾小球疾病,病理特点为肾小球内存在淀粉样物质,但对淀粉样蛋白特殊染色阴性的一种新型的肾小球疾病。国内报道不多,近期确诊1例,现报道如下。     

纤维样肾小球病1例

纤维样肾小球病（fibrillary glomerulopathy,FGP）是指肾小球内存在类似于淀粉样纤维物质,但淀粉样物质特殊染色阴性,且不伴随系统性疾病的一类肾小球疾病。该病少见,至今国内外总共报道约200例,我院发现1例,现报道如下。病例1病例特点患者,女,59岁,主因发现血肌酐升高1年入院。     

纤维样肾小球病1例报告

纤维样肾小球病国外报道 10 0例以上 ,国内报告仅 11例 ,极为少见。兹将本院收治 1例报告如下。病　　例患者 ,女性 2 1岁 ,因双下肢水肿半年于 2 0 0 1年 11月16日住我院。半年前妊娠 6个月时发现双下肢轻度水肿 ,呈指凹性 ,尿蛋白 ,血压正常。经治疗尿蛋白转阴 ,后未注意。     

纤维样肾小球病1例报告

患者女性，54岁，因双眼睑水肿2年，乏力25d而于2003年6月16日入我院。患者2001年6月无诱因出现双眼睑水肿，以晨起时为重，经休息后可自行消退，无四肢关节肿痛，无光过敏，无头痛、头晕，无雷诺氏征，当时未诊断治疗。2003年5月21日出现全身乏力，至当地市级医院就诊，查血压120／80mmHg，尿蛋白 ，潜血 。诊断     

纤维样肾小球病二例

例1女性,54岁,因双眼睑水肿2年,乏力25 d于2003年6月16日入我院.入本院前曾在外院,诊断为慢性肾炎,经治疗无好转.体检除双下肢水肿外,余无异常.     

纤维样肾小球病1例诊治报告

纤维样肾小球病(fibrillary glomerulopathy,FGP)临床极少见,国内报告仅10余例,兹将本院收治1例报告如下。病例患者,男,61岁,因“双下肢浮肿1周”于2006年3月29日入院。4年前曾出现双下肢轻度浮肿及夜尿增多,当时检查尿蛋白1.0g/d,未发现继发性肾脏损害原因,未行肾脏病理检查。     

纤维样肾小球病

纤维样肾小球病王素霞，肖萍综述章友康邹万忠审校纤维样肾小球病（ＦｉｂｒｉｌｌａｒｙＧｌｏｍｅｒｕｌｏｐａｔｈｙＦＧＰ），是指肾小球内存在类似于症粉样纤维物质，但淀粉样物质特殊染色阴性，且不伴随系统性疾病的一类肾小球疾病。近年国外的肾脏病学专著内均已将...     

免疫触须样肾小球病1例报告

免疫触须样肾小球病（immunotactoid glomerulopathy,ITG）是指肾小球内存在中空的微管样结构的纤维性物质,对淀粉样蛋白特殊染色阴性,是一种罕见的原发性肾小球疾病,目前国内仅见数例报道。我科曾收治1例,现报告如下。     

Lipoprotein glomerulopathy: a pediatric case report

We report an 8-year-old girl with lipoprotein glomerulopathy who may have developed this condition as young as 4 years of age. To our knowledge this is the youngest reported case of this disease. Lipid studies of the patient and her family members revealed elevated concentrations of apolipoprotein E (apo E), the apo E phenotype E2/3, and the genotype E3/3. However, other families revealed no urinary abnormalities. Our findings suggest that an apo E abnormality may be responsible for the development of lipoprotein glomerulopathy. Genetic analysis of apo E is needed to clarify the pathogenesis. Received May 12, 1995; received in revised form and accepted September 17, 1996     

肝紫癜病一例及文献复习

目的提高对肝紫癜病(peliosis hepatis,PH)的认识。方法结合1例肝紫癜病病人的临床资料分析及文献复习,探讨肝紫癜病的病因、临床表现、诊断、鉴别诊断及治疗方法等。结果该病病因不明确,缺乏特异临床表现,术前诊断困难,需通过肝脏病理确诊,局灶性肝紫癜病可行手术切除,弥漫性应行肝移植术。结论肝紫癜病是一种罕见的肝良性病变,对非特异性表现的肝脏病变,应考虑此病可能。     

Hemifacial spasm caused by pontine glioma: case report and review of the literature

Hemifacial spasm (HFS) is an involuntary paroxysmal contractions of the facial musculature, caused generally by vascular compression of the seventh cranial nerve at its root exit zone from the brain stem. The case of an adult man harbouring brain stem glioma (BSG) whose only neurological signs were left HFS and mild facial weakness is reported. Radiological and neurophysiological findings are described. No responsible vessel could be identified during surgery, but the causative lesion was found to be an astrocytic tumour encasing the facial nerve at its root exit zone from the brain stem. The rarity of such a condition prompted us to review the literature. Nine cases, including our patient presenting with HFS caused by BSG, are reviewed.     

