A Study on the Effect of Combination Therapy with Anti - Thrombin III and Gabexate Mesilate in Premature Infants for Disseminated Intravascular Coagulation

PURPOSE: Both antithrombin III(ATIII) and Gabexate mesilate(Foy) are effective for the treatment of disseminated intravascular coagulation(DIC). However, their mechanisms of action are slightly different, and combined effect of ATIII and Foy in premature infant with DIC has not been studied. We evaluated therapeutic efficacy of treatments with either ATIII or Foy alone or both in combination. METHODS: We studied 23 premature infants of gestational ages between 30 and 36 weeks with DIC. Group A(n=10) was treated by ATIII only, Group B(n=7) by Foy only and Group C(n=6) by both ATIII and Foy. Three groups were compared for volume of blood sampling and transfusion and hematologic data. RESULTS: Improvement of hematologic data(platelet, PT, aPTT, fibrinogen, FDP) was not significantly different among 3 groups. The mean volume of blood sampling during 5 days of treatment was 30 mL, 22.5 mL, and 30 mL, respectively. The mean volume of packed RBC transfusion was 12.8 mL, 9 mL, and 2.5 mL, respectively: and mean volume of platelet transfusion was 25.9 mL, 10 mL, and 0 mL, respectively, showing no significant statistical difference. But the mean volume of FFP transfusion was 141 mL only in group B, significantly higher compared to other groups. CONCLUSION: The combination therapy of ATIII and Foy significantly decreased the volume of FFP transfusion and may be more effective than monotherapy with ATIII or Foy alone in DIC of premature infant.

A Cse of Partial Trisomy 10q Syndrome / 대한주산의학회잡지

Partial trisomy 10q syndrome is a rare chromosome anomaly characterized by severe mental and growth retardation, craniofacial dysmorphia with prominent forehead, fine arched eyebrows, deep set small eyes and micrognathia, In addition, other physical manifestations have been reported as skeletal anomaly, congenital heart disease, inguinal hernia, and so on. We report a case of partial trisomy 10q syndrorne with certain stigmata which confirmed by chromosome analysis.

A Case of Saethre - Chotzen Syndrome

Anti-HLA antibody related neonatal thrombocytopenia is an uncommon disorder caused by platelet antigen incompatibility between mother and fetus in Korea. Mothers who lack the specific platelet antigen produce the IgG against the platelet antigen which the fetus inherits from the father. These IgG antibodies are then transported across the placenta into the fetal circulation where they lead to the destruction of fetal platelets. We report a case of neonatal alloimmune thrombocytopenia related with anti-HLA antibody in second baby of dizygotic twin who had petechia on trunk and platelet count 43,000/mm. Initially, mother and twin showed the positives in antiplatelet antibodies. In microlym- phocytotoxic test at 6 months of age, anti-HLA antibodies was negative in twins but anti-HLA A2, A24 was positive in their mother. The patient was treated with intravenous immunoglobulin and clinically improved and her platelet count was norrnalized.

Immunohistochemical Study on Expression of the p53 Protein in Medulloblastoma/PNET / 대한암학회지

PURPOSE: The present study explores the expression rate of p53 mutation and the correlation between the expression of p53 protein and prognostic factors in medulloblastoma/ PNET (primitive neuroectodermal tumor). MATERIALS AND METHODS: We studied retrospectively 24 patients with medulloblastoma/ PNET, who were admitted in Dong-A University Hospital, Pusan National University Hospital and Inje University Pusan Paik Hospital from 1988 to 1995. Detection of p53 mutations was made by immunohistochemical staining of p53 protein on paraffin- embedded tissues. The correlation between the expression of p53 protein and prognostic factors was evaluated by the Spearman correlation analysis. RESULTS: p53 protein was expressed in 6 of 24 patients (25%). In 20 patients who could be evaluated for metastasis, 16 patients of M0, 1 patient of M1 and 3 patients of M2 were grouped by M stage, and the expression of p53 was detected in 1 of 16 M0 group (6.3%) and 3 of 3 M2 group (100%). p53 expression was significantly related to the M stage of medulloblastoma/PNET (r=0.73, p<0.001). The detection of p53 was not significantly associated with T stage, cellular differentiation and the relapse rate of medulloblastoma/ PNET. CONCLUSION: The immunohistochemical detection rate of p53 protein in medulloblastoma/ PNET was 25%. The expression of p53 protein was significantly related to the M stage, with higher expression rate in M2 group of medulloblsatoma/PNET.

