An unusual cause of acute renal failure in a newborn: hydrometrocolpos

Here we report on an unusual cause of acute renal failure in a newborn: hydrometrocolpos due to imperforate hymen. Hymenotomy resolved all of the laboratory and clinical abnormalities of the patient and the baby was sent home healthy.     

Lesch-Nyhan syndrome presenting as acute renal failure secondary to obstructive uropathy

Lesch-Nyhan syndrome is a rare genetic disorder characterized by mental retardation, self-mutilation, choreoathetosis, and hyperuricemia. The disease is caused by a mutation in the hypoxanthine-guanine phosphoribosyltransferase gene and is transmitted as a sex-linked recessive disorder. Since hyperuricemia is the primary metabolic problem caused by a hypoxanthine-guanine phosphoribosyltransferase mutation, urologic evaluation and treatment is often necessary for children with this disease. We report a 3-year-old boy who presented with anuric renal failure secondary to bilateral obstructing uric acid calculi. The evaluation of T lymphocytes revealed a hypoxanthine-guanine phosphoribosyltransferase mutation consistent with Lesch-Nyhan syndrome. The diagnosis and urologic management of this disorder is discussed.     

Orthostatic acute renal failure in a renal transplant

Complications due to ureteric obstruction are an occasional cause for renal transplant dysfunction. Here we report an unusual case of orthostatic renal failure in a renal transplant recipient. Our patient had the previously reported predisposing risk factors including: female sex, obesity, and lax abdominal musculature. It is important to recognize this unusual complication of renal transplantation early in order to preserve long-term graft function. Received: 23 December 1996 Received after revision: 6 May 1997 Accepted: 13 May 1997     

Partial hypoxanthine-guanine phosphoribosyltransferase deficiency presenting as acute renal failure

Hyperuricemia and secondary urate nephropathy are uncommon in the paediatric setting outside of tumour lysis syndrome. We describe the case of a 12-year-old boy who presented at 3 years of age with acute renal failure. The cause of this remained unknown until the development of uric acid renal calculi 9 years later. This, and the availability of the previously unknown family history, provided the subsequent diagnosis of partial hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency. Detailed family history is important for early detection of this heterogeneous group of disorders. Early treatment may minimise long-term renal morbidity and mortality from renal insufficiency.     

Acute renal failure due to bilateral xanthine urolithiasis in a boy with Lesch-Nyhan syndrome

Lesch-Nyhan syndrome is a very rare X-linked recessive disorder caused by mutation in the gene encoding enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). A complete deficiency of HPRT leads to severe purine overproduction and to uric acid renal lithiasis as a consequence. This may be effectively prevented by administration of allopurinol; however, its overdosage may result in xanthinuria and xanthine urolithiasis. We report on a 9-year-old boy with Lesch-Nyhan syndrome who developed acute renal failure due to bilateral staghorn xanthine urolithiasis resulting from long-term treatment with excessive doses of allopurinol. To the best of our knowledge, the presented case is the first one in the literature.     

Neonatal transient renal failure with renal medullary hyperechogenicity: clinical and laboratory features

Sonographic findings of renal medullary hyperechogenicity have been observed in the neonate in association with severe perinatal renal injury, kidney malformations or nephrocalcinosis, and, rarely, in newborn infants with transient renal failure. The aim of the study was to describe the entity of neonatal transient renal failure with renal medullary hyperechogenicity (NTRFMH). We studied nine term neonates, born between August 1999 and February 2004 in our institution (0.1% of the live born infants), who developed transient renal dysfunction after birth, and in whom renal sonograms showed bilateral medullary hyperechogenicity. Seven of the infants (78%) had anuria until 30–45 hours of age, and two (22%) had oliguria. Peak serum creatinine levels ranged between 0.61 and 1.62 mg/dL (mean: 1.09±0.27 mg/dL) at 2–3 days of life. Additional findings included proteinuria in nine infants (100%), uric acid crystalluria in seven (78%), hyperuricemia in four (44%), and hypertension in one (11%). Hyperuricosuria was demonstrated in one out of the seven patients in whom this parameter was determined. Urinary excretion rates of calcium, phosphorus and oxalic acid were normal, as were urinary levels of amino acids and organic acids. Full clinical recovery accompanied by normalization of all laboratory parameters was observed in all infants by 4–6 days of life. Subsequent follow-up showed normal renal function, no urinary abnormalities, and normal renal sonograms in all infants. Our summary of the nine infants with NTRFMH reported on here and a review of 19 cases of this condition reported in the literature reveal a not-so-rare entity of unclear etiology, but excellent prognosis. Physicians caring for neonates should be aware of this benign and transient condition.     

