Primary hyperoxaluria diagnosed after kidney transplant: A review of the literature and case report of aggressive renal replacement therapy and lumasiran to prevent allograft loss

Primary hyperoxaluria type 1 is a rare inherited disorder caused by abnormal liver glyoxalate metabolism leading to overproduction of oxalate, progressive kidney disease, and systemic oxalosis. While the disorder typically presents with nephrocalcinosis, recurrent nephrolithiasis, and/or early chronic kidney disease, the diagnosis is occasionally missed until it recurs after kidney transplant. Allograft outcomes in these cases are typically very poor, often with early graft loss. Here we present the case of a child diagnosed with primary hyperoxaluria type 1 after kidney transplant who was able to maintain kidney function, thanks to aggressive renal replacement therapy as well as initiation of a new targeted therapy for this disease. This case highlights the importance of having a high index of suspicion for primary hyperoxaluria in patients with chronic kidney disease and nephrocalcinosis/nephrolithiasis or with end stage kidney disease of uncertain etiology, as initiating therapies early on may prevent poor outcomes.     

Tension band plates have greater risks of complications in temporary epiphysiodesis

PurposeThis study aimed to compare the efficacy of decreasing leg-length discrepancy (LLD) and postoperative complications between tension band plates (TBP) and percutaneous transphyseal screws (PETS).MethodsThis retrospective study reviewed LLD patients who underwent temporary epiphysiodesis at the distal femur and/or proximal tibia from 2010 to 2017 (minimum two years follow-up). Efficacy of decreasing LLD was assessed one and two years postoperatively. Complications were classified with the modified Clavien-Dindo-Sink complication classification system. Knee deformities were assessed by percentile and zone of mechanical axis across the tibial plateau.ResultsIn total, 53 patients (25 boys, 28 girls) underwent temporary epiphysiodesis (mean age, 11.4 years). The efficacy of decreasing LLD at two years between the TBP (n = 38) and PETS (n = 15) groups was comparable. Seven grade III complications were recorded in six TBP patients and in one PETS patient who underwent revision surgeries for knee deformities and physis impingement. Four grade I and two grade II complications occurred in the TBP group. The mechanical axis of the leg shifted laterally in the PETS group and medially in the TBP groups (+7.1 percentile versus -4.2 percentile; p < 0.05). Shifting of the mechanical axis by two zones was noted medially in four TBP patients and laterally in two PETS patients.ConclusionMore implant-related complications and revision surgeries for angular deformities were associated with TBP. A tendency of varus and valgus deformity after epiphysiodesis using TBP and PETS was observed, respectively. Patients and families should be informed of the risks and regular postoperative follow-up is recommended.Level of evidenceLevel III     

冷冻浓缩腹水回输在治疗顽固性肝硬化腹水中的应用

目的 探讨冷冻浓缩腹水回输治疗顽固性肝硬化腹水的疗效。方法 采用透析机对32例顽固性肝硬化腹水患者的腹水进行超滤浓缩,置-18℃环境中冷冻2～3天,37℃温育溶解后静脉回输。结果 冷冻浓缩腹水回输前后,血清总蛋白为(59.1±5.6)g/L和(66.3±4.7)g/L,白/球比值为0.8±0.3和1.1±0.5,腹水总蛋白为(15.2±6.8)g/L和(55.6±8.4)g/L;腹围为(91.3±9.5)cm和(80.8±9.2)cm,腹压为(3.5±0.43)kPa和(2.4±0.32)kPa,24 h尿量为(615.0±185.5)ml和(1 240.0±255.0)ml;治疗前后相比,差异有高度显著性(P<0.01)。血清钾为(3.5±1.1)mmol/L和(4.0±1.3)mmol/L,腹水钾(3.6±0.9)mmol/L和(4.1±1.0)mmol/L;血清钠为(122.2±4.0)mmol/L和(126.5±4.3)mmol/L,腹水钠为(120.8±4.5)mmol/L和(123.3±4.7)mmol/L;血清氯为(90.3±6.2)mmol/L和(95.1±5.7)mmol/L,腹水氯为(92.0±5.9)mmol/L和(96.4±50.5)mmol/L;治疗前后相比,血清钠、腹水钠、腹水钾、血清氯、腹水氯差异均有显著性(P<0.05,P<0.01),血清钾差异无显著性(P>0.05)。冷冻浓缩腹水回输后,除2例出现一过性发热外,未发生低血压、肝昏迷、消化道出血现象,未出现蛋白凝集、堵塞管腔;未出现电解质紊乱现象。结论 冷冻浓缩腹水回输可大量回收腹水     

