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.     

Postpartum hemolytic uremic syndrome in a 17-year-old Filipina primigravid

A 17-year-old Filipina primigravid developed acute renal failure secondary to hemolytic uremic syndrome (HUS) after undergoing emergency cesarean section for severe pre-eclampsia and abruptio placenta. She underwent hemodialysis with concurrent infusions of fresh-frozen plasma and packed red cells for 5 weeks. Renal biopsy revealed findings consistent with HUS with glomerular crescents. She received three doses of pulse methylprednisolone followed by oral prednisone. Renal function improved 5 weeks after the onset of HUS. The pathogenesis, differential diagnosis, and treatment options of postpartum HUS are discussed.     

Prolonged reversible renal failure with nephrotic syndrome

Steroid nonresponsive nephrotic syndrome in a 15-year-old girl with reversible renal failure required dialysis and aggressive nutritional therapy for 1 year. Severe interstitial edema and foot process fusion were the only processes identified to explain the renal failure. Diabetes-like alterations of the glomerular capillary wall basement membrane may have been an outcome of the intense alimentation.     

Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features

From January 1968 to December 1984, 312 infants and children with hemolytic uremic syndrome were admitted to our unit; 8 patients died (2.5%) during the acute phase; 118 children were followed as outpatients at yearly intervals for at least 10 years (mean follow-up 13 years, range 10 – 19.8 years). Four evolution patterns at the end of the follow-up were defined: group 1, complete recovery, 74 (62.7%); group 2, proteinuria with/without hypertension, 21 (17.7%); group 3, reduced creatinine clearance, often in conjunction with proteinuria and hypertension, 19 (16.1%); group 4, end-stage renal failure, 4 (3.4%). We investigated the association between several variables of the acute stage and the long-term evolution. Most non-anuric patients recovered completely (92.5%), while 38.4% of those with 1 – 10 days and 69.2% of those with 11 or more days of anuria had chronic renal sequelae. Similar results were found when analyzing the requirement for peritoneal dialysis. Of the patients with proteinuria at the 1-year control, 86% had renal abnormalities at the end of the follow-up. In our experience, although the final outcome was not predictable in every instance, the severity of acute renal failure – as determined by the days of anuria – and the presence of proteinuria 1 year after the acute phase were the most useful prognostic indicators. Received January 9, 1996; received in revised form April 16, 1996; accepted May 10, 1996     

Cyclosporin in the treatment of idiopathic nephrotic syndrome in children

Thirty-five children (12 girls, 23 boys), aged from 1 year and 5 months to 14 years at the onset of idiopathic nephrotic syndrome, received cyclosporin A (CyA) because of steroid toxicity or failure to respond to steroids. The initial oral dose was 6 mg/kg per day and this was adjusted to obtain trough plasma levels of 50–150 ng/ml. The duration of treatment was between 2 and 8 months. In patients who responded to CyA treatment, the dosage was tapered off; treatment was stopped if found to be ineffective. Of the 35 children, 20 were frequent-relapsing steroid responders who suffered serious side-effects from steroid therapy. Seventeen of them either went into remission or did not relapse despite the withdrawal of prednisone. Prednisone doses could be lowered but not stopped in 1 patient and the remaining 2 patients relapsed when prednisone was tapered off. At the final examination, 10 of the 12 children in whom CyA was tapered off and who had initially responded to CyA had relapsed. A second course was given to these 10 patients and 3 failed to respond. Five children were partial steroid responders and CyA induced a remission in 1 and a partial remission in another. Among the 10 children who were steroid resistant, only 1 responded to CyA, 2 had a partial response and 7 failed to respond to CyA. A reduction of glomerular filtration rate occurred in 8 patients, 7 of whom had either persistent nephrotic syndrome or were in relapse, which suggests that factors other than CyA nephrotoxicity may have been operative. Complete reversal occurred in only 4 patients. Significant histological changes, likely to be related to CyA, were seen in 2 repeat renal biopsies out of the 11 performed.     

