Analysis on the cause of eosinophilia in a neonatal intensive care unit / 소아과

PURPOSE: Although eosinophilia is a common laboratory finding in many neonatal intensive care units (ICUs), its causative mechanisms remain obscure. We aimed to determine the causes of eosinophilia in the neonatal ICU environment. METHODS: Serial eosinophil counts were determined weekly for 288 hospitalized, appropriately grown neonates. Infants were divided into four groups according to gestational age, and the incidence and etiologic factors of eosinophilia were retrospectively studied. RESULTS: Absolute eosinophilia (>700/mm3) was documented in 18% (52/288) of neonates. Twenty-two infants (42.3%) exhibited mild eosinophilia (700-999 cells/mm3), 27 (51.9%) exhibited moderate eosinophilia (1,000-2,999 cells/mm3), and 3 (5.8%) exhibited severe eosinophilia (>3,000 cells/mm3). Of the 288 infants studied, 54 suffered sepsis. Thirty of these 54 infants (55.6%) showed eosinophilia, and 22 out of the remaining 234 infants (9%) without sepsis showed eosinophilia, indicating that eosinophilia was more prevalent in the sepsis group (P <0.05). All 5 infants suffering from bronchopulmonary dysplasia showed eosinophilia, and 47 out of the remaining 283 infants (16.7%) without bronchopulmonary dysplasia showed eosinophilia. Thus, eosinophilia was more prevalent in the bronchopulmonary dysplasia group (P <0.05). Furthermore, increased prevalence of eosinophilia was associated with respiratory distress syndrome, ventilator use, blood transfusion, and total parenteral nutrition (P <0.05). CONCLUSION: Our results suggest that eosinophilia is influenced by sepsis and bronchopulmonary dysplasia, although it can also occur idiopathically at birth. Moreover, the potential role of eosinophils in conditions such as wound healing and fibrosis in sepsis or chronic lung disease may be a cause of eosinophilia.

A Case of a del(8p)/dup(8q) Recombinant Chromosome

A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.1). Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1-->qter. Because 8p21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.

Isolated 3-Methylcrotonyl CoA Carboxylase Deficiency Detected by Newborn Screening Program Using Tandem Mass Spectrometry / 소아과

Isolated deficiency of 3-methylcrotonyl CoA carboxylase is a rare disorder of the catabolic pathway for leucine and many patients have mild symptoms or no symptom. However, the introduction of tandem mass spectrometry in newborn screening has revealed an unexpectedly high incidence of this disorder. We report an asymptomatic premature infant with isolated 3-methylcrotonyl CoA carboxylase deficiency detected by newborn screening program using tandem mass spectrometry. She was born at preterm, 36 weeks of gestation and her birth weight was 1,912 gm. She was delivered by Cesarian section due to maternal preeclampsia and oligohydramnios. An elevation of 3-hydroxyisovalerylcarnitine in a blood sample obtained at Seven days was detected by tandem mass screening. Massively elevated excretion of 3-hydroxyisovalerate and 3-methylcrotonylglycine was detected in the urine collected at 15 days. L-carnitine(100 mg/kg/day) was administrated orally to correct sencondary carnitine deficiency. Carnitine is conjugated with metabolites, to decrease the potential toxic effects. She is asymptomatic to date, and her growth and development are within normal limits.

The Efficacy of Nebulized 3 Percent Hypertonic Saline Solution and Fenoterol in Infants with Bronchiolitis / 소아과