Prophylactic Treatment of Anemia of Prematurity with Recombinant Human Erythropoietin and High Doses of Iron

PURPOSE: We conducted randomized study to determine whether high doses (6mg/kg/ day) of iron would exert a more supplemental effect than low doses (3mg/kg/day), and which regimen of recombinant human erythropoietin (rHuEPO) and iron would be more beneficial in the prophylactic treatment of anemia of prematurity. METHODS: We randomly assigned 38 sick premature infants who were more likely than symptom-free premature infants requiring erythrocyte transfusions for infants with anemia of prematurity to receive rHuEPO, 100unit/kg, tiw, subcutaneously, plus iron, 3mg/kg/day, po, daily from the second day of life (group 1), 100unit/kg and 6mg/kg/ day (group 2), 200unit/kg and 3mg/kg/day (group 3), and 200unit/kg and 6mg/kg/day (group 4), respectively. RESULTS: There were no significant differences of hemoglobin levels and iron balances during treatment among all 4 groups. The rates of increase in reticulocyte counts were greater in group 4 and group 2 compared with group 3 and group 1, respectively, though these rates were statistically not significant. The blood volume differences (volume of phlebotomies-volume of transfusions) during treatment were higher in group 4 compared with group 1 (p<0.05). CONCLUSIONS: High doses of iron may be more effective in rapidly increasing reticulocyte counts, and 200unit/kg, tiw of rHuEPO plus 6mg/kg/day of iron is more beneficial in reducing the need for blood transfusions than any other regimen. Therefore the prophylactic treatment of anemia of prematurity and acute blood loss from frequent blood sampling in risky premature infants with rHuEPO, 200unit/kg, tiw, subcutaneously, plus iron 6mg/kg/day, po, daily from the second day of life is effective in reducing the number of blood transfusions. Additional controlled trials utilizing high doses of iron with rHuEPO and larger numbers of patients are justified.

Neonatal Statistics of Korea in 1996: Collective Results of Live - Births , Neonatal Mortality , and Incidence of Dischage Against Medical Advice at 64 Hospitals

PURPOSE: To evaluate the nation-wide results of statistics related to the neonatal period of 1996, we collected data of a total of 64 hospitals in Korea (42 university hos- pitals and 22 general hospitals). METHODS: We obtained the results of 129,175 inboms and 9,379 outborns, and analyzed the statistics of live-births, ig, distribution of live-births by gestational age and birth weight, incidence of pre-term infants and low birth weight infants (LBWI), neonatal mortality, and incidence of discharge against medical advice (DAMA). RESULTS: According to birth weight, incidence of LBWI, normal birth weight, infant and high birth weight infants was 3.6%, 86.6% and 9.8%, respectively in the case of inborn group. But incidence of LBWI was higher in outborn group as compared with the inbom group. According to gestational age, incidence of preterm, term, and post-term was 11.1%, 87.1Yo and 1.8% respectively in the inbom group. The incidence of preterm in outborn group was higher than that of inborn group, because of the influnce of transpor- tation of high risk neonates to 2nd or 3rd levels of neonatal intensive care units (NICU). Overall neonatal mortality per 1,000 live-births was 9.3 in the inborn group amd 37.6% in the outborn group. These data revealed a high neonatal mortality, because the numbers of DAMA cases was also included. The incidence of DAMA was 0.44% and 1.15% in inborn and outborn groups, respectively. The percentage of the DAMA among the numbers of neonatal mortality was 47.2-48.8M in the inborn group. CONCLUSIONS: We obtained the statistics related to live-birth, incidence of prematurity and LBWI, neonatal mortality, and incidence of DAMA in Korea. The data revealed high levels of neonatal mortality (which included the sum of neonatal death and the number of DAMA) and incidence of DAMA in Korea at present. To achieve a low-level of neonatal mortality, more efforts to decrease the incidence of DAMA are needed. Also, a greater facility for NICU and a stronger support system from a nation-wide govemment policy and system of insurance are seen to be necessary.