Long-term prognosis of hemolytic uremic syndrome and effective renal plasma flow

The long-term prognosis of diarrhea-associated hemolytic uremic syndrome (D+ HUS) was evaluated in a cohort of 127 of 149 children who had survived the acute phase. Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated by serial ⁵¹Cr-EDTA and ¹²³iodine-hippurate clearances. All children had acute renal failure during the initial phase and 74% of patients were dialyzed. During the 1st year, mean GFR and ERPF increased continuously until a plateau was reached. In the 2nd year after the diagnosis of HUS, GFR was below 80 and ERPF below 515 ml/min per 1.73 m² in 16% and 47% of patients, respectively. At the end of a median follow-up of 5.0 (range 2.0–13.2) years, the proportion of children with renal sequelae such as proteinuria ≥300 mg/l, hypertension, or a GFR <80 ml/min per 1.73 m² was 23%. Anuria of more than 7 days’ duration and hypertension during the acute phase were statistically significant risk factors for an unfavorable outcome. A reduced ERPF in the 2nd year was found in 93% of patients with sequelae. Mean filtration fraction (SD) in these patients was 0.26 (±0.07) versus 0.19 (±0.05) in patients without sequelae (P<0.0001). These data suggest that loss of nephrons during the acute phase may implicate hyperfiltration in the residual functioning kidney mass leading to progressive renal disease. ERPF in the 2nd year after D+ HUS may serve as an excellent parameter to detect patients with a high risk of an unfavorable long-term outcome. Received: 17 September 1998 / Revised: 31 March 1999 / Accepted: 1 April 1999     

原发性肾病综合征并发急性肾衰竭56例报告

目的分析56例原发性肾病综合征（PNS）并发急性肾衰竭（ARF）患者临床特点、病理改变及疗效。方法回顾性分析56例PNS并发ARF患者临床和病理改变。结果PNS并发ARF发生率约2．9％左右,肾脏病理改变肾小球多以微小病变为主,肾小管上皮细胞浊肿、空泡变性及蛋白管型常见,但肾间质水肿改变并不明显。经及时有效治疗,91．1％患者肾功能可完全恢复正常,其中抗凝治疗和合适透析方式选择起到较为重要的作用。结论PNS并发ARF多发生于肾脏病变轻微患者,其发病机制有待进一步探讨,经及时有效的综合治疗,患者多预后良好。     

Minor degree of reversible renal insufficiency: a frequent complication of adult minimal-change nephrotic syndrome.

We report on a group of 27 patients with minimal-change nephrotic syndrome who presented complete recovery from the nephrotic syndrome and the other clinical signs during follow-up. At the start of the examination an elevated serum urea was found in 10 (37%) and of serum creatinine in 4 (15%) patients. Creatinine clearance was decreased in 10 (37%), systolic blood pressure was elevated in 8 (29%) and diastolic in 11 (41%). Interstitial oedema was found in 3, tubular parenchymal degeneration in 4, slight interstitial mononuclear infiltration in 6 and slight interstitial fibrosis in 1 patient. Complete recovery of renal function was seen in all patients.     

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1–15.0 years) and the gender ratio was 1.4. Median follow-up period was 7.7 years (1.0–19.7 years). The disease in 45 patients (58%) was initially not steroid-responsive and in 33 (42%) it was later non-responsive. The main therapeutic strategies included administration of ciclosporine (CsA) alone (n = 29; 37%) and CsA + mycophenolate mofetil (n = 18; 23%). Actuarial patient survival rate after 15 years was 97%. Renal survival rate after 5 years, 10 years and 15 years was 75%, 58% and 53%, respectively. An age at onset of nephrotic syndrome (NS) > 10 years was the only independent predictor of end-stage renal disease (ESRD) in a multivariate analysis using a Cox regression model (P < 0.001). Twenty patients (26%) received transplants; ten showed recurrence of the NS: seven within 2 days, one within 2 weeks, and two within 3–5 months. Seven patients lost their grafts, four from recurrence. Owing to better management, kidney survival in idiopathic steroid-resistant nephrotic syndrome (SRNS) has improved during the past 20 years. Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment.     

An unusual cause of acute renal failure: renal lymphoma

Renal involvement is a common finding in non-Hodgkins lymphoma (NHL). Acute renal failure at initial presentation due to lymphomatous infiltration of the kidneys has been described infrequently. We report a 17-year-old male who presented with acute renal failure due to massive lymphomatous infiltration of the kidneys, which necessitated hemodialysis. The diagnosis of B-cell NHL was established by tru-cut biopsy of the kidneys and the patient had an excellent response to high-dose chemotherapy with no major complication. The presence of extrarenal involvement in the testes and the retroperitoneal lymph nodes made the diagnosis of primary renal lymphoma debatable. However, considering the delay in diagnosis and the high proliferative rate of B-cell NHL, we might postulate that the disease had originated primarily in the kidneys. We recommend that in NHL cases with severe renal involvement, full-dose chemotherapy should be instituted with meticulous clinical and laboratory follow-up in order to improve clinical and renal failure status rapidly and to avoid further dissemination of NHL.     

