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1.
Medicina (Kaunas) ; 57(12)2021 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-34946330

RESUMEN

Background and Objectives: Considering developing resistance against neuraminidase inhibitors (NAIs) and their adverse reactions, restricted use of NAIs and use of alternative drugs should be considered for treating influenza. Although glucocorticoids (GCs) have been used for severe influenza, their effects on non-severe influenza have rarely been evaluated. This study aimed to evaluate the clinical responses to NAI therapy and GC therapy in pediatric patients with non-severe influenza. Materials and Methods: A total of 601 pediatric patients (<19 years of age) diagnosed with non-severe influenza were retrospectively recruited to evaluate the effects of NAI therapy and GC therapy. Post-admission fever duration and hospitalization duration were compared among four patient groups divided by the administered treatment: No therapy (n = 52), NAI therapy (n = 154), GC therapy (n = 123), and Both therapies (n = 272). Results: In a multivariate analysis with adjustment for confounding variables, the post-admission fever duration was not significantly different among the four patient groups. The post-admission fever duration tended to shorten with increasing age, longer pre-admission fever duration, and incidence of influenza A virus infection and lower respiratory tract infection. The type of administered treatment showed no significant effects on the post-admission fever duration in any subgroups according to patient age, pre-admission fever duration, influenza virus subtype, and clinical diagnosis. Conclusions: Symptomatic treatment rather than antiviral or GC therapy seems to be sufficient for patients with non-severe influenza, although the effects of NAI therapy and GC therapy according to their administered time and dose should be further evaluated.


Asunto(s)
Antivirales , Glucocorticoides , Gripe Humana , Antivirales/uso terapéutico , Niño , Inhibidores Enzimáticos/uso terapéutico , Glucocorticoides/uso terapéutico , Humanos , Gripe Humana/tratamiento farmacológico , Gripe Humana/epidemiología , Neuraminidasa/antagonistas & inhibidores , Estudios Retrospectivos
3.
BMC Pediatr ; 18(1): 201, 2018 06 23.
Artículo en Inglés | MEDLINE | ID: mdl-29935535

RESUMEN

BACKGROUND: We aimed to compare the antipyretic efficacy, safety, and tolerability between oral dexibuprofen and intravenous propacetamol in children with upper respiratory tract infection (URTI) presenting with fever. METHODS: Patients aging from 6 months to 14 years admitted for URTI with axillary body temperature ≥ 38.0 °C were enrolled and randomized into the study or control group. Patients in the study group were intravenously infused with propacetamol and subsequently oral placebo medication was administered. Patients in the control group were intravenously infused with 100 mL of 0.9% sodium chloride solution without propacetamol and then oral dexibuprofen was administered. We checked the body temperature of all patients at 0.5 h (hr), 1 h, 1.5 h, 2 h, 3 h, 4 h, and 6 h after oral placebo or dexibuprofen had been applied. RESULTS: A total of 263 patients (125 in the study group) were finally enrolled. The body temperatures of patients in the study group were significantly lower until 2 h after administration (37.73 ± 0.58 vs 38.36 ± 0.69 °C (p < 0.001), 37.37 ± 0.53 vs 37.88 ± 0.69 °C (p < 0.001), 37.27 ± 0.60 vs 37.62 ± 0.66 °C (p < 0.001), 37.25 ± 0.62 vs 37.40 ± 0.60 °C (p = 0.0452), at 0.5 h, 1 h, 1.5 h, and 2 h, respectively). The two groups showed no significant differences in terms of the range of body temperature decrease, the Area Under the Curve of body temperature change for antipyretic administration-and-time relationship, the maximum value of body temperature decrease during the 6 h test period, the number of patients whose body temperature normalized (< 37.0 °C), the mean time when first normalization of body temperature, and the development of adverse events including gastrointestinal problem, elevated liver enzyme, and thrombocytopenia. CONCLUSIONS: Intravenous propacetamol may be a safe and effective choice for pediatric URTI patients presenting with fever who are not able to take oral medications or need faster fever control. TRIAL REGISTRATION: CRIS KCT0002888 . Date of registration: July 31st, 2013.


