Uso De Eculizumab En Síndrome Urémico Hemolítico Típico: Una Opción Terapéutica En El Compromiso Neurológico Severo. Reporte De Dos Casos / Use Of Eculizumab In Typical Hemolytic Uremic Syndrome: A Therapeutic Option In Severe Neurological Compromise. Two Cases Report

Resumen El síndrome urémico hemolítico típico es una enfermedad endémica en América Latina. Argentina es uno de los países con más casos reportados, con una tasa de diez casos cada 100.000 menores de cinco años. Es la primera causa de insuficiencia renal aguda, y responsable del 9 % de los trasplantes renales. Esta patología se caracteriza por una tríada clásica: anemia microangiopática, trombocitopenia e insuficiencia renal aguda. El principal agente etiológico del Síndrome Urémico Hemolítico es la bacteria Escherichia coli, productora de la toxina Shiga. El Síndrome Urémico Hemolítico tiene una mortalidad aguda inferior al 5 %.1-2 Existe evidencia acerca del rol activo de la shiga toxina en la activación del complemento a través de su unión al factor H. El eculizumab es un anticuerpo monoclonal que inhibe la formación del complejo de ataque de membrana (C5b-9), por su alta afinidad a C5 de la cascada del complemento. Su infusión está aprobada para el tratamiento del Síndrome Urémico Hemolítico atípico, planteándose su utilidad en casos de Síndrome Urémico Hemolítico típico grave con compromiso neurológico severo como alternativa para inhibir la cascada de complemento, y así detener el daño producido por la toxina. Se presentan dos casos de pacientes pediátricos con diagnóstico Síndrome Urémico Hemolítico con rescate de Shiga toxina, con compromiso neurológico grave y que recibieron tratamiento con eculizumab con respuesta favorable.

Abstract Hemolytic Uremic Syndrome is an endemic disease in Latin America. Argentina is one of the countries where most cases are reported, with a rate of ten cases per 100,000 children under five years old. It is the first cause of acute renal failure (ARF), and responsible for 9% of kidney transplants. This pathology is characterized by a classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. The main etiological agent of HUS is the bacterium Shiga toxin-producing Escherichia coli. HUS has an acute mortality lower than 5 %. There is evidence of the active role of the Shiga toxin in the activation of the complement by binding to factor H. Eculizumab is a monoclonal antibody which inhibits the formation of the membrane attack complex (C5b-9), given its great affinity for C5 of the complement cascade. Its infusion is approved to treat atypical HUS, posing its usefulness to treat severe typical HUS with acute neurological involvement as an alternative to inhibit the complement cascade and stop toxin damage. We present two pediatric patients with SUH diagnosis with shiga toxin rescue; these patients, who showed severe neurological involvement, were treated with Eculizumab and had a favorable response.

Duration of anuria predicts recovery of renal function after acute kidney injury requiring continuous renal replacement therapy

BACKGROUND/AIMS: Little is known regarding the incidence rate of and factors associated with developing chronic kidney disease after continuous renal replacement therapy (CRRT) in acute kidney injury (AKI) patients. We investigated renal outcomes and the factors associated with incomplete renal recovery in AKI patients who received CRRT. METHODS: Between January 2011 and November 2013, 408 patients received CRRT in our intensive care unit. Of them, patients who had normal renal function before AKI and were discharged without maintenance renal replacement therapy (RRT) were included in this study. We examined the incidence of incomplete renal recovery with an estimated glomerular filtration rate < 60 mL/min/1.73 m² and factors that increased the risk of incomplete renal recovery after AKI. RESULTS: In total, 56 AKI patients were discharged without further RRT and were followed for a mean of 8 months. Incomplete recovery of renal function was observed in 20 of the patients (35.7%). Multivariate analysis revealed old age and long duration of anuria as independent risk factors for incomplete renal recovery (odds ratio [OR], 1.231; 95% confidence interval [CI], 1.041 to 1.457; p = 0.015 and OR, 1.064; 95% CI, 1.001 to 1.131; p = 0.047, respectively). In a receiver operating characteristic curve analysis, a cut-off anuria duration of 24 hours could predict incomplete renal recovery after AKI with a sensitivity of 85.0% and a specificity of 66.7%. CONCLUSIONS: The renal outcome of severe AKI requiring CRRT was poor even in patients without further RRT. Long-term monitoring of renal function is needed, especially in severe AKI patients who are old and have a long duration of anuria.

