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Tawadrous H, Kamran H, Salciccioli L, Schoeneman MJ, Lazar J. Evaluation of arterial structure and function in pediatric patients with end-stage renal disease on dialysis and after renal transplantation. Abstract: CVD is a major cause of morbidity and mortality in pediatric patients with CKD. It is unclear whether vascular abnormalities in these patients are reversible, and if transplantation portends salutary effects on arterial function. We compared FMD, PWV, AI75, and CIMT in 15 dialysis (D), 14 transplant patients (T), and 15 controls (C), and their associations with cardiovascular risk factors. There was stepwise lower FMD (p

The differential diagnosis of macroscopic hematuria in patients with sickle cell disease (hgb SS), and more rarely in sickle cell trait (hgb AS), includes papillary necrosis, renal infarction, membranoproliferative glomerulonephritis associated with the hepatitis C virus, interstitial nephritis due to analgesic abuse, pyelonephritis, nephrolithiasis and, very rarely, a renal malignancy, such as renal medullary carcinoma. Acute papillary necrosis is often suspected and is frequently the cause, but further studies are mandatory if the hematuria persists. In this case, the renal ultrasound (Fig. 1) demonstrated an echogenic mass in the upper pole of the left kidney. The computed tomography (CT) scan with intravenous contrast (Fig. 2) revealed a heterogeneous mass in the same location. A tissue diagnosis was required to confirm the diagnosis, and the patient underwent unilateral nephrectomy. Figure 3 shows the gross pathology of the tumor found in the upper pole of the left kidney. The pathologic diagnosis was renal cell carcinoma, papillary cell type, collecting-duct sub-type. Figure 4 shows the microscopic pathology. Our patient had no evidence of metastases and appears to be completely well after 6 months of follow-up.

Background: The mineralocorticoid receptor is protected from excess of glucocorticoids by conversion of active cortisol to inactive cortisone by enzyme 11β-hydroxysteroid dehydrogenase type 2 present in the kidney. The metabolites of cortisol and cortisone are excreted in the urine as tetrahydrocortisol (5αTHF+5βTHF) and tetrahydrocortisone (THE), respectively. Hypothesis: Patients with chronic kidney disease (CKD) and essential hypertension have a functional defect in their ability to convert cortisol to cortisone, thus leading to the activation of mineralocorticoid receptor. Objective: The objective of the investigation was to study the ratio of urinary steroids (5αTHF+5βTHF) to THE in patients with CKD, postrenal transplant, and essential hypertension and to compare the ratio with controls. Design/Methods: We enrolled 44 patients (17 with CKD, eight postrenal transplant, 19 with essential hypertension) and 12 controls. We measured spot urinary 5α-THF, 5β-THF, THE, free active cortisol and inactive cortisone by gas chromatography/mass spectrometry. We collected data on age, sex, cause of kidney disease, height, weight, body mass index, blood pressure, serum electrolytes, aldosterone, and plasma renin activity. Blood pressure percentiles and z-scores were calculated. The glomerular filtration rate was calculated using the modified Schwartz formula. Results: The ratios of 5αTHF+5βTHF to THE were significantly higher in patients with CKD [mean ± sd score (SDS) = 1.31 ± 1.07] as compared with essential hypertension (mean ± SDS = 0.59 ± 0.23; P = 0.02) and controls (mean ± SDS = 0.52 ± 0.25; P = 0.01). In the postrenal transplant group, the ratio was not significantly different (mean ± SDS = 0.71 ± 0.55). The urinary free cortisol to free cortisone ratios were significantly higher in the hypertension and CKD groups as compared with the controls. The 5αTHF+5βTHF to THE ratio negatively correlated with the glomerular filtration rate and positively correlated with systolic and diastolic blood pressure z-scores. The correlation of the blood pressure z-scores with ratios was stronger in the CKD group than the essential hypertension and posttransplant groups. Conclusions: We have elucidated a functional deficiency of 11β-hydroxysteroid dehydrogenase type 2 in children with CKD and a subset of essential hypertension. Urinary 5α-THF, 5β-THF, and THE analysis by gas chromatography/mass spectrometry should be a part of routine work-up of CKD and hypertensive patients.

An 11-year-old African American boy with sickle cell trait (hemoglobin AS) was admitted for evaluation of intermittent painless gross hematuria for 3 months. Initially, the hematuria occurred once every 2 weeks, but it had increased in frequency to two to three times per week. He denied colicky abdominal pain, urinary frequency, or urgency. There was no history of recent trauma. He had never had a urinary tract infection. He was taking no medications and had no recent history of exposure to high altitude. The family history and his review of systems were otherwise unremarkable. Physical examination revealed: weight, 60 kg (>97%); height, 155 cm (95%); temperature, 98°F; heart rate, 87/min; respirations, 18/min; blood pressure, 116/68 mmHg. In general, the patient appeared healthy and not in acute distress. He had no pallor. The abdomen was soft, with mild tenderness in the right upper quadrant. The remainder of the examination was normal. Admission laboratory testing revealed: white blood cell count, 6760/mm; hemoglobin, 12.3 g/dL; hematocrit, 37.7%; mean corpuscular volume, 73.9 fL; platelet count, 390,000/ mm; sodium, 135 mmol/L; potassium, 4.2 mmol/L; chloride, 105 mmol/L; bicarbonate, 21 mmol/L; blood urinary nitrogen, 10 mg/dL; creatinine, 0.54 mg/dL; glucose 107 mg/dL; calcium, 9.5mg/dL; total protein, 7.1 g/dL; albumin, 4.4 g/dL; uric acid, 4 mg/dL; Creactive protein, 1.4 mg/dL (normal); C3 complement, 78 mg/dL (normal); ratio of urinary calcium to creatinine, 0.05 (normal