Jae Sook Ma

Epidemiological features of Kawasaki disease in Korea, 2006–2008

Background:  The aim of this study was to estimate the incidence and describe the epidemiological characteristics of Kawasaki disease among children in Korea.

Epidemiologic picture of Kawasaki disease in Korea, 2000–2002

Background : The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for a 3 year period during 2000 to 2002.

Right ventricular failure in congenital heart disease

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

Clinical significance of pleural effusion in the new influenza A (H1N1) viral pneumonia in children and adolescent

Parapneumonic effusion has been reported to develop either in typical bacterial infection or in viral pneumonia with bacterial co‐infection and to cause death. Swine‐origin influenza A (H1N1) virus infection can be accompanied with pleural effusion; however, there are no reports about the significance of pleural effusion in H1N1 pneumonia. We retrospectively analyzed both the clinical characteristics and the significance of pleural effusion associated with H1N1 pneumonia in children and adolescent.

Mosaic Ring Chromosome 6 in an Infant with Significant Patent Ductus Arteriosus and Multiple Congenital Anomalies

The clinical features of ring chromosome 6 include central nervous system anomalies, growth retardation, facial dysmorphism and other congenital anomalies. Ring chromosome 6 occurs rarely and manifests as various phenotypes. We report the case of mosaic ring chromosome 6 by conventional karyotyping in a 7-day-old male infant diagnosed with a large patent ductus arteriosus (PDA) with hypoplasia of aortic valve and aortic arch. These have not been previously reported with ring chromosome 6. He recovered from heart failure symptoms after ligation of the PDA. He showed infantile failure to thrive and delayed milestone in a follow-up evaluation. To the best of our knowledge, this is the first report of a Korean individual with ring chromosome 6 and hemodynamically significant PDA.

Recurrent Plastic Bronchitis in a Child with 2009 Influenza A (H1N1) and Influenza B Virus Infection

Plastic bronchitis is an uncommon disorder characterized by the formation of bronchial casts. It is associated with congenital heart disease or pulmonary disease. In children with underlying conditions such as allergy or asthma, influenza can cause severe plastic bronchitis resulting in respiratory failure. A review of the literature showed nine cases of plastic bronchitis with H1N1 including this case. We report a case of a child with recurrent plastic bronchitis with eosinophilic cast associated with influenza B infection, who had recovered from plastic bronchitis associated with an influenza A (H1N1) virus infection 5 months previously. To the best of our knowledge, this is the first case of recurrent plastic bronchitis related to influenza viral infection. If patients with influenza virus infection manifest acute respiratory distress with total lung atelectasis, clinicians should consider plastic bronchitis and early bronchoscopy should be intervened. In addition, management for underlying disease may prevent from recurrence of plastic bronchitis.

Henoch-Schönlein purpura nephritis associated with Epstein-Barr virus infection in twins

