Paradoxical Response to Chemotherapy in Tuberculous Pleural Effusion / 소아알레르기및호흡기학회지

It is defined as the paradoxical response when the clinical or radiologic worsening of old lesions or the development of new lesion occur in spite of appropriate antituberculous therapy. The paradoxical response can occur as an intracranial tuberculoma, pleurisy, pericarditis and contralateral new parenchymal lesions. However, poor compliance with therapy, drug resistance, non-tuberculous mycobacterium, or another underlying condition as lung cancer should be ruled out before concluding that the treatment is the cause of the exacerbation. The case reports of paradoxical response have been mainly reported in adults, but extremely rare in children. We report a case of paradoxical response in which a new parenchymal lung lesion developed during antituberculous therapy in a 14-year-old female patient with tuberculous pleurisy. She experienced clinical improvement with steroid therapy in addition to antituberculous therapy.

The relationship between adiponectin, leptin, insulin, insulin-like growth factor and IGF binding protein-3 in cord blood and neonatal anthropometric parameters / 소아과

PURPOSE: This study was designed to examine the effects of adiponectin, leptin, insulin, insulin-like growth factor (IGF)-I and IGF binding protein (BP)-3 levels in cord blood on weight, length, and adiposity at birth in healthy term infants. In addition, we evaluated the mechanism to change the hormone levels in appropriate for gestational age (AGA) during the first month. METHODS: We collected cord blood from 200 term neonates (109 males, 91 females) with no perinatal problems, and measured the hormone levels and anthropometric parameters including weight, length, and skin-fold thickness. Term neonates were divided into 3 groups as follows: birth weight appropriate for gestational age (AGA) (n=132), birth weight less for gestational age (SGA) (n=29), and birth weight more for gestational age (LGA) (n=39). Venous blood samples of 15 fullterm healthy neonates were obtained at 3, 7, and 30 d after birth. RESULTS: The adiponectin, insulin, and IGF-I levels were significantly lower in the SGA group than in the AGA and LGA groups. The leptin levels were significantly higher in the LGA group than in the AGA and SGA groups. Cord blood adiponectin, leptin, insulin, IGF-I, and IGFBP-3 levels correlated significantly and positively with birth weight and the sum of the skin-fold thickness. A significant positive correlation was observed between adiponectin, leptin, and IGF-I levels and birth weight. Adiponectin level correlated significantly with that leptin level (r=0.191, P=0.038), but not with insulin, IGF-I and IGFBP-3 levels. IGF-I levels were higher in females than in males. At 7 d after birth, the leptin level decreased along with physiologic weight loss, and then increased. IGF-I, also decreased at 3 d, significantly increased 1 month later. CONCLUSION: We suggest that adiponectin, leptin, insulin, IGF-I, and IGFBP-3 play an important role in regulating fetal growth. Adiponectin may be involved in regulating fetal growth through mechanisms different from those mediated by insulin or IGF-I. High levels of IGF-I in female neonates indicates a gender difference which serves as evidence for in utero sexual dimorphism. It is likely that IGF-I has a more important role than that of hormones in postnatal growth.

The usefulness of skin test in evaluation of immunity to varicella / 소아과

PURPOSE: The aim of this study is to assess the usefulness of skin test by an inactivated, 1/50 diluted solution of attenuated varicella vaccine in evaluating the immune status to varicella. METHODS: Total 41 subjects (22 males, 19 females, aged 1-32 years) were enrolled from July to August, 2005. Past medical history including varicella infection, varicella vaccination were investigated through questionnaires. The skin test solution was prepared from solution of attenuated varicella vaccine(Oka strain) which was inactivated by exposure to room temperature for 10 days and diluted at 1/50 with normal saline. Skin test was done by injecting 0.1 mL of the solution intradermally into the volar surface of the right forearm and sterile normal saline was used as a control on the left forearm. Positive reaction was defined when the transverse diameter of the induration was 5 mm or more. Serum varicella zoster virus specific IgG antibody test by ELISA (enzyme-linked immunosorbent assay) was done. RESULTS: In adults, the sensitivity of the varicella zoster virus skin test compared to ELISA was 94.7% and the positive predictive value was 100%. In children, both the positive predictive value and specificity were 100% but the sensitivity and the negative predictive value were 50% and 30.7% respectively. Children showed smaller skin test reactivity compared to adults. CONCLUSION: The varicella zoster virus skin test using inactivated, 1/50 diluted solution of attenuated varicella vaccine was proved as one of the useful tools for evaluating the immunity and susceptibility of the varicella zoster virus.