Asunto(s)
Acetaminofén/análogos & derivados , Antipiréticos/uso terapéutico , Fiebre/tratamiento farmacológico , Ibuprofeno/análogos & derivados , Infecciones del Sistema Respiratorio/tratamiento farmacológico , Acetaminofén/efectos adversos , Acetaminofén/uso terapéutico , Administración Oral , Adolescente , Antipiréticos/efectos adversos , Niño , Preescolar , Método Doble Ciego , Femenino , Fiebre/microbiología , Humanos , Ibuprofeno/efectos adversos , Ibuprofeno/uso terapéutico , Lactante , Infusiones Intravenosas , Masculino
4.
J Korean Med Sci ; 30(10): 1471-5, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26425045

RESUMEN

Rotavirus (RV) is one of the most important viral etiologic agents of acute gastroenteritis (AGE) in children. Although effective RV vaccines (RVVs) are now used worldwide, novel genotypes and outbreaks resulting from rare genotype combinations have emerged. This study documented RV genotypes in a Korean population of children with AGE 5 yr after the introduction of RVV and assessed potential genotype differences based on vaccination status or vaccine type. Children less than 5-yr-old diagnosed with AGE between October 2012 and September 2013 admitted to 9 medical institutions from 8 provinces in Korea were prospectively enrolled. Stool samples were tested for RV by enzyme immunoassay and genotyped by multiplex reverse-transcription polymerase chain reaction. In 346 patients, 114 (32.9%) were RV-positive. Among them, 87 (76.3%) patients were infected with RV alone. Eighty-six of 114 RV-positive stool samples were successfully genotyped, and their combinations of genotypes were G1P[8] (36, 41.9%), G2P[4] (12, 14.0%), and G3P[8] (6, 7.0%). RV was detected in 27.8% of patients in the vaccinated group and 39.8% in the unvaccinated group (P=0.035). Vaccination history was available for 67 of 86 cases with successfully genotyped RV-positive stool samples; RotaTeq (20, 29.9%), Rotarix (7, 10.4%), unvaccinated (40, 59.7%). The incidence of RV AGE is lower in the RV-vaccinated group compared to the unvaccinated group with no evidence of substitution with unusual genotype combinations.


Asunto(s)
Vacunación Masiva , Infecciones por Rotavirus/prevención & control , Vacunas contra Rotavirus/inmunología , Rotavirus/clasificación , Rotavirus/genética , Preescolar , Heces/virología , Gastroenteritis/inmunología , Gastroenteritis/prevención & control , Gastroenteritis/virología , Genotipo , Humanos , Lactante , ARN Viral/genética , República de Corea , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Rotavirus/aislamiento & purificación , Infecciones por Rotavirus/inmunología , Infecciones por Rotavirus/virología , Vacunas Atenuadas/inmunología
5.
Immunol Invest ; 43(6): 585-94, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24999735

RESUMEN

Chronic granulomatous disease (CGD) is a rare hereditary disorder that is characterized by a greatly increased susceptibility to life-threatening bacterial and fungal infections. CGD is caused by mutations in any one of the genes encoding subunits of phagocyte NADPH oxidase. X-linked CGD, more than half of all CGD cases, is caused by mutations in CYBB gene encoding gp91-phox subunit. We identified the mutations in the CYBB gene of 29 Korean patients with X-linked CGD from 26 unrelated families. Twenty-three mutations were identified: five splice site mutations (c.45 + 1G > C, c.141 + 5G > A, c.897 + 2T > C c.1461 + 1G > T, c.1586 + 2T > A), four frameshift mutations (c.27dupG, [c.737A > C; c.742delA], c.742dupA, c.1636 del C), seven non-sense mutations (c217C > T, c.469C > T, c.676C > T, c.868C > T, c.1222G > T, c.1272G > A, c.1281T > A), five missense mutations (c.164 C > A, c.422T > C, c.665 A > G, c.1012C > T, c.1461G > T) and two gross deletions. Eight out of 23 mutations identified in this study are novel mutations: two splice mutations(c.897 + 2T > C, c.1586 + 2T > A), two frame shift mutations ([c.737A > C; c.742delA], c.1636 del C), two nonsense mutations (c.1222G > T, c.1281T > A), one missense mutation (c.1461G > T), one gross deletion (c.1667_1629 del.). Our results confirmed that mutations of CYBB gene in the X-CGD are very heterogeneous and not show the peculiarity of the ethnic group.


