干燥综合征、髓质海绵肾并远端肾小管酸中毒1例

作者收治 1例干燥综合征、髓质海绵肾并远端肾小管酸中毒的患者 ,经查阅文献国内外均未见报道 ,现报道如下。1　病例介绍　　患者女 ,5 2岁。因长期低热、右上腹隐痛不适 2年 ,间歇肉眼血尿 10个月为主诉入院。患者于 2年前突发高热伴双侧腮腺肿大 ,持续 1周后高热退 ,遗留长期低热 ,腮腺肿大消失 ,觉上腹部闷痛不适 ,皮肤黄染 ,四肢无力 ,曾诊为“肝炎” ,病情反复。 10个月前解肉眼血尿 ,尿红细胞 ( ) ,不伴尿路刺激征 ,此后反复肉眼或镜下血尿。外院Ｂ超示“双肾泥砂样结石” ,ＣＴ示“髓质海绵肾可能”。患者发病以来 ,曾有两次腮腺…     

原发性干燥综合征并发肾小管酸中毒1例

患者，女，40岁，因反复肢体无力1个月，再发3d于2010年6月10日至中心市陈星海医院就诊。患者1个月前在高温室外工作后出现肢体无力，当时无力行走，双上肢无力抬高，曾在当地医院就诊，     

误诊干燥综合征合并肾小管性酸中毒二例

干燥综合征(SS)病人的22～30%合并远端肾小管性酸中毒(d—RTA),但由于其临床表现不典型,易被漏诊、误诊。笔者1991年遇到2例被误诊为“周期性麻痹”5年多,现报告如下:例1:女,20岁。以烦渴多饮(4000～5000ml/d)、多尿(3000～5000ml/d)、发作性四肢软瘫5年,腰骶部疼痛、乏力1年入院。在外地医院曾诊断为“周期性麻痹”、“尿崩症”。以往     

原发性干燥综合征肾小管酸中毒合并低钾血症的护理体会

总结了21例原发性干燥综合征肾小管酸中毒合并低钾血症的护理措施与经验。主要护理措施包括心理护理、病情观察、补钾治疗与护理、饮食指导、基础护理、唇腺活检的护理及复诊指导等。认为发生原发性干燥综合征肾小管酸中毒合并低钾血症时快速准确诊断,并给予及时有效的治疗护理是至关重要的。     

干燥综合征合并肾小管酸中毒及神经系统损害

1　病例资料女,38岁。因反复四肢乏力10年,加重伴双下肢酸痛2个月入院。患者于10年前无明显诱因出现四肢无力,2天后发展为软瘫,当地医院查血钾明显减低,伴高氯性代谢性酸中毒,诊断为肾小管酸中毒,予枸橼酸钾口服,3天后血钾恢复正常,症状缓解。此后症状反复发作,每年1～2次,每次     

1例干燥综合征并发肾小管酸中毒患儿的护理

干燥综合征是一种以侵犯泪腺、唾液腺等外分泌腺为主的慢性自身免疫性疾病,又称为自身免疫性外分泌腺体病。主要表现为干燥性角膜、结膜炎、口腔干燥症或伴发类风湿性关节炎等其他风湿性疾病,它可累及全身多个脏器和器官,其中肾脏是易受累的器官之一。肾损害主要累及远端肾小管,表现为因Ⅰ     

1例儿童原发性干燥综合征合并肾小管酸中毒的护理

目的 探讨儿童原发性干燥综合征合并肾小管酸中毒的护理.方法 对1例原发性干燥综合征合并肾小管酸中毒患儿进行病情观察,做好症状护理、饮食护理、用药指导、心理护理及出院指导.结果 患儿临床症状缓解,好转出院,经过6个月的随访,患儿病情稳定,未发生其他并发症.结论 儿童原发性干燥综合征合并肾小管酸中毒除了要及时诊断和正规治疗外,全面、精心的护理也是其重点,是延缓疾病发展、促进疾病康复的重要手段.     

