Cancer in thyroid nodules with fine-needle aspiration in Korean pediatric populations.

PURPOSE: To determine the prevalence and clinical findings of benign thyroid nodules and cancer in Korean pediatric patients with thyroid nodules. METHODS: We investigated the medical records of 134 patients aged younger than 18 years who had a goiter, thyroid nodule, thyroid mass, or thyroid cancer who underwent fine needle aspiration biopsy (FNAB). RESULTS: The study population included 113 females (84.3%) and 21 males (13.7%); the mean patient age was 16.1±2.3 years (range, 8-18 years). Of the 134 patients, 24 (18.0%) were finally diagnosed with thyroid cancer, of which 20 (83.3%) were papillary cancer and 4 (16.7%) were follicular cancer. No patient was exposed to radiation. FNAB revealed malignant cancer in 21 of the patients; 3 were initially reported as having benign tumors by FNAB, but were later diagnosed with follicular cancer. An additional 13 patients were suspected to have malignant tumors by FNAB, with a final diagnosis of nodular hyperplasia. Cystic nodules were more common in the benign group. The percentages of cervical lymphadenopathy and irregular nodular margins were higher in the malignant group compared to the benign group. Cervical lymphadenopathy and FNAB malignant findings were highly suggestive of malignant nodules. CONCLUSION: Thyroid cancer prevalence in a Korean pediatric population is comparable to reported estimates of worldwide thyroid cancer prevalence. In this population, cancer predominates on the right thyroid lobe. Papillary thyroid cancers are dominant in the Korean pediatric population but are less prevalent than in Korean adults. As expected, FNAB was highly accurate in predicting malignant nodules.

Dilated cardiomyopathy with Graves disease in a young child.

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.

Massive Cardiomegaly due to Dilated Cardiomyopathy Causing Bronchial Obstruction in an Infant.

Dilated cardiomyopathy (DCMP) remains a life threatening disease in young patients and is often difficult to differentiate from myocarditis. Early recognition and treatment of DCMP are crucial for good prognoses in this patient population. The clinical course of patients with DCMP that result in cardiogenic shock varies according to the etiology as well as patient age. The volumetric expansion of the enlarged heart can compress adjacent structures causing a number of related symptoms, especially in infants with soft cartilaginous bronchi. Therapeutic strategies for treating these issues vary according to the type of complication encountered. We report a case of severe DCMP with sudden onset of massive cardiomegaly with heart failure complicated by bronchial obstruction in an infant.

Recent incidence of congenital heart disease in neonatal care unit of secondary medical center: a single center study.

PURPOSE: With feasibility in the diagnoses of congenital heart disease (CHD) in the antenatal period, we suspect changes have occurred in its incidence. No data have been reported about the current incidence of simple forms of CHD in Korea. We have attempted to assess the recent incidence and characteristics of CHD in the neonatal care unit of a secondary referral medical center. METHODS: Medical records of 497 neonatal care unit patients who underwent echocardiography in the past 5 years were reviewed. Pre-term infants with patent ductus arteriosus and other transient, minimal lesions were excluded from this study. RESULTS: Although the number of inpatients remained stable, the incidence of simple forms of CHD showed a gradual decrease over the 5-year study period; a markedly low incidence of complex forms was seen as well. CHD was observed in 3.7% full-term and 6.8% pre-term infants. CHD was observed in 152 infants weighing >2,500 g (3.5% of corresponding birth weight infants); 65 weighing 1,000 to 2,500 g (9.3%); and 6 weighing <1,000 g (8.0%). The incidence of CHD was higher in the pre-term group and the low birth weight group than in each corresponding subgroup (P<0.001); however, the incidence of complex CHD in full-term neonates was high. The number of patients with extracardiac structural anomalies has also shown a gradual decrease every year for the past 5 years. CONCLUSION: Findings from our study suggest that the recent incidence and disease pattern of CHD might have changed for both complex and simple forms of CHD in Korea.

Polymorphisms of human leukocyte antigen genes in korean children with Kawasaki disease.