PURPOSE: To evaluate the effect of inhaled hypertonic saline solution in hospitalized infants with bronchiolitis. METHODS: A randomized double blind trial was performed from October 2003 to May 2004. A total of eighty patients <1 year of age with a clinical diagnosis of acute viral bronchiolitis were enrolled and assigned to receive either of the following:inhalation of 2 mL(0.5 mg) fenoterol added to 2 mL of 0.9 percent saline solution(group 1; n=40) or 2 mL(0.5 mg) fenoterol added to 2 mL of 3 percent saline solution(group 2; n=40). This therapy was repeated at six hours interval after admission. They were evaluated daily just before and 20 minutes after nebulization. The outcome measures included changes in clinical severity score(based on respiratory rate, presence of wheezing, retraction, and general condition) after nebulization and duration of hospitalization. RESULTS: In the clinical severity score, a significant improvement was observed during the 72 hours of hospitalization in both groups(P<0.05). The basic clinical severity scores before inhalation were decreased significantly faster in group 2 as compared to group 1 on each day of treatment(P<0.05). The mean duration of hospital stay was significantly reduced in group 2 than group 1(5.9+/-1.9 days versus 7.4+/-2.0 days, P<0.05). No adverse effects were associated with inhaled therapy. CONCLUSION: These results suggest that a nebulized 3 percent saline solution plus 0.5 mg fenoterol may be more effective than a 0.9 percent saline solution plus 0.5 mg fenoterol in accelerating the clinical recovery of infants with viral bronchiolitis.

The Efficacy of Nebulized Epinephrine Versus Fenoterol in Hospitalized Infants with Acute Bronchiolitis / 소아과

PURPOSE: Acute bronchiolitis is a lower respiratory tract disease, resulting from inflammatory obstruction of the small airway. The main treatment of acute bronchiolitis is supportive but, numerous investigators have examined the efficacy of beta agonist as bronchodilators. In acute bronchiolitis, mucosal edema in the bronchioles may be an important cause of airway obstruction therefore, an alpha and beta agonist might be useful in the treatment of this disease. So we examined the efficacy and safety of repeated nebulized epinephrine compared to fenoterol. METHODS: This randomized double blind study involved 106 hospitalized infants with wheezing, under one year age and acute onset of respiratory distress. They were randomly separated into two groups, and treated with either nebulized 0.1% epinephrine 0.5 mg in 3.5 mL of 0.9% saline solution (group 1; n=50) or nebulized fenoterol 0.5 mg in 2 mL of 0.9% saline solution(group 2; n=50). This therapy was repeated at six hour intervals after hospital admission. Observations were made at admission and just before, 30 minutes after nebulization. The primary outcome measures used were the degree of change in clinical scores. The secondary outcome measures used were the length of the hospital stay. RESULTS: A significant improvement in the clinical score was noted during 72 hours of hospitalization in both groups(P<0.001). But, there were no significant differences between the groups in clinical score improvement. There were no significant differences between the groups in the length of the hospital stay(P=0.055). No adverse effects were associated with nebulized therapy. CONCLUSION: There were no group differences in the effectiveness of therapy for infants hospitalized with acute bronchiolitis.

The Diagnostic Value of Gross Bloody Stool on Intussusception / 소아과

PURPOSE: To evaluate the diagnostic value of gross bloody stools as a predictor of intussusception. METHODS: We reviewed 159 cases retrospectively that had an ultrasound examination for diagnosis of intussusception from January 2001 to December 2003 at Ilsin Christian Hospital. We compared each symptom and assessed the diagnostic value of gross bloody stools in the patients with suspected intussusception. RESULTS: Thirty-six of the 159 patients had intussusception. Of 36 patients with intussusception, 19 (52.8%) children were male and 28(77.8%) children were younger than two years. The most common symptom was intermittent abdominal pain or irritability. But its frequency among the intussusception (24.2%) was lower than frequency of gross bloody stools(50.9%). The sensitivity, specificity, positive predictive value(PPV) and negative predictive value(NPV) of gross bloody stools in patients with suspected intussusception were as follows; sensitivity=77.8%(95% CI 60.8-89.9), specificity=78.0%(95% CI 69.7-85.0), PPV=50.9%(95% CI 37.1-64.6), NPV=92.3%(95% CI 85.4-96.6)(Diagnostic accuracy 78.0 %; 95% CI 70.7-84.2). CONCLUSION: It is useful for diagnosis of intussusception to confirm the presence of gross blood in stools.

