Prevalence and associations of symptomatic renal papillary necrosis in sickle cell anemia patients in South-Eastern Nigeria.

AIM: To assess the prevalence and associations of symptomatic renal papillary necrosis (RPN) in sickle cell anemia patients. PATIENTS AND METHODS: The case notes of homozygous hemoglobin (Hb) S patients diagnosed with RPN were retrospectively assessed. Diagnosis was based on microscopic hematuria and positive ultrasound findings. Their steady state diastolic blood pressure, Hb, leukocyte count, platelet count, serum direct bilirubin, and aspartate transaminase, were obtained by automated analyzers. These were evaluated for any relationship with the occurrence of RPN. RESULTS: Two hundred and twenty patients were assessed aged 6-55 years with a median age of 24 years. The prevalence of symptomatic RPN was found to be 2.3%. RPN was positively associated with the female gender (Chi-square P value 0.001), but not with any other clinical or laboratory variable. However, other predictors of disease severity were positively associated with RPN such as age, diastolic blood pressure 0.180 (P = 0.016), serum aspartate transaminase, serum bilirubin 0.145 (0.027), Hb, and leukocyte count - 0.155 (P = 0.003). CONCLUSION: The prevalence of symptomatic RPN is low in this group of homozygous S patients and occurs more commonly in females. Improvement in care for these patients will reduce these chronic complications.

Severe anemia in an adolescent male with sickle cell trait: a case report.

BACKGROUND: Sickle cell trait is generally considered a benign condition. However, it has been associated with uncommon comorbidities such as painless gross hematuria secondary to renal papillary necrosis and renal medullary carcinoma. OBSERVATION: We present a 16-year-old African American boy with sickle cell trait and a recent history of prolonged gross hematuria due to renal papillary necrosis. The patient developed severe iron deficiency anemia and required transfusion support. CONCLUSIONS: Although renal papillary necrosis is well-described, it is uncommon in pediatrics and only rarely results in the need for transfusion.

Low dose, oral epsilon aminocaproic acid for renal papillary necrosis and massive hemorrhage in hemoglobin SC disease.

Renal papillary necrosis in sickling hemoglobinopathies can lead to significant complications, including hemorrhage, obstruction, and infection. Despite its frequency, there are limited therapies for protracted hemorrhage. In the past, massive hemorrhage was managed with nephrectomy. Here, we report a patient with hemoglobin SC disease and prolonged, life-threatening hemorrhage from papillary necrosis successfully treated with oral, low-dose epsilon aminocaproic acid (EACA). Although further study is warranted, this case illustrates the need to consider EACA in the conservative management of renal papillary necrosis and significant hemorrhage in sickle cell hemoglobinopathies.

Bilateral ureteric obstruction secondary to renal papillary necrosis.

A 2-year-old male presented to hospital with a 5-day history of vomiting and pyrexia. He was initially treated with non-steroidal medication as an anti-pyretic. Initial investigations demonstrated a raised urea and creatinine and he was treated with intravenous fluids. Within 24 h he became anuric with progressive renal insufficiency. Ultrasound scan demonstrated minimal bilateral hydronephrosis with debris in the lower pole calyces. The bladder was empty. Cystoscopy and retrograde contrast imaging revealed bilateral ureteric obstruction. Double J stents were inserted and his renal function returned to normal within 4 days. We believe the aetiology to be renal papillary necrosis and bilateral ureteric obstruction secondary to the administration of ibuprofen in association with dehydration.

Bilateral ureteral obstruction from papillary necrosis secondary to household cleaner ingestion.

We report a case of a patient who developed bilateral hydroureteronephrosis from papillary necrosis secondary to ingestion of commercial toilet bowl cleaner. Eight days after her ingestion, acute renal failure prompted a renal ultrasound that showed bilateral hydroureteronephrosis. Emergent bilateral percutaneous nephrostomy tubes were placed and subsequent ureteroscopy revealed a large amount of obstructing necrotic material consistent with papillary necrosis. Ureteroscopic removal of the material and bilateral ureteral stents improved renal function. The etiology of this patient's papillary necrosis was likely due to a combination of hypovolemia, systemic acidosis from the ingestion, and direct toxicity of the substance on the renal vasculature. This case demonstrates the importance of early recognition of renal insults and the extra intestinal manifestations of toxic household ingestions.

An unusual cause of renal allograft dysfunction: graft papillary necrosis.

A 43-year-old nondiabetic man, 5 years post-renal transplantation, presented complaining of oliguria, fever and dysuria of 1-day duration. Graft ultrasound did not reveal any obstructive changes. Graft function did not improve in spite of 3 days of antibiotics. On the fourth day he passed fleshy material in urine subsequent to which his urine output improved and fever recovered. His graft function settled near to the previous baseline. Histological analysis of the material revealed necrosed renal papillary tissue. Renal papillary necrosis in allograft is uncommon and generally reported in the immediate postoperative phase, but it can still occur later in transplant follow-up. It is a potentially treatable cause for acute allograft dysfunction and should be suspected in transplant patients presenting with acute pyelonephritis but not getting relief from antibiotic therapy.