Atypical hemolytic uremic syndrome in human immunodeficiency virus-1-infected children

We describe the clinical and pathological findings of the hemolytic uremic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolytic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both patients. None of these children presented with bloody diarrhea, evidence of circulating antibody response to Escherichia coli O157 lipopolysaccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma infusion and renal replacement therapy. Their clinical course was characterized by non-oliguria and lack of significant hypertension throughout the acute phase of the disease. Despite these favorable clinical parameters, both patients developed end-stage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unknown and the role of HIV infection in its pathogenesis, although possible, is unclear. Received March 5, 1996; received in revised form and accepted October 15, 1996     

Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab

BACKGROUND: Therapeutic options are limited in cases of focal and segmental glomerulosclerosis (FSGS) that fail to respond to steroids and calcineurin inhibitors. We describe a case in which steroid-resistant nephrotic syndrome (SRNS) secondary to FSGS did not respond to conventional treatment, but was successfully treated with rituximab. Unlike previous reports in which rituximab was used in conjunction with a calcineurin inhibitor, we present the first case where rituximab was used as the sole therapeutic agent. CASE REPORT: An 11-month-old boy presented with severe manifestations of primary nephrotic syndrome with a subsequent non-responsive steroid course. A renal biopsy confirmed FSGS, with normal staining for podocin and nephrin. Genetic studies for podocin were normal. The child developed ciclosporin-induced hemolytic uremic syndrome (HUS), and the response to plasma exchange, following ciclosporin withdrawal, was only temporary. A trial of a combination of mycophenolate and dexamethasone did not have any effect on proteinuria or fluid status. Four weekly rituximab infusions at 375 mg/m(2)/dose induced a complete remission without any adverse effects. CONCLUSIONS: This case suggests that rituximab may be used as the sole therapeutic agent in the treatment of SRNS secondary to FSGS, especially in cases where calcineurin inhibitors are contraindicated.     

Snakebite-induced acute renal failure

Snakebite is not an uncommon cause of acute renal failure (ARF) in developing countries. We report a12-year-old boy who presented with oliguric ARF following snakebite. He had pallor, icterus, generalized edema, hypertension, and was oliguric. Investigations revealed severe azotemia, microangiopathic hemolytic anemia, thrombocytopenia, prolonged coagulation parameters, and raised fibrin degradation products, suggesting disseminated intravascular coagulation as the cause of ARF. The patient improved with antisnake venom, dialysis, and other supportive treatment.     

Acute renal failure in children with idiopathic nephrotic syndrome

Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, acute tubular necrosis, allergic interstitial nephritis, bilateral renal vein thrombosis, acute pyelonephritis, or rapid progression of the original glomerular disease. It may be termed idiopathic if the underlying cause is undetermined. We present three children with idiopathic nephrotic syndrome who were admitted with acute renal failure. One case was due to drug-induced allergic interstitial nephritis. The other two were idiopathic in nature. Improvement in renal function occurred in the three patients over a variable period of 10 days to 4 weeks. After careful exclusion of well-known causes of acute renal failure, idiopathic acute renal failure (IARF) should be considered as a diagnostic possibility in these patients. The exact pathophysiology of IARF is not understood. Possible proposed explanations include interstitial edema, tubular obstruction, altered glomerular permeability, and unrecognized hypovolemia.     

Reversible renal failure and nephrotic syndrome without interstitial nephritis from zomepirac

This report describes two patients with the clinical syndrome of reversible renal failure and nephrotic syndrome caused by the nonsteroidal anti-inflammatory agent, zomepirac sodium. What is unique about this report are the pathologic findings on renal biopsy which showed fusion of foot processes consistent with minimal change disease without evidence of an interstitial infiltrate. A cause-and-effect relationship of the disease to zomepirac administration is strongly suggested by the resolution of the renal dysfunction when the drug was stopped and by more than eighteen months of follow-up without evidence of any impairment in renal function.     

Acute renal failure at the onset of idiopathic nephrotic syndrome in two children

Two children with idiopathic nephrotic syndrome (INS) developed acute renal failure (ARF) at the onset of the disease. Although the initial renal biopsy showed minimal change (MC) lesions with prominent interstitial edema, repeat renal biopsy revealed focal segmental glomerulosclerosis (FSGS). ARF has been reported to be a relatively rare complication in childhood INS. However, the initial manifestation of ARF may increase the risk for subsequent progression to FSGS in a proportion of children with INS with MC lesions. Received: April 3, 2000 / Accepted: October 31, 2000