Clinical factors causing hyperbilirubinemia in patients with Kawasaki disease / 소아과

PURPOSE: Hyperbilirubinemia, jaundice and gallbladder hydrops are unusual manifestations of Kawasaki disease (KD). In this case, abdominal pain, anorexia and abdominal distension may follow eventfully. We reviewed the clinical and laboratory data to investigate the causative factors of hyperbilirubinemia in patients with KD. METHODS: Two-hundred eighty two children diagnosed and hospitalized with KD were identified by searching patients' charts. Cases were included in the study if diagnosed between January 1997 and December 2004. We reviewed clinical data, age, sex, duration from the onset of fever to admission and duration from start of treatment to defervescence. Clinical and laboratory data were compared between normal (A) and hyperbilirubinemia (B) groups. RESULTS: Thirteen patients (4.6 percent) showed hyperbilirubinemia. In the hyperbilirubinemia group, age and initial ALT value were higher than group A (P=0.003, 0.018 respectively). Duration from the onset of fever to admission and age were relative risk factors in hyperbilirubinemia (P=0.007, 0.003 respectively) in patients with KD. CONCLUSION: Shorter duration from the onset of fever to admission and older age group were relative risk factors of hyperbilirubinemia in patients with KD.

Lower respiratory tract infection of positive antigen test for respiratory syncytial virus on children under 2 years of age / 소아과

PURPOSE: This study was design and performed for evaluations of resent clinical pattern of bronchiolitis caused by RSV infection with children under 2 year of age for 5 years, who were admitted to pediatric ward. METHODS: The inclusion criteria of the patients were children under 24 month-of-age, clinical manifestations of lower respiratory tract infection, and RSV antigen that was detected by a direct immunofluorescence test from the nasal secretions. The additional laboratory and simple chest X-ray findings were reviewed from the medical records of children who were admitted Wonkwang university hospital from Jan. 1999 to Dec. 2003. RESULTS: In the 5 year study duration, 127 patients were enrolled and outbreak of RSV bronchiolitis took place in 2001. The 80 cases(63 percent) of RSV infection were concentrated in later autumn and winter. Number of the cases show coughing were 120(94.5 percent), but rale was audible in 78 cases(61.4 percent). Dyspnea, wheezing, and intercostal retraction were noticed in 27(21.3 percent), 21(16.5 percent), and 4(3 percent) cases respectively. The most common chest X-ray finding was hyperinflation of the lung that was noticed in 110 cases(86.6 percent). Care with mechanical ventilator for more than 2 days required in 5 cases. CONCLUSION: Lower respiratory tract infection by RSV was common in late autumn and winter season but year-round infection was noticed. The severity of RSV respiratory tract infectiontakes in some degree a grave course. So we suggest that population-based surveillance of acute respiratory infection due to RSV is necessary for assessment of prevalence and epidemiology of this disease.

The study on cytotoxicity of cytokines produced by the activated human NKT cells on neuroblastoma / 소아과

PURPOSE: alpha-Galactosylceramide (alpha-GalCer)-stimulated human Valpha24 natural killer T (NKT) cells exert antitumor activity against some leukemia in a CD1d dependent and TCR-mediated manner, but could not kill CD1d-negative neuroblastoma (NB) cells. There are few reports about the direct antitumor effect of highly secreted cytokines by these cells on activation. In this study, using a cell-free supernatant (SPN) collected from plate bound hCD1d/alphaGalCer tetramers-stimulated NKT cells, we examined whether they could be helpful in the immunotherapeutic treatment of NB. METHODS: Cells were cultured in IMDM. The cytokines produced by NKT cells were measured with Cytometric Bead Array (CBA) analysis. Cell viability was evaluated by calcein-AM fluorescence with digital image microscopy scanning (DIMSCAN). The percentage of specific apoptosis was calculated by flow cytometric detection of apoptosis using annexin V and 7-AAD. RESULTS: The activated NKT cells secreted high levels of IL-2, INF-gamma, TNF-alpha. The SPN was significantly cytotoxic against four out of eight tested NB cell lines, through mainly apoptosis as evidenced by annexin-V staining and inhibition with the pretreatment of pancaspase blocker. This apoptosis was significantly inhibited when anti-TNF-alpha and anti-IFN-gamma neutralizing mAbs were used separately and it was completely abolished when the two mAbs were combined. CONCLUSION: IFN-gamma and TNF-alpha produced by NKT cells could exert synergistically direct anti-tumor activity through apoptosis on some NB cell lines.

