A Case of Meconium Peritonitis Accompanied with Hydrocele / 소아과

Meconium peritonitis is a rare disease in neonates, characterized by intraperitoneal calcification, numerous fibrosis with or without pseudocyst formation due to antenatal extravasation of meconium. Meconium peritonitis may result in a number of genital manifestations, including inguinal and scrotal or labial hydrocele containing meconium or calcifications. Recently, increased numbers of fetuses with meconium peritonitis have been prenatally diagnosed by ultrasonography. We report a case of meconium peritonitis in a neonate with ascites and hydrocele which was diagnosed by antenatal ultrasonography.

A Case of Neonatal Hypoxic Ischemic Encephalopathy caused by Amniotic Fluid Embolism / 대한주산의학회잡지

Amniotic fluid embolism, one of the leading causes of maternal death, is a rare event, however, it can cause maternal death and neonatal morbidity when it unrecognized and untreated effectively. Its pathogenesis is unclear and clinical presentations are variable without standardized means of confirming diagnosis. We experienced one case of neonatal hypoxic ischemic encephalopathy possibly due to maternal amniotic fluid embolism, which was diagnosed by brain MRI, EEG and maternal uterine pathology. We report this case with a brief review of literatures.

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.

Changes in Incidence, Survival Rate and Morbidity of Very Low Birth Weight Infants

PURPOSE: The survival rate of very low birth weight infants(VLBWI) has improved by virtue of specialized neonatal care. This study was performed to analyze the changes in incidence, survival rate and morbidity of VLBWI who admitted to Chonnam National University Hospital from 1996 to 2001. METHODS: We enrolled 565 VLBWI, and compared the incidence and the survival rate according to the birth weight or gestational weeks between period I(1996 to 1998) and period II(1999 to 2001). The mortality rate according to the postnatal age, cause of death, morbidity and days of hospital stay were also compared. Morbidity is categorized into 'short term' which is curable until discharge, and 'long term' causing any types of sequelae after discharge. RESULTS: Incidence of VLBWI significantly increased in period II over period I(6.0% vs. 11.0%, P<0.001). The survival rate also increased in period II(71.8% vs. 80.1%, P<0.05), especially in 1,000 to 1,249 gm of birth weight(P<0.001) and in 28 to 30 weeks of gestation(P<0.001). The most common cause of death was respiratory distress syndrome in period I; however it was sepsis in period II. Although overall and short term morbidity rate increased, long term morbidity and days of hospital stay didn't increase in period II. CONCLUSION: Although the incidence of VLBWI significantly increased and the survival improved in period II compared to period I, especially in 1,000 to 1,249 gm of birth weight and 28 to 30 weeks of gestation, 'long term' morbidity rate and hospital days didn't increase.

Frequency of Platelet Transfusions and Outcome in Neonates with Thrombocytopenia

PURPOSE: We compared the underlying or associated diseases according to the frequency of platelet transfusions in neonates with thrombocytopenia to know the factors predicting which patients will require multiple platelet transfusions. We also compared mortality. METHODS: A retrospective study was performed in 72 neonates who received the platelet transfusions in neonatal intensive care unit(NICU) between August 1996 and July 2001. Group I received one platelet transfusion and group II received two or more. We compared the frequency of underlying or assodiated diseases such as sepsis/disseminated intravascular coagulopathy(DIC), respiratory distress syndrome(RDS), intraventricular hemorrhage(IVH), patent ductus arteriosus (PDA), necrotizing enterocolitis(NEC), liver or renal disease, and mortality between two groups. RESULTS: Of the 72 patients, 29(40.2%) received one and 43(59.7%) received two or more transfusions; 16(22.2%) received four or more. There were no statistically significant differences in gestational age, birth weight, sex, and maternal history between two groups. C-section rate was higher in group II(20.7% vs. 55.8%, P<0.05) and the incidence of PDA was higher in group I (55.2% vs. 30.2%, P<0.05). Otherwise, there were no statistically significant differences in the incidence of sepsis/DIC, RDS, IVH, RDS, CLD, NEC, liver or renal disease, pulmonary hemorrhage and hypoxic ischemic encephalopathy, and mortality between group I and group II. CONCLUSION: There was no significant difference in clinical morbidity and mortality according to the frequency of platelet transfusion in neonates with thrombocytopenia. Further study is needed to know the predicting factor for multiple platelet transfusions in neonates with thrombocytopenia.

Viral Hepatitis and Change of Lymphocyte Subpopulation in Hemophiliacs in Chonnam KwangJu area / 대한수혈학회지

