Case 1: The case of the irritable nephrotic

Abstract

A 22-month-old previously healthy boy presented to a tertiary care emergency department with a one-week history of periorbital and bilateral leg swelling. He had no fever, no recent illnesses and no blood in his urine or stool. He was known to have chronic constipation and had a poor diet consisting mainly of cow’s milk. On physical examination, he had no hypertension, was in no apparent distress and displayed normal vital signs. He had marked periorbital edema and pitting edema of his lower limbs. His abdomen was distended and he had mild ascites. The rest of his examination was normal. A urine dipstick showed a high protein level (20 g/L) and a large amount of blood. He had low serum albumin of 18 g/L (normal 32 g/L to 56 g/L), high serum triglycerides of 2.17 g/L (normal 0.31 g/L to 1.41 g/L) and high serum cholesterol of 8.82 mmol/L (normal 3.2 mmol/L to 4.4 mmol/L). His electrolytes and renal function were normal. A complete blood count revealed a low hemoglobin of 79 g/L (normal 110 g/L to 140 g/L) with a low mean corpuscular volume of 53 fL (normal 80 fL to 94 fL). His platelet count was elevated at 934×109/L (normal 150×109/L to 400×109/L). A blood film showed marked hypochromasia and microcytosis. His iron and ferritin levels were both low. He was diagnosed with nephrotic syndrome, as well as iron deficiency anemia (secondary to his poor diet), and was admitted to the hospital. He was initially treated with intravenous methylprednisone because he could not tolerate oral prednisone. He was also started on iron supplementation for his iron deficiency. In addition, the nephrology department recommended starting him on dipyridamole, an antiplatelet agent, because his thrombocytosis and nephrotic syndrome put him at an increased risk for thrombosis. Two days after admission, he was found to be hypertensive and more edematous. He was then treated with two doses of intravenous albumin and furosemide. The next day, his edema had significantly improved. He was switched to oral prednisone and seemed to be doing much better. However, the next day he began vomiting his medications, and was unhappy and irritable. It was thought that he had gastritis from the prednisone and was started on ranitidine. His parents thought he may be constipated and he was also started on lactulose. He had a normal neurological examination. Two days later, he was able to tolerate his medications, and was discharged home despite his irritability. Close outpatient follow-up was arranged. Two days after his discharge, he was seen in the nephrology clinic and was found to be extremely irritable and inconsolable. He had a normal examination, and his edema was markedly improved. He was readmitted to the hospital, and further investigations revealed the etiology of his symptoms.