Clinical significance of follow-up laboratory tests, performed at 6 weeks after the onset of Kawasaki disease / 소아과

PURPOSE: Low dose aspirin is used due to its antiplatelet effect for the subacute phase of Kawasaki disease(KD). It is usually used for 6-8 weeks, then various hematologic laboratory tests and follow up echocardiography for evaluating coronary abnormalities are performed. Our review investigated the usefulness of various follow up laboratory tests performed at 6 weeks after the onset of KD. METHODS: Two hundred eighty-two children diagnosed and hospitalized with KD were identified by reviewing patient's charts. Cases which were diagnosed between January 1997 and December 2004 were included in this study. We reviewed laboratory data including leukocytes, platelet counts, antistreptolysin O(ASO), erythrocyte sedimentation rate(ESR), C-reactive protein(CRP), aspartate aminotransferase(AST), alanine aminotransferase(ALT), urinalysis, and echocardiograms performed at admission and 6 weeks after the onset of KD. Paired t-test and Fisher's exact test, as well as logistic regression tests, were used for the statistical analysis. RESULTS: At 6th week data, ESR and CRP were still elevated in 35(12.4 percent) and 12(4.3 percent) patients, respectively. Sterile pyuria were all normalized. But, 36 patients(12.8 percent) showed thrombocytosis, 22(7.8 percent) elevated AST, 15(5.3 percent) leukocytosis, and 6(2.1 percent) coronary abnormalities. Coronary abnormalities at the 6th week were only shown in patients with initial abnormalites. Younger age and initial thrombocytosis were risk factors for thrombocytosis at the 6th week. CONCLUSION: All children with initial coronary abonormalites should have an echocardiogram at 6 weeks after the onset of fever. In view of case-effectiveness, additional echocardiographic studies are justified only if abnormalities are present at admission. ESR, CRP, and urinalysis performed at the 6 weeks after onset of KD is not significant for clinical information of progression. Platelet count should be estimated at 6th week for a judgement of continuous antiplatelet therapy.

Congenital Anomalies of the Left Heart Valves: Morphology, Pathophysiology and Diagnosis

No abstract available.

An Infantile Case of Sturge-Weber Syndrome in Association with Klippel-Trenaunay-Weber Syndrome and Phakomatosis Pigmentovascularis

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case of the Sturge-Weber syndrome in combination with the Klippel-Trennaunay syndrome and phakomatosis pigmentovascularis in a 4-month-old infant. He showed nevus flameus on the right leg and both part of the face and back, leptomeningeal angiomatosis on right hemisphere, hypertrophy of the right leg, hemiconvulsion on the left and also evidences of congenital glaucoma and nevus of Ota. Very rare case combined with these three kinds of phakomatosis has been reported.

Predisposing Factors of Postoperative Pulmonary Complication in Children with Congenital Heart Disease