Asunto(s)
Enfermedad Granulomatosa Crónica/genética , Glicoproteínas de Membrana/genética , NADPH Oxidasas/genética , Fagocitos/fisiología , Análisis Mutacional de ADN , Familia , Femenino , Humanos , Corea (Geográfico) , Masculino , Mutación/genética , NADPH Oxidasa 2
6.
Children (Basel) ; 11(7)2024 Jun 21.
Artículo en Inglés | MEDLINE | ID: mdl-39062205

RESUMEN

Macrophage activation syndrome (MAS) is potentially fatal; so, early diagnosis and timely treatment are essential. However, detecting MAS is sometimes challenging because its principal features can be observed in other pediatric diseases that cause severe inflammation. Cytokine storm due to immune dysregulation represents the clinical and laboratory features of MAS that are included in the diagnostic criteria. Most cases of MAS occur as an underlying condition worsens and progresses. Therefore, a patient with autoimmune or autoinflammatory disease who shows unexplained clinical deterioration despite appropriate management should be considered at high risk for MAS (i.e., occult MAS). The basic principles of treatment are control of triggering factors, supportive care, and relief of hyperinflammation. Systemic steroids and cyclosporine A are frequently used as a first-line treatment. For the treatment of refractory MAS, cytokine-specific biologic agents such as anakinra have recently become preferred over traditional immunosuppressive agents such as etoposide. MAS might be underrecognized in pediatric patients with infectious and inflammatory diseases due to its diverse clinical presentations. Clinical suspicion of MAS is of the utmost importance for early recognition of the disease.

7.
J Rheum Dis ; 31(1): 49-53, 2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-38130956

RESUMEN

Neonatal lupus can occur in infants born to mother with autoimmune disorders through transplacental auto-antibodies. Clinical manifestations in neonatal lupus include cutaneous lesions and hematologic or hepatobiliary findings resembling those seen in systemic lupus erythematosus. In autoimmune state, macrophage activation syndrome (MAS) represent a critical and potentially fatal complication that can result in mortality if not immediately identified and managed with the appropriate care. Here we present a 33-day-old girl diagnosed with neonatal lupus and serious MAS. She was delivered by a primipara mother who did not exhibit any autoimmune symptoms. The patient visited the hospital due to fever and pancytopenia. Laboratory data were compatible with MAS, including pancytopenia, high level of ferritin, soluble interleukin-2, and decreased natural killer cell activity. In addition, autoimmune study showed positive results for anti-nuclear antibody (ANA), anti-Sjogren syndrome antigen A (SSA), and SSB, The autoimmune study for mother also showed positive results for ANA, anti-SSA, and SSB. The patient recovered after she received high dose steroid and supportive care. Our case indicates that neonatal lupus should be taken into consideration when fever, erythematous skin rash, and pancytopenia are observed in infants, even if their mothers have no prior history of autoimmune conditions.

