ABSTRACT
Pseudohypoaldosteronism (PHA) type l is a rare neonatal disease characterized by salt wasting, dehydration, hyperkalemia and metabolic acidosis. It is unresponsive to mineralocorticoid treatment with elevated aldosterone concentration. The three different modes of inheritance has been described. The autosomal dominant form has a mild clinical course and gradually improves with age. In this form, resistance to aldosterone seems to be restricted to the kidney. The autosomal recessive form displays generalized aldosterone resistance including kidney, colon, lung, sweat and salivary gland system. This form is more severe and requires life-long supplement with high-dose salt. The sporadic form is mild and resembles the autosomal dominant form. In this paper, we describe a male patient diagnosed as PHA type l at the age of 19 months. He presented with recurrent vomiting, diaphoresis accompanying hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma renin activity and aldosterone level. Ultimately he has improved with treatment by oral sodium bicarbonate.
Subject(s)
Humans , Male , Acidosis , Aldosterone , Colon , Dehydration , Hyperkalemia , Hyponatremia , Kidney , Lung , Plasma , Pseudohypoaldosteronism , Renin , Salivary Glands , Sodium Bicarbonate , Sweat , Vomiting , WillsABSTRACT
PURPOSE: We studied the value of clinical signs, laboratory findings and (99m)technetium dimercaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). METHODS: A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. RESULTS: Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. CONCLUSION: An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. Therefore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.
Subject(s)
Child , Humans , C-Reactive Protein , Child, Hospitalized , Escherichia coli , Fever , Leukocyte Count , Retrospective Studies , Sensitivity and Specificity , Succimer , Urinary Tract Infections , Urinary Tract , Vesico-Ureteral RefluxABSTRACT
PURPOSE: To evaluate mortality and morbidity of very low birth weight infants (VLBWI) in Cheongju area from 2002 to 2004. METHODS: Two neonatal intensive care units (NICU), 1 university hospital and 1 general hospital in Cheongju participated in this study. We performed a retrospective review of medical records of VLBWIs in 2 NICUs with literature review. Survival rate, neonatal morbidity, cause of death, and risk factors contributing mortality and neurodevelopmental complications were analyzed. RESULTS: The total number of VLBWI was 152 (4.65%) including 43 (1.31%) extremely low birth weight infants (ELBWI) who were less than 1,000 g of birth weight. The survival rates were 82.9% and 69.8% for VLBWI and ELBWI, respectively. The incidence of respiratory distress syndrome was 60%, bronchopulmonary dysplasia 20.6%, severe retinopathy of prematurity 24%, and severe intravetricular hemorrhage 7.3%. More than half of death occurred in the first week of life, and the main causes were respiratory distress syndrome and sepsis. The major risk factors contributing mortality and neurodevelopmental complications were birth weight, gestational age, Apgar score, respiratory distress syndrome, and high-grade intraventricular hemorrhage. CONCLUSION: Survival rate (82.9%) of VLBWI in Cheongju area, although lower than those of certain NICUS in Seoul, was comparable to those from nation-wide statistics. The incidence of major morbidity seemed somewhat higher than those of other areas in Korea. A systematic national support would be strongly needed for the balanced development of regional NICUs by means of their facilities, personal resources and financial support, etc.
Subject(s)
Humans , Infant , Infant, Newborn , Apgar Score , Birth Weight , Bronchopulmonary Dysplasia , Cause of Death , Financial Support , Gestational Age , Hemorrhage , Hospitals, General , Incidence , Infant, Low Birth Weight , Infant, Very Low Birth Weight , Intensive Care Units, Neonatal , Korea , Medical Records , Mortality , Retinopathy of Prematurity , Retrospective Studies , Risk Factors , Seoul , Sepsis , Survival RateABSTRACT
Moyamoya disease is a rare occlusive cerebrovascular disease characterized by stenosis or occlusion of the main cerebral arteries. It has a tendency for multifactorial inheritance and familial occurrence, although its pathogenesis is not clear. We observed this disease in two girls from the same family:one was eight years old and the other was 45 months. They presented with transient ischemic attacks. We performed cerebral angiography on both patients and magnetic resonance angiography (MRA) on the younger. Both approaches showed the typical features of moyamoya disease, and MRA successfully revealed abnormal findings specific for the disease in the second child. Both children received encephaloduroarteriosynangiosis (EDAS) and this produced good results. MRA is thus a powerful and noninvasive way of detecting individuals at high risk of developing this disease. Considering the reported familial incidence of moyamoya disease in Japan, a careful search for family members using MRA would probably reveal many more such cases in Korea.
