The Role of Capsule Endoscopy in the Diagnosis of Crohn's Disease / 대한소아소화기영양학회지

The examination of small bowel in Crohn's disease (CD) is very important. Capsule endoscopy (CE) has been recognized as a good tool for evaluation of small bowel. The capsule placement is achieved endoscopically for Children not to swallow capsule. CE is superior to any other modalities for examination of small-bowel. The large portion of pediatric patients with known CD were found with CE to have more extensive and newly diagnostic small-bowel disease. All of them had therapeutic changes. The most side effect of CE is capsule retention. The capsule retention rate in pediatric CD is about 7.3%. The patency capsule helps to predict the possibility of capsule retention. For the improving of the diagnostic accuracy, the experience of more than 20 readings of CE is needed.

A Case of Enterovirus 71 Infection Presented with Acute Flaccid Paralysis / 대한소아신경학회지

Hand-foot-mouth disease (HFMD) is characterized by distinctive skin lesions on the hand, foot, and mouth and in general, recovery occurs within one week. However, in cases of HFMD by enterovirus 71 (EV71) infection, the development of neurologic complications such as brainstem encephalitis, acute flaccid paralysis, and aseptic meningitis has been common. Moreover, it has been reported that some patients with neuologic complications have expired in severe cases. Here, we report a case of EV71 infection presented with acute flaccid paralysis of a single similar to paralytic poliomyelitis.

Infections after Living Donor Liver Transplantation in Children

The aim of this study was to evaluate the infectious complications after living donor liver transplantation (LDLT) in children. We enrolled 95 children (38 boys and 57 girls) who underwent LDLT from 1994 to 2004. The median age was 22 months (range, 6 months to 15 yr). We retrospectively investigated the proven episodes of bacterial, viral, and fungal infection. There occurred 150 infections in 67 (70%) of 95 patients (1.49 infections/patient); 74 in 43 patients were bacterial, 2 in 2 were fungal, and 74 in 42 were viral. The most common sites of bacterial infection were the bloodstream (33%) and abdomen (25%). Most of the bacterial infections occurred within the first month after LDLT. Bacterial and fungal infections did not result in any deaths. The most common causes of viral infection were Epstein-Barr virus in 37 patients and cytomegalovirus in 18. Seven of the 14 deaths after LDLT were associated with viral infection. Our study suggests that infection is one of the important causes of morbidity and mortality after LDLT. Especially careful monitoring and management of viral infections is crucial for improving the outcome of LDLT in children.

A Case of Hemophilia A Diagnosed in a Premature Infant

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate(R), USA) because of repeated seizures and intramuscular hematoma.

The Clinical Significance of Soluble Intercellular Adhesion Molecule-1 (sICAM-1) and Soluble Vascular Cell Adhesion Molecule-1 (sVCAM-1) in Kawasaki Disease / 소아과

PURPOSE: Kawasaki disease (KD) is known as an acute multi-systemic vasculitis with various immunologic abnormalities. Adhesion of leukocyte to endothelial cells is a key event in the sequence of inflammatory response. This study was performed to investigate the clinical significance of serum soluble intercellular adhesion molecule-1 (sICAM-1) and soluble vascular cell adhesion molecule-1 (sVCAM-1) in acute and subacute stages of typical KD for diagnostic and prognostic value. METHODS: A typical KD group was 32 patients who were hospitalized from Jan 2002 to Jun. 2004 was enrolled. Control was 16 non-KD patients with febrile illness. sICAM-1 and sVCAM-1 were measured and compared by Echocardiographic and clinical findings and cardiac troponin T and I. RESULTS: sICAM-1 and sVCAM-1 levels of acute KD were significantly elevated over control (P= 0.019 vs. P=0.049, respectively) and sICAM-1 was significantly decreased in subacute stage (P= 0.0015). sICAM-1 and sVCAM-1 had positive correlation with each other in both stages (P=0.0067, P=0.015, retrospectively). Neither sICAM-1 nor sVCAM-1 correctly reflected the coronary abnormalities and responsiveness to intravenous gammaglobulin (IVGG) in both stages. But sVCAM-1 was significantly increased in the carditis group in both stages (P=0.025, P=0.014, retrospectively) and had a positive correlation with troponin T (r=0.63, P=0.00063). CONCLUSION: The levels of sICAM-1 and sVCAM-1 were not very useful tools for detecting and predicting subsequent coronary abnormalities and responsiveness to IVGG in KD patients. However, sVCAM-1 appears to play a significant role in carditis of KD. Further studies are needed about various adhesion molecules and cytokines in the pathogenesis of KD.