8.
J Korean Med Sci ; 28(4): 586-92, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23579367

RESUMEN

This phase II clinical trial was conducted to compare the immunogenicity and safety of a newly developed tetanus-reduced diphtheria (Td) vaccine (GC1107-T5.0 and GC1107-T7.5) and control vaccine. This study was also performed to select the proper dose of tetanus toxoid in the new Td vaccines. Healthy adolescents aged between 11 and 12 yr participated in this study. A total of 130 subjects (44 GC1107-T5.0, 42 GC1107-T7.5 and 44 control vaccine) completed a single dose of vaccination. Blood samples were collected from the subjects before and 4 weeks after the vaccination. In this study, all subjects (100%) in both GC1107-T5.0 and GC1107-T7.5 groups showed seroprotective antibody levels (≥ 0.1 U/mL) against diphtheria or tetanus toxoids. After the vaccination, the geometric mean titer (GMT) against diphtheria was significantly higher in Group GC1107-T5.0 (6.53) and GC1107-T7.5 (6.11) than in the control group (3.96). The GMT against tetanus was 18.6 in Group GC1107-T5.0, 19.94 in GC1107-T7.5 and 19.01 in the control group after the vaccination. In this study, the rates of local adverse reactions were 67.3% and 59.1% in GC1107-T5.0 and GC1107-7.5, respectively. No significant differences in the number of adverse reactions, prevalence and degree of severity of the solicited and unsolicited adverse reactions were observed among the three groups. Thus, both newly developed Td vaccines appear to be safe and show good immunogenicity. GC1107-T5.0, which contains relatively small amounts of tetanus toxoid, has been selected for a phase III clinical trial.


Asunto(s)
Vacuna contra Difteria y Tétanos/inmunología , Difteria/prevención & control , Tétanos/prevención & control , Anticuerpos Antibacterianos/sangre , Artralgia/etiología , Niño , Vacuna contra Difteria y Tétanos/efectos adversos , Método Doble Ciego , Femenino , Cefalea/etiología , Humanos , Masculino , Dolor/etiología , Resultado del Tratamiento , Vacunación
9.
J Rheum Dis ; 30(4): 272-277, 2023 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-37736588

RESUMEN

Multisystem inflammatory syndrome in children (MIS-C) is a serious post-infectious complication of COVID-19 characterized by hyperinflammation and multi-organ dysfunction including shock. Shock is also seen in a severe form of Kawasaki disease (KD) called KD shock syndrome (KDSS). Here, we present one MIS-C and one KDSS case and compare similarities and differences between them. Both MIS-C (case 1) and KDSS (case 2) showed hyperinflammation, KD-related features, gastrointestinal problems, hypotension, and coagulopathy. The extent of systemic inflammation and organ dysfunction was more severe in KDSS than in MIS-C. Case 1 was diagnosed as MIS-C because SARS-CoV-2 was confirmed, and case 2 was diagnosed as KDSS because no pathogen was identified in microbiological studies. We believe that the most important difference between MIS-C and KDSS was whether SARS-CoV-2 was identified as an infectious trigger. Organ dysfunction is a hallmark of MIS-C and KDSS, but not KD, so MIS-C shares more clinical phenotypes with KDSS than with KD. Comparison of MIS-C and KDSS will be an interesting and important topic in the field of KD-like hyperinflammatory disease research.

10.
Children (Basel) ; 10(9)2023 Sep 08.
Artículo en Inglés | MEDLINE | ID: mdl-37761488

RESUMEN

This study aimed to investigate the characteristics of COVID-19-associated multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease shock syndrome (KDSS) and to compare the similarities and differences between the two diseases. The incidence of KDSS and MIS-C was also estimated. Medical records of patients diagnosed with MIS-C or KDSS at four hospitals from January 2013 to December 2022 were retrospectively reviewed. Thirty-one patients were enrolled in the study in either an MIS-C group (n = 22) or a KDSS group (n = 9). The incidence of KDSS in KD was 0.8% (9/1095) and the incidence of MIS-C versus KD was 10.2% (22/216). Compared with the MIS-C group, the KDSS group had longer hospital stays and more severe systemic inflammation (e.g., anemia, elevated C-reactive protein, hypoalbuminemia, and pyuria) and organ dysfunction (e.g., number of involved organs, shock, vasoactive infusion, and intensive care unit admission). All patients in the MIS-C group, but none in the KDSS group, including two patients during the COVID-19 pandemic, had laboratory evidence of SARS-CoV-2 infection. MIS-C and KDSS shared demographic, clinical, and laboratory characteristics; organ dysfunction; treatment; and outcomes. Overall severity was more severe in patients with KDSS than in those with MIS-C. The most important difference between MIS-C and KDSS was whether SARS-CoV-2 was identified as an infectious trigger.