Etiology and outcomes of anuria in acute kidney injury: a single center study

BACKGROUND: It was previously known that anuric acute kidney injury (AKI) is uncommon and its occurrence suggests complete ureteral obstruction, shock, or a major vascular event. As the epidemiology of AKI has significantly changed over the past decade, it is possible that the incidence, etiology, or clinical characteristics of anuric AKI have also changed. METHODS: A prospective cohort study was conducted that included all patients undergoing renal replacement therapy (RRT) for AKI during a 2-year period in a tertiary hospital. Patients were classified as having anuric, oliguric, or nonoliguric AKI based on their volume of urine when RRT started using the modified Acute Kidney Injury Network criteria. RESULTS: Of the 203 patients included in the study, 21.2% met the criteria for anuric AKI. Septic and postoperative AKI were the main causes of anuric AKI, with 60.5% of incidences occurring in hospital. Anuric AKI was associated with a younger age, a lower prevalence of pre-morbid chronic kidney disease and diabetes, more frequent continuous RRT requirement, and multi-organ dysfunction. In addition, patients with anuric AKI had a higher rate of in-hospital mortality and long-term dependence on RRT than patients with nonanuric AKI. CONCLUSION: Anuric AKI is common, with sepsis as the main etiological insult, and is associated with adverse outcomes among patients with AKI who require RRT.

Viper bites complicate chronic agrochemical nephropathy in rural Sri Lanka

Snakebite is a common occupational health hazard among Sri Lankan agricultural workers, particularly in the North Central Province. Viperine snakes, mainly Russell’s viper envenomation, frequently lead to acute renal failure. During the last two decades, an agrochemical nephropathy, a chronic tubulointerstitial disease has rapidly spread over this area leading to high morbidity and mortality. Most of the epidemiological characteristics of these two conditions overlap, increasing the chances of co-occurrence. Herein, we describe four representative cases of viperine snakebites leading to variable clinical presentations, in patients with chronic agrochemical nephropathy, including two patients presented with acute and delayed anuria. These cases suggest the possibility of unusual manifestations of snakebite in patients with Sri Lankan agrochemical nephropathy, of which the clinicians should be aware. It could be postulated that the existing scenario in the Central America could also lead to similar clinical presentations.

Viper bites complicate chronic agrochemical nephropathy in rural Sri Lanka

Snakebite is a common occupational health hazard among Sri Lankan agricultural workers, particularly in the North Central Province. Viperine snakes, mainly Russell’s viper envenomation, frequently lead to acute renal failure. During the last two decades, an agrochemical nephropathy, a chronic tubulointerstitial disease has rapidly spread over this area leading to high morbidity and mortality. Most of the epidemiological characteristics of these two conditions overlap, increasing the chances of co-occurrence. Herein, we describe four representative cases of viperine snakebites leading to variable clinical presentations, in patients with chronic agrochemical nephropathy, including two patients presented with acute and delayed anuria. These cases suggest the possibility of unusual manifestations of snakebite in patients with Sri Lankan agrochemical nephropathy, of which the clinicians should be aware. It could be postulated that the existing scenario in the Central America could also lead to similar clinical presentations.

Acute Kidney Injury and Postobstructive Diuresis Caused by a 4 mm Urinary Calculus

Urinary obstructions from ureteral calculi are one of the causes of postrenal acute kidney injury (AKI). Here we present a case of AKI caused by a 4 mm ureteral calculus with postobstructive diuresis following the spontaneous passage of the calculus. A 13-year-old girl who underwent nephrectomy for the removal of a neuroblastoma eight years previously, visited our institution because anuria had developed over the preceding five days. The serum creatinine level was elevated at 13.4 mg/dL. Radiological examinations showed the right solitary kidney with moderate hydronephrosis and a 4 mm calculus in the upper right ureter. The patient immediately underwent hemodialysis. After the ureteral calculus was passed spontaneously on day 2 of hospitalization, urinary output increased to more than 5,200 mL per day. Intravenous fluid replacement with careful monitoring of weight, intake, output, and serum and urine electrolytes was performed. On day 5 of hospitalization, the patient's condition stabilized.

