• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.292-297
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• 2006

Purpose : In medulloblastoma, craniospinal radiation therapy combined with chemotherapy improves the prognosis of tumors but results in significant endocrine morbidities. We studied the endocrine morbidity, especially growth pattern changes. Methods : The medical records of 37 patients with medulloblastoma were reviewed retrospectively for evaluation of endocrine function and growth. We performed the growth hormone stimulation test in 16 patients whose growth velocity was lower than 4 cm/yr. Results : The height loss was progressive in most patients. The height standard deviation score (SDS) decreased from $-0.1{\pm}1.3$ initially to $-0.6{\pm}1.0$ after 1 year(P<0.01). Growth hormone deficiency(GHD) developed in 14 patients. During the 2 years of growth hormone(GH) treatment, the improvements of height gain or progressions of height loss were not observed. Twelve patients(32.4 percent) revealed primary hypothyroidism. One of six patients diagnosed with compensated hypothyroidism progressed to primary hypothyroidism. Primary and hypergonadotropic hypogonadism were observed in two and one patients respectively. There was no proven case of central adrenal insufficiency. Conclusion : Growth impairment developed frequently, irrespective of the presence of GHD in childhood survivors of medulloblastoma. GH treatment may prevent further loss of height. The impairment of the hypothalamic-pituitary-gonadal and hypothalamic-pituitary-thyroidal axis is less common, while central adrenal insufficiency was not observed.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1126-1133
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• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.944-949
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• 2008

Purpose : As modern society has became more open, interest in healthy internal and external growth has increased, including that pertaining to penile length in children. A micropenis is defined as one where penile length is more than 2 SD (standard deviation) below the mean, and it can be traced back to chromosome and endocrine disorders. The authors executed this study to suggest guidelines for the study of the micropenis and standard information for penile length in Korean newborns. Methods : The subjects of this study were 168 male infants between 37 and 42 weeks of gestational age, none of whom had any complications during pregnancy or birth; each had been born in Daegu Fatima Hospital between February and June 2007. Penile length was measured using conventional stretched penile length measurement (CPLM) and syringe methods. Results : Penile length was $3.02{\pm}0.25cm$ (F=36.467, $R^2=0.180$, P<0.001) when measured with CPLM, and $3.29{\pm}0.26cm$ (F=9.149, $R^2=0.052$, P<0.001) with the syringe method. There was no statistically significant difference in the penile length of newborn infants as a result of taking measurements with the two methods, and both methods showed significance at 0.631 in terms of Pearson's correlation coefficient, at the level of P=0.01. Conclusion : In this study, penile length tended to be longer when gestational age was longer, and a micropenis can be assumed to be one less than 2.5 cm using CPLM and less than 2.8 cm using the syringe method. In the case of a concealed penis, the syringe method is helpful. When a micropenis is assumed, close observation by outpatient department personnel, and additional endocrine and chromosome studies should be undertaken after sufficiently consulting the parents.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.227-236
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• 2002

Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1512-1518
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• 2002

Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1381-1388
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• 2002

Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. Results : A total of 70 patients, consisting of 31 males and 39 females, with a mean age of $2.6{\pm}3.3$ years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. Conclusion : MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.503-509
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• 2010

Purpose : Although neonatal pulmonary hemorrhage is rare, it is associated with high mortality. We aimed to evaluate the risk factors associated with pulmonary hemorrhage in preterm infants and to describe the clinical course, including neonatal morbidity, of infants who developed pulmonary hemorrhage. Methods : We performed a retrospective case-control study of 117 newborn infants aged less than 37 gestational weeks admitted to the neonatal intensive care unit of Daegu Fatima Hospital between January 1995 and December 2008. Control group infants without pulmonary hemorrhage were matched according to the gestational age, duration of mechanical ventilation, and birth weight range (${\leq}100g$). Pulmonary hemorrhage was defined as the presence of hemorrhagic fluid in the trachea and severe respiratory decompensation. Results : Pulmonary hemorrhage occurred in 17 cases of very low birth weight infants (VLBW; birth weight < 1,500 g; median age, 3 days) and 22 cases of low birth weight infants (LBW; $1,500g{\leq}$ birth weight < 2,500 g; median age, 1 day). Antenatal maternal glucocorticoid treatment significantly reduced the incidence of pulmonary hemorrhage in VLBW infants. Low APGAR score (${\leq}3$ at 1 min) and acidosis at birth were associated with significantly high incidence of pulmonary hemorrhage in LBW infants. Conclusion : Antecedent factors and timing of pulmonary hemorrhage of LBW infants were different from those of VLBW infants. The mortality rates of VLBW and LBW infants were 88.2% and 45.5%, respectively. Pulmonary hemorrhage was the principal cause of death in 66.6% VLBW infants and 40.0% LBW infants.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1172-1178
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• 2008

Purpose : Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. Methods : We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007. The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. Results : Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

• Journal of Preventive Medicine and Public Health
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• v.24 no.4 s.36
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• pp.531-548
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• 1991

To assess the adequacy of medical manpower and medical fee for the newborn nursery care, the author visited 20 out of 24 hospitals with the pediatric training program in Youngnam area between July 29 and August 14, 1991. Total number of newborn, both normal and sick, admission and discharge in 1-30 June 1991 was obtained from the logbook of nursery. Head nurse and staff pediatrician of the nursery were interviewed to get the current staffing for the nursery and their subjective opinion on the adequacy of nursery manpower and the difficulties in recruiting manpower. Average medical fee charged for the maternity and normal newborn nursery care was obtained from the division of self-audit of medical insurance claim of each hospital. Average minimum requirement of nursing care time for one normal newborn per day was 179.5 (${\pm}58.6$) minutes; 202.3(${\pm}50.7$) minutes for the university hospitals and 164.2(${\pm}60.5$) minutes for the general hospitals. The ratio of minimum requirement of nursing care time and available nursing time was 1.42 on the average. Taking the additional requirement of nursing care for the sick newborns into consideration, the ratio was 2.06. The numbers of R. N. and A. N. in the nurserys of study hospitals were 31%, and 17%, respectively, of the nursing manpower for the nursery recommended by the American Academy of Pediatrics. These findings indicate that the nursing manpower in newborn nursery is in severe shortage. Ninety percent of the head nurses and 85% of the staff pediatrician stated that the newborn nursery is short of R.N. and 75% of them said that the nurse's aide is also short. Major reason for not recruiting R.N. was the financial constraint of hospital. For the recruitment of nurse's aide, short supply was the second most important reason next to the financial constraint. However, limit of quarter in T.O. was the mar reason for the national university hospitals. Average total medical fee for the maternity and newborn nursery cares of a normal vaginal delivery who stayed two nights and three days at hospital was 219,430won. Out of the total medical fee, 20,323won(9.3%) was for the newborn nursery care. In case of C-section delivery who stayed six nights and seven days, total medical fee was 732,578won and out of the total fee 76,937won (12.0%) was for the newborn care. Cost for a newborn care per day by cost accounting was 16,141won for the tertiary care hospitals and 14,576won for the all other hopitals. The ratio of cost and the fee schedule of the medical insurance for a newborn care per day was 5.0 for the tertiary care hospitals and 4.9 for the all other hospitals. Considering the current wage level of the medical personnel, capital investment for the hospital facilities and equipments, and the cost for hospital maintenance, it is hard to expect adequate quality care in the newborn nursery under the current medical insurance fee schedule.