Hepatic Veno-occlusive Disease Following Hematopoietic Stem Cell Transplantation in Children: Retrospective Analysis in a Single Institution / 대한소아혈액종양학회지

PURPOSE: Hepatic veno-occlusive disease (VOD) is a life-threatening complication occurring early after stem cell transplantation (SCT). Early diagnosis and effective treatment has not been established in severe VOD. Because there are few reports on VOD in Korean children, we evaluated the clinical characteristics of VOD following SCT in children. METHODS: We retrospectively reviewed the chart of all patients (n=116) receiving SCTs in CNUH Pediatric BMT center between May, 1991 and June, 2004. RESULTS: VOD developed in 11 patients (9.5%) (median age, 9.8 years; range, 2 to 13.9). Underlying diagnoses were ALL (n=3), severe aplastic anemia (n=3), AML (n=2), acute biphenotypic leukemia (n=1), neuroblastoma (n=1), and myelodysplastic syndrome (n=1). The median day of onset of VOD was D+9 (range, D-3 to D+19). VOD was classified as moderate in 5 and severe in 6 cases. Maximum level of serum total bilirubin was 2.9 mg/dL (range, 2.1 to 9.2) in moderate VOD and 7.3 mg/dL in severe VOD (range, 2.0 to 24.2) at D+18 (range, D-5 to D+59). We successfully treated VOD with various combinations including tPA and heparin (2/5, 40%), ursodeoxycholic acid (2/5, 40%), N-acetylcysteine (3/5, 60%), and defibrotide (1/2, 50%). All of 5 patients with moderate VOD survived at D+100 (range, 5.5+ to 66.6+ months). Five of 6 (83%) patients with severe VOD died within first 19 day from complications of VOD. CONCLUSION: This retrospective study showed that the incidence of VOD was 9.5%, and the mortality of severe VOD was still high which would necessitate early diagnosis, effective prevention and treatment.

Two Cases of Malignant Lymphomas in Children with Selective IgA and IgG Subclass Deficiency / 대한소아혈액종양학회지

Selective IgA deficiency is one of the most common primary immunodeficiency. Some patients with IgA deficiency also have deficits in one or more immunoglobulin G subclasses. It has been estimated that up to 25% of patients with certain primary immunodeficiencies will develop tumors, primarily B-cell lymphomas during their lifetime. We hereby present 2 cases of malignant lymphomas, one diffuse large cell lymphoma and another mixed cellularity Hodgkin's disease, respectively, which developed in patients with selective IgA and IgG subclass deficiency.

Isolated Intracranial Granulocytic Sarcoma as a Relapse Following Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome in a 1 Year-Old Infant / 소아과

Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple months several GS developed in the scalp and blast counts in BM increased. Induction chemotherapy resulted in partial remission of BM but GS disappeared. Four months after diagnosis, an unrelated BMT was undertaken. Engraftment was uneventful. On D+160, an intracranial GS of 6.5 cm in size developed. A craniotomy and tumor removal was done. There was no evidence of relapse in BM with complete chimerism. Reinduction chemotherapy using IDA-FLAG resulted in profound neutropenia with pneumonia. She succumbed to respiratory failure despite leukocyte recovery. The optimal management for isolated relapse as GS following BMT should be established.

Complications and Perinatal Factors According to the Birth Weight Groups in the Infants of Diabetic Mothers

PURPOSE: This study was performed to compare complications and perinatal factors according to the birth weight groups in the infants of diabetic mothers(IDM). METHODS: Three hundred and one singleton diabetic mothers and their babies of more than 30 weeks' gestational age admitted in the department of Pediatrics, Chonnam University Hospital from January 1996 to March 2002 were enrolled. Complications and perinatal factors were compared between large for gestational age(LGA) and appropriated for gestational age(AGA) infants. RESULTS: Hypomagnesemia was observed in 37.5%, jaundice in 21.3%, hypoglycemia in 11.1%, hypocalcemia in 7.0%, and birth injury in 19.6%. Congenital anomaly was noted in 24.9% with cardiovascular anomaly most commonly. In the LGA group, the frequencies of jaundice, hypoglycemia, tachypnea, and birth injuries were higher, and the interventricular septum was thicker than the AGA group. In the LGA group, Cesarean section rate, maternal height, weight before pregnancy, weight gain during pregnancy, and the incidence of unawareness of gestational DM were significant compared with the AGA group. CONCLUSION: In the LGA group, the frequencies of jaundice, hypoglycemia, tachypnea, and birth injuries were higher, and the interventricular septum was thicker than the AGA group. In the LGA group, maternal height, weight before pregnancy and weight gain during pregnancy were larger, and the incidence of unawareness of gestational DM was higher compared with the AGA group. These results suggest that careful examination and management are needed to detect the high risk, pregnant DM mothers with possible LGA babies.