PURPOSE: Postoperative care usually required 24-48 hours at intensive care unit (ICU) in children with congenital heart disease. More longer ICU stay may give more chances to produce the postoperative complications. Postoperative pulmonary complication is produced to a much higher incidence after longer immobilized state with keeping catheters and arterial and venous lines. So, we evaluated the predisposing factors those are oriented to ICU stay factors and age, hematologic abnormalities, hepatic dysfunction, infections during ICU care in children with congenital heart disease. METHODS: A retrospective review was performed of postoperative factors for children undergoing open heart surgery in intensive cardiac unit, Wonkwang medical cardiac center. A total of 193 pediatric patients who had cardiac surgery with cardiopulmonary bypass in a 10 year period from Jan. 1995 until Dec. 2004 were reviewed. After logistic regression test, predisposing factors were deemed significant if associated with a pulmonary complication with P<0.05. RESULTS: Children who fell postoperative pulmonary complication in our institution occupied 15% of 193 patients with congenital heart disease. Of all clinical factors considered, those significantly associated with postoperative pulmonary complication were as follows: high ALT level, longer duration of mechanical ventilation and arterial line maintenance. CONCLUSION: Prompt weaning of mechanical ventilation and removal of arterial line during ICU stay in children underwent open heart surgery may be necessary to decrease the risks of postoperative pulmonary complication.

A Case of Intussusception Caused by Meckel's Diverticulum in a Newborn / 소아과

Intussusception and Meckel's diverticulum are very rare disorders in intrauterine or neonatal periods, which are causes of intestinal obstruction. We experienced a case of intussusception due to Meckel's diverticulum which caused intestinal obstruction in the neonate who had bilious vomiting a few hours after birth. We report this case with a brief review of the literature.

Etiologic Diagnosis and Clinical Characteristics of Chronic Recurrent Chest Pain in Children / 소아과

PURPOSE: To identify the causes and clinical characteristics, and the efficacy of various diagnostic approaches, we studied, prospectively, pediatric patients with chronic recurrent chest pain. METHODS: A prospective study of 122 patients with chronic recurrent chest pain from June 1998 to June 2003 was performed. The male and female ratio was 81:41, age 9.3+/-3.1 year. A single chart including pain description(histoy) and pain questionaire, with associated symptoms, was used for systematic history taking. The patients were devided in two groups, Group A, before June 2001(n=70), Group B, after June 2001(n=52). Chest X-ray and ECG were checked in all patients. Allergy tests and echocardiography, 24 hour ECG monitoring, exercise tests, pulmonary function tests, and gastrointestinal fiberendoscopy were performed selectively. RESULTS: Idiopathic origins were most common(32%). The remaining causes were psychogenic(23%) and exercise-induced asthma(20.5%), hyperventilation syndrome(9.1%), tachyarrhythmia(4.9%), cardiac (4.1%), pulmonary(3.3%), reflux esophagitis(2.5%), in order. The positive rate of allergy tests(24%) was higher than that of cardiac examinations(11-20%). Exercise-induced asthma was more common than psychogenic causes after June, 2001. Various abdominal symptoms accompanied idiopathic and psychogenic chest pains, and hyperventilation syndrome. Headaches were more commonly found in psychogenic pain, but chest tightness and dyspnea were common in hyperventilation syndrome. About half of exercise-induced asthma patients had symptoms of allergic rhinitis. CONCLUSION: In the majority(72%) of exercise-induced asthma, the chest pain was induced or aggravated by exercise, and relieved by rest. Causative antigens were detected in 69.2% of group B patients with exercise-induced asthma. Exercise-induced chest pain might be a first manifestation in allergy patients. So, we recommand allergy tests for patients with recurrent chest pains induced or aggravated by exercise. Hence, cardiac examinations such as echocardiography or 24 hour ECG monitoring could be performed selectively, case by case.

Incidence and Risk Factors of Rehospitalization with Respiratory Syncytial Virus Infection in Premature Infants / 소아과