BACKGROUND: Hemophiliacs are known to have higher risk of exposure of hepatitis virus and immunosuppression. The aim of this study is to investigate the positive rate of viral markers for hepatitis and anti-HIV and the changes of lymphocyte subpopulations in Hemophiliacs in Chonnam GwangJu area. METHODS: One hundred four patients who had visited to the Hemophilic Clinic, Chonnam University Hospital from 1999 to 2001 were enrolled. They were checked for type A, B, C hepatitis viral markers, anti-HIV and lymphocyte subpopulations. The prevalence of hepatitis and lymphocyte subpopulation were compared according to severity and age of hemophiliacs. RESULTS: Anti-HAV IgM, anti-HAV IgG, HBsAg, anti-HBs, anti-HCV were positive in 40%(22/55), 65.5%(66/101), 42.3%(42/97) of cases tested. Positivity of anti-HCV showed trends of increase according to the severity of hemophiliacs (P11 years old) (P=0.003, P<0.001, respectively). Although all enrolled patients were negative for anti-HIV, absolute T and B cells counts were decreased in 71.6% and 14.9% of patients, respectively and inversion of CD4/CD8 ratio were found in 65.7%. But there were no statistical difference in not only decrease of T and B cells but also inversions of CD4/CD8 ratio according to age and severity. CONCLUSION: The number of hemophiliacs with previous history of hepatitis B virus infection and seropositivity of anti-HCV were increased according to the age and severity of hemophilia. Active vaccinations of hepatitis B may be required in hemophiliacs. The greater part of hemophiliacs showed decrease in T cell count and inverted CD4/CD8 ratio. The hemophiliacs need a cautiion for infection and follow up tests for immunologic function.

Different Outcomes of Intractable Temporal Lobe Epilepsy with Regard to the Timing of Surgical Intervention / 대한소아신경학회지

PURPOSE: We like to examine the clinicopathologic findings of intractable temporal lobe epileptic patients who underwent epilepsy surgery, and different outcomes with regard to the timing of surgical intervention. METHODS: One hundred fourty six patients underwent anterior temporal lobectomy for medically intractable epilepsy between the year of 1993 and 2000. Except 5 patients who had malignancy, 132 patients were included in this study and were followed up for longer than 12 months after surgery. Two groups, under 15 years old(pediatric group) and over(adult group) according to the timing of surgery, were compared with clinical variables(seizure patterns, EEGs and brain MRI findings), pathologic findings and seizure outcomes. Seizure outcomes were divided as favorable in Class I and II or unfavorable in Class III and IV by using Engel's classification. RESULTS: Among 132 patients, 103 patients(78.0%) were classified as favorable and 29 patients(22.0%) unfavorable. Adult group had more favorable outcomes than pediatric group(82.1% vs 55.0%, P=0.007). Pathologic findings were as follows:48 patients(36.4%) had only hippocampal sclerosis, 50(37.9%) hippocampal sclerosis with other pathologic findings, 20(15.2%) cortical dysplasia, 6(4.5%) cortical dysplasia and gliosis, 7(5.3%) only gliosis and 1(0.7%) hippocampal atrophy. Among 98 patients who had hippocampal sclerosis, 81(82.7%) had favorable outcomes. Among 26 patients who had cortical dysplasia, 16(61.5%) had favorable outcomes. In case of hippocampal sclerosis only, pediatric group had more favorable outcomes(85.7% vs 82.9%, P=0.86). But in case of hippocampal sclerosis with other pathologic findings, adult group had more favorable outcomes(50.0% vs 86.4%). In case of cortical dysplasia only, adult group had more favorable outcomes(40.0 % vs 74.3%). CONCLUSION: The seizure outcomes after surgery, pediatric group showed less favorable outcomes than adult group. In case of hippocampal sclerosis only, the outcome of early surgery was good too.

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.

Endocrinopathy in Hemochromatosis Patients Multi-Transfused for Aplastic Anemia / 대한소아혈액종양학회지

PURPOSE: Chronic blood transfusions result in excessive iron deposition leading to eventual tissue damage and impaired function of organs, such as the liver, spleen, pancreas, skin, thyroid, and heart. We evaluated the body iron status and endocrinopathy in repeatedly transfused patients with aplastic anemia (AA). METHODS: Fourteen patients with AA who were transfused with more than 10 Units of packed RBC since 1996 were evaluated. We evaluated the correlation of amount of blood transfused with status of iron stores (determined by serum iron, TIBC, ferritin and transferrin saturation) and organ damage. RESULTS: Patients received a median of 61 units (range 11~168 units) of PRC. Twelve patients (85.7%) had elevated serum ferritin levels, and 11 (78.6%) had elevated transferrin saturation. Serum ferritin (P<0.01; r=0.868), and transferrin saturation (P<0.05; r=0.569) were significantly correlated with the amount of PRC transfused, respectively. Five patients had clinically significant iron overload despite the use of deferoxamine. Organ damage caused by transfusion iron overload were skin pigmentation (N=3), hepatic (N=1) and endocrinologic abnormalities. Diabetes (N=3), hypothyroidism (N=3), and hyogonadotropic hypogonadism (N=1) were observed. No patient developed clinically significant arthropathy or cardiac disease. CONCLUSION: AA patients who received chronic blood transfusions develop iron overload which may lead to endocrinopathy. Iron status and organ dysfunction should be monitored and effective measures to prevent iron overload should be applied in patients who need chronic transfusions.

Three Cases of Factor XI Deficiency / 대한소아혈액종양학회지

Factor XI deficiency is a very rare autosomal recessive coagulation factor deficiency, comprising 1/million in ethnic groups other than Ashkenazi Jews. The clinical manifestations are extremely variable, and generally milder than those of hemophilia A and B. We describe herewith 3 children with factor XI deficiency, who were found to have prolonged aPTT in routine laboratory studies, or in evaluation of intermittent epistaxis.