11.
Mol Ther ; 19(11): 2092-101, 2011 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-21878903

RESUMEN

X-linked chronic granulomatous disease (CGD) is an inherited immunodeficiency caused by a defect in the gp91(phox) gene. In an effort to treat X-CGD, we investigated the safety and efficacy of gene therapy using a retroviral vector, MT-gp91. Two X-CGD patients received autologous CD34(+) cells transduced with MT-gp91 after a conditioning regimen consisting of fludarabine and busulfan. The level of gene-marked cells was highest at day 21 (8.3 and 11.7% in peripheral blood cells) but decreased to 0.08 and 0.5%, respectively, 3 years after gene transfer. The level of functionally corrected cells, as determined by nicotinamide adenine dinucleotide phosphate (NADPH) oxidase assay, reached a peak at day 17 (6.5% patient 1 (P1) and 14.3% patient 2 (P2) of total granulocytes) and declined to 0.05% (P1) and 0.21% (P2), 3 years later. Some retroviral vectors were found to have integrated within or close to the proto-oncogenes MDS1-EVI1, PRDM16, and CCND2; however, no abnormal cell expansion or related hematological malignancy was observed. Overall, the gene transfer procedure did not produce any serious adverse effects and was able to convert a significant fraction of blood cells to biologically functional cells, albeit for a short period of time.


Asunto(s)
Terapia Genética , Vectores Genéticos , Enfermedad Granulomatosa Crónica/terapia , Retroviridae/genética , Adolescente , Niño , Perfilación de la Expresión Génica , Vectores Genéticos/efectos adversos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Células Madre Hematopoyéticas/metabolismo , Humanos , Masculino , Glicoproteínas de Membrana/genética , NADPH Oxidasa 2 , NADPH Oxidasas/genética , Transducción Genética , Resultado del Tratamiento , Integración Viral
12.
J Korean Med Sci ; 27(7): 788-93, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22787376

RESUMEN

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152 patients with PID (under 19 yr of age), who were observed from 2001 to 2005, have been entered in this registry. The period prevalence of PID in Korea in 2005 is 11.25 per million children. The following frequencies were found: antibody deficiencies, 53.3% (n = 81), phagocytic disorders, 28.9% (n = 44); combined immunodeficiencies, 13.2% (n = 20); and T cell deficiencies, 4.6% (n = 7). Congenital agammaglobulinemia (n = 21) and selective IgA deficiency (n = 21) were the most frequently reported antibody deficiency. Other reported deficiencies were common variable immunodeficiencies (n = 16), X-linked agammaglobulinemia (n = 15), IgG subclass deficiency (n = 4). Phagocytic disorder was mostly chronic granulomatous disease. A small number of patients with Wiskott-Aldrich syndrome, hyper-IgE syndrome, and severe combined immunodeficiency were also registered. Overall, the most common first manifestation was pneumonia. This study provides data that permit a more accurate estimation PID patients in Korea.


Asunto(s)
Síndromes de Inmunodeficiencia/epidemiología , Adolescente , Agammaglobulinemia/congénito , Agammaglobulinemia/epidemiología , Distribución por Edad , Niño , Preescolar , Inmunodeficiencia Variable Común/epidemiología , Femenino , Enfermedades Genéticas Ligadas al Cromosoma X/epidemiología , Humanos , Deficiencia de IgA/epidemiología , Deficiencia de IgG/epidemiología , Lactante , Recién Nacido , Síndrome de Job/epidemiología , Masculino , Prevalencia , Sistema de Registros , República de Corea/epidemiología , Inmunodeficiencia Combinada Grave/epidemiología , Distribución por Sexo , Encuestas y Cuestionarios , Síndrome de Wiskott-Aldrich/epidemiología , Adulto Joven
13.
J Rheum Dis ; 29(1): 14-21, 2022 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-37476697

RESUMEN

Juvenile dermatomyositis (JDM) is a systemic capillary vasculopathy. Patients present with proximal muscle weakness, raised muscle enzymes, and pathognomic skin rashes such as heliotrope rash, Gottron's papules. Main complications are calcinosis, lipodystrophy, osteoporosis. Complement-mediated damage of vessels is a major mechanism. Magnetic resonance imaging is currently widely used to diagnosis of JDM. The goals of treatment are to control inflammatory myositis and prevent disease complication. Early, aggressive treatment of JDM associated with a better prognosis. High-dose corticosteroids in combination with methotrexate is the mainstay of treatment. The course of JDM is variable.

