RESUMO
We reviewed the medical records of five patients with T-B+NK- severe combined immunodeficiency (SCID) who received minimal dose allogeneic hematopoietic cell transplantation (HCT) (total nucleated cell count (TNC) lower than 1.0 × 108/kg). Patients were administered a median of 5.0 mL of bone marrow or peripheral blood without conditioning (in four) or with anti-thymocyte globulin alone (in one). Three patients received HCT from a matched sibling donor, one from unrelated donor, and one from familial mismatched donor. The median TNC and CD34+ cells were 0.54 (0.29-0.84) × 108/kg and 0.61 (0.35-0.84) × 106/kg, respectively. Engraftment was achieved in all. Total T cell, CD4+ cell, and CD8+ cell recovery was obtained within a year in four, and immunoglobulin replacement was discontinued in all. All patients survived, exhibiting stable donor chimerism. We obtained sufficient therapeutic effects with minimal dose transplantation without intensive conditioning in patients with T-B+NK- SCID.
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Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Imunodeficiência Combinada Severa , Humanos , Imunodeficiência Combinada Severa/terapia , Condicionamento Pré-Transplante , Linfócitos T CD4-Positivos , Células Matadoras NaturaisRESUMO
The gastrointestinal (GI) tract is frequently affected by inborn errors of immunity (IEI), and GI manifestations can be present in IEI patients before a diagnosis is confirmed. We aimed to investigate clinical features, endoscopic and histopathologic findings in IEI patients. This was a retrospective cohort study conducted from 1995 to 2020. Eligible patients were diagnosed with IEI and had GI manifestations that were enough to require endoscopies. IEI was classified according to the International Union of Immunological Societies classification. Of 165 patients with IEI, 55 (33.3%) had GI manifestations, and 19 (11.5%) underwent endoscopy. Among those 19 patients, nine (47.4%) initially presented with GI manifestations. Thirteen patients (68.4%) were male, and the mean age of patients 11.5 ± 7.9 years (range, 0.6 - 26.6) when they were consulted and evaluated with endoscopy. The most common type of IEI with severe GI symptoms was "Disease of immune dysregulation" (31.6%) followed by "Phagocyte defects" (26.3%), according to the International Union of Immunological Societies classification criteria. Patients had variable GI symptoms such as chronic diarrhea (68.4%), hematochezia (36.8%), abdominal pain (31.6%), perianal disease (10.5%), and recurrent oral ulcers (10.5%). During the follow-up period, three patients developed GI tract neoplasms (early gastric carcinoma, mucosa associated lymphoid tissue lymphoma of colon, and colonic tubular adenoma, 15.8%), and 12 patients (63.2%) were diagnosed with inflammatory bowel disease (IBD)-like colitis. Investigating immunodeficiency in patients with atypical GI symptoms can provide an opportunity for correct diagnosis and appropriate disease-specific therapy. Gastroenterologists and immunologists should consider endoscopy when atypical GI manifestations appear in IEI patients to determine if IBD-like colitis or neoplasms including premalignant and malignant lesions have developed. Also, if physicians in various fields are better educated about IEI-specific complications, early diagnosis and disease-specific treatment for IEI will be made possible.