We recently observed 6-year-old twin boys with HenochSch nlein purpura (HSP) nephritis associated with Epstein-Barr virus (EBV) infection. These twin boys were identical and healthy up to the age of 6 years. Twin 1 was admitted to our hospital because of abdominal pain, arthralgia in the left knee joint, and purpura on both lower extremities; 15 days before admission, he showed mild fever and cough; 3 days later, he developed intermittent abdominal pain and diffuse purpuric rash on his lower extremities. He was given acetaminophen for 3 days; 2 days before admission, he developed pain and swelling in the left knee joint. On admission, laboratory investigations revealed hemoglobin of 13.3 g/dl, white blood cell count of 15,600/mm3, platelet count of 296,000/mm3, erythrocyte sedimentation rate of 33 mm/h, blood urea nitrogen of 11.3 mg/dl, serum creatinine of 0.7 mg/dl, and serum albumin of 3.8 g/dl. Serum electrolytes, liver enzymes, blood coagulation tests, C3, C4, antinuclear antibody, rheumatoid factor, antistreptolysin O titer, and Mycoplasma antibody were normal. Urinalysis showed 3+ protein and 3+ blood, with many red blood cells (RBC) per high-power field (HPF). Urine protein excretion was 260 mg/m2 per day. EBV serology showed anti-EBV capsid antigen (VCA) IgM ( ), anti-EBV VCA IgG (+), anti-EBV early antigen (EA) IgG (+), and anti-EBV nuclear antigen (NA) IgG ( ). Other viral investigations for parvovirus B19, adenovirus, hepatitis B virus, and hepatitis A virus were negative. The patient was treated with intravenous hydration and 1 mg/kg per day oral prednisolone. The clinical findings of arthralgia, abdominal pain, and purpuric rash improved gradually over 10 days. He was discharged after 10 days with normal urinalysis. Physical examination and urinalysis were normal 6 months after discharge. Twin 2 was admitted on the same day as twin 1; 4 days before admission, he developed a mild cough. He was not receiving any medication; 1 day before admission, he had a painful left ankle joint and purpura on both lower extremities. On admission, he showed diffuse purpuric rash on the lower extremities and buttocks. He also had painful and swollen knees and ankles. Laboratory studies revealed hemoglobin of 11.7 g/dl, white blood cell count of 8,700/mm3, platelet count of 346,000/mm3, and normal microscopic urinalysis. EBV serology showed anti-EBV VCA IgM ( ), anti-EBV VCA IgG (+), anti-EBV EA IgG (+), and anti-EBV NA IgG ( ). Other laboratory studies and viral investigations were negative. He was treated with intravenous hydration and bed rest; 3 days after admission, he complained of severe abdominal pain and edema on the right ear auricle. He was treated with 1 mg/ kg per day oral prednisolone for 7 days. The clinical findings resolved gradually, so he was discharged with twin 1; 2 weeks after discharge, urinalysis revealed 2+ proteinuria and hematuria of 30–39 RBC/HPF. Other clinical findings were normal. He showed isolated microscopic hematuria for 1 month. Physical examination and urinalysis were normal 6 months after discharge. The cause of HSP is unknown, but HSP typically follows an upper respiratory tract infection. EBV has been reported to precede HSP [1]. The results of EBV serology in children are often different from adults or adolescents. In patients with negative heterophil antibody and antiEBV VCA IgM, tests for anti-EBV VCA IgG or anti-EBV C. J. Kim · Y. J. Woo · H. Kook · Y. Y. Choi · J. S. Ma · T. J. Hwang Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea

Radiodensity on serial chest X-rays for the diagnosis of foreign body aspiration in children

ObjectivesTo evaluate the utility of measuring lung radiodensity from chest X-ray for the diagnosis of foreign body aspirationMethodsRecords of 59 children with foreign body aspiration were retrospectively reviewed. Lung radiodensity and radiodensity ratio (right/left lung radio density) before and after foreign body removal were measured. Radiodensity was calculated as the relative score compared with the tenth thoracic vertebra body (100 points) and the background (0 point). The change of radiodensity ratio (difference in radiodensity ratio of the second X-ray from that of first X-ray) was compared between 22 patients (foreign body group) and 22 normal subjects (control group).ResultsIn the group of foreign body in the left bronchus, the mean (SD) radiodensity of the left lung [53.5 (12.8)] was lower than that of the right lung [60.8 (7.7), P<0.01] and it increased after foreign body removal [60.0 (6.9), P=0.02]. The radiodensity ratio decreased from 1.20 (0.30) to 0.96 (0.09) (P<0.01) after foreign body removal. In the group with a foreign body in the right bronchus, the radiodensity of the right lung [51.8 (12.8)] was lower than that of left lung [62.0 (11.7), P=0.03], and it also increased after foreign body removal [58.4 (9.6), P=0.03]. The change of radiodensity ratio in the foreign body group [15.7 (17.8)%] was higher than the control group [5.4 (4.3)%, P=0.01] and the cutoff value was 7.5%.ConclusionRadiodensity from chest X-ray could be a useful tool for diagnosing foreign body aspiration in children.

Hypokalemic paralysis and rhabdomyolysis in distal renal tubular acidosis

Distal renal tubular acidosis (RTA) is caused by impaired distal acidification and is characterized by the inability to lower urine pH maximally (below 5.5) under the stimulus of systemic acidemia. 1 Characteristic symptoms of distal RTA include polyuria, hypercalciuria, nephrocalcinosis, growth retardation, acidosis, and hypokalemia. 1 The most prominent clinical features of hypokalemia are neuromuscular manifestations, that include muscle weakness and paralysis, as well as an increased incidence of rhabdomyolysis. 2 Rhabdomyolysis can be a life-threatening disorder, with acute renal failure being one of the most serious complications. 3

Nationwide survey of coronary aneurysms with diameter >6 mm in Kawasaki disease in Korea.

We investigated the course of coronary aneurysm with diameter >6 mm in Kawasaki disease (KD), as well as related therapeutic trends and prognosis in Korea.