A Study on Blood Lead Levels in Pediatric Inpatients

Lead is one of the most widespread environmental toxins and its poisoning in children was considered a rarity, usually resulting from unique circumstances such as inappropriate use of leadbased body cosmetics or direct administration of lead-containing folk medicines. The increasing concern about children with lower levels of lead exposure has developed, but there are no data regarding the mean blood lead levels and the incidence of symptomatic or asymptomatic lead poisoning in Korean children. We analyzed the zinc protoporphyrine (ZPP) values and blood lead concentrations in 163 pediatric inpatients for a prospective study of lead exposure. The blood lead concentrations in all 163 children were 15~54 g/dl, of whom 111 children (68.1%) were 25~54 g/dl which needs decision to chelate based on the EDTA provocation test. Among 111 children whose blood lead concentrations are 25~54 g/dl, 59 children(53.2%) were between 7 months and 3 years of age, which revealed no significant higher incidence of lead exposure than any other age group. The ZPP values in 126 children (77.3%) were above 35 g/dl. The mean blood lead concentration and ZPP values are 27.8 g/dl and 48.8 g/dl, respectively. We conclude that there are many asymptomatic children with increased absorption of lead in the urban area of Korea, and we need further studies regarding lead poisoning. It is important that there must be a national counterplan and that pediatricians continue to pay attention to lead posioning in children.

A clinical study of neonatal sepsis

The 35 newborns with neonatal sepsis admitted to the Neonatal Intensive Care unit of Dong-A University Hospital during 2 years and 6 months from April 1990 to October 1992, and were reviewed on the bases of incidence, clinical manifestations, underlying conditions, etiologic organisms, results or antibiotics sensitivity test and mortality rates. The results were summarized as follows; 1) The incidence of neonatal sepsis was 1.2% and male predominated. Sepsis was more prevalent in premature babies (7.5%) than in full term babies (0.8%). 2) Neonatal sepsis occured more frequently in low birth weight infant below 2500 g(6.3%) than in normal birth weight infant. 3) Underlying conditions associated with neonatal sepsis were as follows; pneumonia (25.7%), HMD (17.1%), urinary tract infection (11.4%), DIC (8.6%). 4) Common clinical manifestations observed in neonatal sepsis were jaundice (45.7%), poor feeding (22.9%), abdominal distension (20.0%), lethargy, convulsion, apnea and diarrhea in order. 5) Blood culture report revealed that gram negative organisms (57.1%) were more frequently associated with neonatal sepsis than gram positive organisms (37.1%). Klebsiella pneumoniae was the most common microorganisms in neonatal sepsis. 6) Vancomycin (100%), cephalothin (84.6%) and chloramphenicol (84.6%) were the sensitive drug to gram positive organisms. Gram negative organisms were sensitive to amikacin (100%), and cephalothin (95%). 7) Overall mortality rate was 17.1% in all patients with neonatal sepsis, 7.7% in gram positive sepsis and 25% in gram negative sepsis. We conclude that the etiologic organisms of neonatal sepsis have been altered, and have to choose appropriate antibiotics which particularly sensitive to these gram negative organisms such as Klebsiella pneumoniae, Pseudomonas aeruginosa, and also have to specify antibiotics according to the predominant organisms of the each institute.

Nesidioblastosis of the Pancreas

The morphologic abnormalities of the endocrine pancreas that underlie persistent neonatal hyperinsulinemic hypoglycemia and are included under the heading "nesidioblastosis" appears to be heterogeneous. This characteristic morphologic finding is ductuloinsular complexes showing endocrine cells budding off the ductoepithelium and merging with adjacent endocrine cell clusters. A case of nesidioblastosis associated with hyperinsulinemic hypoglycemia occurred in a 6/365 year-old male neonate. Microscopic finding of near totally resected pancreas revealed irregular sized islets and ductuloinsular complexes, both of which contained hypertrophied B cells with a few mitosis. Because of persistent hypoglycemia after first operation, he received second operation 8 days after. This histologic finding was more severe comparative to that of first operation. According to these findings, the pathogenesis of nesidioblastosis may be congenital or developmental defect of a kind of compensatory mechanism by unknown stimuli to acquire persistent hypoglycemia.

A case of Crohn disease

No abstract available.