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Gastroenteropatias/etiologia , Síndromes de Imunodeficiência/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Gastroenteropatias/patologia , Humanos , Síndromes de Imunodeficiência/patologia , Lactente , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Prenatal maternal diet may influence disease susceptibility in offspring with specific genetic backgrounds. We hypothesized that interactions between prenatal antioxidant intake and polymorphisms in immunity genes influence respiratory tract infection (RTI) susceptibility in infants at 12 months of age. METHODS: This study included 550 infants. In the Cohort for Childhood Origin of Asthma and Allergic Diseases (COCOA) birth cohort study, prenatal maternal diet was assessed by administering a food frequency questionnaire. Infants' cord blood was genotyped for CD14 (rs2569190), TLR4 (rs1927911), and GSDMB (rs4794820) polymorphisms by the TaqMan method. RESULTS: Higher prenatal intake of total fruit and vegetables (FV) was associated with the decreased risk of RTI in offspring (P-trend=0.0430). In children with TT genotype at rs2569190, a higher prenatal intake of vitamins A and C, fruits, and total FV decreased RTI risk (P-trend <0.05), while in infants with TC+CC genotype, a higher prenatal intake of fruit increased RTI risk (P-trend <0.05). When analyzing the 3 genotypes, children with TT genotype at rs2569190 were more protected against RTIs compared with those with CC genotype with respect to vitamin C and fruits [odds ratio (OR)=5.04 and OR=10.30, respectively]. In children with CC genotype at rs1927911, RTI risk showed a dose-response association with a higher prenatal intake of vitamin C (P for interaction<0.05). A higher prenatal intake of fruits and total FV reduced RTI risk in infants with GA+AA genotype of rs4794820 (P for interaction<0.05). CONCLUSIONS: Prenatal antioxidant intake may reduce RTI risk in infants and this relationship may be modified by CD14, TLR4, and GSDMB polymorphisms.
Assuntos
Antioxidantes/administração & dosagem , Predisposição Genética para Doença , Recém-Nascido Prematuro , Receptores de Lipopolissacarídeos/genética , Infecções Respiratórias/prevenção & controle , Adulto , Dieta , Feminino , Seguimentos , Idade Gestacional , Humanos , Incidência , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Exposição Materna , Polimorfismo Genético , Gravidez , Estudos Prospectivos , Valores de Referência , República da Coreia , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/genética , Medição de RiscoRESUMO
BACKGROUND: Exposure to perinatal anxiety affects disease susceptibility in offspring but studies on the association between perinatal anxiety and gene polymorphisms are lacking. This study aimed to elucidate the interaction between perinatal anxiety and polymorphisms in antioxidant defense and innate immunity genes on the development of respiratory tract infections (RTIs) during early infancy. METHODS: Trait anxiety levels in 440 women were assessed by the State-Trait Anxiety Inventory during late gestation. The occurrence of RTIs, including bronchiolitis, during the first year of life was assessed by parent-reported doctor diagnosis. Polymorphisms in glutathione S-transferase P-1 (GSTP1, rs1695) and CD14 (rs2569190) were genotyped using the TaqMan assay. Copy number variations of GSTT1 were measured by real-time polymerase chain reaction. RESULTS: Exposure to high levels of perinatal anxiety increased the risk of bronchiolitis in the first year of life (adjusted odds ratio [aOR], 1.30; 95% confidence interval [CI]: 1.00-1.80), in particular among children with the AG + GG genotype of GSTP1 or the GSTT1 null genotype (aOR 3.36 and 2.79). In infants with the TC + CC genotype of CD14, high levels of perinatal anxiety were associated with an increased risk of upper RTI, lower RTI, and bronchiolitis (aOR 2.51, 4.60, and 4.31, respectively). CONCLUSIONS: Perinatal maternal anxiety levels affect the occurrence of bronchiolitis in offspring. The effect of perinatal anxiety on the occurrence of bronchiolitis during infancy was influenced by genetic polymorphisms in antioxidant defense and innate immunity genes.
