A 19-month-old African American male was transferred from an outside hospital to our pediatric unit for new-onset status epilepticus and hyponatremia (serum sodium of 126 mmol/L). He initially presented with generalized tonic–clonic seizures unresponsive to rectal Diazepam, but eventually resolved with intravenous Lorazepam after approximately 15 min. Upon admission, the patient appeared lethargic but responsive. Vital signs on admission: blood pressure 218/144, heart rate of 210, respiratory rate was 32, and temperature was 36.8°C. Lab work revealed hyponatremia, an elevated peripheral renin activity, aldosterone and ADH levels, and a normal serum creatinine (Table ). His weight was 10.6 kg (29th‰ for age) and his height was 87.3 cm (88th‰ for age). His physical exam was unremarkable. He was awake and alert, normal heart, and lung sounds. Abdomen was soft, non-tender, and non-distended with no evidence of organomegaly. Extremities were warm with normal pulses and capillary refill.\nElectrocardiogram (ECG) showed sinus tachycardia and a waveform characteristic concerning for left ventricular hypertrophy. Echocardiogram demonstrated normal biventricular size with qualitatively mild concentric left ventricular hypertrophy.\nUpon review of systems, the family revealed a 1 kg weight loss over the course of 10 days. He was seen by a pediatric gastroenterologist for daily non-bloody, non-bilious emesis, and was treated for gastroesophageal reflux disease (GERD). During this time, he also demonstrated signs of polyuria and polydipsia with water intake increasing to 8–10 bottle per day.\nShortly after admission, his blood pressure increased to 248/150 mmHg. He became lethargic and suffered a second episode of seizure activity, which was successfully treated with Lorazepam followed by a loading dose of Phenobarbital. His blood pressure was titrated with a nicardipine infusion for a target range of 120–140 mmHg systolic and 70–80 mmHg diastolic.\nA renal ultrasound revealed a smaller right kidney (length 6.4 cm) as compared to the left (length 7.6 cm). The right kidney when compared to the left had less cortical thickness and increased echogenicity suggestive of vascular insult. The vessels were not completely visualized via color Doppler.\nMRI of the brain without contrast demonstrated features consistent with PRES (Figure ). MRI of the abdomen with contrast showed a small right kidney and a high-grade proximal renal artery stenosis (Figures and A). Upper pole hypointense lesions of the right kidney consistent with long-standing ischemic insults. Diagnostic angiography later confirmed a complete occlusion of his renal artery accompanied by a 20% narrowing of the perirenal aorta. Video EEG was performed which exhibited frequent, focal spikes and sharp waves in the left temporal region. Seizure activity was thought to emanate from accrued pathological alterations in the brain regions specified.\nThe right renal artery was balloon dilated to 50% as shown in Figure B. This was complicated by renal artery re-stenosis, with a peak systolic velocity of 200 cm/s (normal <180 cm/s) on serial renal Doppler ultrasounds. Repeat angiogram showed a 30% opening. Following repeat balloon angioplasty, the patient’s renal artery progressively re-occluded over 15 min requiring a 4 × 14 Genesis stent to maintain adequate flow, as shown in Figures C,D. He was weaned off the nicardipine to amlodipine 2.5 mg in a.m. and 5 mg in p.m. to good effect. Since his hospitalization, the patient has been free of seizures and is neurologically intact.
Eight years old boy presented with complain of headache for a week with increasing thirst and polyuria. He was having altered sensorium and convulsions prior to admission. He appeared hypovolemic with feeble peripheral pulses, possibly contributed by the polyuria. His BP was 184/110 mmHg (95th\nP = 114/75). There was left sided hemiparesis along with 6th and 7th nerve palsy. Ophthalmological examination was consistent for hypertensive retinopathy. Investigations revealed hyponatremic, hypokalemic, metabolic alkalosis along with normal creatinine (Table ). USG with Doppler revealed bilateral hyper-echogenic kidneys with right relatively bigger (7.5 cm) than the left (6.2 cm) and suspected decreased blood flow in the left kidney. Renin and aldosterone was elevated. Digital subtraction angiography (DSA) of kidneys confirmed 90% stenosis of the left renal artery. MRI brain was consistent for PRES (Figure ).