“Recurrent Papillary Necrosis and Nephrocalcinosis Induced by Nonsteroidal Anti-Inflammatory Drugs for Gouty Arthritis Associated with Congenital Chloride-Losing Diarrhea: A Major Risk for Kidney Loss”

Congenital chloride-losing diarrhea (CCLD) is a rare genetic disorder due to autosomal recessive mutation in the SLC26A3 gene on chromosome 7. It is characterized with chronic watery diarrhea with high fecal chloride (Cl: >90 mmol/L), low potassium (K), and metabolic alkalosis with low urinary Cl and K. The overall long-term prognosis is favorable with optimal life-long salt and K supplementation. In this case report, we describe a man with progressive renal failure and small kidneys that showed nephrocalcinosis and papillary necrosis. His disease was diagnosed since birth and was confirmed by our tests. He was incompliant with therapy and had developed gout. The latter complication of his disease has led to excessive NSAID use over the past years. Reinstitution of diet, drug therapy, and allopurinol had stabilized his renal disease for 1 year of follow-up. In conclusion, excessive analgesic use is a risk factor for renal failure in CCLD.

Uncovering Silent Fungal Infections complicating Obstructive Uropathy due to Renal Papillary Necrosis in Diabetic patients – A step towards preventing Recurrent Obstructive Uropathy.

Introduction and Objectives: Renal papillary necrosis is one of the common causes of obstructive uropa-thy in diabetic patients. During removal of necrosed renal papilla, many patients were observed to have fluffy necrotic material in the ureter, and renal pelvis, and a few among them present again with recurrent episodes of obstructive uropathy and sepsis following DJ stent removal. Our study aimed to identify thesignificance of this fluffy necrotic material and its evaluation by histopathological examination (HPE). Materials and Methods: This is a prospective observational study done in our institute by compiling data of 56 patients admitted with obstructive uropathy secondary to renal papillary necrosis who underwent a protocol-based treatment from 2016 to 2019. All these patients underwent initial DJ stenting followed by check flexible ureteroscopy or nephroscopy after 6 weeks. The white, necrotic fluffy material collected during initial DJ stenting or with subsequent flexible ureteronephroscopy was sent for HPE. All these patients were followed up for 1-3 years. Results: Out of 56 patients, 15 patients had fluffy necrotic material in the bladder on initial cystoscopy, of which 1 patient was diagnosed with aspergillosis and 1 patient with candida infection on HPE. During check flexible ureteroscopy (FU), 19 patients had minimal burden of fluffy necrotic material in renal pelvis, of which one patient was diagnosed with aspergillosis, one with candida (same patient diagnosed on cystoscopy), and one patient with both aspergillus and candida colonies on HPE. 5 patients had the significant burden of fluffy necrotic material in the renal pelvis, requiring removal via percutaneous nephroscopic access. Among these 5 patients, 2 were diagnosed with aspergillosis and 1 with candida infection on HPE. A total of 32 patients had single papilla, and 24 had multiple papillae in the pelvicalyceal system. 5 out of 7 patients with positive fungal pathology had negative fungal cultures. Compared to our historical data of 4% mortality and 22% recurrent obstructive uropathy in the 3 years preceding the adoption of this protocol, with the present protocol, no patient developed recurrent pyelonephritis during follow-up of 1–3 years after DJ stent removal following complete evacuation of necrotic material and appropriate antifungal treatment. Conclusion: This study highlights the need for check ureterorenoscopy and removal of all necrotic papillae and debris to establish a microbiological and histopathological diagnosis along with proper antifungal treatment to prevent episodes of recurrent pyelonephritis and obstructive uropathy.

