A Case of ARCI Syndrome with Hypoplasia of Corpus Callosum and Heart Anomaly

ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized due to pneumonia at four months of age. Corpus callosum hypoplasia and atrial septal defect were detected. The case was treated with a mechanical ventilator because pneumonia was aggravated and respiratory failure occurred. The patient expired at five months of age.

Pattern of Occurrence of Mycoplasma pneumoniae Pneumonia in Admitted Children: Southern Central Korea, from 1989 to 2002

PURPOSE: The determination of exposure and prevalence of Mycoplasma pneumoniae pneumonia of any region should be helpful for clinical diagnosis. We studied the pattern of occurrence of M. pneumoniae pneumonia among children living in southern central Korea during the last 13 years. This area has a relatively small population and less mobility compared with metropolitan areas. METHODS: We performed a retrospective descriptive study of 143 patients admitted to the pediatrics ward among all the patients aged less than 15 years old diagnosed with M. pneumoniae pneumonia at our hospital, from July 1989 to June 2002. RESULTS: The percentiles of incidence per year compared to the total patients admitted to a pediatric ward per year were 0.52% in 1989, 0.48% in 1990, 0.13% in 1991, 0% in 1992, 1.85% in 1993, 1.00% in 1994, 0.15% in 1995, 0.53% in 1996, 1.75% in 1997, 0.65% in 1998, 0.24% in 1999, 1.14% in 2000, 1.30% in 2001, 0.53% in 2002. In the peak incidence of monthly distribution for two consecutive years with outbreaks, its outbreak in the first year was concentrated in late fall and winter, but its outbreak in the following year was earlier than first year. There was peak incidence of age distribution in 4-5 years. It is shown gradually that age distributions per year of outbreak became earlier in age. CONCLUSION: The occurrences of M. pneumoniae pneumonia in southern central Korea occurred at 3-4 year intervals, 1-2 years in duration, compared to three year intervals, one year in duration, according to reports from metropolitan areas before 1997.

Serum C-Reative Protein As a Predictor for Cardiac Sequelae in Kawasaki Disease

PURPOSE: The aim of this study was to identify the predictive factors in the early laboratory findings for cardiac sequelae in Kawasaki disease(KD). METHODES: A retrospective review of the records was conducted of all children with KD who were admitted to the Ulsan Dongkang General Hospital, Masan Samsung Hospital, and Gyeongsang National University Hospital between January 1995 and December 1999. We analyzed and compared the early laboratory findings between the patients with and without coronary artery dilatation. RESULTS: A total of 981 patients were divided into two groups : 826 patients(84.3%) with normal coronary artery and 155 patients(15.7%) with coronary artery dilatation. Age and sex were not significantly different between the two groups. The mean serum C-reactive protein(CRP) in the coronary artery dilatation group and in the normal coronary artery group were 5.0 mg/dl(+/-5.3) and 4.1 mg/dl(+/-5.0), respectively, with a significant difference(P<0.05), whereas the other early laboratory findings had no difference between the groups. CONCLUSION: This study shows that the early serum CRP was higher in patients with KD who had coronary artery dilatation than in those with normal coronary artery. There may be a strong possibility of cardiac sequelae at a high level of serum CRP. However, the cut-off value of serum CRP could not be determined for the prediction of cardiac sequelae in patients with KD.

Gastrointestinal Foreign Bodies: Review of 96 Cases / 대한소아소화기영양학회지

PURPOSE: Foreign body swallowing is common in children. Most of foreign bodies passed upper esophagus are removed spontaneously. But recently, therapeutic endoscopy in children is widely used. In this study, we reviewed gastric foreign bodies regarding types, location, interval from swallowing, complications, and treatment or method of removal. METHODS: We reviewed medical records of 96 cases with foreign bodies in gastrointestinal tract at Department of Pediatrics in Gyeongsang National University Hospital (GNUH) from Feb 1987 to Feb 2002. RESULTS: The peak age of patients (male=60, female=36) with foreign bodies in gastrointestinal tract was 2 to 5 years of age. Sixty two patients (64.5%) were asymptomatic. The location of foreign bodies in gastrointestinal tract were detected by simple X-ray in eighty one patients (86.0%), barium study (1 case), and gastroduodenoscopy in 37 cases. The most common location was stomach (63.5%). The most common foreign body was coin (41.7%). In thirty seven cases (38.5%), foreign bodies were removed with flexible gastroduodenoscopy. Fifty four patients (56.3%) visited GNUH in 24 hours after swallowing foreign bodies. The long interval (over 24 hours) of swallowing of foreign bodies is related with high frequency of endoscopic removal. CONCLUSION: The flexible gastroduodenoscopy was effective in diagnosis and removal of foreign bodies. Further studies for indication and validity of endoscopic removal of foreign bodies in upper gastrointestinal tract are needed.

A Case of Trisomy 22 with Pulmonary Hypoplasia in a Liveborn Infant

We report a case of trisomy 22 in a liveborn male infant which was confirmed by fluorescence in situ hybridization(FISH), macrocultures and GTG-banding, and RHA-banding procedures of peripheral white blood cells. The infant showed lung hypoplasia, which is a unique presentation, with other clinical manifestations of previously reported cases of trisomy 22, such as intrauterine growth retardation, cleft palate, micrognathia, large atrial septal defect, limb anomalies, imperforate anus, and hypospadias. Our report gives weight to the previously reported observation that pulmonary hypoplasia may be associated in trisomy 22.

A Mesenteric Lymphangioma Causing Billous Vomiting and Bloody Stool in a 3-day-old Infant

Mesenteric lymphangioma, which is rare and often diagnosed incidentally, is a benign tumor in infants and children. Most cases of lymphangioma are in the neck and axilla. About 5% of these lesions are in the mediastinum, mesentery or retroperitoneal region. Although most lymphangioma produce no symptoms, it may present an acute surgical condition suggesting acute intestinal obstruction. We report one case of histologically confirmed mesenteric lymphangioma causing billous vomiting and bloody stool in a 3-day-old infant.

A Case of Gastric Perforation Presenting with Secondary Localized Peritonitis Due to Bamboo in Stomach

Perforation after foreign body ingestion is rare. We report a 25-month-old girl who was diagnosed with a stomach perforation with secondary localized peritonitis caused by long, sharp bamboo. On admission, she had abdominal pain and fever. Simple abdominal erect & supine X-ray, abdominal ultrasonogram and computed tomography scan were normal. On the 6 th hospital day, gastrofiberoscopy was performed because a sharp mass was palpated by doctor on abdominal examination. Endoscopic findings revealed a 8 cm-long sharp bamboo tip was pierced to the posterior wall of antrum. This foreign body was removed with Olympus FG-8L alligator forcep. She was managed with NPO and prompt intravenous antibiotics and discharged with clinical improvement.