Subject(s)
Child , Female , Humans , Cerebral Angiography , Cerebral Arteries , Constriction, Pathologic , Incidence , Ischemic Attack, Transient , Japan , Korea , Magnetic Resonance Angiography , Moyamoya Disease , Multifactorial InheritanceABSTRACT
PURPOSE: Most neonatal abdominal masses are benign in nature, but early detection and management is important to avoid the development of complications. So, we studied the causes of neonatal abdominal masses and the efficacy of ultrasonography for early detection and diagnosis. METHODS: We reviewed 36 newborn infants with abdominal masses who had been admitted to the nursery and Neonatal Intensive Cave Unit of Seoul National University Children' s Hospital from Jan. 1, 1989 to Dec. 31, 1995. RESULTS: The mean gestational age was 38 weeks and the male to female ratio was 22 : 14. All masses were benign and the most common etiology was hydronephrosis (41.7%). The following etiologies were multicystic dysplastic kidney, polycystic kidney, ovarian cyst, hemangioendothelioma, teratoma, meconium peritonitis, mesenteric cyst and intestinal duplication cyst. All except one were detected before the first 48hours, and among them, 32 cases (88.9%) detected antenatally. All patients except one who died shortly after birth, were examined by abdominal ultrasonography, and 31 of 35 masses (88.6%) had the same diagnosis of ultrasonography. Five cases had associated abnormalities in the opposite kidney and other organs. CONCLUSION: The most common etiology of neonatal abdominal masses was hydronephrosis and majority of masses originated from genitourinary system. Most abdominal masses were detected antenatally and abdominal ultrasonography was an efficient method for the diagnosis of neonatal abdominal masses.
Subject(s)
Female , Humans , Infant, Newborn , Male , Diagnosis , Gestational Age , Hemangioendothelioma , Hydronephrosis , Kidney , Meconium , Mesenteric Cyst , Multicystic Dysplastic Kidney , Nurseries, Infant , Ovarian Cysts , Parturition , Peritonitis , Polycystic Kidney Diseases , Seoul , Teratoma , Ultrasonography , Ultrasonography, Prenatal , Urogenital SystemABSTRACT
PURPOSE: Patent ductus arteriosus (PDA) of prematurity is very important disease to study because it causes many cases of perinatal morbidity and its incidence is now increasing. Nowadays indomethacin is the drug of choice for PDA closure, but its use has been limited due to its side effects. Therefore, we compared the effect and side effects of indomethacin according to the infusion method, continuous versus intermittent infusion, to find better an administration method. METHODS: Twenty-five preterm infants who were admitted to Seoul National University Children's Hospital (SNUCH) NICU from March 1995 to August 1996 with a diagnosis of respiratory distress syndrome (RDS) and PDA, were enrolled. They were randomly assigned to intertmittent lV group or continuous lV group. Each group received three intermittent doses or continuous infusion over 36 hours, respectively. We analyzed the perinatal history, time of diagnosis and treatment of PDA, size of PDA, and compared the laboratory parameters, intraventricular hemorrhage (IVH), periventricular echogenecity (PVE), ductal closure and perinatal morbidity before and 48 hours after indomethacin administration between the two groups. RESULTS: Fourteen infants (birth weight 1,149 +/- 373g) were intermittent lV group and eleven infants (birth weight 1,212 +/- 504g) were continuous lV group. There were no significant difference between the groups in perinatal history, pretreatment laboratory parameters, ductal closure, and perinatal morbidity. Patients with IVH of grade 2 or more increased significantly in intermittent lV group (50%) compared to continuous lV group (9%), and PVE progressed significantly in intermittent lV group (64%) compared to continuous lV group (18%). CONCLUSION: Continuous infusion of indomethacin for PDA closure in preterm infants with RDS appears to be as effective as intermittent infusion of indomethacin in closing PDA and have less side effects such as IVH and PVE progress.