PURPOSE: We performed this study to evaluate the risk of rehospitalization for respiratory syncytial virus(RSV) infection among premature infants discharged from a neonatal intensive care unit(NICU). METHODS: We performed a retrospective study for rehospitalization for RSV infection and risk factors among premature infants who were admitted to NICU and discharged between May 2001 and April 2003 in Wonkwang University Hospital. RSV detection was utilized by direct fluorescent antibody tests in nasopharyngeal aspirates. We also reviewed various risk factors including gestational age, birth weight, sex, ventilatory care, surfactant administration, chronic lung disease(CLD), siblings in school or kindergarten age, and month of discharge. RESULTS: The rehospitalization rate for RSV infection was 6.6%(26/381) in premature infants and 22.2%(4/18) in premature infants with CLD. The most common season of rehospitalization for RSV infection was between November to January, this was 69.2%(18/26) in premature infants, the same as children: 61.2%(93/152). The risk factors for RSV rehospitalization among premature infants were CLD, siblings in school or kindergarten age and discharge between October to December from NICU. CONCLUSION: The risk for RSV rehospitalization among premature infants from NICU was low. Preterm infants subject to risk factors of CLD, siblings in school or kindergarten age, and discharge between October to December from NICU, were most likely to require hospitalization for RSV disease. In CONCLUSION: Prophylaxis for RSV infection should be considered one month before discharge from NICU in the RSV season between October and December.

Physical Examination in Pediatric Patients with Heart Diseases / 소아과

No abstract available.

A Case of Thyroid Papillary Carcinoma Presenting as a Miliary Pulmonary Infiltration in 8 Year Old Girl / 소아알레르기및호흡기학회지

Papillary carcinoma of the thyroid is the most common thyroid cancer in children. It usually occurs in the teenage female and presents as a mass within the thyroid itself. In the great majority of cases the only presenting sign is a neck mass. In a high percentage (60-80%) there are also palpable lymph nodes. A 8 years old female patient was admitted because of palpable neck mass and miliary shadow in chest X-ray. On examination, a soft nontenter nodular mass 1x1 cm in size was palpated at supraclavicular area of left mid-neck. In chest X-ray and CT, well demarcated nodular milialy infiltration was fully distributed in both lung. After the initial treatment failure for the miliary tuberculosis, the fine needle aspiration biopsy was performed. The Papillary carcinoma of the thyroid was diagnosed. Total thyroidectomy and entire lymph node excision were performed, followed by radioiodine ablation and hormonal therapy. The patient has been improved clinically. We are reporting a case of thyroid papillary carcinoma presenting as a pulmonary miliary infiltration with brief review of the literature.

Comparative Prospective Study of Intravenously and Subcutaneously Administered Recombinant Human Erythropoietin(Epokine(R)) in End-Stage Renal Disease Patients : A Phase IV Single Center Study / 대한신장학회잡지

BACKGROUND: We evaluated the clinical efficacy and safety of recombinant human erythropoietin(Epokine(R)). METHODS: A comparative prospective study of intravenously and subcutaneously administrated Epokine(R) conducted 13 patients performing hemodialysis and 28 patients performing continuous ambulatory peritoneal dialysis with end-stage renal disease. Epokine(R) was given initially at a dosage of 100 unit/kg, subcutaneously, two times a week. The patients had achieved stable or more than 10 g/dL of hemoglobin level for 12 weeks and then we randomized switching intravenously or subcutaneously administrated Epokine(R) for another 12 weeks. RESULTS: Hemoglobin(g/dL) and hematocrit(%) increased significantly from baseline levels beginning from 2 weeks after Epokine(R) administration. In HD patients, hemoglobin increased significantly from 7.3 to 9.5 after 12 weeks and to 10.6 after 24 weeks. In CAPD patients, hemoglobin increased significantly from 6.8 to 10.2 after 12 weeks and then 10.8 after 24 weeks(p 0.05). In HD patients, intravenously administrated Epokine(R) group was more dosage than subcutaneously group(97.4+/-15.4 vs 145.4+/-2.9 U/kg/wk, p 0.05). The 9 cases(18.8%) were suffered from headache and flu-like syndrome, but these side effects were not severe and disappeared from conventional therapy. CONCLUSION: Epokine(R) administration is safe and effective in treating anemia of ESRD patients and subcutaneously administration is significantly more effective than intravenously.