以肾小管酸中毒为首发表现的干燥综合征一例

1 病历摘要 女,29岁.因"反复全身乏力1年,腰背部持续性钝痛6个月余"入院.1年前某清晨无明显诱因醒来后觉全身明显乏力,双下肢为甚,不能站立,无呼吸困难等症状,遂至当地医院检查发现血钾低至2.8 mmol/L,给予静脉补钾后乏力明显好转.首次发作后半年内未再有类似发作.     

以肾小管酸中毒为首要表现的干燥综合征1例

干燥综合征为一种自身免疫性疾病 ,约有半数可以合并有肾脏损害[1] ,但以肾小管酸中毒为首要表现的病例较少见 ,近日我院收治了这样 1例患者 ,现报道如下。患者 ,女性 ,17岁 ,因“间断恶心、双下肢痛、全身乏力 1个月 ,加重 2天”于 2 0 0 0年 10月 12日住院。入院前 1个月无明显诱因出现恶心、呕吐、反酸、呃逆、双下肢疼痛、全身乏力 ,吞咽困难 ,在外院查血钾 1.98ｍｍｏｌ/Ｌ ,ＣＯ2 13 .5ｍｍｏｌ/Ｌ ,血淀粉酶 478ＩＵ/Ｌ ,诊为“胰腺炎 ?” ,给予解痉、补钾、纠酸治疗 2周后好转 ,自行中断治疗。入院前 2天 ,无明显诱因 ,上述症状再次…     

原发性干燥综合征并肾小管酸中毒、病理性骨折误诊1例分析

原发性干燥综合征（pSS）是一种以外分泌腺高度淋巴细胞浸润为特征的自身免疫性疾病，除外分泌腺受累所致口干、眼干外，常伴其他系统损害，其中肾脏损害以肾小管酸化功能障碍较为常见，重可出现低钾性麻痹、肾性骨病。由于患极少以口干、眼干为主症就诊，临床诊治中极易误诊。本就长期误诊的pSS合并RTA、反复低钾性麻痹、肾性骨病、病理性骨折1例分析如下。     

Endemic Primary Distal Renal Tubular Acidosis in Thailand

Within a period of 3 years, 103 patients with primary type Idistal renal tubular acidosis were reported from two hospitalsin the northeast of Thailand, with cases peaking in the summer.The patients' age ranged from 18 to 76 with a mean and modeof 39 and 47 years, respectively. The female to male ratio was3.3:1. All patients presented with generalized muscle weaknessand bone pain and nocturia were frequent manifestations. Hypokalaemiaand hyperchloraemic metabolic acidosis were constant findingsand were more pronounced in patients with roentgenographic evidenceof nephrocalcinosis and osteomalacia. Urinary citrate was extremelylow in most of the patients. Nephrocalcinosis and/or renal stoneswere present in 27.2% of the patients and osteomalacia was foundin 23.3%. All patients were Lao-Thai. This is the first reportof a large group of patients with distal renal tubular acidosis,endemic within a particular geographical region.     

Haematological Manifestations of Primary Sjogren's Syndrome: A Clinicopathological Study

Clinically significant cytopenias are thought to be uncommonin primary Sjögren's syndrome: only a few cases have beenreported in the literature. Over a 3-year period we identifiedhaematological abnormalities in 11 of 27 patients with Sjögren'ssyndrome. Six patients had a positive direct antiglobulin test,including one patient with all the features of autoimmune haemolyticanaemia and two others with some features of this condition.Four patients had immune thrombocytopenia and two patients hadmyelodysplastic syndrome. Neutropenia was noted in two patients,one patient had aplastic anaemia and one had pure red cell aplasia.Haematological disorders were found to be common in patientswith Sjögren's syndrome (40 per cent). Accordingly, wesuggest that patients with immune cytopenia(s) should be screenedfor Sjögren's syndrome using sensitive assays for anti-SS.Aand anti-SS.B antibodies, and that patients with Sjögren'ssyndrome should be periodically monitored, with a full bloodcount to rule out any haematological abnormality.     