Subject(s)
Humans , Infant , Infant, Newborn , Diagnosis , Ductus Arteriosus, Patent , Hemorrhage , Incidence , Indomethacin , Infant, Premature , SeoulABSTRACT
PURPOSE: Patent ductus arteriosus (PDA) of prematurity is very important disease to study because it causes many cases of perinatal morbidity and its incidence is now increasing. Nowadays indomethacin is the drug of choice for PDA closure, but its use has been limited due to its side effects. Therefore, we compared the effect and side effects of indomethacin according to the infusion method, continuous versus intermittent infusion, to find better an administration method. METHODS: Twenty-five preterm infants who were admitted to Seoul National University Children's Hospital (SNUCH) NICU from March 1995 to August 1996 with a diagnosis of respiratory distress syndrome (RDS) and PDA, were enrolled. They were randomly assigned to intertmittent lV group or continuous lV group. Each group received three intermittent doses or continuous infusion over 36 hours, respectively. We analyzed the perinatal history, time of diagnosis and treatment of PDA, size of PDA, and compared the laboratory parameters, intraventricular hemorrhage (IVH), periventricular echogenecity (PVE), ductal closure and perinatal morbidity before and 48 hours after indomethacin administration between the two groups. RESULTS: Fourteen infants (birth weight 1,149 +/- 373g) were intermittent lV group and eleven infants (birth weight 1,212 +/- 504g) were continuous lV group. There were no significant difference between the groups in perinatal history, pretreatment laboratory parameters, ductal closure, and perinatal morbidity. Patients with IVH of grade 2 or more increased significantly in intermittent lV group (50%) compared to continuous lV group (9%), and PVE progressed significantly in intermittent lV group (64%) compared to continuous lV group (18%). CONCLUSION: Continuous infusion of indomethacin for PDA closure in preterm infants with RDS appears to be as effective as intermittent infusion of indomethacin in closing PDA and have less side effects such as IVH and PVE progress.
Subject(s)
Humans , Infant , Infant, Newborn , Diagnosis , Ductus Arteriosus, Patent , Hemorrhage , Incidence , Indomethacin , Infant, Premature , SeoulABSTRACT
Blood glucose levels were measured in 89 healthy term neonates during the first 72 hours using the SureStep, a newly developed reagent test strip method by LifeScan. The blood samples were obtained by heel-stick puncture and blood glucose concentrations were monitored at birth(0), 2, 4, 6, 12, 24, 48, and 72 hours after birth. Mean and standard deviation of their measurement were compared according to postnatal hours and type of delivery. Comparison of significance between mean plasma glucose levels were made with the Wilcoxon rank sum test and significance level of 0.05 was used to determine which pair-wise comparisons were significantly different. The mean plasma glucose concentrations of first 6 hours were significantly lower than those of 12, 24, 48, 72 hours. This finding indicates that plasma glucose stabilization in healthy fullterm neonates can be expected with the feedings. The mean plasma glucose concentration at birth in the neonates of cesarean section (64.5+-8.06 mg/dl) was significantly lower than that of vaginal delivery (80.3+-20.7 mg/dl), but there were no significant differences after 2 hours. This may be due to the difference in prenatal care of obstetric department of Horamae hospital (C/S: midnight NPO and Hartmann solution, V/D: NPO with labor pain and 5% dextrose solution intravenously). In summary, the changes in perinatal care, especially prenatal fluid therapy and time of first feeding should be considered in defining neonatal hypoglycemia. Our data suggest that hypoglycemia should he defined as below 40 mg/dl during the first 6 hours and below 55 mg/dl thereafter.