14.
Clin Exp Pediatr ; 65(4): 153-166, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34809418

RESUMEN

During the coronavirus disease 2019 (COVID-19) pandemic, a novel multisystem inflammatory syndrome in children (MIS-C) has been reported worldwide since the first cases were reported in Europe in April 2020. MIS-C is temporally associated with severe acute respiratory syndrome coronavirus 2 infection and shows Kawasaki disease (KD)-like features. The epidemiological and clinical characteristics in COVID-19, KD, and MIS-C differ, but severe cases of each disease share similar clinical and laboratory findings such as a protracted clinical course, multiorgan involvement, and similar activated biomarkers. These findings suggest that a common control system of the host may act against severe disease insult. To solve the enigmas, we proposed the protein-homeostasis-system hypothesis in that every disease involves etiological substances and the host's immune system controls them by their size and biochemical properties. Also, it is proposed that the etiological agents of KD and MIS-C might be certain strains in the microbiota of human species and etiological substances in severe COVID-19, KD, and MIS-C originate from pathogen-infected cells. Since disease severity depends on the amounts of inflammation-inducing substances and corresponding immune activation in the early stage of the disease, an early proper dose of corticosteroids and/or intravenous immunoglobulin (IVIG) may help reduce morbidity and possibly mortality among patients with these diseases. Corticosteroids are low cost and an analogue of host-origin cortisol among immune modulators. This study's findings will help clinicians treating severe COVID-19, KD, and MIS-C, especially in developing countries, where IVIG and biologics supplies are insufficient.

15.
Paediatr Drugs ; 24(6): 689-697, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-36156791

RESUMEN

BACKGROUND: Intravenous immunoglobulin (IVIG) resistance in patients with Kawasaki disease (KD) is defined as persistent or recrudescent fever ≥36 hours after IVIG infusion. We have experienced an increase in IVIG resistance in patients with KD since the substitution of 10% IVIG for 5% IVIG. This study aimed to determine the independent association between increased IVIG resistance and 10% IVIG therapy. METHODS: Medical records of pediatric patients with KD were retrospectively reviewed. Clinical and laboratory characteristics were compared between patients receiving 5% IVIG therapy and those receiving 10% IVIG therapy. Between IVIG-responsive and IVIG-resistant patients, a multivariate analysis was performed to determine the independent factors for IVIG resistance. RESULTS: A total of 119 patients were included in this study: 81 (68.1%) and 38 (31.9%) patients received 5% and 10% IVIG therapy, respectively. IVIG resistance was identified in 34 (28.6%) patients: 44.7% of patients receiving 10% IVIG therapy and 21.0% of patients receiving 5% IVIG therapy (p = 0.008). The clinical manifestations and outcomes were comparable between patients who received 5% IVIG therapy and those who received 10% IVIG therapy. IVIG resistance was significantly associated with fewer fever days at IVIG administration (p = 0.032), a higher percentage of neutrophils (p = 0.013), and 10% IVIG treatment (p = 0.004) in the multivariate analysis. CONCLUSION: 10% IVIG therapy was significantly associated with increased reporting of IVIG resistance. However, the increase in patients with fever patterns consistent with IVIG resistance seemed to represent adverse febrile reactions resulting from using high-concentration IVIG rather than increased severity of KD.