POEMS综合征合并肾损害一例

1 病例资料 男,53岁.因间断双小腿麻木2年,腹胀10 d,加重4 d入院.2年前不明原因出现双小腿麻木不适,当地医院诊断为周围神经炎,间断应用泼尼松(10 mg/d)1年余,症状时轻时重,摄腰椎X线片及CT检查示腰椎骨质增生,腰椎间盘突出.     

原发性干燥综合征疲劳的机制与治疗进展

原发性干燥综合征是一种主要侵犯泪腺、唾液腺等外分泌腺的慢性系统性自身免疫性疾病,除了表现为口干、眼干外,通常还伴有明显的疲劳症状,严重影响患者的生活质量.疲劳常与抑郁、疼痛、嗜睡相互影响.肾小管性酸中毒、热休克蛋白和遗传变异是疲劳较为明确的机制,目前还缺乏有效的药物治疗,有氧运动能一定程度缓解疲劳.     

原发性肾病综合征合并甲状腺功能减退症的治疗观察

目的 :探讨原发性肾病综合征 (primarynephroticsyndrome ,PNS)合并甲状腺功能减退症 (甲减 )的发生机制 ,以及应用左甲状腺素治疗的效果。方法 :采用放射免疫法测定 5 0例健康体检者 (正常对照组 )及 5 0例PNS合并甲减 (左甲状腺素组与一般治疗组 )治疗前后甲状腺功能 (TT3 、TT4、FT3 、FT4及TSH)水平 ;观察部分难治者在常规治疗基础上加用左甲状腺素的治疗效果。结果 :PNS综合征合并甲减者TT3 、TT4、FT3 、FT4水平均有不同程度的降低 ,且TT3 及FT3 下降较为明显 (P <0 .0 1) ,治疗后以上指标有所回升 ,TT3 、TT4、FT3 水平与治疗前比较 ,升高较明显 (P <0 .0 5 ) ;左甲状腺素组缓解率明显高于对照组 (P <0 .0 5 ) ,完全缓解时间也明显短于对照组。结论 :PNS综合征合并甲减者在常规治疗基础上给予小剂量左甲状腺素不仅有利于甲状腺功能恢复 ,且有利于尿蛋白转阴 ,缩短病程     

肾病综合征并发急性肾功能衰竭和黄色肉芽肿性肾盂肾炎1例报告

1　病例简介患者男 ,5 0岁 ,农民。因全身水肿 ,尿少 10ｄ入院。以往无肾病、糖尿病史及泌尿系统器械检查史。入院查体 :Ｔ36℃ ,ＢＰ130 / 80ｍｍＨｇ ,全身重度水肿 ,心脏 (一 ) ,双肺底呼吸音低 ,腹膨隆 ,肝脾未触及 ,腹水征 (+) ,双肋腰点有压痛。辅助检查 ;血Ｈｂ119ｇ/Ｌ ,ＷＢＣ 7.9× 10 9/Ｌ ,Ｎ75 .1% ,Ｌ2 4.9% ,Ｔｐ49.0ｇ/Ｌ ,ＡＬＢ2 1.0 ｇ/Ｌ ,ＣＨＯ7.5ｍｍｏｌ/Ｌ ,ＴＧ 3.12ｍｍｏｌ/Ｌ ,Ｃｒ132ｍｏｌ/Ｌ ,ＢＵＮ2 8.8ｍｍｏｌ/Ｌ ,ＨＣＯ3 2 3.2ｍｍｏｌ/Ｌ ,ＦＤＰ <10 .0 μｇ/ｍｌ,免疫学检查 (一 ) ,尿Ｒｔ:Ｐｒｏ…     

Sweet综合征合并骨髓增生异常综合征伴肺部浸润一例报道

病人,女,52岁,1998年7月在无明显诱因情况下右侧面部出现米粒大小皮疹,红色,高出皮面,自觉略痛,并逐步发展至双侧面部、耳部,皮疹在数日内逐渐增大,予外用药半月后渐消退,但不久皮疹复发并伴发热(37～38℃)及咳嗽,胸片示肺部感染.经抗生素治疗体温略下降,但皮疹不退,且增大,至溃破结痂.     