Papillary necrosis causing hydronephrosis in renal allograft treated by percutaneous retrieval of sloughed papilla.

Obstructive uropathy is the second most common urological complication in a transplanted kidney. The usual causes of obstruction are ureteral stenosis and calculi. Papillary necrosis as a cause of obstruction in a transplant kidney is extremely rare with only one prior report published. Moreover, percutaneous removal of sloughed papilla in a transplant kidney has not previously been reported. We report an unusual case of a sloughed papilla causing hydronephrosis of a transplant kidney and its successful percutaneous removal. The recognition of renal papillary necrosis is important, not only because it can be a sign of acute rejection but also it because it can lead to obstruction, infection and potentially the loss of the transplant as exemplified by our case. Rapid diagnosis and meticulous retrieval technique are the crucial factors in minimizing the complications due to obstruction of a transplanted kidney by sloughed papilla.

[Diagnosis and conservative treatment of acute pyelonephritis in diabetes mellitus].

AIM: To raise efficacy of conservative treatment of patients with acute pyelonephritis (AP) which developed in the presence of diabetes mellitus (DM). MATERIAL AND METHODS: Elderly patients (n = 218, 182 females and 36 males) with DM and AP enered the study. DM type 1 and 2 were diagnosed in 41 and 177 of them. Pyelonephritis was diffuse-purulent, pyodestructive, calculous, pyocalculous. RESULTS: Conservative treatment was made in 160 patients, surgical treatment--in 58. Lethal outcomes (14 cases) were registered as a rule in patients with bilateral pyodestructive pyelonephritis. CONCLUSION: AP is a severe complication of DM. The severity of DM decompensation and metabolic disorders is proportional to AP severity. AP in diebetics runs often an asymptomatic course and the diagnosis is difficult. Therapeutic policy is individual with consideration of a clinical course. Positive results were achieved in 80% patients.

Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations.

Obstructive uropathy causes tubular resistance to aldosterone and severe metabolic imbalance may be precipitated by an episode of pyelonephritis. In the last 3 years we investigated 52 episodes of pyelonephritis (positive urine culture, elevated C reactive protein, fever, elevated neutrophil count) in 50 children between 15 days and 15 months of age. Ultrasonography voiding cystography and renal scintiscan were performed in all cases and i.v. urography in some. A salt-losing syndrome with hyponatremia and hyperkalemia (Na < 125 meq/liter; K > 6.3 meq/liter) was observed in 17 infants < 3 months, accompanied by plasma aldosterone concentration of 5000 to 23,000 pg/ml (normal value, < 1000 pg/ml). All these children had a severe urinary tract (UT) malformation (ureteropelvic junction stenosis in 7 cases, vesicoureteral reflux in 7, posterior urethral valves in 2, double system in 1). Thirteen infants < 3 months, 7 with no urinary tract malformations, did not have electrolyte imbalance. Pyelonephritis was diagnosed in 20 other patients ages 4 to 15 months, including 16 with severe UT malformations; 4 had normal UTs. We conclude that a salt-losing syndrome with tubular resistance to aldosterone can occur during pyelonephritis in young infants with congenital UT malformation, that the risk diminishes considerably or disappears after 3 months of age and that in the absence of UT malformation pyelonephritis does not cause acute sodium loss of clinical relevance.

The renal collecting system after medullary damage.

The micromorphology of the renal collecting system after unilateral papillectomy in rabbits was investigated. Two weeks to six months after papillectomy, tubular micropuncture studies were performed. Cleared specimens were studied by light microscopy and microradiography. Histologic and physiologic correlations were made. At two to four weeks, the papillectomized kidneys were smaller and had smooth, mottled surfaces. At one to six months, there was cortical scarring with decreased concentrating ability. Ductal micropuncture revealed dilated collecting tubules and ducts which were obstructed at the papillectomy site. It is concluded that obstructed collecting ducts may be responsible for the cortical atrophy and are the primary cause of renal cortical damage after papillectomy in our model.

Renal papillary necrosis in thyroid carcinoma.

A thirty-four-year-old man with progressive visual impairment was found to have thyromegaly and renal insufficiency at the time of admission. Subsequent evaluation demonstrated bilateral optic neuritis and a thyroid nodule which proved to be a follicular carcinoma. Nephrologic studies revealed bilateral papillary necrosis and chronic interstitial nephritis on biopsy. The patient's renal function stabilized and twenty-four-hour protein excretion diminished after hemithyroidectomy. Carcinoma-related protein production may have played a role in the development of the observed renal lesions. Carcinoma should be a consideration in patients with unexplained papillary necrosis.