Mortality and Morbidity of Very Low Birth Weight Infants in Busan Area during Recent 6 Years

PURPOSE: To evaluate mortality and morbidity of very low birth weight (VLBW) infants born in Busan from 1996 to 2001. METHOD: Seven neonatal intensive care units (including 3 university hospitals and 4 general hospitals) in Busan participated for this study. We performed retrospective review of 756 VLBW infants who were born in this area during study period was done to assess their mortality and morbidity. RESULT: The total number of VLBW infant was 756 (incidence: 0.97%, mean gestational age: 29.5 +/- 2.9 wk, mean birth weight: 1165+/-232 g) including 187 extremely low birth weight infants who were less than 1, 000 g birth weight (24.7%). The survival rate was 61.0% (461 of 756). It increased from 56.4% in early period (1996 to 1998) to 65.2% in late period (1999 to 2001) (P<0.01). The incidence of respiratory distress syndrome was 61.5% (466 of 756), patent ductus arteriosus 12.3% (93 of 756), blood culture positive sepsis 10.2% (77 of 756), bronchopulmonary dysplasia 9.3% (70 of 756), severe intracranial hemorrhage 6.5% (49 of 756), necrotizing enterocolitis 4.9% (37 of 756), severe retinopathy of prematurity 3.7 % (28 of 756). The main causes of death were respiratory distress syndrome and sepsis. CONCLUSION: The survival rate of VLBW infant in Busan area during recent 6 years was 61.0%, which is lower than other single unit result of our country, recent Japanese or North American data. We think both modernized facilities in NICU and well-trained medical personnel are needed to improve survival.

A Case of Maple Syrup Urine Disease Associated with Acrodermatitis Enteropathica-like Syndrome Due to Iisoleucine Deficinecy During Diet Therapy

Maple syrup urine disease(MSUD) is an autosomal recessive disorder involving the metabolism of the branched-chain amino acids(BCAA) such as leucine, isoleucine and valine. The disorder is due to a defect in branched-chain alpha-ketoacid dehydrogenase(BCKAD) and the classic form causes rapid progressive and overwhelming illness beginning in the first weeks of life, present with poor feeding, lethargy, change in muscle tone, acidosis, seizures and coma. The goal of therapy in acutely ill patients with MSUD is an immediate reduction in the plasma levels of the BCAAs and branched-chain ketoacids. In this report, we describe an infant with MSUD who was treated by dietary therapy alone. During the therapy, acrodermatitis enteropathica-like syndrome developed with low plasma isoleucine concentration while she was receiving a formula deficient in BCAAs.

One Case of Subcapsular Hematoma with Persistent Neonatal Hyperbilirubinemia

A neonate may incur liver injury during spontaneous delivery. The liver is the organ most likely to be injured during the birth process. Breech presentation and manipulation are the most common causes of hepatic trauma. However, hepatic hemorrhage can occur in any infant, regardless of size and the type of delivery. Infants with subcapsular hemorrhage are usually asymptomatic at birth, and gross hepatic rupture from these lesions is unusual. In patient with primary rupture, major bleeding takes place immediately, explaining the high percentage of neonatal deaths with massive liver rupture. If subcapsular hemorrhage remain intracapsular with spontaneous resolution, the only treatment needed in subcapsular hemorrhage may be transfusion. We experienced one case of neonatal subcapsular hemorrhage with persistent hyperbilirubinemia. We report this case with the brief review of related literature.

A Case of Transient Hyperammonemia of the Newborn Infant

Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The etiology is unknown. Infant had mild respiratory distress that progressed within 48 hours to deep coma requiring ventilatory assistance and had marked hyperammonemia. The degree of neurologic impairment and developmental delay in this disorder depends on the duration of the hyperammonemic coma. So, treatment of hyperammonemia should be initiated promptly and continued vigorously. We report of a preterm infant (34+5 weeks of gestation) presenting with respiratory distress, seizure, coma, and marked elevated plasma ammonia level.