Asunto(s)
Síndrome Mucocutáneo Linfonodular , Humanos , Niño , Lactante , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Inmunoglobulinas Intravenosas/efectos adversos , Estudios Retrospectivos , Fiebre/tratamiento farmacológico
16.
Children (Basel) ; 9(10)2022 Oct 20.
Artículo en Inglés | MEDLINE | ID: mdl-36291524

RESUMEN

Recognition of macrophage activation syndrome (MAS) in patients with refractory Kawasaki disease (KD) can be challenging. This study aimed to investigate the incidence of MAS in patients with refractory KD and to compare the characteristics of refractory KD and MAS. Medical records of 468 patients diagnosed with KD from January 2010 to December 2019 were retrospectively reviewed. Of the 468 KD patients, 63 were enrolled in the study as a refractory KD group (n = 59) and an MAS group (n = 4). The incidence of MAS was 0.8% (4/468) in patients with KD and 6.3% (4/63) in patients with refractory KD. Compared to the refractory KD group, the MAS group had higher frequencies of incomplete KD, hepatosplenomegaly, third-line treatment, and MAS screening, and showed lower levels of albumin. No significant differences were found in other clinical and laboratory findings. In addition to four patients with MAS, five patients with refractory KD who received third-line treatment showed severe systemic inflammation and organ dysfunction, but only one in five patients underwent MAS screening, including ferritin levels. In conclusion, given the relatively high incidence of MAS in children with refractory KD and the similar phenotype between refractory KD and MAS, we propose that MAS screening should be included in routine laboratory tests for refractory KD.

17.
Pediatr Rheumatol Online J ; 20(1): 7, 2022 Feb 02.
Artículo en Inglés | MEDLINE | ID: mdl-35109877

RESUMEN

BACKGROUND: In women with autoimmune rheumatic disorders (ARD), pregnancy complications or postpartum events are more frequent compared to the general population. Transplacental autoantibodies or cytokines influence various fetal and neonatal outcomes. We compared the growth patterns of babies born to mothers with ARD versus healthy mothers to assess the long-term growth outcomes of children born to women with ARD. METHODS: This was a retrospective age-matched cohort analyses of babies born to mothers with ARD from the hospitals belonging to the Catholic University of Korea between 2010 and 2017. Demographic and autoimmune laboratory test data of the mothers and newborns were assessed. Neonatal growth was measured in terms of height and weight, measured at birth and follow-up examinations. RESULTS: We enrolled 142 infants from mothers with ARD and 149 infants from healthy mothers. There was no significant difference between mothers with ARD and healthy mothers in terms of delivery age, parity, abortion, and premature delivery history. The mothers with ARD were diagnosed with systemic lupus erythematosus (81%), Sjogren syndrome (6%), and other autoimmune phenomena (11%). The groups were significantly different in terms of neonatal characteristics such as prematurity, gestational age, birth weight, and height, but not in Apgar score and delivery type. For most neonates, autoimmune laboratory results were normalized within 1 year, except for anti-La/SSB antibody, which remained high in some. The height and weight for age z-score were lower than the normal age groups at birth but showed catch-up growth by 2 years of age. CONCLUSIONS: Low birthweight and prematurity at birth for neonates born to mothers with ARD could be caught up by 2 years of age, and maternal ARD does not affect the growth of their offspring.


Asunto(s)
Desarrollo Infantil , Trastornos del Crecimiento/etiología , Recién Nacido de Bajo Peso , Enfermedades del Prematuro/etiología , Complicaciones del Embarazo/etiología , Enfermedades Reumáticas/complicaciones , Adulto , Estudios de Cohortes , Femenino , Humanos , Recién Nacido , Masculino , Embarazo , Estudios Retrospectivos
18.
J Rheum Dis ; 29(1): 52-55, 2022 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-37476696