A Case of Intussusception Diagnosed by Exploratory Laparotomy in a Very Low Birth Weight Infant

Intussusception is the most common cause of intestinal obstruction between 5 month and 11 month of age. However, it is very rare in neonatal period and only 1% incidence was reported. It is exceedingly rare among preterm infants and the signs are similar to those of necrotizing enterocolitis (NEC). So the time from onset of symptoms to diagnosis may be prolonged. The authors report a case of intussusception in very low birth weight infant born at 28 weeks of gestation diagnosed after exploratory laparotomy.

The Prevalence of Metabolic Syndrome in Children and Adolescents Born Small for Gestational Age / 대한소아내분비학회지

PURPOSE: Type 2 diabetes mellitus, dyslipidemia, hypertension, cardiovascular disease, called metabolic syndrome (MS), occur more frequently among individuals who were born small for gestational age (SGA). SGA children with catch-up growth in height and high BMI are the most insulin resistant. We investigated the prevalence of MS and evaluated the risk factors affecting the development of MS in children and adolescents born SGA. METHODS: The study population (n=65) were born less than 10th percentile in body weight for their gestational age and the body weights of the control group (n=34) were more than 10th percentile at birth. The SGA and control subjects divided into prepubertal and pubertal groups. We measured serum lipid levels (total cholesterol, triglyceride, HDL cholesterol, LDL cholesterol), fasting sugar levels and insulin levels. Insulin resistance was determined by homeostasis model assessment, fasting insulin glucose ratio and quantitative insulin sensitivity check index. RESULTS: Systolic blood pressure was significantly higher in the pubertal SGA group than in the control group (113.2+/-0.3 vs. 98.7+/-6.4, P=0.001). The prevalence of high triglyceride and high fasting glucose levels were significantly increased in pubertal SGA group than prepubertal SGA group (P<0.05). Insulin resistance was correlated to systolic blood pressure, triglyceride levels, HDL-cholesterol levels, fasting glucose, insulin levels at fasting and 2 hours after oral glucose tolerance test and body fat mass in SGA group (P<0.05). CONCLUSION: We suggest that the monitoring of these risk factors including systolic blood pressure, triglyceride, HDL-cholesterol levels and body fat mass is important for the prevention of MS in children and adolescents born SGA.

A Case of Right Atrium thrombosis Caused by Umbilical Venous Catheterization

Insertion of intravascular catheters may be the most common procedure in neonatal intensive care units. One of these kinds of catheters, umbilical venous catheter is generally used for the small and sick neonates. But the indwelling umbilical venous catheter can cause many complications. The most common complication is thrombus formation in the lumen of the catheter. Neonatal thrombosis has symptomatic or asymptomatic clinical manifestations, which may have serious complications in case of intracardiac thrombosis. In our experience, the infant who has no symptom of thrombosis was treated with low dose aspirin and warfarin for right atrium thrombosis caused by umbilical venous catheterization. So, we report a case of right atrium thrombosis following umbilical catheterization in neonatal intensive care units.