A Case of Neonate with Acute Renal Failure after Maternal Treatment with Angiotensin II Receptor Blocker / 대한주산의학회잡지

Hypertension is common medical problem encountered during pregnancy. However medication administered for maternal hypertension may cause fetal or neonatal complications. Angiotensin converting enzyme inhibitor or angiotensin II receptor blocker are rarely used during pregnancy, and there are few reports about the effect of them, because administration of these drugs during pregnancy may cause oligohydramnios, renal tubular dysplasia, hypocalvaria, pulmonary hypoplasia, intrauterine growth retardation, neonatal anuria and persistent ductus arteriosus. We report a case of neonatal acute renal failure by angiotensin II receptor blocker during pregnancy. In this case, the neonate with meconium aspiration was admitted to neonatal intensive care unit (NICU). During the NICU stay, neonatal anuria occurred, and there was a medical history that his mother took Candesartan Cilexeril (Atacand(R)), one of angiotensin II receptor blockers during pregnancy. The neonate showed intrinsic acute renal failure, so fluid was restricted and diuretics were administered to the neonate, and after 10 days, anuria improved.

Anuria in a 9-Month-Old Infant Resulting from Ureteral Cystine Stones

Pediatric urolithiasis and calcular anuria in early infancy are rare. Cystine stones may develop in utero or during early infancy. We report the case of a female 9-month-old infant with obstructive anuria resulting from cystine stones in a single functioning unit. She presented to the emergency department owing to the absence of micturition for 3 days. Radiological investigations revealed four left ureteral stones and an atrophic right kidney resulting from a calcular obstruction. Her laboratory values were as follows: serum creatinine 6.7 mg/dl, Na 132 mEq/l, K 6 mg/dl, and hematocrit 32%. An urgent percutaneous nephrostomy tube was inserted into the left side for urinary drainage, and her serum levels of creatinine and K returned to normal within 3 days. A left ureterolithotomy was the final management. Stone analysis revealed pure cystine crystals.

A Case of Bilateral Acute Renal Cortical Necrosis Complicated by Tranexamic Acid Administration / 대한내과학회지

Acute renal cortical necrosis is an anuric form of acute renal failure. We experienced a case of renal cortical necrosis complicated by tranexamic acid administration. To our knowledge, only three cases of renal cortical necrosis have been reported worldwide. A 49-year-old man was referred with hemothorax and multiple bone fractures following a traffic accident. Tranexamic acid, and hemocoagulase were injected three times a day. After the 4th dose of hemostatics, anuria developed abruptly, the platelet count decreased to 84,000 /microL, and the serum creatinine was increased to 2.56 from 1.06 mg/dL. On the 4th Intensive Care Unit (ICU) day, computed tomography (CT) showed bilateral renal cortical necrosis with normal renal arteries and aorta. The oliguria persisted for 14 days and temporary hemodialysis was performed. The serum creatinine had decreased to 2.12 mg/dL 8 months after discharge.

A Case of Leriche Syndrome Manifested by Anuria / 대한신장학회잡지

Leriche syndrome is an aortoiliac occlusive disease. The aortoiliac junction is the most common sites of chronic obliterative atherosclerosis. Leriche syndrome has a variety of clinical symptoms attributed to the obstruction of the infrarenal aorta. Common clinical symptoms include thigh, hip, and buttock claudication as well as erectile impotence, usually in association with diminished femoral pulses. But acute anuric renal failure as first manifestation of Leriche syndrome is very uncommon. Contrast-enhanced 3D MRA appears to be well suited for assessment of patients with suspected Leriche syndrome. We report a 75-year-old man who presents anuria as first manifestation of Leriche syndrome.

Adequacy of Dialysis in Anuric CAPD Patiens / 대한신장학회잡지

BACKGROUND: Failure to achieve target values for both urea (Kt/V) and creatinine clearance has been associated with increased morbidity and mortality in CAPD patients. Current standard of adequacy CAPD is to provide a weekly normalized urea clearance of 2.0 or more and a creatinine clearance of 60 liter/ 1.73 m2 or more. Conventional CAPD in patients without residual renal function is associated with worse clinical outcomes. This study was designed to study the effect of increasing daily exchange frequency on dialysis adequacy in anuric CAPD patients. METHODS: The 27 anuric CAPD patients (patients on 4x2 L daily exchanges for 7 days) were selected and then they received standard dose dialysis (4x2 L daily exchanges for 7 days) followed by high dose dialysis (5x2 L daily exchanges). Weekly Kt/Vurea and weekly Ccr were measured at the end of standard and high dose dialysis. Adequate dialysis was defined as satisfying both weekly Kt/Vurea >2.0, weekly Ccr >60 L/1.73 m2 according to DOQI guideline. RESULTS: Selected patients were 12 men and 15 women, mean age was 49+/-2 years, mean weight was 59.2+/-0.1 kg , mean peritoneal dialysis duration was 51+/-5 months. Weekly Kt/V was 1.7+/-.3 in standard dose dialysis patients and 2.1+/-.4 in high dose dialysis patients, mean Ccr was 48.8+/-.2 L/ week/1.73 m2 in standard dose dialysis patients and 63.1+/-2.1 L/week/1.73 m2 in high dose dialysis patients. This difference is statistically significant (p< 0.05). Among 27 patients, only 2 standard dose dialysis patients were on adequate dialysis but in high dose dialysis group, 14 patients were on adequate dialysis according to DOQI guideline. In high dose dialysis, 14 on adequate dialysis and 13 inadequate dialysis were divided and their clinical factors were analyzed. Only volume of urea distribution (30.9+/-.9 L vs 37.7+/-.6 L) was significantly different (p<0.05). CONCIUSION: This study revealed most standard dose of anuric CAPD patients, who receiving daily 8 L dialysis did not dialyzed adequately by DOQI guideline. Increasing the number of exchanges effectively increased Kt/Vurea and weekly creatinine clearance in anuric CAPD patients.