原发性肾滑膜肉瘤一例报告并文献复习

目的探讨原发性肾滑膜肉瘤的临床特点及诊治方法。 方法回顾性分析我院原发性肾滑膜肉瘤1例的临床资料,并对国内外相关文献进行回顾。 结果患者接受根治性左肾切除术,术后病理回报为原发性肾滑膜肉瘤。 结论原发性肾滑膜肉瘤非常罕见,无特异性临床特点及影像表现,诊断主要依靠病理及免疫组化,SYT-SSX融合基因有提示作用,主要治疗方案为手术,化疗对生存率的影响还需进一步研究。     

Membranoproliferative glomerulonephritis in two siblings: report and literature review

There is evidence of a genetic basis in some cases of idiopathic membranoproliferative glomerulonephritis (MPGN) types I and III, particularly those occurring in families. The clinical and morphological features and disease course in two siblings with MPGN are described. In the male sibling, both clinical and morphological features as well as serum complement profile suggested type I MPGN; electron microscopy appearance in the female sibling was consistent with type III MPGN. Both patients had treatment-resistant nephrotic syndrome which evolved into renal insufficiency in the girl. No hereditary complement deficiencies were found in siblings or their parents. Both children exhibited HLA-A24; -B27, w4; -DR11, 52; -DQ3 antigens. Between 1981 and 1996, 18 patients from eight families with unequivocal diagnosis of MPGN I or III had been described. The mode of inheritance appeared to be autosomal dominant or X-linked in four of these families. In 11 patients, including our 2, in whom HLA typing was performed, eight had the HLA-A2 antigen. Similarities and discrepancies regarding clinical and morphological features and outcomes were evident in these intrafamilial cases, suggesting either a similar genetic background or a multigenic origin of MPGN. The familial occurrence of the MPGN, highlighted by our report, supports the concept that genetically determined factors may be involved in the pathogenesis of the disease. Received: 2 September 1998 / Revised: 10 August 1999 / Accepted: 13 August 1999     

HELLP综合征二例诊治报告及文献复习

目的报道2例HELLP(hemolysis,elevated liver enzymes and low platelets)综合征患者诊治情况,并结合文献系统复习HELLP综合征的临床表现、早期预警标志、鉴别诊断、治疗及随访管理,以期提高对该病的认识和重视,避免误漏诊,早期获得正确合理的治疗。方法回顾分析2例HELLP综合征患者的临床表现、诊治经过,并总结复习相关文献。结果 2例妊娠早中期各项指标均未见异常。例1患者妊娠晚期出现血小板计数低下,肝酶升高,总胆红素异常,为本病诊断提供了线索;例2患者妊娠晚期出现高血压、蛋白尿,是HELLP综合征患者高危人群。2例均为终止妊娠后并发急性肾损伤,患者经过药物及肾脏替代(renal replacement therapy,RRT)治疗,例1患者至今未脱离腹膜透析治疗,例2患者病情完全缓解。结论 HELLP综合征患者,及时进行连续性血液净化(continuous blood purification,CBP)治疗对缓解HELLP综合征、降低病死率非常关键,HELLP综合征是一种罕见的以血栓性微血管病变为特征的临床综合征,其发病机制尚不清楚,早期预警措施非常有限,临床表现缺乏特异性,而且病情恶化迅速。     

肾上腺嗜酸细胞腺瘤一例报告并文献复习

目的报道1例肾上腺嗜酸细胞腺瘤患者的临床及病理特点。方法患者,男,37岁,体检发现左肾上腺肿物15 d入院。既往否认高血压、低血钾等病史。实验室检查血钾、血儿茶酚胺、血皮质醇、立卧位肾素血管紧张素、醛固酮、尿3-甲基-4-羟基苦杏仁酸、尿17-羟皮质类固醇、17-酮皮质类固醇均正常。B超及CT检查示左肾上腺区肿物,直径约7 cm。术前诊断为左肾上腺区肿物,行后腹腔镜下左肾上腺肿物切除术。结果术中发现肿物体积较大,包膜完整光滑,与周围组织无粘连,肿物表面血管较为丰富,术中碰触肿物,血压无波动,完整切除肿物。术后病理检查示肿瘤细胞嗜酸性,内部充满颗粒,诊断为肾上腺嗜酸细胞腺瘤。随访2个月无复发。结论肾上腺嗜酸细胞腺瘤是一种具有潜在恶性的良性肿瘤,可手术切除,但需密切随访。