“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”

Kamel El-Reshaid,Shaikha Al-Bader,Hossameldin Sallam,Yoshihide Fujigaki

doi:10.1155/2021/3558278

Abstract

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.

Highlights

Congenital chloride-losing diarrhea (CCLD) is a rare genetic disorder due to autosomal recessive mutation in the SLC26A3 gene on chromosome 7. e latter leads to defective intestinal chloride-bicarbonate exchanges, in epithelial sodium (Na)/hydrogen (H) transport through the Na/ H exchangers (NHE2 and/or NHE3), with the resultant intestinal loss of chloride and retention of bicarbonate [1]
In Arab, only few cases have been reported in Saudi Arabia and Kuwait [5, 6]. e overall longterm prognosis is favorable with optimal life-long salt and K. Substitution though complications such as renal disease, hyperuricemia, inguinal hernias, spermatoceles, and decreased fertility reported in incompliant patients [7]. In this case report; we describe an adult patient, with this rare disease, who lost his kidneys due to chronic analgesic abuse for maltreated gouty arthritis
With adequate salt and K, are essential for normal growth and development and prevention of other severe complications. e optimal dosage of electrolytes are Na 6 mmol/kg/day, K 6.5 mmol/kg/day, and Cl ranging from 12.5 mmol/kg/day, mmol/kg/day in infants, and less in older patients [5, 7]. e long-term disease complications have been attributed to chronic hypovolemia-induced nephrocalcinosis [10]

Summary

Introduction

Congenital chloride-losing diarrhea (CCLD) is a rare genetic disorder due to autosomal recessive mutation in the SLC26A3 gene on chromosome 7. e latter leads to defective intestinal chloride-bicarbonate exchanges, in epithelial sodium (Na)/hydrogen (H) transport through the Na/ H exchangers (NHE2 and/or NHE3), with the resultant intestinal loss of chloride and retention of bicarbonate [1]. Congenital chloride-losing diarrhea (CCLD) is a rare genetic disorder due to autosomal recessive mutation in the SLC26A3 gene on chromosome 7. E first description of the disease was in 1945 by Gamble and Darrow, and nearly 250 cases have been reported to date [3]. E overall longterm prognosis is favorable with optimal life-long salt and K substitution though complications such as renal disease, hyperuricemia, inguinal hernias, spermatoceles, and decreased fertility reported in incompliant patients [7]. In this case report; we describe an adult patient, with this rare disease, who lost his kidneys due to chronic analgesic abuse for maltreated gouty arthritis

The Case

Discussion