A Rare Presentation of Renal Papillary Necrosis in a COVID-19-Positive Patient

In this case report we describe an unusual presentation of severe acute papillary necrosis in a COVID-19-positive patient. An emergency flexible ureteroscopy greatly helped in the establishment of the diagnosis. In the international literature, there is a paucity of intraoperative endoscopic images representing severe renal papillary necrosis. We present a case of severe acute renal papillary necrosis in a 49-year-old south-Asian, COVID-19-positive male patient who needed emergency urological intervention for macroscopic hematuria and urinary retention due to clot formation in the urinary bladder. The patient underwent emergency cystoscopy, clot evacuation, and by rigid and flexible ureteroscopy. The diagnosis was only confirmed in the postoperative period, retrospectively. Finally, the patient fully recovered due to the multidisciplinary management. Diagnosis of rare clinical entities can be sometimes challenging in the everyday routine practice. Having atypical clinical course, the surgeon should be prepared and sometimes must take responsible decisions promptly, even if needed intraoperatively, to manage unexpected findings in order to get the right diagnosis without compromising the patient’s safety.

Renal Papillary Necrosis as the First Presenting Clinical Feature in a Sickle Beta and Thalassemic Child

In sickle cell disease (SCD), the clinical manifestations are due to episodes of vascular occlusion and haemolysis. Most of the children experience vaso-occlusive pain episodes by the age of six years. Renal injury in sickle cell disease referred to as sickle cell nephropathy is a frequent yet under-recognised complication. Renal papillary necrosis (RPN) as the first presentation in SCD is rare. We are reporting a 12 year old child with no prior vaso-occlusive episodes, presenting with renal papillary necrosis due to Sickle Beta and Thalassemia.

SAT-363 Renal Papillary Necrosis Associated with Normocalcemic Hyperparathyroidism

IntroductionHypercalciuria is generally considered to be the most common identifiable metabolic risk factor for calcium nephrolithiasis. Important renal manifestations of primary hyperparathyroidism (PHPT) include asymptomatic nephrolithiasis, hypercalciuria, nephrocalcinosis, and chronic renal insufficiency. However renal papillary necrosis (RPN) occurring in PHPT has not been reported previously. We report a 50-year-old woman who manifested RPN associated with hypercalciuria and normocalcemic PHPT. Case ReportA 50-year-old Caucasian woman was evaluated in 2006 for hypercalcemia. She had no history of nephrolithiasis, fractures, or symptoms of hypercalcemia. Laboratory: serum calcium 11.8 mg/dL, ionized calcium 6.3 mg/dL, phosphorus 1.8 mg/dL, intact PTH 98 pg/mL (ref 15–65), urine calcium 543 mg/24 hrs (ref <235). Renal ultrasound showed no evidence of nephrocalcinosis or nephrolithiasis. A parathyroid scan was consistent with a left superior parathyroid adenoma. Patient underwent parathyroidectomy and became normocalcemic with normal serum PTH levels postoperatively. One year later she was diagnosed with a left sided bronchial carcinoid tumor. Surveillance Gallium-68 PET/CT scan done 2 years later was negative for any metastases. Twelve years later she reported to our clinic for follow up. She had no symptoms of hypercalcemia, fractures, nephrolithiasis, history of pyelonephritis, diabetes mellitus, analgesic use, or hypertension. Serum calcium was 9.1 mg/dL, serum phosphorous 3.8mg/dL, PTH 82 pg/mL, 25-OH vitamin D 34 ng/mL, 1,25-vitamin D 38 pg/mL, and a urorisk panel was normal except for a 24-hour urine calcium of 410 mg. However renal ultrasound showed bilateral RPN and this diagnosis was also confirmed by a CT scan. A urinalysis showed only microalbuminuria with no red cells. She had no history of any analgesic drug abuse, pyelonephritis, sickle cell disease, or diabetes mellitus. A glucose tolerance test was completely normal. Discussion RPN is characterized by coagulative necrosis of the renal medullary pyramids and papillae brought on by several associated disorders and toxins that exhibit synergism toward the development of ischemia. Although the initial kidney US was normal, a repeat US done 12 years later showed evidence of RPN. This finding along with hypercalciuria and a diagnosis of normocalcemic PHPT suggests that RPN may be associated with hypercalciuria and normocalcemic PHPT. Furthermore she had no other risk factors for RPN. Additional studies with large number of patients are needed to confirm the association between these 2 disorders.