Cytotoxicity of Diamide and the Protective Effect of Thioredoxin on Diamide-Induced Vasculotoxicity in Vascular Endothelial Cells

BACKGROUND AND OBJECTIVES: This study was designed to examine the effects of diamide and thioredoxin (TRX) on vascular endothelial cells in order to clarify the mechanism by which vascular damage is mediated by oxygen free radicals. MATERIALS AND METHODS: The pulmonary artery endothelial cell (PAEC) line derived from bovine serum was cultured for 8 hours in media supplemented with various concentrations of diamide and TRX. The XTT assay, MTS assay, SRB assay, LDH activity and lipid peroxidation tests were perfomed. RESULTS: In XTT and MTS assays, diamide significantly decreased the cell viability of cultured PAEC in a dose- and time-dependent manner. Diamide showed a decrease in the amount of total protein, although it showed an increase of lipid peroxidation and LDH activity in cultured PAEC. In regards to the protective effect of TRX on diamide-induced cytotoxicity, this showed an increase of total protein, however it showed a decrease of lipid peroxidation and LDH activity. CONCLUSION: Our results suggest that diamide has a vasculotoxic effect on cultured bovine PAEC and that TRX is very effective in the protection of diamide-induced cytotoxicity by duye to the increase of total protein and the decrease of lipid peroxidation and LDH activity in these cultures.

Significance of Postoperative Peritoneal Drainage and Peritoneal Dialys is in Infants with congenital Heart Disease Who Underwent Open Heart Srugery

PURPOSE: We reviewed 5 years worth of experience with peritoneal drainage and dialysis in infants who underwent open heart surgery. The aim of this study was to investigate the effect of peritoneal drainage and peritoneal dialysis on fluid balance and several parameters of intensive care. METHODS: Six(10%) of 60 consecutive infants who underwent open heart surgery required peritoneal dialysis during peritoneal drainage. Simple peritoneal drainage was performed in the remaining 54 infants. The silicone rubber peritoneal dialysis catheter was inserted into the center of abdominal cavity just after operation, and subsequent peritoneal drainage was maintained during intensive care. RESULTS: Mean age of the study group was 0.48+/-0.21 years(M: F=32: 28). Early postoperative mortality amang the infants with congenital heart disease was 1.6%. Total amount of output was 7.0+/-2.28mL/kg/hr, urine output 5.14+/-2.9lmL/kg/hr, pleural fluid 0.80+/-0.5lmL/kg/hr, and peritoneal fluid 1.20+/-0.90mL/kg/hr. The ratio of output to intake(O/I) was 1.06. None of the complications required early termination of peritoneal drainage or peritoneal dialysis. Hemodynamics and pulmonary function were maintained steadily during postoperative intensive care. CONCLUSION: The early institution of peritoneal drainage and peritoneal dialysis in infants with congenital heart disease after cardiac operations not only removes fluid, thus easing fluid restriction, but may also improve cardiopulmonary function.

Clinical Implication of Atrial Isomerism

No Abstract available.

Relation of Postoperative Complication and Duration of Artificial Ventilation in Infants with Large Ventricular Septal Defect

PURPOSE: The importance of postoperative management of those who have undergone intracardiac repair for congenital heart diseases has increased in recent years. In this study, we investigated postoperative complications and their relations to preoperative age or duration of the intubation and artificial ventilation in infants with large symptomatic ventricular septal defect. METHODS: Between January, 1993 and December, 1996, sixty infants underwent primary closure of a ventricular septal defect (VSD). The patients were divided into two groups based on preoperative age : group 1, infants aged 6 month or less (n=40), and group 2, infants aged more than 6 month (n=20). And, another three groups were divided into 3 groups due to the duration of the intubation and artificial ventilation including 48 hour, respectively. We compared the incidence of complications such as infection, respiratory or cardiovascular complications among each group. RESULTS: No specific differences between two age groups were found, but the incidence of right bundle branch block was high in age group of 6 month or less (P48 hour (P<0.05). And also, arrhythmias, atelectasis, toxic hepatitis were more frequently observed in the former group. CONCLUSION: These results indicate that early weaning of the artificial ventilation in infants with large symptomatic ventricular septal defect could protect them from development of severe life- threatening infection such as sepsis. We suggest 48 hours or less as the optimal duration of artificial ventilation. Additionally, earlier weaning could provide earlier oral feeding or earlier ambulation, which is improve respiratory homeostasis.

