Life-threatening hypokalaemia associated with ibuprofen-induced renal tubular acidosis

Renal tubular acidosis is an underreported complication of ibuprofen misuse, and can result in life-threatening hypokalaemia. We describe four patients who presented with profound hypokalaemia and muscle weakness associated with excessive ibuprofen ingestion. Ibuprofen cessation and supportive management resulted in complete biochemical resolution within a few days. These cases remind practitioners about potential complications of unmonitored use of over-the-counter analgesics, including those with potential for misuse due to their codeine content.     

Familial hypokalaemic periodic paralysis in papua nuiginians--a reappraisal

Studies of 11 Papua Nuiginians with recurrent muscular paralysis are presented. The clinical pattern, the geographical clustering, the lack of relation with dietary change, and the normal aldosterone levels in two patients support the view that it is a variant of familial hypokalaemic periodic paralysis.     

Type IV renal tubular acidosis associated with Alport's syndrome.

A case of hereditary nephritis with mild reduction of renal function associated with renal tubular acidosis type IV is described. The patient was admitted with life-threatening hyperkalaemia. To our knowledge, type IV renal tubular acidosis has not been reported previously in association with Alport''s syndrome in an adult patient.     

Purpura associated with hypergammaglobulinemia, renal tubular acidosis and osteomalacia.

Two patients with hyperglobulinemia associated with purpura were studied. One had features of Sjögren's syndrome, while the other appeared to have a primary condition -- "chronic benign purpura". Both patients also had renal tubular acidosis, osteomalacia and renal calculi, with disturbed calcium metabolism and acid-base balance. Autoantibodies were detected in the serum of both patients, and mononuclear cell infiltrates were noted in skin and kidney biopsies from both.     

Incomplete distal renal tubular acidosis with nephrocalcinosis

We report the case of a female patient with incomplete distal renal tubular acidosis with nephrocalcinosis. She was admitted to the hospital because of acute pyelonephritis. Imaging studies showed dual medullary nephrocalcinosis. Subsequent evaluations revealed hypokalemia, hypocalcemia, hypercalciuria, and hypocitraturia with normal acid-base status. A modified tubular acidification test with NH4Cl confirmed a defect of urine acidification, which is compatible with incomplete distal tubular acidosis. We treated our patient with potassium citrate, which corrects hypokalemia and prevents further deposition of calcium salts.     

合并低钾型周期性麻痹的手术患者基因突变筛查及麻醉处理

目的 通过筛查原发性低钾型周期性麻痹(hypokalaemic periodic paralysis,HOKPP)相关基因突变位点,探讨合并HOKPP患者的手术麻醉处理方法 .总结该类患者麻醉处理注意事项.方法 应用PCR-直接测序技术.对5例HOKPP患者进行候选基因CACNAlS和SCN4A筛查.并对该5例患者在静脉复合气管插管全麻下,分别施行上腹部手术、声带息肉摘除术及鼻息肉摘除术,麻醉诱导用眯达唑仑0.1mg/kg.丙泊酚2mg/kg,芬太尼4 μg/kg,罗库澳铵0.6mg/kg.麻醉维持间断静注芬太尼、罗库溴铵,持续微泵静注丙泊酚.结果 DNA测序结果 发现1例患者的CACNA1S基因外显子30上存在3716(G→A)杂合突变.导致氨基酸序列R1239H改变.该组HOKPP患者在围手术期生命体征稳定,手术过程顺利.未出现血电解质紊乱、心律失常、HOKPP发作、恶性高热等麻醉意外和并发症.术毕至气管拔管的时间为40～90min.结论 HOKPP存在CACNA1S基因突变,在手术过程中应注意HOKPP患者的血钾浓度变化,心电图监测,避免心律失常发生,同时注意麻醉方式和麻醉药物的选择,避免麻醉药物选择不当诱发HOKPP、恶性高热等麻醉意外和严重并发症.     

Idiopathic hypergammaglobulinaemia associated with nephrogenic diabetes insipidus and distal renal tubular acidosis.

Renal tubular dysfunction may be recognized in patients suffering from urinary light chain disease or non-myelomatous hypergammaglobulinaemia. We report a patient who has the combination of distal renal tubular acidosis and nephrogenic diabetes insipidus in association with hypergammaglobulinaemia due solely to increased IgG. We postulate that the abnormalities of distal nephron function resulted from cell-mediated immune damage.     

