Clinical characteristics of severe meconium aspiration syndrome / 소아과

PURPOSE: This study aims to describe the clinical characteristics of severe meconium aspiration syndrome (MAS) which required mechanical ventilation over 48 h and to delineate the progress of respiratory failure and radiographic findings in severe MAS. METHODS: Twelve infants admitted to the Neonatal Intensive Care Unit (NICU) of the Seoul National University Bundang Hospital diagnosed with severe MAS from January 2004 to July 2007 were analyzed retrospectively. RESULTS: The presence of persistent pulmonary hypertension of the newborn (PPHN) is the only independently significant risk factor for a longer hospital stay and longer duration of mechanical ventilation. Surfactant replacement therapy (SRT) was not randomized but only performed in infants with radiographic findings for respiratory distress syndrome (RDS). In the presence of radiographic findings for RDS, the duration of high-frequency oscillatory ventilation was significantly longer. PPHN developed in 8 infants (75%). The PPHN group had a significantly longer duration of mechanical ventilation. All infants who received SRT showed radiographic improvement within 12 h, but there was no significant change in the severity score during the same period. Infants without the PPHN complications showed significant decrease in the severity score within 12 h after SRT, whereas infants with PPHN complications did not. CONCLUSION: The clinical course of severe MAS differed significantly depending on the development of PPHN. SRT conferred radiographic improvement in infants who showed radiographic findings for RDS, but did not influence the clinical course of MAS significantly.

The Effects of Bone Marrow-Derived Mesenchymal Stem Cells on Alveolarization Inhibition Induced by Hyperoxia in Neonatal Rat

PURPOSE: We sought to determine whether bone marrow-derived mesenchymal stem cells (BMMSC) could attenuate the inhibition of alveolarization induced by hyperoxia. METHODS: Human BMMSC (SNU-hMSC) were infused into the peritoneal cavity (IP) or trachea (IT) of neonatal rats exposed to hyperoxia (90% O2 from D1) on D5. The rats were then exposed to the same degree of hyperoxia for another 9d and sacrificed on D21. Morphometric analysis of the lungs and immunofluorescent staining in order to determine cell fates of infused SNU-hMSC were performed. RESULTS: The airspace of the hyperoxia control group (90% O2 for 14d) was significantly larger and more simple (mean linear intercept [Lm] : 68+/-16 micrometer vs 33+/-3 micrometer) and the alveolar surface area [SA] was significantly smaller (646+/-72 cm2 vs 1,042+/-477 cm2) than those of the normoxia control group. The Lm of the BMMSC- infused groups was significantly shorter irrespective of infusion route (52+/-2 micrometer [IP], 50+/-8 micrometer [IT] vs 68+/-16 micrometer) and the SA of the BMMSC IP infusion group was significantly larger (646+/-172 cm2 vs 346+/-142 cm2) than those of the hyperoxia control group. The IT-, but not IP-, infused BMMSC groups were observed in lung tissue and assumed to be type I and type II alveolar epithelial cell phenotypes. CONCLUSION: BMMSC, when infused into neonatal rats exposed to hyperoxia, significantly attenuated the inhibition of alveolarization irrespective of the infusion route. It seems that BMMSC, when infused IT, engrafts into lung tissue and differentiates into alveolar epithelial cells. These results indicate that BMMSC could be considered as a potential candidate therapy for bronchopulmonary dysplasia.

Immediate Postnatal Serum Tumor Necrosis Factor-Alpha Concentration and the Development of Bronchopulmonary Dysplasia

PURPOSE: We tested the hypothesis that preterm infants who develop bronchopulmonary dysplasia (BPD) have higher concentration of serum tumor necrosis factor-alpha (TNF-alpha) within 8 hours after birth than those who do not. METHODS: Serum and tracheal aspirate (TA) TNF-alpha concentrations were measured by enzyme-linked immunosorbent assay in the thirty-four preterm infants born before 32 weeks of gestation. Multiple logistic regression analysis was done. RESULTS: Median concentrations of both serum and TA TNF-alpha were higher in BPD group than in non BPD group [serum TNF-alpha, 214.52 pg/mL (84.20-244.20) versus 100.07 pg/mL (78.43-225.52), P=0.037],[TA TNF-alpha, 13.12 pg/mL (10.43-64.67) versus 11.58 pg/ mL (9.76-58.53), P=0.038]. After making adjustments for the effects of gestational age at birth, 5 minute Apgar score less than 7 and histologic chorioamnionitis, only serum TNF-alpha concentration was independently significant [P=0.045 Odds ratio, 1.381 95% confidence interval, 1.007-1.895]. The diagnostic indices of TNF-alpha (cutoff, 97.1 pg/mL) as a predictor of development of BPD were: sensitivity of 85.7%, specificity of 38.5%, positive predictive value of 69.2%, negative predictive value of 62.5% (P=0.037). CONCLUSION: Increased serum TNF-alpha concentration within 8 hours after birth may be a significant risk factor of later development of BPD.