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Ansiedade/psicologia , Bronquiolite/epidemiologia , Glutationa S-Transferase pi/genética , Glutationa Transferase/genética , Imunidade Inata/genética , Receptores de Lipopolissacarídeos/genética , Infecções Respiratórias/epidemiologia , Adulto , Ansiedade/imunologia , Bronquiolite/etiologia , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Lactente , Recém-Nascido , Masculino , Estresse Oxidativo/imunologia , Período Periparto/psicologia , Polimorfismo Genético , Gravidez , Infecções Respiratórias/etiologiaRESUMO
BACKGROUND: Although cesarean delivery and prenatal exposure to antibiotics are likely to affect the gut microbiome in infancy, their effect on the development of atopic dermatitis (AD) in infancy is unclear. The influence of individual genotypes on these relationships is also unclear. To evaluate with a prospective birth cohort study whether cesarean section, prenatal exposure to antibiotics, and susceptible genotypes act additively to promote the development of AD in infancy. METHODS: The Cohort for Childhood of Asthma and Allergic Diseases (COCOA) was selected from the general Korean population. A pediatric allergist assessed 412 infants for the presence of AD at 1 year of age. Their cord blood DNA was subjected to interleukin (IL)-13 (rs20541) and cluster-of-differentiation (CD)14 (rs2569190) genotype analysis. RESULTS: The combination of cesarean delivery and prenatal exposure to antibiotics associated significantly and positively with AD (adjusted odds ratio, 5.70; 95% CI, 1.19-27.3). The association between cesarean delivery and AD was significantly modified by parental history of allergic diseases or risk-associated IL-13 (rs20541) and CD14 (rs2569190) genotypes. There was a trend of interaction between IL-13 (rs20541) and delivery mode with respect to the subsequent risk of AD. (P for interactionâ=â0.039) Infants who were exposed prenatally to antibiotics and were born by cesarean delivery had a lower total microbiota diversity in stool samples at 6 months of age than the control group. As the number of these risk factors increased, the AD risk rose (trend p<0.05). CONCLUSION: Cesarean delivery and prenatal antibiotic exposure may affect the gut microbiota, which may in turn influence the risk of AD in infants. These relationships may be shaped by the genetic predisposition.
Assuntos
Antibacterianos/efeitos adversos , Dermatite Atópica/genética , Interleucina-13/genética , Efeitos Tardios da Exposição Pré-Natal/genética , Adulto , Antibacterianos/uso terapêutico , Cesárea , Dermatite Atópica/induzido quimicamente , Feminino , Interação Gene-Ambiente , Estudos de Associação Genética , Predisposição Genética para Doença , Humanos , Lactente , Receptores de Lipopolissacarídeos/genética , Masculino , Polimorfismo de Nucleotídeo Único , Gravidez , Efeitos Tardios da Exposição Pré-Natal/induzido quimicamente , RiscoRESUMO
BACKGROUND: This study was performed to examine how the characteristics of soybean 2S protein influence allergenicity after enzymatic hydrolysis. Soybean 2S protein was extracted and enzymatic hydrolysis was performed using pepsin and chymotrypsin. Allergenicity was observed using soybean-sensitive patients' sera. RESULTS: Only 13.3% (6/45) of soybean-sensitive patients reacted to soybean Kunitz trypsin inhibitor (SKTI), known as the major allergen of soybean 2S protein. After peptic hydrolysis for 90 min at pH 1.2, the intensity of SKTI decreased to 25% but was still visible on SDS-PAGE. Chymotryptic hydrolysis following peptic hydrolysis at pH 8 for 60 min showed a limited hydrolytic effect on soybean 2S protein. Peptic hydrolysis of soybean 2S protein partially reduced the allergenicity of soybean 2S protein, while chymotryptic hydrolysis following peptic hydrolysis increased slightly the allergenicity. CONCLUSION: Food allergy caused by soybean 2S protein occurred in part of the soybean-sensitive patients. SKTI was partially digested after peptic hydrolysis for 90 min. The allergenicity was decreased with peptic hydrolysis, while subsequent treatment of chymotrypsin increased slightly the allergenicity.