Purine enzyme defects as a cause of acute renal failure in childhood

Acute renal failure (ARF) is not listed as a usual form of presentation in hypoxanthineguanine phosphoribosyltransferase deficiency, despite the gross uric acid overproduction in the defect. We found that a third of such patients may present in ARF when the urinary uric acid/creatinine ratio may be normal, not raised, and the defect may be suspected from the disproportionate increase in plasma uric acid. This is important in view of the potential confusion of uric acid with 2,8-dihydroxyadenine, the even more insoluble purine excreted in the other salvage enzyme disorder, adenine phosphoribosyltransferase deficiency. In that disorder, presentation in ARF is well recognised, the uric acid/creatinine ratio is also normal, but plasma urate is not raised. Our combined experience in these two disorders underlines the importance of early recognition and treatment with carefully adjusted doses of allopurinol, which may reverse or postpone renal failure.     

Risk factors of acute renal injury in patients with acute left heart failure

Objectives To investigate the risk factors of acute renal injury (acute kidney injury) in patients with acute left heart failure. Methods Clinical data of 188 patients with acute left heart failure who were admitted to our hospital were retrospectively analyzed. Logistic regression analysis was used to assess the risk factors for AKI. Results Among 188 patients with acute left heart failure, incidence of acute kidney injury was 33.51%. Univariate and Multivariable logistic regression analyses showed that the independent predictors of acute kidney injury were lower baseline eGFR (OR=4.294, P＜0.001) and anemia (OR=3.573, P=0.006). Conclusions The incidence of acute left heart failure complicated with AKI was high. Basic state of renal function and anemia were the independent risk factors for AKI.     

Pheochromocytoma presenting with rhabdomyolysis and acute renal failure: a case report

Rhabdomyolysis ranges from an asymptomatic illness with elevated creatine kinase levels to a life-threatening condition associated with extreme elevations in creatine kinase, electrolyte imbalances, acute renal failure, and disseminated intravascular coagulation. The most common causes are crush injury, overexertion, alcohol abuse, certain medicines, and toxic substances. A number of electrolyte abnormalities and endocrinopathies, including hypothyroidism, thyrotoxicosis, diabetic ketoacidosis, nonketotic hyperosmolar state, and hyperaldosteronism, cause rhabdomyolysis. Rhabdomyolysis and acute renal failure are unusual manifestations of pheochromocytoma. There are a few case reports with pheochromocytoma presenting rhabdomyolysis and acute renal failure. Herein, we report a case with pheochromocytoma crisis presenting with rhabdomyolysis and acute renal failure.     

320例急性肾功能衰竭患者的临床分析

目的 探讨急性肾功能衰竭（ARF）住院患者的病因、预后及影响预后的因素。 方法 回顾性研究我院2003年12月至2006年12月期间急性肾功能衰竭患者的临床资料。 结果 观察期间住院患者共108 744例次，其中ARF患者320例，老年ARF患者135例，占42.2%。ARF主要病因为感染、心力衰竭和药物。ARF患者总体病死率为31.9%，老年人病死率较高。Logistic回归分析显示心力衰竭、呼吸衰竭及恶性肿瘤是与预后相关的危险因素。接受肾替代治疗组患者病死率低于保守治疗组（23.2％比35.6％，P < 0.05）。 结论 住院患者中ARF的发生率、病死率高，替代治疗组预后较好。     

Staghorn calculus in renal allograft presenting as acute renal failure

Background: Urolithiasis is a rare complication in renal transplant recipients. We report a case of a staghorn calculus occurring in renal allograft, presenting as anuric renal failure with Gram-negative sepsis. Methods and Results: A 48-year-old Caucasian female, with end-stage renal disease due to autosomal dominant polycystic kidney disease, underwent cadaveric renal transplantation in 1986. Sixteen years after transplant, she presented with Gram-negative sepsis with Proteus mirabilis and acute anuric renal failure in the allograft. After undergoing an emergency nephrostomy and treatment of sepsis, a staghorn calculus was subsequently removed by percutaneous nephrolithotomy. Based on the stone analysis and history of urinary tract infections with urease splitting bacteria, the calculus was thought to be infection-induced. Conclusion: Although a rare complication, urolithiasis in an allograft can be associated with significant morbidity. Immediate recognition is critical to restore renal allograft function and to treat associated serious infection in an immunocompromised patient.     

