Posttransplant lymphoproliferative disease (PTLD) represents a diverse lymphoproliferative disorder ranging from nonspecific reactive hyperplasia to malignant immunoblastic sarcoma developed in a setting of immunosuppression following organ or cellular transplantation. It is often associated with Epstein-Barr virus (EBV) infection and high dose immunosuppression. PTLD after renal transplantation was reported at first in adult in Korea in 1997. In children there have been several cases of PTLD after liver transplantation but PTLD after renal transplantation has not been reported. This is a case report of PTLD developed 4 months after renal transplantation in a 9-year-old boy. The major clinical manifestations were fever, multiple lymph nodes enlargement and blood-tinged stool. EBV was detected by in-situ hybridization in the enlarged cervical lymph node and the colonic tissue. Histological examination revealed B-cell lineage. Use of ganciclovir and reduction of the immunosuppression level resulted in complete remission of PTLD. This is the first pediatric case report of PTLD following renal transplantation in Korea.
Adult ; B-Lymphocytes ; Child* ; Colon ; Fever ; Ganciclovir ; Herpesvirus 4, Human ; Humans ; Hyperplasia ; Immunosuppression ; Kidney Transplantation* ; Korea ; Liver Transplantation ; Lymph Nodes ; Lymphoma, Large-Cell, Immunoblastic ; Lymphoproliferative Disorders ; Male

A 9-year-old boy of B blood group with end-stage renal disease due to IgA nephropathy received group O kidney transplantation from his father. On day 9, he developed intravascular hemolysis, and anti-B autoantibody formation was confirmed. We diagnosed as immune hemolytic anemia due to passenger lymphocyte from donor, and cyclosporine withdrawl was done. Anemia resolved spontaneously, but on day 18, graft dysfunction developed, and graft biopsy revealed acute allograft rejection. Although hemolysis due to autoantibody is very rare and often mild, and the role of hemoglobinuria on acute rejection in this case is not certain, we recommend consideration of aggressive management on severe hemolysis after minor mismatched kidney transplantation.
Allografts ; Anemia ; Anemia, Hemolytic ; Biopsy ; Child ; Cyclosporine ; Fathers ; Glomerulonephritis, IGA ; Hemoglobinuria ; Hemolysis* ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Lymphocytes ; Male ; Tissue Donors ; Transplants

Takayasu's arteritis(TA) is a chronic idiopathic vasculitis mainly involving the aorta and its main branches, such as brachiocephalic, carotid, subclavian, vertebral and renal arteries, as well as coronary and pulmonary arteries. The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of involved arteries. We experienced a case of idiopathic Takayasu's arteritis with negative tuberculin test involving multiple main branch arteries at active stage without pulse. We treated this patient with combined therapy of steroid and azathioprine, with remission of disease activity.
Aorta ; Arteries ; Azathioprine ; Constriction, Pathologic ; Extremities ; Humans ; Ischemia ; Pulmonary Artery ; Renal Artery ; Takayasu Arteritis* ; Tuberculin Test ; Vasculitis

The tethered cord syndrome is characterized by sensory and motor disturbances of the lower extremities and incontinence. We report a 12-year-old boy with sacral lipoma and a tethered cord syndrome, whose chief complaint is enuresis. This complication of tethered cord syndrome was most likely due to a renal failure and hydronephrosis secondary to a neurogenic bladder. Thus, the spinal lesions have to be considered in patients with enuresis.
Child ; Enuresis* ; Humans ; Hydronephrosis ; Lipoma* ; Lower Extremity ; Male ; Neural Tube Defects* ; Renal Insufficiency* ; Urinary Bladder, Neurogenic

We report two cases of hemolytic uremic syndrome (HUS) associated with Escherichia coli O114. Two cases were similar and showed the same clinical courses. After prodrome of diarrhea and vomiting lasting 1-2 days, azotemia persisted for about 10 days, and during that period, the patients were on peritoneal dialysis. They recovered without any sequelae after about 15 days. Direct multiplex PCR of stool culture revealed eae and stx2 gene and the result of ELISA done on the colony positive of eae gene confirmed Escherichia coli O114. This is the first report of HUS associated with Escherichia coli O114. We recommend, Shiga toxin producing bacterial infection must be considered and efforts should be made to scrutinize the organism in all diarrhea-prodrome HUS patients.
Azotemia ; Bacterial Infections ; Diarrhea ; Enzyme-Linked Immunosorbent Assay ; Escherichia coli* ; Escherichia* ; Hemolytic-Uremic Syndrome* ; Humans ; Multiplex Polymerase Chain Reaction ; Peritoneal Dialysis ; Shiga Toxin ; Vomiting

Fraley's syndrome is a disorder of vascular impression on the renal infundibulum which is associated with unexplained disabling flank pain, painless microscopic hematuria, and urinary tract infection. We experienced one case of Fraley's syndrome in a 12-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures.
Child ; Female ; Flank Pain ; Hematuria* ; Humans ; Urinary Tract Infections

Oligomeganephronia is rare congenital anomaly characterized by striking reduction of the number of nephrons, which are markedly hypertrophied in renal hypoplasia. Since the first description of oligomeganephronia in bilateral renal hypoplasia in 1962, dozens of cases were reported. Van Acker reported the first case of oligomeganephronia developed in the solitary kidney and 8 cases were searched in the literature. We report a case of oligomeganephronia in the solitary kidney in 13 years old boy, presented with isolated proteinuria as a first case in Korea.
Adolescent ; Humans ; Kidney* ; Korea ; Male ; Nephrons ; Proteinuria* ; Strikes, Employee

PURPOSE: Phosphodiesterase (PDE) inhibitor increases the cellular content of cAMP, and cAMP suppresses connective tissue growth factor (CTGF) expression induced by TGF-beta1. Therefore, we investigated whether PDE inhibitor suppresses renal fibrosis without suppression of TGF-beta. MATERIALS AND METHODS: Renal interstitial fibrosis was produced by ligation of left ureter in Sprague-Dawley rats. Cilostazol, a selective PDE3 inhibitor, and dipyridamole, a hybrid PDE5, PDE6, and PDE8 inhibitor, were provided in drinking water for 7 days. In addition to the Masson-trichrome score of renal tissue, the concentration of fibronectin and TGF-beta1 in renal tissue-conditioned media was measured by ELISA. RESULTS: Masson-trichrome score and fibronectin concentration were significantly lower in cilostazol-treated group compared to the control group (P<0.05). Though dipyridamole treatment seemed to suppress the Masson-trichrome score and fibronectin concentration too, the decrements were not statistically significant. There was no difference in TGF-beta1 concentration among the groups. CONCLUSION: A selective PDE3 inhibitor cilostazol suppresses renal fibrosis without alteration of TGF-beta expression.
Animals ; Connective Tissue Growth Factor ; Dipyridamole ; Drinking Water ; Enzyme-Linked Immunosorbent Assay ; Fibronectins ; Fibrosis* ; Ligation ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta ; Transforming Growth Factor beta1 ; Ureter* ; Ureteral Obstruction*

No abstract available.

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Schonlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.
Ambulatory Care Facilities ; Child ; Emergencies ; Epididymis ; Epididymitis ; Exanthema ; Humans ; Infant ; Infant, Newborn ; Male ; Porphyrins ; Purpura, Schoenlein-Henoch ; Scrotum ; Spermatic Cord Torsion ; Testis ; Ultrasonography, Doppler