Subject(s)
Female , Humans , Infant, Newborn , Pregnancy , Blood Glucose , Cesarean Section , Fluid Therapy , Glucose , Hypoglycemia , Labor Pain , Parturition , Perinatal Care , Plasma , Prenatal Care , Punctures , Reagent StripsABSTRACT
PURPOSE: Patent ductus arteriosus (PDA) is a common disease in very low birth weight infants (VLBWI). Hemodynamically significant PDA increases the morbidity and mortality of premature infants. Based on experimental model, light inhibits the constriction of immature piglet's ductal rings. No specific mechanism adequately explains the effect of light on the relaxation of PDA. Several hypotheses, including activation of photosensitive metabolites, alterations in receptors, or alterations in prostaglandin metabolism, have been postulated. The purpose of this study was to evaluate the influence of phototherapy on incidence of PDA in VLBWI. Mehtods : Sixty-three infants with birth weights less than 1,500 gm from March 1994 to February 1996 who were admitted in NICU of Seoul National University Children's Hospital were included. Thirty-four infants from March 1995 to February 1996 were shielded with aluminium foils on left chest during phototherapy (Shield group) and twenty-nine infants from March 1994 to February 1995 were not shielded (No shield group : control group). We investigated the incidence and the perinatal risk factors of PDA. RESULTS: 1) The incidence of PDA was 18% in shield group and 41% in control group. There was statistically significant between the two groups (P<0.05). 2) There was not statistically significant between two groups with gestational age, birth weight, sex, delivery mode, etc. 3) The perinatal risk factors which were statistically significant were group and presence of respiratory distress syndrome (RDS), and use of artificial surfactant. With linear logistic regression analysis, only group (OR=8.3, 95% CI=1.17-58.69) and presence of RDS (OR=21.3, 95% CI=1.39-329.81) were proved to be related to the occurrence of PDA. CONCLUSIONS: We conclude that chest shielding during phototherapy is a simple and inexpensive method to decrease the incidence of PDA.
Subject(s)
Humans , Infant , Infant, Newborn , Birth Weight , Constriction , Ductus Arteriosus, Patent , Gestational Age , Incidence , Infant, Premature , Infant, Very Low Birth Weight , Logistic Models , Metabolism , Models, Theoretical , Mortality , Phototherapy , Relaxation , Risk Factors , Seoul , ThoraxABSTRACT
PURPOSE: Retinopathy of prematurity(ROP) continues to be a serious problem in infants who survived with neonatal intensive care. Nevertheless, there is neither available data about the incidence of ROP which may be the basis of the epidemiology, nor analysis of the risk factors of ROP in Korea. The purpose of this retrospective study was to evaluate the incidence of ROP and to analyze perinatal risk factors of ROP. METHODS: The study population was inborn neonates of Seoul National University Hospital during the three-year period, from Jan. 1991 to Dec. 1993. The total number of infants was 5,764, and the incidence of ROP was evaluated according to gestational age and birthweight. Infants with birthweight under 1,500gram or with gestational age less than 33 weeks were involved in this study, in 168 of the 242 infants, their eyes were examined. Of these, 71 developed some degree of ROP, and 12 received cryotherapy. The comparisons of perinatal risk factors of ROP between the group with and without ROP, and in the group with ROP, between with and without cryotherapy have been made. RESULTS: 1) The cumulative incidence of ROP according to gestational age was 100% in less than 28 weeks, 96.0%, 80.4%, 66.7%, 57.3%, 44.1%, 32.2%, 23.1%, 15.8%, and 10.6% in less than 29, 30, 31, 32, 33, 34, 35, 36, and 37 weeks, respectively. 