Adequacy of Dialysis in Anuric CAPD Patiens / 대한신장학회잡지

BACKGROUND: Failure to achieve target values for both urea (Kt/V) and creatinine clearance has been associated with increased morbidity and mortality in CAPD patients. Current standard of adequacy CAPD is to provide a weekly normalized urea clearance of 2.0 or more and a creatinine clearance of 60 liter/ 1.73 m2 or more. Conventional CAPD in patients without residual renal function is associated with worse clinical outcomes. This study was designed to study the effect of increasing daily exchange frequency on dialysis adequacy in anuric CAPD patients. METHODS: The 27 anuric CAPD patients (patients on 4x2 L daily exchanges for 7 days) were selected and then they received standard dose dialysis (4x2 L daily exchanges for 7 days) followed by high dose dialysis (5x2 L daily exchanges). Weekly Kt/Vurea and weekly Ccr were measured at the end of standard and high dose dialysis. Adequate dialysis was defined as satisfying both weekly Kt/Vurea >2.0, weekly Ccr >60 L/1.73 m2 according to DOQI guideline. RESULTS: Selected patients were 12 men and 15 women, mean age was 49+/-2 years, mean weight was 59.2+/-0.1 kg , mean peritoneal dialysis duration was 51+/-5 months. Weekly Kt/V was 1.7+/-.3 in standard dose dialysis patients and 2.1+/-.4 in high dose dialysis patients, mean Ccr was 48.8+/-.2 L/ week/1.73 m2 in standard dose dialysis patients and 63.1+/-2.1 L/week/1.73 m2 in high dose dialysis patients. This difference is statistically significant (p< 0.05). Among 27 patients, only 2 standard dose dialysis patients were on adequate dialysis but in high dose dialysis group, 14 patients were on adequate dialysis according to DOQI guideline. In high dose dialysis, 14 on adequate dialysis and 13 inadequate dialysis were divided and their clinical factors were analyzed. Only volume of urea distribution (30.9+/-.9 L vs 37.7+/-.6 L) was significantly different (p<0.05). CONCIUSION: This study revealed most standard dose of anuric CAPD patients, who receiving daily 8 L dialysis did not dialyzed adequately by DOQI guideline. Increasing the number of exchanges effectively increased Kt/Vurea and weekly creatinine clearance in anuric CAPD patients.

Acute Renal Artery Thromboembolism during Abdominal Aortic Bypass Graft Surgery / 대한마취과학회지

Acute occlusion of the artery to a single functioning kidney is a rare but surgically correctable cause of acute renal failure. A young-aged woman with acute renal failure and anuria due to a thromboembolism of the right renal artery was surgically treated 2 hours after the onset of anuria. Revascularization resulted in the reversal of renal failure and complete recovery of renal function. An aggressive diagnostic and therapeutic approach is important whenever occlusion of the renal artery is suspected during surgery in Takayasu arteritis patients.

Clinical management of anuria caused by congenital bilateral ureteral stenotic obstruction / 中华泌尿外科杂志

Objective To investigate the clinical features of anuria caused by pediatric congenital bilateral ureteral stenotic obstruction,and to improve the diagnosis and treatment of the disease.Methods This series included 7 infants(2 males and 5 females;age range,35-57 d) with congenital bilateral ureteral stenotic obstruction.The clinical presentations were characterized by sudden anuria or oliguria.B-ultrasound,cystography and puncture pyelography were performed in all of them,and magnetic resonance urography(MRU) in 3.Based on the definite diagnosis,resection of stenotic segment of the ureter or ureteral implantation was performed on them.Results The diagnosis was confirmed by puncture pyelography in all of the 7 cases.Six cases underwent phase I surgery and recovered.No ureteral stenosis and cystoureteral reflux occurred during a follow-up of 2-4 years.The renal function was normalized in them.One case had stenosis recurrence due to scar formation,and was cured by the second operation.Conclusions Anuria caused by pediatric congenital bilateral ureteral stenosis should be diagnosed and treated as early as possible.Definite diagnosis can be established by puncture pyelography and MRU.Resection of the obstructive segment of the ureter,protection of renal function,and ureteral implantation are the main therapies.

