Polycystic horseshoe kidney

AK Prasad¹, R Ram², G Swarnalatha², M Shetty¹, GD Naidu², KV Dakshinamurty²,  
¹ Department of General Medicine, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India
² Department of Nephrology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India

Correspondence Address:
R Ram
Sri Venkateswara Institute of Medical Sciences, Tirupati, AP
India

Full Text

A 34-year-old man was admitted with a history of fever, chill, rigor, and dysuria for 15 days. There was a history of abdominal pain in the right and left lumbar regions and in the hypogastric regions for 1 week. There was a history of vomiting for 4 days. Investigations revealed blood pressure of 120/80 mmHg, pulse rate 100 beats/min and oral temperature was 104°F. On examination, there was tenderness in all quadrants of the abdomen. Cardiovascular and respiratory system examination was unremarkable. Investigations revealed fasting blood glucose 107 mg/dL, serum creatinine 1.2 mg/dL, blood urea 78 mg/dL, serum sodium 134mEq/L, serum potassium 4.2 mEq/L, alanine transaminase 15 U/L, aspartate transaminase 21 U/L, serum alkaline phosphatase 263 U/L, bilirubin 0.6 mg/dL, serum proteins 7.5 g/dL, serum albumin 3.9 g/dL, haemoglobin 14.4 g/dL, total leukocyte count 15,200/cu. mm, differential cell count P70, L25, M4, E1%, platelet count 5.4 lakhs/ cu.mm, urine examination pH 5.00, specific gravity 1,030, albumin 2+, sugar nil, ketones negative, red blood cells nil, White blood cells 2-3/hpf, blood and urine cultures Escherichia coli. Ultrasound of the abdomen and computed tomography (CT) scan of the abdomen showed polycystic horse shoe kidney [Figure 1]. CT of the abdomen revealed cysts in the liver and spleen. The patient was treated according to the antibiogram. At 4 weeks, he was asymptomatic and serum creatinine was 1.4 mg/dL.{Figure 1}

About 20 patients of polycystic horseshoe kidney have been reported in the literature. [1],[2] It is thought to represent two separate renal diseases. Horseshoe kidney is a renal fusion anomaly during embryogenesis; autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder due to mutations in the genes responsible for the expression of the proteins polycystin 1 (ADPKD1) and polycystin 2 (ADPKD2). Horseshoe kidney occurs in 1 per 400-800 live births and ADPKD in 1 in 400-1,000 live births. Polycystic horseshoe kidney occurs 1 in 134 000 to 1 in 8 000 000 live births. [2] To date, no genetic association has been described between the ADPKD loci (PKD1 on chromosome 16 and PKD2 on chromosome 4) and horseshoe kidneys.

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