老年骨质疏松症合并桡骨远端骨折的治疗

应用中西医结合疗法治疗39例老年骨质疏松症患者,结果治愈率达100%。     

A Microperfusion Study of Phsophate Reabsorption by the Rat Proximal Renal Tubule EFFECT OF PARATHYROID HORMONE

To study the mechanism of phsophate reabsorption by the proximal tubule and the effect of parathyroid hormone (PTH), microperfusion experiments were carried out in rats. Segments of proximal tubule isolated by oil blocks were perfused in vivo with one of three solutions, each containing 152 meq/liter Na(+) and 2 mmol/liter phosphate, but otherwise differing in composition. The pH of solution 1 was 6.05-6.63, indicating that 60-85% of the phosphate was in the form of H(2)PO(4) (-). The pH of solution 2 was 7.56-7.85, and 85-92% of the phosphate was in the form of HPO(4) (=). Solution 3 contained HCO(3) (-) and glucose and had a pH of 7.50-7.65. When the proximal tubules were perfused with solution 1, the (32)P concentration in the collected perfusate was found to be consistently lower than in the initial perfusion solution. In sharp contrast, when the tubules were perfused with solutions 2 or 3, (32)P concentration usually rose above that in the initial solution. Water (and persumably Na(+)) reabsorption, as measured with [(3)H]inulin, was the same with the acid and alkaline solutions. Administration of partially purified PTH clearly prevented the fall in phosphate concentration with the acid solution, but had a less discernible effect on phosphate reabsorption with the two alkaline solutions. Measurements of pH within the perfused segments with antimony microelectrodes demonstrated that PTH enhanced alkalinization of the acid perfusion solution. The findings are consistent with the view that H(2)PO(4) (-) is reabsorbed preferentially over HPO(4) (=). This can be attributed to either an active transport mechanism for H(2)PO(4) (-) or selective membrane permeability to this anion. PTH appears to either inhibit an active transport process for H(2)PO(4) (-), or to interfere with passive diffusion of phosphate by alkalinizing the tubular lumen.     

生脉散加减联合养阴润喉茶治疗原发性干燥综合征

目的:观察生脉散加减联合养阴润喉茶治疗原发性干燥综合征的临床疗效。方法:将60例原发性干燥综合征患者随机分为治疗组和对照组各30例。对照组予白芍总苷胶囊口服;治疗组在对照组用药基础上,予生脉散加减联合养阴润喉茶口服。2组均治疗12周后观察并比较临床疗效、中医证候评分、免疫球蛋白G(Immunoglobulin G,IgG)、免疫球蛋白A(Immunoglobulin A,IgA)、免疫球蛋白M(Immunoglobulin M,IgM)、超敏C反应蛋白(Hypersensitive C-rective protein,CRP)、红细胞沉降率(Erythrocyte sedimentation rate,ESR)、干燥三项(角膜荧光染色评分、泪膜破裂时间测定、静态唾液流率)的变化情况。结果:治疗组临床有效率为90%,高于对照组66.67%,差异有统计学意义。治疗后2组中医症状积分均有下降,差异有统计学意义,相较于对照组,治疗组两目干涩、咽干口燥、乏力自汗、五心烦热、阴道干涩或大便干、舌红瘦干及脉细涩的症状积分下降更为明显,差异有统计学意义。治疗后2组IgG、CRP、ESR指标均较治疗前下降,相较于对照组,治疗组IgG、CRP指标改善更为明显,差异有统计学意义。治疗后2组泪膜破裂时间、静态唾液流率较前上升,角膜荧光染色评分较前下降;相较于对照组,治疗组泪膜破裂时间、静态唾液流率改善更为明显,差异有统计学意义,角膜荧光染色评分差异无统计学意义。结论:生脉散加减联合养阴润喉茶治疗气阴两虚型原发性干燥综合征临床疗效显著,明显缓解患者临床症状,一定程度上改善实验室指标,安全性高。