急性肾功能衰竭犬血液流变学特性的动态变化

对７只比格（Ｂｅａｇｌｅ）犬注射甘油前及注射甘油后５个不同时间的血液流变学进行动态研究。结果发现：在注射甘油后０．５小时即出现高、低切变率１２５ｓ－１及１．２４３５ｓ－１条件下的全血粘度增高，并维持至注甘油后２４小时（１．８±１．０ｍＰａ·ｓ，Ｐ＜０．００１；２０．１±８．０ｍＰａ·ｓ，Ｐ＜０．００１）；血浆粘度在注射甘油后１小时开始增高，第２４小时恢复；红细胞聚集性在注射甘油后０．５小时开始增高且维持至第２４小时；红细胞压积在注射甘油后１小时即增高并维持第２４小时，全血粘度增高与血浆粘度及红细胞压积呈显著正相关；血浆粘度与红细胞压积呈明显正相关。认为在急性肾功能衰竭发病过程中自始至终存在血液流变学特性变化，是急性肾功能衰竭发生、发展及维持的重要原因     

Novel HGPRT 293 A>G point mutation presenting as neonatal acute renal failure

We report on a rare case of hypoxanthine guanine phosphoribosyl transferase (HGPRT) deficiency that presented in the newborn period with acute renal failure (ARF). The clinical diagnosis was made on the basis of non-oliguric ARF and evidence of crystal nephropathy on renal biopsy. HGPRT deficiency was eventually confirmed by enzymatic and genetic testing, showing a novel point mutation, 293 A>G. Immediate treatment consisted of peritoneal dialysis with, initially, lactate- then bicarbonate-buffered 1.36% glucose solution together with oral administration of allopurinol. Follow-up after more than 4 years continued to show hyper-echogenic kidneys with almost normal renal glomerular function. There continues to be no neurobehavioural abnormalities.     

Cholesterol embolic disease presenting as renal failure with acute pancreatitis

Renal failure and acute pancreatitis developed in a 63-year-old man after 2 angiography procedures and iliac artery surgery. The results of the renal biopsy showed no specific abnormalities and the cause of the pancreatitis could not be determined. The patient was treated with hemodialysis and conservative management for pancreatitis, however he died due to sepsis approximately 50 days after diagnosis. The autopsy revealed cholesterol crystals in several organs including the kidneys, pancreas, and spleen. Therefore, systemic cholesterol emboli may have contributed to the development of renal failure and acute pancreatitis in this case. Cholesterol crystals in the pancreas have frequently been observed at autopsy in patients with cholesterol emboli. The clinical development of acute pancreatitis, however, is quite rare in these cases. The present case is reported to increase awareness of the possible clinical presentations of systemic cholesterol embolic disease in an effort to increase the correct diagnosis of this condition.     

输尿管镜在婴儿肾结石合并急性肾后性肾功能衰竭手术治疗中的应用

目的 探讨输尿管镜在婴儿肾结石合并急性肾后性肾功能衰竭手术中的临床应用.方法双肾结石合并急性肾后性肾功能衰竭患儿13例.男11例,女2例.年龄5～12个月,平均9个月.其中双肾结石并肾积水5例,一侧肾结石合并对侧输尿管结石嵌顿3例,双侧输尿管结石嵌顿3例,双肾结石合并肾积水伴尿道结石2例.无尿1～3 d者11例,少尿5～10 d者2例.实验室检查血BUN及SCr均高于正常,高尿酸血症8例,高血钾症9例.13例尿pH均<6.5,平均5.5.KUB检查均未见阳性结石.B超检查13例均有不同程度肾积水,9例肾积水直径>2.5 cm.CT检查结石直径0.5～1.5 cm.均未行透析治疗.13例均行输尿管镜下溶石、碎石、置入双J管内引流术治疗. 结果 13例患儿术后24 h内出现多尿,出现多尿期平均时间为术后12 h,多尿期持续24～72 h,24 h尿量约800～2500 ml,术后48～96 h尿量逐渐恢复正常.术后1～5 d BUN及SCr均恢复正常,8例高尿酸血症患儿术后1～7 d血尿酸恢复正常,9例高钾血症患儿术后1～3 d血钾恢复正常.13例患儿留置双J管2～4周,复查B超,结石排净11例、结石变小2例. 结论 对肾结石合并急性肾后性肾功能衰竭要儿应用输尿管镜下腔内手术治疗,采用溶石、碎石,置入双J管内引流,可立即解除上尿路梗阻,恢复排尿,改善肾功能.具有安全有效、损伤小的优点.可作为婴儿肾结石合并急性肾后性肾功能衰竭手术解除梗阻的首选方法.     

ALG和AHTG在肾移植术后急性肾功能衰竭中的应用

对１４例肾移植术后４８小时内出现肾功能衰竭的患者应用猪抗人胸腺细胞球蛋白（ＡＨＴＧ）和进口马抗人胸腺淋巴细胞球蛋白（ＡＬＧ）。结果发现７１．４％患者肾功能恢复，同期对照组仅４４．４％；一年内４０．０％患者出现排斥反应，而对照组为６２．５％；一年内排斥次数，肾功能恢复时间和进程等则与对照组无明显差异。肾移植术后早期肾功能衰竭的最常见原因为排斥因素，其次为急性肾小管坏死以及手术并发症。