BACKGROUND: Kawasaki disease is a leading cause of acquired heart disease in children. The prevalence rate varies in different ethnic groups. Recently, with the clinical application of molecular genetic technology, human leukocyte antigen (HLA) polymorphisms associated with several diseases have been identified by DNA analysis. This study aimed to assess the association of HLA alleles with susceptibility and complications of Kawasaki disease in Korean children. METHODS: In this study, DNA was extracted from 74 children with a diagnosis of Kawasaki disease. The polymorphisms of the HLA-A, -B, -C, and -DRB1 alleles of patients with Kawasaki disease were determined by polymerase chain reaction (PCR)-amplification refractory mutation system (ARMS) and PCR-sequence-specific primer (SSP) analysis. The polymorphisms identified were compared with those of 159 normal healthy control subjects. RESULTS: There was a significant increase in the frequencies of the HLA-B35, -B75, and -Cw09 alleles in patients with Kawasaki disease compared with the healthy control group. There was no increase in the frequency of HLA-DRB1 alleles among the Kawasaki disease patients compared with a healthy control group. When the patients with Kawasaki disease were divided into two subgroups, with or without coronary complications, the Kawasaki disease patients with coronary complications showed a significantly increased frequency of the HLA-DRB1*11 allele compared with the healthy control group and increased frequency of HLA-DRB1*09 in a comparison of the subgroups. CONCLUSIONS: This study suggests that polymorphisms in some alleles of B and C in HLA class I genes are associated with Kawasaki disease in Korean children.

Alteration of the influences of HLA classes I and II alleles on the perinatal hepatitis B virus infection after immunoprophylaxis in Korean children.

Some HLA alleles are known to be associated with hepatitis B virus (HBV) persistence. In order to find out the relationship between HLA and perinatal HBV infection after prophylaxis, we typed HLA classes I and II in 38 HBeAg-positive mothers, their children (19 succeeded and 19 failed in prophylaxis) and 198 HBsAg-negative healthy controls. HLA-B35 (RR=2.8, p<0.03), Cw*07 (RR=2.7, p<0.02), DRB1*07 (RR=3.6, p<0.006), DQA1*02 (RR=3.6, p<0.02) and DQB1*02 (RR=2.4, p<0.05) alleles were higher and DRB1*13 (RR=0.3, p<0.03) and DPB1*0401 (RR=0.1, p<0.01) alleles were lower frequencies in HBeAg-positive mothers than in the control. In failed children to the perinatal HBV prophylaxis, HLA-Cw*0303 allele was significantly higher (p<0.05) and DPB1*0202 allele was lower (p<0.03) than in succeeded children. These results suggest the influences of certain HLA alleles on naturally acquired chronic HBV infection may be changed by perinatal HBV prophylaxis.

[The incidence rate of hepatitis B virus surface gene variants in Korean children with immunoprophylaxis failure of perinatal infection].

BACKGROUND/AIMS: Perinatal infection with hepatitis B virus (HBV) may occur despite immunoprophylaxis. One of the important mechanisms for perinatal prophylaxis failure, might include HBV surface gene variants. Therefore, we screened Korean children, in whom perinatal prophylaxis failed, for HBV surface gene variants. METHODS: Thirty-one children with perinatal HBV prophylaxis failure were selected. To amplify the major hydrophilic region of the HBV surface gene, nested PCR with primers targeted to nucleotides 237 to 706 was performed, and then sequencing was done. RESULTS: All cases were shown to be PCR positive for HBV-DNA and genotype C. Nine out of 31 (29%) with perinatal prophylaxis failure had a nucleotide substitution at the major hydrophilic region of the gene; but only two cases (6.5%) had an amino acid substitution. One case was infected by wild type and variants of I126S, and the other by wild type and S114A+I126S, respectively. CONCLUSIONS: In Korea, compared to the previous studies of other nations, gene surface variants such as G145R do not appear to play an important role in perinatal immunoprophylaxis failure.