Assuntos
Alérgenos/imunologia , Quimotripsina/metabolismo , Hipersensibilidade Alimentar/imunologia , Glycine max/química , Pepsina A/metabolismo , Hidrolisados de Proteína/imunologia , Inibidor da Tripsina de Soja de Kunitz/imunologia , Pré-Escolar , Feminino , Humanos , Hidrólise , Lactente , Masculino , Hidrolisados de Proteína/metabolismo , Proteínas de Soja , Inibidor da Tripsina de Soja de Kunitz/metabolismo , Inibidores da Tripsina/imunologiaRESUMO
BACKGROUND: Maternal prenatal depression is associated with lower offspring birth weight, yet the impact of gestational age on this association remains inadequately understood. AIMS: We aimed to investigate the effect of prenatal depression on low birth weight, gestational age, and weight for gestational age at term. STUDY DESIGN: Prospective cohort study. SUBJECT: Data were collected from 691 women in their third trimester of pregnancy who went on to give birth to a singleton at term without perinatal complications. One hundred and fifty-two women had a Center for Epidemiologic Studies Depression Scale-10 score ≥10 and were classed as prenatally depressed. OUTCOME MEASURES: Low birth weight (<2500g), gestational age at birth, and birth weight percentile for gestational age. RESULTS: Offspring of prenatally depressed women were more likely to be low birth weight (Odds ratio [OR] 2.94, 95% confidence interval [CI] 1.14-7.58) than offspring of prenatally non-depressed women, but the association was attenuated (OR 1.66, 95% CI 0.55-5.02) when adjusted for gestational age. Offspring of prenatally depressed women had lower gestational age in weeks (OR for one week increase in gestational age: 0.66, 95% CI 0.47-0.93) than offspring of prenatally non-depressed women. There was no association between prenatal depression and birth weight percentile for gestational age. CONCLUSIONS: Prenatal depression was not associated with low birth weight at term, but was associated with gestational age, suggesting that association between maternal depression and birth weight may be a reflection of the impact of depression on offspring gestational age.
Assuntos
Peso ao Nascer , Depressão/fisiopatologia , Idade Gestacional , Complicações na Gravidez/fisiopatologia , Adulto , Estudos de Casos e Controles , Estudos de Coortes , Feminino , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Coreia (Geográfico) , Gravidez , Estudos ProspectivosRESUMO
Previous studies suggest that maternal characteristics may be associated with neonatal outcomes. However, the influence of maternal characteristics on birth weight (BW) has not been adequately determined in Korean populations. We investigated associations between maternal characteristics and BW in a sample of 813 Korean women living in the Seoul metropolitan area, Korea recruited using data from the prospective hospital-based COhort for Childhood Origin of Asthma and allergic diseases (COCOA) between 2007 and 2011. The mean maternal age at delivery was 32.3 ± 3.5 yr and prepregnancy maternal body mass index (BMI) was 20.7 ± 2.5 kg/m(2). The mean BW of infant was 3,196 ± 406 g. The overall prevalence of a maternal history of allergic disease was 32.9% and the overall prevalence of allergic symptoms was 65.1%. In multivariate regression models, prepregnancy maternal BMI and gestational age at delivery were positively and a maternal history of allergic disease and nulliparity were negatively associated with BW (all P < 0.05). Presence of allergic symptoms in the mother was not associated with BW. In conclusion, prepregnancy maternal BMI, gestational age at delivery, a maternal history of allergic disease, and nulliparity may be associated with BW, respectively.
Assuntos
Peso ao Nascer , Mães , Adulto , Índice de Massa Corporal , Estudos de Coortes , Feminino , Idade Gestacional , Humanos , Hipersensibilidade/diagnóstico , Hipersensibilidade/epidemiologia , Recém-Nascido , Modelos Lineares , Masculino , Paridade , Gravidez , República da Coreia/epidemiologiaRESUMO
A retropharyngeal abscess is a rare disease entity in young infants but can develop after nasopharyngeal viral infection. Group B Streptococcus and Staphylococcus aureus are the most common pathogens in young infants, however, Mycobacterium tuberculosis is very rare. We report the case of retropharyngeal abscess and coinfection with S. aureus and M. tuberculosis in a very young infant presenting with respiratory symptoms due to upper airway obstruction. Usually tuberculous retropharyngeal abscesses are caused by the direct invasion of the bacteria from the spine via anterior longitudinal ligament of the spine. However, in this case, no associated spinal disease was observed.
