A Case of Posttransplant Lymphoproliferative Disease (PTLD) Following Renal Transplantation in a Child

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.

Clinical Profiles and Anatomic Classification of Intestinal Malrotation

PURPOSE: Intestinal malrotation is a developmental anomaly and may be complicated by volvulus and intestinal necrosis. We performed anatomic classification and compared clinical profiles to understand their relationship. METHODS: Twenty-eight children were diagnosed as intestinal malrotation at Seoul National University Children's Hospital between Jan. 1980 to Dec. 1995. The patients were classified into 4 groups by age at initial presentation(=x). [group 1 : x<1 week, group 2 : 1 week< OR =x<1 month, group 3 : 1 month< OR =x<1 year, group 4 : 1 year< OR =x] Anatomic classification was performed by radiologic and operative findings. RESULTS: There were 20 males and 8 females. More than half(15/28) of the patients presented in the first month of life, and most of whom presented with bilous vomiting. Most patients older than 1 year of age presented with recurrent abdominal pain and vomiting. The anatomic classification contained 12 patients of type I, 3 patients of type II(IIA : 1, IIC : 2) and 13 patients of type III(IIIA : 5, IIIB : 8). Volvulus occurred in patients of type I and type IIIA and Ladd's band was a major cause of obstruction in patients of type IIIB. Twenty-six patients underwent operation and most had good outcomes except 3 patients. CONCLUSION: Intestinal malrotation should be considered in children with chronic abdominal pain and vomiting as well as infants with bilous vomiting. By performing anatomic classification, we can understand more precisely this disease and care for patients more effectively.

Congenital Esophageal Stenosis: with Special Reference to Diagnosis and Postoperative Complications

PURPOSE: Congenital esophageal stenosis(CES) is one of the rare causes of recurrent vomiting during infancy and childhood. We studied the diagnostic and therapeutic tools and postoperative complications for early diagnosis and adequate management of CES. METHODS: Fourteen cases of CES were evaluated for clinical manifestations, findings of esophagogram and esophagoscopy, classification of pathologic findings and postoperative complications. RESULTS: Most common clinical manifestations at onset were non-projectile vomiting(14), dysphagia to solids(13). Age at onset of symptoms corresponded with the introduction of solids in 11 cases. Esophagogram showed segmental stenosis of variable length in the lower portion of the esophagus in all cases with marked proximal dilatation in 11 cases. Esophagoscopy revealed no signs of esophagitis or ulcer at the area of stenosis. Segmental resection and primary anastomosis were performed as a definitive treatment modality in all cases except one with fibromuscular stenosis. Bronchial cartilage were present in all cases of tracheobronchial remnants(10). Abnormal arrangement and thickening of muscularis mucosae and inner circular muscle were found in all cases of fibromuscular stenosis(4). Postoperative complications were gastroesophageal reflux(5), stricture of anastomotic sites, reflux esophagitis, and so on. CONCLUSION: CES is rare but should be considered as a cause of recurrent vomiting and dysphagia to solid food beginning in infancy and childhood especially in the weaning period. Esophagogram and esophagoscopy are useful tools for diagnosis and differential diagnosis. The stricture of anastomosis site, gastroesophageal reflux and esophagitis need to be evaluated in the follow-up postoperative periods.