Severe hypertension and encephalopathy due to renin- producing hepatoblastoma

Paraneoplastic syndromes result from secretion of hormones, peptides or cytokines by tumor or immune cross-reactivity between malignant and normal tissues. These conditions are rare in children, but when the clinical presentation of patients with a tumor is unusual, these syndromes should be emphasized. Extrarenal tumors with renin-secretion are rare in children. They may be related to paraneoplastic syndromes. We report a 22-month-old infant with hepatoblastoma presented with severe hypertension and related neurologic symptoms due to high plasma renin activity. To the best of our knowledge, this is the second report of renin producing hepatoblastoma in the literature. However, due to lack of laboratory facilities such as immunohistochemical study or polyclonal antibody for human renin activity, we could not prove the secretion of renin just by tumor tissue cells, but this potentiality is very likely. Other intensive investigations did not show any other origin for rennin secretion or hypertension in this patient

Quantification of proteinuria with urinary protein to osmolality ratios in children with and without renal insufficiency

Spot urine is recommended as an accurate method to determine proteinuria in children and adults. However, urinary excretion of creatinine may vary in newborns and spot urine may be influenced by the hydration-dehydration condition of patients. The study was done to assess the validity of the urine protein to osmolality ratio versus the urine protein to creatinine ratio in health and disease conditions. We studied the correlation of the urine protein-osmolality ratio [Uprot/Uosm] and the urine protein to creatinine ratio [Up/Ucr] and compared results with the 24-hour urinary protein excretion. Three groups were compared: children with normal renal function and without proteinuria [group 1, n=53], children with normal renal function and with proteinuria [group 2, n=52] and patients with renal insufficiency [group 3, n=45]. Early morning urine samples and 24-hour urine specimens were collected for protein, creatinine, and osmolality. The optimal cutoff value of the Uprot/Uosm ratio was determined to be 0.33 mg/L/mosm/kgH2O for abnormal proteinuria and 1.75 mg/L/mosm/kgH2O for nephrotic range proteinuria. In comparing ROC curves, we found no differences between the Uprot/Uosm and Up/Ucr ratios in detecting abnormal proteinuria or nephrotic syndrome in children with normal or decreased renal function [P>0.05]. Both the Uprot/Uosm and Up/Ucr ratios from random urine specimens are good predictors of 24-hour urinary total protein excretion in children with and without renal insufficiency