A Case of Labial Adhesion

Labial adhesions are postnatal fusion of the labia minora in the midline of varying degrees. They are postulated to be the result of low estrogen levels in the prepubertal child and possibly of a chronic inflammatory process. Topical treatment with conjugated estrogens has been the mainstay of therapy. We experienced one case of labial adhesion in an infant who was treated with estrogen cream for three weeks without any complication. Here we present this case with a brief review of the literature.

Three Cases of Internal Jugular Phlebectasia

Phlebectasia is an abnormal dilatation of an isolated vein and a rare venous anomaly and is usually asymptomatic. Clinically internal jugular phlebectasia is a self limited benign condition and usually no treatment is required after initial diagnosis. So suspection of this disease and appropriate diagnostic approaches are essential to avoid unnecessary surgical intervention. We present three cases of internal jugular phlebectasia of which diagnosis was made by neck sonography and CT.

Hematologic Findings of Toxemia Mothers and Their Babies / 대한주산의학회잡지

OBJECTIVE: Hematologic abnormalities in toxemia mothers and their babies have been documented. The purpose of this study was to explore the hematologic findings in mothers with toxemia and postnatal hematologic findings in their babies. The relationship of hematologic findings between toxemia mothers before delivery and their babies immediately after birth was also examined. METHODS: Forty-six pairs of singleton toxemia mothers and their babies born by C-section with 28 to 35 weeks of gestation from Jan. 1995 to Dec. 1999 were enrolled. Fifty pairs of singleton normotensive mothers and their babies with the same gestational period and delivery method were matched for control group. Blood samples of mothers were performed before delivery, and those of their babies, were done immediately after birth, day 3 and 7 of life. Hemoglobin(Hb) concentration, total white blood cell(WBC) count, absolute neutrophil count(ANC), and platelet count were examined. RESULTS: Toxemia mothers have significantly lower platelet count compared with controls(191,000+/-83,200/mm3 vs. 252,000+/-92,700/mm3, p<0.05). There were no difference between both groups in Hb, WBC and ANC. On the first day of life, Hb was significantly higher(16.97+/-2.36g/dL vs 14.32+/-1.5g/dL, p<0.05), but WBC, ANC and platelet count were significantly lower in babies born to toxemia mothers than those of control group(p<0.05 for all). In babies born to toxemia mothers, Hb was significantly decreased until the seventh day of life(16.97g/dL, 15.48g/dL, 14.61g/dL, p<0.05), however, WBC and platelet count were significantly decreased until the third day of life and then incresed until the seventh day of life(11,832/mm3, 8,334/mm3, 10,104/mm3 for WBC, p<0.05, and 152,220/mm3, 144,720/mm3, 214,090/mm3 for platelet, p<0.05, respectively). CONCLUSION: Hemoglobin concentration was significantly higher, whereas WBC, ANC and platelet were significantly lower in babies of toxemia mothers than those of control group. In babies born to toxemia mothers, Hb was significantly decreased until the seventh day of life, however, WBC and platelet count were significantly decresed until the third day and then increased until the seventh day of life. There was no relationship between hematologic findings of toxemia mothers and their babies.

A Case of Satoyoshi Syndrome Presented with Progressive Muscular Spasm and Alopecia

Satoyoshi syndrome(generalized Komuragaeri disease) is a rare disorder of unknown cause, characterized by progressive, painful, intermittent muscle spasms and alopecia. Endocrinopathy with amenorrhea, secondary skeletal abnormalities, and diarrhea or unusual malabsorption are frequently seen. It seems that autoimmunity may play a role in its pathogenesis. We report a 13-year-old girl with characteristic manifestations of the syndrome. She was treated with intravenous gammaglobulin and Prednisolone. Painful muscle cramps were gradually improved, but the scalp condition did not change. Satoyoshi syndrome should be considered in children with unexplained muscle spasms and alopecia.

Multifocal Brain Infarction and Intraventricular Hemorrhage in an Infant during Treatment of Diabetic Ketoacidosis / 대한소아내분비학회지

Fatal complications including cerebral edema and neurologic collapse occur during treatment of diabetic ketoacidosis(DKA). A 6-week-old female infant with fever, dehydration and drowsy mental status was diagnosed as DKA and neurologically deteriorated during treatment. The cranial computed tomography scan revealed multifocal brain infarctions of the left caudate nucleus, bilateral frontal periventricular white matter, and right parietal cortex. A moderate amount of hemorrhage was also noted in both lateral ventricles. She recovered rapidly with supportive treatment over time. The clinical course and radiologic findings of this patient emphasize the importance of brain infarction as a cause of persistent neurologic loss in children with DKA.