肾小管性酸中毒148例临床分析

目的探讨肾小管性酸中毒(RTA)的病因、分型、临床特点,以助早期诊断及治疗,减少严重并发症的发生。方法回顾性分析148例RTA患者的临床资料。结果病因原发性RTA占34.5%、继发性RTA占65.5%;临床分型Ⅰ型占77.0%、Ⅱ型占9.5%、Ⅲ型占11.5%,Ⅳ型占2.0%。女性RTA病因以原发性干燥综合征多见(27.5%);成人RTA最常见的临床表现为肌无力(75.5%),儿童以生长发育落后多见(57.1%)。结论 RTA临床表现多样,多种疾病可以继发RTA,临床医生要提高对RTA的认识;RTA伴生长发育落后的儿童使用生长激素追赶生长治疗值得重视。     

Hypokalaemic paralysis secondary to distal renal tubular acidosis as the presenting symptom of systemic lupus erythematosus

We report hypokalaemic quadriparesis presenting in a 43-year-old woman. Evaluation revealed hypokalaemic quadriparesis secondary to underlying distal renal tubular acidosis, also known as type 1 RTA. Four years after the diagnosis of RTA, the patient developed joint pain, and investigation revealed systemic lupus erythematosus with lupus nephritis. RTA is one of the very rare presentations of systemic lupus erythematosus. Thus, tubular dysfunction should be carefully assessed in patients with systemic lupus erythematosus. Similarly, patients with RTA should be evaluated for underlying lupus. Our patient was successfully treated with mycophenolate mofetil and steroids.     

甲状腺功能亢进并周期性麻痹的发病机制探讨

目的探讨甲状腺功能亢进(甲亢)并周期性麻痹的发病机制。方法15例男性甲亢并周期性麻痹的Graves病患者与15例男性甲亢非周期性麻痹的Graves病患者行临床对比分析，比较其一般情况，测定其FB、Fr4、s-TSH、空腹血糖(FBG)、餐后2h血糖(2h PBG)、空腹胰岛素(FINS)，计算胰岛素敏感指数(QUICKI)。结果甲亢并周期性麻痹组QUICKI指数明显低于甲亢非周期性麻痹组，空腹胰岛素水平明显高于甲亢非周期性麻痹组。结论胰岛素抵抗可能为甲亢患者发生周期性麻痹的原因之一。     

Medullary sponge kidney presenting with hypokalaemic paralysis.

Medullary sponge kidney associated with a defect in urine acidification is rare and usually of no clinical significance. We report a case presenting as hypokalaemic paralysis due to associated congenital distal renal tubular acidosis.     

干燥综合征合并肾小管性酸中毒

目的：结合实验检测及肾脏病理，了解干燥综合征中肾小管性酸中毒的发生情况。方法：对我科１９８８年１月～１９９７年１月收治的４０例干燥综合征（ＳＳ）肾脏损害患者进行常规、免疫学、肾小管功能及肾活检等检查。结果：２８／４０为远端肾小管性酸中毒（ｄＲＴＡ），１３／２８合并其他自身免疫性疾病，１８／２８合并低钾性麻痹，３／２８合并肾性尿崩症。６７．９％患者为高免疫球蛋白Ｇ血症，５７．１％和５０％患者抗ＳＳ－Ａ、抗ＳＳ－Ｂ抗体阳性。１１／２８行肾活检，发现肾小管间质中有大量淋巴细胞和浆细胞浸润，部分小管萎缩，间质纤维化。２０／２８例患者用激素治疗，４／２８例合并用环磷酰胺治疗。结论：ＳＳ合并ｄＲＴＡ常见，长期中、小剂量激素治疗可减少肾小管及间质损害，改善肾功能     

干燥综合征合并完全型肾小管性酸中毒

目的：为提高对干燥综合征（ＳＳ）并发完全型肾这性酸中毒（ＲＴＡ）的认识。方法：对２２例ＳＳ合并完全型ＲＴＡ患者的临床资料进行总结。然后对其中２１例原发ＳＳ（ＰＳＳ）合并完全型ＲＴＡ的患者与另外３７例无肾脏受累的ＰＳＳ进行对比分析。结果：列同时合并有提示近端肾小管受损的糖尿和尿酸尿外，均突出表现为１型肾小管性要到中毒。发作性软瘫、多饮多尿和骨痛／关节痛是最常见的症状；多数患者（６８．１８％，１５／２