RESUMO
Since the risk of developing allergic disease increases in individuals exposed to allergens previously, even during the neonatal period, the immunologic status of a fetus may be important in the subsequent development of allergy. We evaluated the fetal factors to predict atopic dermatitis (AD) at 12 months in 412 infants of a COhort for Childhood Origin of Asthma and Allergic Diseases (COCOA) in the general Korean population. Cord blood mononuclear cells (CBMCs) were stimulated with ovalbumin and phytohemagglutinin and cellular proliferative response and concentrations of interleukin-13 and interferon-γ, were measured. The risk of developing AD was greater in boys than girls (OR 1.97, 95% CI 1.26-3.09), infants delivered by cesarean section than vaginally (OR 1.93, 95% CI 1.14-3.26) and infants with than without parental history of AD (OR 2.34, 95% CI 1.29-4.24). The CBMC proliferative response to phytohemagglutinin stimulation was higher in infants with than without AD (P = 0.048), but no difference was observed in ovalbumin-stimulated cells (P = 0.771). Risk factors for the development of AD at 12 months include male gender, delivery by cesarean section and parental history of AD. Increased CBMC proliferative response to phytohemagglutinin stimulation may predict the development of AD at 12 months.
Assuntos
Dermatite Atópica/diagnóstico , Sangue Fetal/citologia , Leucócitos Mononucleares/metabolismo , Adulto , Proliferação de Células , Cesárea , Dermatite Atópica/metabolismo , Feminino , Sangue Fetal/metabolismo , Humanos , Lactente , Interferon gama/metabolismo , Interleucina-13/metabolismo , Leucócitos Mononucleares/efeitos dos fármacos , Masculino , Razão de Chances , Ovalbumina/toxicidade , Fito-Hemaglutininas/toxicidade , Valor Preditivo dos Testes , Gravidez , Fatores de Risco , Fatores SexuaisRESUMO
BACKGROUND: The importance in asthma management of monitoring of peak expiratory flow rate (PEFR) has been emphasized. OBJECTIVE: For effective asthma management in Korean children, we established reference ranges for the PEFR in children 4-18 years of age. METHODS: The Mini Wright Peak Flow Meter (MWPFM) and spirometry were used in this study. All tests were conducted using a standardized method recommended by the American Thoracic Society. RESULTS: From a total sample of 2389 children, 826 (34.5%) were excluded based on our exclusion criteria. For both sexes, the PEFR increased with height, age, weight, sitting height and body surface area (BSA). Height and BSA were found to be better predictors of PEFR than the other parameters. The correlation coefficient between FEV1 and PEFR using the MWPFM was 0.886 (p < .001). The reference values of PEFR for height in our study were higher for both sexes than for those previously reported in Korea (p < .005). When we compared our results with those from other countries, the values for boys of the same height were lower than those for European children, but higher than those for African and Turkish children (p < .001). CONCLUSIONS: We have established reference values for PEFR obtained by MWPFM in Korean children in Seoul, and have provided the percentile curves for PEFR as a function of height asthma to assist clinical practices in treating children with asthma in Korea.
Assuntos
Asma/diagnóstico , Asma/epidemiologia , Grupos Populacionais/estatística & dados numéricos , Espirometria/estatística & dados numéricos , Adolescente , Antropometria , Asma/etnologia , Criança , Pré-Escolar , Feminino , Humanos , Coreia (Geográfico) , Masculino , Guias de Prática Clínica como Assunto , Valor Preditivo dos Testes , Prognóstico , Padrões de Referência , Espirometria/normasRESUMO
BACKGROUND: Cystic fibrosis (CF) is one of the most common hereditary disorders among Caucasians. The most common mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been well established among Caucasian populations. In Koreans, however, there are very few cases of genetically confirmed CF thus far, and the spectrum of mutations seems quite different from that observed in Caucasians. METHODS: In the present study, we describe the cases of 2 Korean CF patients, present sequencing results identifying mutations in their CFTR gene, and summarize the results of CFTR mutational spectrum from previously reported Korean CF patients. The mutations described were identified by performing direct sequencing analysis of the complete coding regions and flanking intronic sequences of the CFTR gene, followed by multiplex ligation-dependent probe amplification (MLPA) analysis in order to detect gene deletions or duplications that could not be identified by a direct sequencing method. RESULTS: Three CFTR mutations were identified in the 2 patients, including p.Q98R, c.2052delA, and c.579+5G>A. In an analysis of 9 Korean CF patients that included the 2 patients presented in this study, p.Q98R mutation was the only recurrently observed mutation with a frequency of 18.8% (3/16 alleles). Furthermore, only one of the mutations (c.3272-26A>G) was found among the 32 common mutations in the screening panel for Caucasians from the Cystic Fibrosis Mutation Database. CONCLUSIONS: Sequencing of the entire CFTR gene followed by MLPA analysis, rather than using the targeted sequencing-based screening panel for mutations commonly found in Caucasian populations, is recommended for genetic analysis of Korean CF patients.