Clinical Manifestations of Ureaplasma urealyticum Colonization in Infants / 대한주산의학회잡지

OBJECTIVE: Ureaplasma urealyticum colonization is a significant cause of fetal and neonatal pneumonia and is associated with the prevalence of bronchopulmonary dysplasia (BPD) in preterm infants. This study was aimed to evaluate the relationship between U. urealyticum colonization and neonatal morbidity. METHODS: We tested 476 infants for U. urealyticum on the first day of life among infants admitted to the neonatal intensive care unit of Bun-dang Seoul National University Hospital from June 2003 to July 2006. Urine and endotracheal aspirates were processed by PCR (polymerase chain reaction) or culture for U. urealyticum colonization. We compared the morbidity in the colonized group with the non-colonized group. RESULTS: The study group consisted ot 136 infants less than 32 weeks of gestational age and 340 infants more than 32 weeks of gestational age. In infants less than 32 weeks of gestational age, 18 (13%) of 136 infants had 1 or more positive specimens by culture or PCR. BPD occurred more in the colonized group than in the non-colonized group (p=0.058) and respiratory distress syndrome (RDS) occurred significantly less in the colonized group (p=0.043). Total WBC counts on the third day of life was significantly increased in the colonized group (p=0.003) and this significance was prominent in the neutrophil fraction (p=0.001). There was no significant relation between U. urealyticum colonization and IgM levels nor C-reactive protein (CRP) level nor U. urealyticum colonization of the mother. Twenty-three(7%) of 340 infants more than 32 weeks of gestational age had 1 or more positive specimens by culture or PCR. BPD didn't occur and RDS didn't decrease significantly in the colonized group (p=0.605). Total IgM levels didn't increase significantly in the colonized group (p < 0.006) but total WBC counts and CRP levels didn't increase significantly in the colonized group. There was no significant relation between U. urealyticum colonization and U. urealyticum colonization of the mother (p=0.21). CONCLUSION: U. urealyticum colonization in infants less than 32 weeks of gestational age was associated with an increased prevalence of BPD and a decreased prevalence of RDS.

Clinical Usefulness of the New Definition of Bronchopulmonary Dysplasia

PURPOSE: The aim of our study was to determine the incidence of bronchopulmonary dysplasia (BPD) using the newly proposed definition for BPD and investigate the relationship between the severity of BPD by new definition and clinical severity of illness and radiographic change. METHODS: We selected very low birth weight infants <1,500 g with gestational age <32 weeks born in the Bun-Dang Seoul National University Hospital from June 2003 to May 2005. The datas were categorized in four weight groups, <751, 751 to 1,000, 1,001 to 1,250 and 1,251 to 1,500 g and the incidence of BPD was computed in survivors based on the oxygen need at postnatal 28 days and 36 weeks postmenstrual age (PMA). Further, BPD-associated comorbidities and radiographic changes at 36 weeks PMA were compared among the groups defined by the new severity of BPD criteria. RESULTS: Among VLBW <1,500 g, the incidence of BPD at 28 days and 36 weeks PMA were 67 and 39%, respectively. Using the newly defined criteria, the incidence of mild, moderate and severe BPD were 29, 33 and 6%, respectively. Associated comorbidities correlated significantly with severity of BPD. Significantly longer hospital stay, longer duration of mechanical ventilation, higher score of radiography at 36 weeks PMA was observed with increasing severity of BPD. CONCLUSIONS: The severity of BPD by new definition significantly related to clinical severity and radiographic changes. Whether it will have a role in predicting long-term pulmonologic and neurologic outcome remains to be determined.

