Stage 2 hypertension in a child with a rapidly enlarging kidney: question

Abstract

Case summarySevere headache, vomiting, and blurred vision preceded confusion, irrational behaviours and afebrile generalized tonic-clonic seizures by 3 days in this 11-year-old boy. All four seizure episodes occurred within 24 h and before hospital arrival. Each episode lasted 3-5 min. A progressively increasing painless left abdominal swelling preceded all these complaints by 3 weeks. There were no urinary symptoms. He was neither tremulous nor sweating excessively.The patient was drowsy, with a Glasgow coma score of 10/15. There was neither neck stiffness nor positive Kerning's sign. Muscle tone and tendon reflexes were normal. His blood pressure (BP), 160/120 mmHg (mean arterial pressure, 133 mmHg), was in the stage 2 hypertension (HTN) range (> 5 mmHg above the 99th percentile for age, gender and height [1]). There was no femoral pulse delay. Temperature, pulse and respiratory rate were 36.5°C, 96 beats/min, and 26 cycles/min, respectively. He was neither pale, dehydrated nor dyspnoeic. There was no papilloedema. Skin lesions suggestive of neurofibromatosis or systemic lupus erythematosus were absent. There was generalized discrete, painless, significant peripheral lymphadenopathy. Moderate ascites was present, but no peripheral oedema. The left kidney was markedly enlarged, but the right kidney was not palpable. Spleen and liver were not palpable. He had no abdominal bruit. His weight, height and body mass index were 28.0 kg, 131.0 cm and 16.32 kg/m 2 , respectively. Table 1 shows the results of biochemical and renal ultrasound investigations at baseline and at 4 weeks. Pretreatment haematocrit was 34.0%; white blood cell count, differentials, and platelets count were within normal limits. Haemoglobin electrophoresis was AS. Serology was negative for hepatitis B and C viruses and human immunodeficiency virus. Figure 1a shows a remarkably enlarged hypoechoic left kidney, with loss of corticomedullary differentiation. The right kidney was normal (Fig. 1b). The ultrasound was repeated 24 h later when an increase in the left kidney size was clinically observed as a posterior bulge in the lumbar region. It revealed a more than threefold increase in the kidney volume (Table 1, footnote a). Right kidney dimensions remained unchanged. Cerebrospinal fluid and bone marrow examinations were normal. The provisional diagnosis was nephroblastoma. The final diagnosis, however, turned out not to be nephroblastoma. His response to definitive treatment was good. He was discharged home for completion of treatment as outpatient on admission day 45 but was immediately lost to follow-up. His discharge BP was 90/60 mmHg.The answer to this question can be found at http://dx.