Therapeutic Efficacy of Lamivudine in Children and Adolescents with Chronic Hepatitis B

PURPOSE: This prospective study aimed to investigate the therapeutic efficacy of lamivudine in children with chronic hepatitis B virus (HBV) infection. METHODS: During July 2003 through October 2015, children with chronic hepatitis B who visited our institution were included in this study. Fifty-five patients, who received first-line treatment of lamivudine (3 mg/kg, 100 mg maximum) for over three months, were enrolled. After initiating lamivudine, alanine aminotransferase (ALT), HBV-DNA, and HBV markers were followed up at 1 month, 3 months, and every 3 months, thereafter. The treatment endpoint was determined as 1) normalization of ALT, 2) HBeAg seroconversion, and 3) anti-HBe positivity for twelve consecutive months. RESULTS: Thirty-one male (56.4%) and 24 female (43.6%) patients were included. The mean age at treatment initiation was 8.1 years. The mean duration of treatment was 23.4 months. ALT normalization was found in 98.2% (54 of 55). Anti-HBe seroconversion was found in 70.6% (36/51). Loss of HBsAg was found in 10.9% (6/55). All biochemical responses occurred under age seven. The rate of virologic response (defined as HBV-DNA <2,000 IU/mL) at six months after treatment initiation was 78.7% (37/47). At twelve months after reaching treatment endpoint, 87.2% (34/39) maintained their virologic response. Resistance to lamivudine was found in 16.4% (9/55). CONCLUSIONS: Lamivudine treatment in Korean pediatric patients with chronic hepatitis B showed better outcomes compared with other studies that implemented similar protocols in foreign populations. Further studies are needed to investigate the efficacy of newly recommended antiviral drugs on the Korean pediatric population.

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. CONCLUSIONS: 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.

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.

A Case of Epidural Abscess Complicated from Acute Mastoiditis Caused by Streptococcus pneumoniae / 소아감염

Acute otitis media (AOM) is one of the most common childhood infectious diseases. Despite antibiotic treatment for AOM, AOM and its complication still continue to develop. Acute mastoiditis is a serious complication of AOM and epidural abscess constitutes the commonest of all intracranial complication of AOM. Neurological complication of acute mastoiditis are rare but can be life threatening. Their presentation may be masked by the use of antibiotics. We report the rare case of acute otitis media progressing to acute mastoiditis, epidural abscess formation and lateral sinus thrombophlebitis caused by Streptococcus pneumoniae in a child. She was admitted with acute otitis media with fever. Despite proper antibiotics, acute mastodititis and epidural abscess were developed, and after surgical drainage and antibiotics therapy she was recovered without sequalae.

Comparison among Known Severity Scoring Scales in the Evaluation of Acute Gastroenteritis in Children / 소아감염

PURPOSE: This study aimed to evaluate the disease severity of children suffering from gastroenteritis using different scales. The results are compared and subsequently classified on the basis of the type of virus causing the disease in order to investigate the differences in clinical characteristics and disease severity according to pathogen. METHOD: This study was conducted prospectively with patients under 5 years of age diagnosed with acute gastroenteritis and hospitalized at 9 medical institutions in 8 regions across the Republic of Korea. Disease severity was evaluated using the Vesikari Scale, the Clark Scale, and the modified Flores Scale. Fecal samples collected from patients were used to detect rotavirus and enteric adenovirus by enzyme immunoassay, and for RT-PCR of norovirus, astrovirus, and sapovirus. RESULTS: There were a total of 214 patients with a male : female ratio of 1.58 : 1, of which 35 were under the age of 6 months (16.4%), 105 were aged 6-23 months (49.1%), and 74 were aged 24-59 months (34.5%). The rate of concordance between the Vesikari and Clark Scales was 0.521 (P<0.001) and, in severe cases, the Vesikari Scale was 60.7% and Clark Scale was 2.3%, indicating that the Clark Scale was stricter in the evaluation of severe cases. CONCLUSIONS: In children with gastroenteritis, there were differences in disease severity based on the scale used. Therefore, to achieve consistent results among researchers, either only a single scale or a measure of all scales should be used to determine disease severity.

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 / 소아과

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.

A Pediatric Case of Primary Sjogren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis

Sjogren's syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.

