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Delayed Presentation of Short Bowel Syndrome Complicated with Severe Degree of Nutritional Deficiencies, Nephrocalcinosis and Distal Renal Tubular Acidosis

Herath T, Kulatunga A

Background: Short bowel syndrome (SBS) is a malabsorptive state due to functional or anatomic loss of extensive segments of small intestine which can lead to nutritional deficiencies and metabolic disarrangements. Here we describe a young patient with short bowel presenting with severe nutritional deficiencies, nephrocalcinosis and distal renal tubular acidosis. Nephrocalcinosis and distal renal tubular acidosis are closely associated and each can lead to the other. There are only rare case reports of short bowel syndrome complicated with nephrocalcinosis and distal RTA and severe degree of metabolic derangement with nutritional deficiencies. And this case highlights the importance of early management of short bowel syndrome in order to prevent long-term complications. Case presentation: A forty two-year-old Sri Lankan male patient who had undergone appendectomy at the age of nineteen years, which was complicated by bowel gangrene and about 240 cm of small bowel was resected. Later he presented with recurrent urolithiasis and urinary tract infections and ten years after the surgery imaging revealed bilateral nephrocalcinosis and obstructive uropathy. Distal Renal tubular acidosis (RTA) was diagnosed at the same time. He also had osteomalacia, proximal myopathy, multiple vitamin deficiencies, mixed deficiency anemia. He was started on calcium, iron supplements, parenteral B12, vitamin D injections and alkali therapy to achieve a normal serum bicarbonate concentration. His calcium and Vitamin D supplementation is currently being monitored with serum calcium, 25-hydroxyvitamin D, serum alkaline phosphatase, serum PTH and urinary calcium/creatinine ratio. Conclusion: Patients with extensive small bowel resection are at risk for nutrient, mineral, and vitamin deficiencies because of the loss of absorptive surface. It can also be complicated with nephrocalcinosis and distal RTA which is rare in short bowel syndrome. It is therefore important to identify patients at risk of short bowel syndrome and institute an early management, follow up plan to prevent complications.