RESUMEN

Ischemic vaso-occlusive retinopathy as an initial manifestation is rare in pediatric systemic lupus erythematosus (pSLE). A 13-year-old girl presented with two months' history of papules and crusts with fatigue, weight loss, and abrupt hair loss. Pancytopenia and findings compatible with SLE, including positive direct Coombs' test, antinuclear antibody (Ab), anti-double stranded DNA Ab, anti-Smith Ab, anti-ribonucleoprotein Ab, lupus anticoagulant, anti-ß2 glycoprotein Immunoglobulin G, and anti-cardiolipin Ab, were detected. Bi-nasal hemianopsia was detected. Initial visual acuity was hand motion in the right eye and 15/20 in the left. Fundoscopy showed massive exudation around the optic disc with macular edema, vascular sheathing with perivascular hemorrhage in the whole retina, and ghost vessels in the peripheral retina. Intravitreal triamcinolone injection and dexamethasone implant injection were administered. Visual symptoms improved but did not recover. Methylprednisolone therapy and photocoagulation improved visual acuity and fever. Early intervention for retinopathy in pSLE can help prevent vision-loss.

19.
BMC Infect Dis ; 11: 225, 2011 Aug 24.
Artículo en Inglés | MEDLINE | ID: mdl-21864391

RESUMEN

BACKGROUND: There was a pandemic influenza around the world in 2009 including South Korea since last pandemic occurred four decades ago. We aimed to evaluate the epidemiological and clinical characteristics of this infection in childhood. METHODS: We evaluated the epidemiologic characteristics of all the subjects infected with the 2009 H1N1 influenza A virus (2,971 patients, ≤ 15 years of age), and the clinical and laboratory findings of the inpatients (217 patients, 80 had pneumonia) between 1 September 2009 and 31 January 2010 in a single hospital throughout the epidemic. RESULTS: The age distribution of all the subjects was relatively even. Over 90% of cases occurred during a two-month period. Two hundred and five patients (94.5%) received oseltamivir within 48 h of fever onset, and 97% of inpatients defervesced within 48 h of medication. The group with pneumonia included more males than females, and had higher leukocytes counts with lower lymphocyte differentials than the group without pneumonia. The white blood cell count and lymphocyte differential were associated with the severity of pneumonia. Corticosteroid treatment for severe pneumonia patients was highly effective in preventing disease progression. CONCLUSION: Children of all ages affected with even rates of infection, but males were predominant in pneumonia patients. Pneumonia patients showed lymphopenia and its severity was associated with the severity of illness. Our results suggest that the mechanism of lung injury in 2009 H1N1 virus infection may be associated with the host immune response.


Asunto(s)
Subtipo H1N1 del Virus de la Influenza A/aislamiento & purificación , Gripe Humana/epidemiología , Gripe Humana/patología , Pandemias , Adolescente , Distribución por Edad , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Recién Nacido , Gripe Humana/complicaciones , Gripe Humana/virología , Masculino , Neumonía/epidemiología , Neumonía/inmunología , Neumonía/patología , Prevalencia , República de Corea/epidemiología , Índice de Severidad de la Enfermedad , Factores Sexuales
20.
Children (Basel) ; 8(7)2021 Jun 24.
Artículo en Inglés | MEDLINE | ID: mdl-34202436

RESUMEN

This study aimed to determine the subjects for bacterial multiplex polymerase chain reaction (mPCR) testing and to interpret the mPCR test results based on patients' clinical symptoms and diagnoses. The medical records of 710 pediatric patients who underwent a bacterial mPCR test were retrospectively reviewed. Clinical characteristics and mPCR test results were compared between patients with positive (n = 199) and negative mPCR test results (n = 511) and between patients with invasive pathogens (n = 95) and toxigenic pathogens (n = 70). Positive mPCR test results were significantly associated with older age (p < 0.001), diagnosis of acute gastroenteritis (p = 0.021), presence of hematochezia (p < 0.001), and absence of cough (p = 0.004). The diagnosis of acute gastroenteritis (p = 0.003), presence of fever (p = 0.027) and diarrhea (p = 0.043), and higher C-reactive protein levels (p = 0.025) were significantly associated with the identification of invasive pathogens in patients with positive mPCR test results. Thus, selective bacterial mPCR testing should be performed based on the patients' clinical symptoms and diagnoses, and the results should be interpreted in consideration with identified pathogens.

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