Diagnosis of acute anuria caused by upper urinary tract obstruction / 中华泌尿外科杂志

Objective To study the diagnostic techniques of acute anuria caused by upper urinary tract obstruction. Methods Fifty-eight patients with acute anuria caused by upper urinary tract obstruction were analyzed on clinical manifestations,etiology of obstruction and imageologic findings. Results The main clinical manifestations consisted of anuria in 58 cases,pain in renal region in 41,percussion tenderness over kidney region in 33,hypertension in 20,high levels of BUN and creatinine in 57 and hyperkaliemia in 8.The main adjuvant examinations included MRU,B-ultrasound,X-ray (KUB,IVU and retrograde pyelography),CT and MRI.The detection rates of upper urinary tract obstruction by above-mentioned examinations were as follows:100% (25/25) with MRU,88% (50/57) with B-ultrasound,60% (6/10) with CT/MRI,49% (19/39) with X-ray. The detection rates of MRU and B-ultrasound were significantly higher than those of X-ray and CT/MRI (P

A Case of Anuria and Urinary Ascites in a Premature Infant due to Bilateral Ureteropelvic Fungal Bezoars / 대한비뇨기과학회지

A 3-month-old female presented with anuria and abdominal distention for 1 day. The patient was twin and was delivered at 30 week-term with low birth weight. Emergent ultrasonography revealed both hydronephrosis and hyperechogenic mass within the both renal pelves. Urinoma around left kidney was also found. Computed tomography showed high density material filled dilated pelvises. Urinoma measured 3x4cm in the left prerenal space and urinary ascites in the right abdomen were found. Candida albicans was cultured from catheter drained urine. We started the care with percutaneous nephrostomy with amphotericin B irrigation, coupled with systemic antifungal therapy. And this management had a major role in the successful outcome.

Anuria in a Infant due to Bilateral Ureteropelvic Fungus Balls / 대한신장학회잡지

Fungal infection has been observed with increasing frequency in recent years because the use of combinations of broad spectrum antibiotics, immunosuppressive agents, and antineoplastic agents is increasing and the survival rate of premature baby is increasing. We experienced a 3 month old male infant with anuria due to bilateral ureteropelvic fungus balls. He was born at 31 weeks gestation period and had been treated with broad spectrum antibiotics for 5 weeks after birth. We removed fungus balls surgically and made nephrostomy bilaterally. And then irrigation of amphotericn B through nephrostomy and systemic amphotercin B injection had performed for 3 weeks. Thereafter fungus balls completely disappeared.

A Clinical Observation on Complete Anuria of Postrenal Type / 대한비뇨기과학회지

A clinical observation was made on 7 patients with postrenal complete anuria in the department of Urology. Medical School, Jeonbug National University during recent 3 years. The results were as follows. 1. Most common age groups were the 5th and 6th decades (57.1%), and the male to female ratio was 1.3:1. 2. Of etiology, ureteral stones were most common (3 cases), and the others were malakoplakia of both ureteral surrounding tissues (1 case), both ureteral obstruction due to renal bleeding (1 cases) post-catheterization ureteral obstruction (1 case) and ureteral compression due to metastatic cervical cancer involved lower ureter (1 case) Incidence of complete anuria were relatively high in the solitary kidney. 3. In most cases. The BUN and the creatinine levels were nearly normalized in 6th postoperative day. And 5 cases of high blood level in preoperative state showed massive diuresis after operation.

A Clinical Observation on Complete Anuria / 대한비뇨기과학회지

A clinical observation was made on 8 cases with complete anuria of the in-patients in the Department of Urology, Chonnam University Medical School during the period from January. 1971 to December. 1975. The results are as followings: 1) Of 8 cases with complete anuria, 7 cases were postrenal anuria and only one case was renal anuria. 2) In postrenal anuria, the most common site of ureteral obstruction was lower ureter. S) Massive diuresis after relief of urinary tract obstruction was noted in 6 cases and a week after relief of urinary tract obstruction. B. U. N. level was almost normal range in 6 cases.