顺铂术中腹腔化疗对妇科恶性肿瘤患者术后的近期影响

目的 探讨顺铂术中腹腔化疗治疗妇科恶性肿瘤的可行性。方法 60例妇科恶性肿瘤患者术中腹腔置入100mg顺铂稀释液,观察其对患者术后胃肠功能、体温、切口愈合、腹水控制及术后并发症的影响。结果 术后24h内呕吐发生率增高,但使用止吐剂及水化效果好,尿量达标;胃肠道功能按期恢复;术后发热发生率低;服务部切口无一例丙级愈合;无并发症发生。14例伴发大量腹水患者11例得到控制。肝肾功能未发现损害。结论 术中腹腔化疗比较安全,切实可行。     

改良式剖宫产术50例临床分析

探讨改良式剖宫产术的临床价值。方法：采用香港周杰医师１９９１年创立的一套改式剖宫产术对５０例产妇进行手术并与同期腹部横切口传统剖宫产术进行比较。结果：改良式剖宫产术所用时间短,术中出血小,术后排气暗暗科痛轻、产褥病发生率低。住院时间短,与对照组比较有明显差异。     

脾切除手术指征及近期疗效分析

【目的】分析总结脾切除手术的近期疗效以提高其安全性、降低死亡率 ,减少并发症并指导正确掌握切脾适应征。【方法】统计 430例脾切除手术病人的临床资料 ,包括切脾指征、手术种类、术后并发症和手术死亡率。【结果】切脾指征中的原发疾病以肝为最多 192例 (4 4% ) ,尤其是门脉高压性脾亢 16 4例 (38% ) ;430例患者共行 76 8例次不同类型的手术 ,除切脾外 ,以分流断流为最多 75例次 (2 3% ) ;术后发生 111例次并发症 (2 6 % ) ,以感染为最多 (12 % ) ,术后死亡 12人 ,术后受治病死率 2 %。【结论】认识脾的免疫功能 ,正确掌握适应征和加强围术期处理是提高脾切除手术安全性的关键。     

新生儿败血症107例临床分析

目的：对新生儿败血症进行早期诊断,减少并发症,降低病死率。方法：对我院近10年来107例血培养阳性患儿的临床资料进行分析。结果：新生儿败血症临床表现无特殊性,并发症多,未梢血象、C反应蛋白均不能作为诊断依据。血培养仍是确诊的主要手段,但阳性率不高。病原菌以金黄色葡萄球菌（24．29％）为主,四联球菌（21．50％）为次,机会菌感染占50．46％。病死率以鼠伤寒沙门氏菌、大肠杆菌所致为高,分别是43．75％、33．33％。目前病原菌普遍敏感的抗生素有头孢唑啉、头孢哌酮、丁胺卡那霉素、万古霉素。结论：对拟诊病例开展L型血培养,以提高诊断率。早期应用敏感抗素控制感染,积极防治并发症,减少病死率。     

准分子激光角膜原位磨镶术治疗高度近视疗效分析

目的:观察准分子激光角膜原位磨镶术(LASIK)治疗高度近视的疗效.方法:采用LASIK治疗高度近视眼(平均-11.2±1.86D)195只,术后第1,6,12个月随访观察.结果:术后第1,6,12个月裸眼视力达到0.5和1.0者分别为84.1%,79.0%,74.9%和61.0%,57.9%,55.9%;术后第1,6,12个月屈光度在预测矫正度±0.50D和±1.0D者分别是68.7%,63.1%,61.5%和79.0%,75.9%和74.9%.结论:LASIK治疗高度近视有较好的效果,手术中并发症与术者手术技巧有关.     

妊娠期糖尿病对妊娠结局的影响

目的研究GDM对母婴的影响。方法对112例GDM孕妇和112例健康孕妇的妊娠结局进行分析。采用11配对病例—对照研究,配对的条件是孕妇年龄相近(±2),同年同月同医院分娩。结果GDM孕妇手术分娩率、早产率、妊娠高血压综合征和巨大儿发生率都较对照组高。GDM的及早诊断和及时治疗可使巨大儿发生率降低。与新生儿病率有关的因素是GDM的严重程度、血糖控制情况及巨大儿。结论早期诊断GDM及控制血糖是减少妇婴并发症的关键。