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Povo Asiático/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Adulto , Alelos , Feminino , Heterozigoto , Humanos , Masculino , Mutação , República da Coreia , Análise de Sequência de DNA , Adulto JovemRESUMO
Many patients assume that allergic reactions against foods are responsible for triggering or worsening their allergic symptoms. Therefore, it is important to identify patients who would benefit from an elimination diet, while avoiding unnecessary dietary restrictions. The diagnosis of food allergy depends on the thorough review of the patients's medical history, results of supplemented trials of dietary elimination, and in vivo and in vitro tests for measuring specific IgE levels. However, in some cases the reliability of such procedures is suboptimal. Oral food challenges are procedures employed for making an accurate diagnosis of immediate and occasionally delayed adverse reactions to foods. The timing and type of the challenge, preparation of patients, foods to be tested, and dosing schedule should be determined on the basis of the patient's history, age, and experience. Although double-blind, placebo-controlled food challenges(DBPCFC) are used to establish definitively if a food is the cause of adverse reactions, they are time-consuming, expensive and troublesome for physician and patients. In practice, An open challenge controlled by trained personnel is sufficient especially in infants and young children. The interpretation of the results and follow-up after a challenge are also important. Since theses challenges are relatively safe and informative, controlled oral food challenges could become the measure of choice in children.
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Controversy exists regarding the preventive effect of probiotics on the development of eczema or atopic dermatitis. We investigated whether supplementation of probiotics prevents the development of eczema in infants at high risk. In a randomized, double-blind, placebo-controlled trial, 112 pregnant women with a family history of allergic diseases received a once-daily supplement, either a mixture of Bifidobacterium bifidum BGN4, B. lactis AD011, and Lactobacillus acidophilus AD031, or placebo, starting at 4-8 wks before delivery and continuing until 6 months after delivery. Infants were exclusively breast-fed during the first 3 months, and were subsequently fed with breastmilk or cow's milk formula from 4 to 6 months of age. Clinical symptoms of the infants were monitored until 1 yr of age, when the total and specific IgE against common food allergens were measured. A total of 68 infants completed the study. The prevalence of eczema at 1 yr in the probiotic group was significantly lower than in the placebo group (18.2% vs. 40.0%, p=0.048). The cumulative incidence of eczema during the first 12 months was reduced significantly in probiotic group (36.4% vs. 62.9%, p=0.029); however, there was no difference in serum total IgE level or the sensitization against food allergens between the two groups. Prenatal and postnatal supplementation with a mixture of B. bifidum BGN4, B. lactis AD011, and L. acidophilus AD031 is an effective approach in preventing the development of eczema in infants at high risk of allergy during the first year of life.