The Changes of Intestinal Normal Flora in Neonates for Seven Days Postnatally / 대한소아소화기영양학회지

PURPOSE: Microbial colonization of the intestine begins just after birth and development of the normal flora is a gradual process. The first bacteria colonizing the intestine in newborns are Staphylococcus, Enterobacteriaceae and Streptococcus. For several days after birth, the number of Bifidobacterium spp. increase. The aim of this study was to investigate the changes of microflora for seven days postnatally in neonatal stool. METHODS: Fifteen neonates (breast : formula : mixed feeding 1 : 8 : 6, vaginal delivery : cesarean section 3 : 12) who were born at the Kangdong Sacred Heart Hospital, Hallym University were enrolled. First meconium and stools of postnatal 1-, 3-, and 7-day were innoculated. Blood agar plates for total aerobes, trypton bile X-glucuronide agar for E. coli, phenylethyl alcohol agar for gram positive anaerobes, MRS agar for Lactobacillus spp., bifidobacterium selective agar for Bifidobacterium spp. and cefoxitin-cycloserine-fructose agar for Clostridium difficile were used in the general incubator (CO2 free incubator), CO2 incubator or the anaerobic chamber for 48 or 72 hours at 37oC and then colony forming units were counted. RESULTS: No microflora was identified in the first meconium. Total aerobes, E. coli, and gram positive anaerobes were significantly increased with advancing postnatal days. In only one baby, Lactobacillus acidophilus was detected 2x105 CFU/g in the seven-day stool. Bifidobacterium spp. was detected in two babies. Clostridium difficile was not detected during the seven days. There were no significant differences in the bowel flora depending on the delivery pattern and feeding method. CONCLUSION: This study shows many changes in the intestinal normal flora in neonatal stool during seven days postnatally. If these findings are confirmed with larger studies, the data may be preliminary findings to support use of probiotics in neonates.

The Effect of Skin-colonizing Staphylococcus aureus and Its Exotoxins on Childhood Atopic Dermatitis / 소아알레르기및호흡기학회지

PURPOSE: Staphylococcus aureus and its exotoxins have been regarded as having an influence on atopic dermatitis(AD). We aimed to examine the prevalence of S. aureus in the AD lesion, the types of the exotoxins, and the relationship between S. aureus and AD. METHODS: AD patients(n=32) and a normal control group(n=20) were enrolled. The severity of AD was measured by SCORAD index. Through skin culture and PCR, we tried to identify S. aureus and its exotoxins. RESULTS: S. aureus was isolated from 18(56 percent) out of 32 AD patients and its exotoxins were identified from 10(31 percent) out of them. The exotoxin types were as follows; sea in 4, eta in 3, sea+tst-1 in 1, sea+see in 2 patients. On the contrary, S. aureus was isolated from only 1(5 percent) out of 20 subjects of the normal control group, and its exotoxin type was sea. The SCORAD index in the S. aureus(+) group was higher than in the S. aureus(-) group, however it was not significant.(44+/-14.2 vs 38+/-17.1, P= 0.304) The SCORAD index was higher in the exotoxin(+) group than in the exotoxin(-) group(49+/-11.2 vs 38+/-16.2, P<0.05). The prevalence of S. aureus and its exotoxins in the AD group was higher than in the normal control group(P<0.001, P<0.05, respectively). The difference of SCORAD index was significant between the exotoxin(+) group and the exotoxin(-) group, but not between the S. aureus(+) group and S. aureus(-) group.(P<0.05, P= 0.304, respectively) CONCLUSION: The exotoxins of S. aureus were found to influence the severity of AD.

Immediate Improvement of Chaotic Atrial Tachycardia after Ligation of Patent Ductus Arteriosus

Chaotic or multifocal atrial tachycardia is very rare abnormality in cardiac rhythm for pediatric patients. A newborn female baby was brought to the hospital for tachycardia and showed heart failure symptoms such as poor oral intake, tachypnea, and chest wall retraction. Electrocardiogram and 24-hour holter monitoring revealed chaotic atrial tachycardia and echocardiogram revealed large patent ductus arteriosus and small secundum atrial septal defect associated with pulmonary hypertension. Medical treatment had been unsuccessful and large patent ductus arteriosus was ligated with subsequent improvement of chaotic atrial tachycardia.

