Successful continuous venovenous hemofiltration in a neonate with hyperammonemia from ornithine transcabamylase deficiency.

Ornithine transcabamylase (OTC) deficiency is the most common and severe form of abnormal urea synthesis. It can result in hyperammonemia, severe neurologic manifestation, brain edema, and early death. Rapid removal of ammonia by hemodialysis can decrease mortality and morbidity in the patients with severe increase of ammonia levels.

Clinical outcome of children with Henoch-Schönlein purpura nephritis.

Objective: Evaluate the outcomes of pediatric patients with Henoch-Sch6nlein purpura nephritis andfind the parameters correlated with outcomes of treatment.

Material And Method: Review of medical records was performed in twenty patients diagnosed with Henoch-Schinlein purpura nephritis. Demographic data, clinical parameters and records of treatment at diagnosis and the last visit were collected and analyzed.

Depletion of alloreactive T cells for tolerance induction in a recipient of kidney and hematopoietic stem cell transplantations.

The present case report represents a successful attempt to induce transplantation tolerance to organ allograft by combined administration of donor hematopoietic cells and kidney based on in vivo deletion of alloreactive host-vs-graft and graft-vs-host alloreactive T cells following non-myeloablative conditioning. We were able to induce mixed and eventually full donor chimerism and tolerance of kidney allograft in a 15-yr-old male with ESRD after cisplatin treatment and autologous HSCT for mediastinal germ cell tumor. Our approach to induce tolerance was based on preferential depletion of alloreactive T cells induced by exposure to donor's alloantigens and administration of cyclophosphamide at day 2 and day 3 after stem cell infusion.

Clinical outcome of children with primary distal renal tubular acidosis.

Objective: To evaluate the clinical course of children with primary distal renal tubular acidosis and to determine parameters correlated with the outcomes.

Material And Method: A retrospective review of medical records was carried out. The parameters at initial diagnosis and the last visit were collected including height standard deviation score (SDS), weight SDS, ultrasonography of kidneys, serum electrolytes, urine electrolytes, urine calcium to urine creatinine ratio (urine Ca/Cr), serum creatinine, bicarbonate dosage and glomerular filtration rate estimated by Schwartz's formula (eGFR).

Antineutrophilic cytoplasmic antibody-positive systemic vasculitis associated with propylthiouracil therapy: report of 2 children with Graves' disease.

Systemic vasculitis is a rare complication of therapy with antithyroid medication. Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis has been described in patients treated with propylthiouracil (PTU) and methimazole (MMI). The majority of cases have underlying Graves' disease.

Cystinuria: cause of recurrent renal stones in a 4-year-old girl.

This paper presents the case report of a 4-year and 6-month old girl with cystinuria. She clinically presented with recurrent radiopaque renal stones since the age of 3 years. She received 2 subsequent operations of pyelolithotomy combined with ureterolithotomy at the age of 3 years 6 months, and pyelolithotomy alone at the age of 5 years.

The optimal dose of potassium citrate in the treatment of children with distal renal tubular acidosis.

Background: Distal renal tubular acidosis (RTA) is a common cause of intractable calcium nephrolithiasis. In adults, the use of potassium citrate (PC) in distal RTA effectively decreases metabolic acidosis and the risk of calcium oxalate stone but it cannot decrease the risk of calcium phosphate stone. However, there is no report for the optimal dose of PC and the risk of calcium stone in distal RTA in children.

Dosage of potassium citrate in the correction of urinary abnormalities in pediatric distal renal tubular acidosis patients.

Potassium citrate is an alkaline agent that has been recommended for the prevention of nephrolithiasis in distal renal tubular acidosis (RTA). Information on the effectiveness and the optimal dose of potassium citrate in the correction of urinary abnormalities in pediatric distal RTA is limited, however. We conducted this study to determine the effectiveness and the optimal dose of potassium citrate for the correction of urinary abnormalities and the prevention of nephrolithiasis in children with distal RTA.