Comparison of Preventive Effect of RDS and Neonatal Morbidity between Antenatal Dexamethasone versus Ambroxol Administration

PURPOSE: Antenatal dexamethasone administration is associated with a significant lowering respiratory distress syndrome (RDS) incidence, but can increase neonatal infection. Ambroxol has been accepted as an alternative treatment to dexamethasone and is of at least equal efficacy but without adverse reaction. The aim of this study was to evaluate the effectiveness of ambroxol versus dexamethasone in RDS incidence and neonatal infection. METHODS: In this study, 30 infants, who received prenatal dexamethasone therapy, were compared retrospectively to 19 infants who received prenatal ambroxol therapy and 45 infants who received placebo during 28 to 34 weeks' of gestation. RESULTS: RDS incidence was comparable in both the dexamethasone (10.0%) and ambroxol (10.5%) groups but higer in the control group (26.6%). The puerperal infection rate in the mothers of these infants was 33.3% in the dexamethasone group, 10.5% in the ambroxol group and 20.0% in the control group. Neonatal infection in the 28 days following delivery was 56.6% in the dexamethasone group, 26.3% in the ambroxol group and 26.6% in the control group. Neonatal infection rate of the dexamethasone group was higher than ambroxol and control groups (P<0.05). When premature rupture of membrane was controlled, the sepsis rate (<28 days) was significantly lower in the ambroxol group than in the dexamethasone group (P<0.05), but puerperal infection and sepsis (<7 days) were not significantly different. CONCLUSOIN: Ambroxol was as effective as the dexamethasone in reducing the RDS incidence. Neonatal and puerperal infection were significantly higher in the dexamethasone group than in the ambroxol group.

Cord Blood Interleukin 6 and Intrauterine Infection in Preterm Infant

PURPOSE: Our purpose was to examine the relationship between the intrauterine infection and the concentration of cord blood interleukin 6 in preterm infants. METHODS: Patients delivering preterm infants because of spontaneous preterm labor were evaluated for clinical signs of chorioamnionitis, which was defined as a temperature of >or=37.8 degrees C along with >or=2 of the followings : Significant maternal tachycardia (>or= 100/min), fetal tachycardia (>or=160/min), purulent discharge, uterine tenderness, and leukocytosis (WBC >or=15,000c ells/mm3). Their placentas were examined for histologic chorioamnionitis. Cord blood was assayed for Interleukin 6 and CRP. And Interleukin 6 and CRP were sequentially determined at 12-24hour and at 36-48 hour. An elevated interleukin 6 was determined to be 20 pg/mL. Infants were evaluated for the evidences of early and late neonatal sepsis, respiratory distress syndrome, necrotizing enterocolitis, intraventricular hemorrhage, neonatal pneumonia, and bronchopulmonary dysplasia. RESULTS: Umbilical cord blood interleukin 6 was significantly higher in the presence of intrauterine infection than in the absence of intrauterine infection (208.4+/-426.3 pg/mL VS 13.3+/-20.6 pg/mL P=0.018). And the frequencies of histologic chorioamnionitis, early neonatal sepsis, neonatal pneumonia, and morbidity were significantly higher in the presence of intrauterine infection than in the absence of intrauterine infection (P=0.003, P=0.000, P=0.042, and P=0.000, respectively). There was significant difference in gestational age, but no significant differences in maternal age, parity, birth weight, method of delivery, respiratory destress syndrome, necrotizing enterocolitis, intraventricular hemorrhage, and bronchopulmonary dysplasia. Using receiver operator characteristic curves, the best cutoff for interleukin 6 was found to be 20 pg/mL. CONCLUSION: Cord blood interleukin 6 was significantly elevated in the intrauterine infection in the preterm infant.