A Survey for Changed Control Policies of Hepatitis B in Republic of Korea / 소아감염

PURPOSE: We have tried to search all concerning data on the policies of hepatitis B vaccination or surveillance of medical equipments that can transmit blood mediated diseases, including disposable syringe, in Republic of Korea, to propose references to other medical professionals. METHODS: Data from domestic journals, government websites, computerized newspapers, etc. were reviewed. The data were arranged into four categories (governmental policies on hepatitis B control, hepatitis B mass immunization program in school, using process of hepatitis B vaccine, and policies on medical equipments surveillance, including disposable syringes, which is considered as source of blood mediated infection), and the periodic changes on each subjects in policies were reviewed. RESULTS: Due to growing social concerns on hepatitis B in early 1980s, swift introduction and production of hepatitis B vaccine was made. After establishment of "5 year project for eradication of hepatitis B" in late 1983, number of vaccinated population jumped up to 6 million within 2 years (1984-85). However, since the immunization targets were mainly adult, not infant, this project was annulled in late 1985. Following this project, hepatitis B mass immunization program in school was carried out. In year 1995, hepatitis B vaccine was included in national immunization program. The use of disposable syringe was recommended from year 1980 but legislated in year 1985, finally. CONCLUSION: The mainstay in controlling hepatitis B in Republic of Korea was coordination of prompt introduction of vaccine, mass immunization, high vaccination coverage rate, or use of disposable syringes. However, since there is lack of official data available, it is urgent to arrange and computerize all government data related to infectious diseases.

A Comparison of Clinical Findings According to the Duration of Pyuria in Infants with Urinary Tract Infections / 소아감염

PURPOSE: Urinary tract infection (UTI) in children is the most common disease during the infantile period, therefore early diagnosis and treatment are important. Pyuria is a useful clinical parameter for the initial diagnosis of a UTI. In this study we aimed to compare the clinical, laboratory, and imaging findings in relation to the duration of pyuria in infants with UTIs. METHODS: Three hundred seventy-four infants <12 months of age who were admitted between January 1995 and December 2005 for the first episode of a febrile UTI were retrospectively reviewed. Patients were divided into two groups according to the duration of pyuria as follows: group 1, pyuria resolved <3 days after initial treatment; and group 2, pyuria lasted at least 3 days after initial treatment. RESULTS: There were no significant differences between the two groups in relation to gender, age, total duration of fever, and organisms in the urine. Group 2 had a significantly higher peripheral blood leukocyte count (14,360.86+/-5,526.16 cells/mm3 vs. 11,822.55+/-5,687.26 cells/mm3, P<0.001), erythrocyte sedimentation rate (32.81+/-19.34 mm/hr vs. 23.74+/-20.43 mm/hr, P<0.001), and C-reactive protein (6.84+/-5.68 mg/dL vs. 3.78+/-3.99 mg/dL, P<0.001) than group 1. There was a significantly higher incidence of hydronephrosis and a higher grade of vesicoureteral reflux (VUR) in group 2 compared to group 1. CONCLUSION: In infants with UTI, pyuria of longer duration is related to severe UTI and higher grade VUR, therefore aggressive radiologic studies may be necessary.

The Change of Immunologic Parameters in Acute Poststreptococcal Glomerulonephritis

PURPOSE: This study was aimed to evaluate the changes of immunologic parameters during hospitalization, and the relationship between IgG and other laboratory or clinical indices in patients with acute poststreptococcal glomerulonephritis (APSGN). METHODS: We reviewed the medical charts of 36 children with APSGN who showed ASO titer >250 Todd U/L and C3125 mmHg), gross hematuria, and weight gain were observed in 27.8% (10/36), 80.1% (29/36), and 80% (24/30) of the patients, respectively. The mean IgG level was 1,432+/-322 mg/dL (1,025+/-234 mg/dL in control group, P8 years of age (15 cases) (-0.6% vs. -5.7%, P=0.01). The IgG and ASO levels did not change, but C3 (P=0.001) and IgM (P=0.02) levels increased during admission. CONCLUSION: Increased IgG and ASO levels in APSGN did not change, but C3 level increased during admission. IgG level was not correlated with other laboratory parameters (ASO and C3) and the severity of the disease. Younger children seem to have less severe clinical course compare to older children. With our hypothetic pathogenesis of APSGN, further studies are needed to resolve the pathogenesis of the disease including the increase of IgG.