Assuntos
Bifidobacterium/imunologia , Eczema/epidemiologia , Lactobacillus acidophilus/imunologia , Probióticos/administração & dosagem , Bifidobacterium/classificação , Aleitamento Materno , Dermatite Atópica/epidemiologia , Dermatite Atópica/imunologia , Dermatite Atópica/prevenção & controle , Método Duplo-Cego , Eczema/imunologia , Eczema/prevenção & controle , Feminino , Hipersensibilidade Alimentar/prevenção & controle , Humanos , Imunoglobulina E/sangue , Incidência , Lactente , Recém-Nascido , Cuidado Pós-Natal , Gravidez , Cuidado Pré-Natal , Prevalência , Resultado do TratamentoRESUMO
BACKGROUND: Cystic fibrosis is a chronic progressive autosomal recessive disorder caused by the CFTR gene mutations. It is quite common in Caucasians, but very rare in Asians. Sweat chloride test is known to be a screening test for the cystic fibrosis due to the fact that electrolyte levels in sweat are elevated in patients. In this study, sweat chloride levels in Korean population were measured and analyzed by using standardized pilocarpine iontophoresis sweat chloride test. METHODS: The sweat chloride test was performed in 47 patients referred to Yondong Severance Hospital from August, 2001 to April, 2007 and 41 healthy volunteers. The sweat chloride tests were conducted according to the CLSI C34-A2 guideline using pilocarpine iontophoresis method, and the chloride concentrations in sweat were measured by mercurimetric titration. RESULTS: Four patients showed sweat chloride concentrations higher than 60 mmol/L. Reference interval was calculated as 1.4-44.5 mmol/L by analysis of the results of healthy volunteers (n=41). Four patients who exhibited high sweat chloride levels, had characteristic clinical features of cystic fibrosis and their diagnoses were confirmed either by repeated sweat chloride test or genetic analysis. CONCLUSIONS: Standardized sweat chloride test can be utilized as a useful diagnostic tool for cystic fibrosis in Koreans. In cases of sweat chloride levels higher than 40 mmol/L, the test should be repeated for the possible diagnosis of cystic fibrosis. All the confirmed Korean cases of cystic fibrosis showed sweat chloride level above 60 mmol/L.
Assuntos
Cloretos/análise , Cloretos/normas , Fibrose Cística/diagnóstico , Suor/metabolismo , Adolescente , Adulto , Criança , Pré-Escolar , Fibrose Cística/genética , Feminino , Humanos , Lactente , Iontoforese/métodos , Coreia (Geográfico) , Masculino , Pessoa de Meia-Idade , Pilocarpina/química , Análise de Sequência de DNA , Suor/químicaRESUMO
BACKGROUND: Although parenchyma-saving resection makes it possible to preserve the lung parenchyma, most surgeons are reluctant to perform it for congenital cystic adenomatoid malformation (CCAM) because it could also result in recurrent pulmonary infection or residual lesion. This study compared the early and late postoperative outcomes according to the extent of resection in CCAM patients to determine if the extent of resection would influence the short- and long-term results. METHODS: Between 1995 and 2006, 45 patients underwent surgical resection for CCAM. Ten patients received a segmentectomy and 2 a wedge resection (the parenchyma-saving group), and 32 received a lobectomy and 1 a pneumonectomy (the lobectomy group). A retrospective analysis was done to compare the early and late postoperative outcomes between two groups. RESULTS: No significant differences were observed for severity and duration of preoperative symptoms. No in-hospital or late deaths occurred. There were no significant differences in the incidence of early postoperative complications and late morbidities between the two groups. No significant differences were observed between the two groups for hospital length of stay and duration of chest tube placement. CONCLUSIONS: The early and late outcomes were excellent even after parenchyma-saving resection in patients with CCAM. We suggest that parenchyma-saving resection can be safely performed in selected patients with a well-confined CCAM lesion and thereby avoiding pneumonectomy in children.