Successful Treatment of Candida albicans Endocarditis with Liposomal Amphotericin B (AmBisome(R)) in a Very Low Birth Weight Infant

Candida albicans endocarditis is an uncommon manifestation of systemic candidiasis in newborn infants who require intensive care and develops mostly in patients with congenital heart disease; open heart surgery is the majority of predisposing factor. Improvement of techniques managing premature infants leads to increased survival rates, which give much more chances to develop fungal infections and its complications. We report a case of very low birth weight infant who had candidemia and Candida endocarditis, who was successfully treated with AmBisome(R) because of no response to conventional amphotericin B therapy.

Overview and Evaluation of Pulmonary Hypertension

The Prevalence of pediatric pulmonary hypertension is rare in general population, and the etiology of this disease is diverse or unknown. But, in recent years, remarkable understanding of vascular biology, normal and hypertensive pulmonary circulation has made an effort to the pathophysiology of pulmonary arterial hypertension. These efforts are adapted the patients treatments with new advanced drugs. In this reviw, we discuss the definition and the clinical classification of pulmonary hypertension, current concepts in the pathophysiology, diagnosis, and managements of pulmoanry hypertension. But, Despite of significant development of therapy, the patients with pulmonary hypertension in pediatric division are rare, so multicenter study in clinical setting is necessary to improve the quality of life and survival for them.

Overview and Evaluation of Pulmonary Hypertension

The Prevalence of pediatric pulmonary hypertension is rare in general population, and the etiology of this disease is diverse or unknown. But, in recent years, remarkable understanding of vascular biology, normal and hypertensive pulmonary circulation has made an effort to the pathophysiology of pulmonary arterial hypertension. These efforts are adapted the patients treatments with new advanced drugs. In this reviw, we discuss the definition and the clinical classification of pulmonary hypertension, current concepts in the pathophysiology, diagnosis, and managements of pulmoanry hypertension. But, Despite of significant development of therapy, the patients with pulmonary hypertension in pediatric division are rare, so multicenter study in clinical setting is necessary to improve the quality of life and survival for them.

The Efficacy of Modified Puberty Suppression Score by Single Blood Sample for Evaluation of GnRH Agonist Treatment in Central Precocious Puberty / 대한소아내분비학회지

PURPOSE: GnRH stimulation test is golden standard for the diagnosis of central precocious puberty as well as evaluation of treatment, however, it is more expensive and inconvenient. This is the reason why many other tests have been suggested. We studied the efficacy of modified puberty suppression score by single blood sample for evaluation of GnRH agonist treatment in central precocious puberty. METHODS: Twenty-four girls (age, 9.56+/-1.56 years) diagnosed with early puberty or precocious puberty at Kangdong Sacred Heart Hospital from March 2002 to May 2005 were included in this study. All of patients were treated with leuprorelin acetate (83.66-115.12 microgram/kg). Total 24 patients including 11 suppression and 13 non-suppression cases were analyzed. The serum levels of LH, FSH, estradiol and progesterone were measured before and 8 weeks after treatment. The height, weight, bone age and Tanner stage of breast development in each patient were also measured before and 12 weeks after treatment. We modified puberty suppression score by Mul et al. in 1999. We defined scores based on statistical significance - estradiol, 2 points (>=1.36 ng/dL), progesterone, 2 points (>=0.31 ng/dL), LH, 1 point (>=2.0 IU/L), delta BA/delta CA, 1 point (>=0), delta HtSDS, 1 point (>=0.25/6 mo). Total score is 7 points and we defined suppression is less than 3 points. RESULTS: The serum levels of estradiol (<1.36 ng/dL, P=0.000) and progesterone (<0.31 ng/dL, P= 0.003) are significantly lower in suppression group than nonsuppression group. If the score according to modified puberty suppression score (MPSS) is less than 3 points, which is considered as a successful suppression by GnRH agonist. The sensitivity, specificity, positive predictive value and negative predictive value of MPSS are 100%, 92.8%, 90.9% and 100% respectively. CONCLUSION: Single blood sample is simpler and easier than GnRH stimulation test for the evaluation and monitoring of GnRH agonist treatment in central precocious puberty and MPSS by single blood sample may be useful in outpatient clinic.