Frequency and Etiologies of Hypotension in Extremely Low Birth Weight Infants

PURPOSE:Hypotension is common in extremely low birth weight infants (ELBWI) and the treatment becomes important as the survival rate of ELBWI is increasing. This study is to investigate frequency and etiologies of hypotension in ELBWI. METHODS:40 patients admitted to the NICU of Seoul National University Children's Hospital from September 2004 to June 2006 were included retrospectively. Definition of hypotension was 1) mean arterial blood pressure below 30 mmHg or below the gestational age, 2) decreased urine output or metabolic acidosis, and 3) use of inotropics. Hydrocortisone was used for inotrope-resistant hypotension. Clinical characteristics of patients with hypotension were compared with those of others without hypotension. RESULTS:Mean gestational age and mean birth weight of 40 patients was 26(+6)+/-2(+1) weeks and 787+/-149 g. 20 patients had hypotension. 17 events were within 1 week of postnatal age, 5 from 1 to 2 weeks, and 9 after 2 weeks. The etiologies of hypotension within 1 week were PDA in 12 cases, and bleeding in 4 cases. PDA, post-operative condition, adrenocortical insufficiency, and sepsis were the probable etiologies of hypotension after 2 weeks. Among 12 patients recieved hydrocortisone, 9 responded to hydrocortisone. Patients with hypotension were significantly low in gestational age and birth weight. Incidence of IVH and ROP were significantly high in patients with hypotension. CONCLUSIONS:Hypotension was frequent in ELBWI and the etiologies of hypotension were various according to postnatal ages. Significant proportion of hypotension was inotrope-resistant in ELBWI. Further studies about etiologies are in need with reference of this study.

A Comparison of Clinical Manifestations in Neonates and Infants Infected by Respiratory Syncytial Virus / 소아과

PURPOSE: This study was performed to evaluate of clinical manifestations of neonatal respiratory syncytial virus(RSV) infection, and to evaluate of differences of clinical manifestations between the neonates and infants who were infected by RSV. METHODS: We reviewed the medical record of 75 children who were younger than 12 months of age and infected by RSV. We classified then into a neonatal group(n=30) and an infantile group(n=45) and compared the neonatal group with the infantile group by clinical manifestation and chest X-ray. RESULTS: Fever was more significant in the infantile group than the neonatal group(P=0.0256). The chest wall retraction was seen significantly in the neonatal group(P=0.0034). The study didn't show a significant difference in wheezing or rale between the two groups. There was not any significant difference in cyanosis and apnea between the two groups but the symptoms appeared more frequently in the neonatal group. With regard to chest X-rays, pneumonia is seen more frequently in the neonatal group(23/30, 76.67%) and bronchiolitis is seen more frequently in the infantile group (25/45, 55.55%). CONCLUSION: Neonatal RSV infections appear with fever less than infantile RSV infection and may appear with mild clinical manifestations, but chest retraction and pneumonia are more frequently present than in the infantile group. Therefores, neonate needs careful observation and treatment.

Intestinal Neuronal Dysplasia in Twins / 대한소아소화기영양학회지

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

Clinical Reviews of Landau-Kleffner Syndrome / 대한소아신경학회지

PURPOSE: We intended to describe the clinical features including characteristic etiologies, therapeutic approaches and outcomes for Landau-Kleffner syndrome(LKS). METHODS: A retrospective chart reviews were done to reveal the clinical and electrophysiological features in 5 patients who were diagnosed as LKS and undergone extensive diagnostic work-up and various therapeutic interventions. RESULTS: Among five LKS patients, 2 patients were males and 3 were females. All patients showed well controlled seizure outcomes but cognitive function including auditory and/or expressive aphasia were not improved in spite of using various anticonvulsants. Only two patients responded to steroid therapy but one patient showed repeated deterioration after discontinuation of the durg. Among 3 patients who were on ketogenic diet(KD), one patient showed a dramatic improvement in liguistic and cognitive functions in spite of underlying mitochondrial complex I deficiency. Multiple subpial transections (MST) were done in the other two patients because of incomplete recovery from ketogenic diet, and only one patient showed fairly successful improvement. CONCLUSION: Cognitive regression induced by LKS could be successfully improved by various therapeutic modalities including steroid, KD and palliative MST in most patients.

A Case of 4q Deletion with Partial Agenesis of Corpus Callosum

Syndrome of 4q deletion is characterized by an abnormal shape of the skull, craniofacial dysmorphism, cardiovascular malformations, genitourinary defects, limb and digital anomalies, and developmental delay. We experienced a case of 4q interstitial deletion in a 2 day-old female neonate who showed short extremities, partial agenesis of corpus callosum and congenital heart defects. We report the case with a brief review of the literature.