Surgical Treatment of 3 cases of Takayasu Arteritis with Renovascular Hypertension

Renovascular hypertension secondary to renal artery vascular disease is the most common form of surgically correctable hypertension. The common causes of renovascular hypertension are atherosclerosis, fibromuscular dysplasia, and Takayasu arteritis. Takayasu arteritis is a chronic nonspecific arteritis of unkown cause that is relatively prevalant in young female subjects. It has been well known that the pathologic feature of the disease consist predominantly of occulsive changes in the aorta and the origin of its major branches. The most important pathogenetic mechanism of hypertension seems to be through renal artery stenosis. We have experimented three hypertensive patients with Takayasu arteritis experienced. Basic diagnosis was established by angiographic study. This article presents surgical treatment methods of Takayasu arteritis with renovascular hypertension and brief review of literatures.

Postoperative Weight-Gain in Early Infants with Symptomatic Large Ventricular Septal Defect

PURPOSE: To investigate the postoperative patterns of weight-gain in early infants with ventricular septal defects (VSDs), we analyze 48 patients who received primary surgical closure of the symptomatic VSDs during 6 months of age in the period of 1993 through 1995. METHODS: Forty-sixt infants met criteria for inclusion in the study. The mean follow up period was 14 months (3 to 36 months). Weight percentile was extrapolarated from data of the Korean boys and girls measured in 1985. The data was compared by one-group paired student t-test. RESULTS: The overall mortality rate of this study was 4% (2/48). No surviving patients required a second ventricular septal defect operation, and the majority no longer received anticongestive therapies. The mean percentile of the preoperative weight was 3.53. In postoperative follow up period, they grew steadily. But, the mean weight percentile was only 5.46 in age under 6 months. Mean 12 percentile was achieved in age of 6 to 8 months, hence 25.4 percentile in 9 to 12 months, 33.5 percentile over 12 months, 64.8 percentile over 24 months of age. The weighing pattern of the patients with smaller defect was similar in 24 patients (52%) with large VSD, which size were same or larger than diameter of the aortic root. CONCLUSIONS: This study indicated that the patients under 6 months who have undergone primary surgical closure of the symptomatic large ventricular septal defects could gain the weight normally in postoperative period. Although the weight-gain was not sufficient when the age was under 12 months, It became normal pattern of weighing in age over 12 months.

Postoperative Weight-Gain in Early Infants with Symptomatic Large Ventricular Septal Defect

PURPOSE: To investigate the postoperative patterns of weight-gain in early infants with ventricular septal defects (VSDs), we analyze 48 patients who received primary surgical closure of the symptomatic VSDs during 6 months of age in the period of 1993 through 1995. METHODS: Forty-sixt infants met criteria for inclusion in the study. The mean follow up period was 14 months (3 to 36 months). Weight percentile was extrapolarated from data of the Korean boys and girls measured in 1985. The data was compared by one-group paired student t-test. RESULTS: The overall mortality rate of this study was 4% (2/48). No surviving patients required a second ventricular septal defect operation, and the majority no longer received anticongestive therapies. The mean percentile of the preoperative weight was 3.53. In postoperative follow up period, they grew steadily. But, the mean weight percentile was only 5.46 in age under 6 months. Mean 12 percentile was achieved in age of 6 to 8 months, hence 25.4 percentile in 9 to 12 months, 33.5 percentile over 12 months, 64.8 percentile over 24 months of age. The weighing pattern of the patients with smaller defect was similar in 24 patients (52%) with large VSD, which size were same or larger than diameter of the aortic root. CONCLUSIONS: This study indicated that the patients under 6 months who have undergone primary surgical closure of the symptomatic large ventricular septal defects could gain the weight normally in postoperative period. Although the weight-gain was not sufficient when the age was under 12 months, It became normal pattern of weighing in age over 12 months.