No abstract available.
Nephrotic Syndrome*

Pyriform sinus fistula (PSF) is a persistent embryologic third or fourth pharyngeal pouch, which typically presents as a congenital sinus tract that originates from the pyriform sinus. PSF is among the most uncommon congenital malformation of the neck. The usual clinical manifestations are either acute thyroiditis or abscess, usually on the left side of the neck. We report a case of PSF in a 9-year old female with a left cervical abscess who had been diagnosed by barium esophagogram and treated endoscopically by injection of histoacryl.
Abscess ; Barium ; Child ; Enbucrilate* ; Female ; Fistula* ; Humans ; Neck ; Pyriform Sinus* ; Thyroid Gland ; Thyroiditis

Kidney allograft transplantation is the most effective method of renal replacement for end stage renal disease patients. Still, it is another kind of 'disease', requiring immunosuppression to keep the allograft from rejection(allograft immune reaction). Immune system of the allograft recipient recognizes the graft as a 'pathogen(foreign or danger)', and the allograft-recognizing commander- in-chief of adaptive immune system, T cell, recruits all the components of immune system for attacking the graft. Proper activation and proliferation of T cell require signals from recognizing proper epitope(processed antigen by antigen presenting cell) via T cell receptor, costimulatory stimuli, and cytokines(IL-2). Thus, most of the immunosuppressive agents suppress the process of T cell activation and proliferation.
Humans ; Immune System ; Immunosuppression ; Immunosuppressive Agents ; Kidney ; Kidney Failure, Chronic ; Receptors, Antigen, T-Cell ; Rejection (Psychology) ; Transplantation Tolerance ; Transplantation, Homologous ; Transplants

For solid organ transplant, ABO blood type of donor and recipient should be compatible in principle. Recent improvement of immunosuppressant made HLA typing not so important while no-mismatch transplant still shows the longest graft survival. PRA(panel reactive antibody) test is to screen and identify recipients with HLA sensitization. When solid organ transplant is scheduled, cross-match test of donor cell and recipient serum should be performed and positive result of cross-match prohibits transplantation. Donor specific antibody (DSA) test can predict the severity of recipient immune reaction against donor organ. Today's mainstay of allograft immunosuppressant regimen is triple therapy of steroid, calcineurin inhibitor(cyclosporine, tacrolimus), azathioprine or mycophenolate mofetil(MMF). Antibody induction using Thymoglobulin or anti-IL-2 receptor antibody(basiliximab or daclizumab) is frequently practiced as well.
Antilymphocyte Serum ; Azathioprine ; Calcineurin ; Graft Survival ; Histocompatibility Testing ; Humans ; Immunosuppression ; Kidney ; Kidney Transplantation ; Tissue Donors ; Transplantation, Homologous ; Transplants

Nocturnal polyuria is one of the main pathogenic mechanisms of enuresis. Disturbance of circadian rhythm of antidiuretic hormone (ADH or AVP), hypercalciuria, and/or solute diuresis are considered to cause nocturnal polyuria, which in turn causes enuresis in patients with relatively small bladder capacity and high threshold for awakening. Evaluation of these factors would guide the therapeutic approach for enuresis.
Circadian Rhythm ; Diuresis ; Enuresis ; Humans ; Hypercalciuria ; Polyuria ; Urinary Bladder

Last year, an epidemic of infantile urinary stone disease developed in China. Investigation revealed that melamine-tainted diary product caused urinary stone in these infants. Young infants were susceptible to the melamine toxicity and dehydration or other stone-prone factors aggravated the toxicity. Melamine-related urinary stones were small, multiple, and mainly composed of uric acid, thus conservative treatment of hydration and urine alkalinization worked well in majority of the patients.
China ; Dehydration ; Humans ; Infant ; Triazines ; Uric Acid ; Urinary Calculi

Pediatric kidney transplantation is the best option since it can achieve near normal glomerular filtration rate, adequate fluid balance, and autonomic endocrine function of the kidney in end-stage kidney disease. However, pediatric kidney transplantation is difficult because children are developing and growing, management and complications of pediatric kidney transplantation are different from those of adults. This review covers the current status of pediatric kidney transplantation in Korea, key considerations that must be taken before kidney transplantation in children, and management strategy of immunosuppression and common complications.

Pediatric kidney transplantation is the best option since it can achieve near normal glomerular filtration rate, adequate fluid balance, and autonomic endocrine function of the kidney in end-stage kidney disease. However, pediatric kidney transplantation is difficult because children are developing and growing, management and complications of pediatric kidney transplantation are different from those of adults. This review covers the current status of pediatric kidney transplantation in Korea, key considerations that must be taken before kidney transplantation in children, and management strategy of immunosuppression and common complications.

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.
Adolescent ; Child ; Cilia ; Diagnosis ; Hearing ; Humans ; Renal Insufficiency, Chronic ; Retina ; Skeleton

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.
Adolescent ; Child ; Cilia ; Diagnosis ; Hearing ; Humans ; Renal Insufficiency, Chronic ; Retina ; Skeleton