Radiolucent defects within renal pelvis.

Radiolucent filling defects within the renal pelvis are common findings in diagnositc urography, and because of their myriad causes the diagnostician is often faced with a challenging problem. Several of the more unusual causes of renal pelvis filling defects are described and their diagnosis, pathophysiology, and therapy discussed.

Urologic complications of renal papillary necrosis.

Fifty-six patients demonstrating roentgenographic evidence of renal papillary necrosis were reviewed. Seven patients had an acute fulminating form of the disease. Urologic complication incident to sloughed renal papillae required urgent surgical intervention. Management ranging from ureteral catheterization to nephrectomy in 3 of 7 patients is discussed, and associated disease states are scrutinized.

Simultaneous occurrence of transitional cell carcinoma and urothelial adenocarcinoma associated with xanthogranulomatous pyelonephritis.

We report on a patient with xanthogranulomatous pyelonephritis, in situ transitional cell carcinoma, and focal prosoplasia revealing abrupt conversion of transitional epithelium to moderately well-differentiated adenocarcinoma. The etiology and pathogenesis of mucinous adenocarcinoma in the renal pelvis very likely involves the prosoplastic transition of pre-existing transitional carcinoma to adenocarcinoma. The urologist should be aware of the increased possibility of this tumor developing in a patient with longstanding infection, and frozen section should be performed more often because the gross structure of the tumor frequently appears normal. This permits the urologist to change his surgical strategy if frozen section is positive for tumor.

The role of apoptosis in the development of renal cortical tubular atrophy associated with healed experimental renal papillary necrosis.

An animal model of chronic analgesic nephropathy, in which renal papillary necrosis was induced by the administration of a single injection of bromoethylamine 2-hydrobromide in male Sprague-Dawley rats, was used to investigate the pathogenesis of the atrophy of tubules that leads to cortical atrophy or 'scarring' in analgesic nephropathy. One of the major objectives was documentation of the participation of apoptosis, a distinctive mode of cell death, in the process of cortical tubular atrophy. Control and treated groups of animals were studied at 2 wks, and at subsequent monthly intervals up to 4 mths. At each time, light microscopy and ultrastructure were used to relate changes in cellular pathology to alterations in renal mass. Apoptosis was quantitated in paraffin sections, and autoradiographic identification of cells showing tritiated thymidine uptake was used as an indication of cell proliferation. In animals with total renal papillary necrosis (RPN), focal or diffuse cortical atrophy developed, the extent of which appeared to be proportional to the extent of the RPN. Renal mass was reduced only in those kidneys that developed extensive, diffuse lesions. Compensatory renal growth occurred in the areas of healthy tissue adjacent to the foci of atrophy, with both cellular hyperplasia and hypertrophy playing roles in its development. One of the prominent cellular events was the appearance of apoptotic cells and bodies, with invading intraepithelial macrophages involved in their phagocytosis and degradation. We propose that this form of cell death plays an important role in the pathogenesis of cortical atrophy. Current descriptions of the cortical lesions that occur in analgesic nephropathy refer to the changes as 'scars'. Although the focal lesions have a macroscopic appearance that resembles scars, the results of the present study indicate that usage of this terminology may be misleading, since scarring is often described after severe tissue injury or necrosis, which was not identified in the present study.

Prostaglandin E1 for renal papillary necrosis in a patient with diabetes mellitus.

We report a case of renal papillary necrosis with diabetes mellitus which was treated with prostaglandin E1. An intravenous infusion of 40 mg/day prostaglandin E1 was given for 14 days in an attempt to improve renal circulation. Treatment resulted in an improved creatinine clearance, renal plasma flow and renogram, and proteinuria was decreased. The administration of prostaglandin E1 produced an improvement in renal haemodynamics and can be considered as a possible therapy for renal papillary necrosis in diabetic patients.

Paraproteinaemia and renal papillary necrosis in three women: coincidence or not?

Three women with acute-on-chronic renal failure and renal papillary necrosis were found to have a paraproteinaemia. One of the patients also had Bence Jones proteinuria. Although this may be a coincidental finding, it is suggested that the paraproteinaemia may be a sequel to analgesic nephropathy.

Renal papillary necrosis in diabetes mellitus.

Renal papillary necrosis in 4 diabetic patients is described. Two of them had underlying diabetic nephropathy. Urinary tract infection was present in all of them. Three patients had passed fleshy material in the urine while in one the diagnosis was established by excretory urography. Two patients required haemodialysis for acute renal failure caused by sloughed papillae. The condition should be suspected in diabetic subjects who develop recurrent episodes of urinary tract infection, renal colic, haematuria or obstructive uropathy.