Comparison of the Clinical Characteristics of Intestinal Malrotation in Infants and Children

Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50%) boys and 20 (50%) girls. At the time of operation, 27 (67.5%) patients were younger and 13 (32.5%) were older than 1 year. Vomiting was seen in 20 cases (74.1%) of the younger group compared to 2 cases (15.4%) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4%) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5%) of the younger group and 1 patient (7.7%) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.

Clinical Manifestations of Posttransplantation Lymphoproliferative Disorder (PTLD) after Liver Transplantation / 감염과화학요법

BACKGROUND: Post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation is a rare but potentially fatal disease. Clinical manifestations and prevalence of PTLD after liver transplantation in Korea have not been investigated thoroughly. MATERIALS AND METHODS: A retrospective chart review was done for 284 liver transplant recipients at Samsung Medical Center, Seoul, Korea during the period from 1996 to 2003. RESULTS: The incidence of PTLD after liver transplantation was 3.9% (11/284). PTLDs were more prevalent in children (9/55, 16.4%) than in adults (2/237, 0.9%; P<0.01). Among the PTLD patients, four cases were male (36.3%) and seven were female (63.7%). Median time from the transplantation to PTLD diagnosis was 9 months. The type of PTLD was as follows:early lesion (6 cases, 54.5%), polymorphic PTLD (3 cases, 27.3%), and B cell lymphoma (2 cases, 18.2%). PTLDs were more prevalent in the patients with cyclosporine use (OR 13.28, 95% CI:1.29-136.31, P=0.03), acute rejection (OR 5.63, 95% CI:1.03-30.62, P=0.04), and negative serology for EBV VCA IgG (OR 19.15, 95% CI:1.99-183.98, P=0.01) by multivariate logistic regression. Three patients (27.3%) died of B cell lymphoma (2 cases) and polymorphic PTLD (1 case). The remaining patients were improved with reduction of immunosuppression and treatment with acyclovir. CONCLUSION: The incidence of PTLD was high in children. The risk factors of PTLD were negative serology for EBV VCA IgG, history of acute rejection, and cyclosporine use. Considering the poor prognosis of PTLD, effective strategies for prevention and early diagnosis for early treatment should be emphasized.

Clinical Manifestations of Posttransplantation Lymphoproliferative Disorder (PTLD) after Liver Transplantation / 감염과화학요법

BACKGROUND: Post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation is a rare but potentially fatal disease. Clinical manifestations and prevalence of PTLD after liver transplantation in Korea have not been investigated thoroughly. MATERIALS AND METHODS: A retrospective chart review was done for 284 liver transplant recipients at Samsung Medical Center, Seoul, Korea during the period from 1996 to 2003. RESULTS: The incidence of PTLD after liver transplantation was 3.9% (11/284). PTLDs were more prevalent in children (9/55, 16.4%) than in adults (2/237, 0.9%; P<0.01). Among the PTLD patients, four cases were male (36.3%) and seven were female (63.7%). Median time from the transplantation to PTLD diagnosis was 9 months. The type of PTLD was as follows:early lesion (6 cases, 54.5%), polymorphic PTLD (3 cases, 27.3%), and B cell lymphoma (2 cases, 18.2%). PTLDs were more prevalent in the patients with cyclosporine use (OR 13.28, 95% CI:1.29-136.31, P=0.03), acute rejection (OR 5.63, 95% CI:1.03-30.62, P=0.04), and negative serology for EBV VCA IgG (OR 19.15, 95% CI:1.99-183.98, P=0.01) by multivariate logistic regression. Three patients (27.3%) died of B cell lymphoma (2 cases) and polymorphic PTLD (1 case). The remaining patients were improved with reduction of immunosuppression and treatment with acyclovir. CONCLUSION: The incidence of PTLD was high in children. The risk factors of PTLD were negative serology for EBV VCA IgG, history of acute rejection, and cyclosporine use. Considering the poor prognosis of PTLD, effective strategies for prevention and early diagnosis for early treatment should be emphasized.

Lymphoproliferative Disorder as an Unusual Causen of Stridor in a Post-Liver Transplant Child / 소아알레르기및호흡기학회지

Otolaryngologic symptoms and findings are often the first manifestations of post-transplant lymphoproliferative disorder (PTLD) in children. We report a case of lymphoproliferative disorder which occurring in the epiglottis and aryepiglottic fold of a post-liver transplant, 28-month-old girl. Inspiratory stridor and mild respiratory distress were presented 7 months after the transplantation. Bronchoscopy revealed swelling of the epiglottis, multiple nodular lesions in the arytenoid and the pharyngeal wall, and collapse of the false vocal cord due to a redundant nodular mass during inspiration. Microscopic examination showed polymorphic, polyclonal small lymphoid aggregates, which were dominantly stained by monoclonal antibody to CD20. In this case, PTLD was associated with Epstein-Barr virus (EBV) infection. The patient was treated successfully by the surgical excision of the obstructing redundant mass in the arytenoids, dose reduction of immunosuppressive drugs and the administration of an antiviral agent. A diagnosis of PTLD should be considered in transplanted children who later develop upper airway obstruction.