Comparison of Urinary Tract Infections Caused by Escherichia coli and Non-E. coli in Infants / 소아감염

PURPOSE: Urinary tract infection (UTI) is one of the most common bacterial infectious disease in childhood. Renal scarring is an important complication of UTIs. Known risk factors for renal scarring are younger age, anatomic defects, delayed treatment, and causative pathogens other than Escherichia coli. The aim of this study was to compare the characteristics of clinical and laboratory features of UTI with E. coli to those with non-E. coli in infants. METHODS: We reviewed the medical records of 1,120 infants under 12 months of age who had been admitted for UTIs between January 1998 and December 2007. All patients who were diagnosed with UTIs were divided into two groups (E. coli and non-E. coli UTIs). RESULTS: Three hundred twenty-four of 1,120 cases met the inclusion criteria. The number of E. coli and non-E. coli UTIs was 273 (84.3%) and 51 (15.7%), respectively. As compared to the non-E. coli UTI group, the E. coli UTI group was younger (3.59 vs. 4.47 months, P=0.008), a longer duration of pyuria (3.96 vs. 3.06 days, P=0.01), higher peripheral white blood cell counts (13.89 vs. 12.13x10(3)/mm3, P=0.043), and lower rates of high degree (III-V) vesico-ureteral reflux (P=0.005). CONCLUSION: UTIs with E. coli might have more severe clinical features and a lower prevalence of high grade vesico- ureteral reflux than UTIs with non-E. coli. However, no difference was noted in the clinical response to antibiotic therapy between the two groups.

The Clinical Features of Pertussis in Infancy / 소아감염

PURPOSE: Pertussis was very common in the past, but reported cases have dramatically decreased. The improvement of vaccination programs and unreadiness of laboratory confirmation seems to have developed this situation. This study investigated the frequency of pertussis among infants with a paroxysmal cough and compared the clinical characteristics between infants with and without pertussis. METHODS: Between June and November 2006, 27 infants admitted to the hospital that were 15-90 days old with a history of a cough for more than seven days were enrolled. The cough was described as: paroxysmal, whooping, and post-tussive vomiting. PCR and cultures for Bordetella pertussis with nasopharyngeal aspirates were obtained. The patients were divided into two groups: (1) the pertussis group that had positive results by PCR or culture; (2) the control group that had negative results by PCR and culture. Clinical and laboratory characteristics were compared between the two groups. RESULTS: Among the 27 cases, five (18.5%) were finally diagnosed with pertussis. Only one out of the five pertussis cases was initially diagnosed with a pertussis-like syndrome on admission. Compared to the group without pertussis, the pertussis group had a significantly higher frequency of: no fever (P=0.043), a paroxysmal cough (P=0.040), cyanosis (P=0.001), non-immunized status for DTaP (P=0.047), normal auscultation (P=0.028), normal chest X-ray findings (P=0.027), high absolute lymphocyte count (P=0.039), and low CRP (P=0.046). The patients with the diagnosis of pertussis had a significantly longer duration of coughing (27.2+/-10.6 vs. 12.6+/-5.6 days, P=0.039). CONCLUSION: Pertussis should be suspected in any infant with typical symptoms of pertussis in addition to: a persistent cough without fever, accompanied by paroxysms or cyanosis prior to the age of DTaP immunization. Active laboratory confirmation should be carried out to confirm more cases with pertussis.

A Survey for Understanding of Breastfeeding among Mothers with Chronic Hepatitis B Infection / 소아감염

PURPOSE: Breastfeeding should be recommended for infants born to mothers with chronic hepatitis B Infection after postexposure prophylaxis. However, high proportion of these mothers are reluctant to engage in breastfeeding in Korea. This survey was taken to identify the cause of that reluctance. Method:Questionnaires were given to mothers with chronic hepatitis B infection who were registered at the 'Hepatitis B Perinatal Transmission Prevention Program' operated by Korea Center for Disease Control and Prevention. They visited a community health center for blood sample collection and signed a consent paper. The questionnaires were sent to the mothers. Result:Among 839 mailed questionnaires, 114 were returned marked 'address unknown'. The overall reply rate was 17% (n=125). Among responders, 52% (n=62) were breastfeeding and 48% (n=60) were formula-feeding. The most influential factor for breastfeeding was the mother's own decision (75%) and the obstetrician's recommendation (17%). For formula- feeding mothers, their decisions were influenced by obstetricians (57%), and by their own thinking (28%). The relationship between breastfeeding and perinatal prophylaxis failure was recognized as 45% 'related' and 50% 'not-related'. A total of 91% of breastfeeding mothers replied that they will breast-feed again. Among formula-feeding mothers, 78% answered that they will breast-feed if they were known that 'there is no direct relationship between breastfeeding and perinatal prophylaxis failure'. Conclusion:Despite the fact that there is no direct relationship between breastfeeding and perinatal prophylaxis failure, many were reluctant to breast-feed. Healthcare professionals have influence over the mothers for decision making. It will be necessary to educate healthcare personnel so that they can make a conceptual change as well as to promote the fact to the general public.