Gastric Emptying Time in Preterm Infents: Comparison between Breast Milk and Formula Milk

PURPOSE: Failure of adequate gastric emptying frequently interferes with successful enteral nutrition in infants. Study of gastric emptying in preterm infants has been hampered by the absence of a suitable and, valid technique, but ultrasonic assessment is well tolerated by infants. The aim of this study is to compare gastric emptying rates of breast milk and formula within preterm infants using an ultrasonic technique. METHODS: Ten infants(6 males, 4 females) were studied on 20 occasions. Median gestation of the group was 33 wks(29-38 wks), birthweight was 1506gms(850-1870gms). Each infant was receiving a minimum of 150 ml/kg/day of breast milk. We conducted the study with breast milk first and with formula milk 2 days later. With the infant in the right lateral position, ultrasonic images of the gastric antrum were obtained using the aorta bifurcational branch of superior mesenteric artery as constant landmarks. Measurements of antral cross sectional area(ACSA) were made before the feed and then sequentially, following its completion until ACSA returned to its pre-feeding state. Half-emptying time(t1/2) was calculated as the time taken for the ACSA to fall to half the maximal increment. The test was well tolerated by all subjects. RESULTS: There was a strong linear relationship between gastric volume and ACSA. The half-emptying time for breast milk was less than formula : t1/2 breast milk 32.0+/-9.8 min; t1/2 formula 45.0+/-11.6 min, P=0.0004. CONCLUSION: The data demonstrate that human milk increases gastric emptying compared to formula. This has important implications for the management of preterm infants who have feeding intolerances.

A Case of Citrullinemia Controlled by Diet and Arginine

Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The clinical symptoms include vomiting, lethargy, seizure, coma and ultimately death if hyperammonemia is not controlled. We report a case of 9- day old male with citrullinemia who was initially treated with sodium benzoate during acute stage followed by gradual weaning to discontinuation. Hyperammonemia was well controlled by low protein milk diet and arginine.

A Case of Hydranencephaly Caused by Internal Carotid Artery Stenosis: Diagnosis with Doppler Sonogram

Hydranencephaly is congenital absence of the cerebral hemispheres which are replaced by a large fluid-filled cavity. The brain stem and basal ganglia are well formed and rudiments of frontal k occipital cortex may be present. We experienced a case of hydranencephaly caused by both internal carotid artery stenosis. We diagnosed it through the brain CT sonogram and doppler sonogram. A brief review of the related literatures was made.

Bone Mineral Metabolism and Rickets Compared between Fortified Human Milk and Preterm Formula Feeding in Prematurity

PURPOSE: To compare the effect of breast milk containing fortifier with preterm formula on bone mineral metabolism in premature infants, we evaluated the state of bone mineralization, biochemical alteration and the frequency of rickets during the first five months of life. METHODS: Fourteen fortified breast milk-fed infants and eleven preterm formula-fed infants who were born at Il-Sin Christian Hospital from August, 1996 through July, 1997, were studied. The breast milk-fed group received human milk fortifier. The intake of Ca, P, Mg, protein, and fat was calculated at one month of age. Birth weight and weight at one, two and five months of age were measured. Serum Ca, P, alkaline phosphatase and 25-hydroxyvitamine D were measured at one month of age, and the wrist received X-ray examinations monthly. Total body bone mineral content was measured by Dual energy X-ray absorptiometry (Lunar WI) at two and five months of age. RESULTS: The two groups were similar in birth weight, gestational age, and weight at one, two and five months of age. Enteral Ca, P, protein, Mg, and fat intake, and urinary excretion of Ca, P were similar among the two groups. Serum Ca, P, ALP and 25-hydroxyvitamin D were not different. Occurrence of rickets and bone mineral content were similar among the two groups. CONCLUSION: Fortified breast milk-fed infants and preterm formula-fed infants showed no difference in total body bone mineral content and occurrence of rickets. Ultimately, we could enhance the advantage of breast milk fed to premature infants by adding fortifier.