2) The cumulative incidence of ROP according to birthweight was 100% in under 750gram, 90.9%, 69.8%, 56.8%, 39.1%, 26.8%, 16.0%, and 9.9% in under 1,000gram, 1,250gram, 1,500gram, 1,750gram, 2,000gram, 2,250gram, and 2,500gram, respectively. 3) The perinatal risk factors which were statistically significant were gestational age, birthweight, Apgar score at 1 and 5 minutes, duration of hospitalization, ventilation and oxygen supply, number of transfusions, acidosis, hyperoxia, maximum fraction of inspired oxygen, maximum peak inspiratory pressure, maximum frequency, and the presence of pneumonia, apnea-bradycardia syndrome, intraventricular hemorrhage, sepsis, hypocalcemia, hyperglycemia, bronchopulmonary dysplasia, administration of aminophylline or furosemide. With linear logistic regression analysis, only gestational age (OR=0.930, 95% CI=0.887-0.975) and hyperoxia (OR=1.006, 95% CI=1.003-1.009) were proved to relate to the occurrence of ROP independently. CONCLUSIONS: ROP was developed in 44.1% of infants less than 33 weeks and in 56.8% of infants under 1,500gram and the occurrence of ROP was related significantly only to gestational age and hyperoxia.
Subject(s)
Humans , Infant , Infant, Newborn , Acidosis , Aminophylline , Apgar Score , Bronchopulmonary Dysplasia , Cryotherapy , Epidemiology , Furosemide , Gestational Age , Hemorrhage , Hospitalization , Hyperglycemia , Hyperoxia , Hypocalcemia , Incidence , Intensive Care, Neonatal , Korea , Logistic Models , Oxygen , Pneumonia , Retinopathy of Prematurity , Retrospective Studies , Risk Factors , Seoul , Sepsis , VentilationABSTRACT
Eimeria tenella, an intracellular protozoan parasite infecting the epithelial cells of the ceca of chickens, causes severe diarrhea and bleeding that can lead its host to death. It is of interest that E. tenella first penetrate into the mucosal intraepithelial lymphocytes (IEL) before they parasitize crypt or villous epithelial cells. This in vitro study was undertaken to know whether the penetration of E. tenella into such a lymphoid cell is a beneficial step for the parasite survival and development. Three sequential experiments were performed. First, the in vitro established bovine kidney cell line, MDBK cells, were evaluated for use as host cells for E. tenella, through morphological observation. Second, the degree of parasite development and multiplication in MDBK cells was quantitatively assayed using radioisotope-labelled uracil (3H-uracil). Third, the E. tenella sporozoites viability was assayed after preincubation of them with chicken spleen cells. E. tenella oocysts obtained from the ceca of the infected chickens were used for the source of the sporozoites. Spleen cells (E) obtained from normal chickens (FP strain) were preincubated with the sporozoites (T) at the E:T ratio of 100:1, 50:1 or 25:1 for 4 or 12 hours, and then the mixture was inoculated into the MDBK cell monolayer. Morphologically the infected MDBK cells revealed active schizogonic cycle of E. tenella in 3-4 days, which was characterized by the appearance of trophozoites, and immature and mature schizonts containing merozoites. The 3H-uracil uptake by E. tenella increased gradually in the MDBK cells, which made a plateau after 48-60 hours, and decreased thereafter. The uptake amount of 3H-uracil depended not only upon the inoculum size of the sporozoites but also on the degree of time delay (preincubation; sporozoites only) from excystation to inoculation into MDBK cells. The 3H-uracil uptake became lower as the preincubation time was prolonged. In comparison, after preincubation of sporozoites with spleen cells for 4 or 12 hours, the 3H-uracil uptake was significantly increased compared with that of control group. From the results, it was inferred that, although the penetration of E. tenella sporozoites into the lymphoid cells such as IEL is not an essential step, it should be at least a beneficial one for the survival and development of sporozoites in the chicken intestine.
ABSTRACT
No abstract available.