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Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Pulmão/cirurgia , Pneumonectomia/métodos , Biópsia por Agulha , Pré-Escolar , Estudos de Coortes , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Feminino , Seguimentos , Humanos , Lactente , Tempo de Internação , Masculino , Dor Pós-Operatória/fisiopatologia , Pneumonectomia/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Probabilidade , Estudos Retrospectivos , Medição de Risco , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Nasal swabs were collected to isolate S. aureus in 296 children, who visited the pediatrics department with a variety of symptoms. Staphylococcus aureus was isolated from 95 children (32.1%). Of the isolates, 18 were methicillin-resistant S. aureus (MRSA) (18.9%). Antimicrobial susceptibility testing was performed for all S. aureus cultured and the molecular characteristics were investigated. Forty-nine spa types were identified among the S. aureus isolates, and were classified into 13 spa groups (A-L). The most prevalent clone (34 isolates, 35.8%) belonged to the spa group B (spa repeat motif, WG/FKAOMQ), which corresponded to sequence type 30 (ST30) and its variants. Sixteen different spa types, within the spa group B, suggested that this group has evolved over a long period of time. In addition, all S. aureus isolates belonging to the spa group B were methicillin-susceptible, indicating that this group might represent successful adaptation of this clone in the community setting with low antibiotic pressure. The most frequently found clone in the MRSA group was spa group C (spa repeat motif, DMGGM) and SCCmec type IVA, which represented half of the MRSA isolates and corresponded to ST72. ST5-MRSA-II, the most prevalent MRSA clone in Korean hospitals, was found in only two isolates. These findings suggest that strains of S. aureus nasal carriage in Korean children visiting an outpatient pediatric department were different from the strains identified in hospital infections.
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Instituições de Assistência Ambulatorial , Resistência a Meticilina , Nariz/microbiologia , Staphylococcus aureus/efeitos dos fármacos , Staphylococcus aureus/isolamento & purificação , Distribuição por Idade , Portador Sadio , Criança , Pré-Escolar , Genes Bacterianos , Humanos , Lactente , Coreia (Geográfico) , Testes de Sensibilidade Microbiana , Análise de Sequência de DNAAssuntos
Aditivos Alimentares/efeitos adversos , Hipersensibilidade Alimentar/diagnóstico , Hipersensibilidade/diagnóstico , Adolescente , Adulto , Asma/diagnóstico , Asma/imunologia , Criança , Pré-Escolar , Doença Crônica , Método Duplo-Cego , Feminino , Hipersensibilidade Alimentar/imunologia , Humanos , Hipersensibilidade/imunologia , Lactente , Masculino , Rinite Alérgica Perene/diagnóstico , Rinite Alérgica Perene/imunologia , Rinite Alérgica Sazonal/diagnóstico , Rinite Alérgica Sazonal/imunologia , Testes Cutâneos , Urticária/diagnóstico , Urticária/imunologiaRESUMO
OBJECTIVE: The aim of this study was to determine how long postoperative pulmonary care is needed in patients after rigid bronchoscopic retrieval of foreign body from the airway and to identify the factors affecting pulmonary recovery. MATERIALS AND METHOD: A retrospective review of 98 pediatric patients who had foreign body removed from the airway by rigid bronchoscopic procedure was conducted. After the removal of the foreign body, the time required for complete clinical and radiologic pulmonary recovery was reviewed for each subject. Factors examined included age, sex, preoperative symptoms, preoperative radiologic findings, duration between the aspiration event and treatment, the type of foreign body, the location of foreign body impaction, operation time, immediate postoperative radiologic changes, and treatment outcome. RESULTS: More than 1 week for the complete pulmonary recovery was required in 25 (25.5%) cases, whereas 74.5% of subjects fully recovered within 1 week after the removal of foreign body. In the univariate analysis, preoperative inflammatory symptoms, and radiologic findings, an operation time of over 50 minutes and the aggravation of immediate postoperative radiologic findings predicted a delayed pulmonary recovery (more than 1 week). In the multivariate analysis, three of four variables previously noted, except preoperative inflammatory symptoms, showed significant predictability for a delayed pulmonary recovery. CONCLUSION: The recovery time of more than 1 week after rigid bronchoscopic retrieval of airway foreign body was associated with preoperative inflammatory findings by radiologic study, a prolonged procedure over 50 minutes, and aggravation of the immediate postoperative radiologic findings. Therefore, long-term pulmonary care is required for this group of patients.