MELAS Syndrome Confirmed by Mitochondrial DNA Analysis in Siblings

MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome is a major subgroup of mitochondrial myopathy. Recent advances in molecular genetics revealed specific mutations in mitochondrial DNA which cause MELAS. We described here clinical and molecular genetic findings of sister and brother with MELAS syndrome. For molecular genetic studies, DNAs from peripheral blood nucleated cells were used. And the substitution of adenine to guanine at the nucleated position 3243 in the mitochondrial tRNALeu(UUR) gene was confirmed in the patients. Their mother was a heteroplasmic pattern which supports maternal transmission.

A Study of Vascular Reactivity Change in Nephrotic Syndrome Children Using Plethysmography

PURPOSE: The nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema and hyperlipidemia. These can change body homeostasis and cause hypertension. This study was designed to determine the relationship between the forearm vasodilating capacity and serum cholesterol level of nephrotic syndrome patients. METHODS: 15 Nephrotic syndrome patients and 17 normal control children who visited Chung-ang University Youngsan Hospital from Sep. 1995 to Sep 1996, were investigated. Each subject underwent forearm plethysmography for mearsurement of blood flow and vascular resistance. RESULTS: 1) Resting blood pressure, heart rate, forearm blood flow, vascular resistance was not significantly different in nephrotic syndrome group and control group. 2) After peak hyperemic periods, blood pressure, heart rate was not significantly different in nephrotic syndrome group and control group. 3) After hyperemic periods, peak forearm vascular blood flow was lower in nephrotic syndrom group (52.0+/-10.6mL/min/100ml) than control group (59.5+/-4.5mL/min/100mL), and minimal forearm vascular resistance was significantly higher in nephrotic syndrome group (1.8+/-0.5mmHg/mL/min/100mL) than control group (1.5+/-0.4mmHg/mL/min/100mL) (p<0.05). 4) vascular dilatation capacity (resting-hyperemic forearm vascular resistance difference) was also significantly lower in nephrotic syndrome group (6.3+/-1.6mmHg/mL/min/100) than control group. 5) Serum cholesterol level is significantly higher in nephrotic syndrome group (253.1+/-133.4mg/dL) than control group (183.5+/-41.0mg/dL). High cholesterol level related with nephrotic duration. 6) resting-hyperemic forearm vascular resistance difference is associated with relapsing frequence, but not associated with cholesterol level and nephrotic syndrome duration. CONCLUSIONS: These data suggest that reactive vascular changes in the forearm of nephrotic syndrome demonstrate early abnormalities of subclinical vascular changes, and these vascular change may contribute to cardiovacular disease and artherosclerosis.

A case of adult Wilms' tumor / 대한비뇨기과학회지

Wilms' tumor is malignant renal tumor, originated from undifferentiated mesonephric blastema. Adult Wilms' tumor, unlike that of childhood, is a rare disease and a total of l67 cases have been reported in the world medical literature until 1980. Diagnosis of adut Wilms` tumor is very difficult preoperatively and the pathologic hallmark is the presence of abortive or embryonal glomerulotubular structure with an immature spindle cell stroma. The therapeutic guidelines and surgical principles that govern childhood Wilms' tumor should be applied to adult Wilms' tumor. We reported a case of Wilms' tumor developed in 34 year-old male patient complaining of right flank pain with gross hematuria for 2 months.

A Case of Malignant Fibrous Histiocytoma of the Left Atrium

Primary malignant neoplasms of the heart are very rare disorders, which are found at less 1/1000 necropsies. These malignancies are almost exclusively sarcomas and histologically assume a wide veriety of morphological subtypes, angiosarcomas, rhabdomysarcomas, and fibrosarcomas(or MFH) being the most frequent. We experienced a case in which left atrial myxoma was suspected by 2-D echocardigraphy and the histologic diagnosis of promary MFH was confirmed by operation. A 45-year old woman with primary MFH arising from posterior wall of left atrium, interatrial septum and mitral annulus is presented with a brief view of the literatures. The patient was admitted to our hospital on March 1988, because of exertional dyspnea and intermittent palpitation resction of the masses. To date, more than thirteen months after surgery, the patient is alive and well in state of NYHA class II.