A Case of Typical Kawasaki Disease with Vulvar Erythema and Edema in 21 Days Old Neonate

Kawasaki diseases occur frequently in children beyond the age of 4 years, but classic Kawasaki disease in newborns are rare and have not been reported in Korea yet. We report a case of classic Kawasaki disease in neonate who is 21 days old girl and has no response to gamma-globulin although early diagnosis and treatment. Her clinical features met typical Kawasaki disease, but unusually she had a non suppurative labia major erythema and severe edema instead of cervical lymphadenitis. She had persistent fever and progressive carditis with coronary dilatation in spite of 3 doses of gamma globulin. Her fever was subsided after 3 doses of intravenous methyl prednisolone. In the subacute stage her other symptoms subsided and appeared typical desquamation of fingers and toe. Finally she had mild mitral regurgitation and diffuse coronary dilatation when she was discharged. Since one year follow up, with continuous aspirin medication, now she is very healthy, and her coronary dilatation was normalized and not any more valvular regurgitation.

Changes of Lipid and Lipoprotein Compositions in Kawasaki Disease and its Impact on Cardiac Complications / 소아과

PURPOSE: Delineation of serum lipid and lipoprotein values in children after Kawasaki disease (KD) is important because of the predilection of this disease for the coronary arteries. METHODS: The KD group was composed of 51 patients who were hospitalized from Jan. 2002 to Dec. 2003. Control was 25 patients with non-KD febrile illness. The levels of total lipid, phospholipid, triglyceride, HDL-cholesterol (HDL-C), LDL-cholesterol, total cholesterol, apolipoprotein A1 (apo A1), apolipoprotein B, and Lipoprotein (a) were measured and compared with Echocardiographic findings. Measurements were obtained in four time periods: acute febrile, subacute, convalescent phase and 1 year after KD. RESULTS: HDL-C (33.64+/-7.49 mg/dL vs 50.43+/-14.41 mg/dL, P< 0.01) and apo A1 (99.75+/-6.39 mg/dL vs 113.34+/-11.35 mg/dL, P< 0.05) were decreased more in the acute febrile period of KD than in the control, but these changes were not correlated with cardiac complications. All lipid profiles were markedly elevated in the subacute stage and normalized in the convalescent stage; there were no changes until 1-year follow up. There were no significant differences in the changes of lipid profiles, including Lp (a) and coronary dilatation, in any time periods. CONCLUSION: KD is associated with important abnormalities in lipid metabolism, but these changes were transient and appear to be due to the disease itself. These data lead us to infer that KD dose not cause such permanent changes in lipid abnormalities as to be considered a risk factor for atherosclerosis, beyond that caused by the disease itself.

A Case of Bronchogenic Cyst with Nausea and Epigastric Pain / 소아과

Bronchogenic cysts are rare congenital anomalies that arise early in gestation from abnormal budding of the developing respiratory system. Mediastinal bronchogenic cysts account for 10-15 percent of all primary mediastinal masses; 63.7 percent of patients are symptomatic. Common symptoms are fever, chest pain, cough, dyspnea, and dysphagia. Gastrointestinal symptoms except dysphagia are rare. It can be life threatening with compression, infection, hemorrhage, or rupture. Symptoms and signs of compression are more frequent in infants and children than in adults. It may be asymptomatic, or cough, infection, and hemoptysis may be observed. Complete excision is recommended. We report a case of bronchogenic cyst misdiagnosed as chronic gastritis with nausea and epigastric pain for a year.

A Case of Pfeiffer Syndrome with Hydrocephalus and Multiple Congenital Anomalies

Pfeiffer syndrome is one of a rare form of craniosynostosis syndrome, showing variable degree of craniosynostosis, midface hypoplasia, broad thumbs and toes and syndactyly. This is transmitted in autosomal dominant pattern and known to be related to mutations in FGFR (Fibroblast Growth Factor Receptor) 1 or FGFR 2. We experience a case of newborn Pfeiffer syndrome type 3 who had multiple facial anomalies, thumbs and great toes anomalies, ankylosis of radius and ulnar and hydrocephalus.