Effect of Indomethacin Therpy on Prevention of Intraventricular Hemorrhage in Very

PURPOSE: Intraventricular hemorrhage(IVH) is a major risk factor for neurodevelop- mental handicap in very low birth weight infant. So we carried out to determine the efficacy of indomethacin in reducing the incidence of intraventricular heorrhage in very low birth weight infant. METHODS: A prospective, random trial was carried out to determine the efficacy of indomethacin in preventing intraventricular hemorrhage. A total of 46 infants of less than 1500g birth weight admitted to NICU of IL Sin Christian Hospital from August 1995 to June 1997 were analyzed. Randomly, 23 infants were given indomethacin and 23 infants were not given as control group. Serial cranial ultrasound examination was performed. RESULTS: There were no differences in the birth weight, gestational age, sex, Apgar score, blood pressure, intake and output within the first 3 days between the indornethacin and control groups. Of 23 infants given indomethacin, four had germinal matrix or intraventricular hemorrhage, in comparison with 10 of 23 control infants(P=0.045). CONCLUSION: Prophylactic indomethacin lowers the incidence of IVH in very low birth weight infants.

An Analytic Study for Incidence of Childhood Leukemia in Pusan City, Korea / 대한소아혈액종양학회지

BACKGROUND: Cancer is the second cause of childhood death following accident, and leukemia is the most frequent childhood cancer in Korea. For the desirable control of childhood leukemia, of which the mortality is still high, the basic data for the incidence has a great importance. This is the second report from the data during 1991~1995 following the first one that analyzed the data from 328 cases of childhood leukemia during 1981~1990 in the same area, Pusan city, Korea. METHODS: The data were obtained from 138 new cases(84 males and 54 females from 0 to 15 years old) of childhood leukemia who had been living in the city of Pusan and were admitted to the 4 university hospitals and 11 general hospitals from 1991 to 1995. The cases were confirmed by CBC and bone marrow examination. RESULTS: The crude annual incidence rate per 100,000 population varied 1.50~5.30, 2.59~6.00 and 1.58~2.61 in the age group of 0~4 years, 5~9 years and 10~14 years, respectively. The standardized annual incidence rate per 100,000 population varied from 2.05 to 3.46(male 2.96~4.89, female 0.98~3.57). Sex ratio(male to female) was 1.58:1, 1.44:1, and 1.82:1 in total cases, ALL and AML, respectively, while incalculable in CML. By the major types of childhood leukemia, the cases were composed of 105 ALL (76.1%), 31 AML(22.5%), 2 CML(1.4%). CONCLUSION: It was concluded that the annual incidence rate of childhood leukemia per 100,000 population in Pusan city during 1991~1995 was similar to that of previous report during 1981~1990, while the proportion of ALL had tendency to increase up to that of United States, in contrast to the low proportions of ALL in the previous reports.

A Case of Neuronal Heterotopia

Neuronal migrational disorders of the brain represent abnormalities in the formation of the neocortex caused by faulty migration of the subependymal neuroblasts. The neuroblasts normally migrate between the sixth and 15th gestational week and in doing so form the six-layered neocortex. When the migration does not occur in a normal fashion the resultant brain anomalies include lissencephaly, pachygyria, schizencephaly, hemimegalencephaly, heterotopia, and polymicrogyria. Neuronal heterotopia is a collection of nerve cells in abnormal locations as a result of arrest of their radial migration, improper formation, or destruction of the radial glial fiber. We reported a case of neuronal heterotopia with brief review of related literatures.

A Case of Tetrasomy 9P

Supernumerary isochromosome resulting in autosomal tetrasomy are rare and have been described only for 12P, 18P, and 9P. Tetrasomy 9P, initially described by Ghymer et al, is a rare chromosomal aberration that has been described in 20 patients. Affected subjects show both cytogenetic and ohenotypic variability. Some patients have the abnormal cell line in all cells, but many display tissue limited mosaicism. The phenotype varies in severity from prenatal death to mild developmental delay and minor anomalies. We reported a infant with mild manifestations of tetrasomy 9p with brief review of related literatures.