Chromosome 7q Deletion Syndrome 46, XX, del(7)(q34) with Hydronephrosis

Chromosome 7q deletion, relatively rare syndrome, was first described by de Grouchy in 1969. The most frequent clinical manifestations of a "7q deletion syndrome" include; low birth weight, postnatal growth retardation, mental retardation, developmental delay, microcephaly, congenital heart disease, hypotonia, bulbous nasal tip and abnormal ears. We report a case of 7q deletion syndrome with microcephaly, upslanting palpabral fissure, micrognathia, bulbous nasal tip, developmental delay and hydronephrosis.

The Efficacy and Safety of Inhaled Steroid Therapy for Prevention of Recurrent Wheezing after Bronchiolitis / 소아알레르기및호흡기학회지

PURPOSE: The purpose of this study was to evaluate the efficacy and metabolism of inhaled steroids to prevent recurrent wheezing after bronchiolitis. METHODS: Sixty two patients were randomly divided into study (n=31) and control (n=31) groups. All of them received budesonide 500 microgram and salbutamol 1.25 mg 4 times a day via nebulizer during admission period (5.5+/-2.5 days). After discharge, the study group patients received fluticasone 50 microgram twice a day with metered dose inhaler with mask spacer for 12 weeks, and the control group received none of inhaled steroids. Serum cortisol and dehydroepiandrosterone sulfate (DHEA-S) concentrations were measured at admission and at the end of the inhaled corticosteroid (ICS) therapy. Weight and height of all patients were checked during the follow-up period. RESULTS: Atopic dermatitis of the patient and allergy family history proved statistically significant. Among high risk group patients, the attack rate of recurrent wheezing in the study group was significantly reduced as compared with the control group. Cortisol levels checked at the end of the ICS therapy were not significantly different from the level checked at admission. In the study group, there was no statistically significant difference between dehydroepiandrosterone sulfate (DHEA-S) level at admission and at the end of the ICS therapy. There was no statistically significant difference of height and weight SDS (standard deviation score) between baseline and 12 weeks later. CONCLUSION: This study suggest that inhaled corticosteroid can be used prophylactically for reducing recurrent wheezing after bronchiolitis in high risk group for asthma.

Morphologic Changes of the Pulmonary Arteries after Stent Implantation on Branch Pulmonary Artery Stenosis: Impact of Pulmonary Insufficiency

PURPOSE: Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. METHODS: The subjects in our study were 23 children who had undergone stent implantaion from Jan. 1995 to Jul. 2002 in the Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. RESULTS: The CSAI of the RPA without stenosis changed from mean 238+/-17 mm2/BSA to mean 249+/-20 mm2/BSA(P=0.47), but didn't regress. The CSAI of the LPA with stenosis was increased effectively by stent implantation from the mean 102+/-12 mm2/BSA to mean 125+/-11 mm2/BSA(P< 0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. CONCLUSION: In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPA.

High-dose Methylprednisolone Pulse Therapy in Measles Postinfectious Encephalomyelitis: Two Cases of Successful Recovery / 대한소아신경학회지

We treated two patients of measles postinfectious encephalomyelitis with intravenous methylprednisolone pulse therapy(1 g/1.73 m2/day for 5 days) 38 hours and 5 days respectively after the onset of neurologic symptoms. Despite extensive white matter involvement shown in MRI and severe clinical symptoms, the patients recovered from their neurologic symptoms dramatically following intravenous methylprednisolone pulse therapy. Because acute postinfectious encephalomyelitis has been postulated to be immunologically mediated disease, instead of direct viral invasion, ACTH and dexamethasone are widely used and the outcome is variable. This case report of successful recovery from fulminant ADEM with pulse intravenous methylprednisolone therapy, although uncontrolled, suggests that this therapeutic regimen should be studied in other cases.