A Case of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome

Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by catheter. Prenatal ultrasonic findings were bladder distension, hydronephrosis and possible intraabdominal mass. Supine view of abdomen on third day of life showed dilated loops of proximal small bowel and stomach without colonic gas shadow. Preoperative diagnosis was intestinal obstruction. When the abdomen was opened, the distal ileum was filled with meconium and postoperative diagnosis was meconium plug syndrome. Ganglion cells were present in the ileal biopsy. All postoperative attempts to feed her resulted in bilous vomiting. Voiding cystourethrography done on the 4th day after colon study showed markedly distended bladder, contrast enhanced microcolon and hypoperistalsis. She was dischaged against medical advice by her parents at the age of 23 days and died at home at the age of 33 days.

Predicting Intraventricular Hemorrhage by the Nucleated Erythrocytes Counts in Preterm Neonates

PURPOSE: Prolonged fetal hypoxia stimulates erythropoiesis in fetal life and induces increased nucleated erythrocytes(NRBC) counts at the early newborn period. To evaluate the relationship between prolonged fetal hypoxia and neonatal intraventricular hemorrhage (IVH), and the prediction of neonatal IVH by neonatal NRBC. METHODS: We compared the daily courses of the absolute NRBC count in preterm new- boms at 34 weeks' gestation or earlier with(n=17) and without(n=20) IVH for 7 days of life. RESULTS: Absolute NRBC counts at birth were higher in neonates with IVH than in control neonates(2,499/mm3+/-3,748 and 412/mm3+/-272, respectively, P=0.0022). The cut-off value of 1,000/mm3 for absolute NRBC counts at birth showed the best parameter estimate of the predictive model for IVH at early newborn period with 100% of positive predictive value and 74.1% of negative predictive value. CONCLUSION: Prolonged fetal hypoxia inducing fetal erythropoiesis near labor is closely related to IVH at early newborn period. Thabsolute NRBC counts at birth is the very important predictable marker for the condition.

A Case of Congenital Lobar Emphysema on Right Lower Lobe

Congenital lobar emphysema is a rare disease and a cause of respiratory disturbance in newborns and infancts. The common locations of pulmonary involvement are the left upper lobe, right middle and upper lobe. We experienced a case of congenital lobar emphysema in a 9-month-old male infant. Chief complaints were dyspnea and tachypnea. Chest X-ray or CT scan revealed a hyperinflated right lower lobe, mediastinal shift to the left side and retrosternal herniaton of right lung. There was no evidence of infection and foreign body. This patient was successfully treated with right lower lobectomy. We report this case with a brief review of related literatures.

A Case of Congenital Lobar Emphysema on Right Lower Lobe

Congenital lobar emphysema is a rare disease and a cause of respiratory disturbance in newborns and infancts. The common locations of pulmonary involvement are the left upper lobe, right middle and upper lobe. We experienced a case of congenital lobar emphysema in a 9-month-old male infant. Chief complaints were dyspnea and tachypnea. Chest X-ray or CT scan revealed a hyperinflated right lower lobe, mediastinal shift to the left side and retrosternal herniaton of right lung. There was no evidence of infection and foreign body. This patient was successfully treated with right lower lobectomy. We report this case with a brief review of related literatures.

A Case of Hutch's Diverticulum Associated with Severe Bilateral Vesicoureteral Reflux

We experienced a case of primary hepatic actinomycosis which was initially diagnosed by means of fine needle aspiration. The patient was a 31-year-old emaciated man with a 2-month history of 10 kg weight loss, right upper quadrant pain and flank pain. The liver was palpable and tender on physical examination. Computerized tomography scan of the liver showed two ill-defined hypodense masses in gallbladder fossa and inferior pole of right lobe. Hepatocellular carcinoma was clinically suspected. A CT-guided fine needle aspiration was performed. Microscopically, smears showed numerous radiating clusters of filamentous bacteria with many neutrophils and monocytes in necrotic background. The symptoms were improved by incision and drainage and massive administration of penicillin.

A Case of Secondary Pulmonary Hemosiderosis in a severe Hemophilia A with High Titer of FactorVIII Inhibitor / 대한소아혈액종양학회지

An 18-Year-old male hemophiliac with high titer of factorVIII inhibitor, stage V hemophilic arthropathy in right knee joint and a history of hematuria and retroperitoneal hemorrhage was admitted because of acute and massive bleeding of epistaxis, pulmonary hemorrhage and intestinal bleeding. The bleeing was not controolled by massive infusion of factorVIII concentrates but by prothrombin complex concentrates and high dose of factorVIII concentrates. He showned symptoms of sustained fever and diffuse pulmonary infiltration which was diagnosed as pulmonary hemosiderosis by MRI. We suppressed his immune reaction by prednisolne to prevent the formation of factorVIII inhibitor. He has been followed up for 3 years and shown no massive bleeding there-after.