Correlation between Serum Albumin Level and IgG Level in Minimal Change Nephrotic Syndrome

PURPOSE: Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated the relationship between the serum IgG and albumin levels for children with minimal change nephrotic syndrome(MCNS). METHODS: The levels of immunoglobulin G(IgG), albumin and total cholesterol of a total of 46 children with MCNS(proteinuria >40 mg/m2/h, and serum albumin level <2.5 g/dL) were analyzed. RESULTS: The mean values of albumin, IgG and total cholesterol in MCNS children were 1.7+/-0.3 g/dL, 368+/-143 mg/dL and 431+/-78 mg/dL, respectively. There was an inverse correlation between the albumin values and the total cholesterol values(r=0.68, P=0.0001), whereas there was a direct-proportional correlation between albumin values and the IgG values(r=0.4, P=0.01). CONCLUSION: The IgG level is associated with albumin level, and it may reflect the severity of urinary protein loss in MCNS. Further studies are needed to evaluate this phenomenon.

Coronary Complication according to Retreatment of Immune Globulin in Kawasaki Disease

PURPOSE: Recently most of patients with Kawasaki disease (KD) get treatment within several days of illness. But, some patients still suffer from coronary complication (CC) despite early initial treatment with intravenous immune globulin (IVIG) and even the additional therapy. We investigated the risk factors of CC in non-responders to initial therapy who needed additional IVIG infusion among patients with KD. METHODS: Forty five non-responders to initial IVIG who got additional IVIG infusion were reviewed from 1996 to 2007. We devided patients into two groups with CC (group A, n=17) or without CC (group B, n=28). Clinical characteristics, timing of additional IVIG infusion and laboratory results were reviewed. And we investigated the differences between the two groups and tried to find risk factors of CC. RESULTS: In comparison between the two groups, CC, clinical characteristics and timing of IVIG infusion were not different between two groups. But, total febrile days were significantly longer and peak platelets counts were significantly higher in group A (P=0.006, P=0.013). On the logistic regression analysis, total febrile days longer than 10.5 days was the only risk factor of CC in these patients. CONCLUSION: Our results showed that patients with CC inspite of repeated IVIG therapy had longer fever duration. So, additional therapy besides re-treatment with IVIG aiming at shortening total duration of fever seems to be important in refractory KD to prevent CC.

Congenital central hypoventilation syndrome combined with Hirschsprung disease diagnosed in the neonatal period / 소아과

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

Comparison of Intervals to Peak Values of Laboratory Findings in Patients with Coronary Complication to Those without Complication in Kawasaki Disease

PURPOSE: Intravenous immune globulin(IVIG) as a treatment for the Kawasaki disease (KD) has reduced the coronary complications. But, some patients suffer from coronary complication despite early IVIG infusion, and it is difficult to discriminate the susceptible patients in the acute phase. It is also challenging to decide additional therapy in cases showing fever after IVIG therapy. We investigated the relationship between intervals from the onset of fever to the day of peak laboratory values and coronary complications. METHODS: We reviewed the charts of KD patients with coronary aneurysm(group A, n=13) and without aneurysm(group B, n=35). All patients got IVIG therapy early in the acute phase and additional therapy in cases fever recurred. We counted the days from onset of fever to the peak level of acute phase reactants and analyzed the differences between two groups with t-test. RESULTS: In the comparison of two groups, the mean intervals from the onset of fever to peak CRP level was 9.23+/-4.71 days in group A, 6.63+/-2.47 days in group B. The mean intervals to peak ESR was 13.31+/-7.06 days in group A, 8.37+/-3.01 days in group B. The mean intervals to highest platelets counts was 14.62+/-4.96 days in group A, 11.14+/-3.59 days in group B. All of these results showed statistically significant differences. CONCLUSION: Our results show that the KD patients with coronary aneurysm have longer intervals between the onset of fever to day of peak acute reactants in spite of the aggressive treatment than those without aneurysm. So, in cases of KD with relapsing fever in spite of IVIG and the acute reactants are in the course of increment, additional immune modulation therapy and short term follow ups with echocardiography would be needed.

The Levels of IgG Subclasses and Vaccine-induced Viral Antibodies in Childhood Minimal Change Nephrotic Syndrome

PURPOSE: Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). METHODS: Using the stored sera, the levels of immunoglobulin(IgG, IgM, IgA, and IgE) and IgG subclasses(IgG 1, 2, 3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. RESULTS: The mean values of IgG and IgG1 were 390+/-187 mg/dL and 287+/-120 mg/dL in nephrotic children, and 1,025+/-284 mg/dL and 785+/-19 mg/dL in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM(251+/-183 mg/dL vs. 153+/-55 mg/dL, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9%(9 of 21 cases) in the MCNS group and 52%(13/25) in the control group, and that of anti-measles IgG was 76%(16/21) and 92%(23/25), respectively, but there was no statistical difference between the two groups. CONCLUSION: IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS.