Immunoglobulin deficiency in patients with chronic rhinosinusitis: Systematic review of the literature and meta-analysis.

BACKGROUND: Several studies have shown a high prevalence of immunoglobulin deficiencies in patients with chronic rhinosinusitis (CRS). OBJECTIVE: We sought to perform a systematic review and meta-analysis to estimate this prevalence more precisely and to identify patients who need substitution treatment. METHODS: All case series published after 1990 describing patients with CRS, which was defined as symptomatic rhinosinusitis for more than 12 weeks and documented immunoglobulin deficiencies (including deficiencies of IgG with subclasses, IgA, and IgM; specific antibody deficiencies; and potential common variable immunodeficiency), were retrieved. A meta-analysis of the proportion of any combination of common variable immunodeficiency, IgG deficiency, IgA deficiency, and IgM deficiency in patients with CRS was performed by using logit transformation of the prevalence. Recurrent CRS was defined as rhinosinusitis not controlled by appropriate conservative management for 4 months, and difficult-to-treat CRS was defined as noncontrollable rhinosinusitis despite successful sinus surgery and appropriate conservative management for at least 1 year. RESULTS: The meta-analysis revealed a prevalence of pooled IgG, IgA, and IgM deficiencies in 13% of patients with recurrent CRS and 23% of patients with difficult-to-treat CRS. The prevalence of IgG subclass deficiency (5% to 50%) and specific antibody deficiency (8% to 34%) was increased in patients with CRS, as was the prevalence of respiratory allergies in patients with recurrent CRS (31% to 72%). CONCLUSION: Immunoglobulin deficiency is a frequent condition in patients with CRS. An even higher prevalence of atopy was observed in patients with recurrent CRS. Therefore immunoglobulin titers and accurate allergy diagnostic workups are strongly recommended in these patients to provide specific treatments for symptom alleviation. However, there is a need for larger prospective studies addressing the effect of specific therapeutic interventions for CRS.

Primary immunodeficiency from B-cell defect: a case series of 6 patients seen in a national tertiary hospital in the Philippines.

Primary immunodeficiency disorders, although rare, pose a significant burden in the quality of life of afflicted patients and their families. The most common of these disorders are caused by B-cell defects. A total of 6 patients were seen and diagnosed in a national tertiary hospital in the Philippines from 1996 to 2018. These patients were admitted due to various infections, and were subsequently diagnosed to have B-cell defects. Four out of the 6 patients have genetic studies confirming the diagnosis of X-linked agammaglobulinemia. One patient succumbed to sepsis at 10 years of age, while the rest are on follow-up at the Philippine General Hospital for intravenous immunoglobulin infusion.

Repercussions of inborn errors of immunity on growth.

OBJECTIVES: This study aimed to review the literature on the repercussions of the different inborn errors of immunity on growth, drawing attention to the diagnosis of this group of diseases in patients with growth disorders, as well as to enable the identification of the different causes of growth disorders in patients with inborn errors of immunity, which can help in their treatment. DATA SOURCES: Non-systematic review of the literature, searching articles since 2000 in PubMed with the terms "growth", "growth disorders", "failure to thrive", or "short stature" AND "immunologic deficiency syndromes", "immune deficiency disease", or "immune deficiency" NOT HIV. The Online Mendelian Inheritance in Man (OMIN) database was searched for immunodeficiencies and short stature or failure to thrive. DATA SUMMARY: Inborn errors of immunity can affect growth in different ways, and some of them can change growth through multiple simultaneous mechanisms: genetic syndromes; disorders of the osteoarticular system; disorders of the endocrine system; reduction in caloric intake; catabolic processes; loss of nutrients; and inflammatory and/or infectious conditions. CONCLUSIONS: The type of inborn errors of immunity allows anticipating what type of growth disorder can be expected. The type of growth disorder can help in the diagnosis of clinical conditions related to inborn errors of immunity. In many inborn errors of immunity, the causes of poor growth are mixed, involving more than one factor. In many cases, impaired growth can be adjusted with proper inborn errors of immunity treatment or proper approach to the mechanism of growth impairment.

Alcohol and tobacco use among methadone maintenance patients in Vietnamese rural mountainside areas.

INTRODUCTION: The expansion of methadone maintenance treatment (MMT) program requires more data about the factors affecting the effectiveness of treatment, especially behavioral data such as smoking and alcohol use among patients. This study aimed to examine the prevalence of tobacco and alcohol consumption and identify related factors among MMT patients in the Vietnamese rural mountainside. METHODS: We interviewed 241 MMT patients in two clinics in Tuyen Quang, a mountainous province in Vietnam. Patients were asked to report the smoking status (current smoker or not), nicotine dependence (by Fagerström test for nicotine dependence - FTND) and alcohol use (by using the Alcohol Use Disorders Identification Test - AUDIT-C). EuroQol-5 dimensions-5 levels (EQ-5D-5L) and EQ-Visual analogue scale (EQ-VAS) were employed to measure health-related quality of life. Multivariate logistic and Tobit regressions were used to identify the associated factors. RESULTS: The majority of respondents were current smokers (75.7%) and a low proportion were hazardous drinkers (18.3%). People receiving treatment in a rural clinic (OR = 0.45; 95%CI = 0.22-0.92) and had problems in usual activities (OR = 0.20; 95%CI = 0.06-0.70) were less likely to be smokers. Q-VAS score (Coef. = 0.03; 95%CI = 0.02-0.05) and having problems in mobility (Coef. = 0.72; 95%CI = 0.03-1.42) was found to be associated with the increase of nicotine dependence. In terms of alcohol drinking, people with other jobs were more likely to drink hazardously compared to unemployed patients (OR = 2.86; 95%CI = 1.20-6.82). Similarly, patients having higher duration of MMT had higher likelihood of being hazardous drinkers (OR = 1.07; 95%CI = 1.01-1.13). CONCLUSIONS: This study highlights the low rate of alcohol abusers but a considerably high proportion of current smokers among MMT patients in the rural mountainside area. Alcohol and tobacco counseling programs combined with social and family support also play an essential role in alcohol and tobacco control. In addition, implementing mass community-based behavioral change campaigns to reduce drug addiction-related stigmatization should also be prioritized.

Avaliação do emprego da imunoglobulina humana endovenosa sobre a densidade de infecções em pacientes com mieloma múltiplo / Evaluation of the use of human intravenous immunoglobulin on the incidence density of infections in patients with multiple myeloma

Introdução: O mieloma múltiplo (MM) é uma neoplasia hematológica que cursa com hipogamaglobulinemia e consequente imunodeficiência secundária. Uma das principais causas de morbimortalidade desses pacientes são infecções. Objetivou-se com esse estudo avaliar o impacto da reposição de imunoglobulina endovenosa (IgIV) na taxa de infecções em pacientes portadores de MM. Métodos: Trata-se de um estudo de análise documental, com variáveis qualitativas e quantitativas, com objetivo de realizar análise retrospectiva dos prontuários de pacientes com MM que receberam tratamento com imunoglobulina humana endovenosa em um hospital privado na cidade de Patos de Minas, MG, Brasil, no período de 01/05/2016 a 31/12/2020. Foram coletados dados epidemiológicos, resultados de exames, episódios de infecções, eventos adversos da medicação e desfecho dos pacientes nos prontuários analisados. Resultados: Foram identificados 10 pacientes com diagnóstico de MM, todos receberam IgIV na dose de 300 a 400 mg/kg/mês. Nenhuma reação adversa relacionada ao uso da IgIV foi registrada nos prontuários. Foram identificados seis quadros infecciosos que ocorreram em quatro pacientes. Nenhum diagnóstico de sepse foi registrado. A densidade de incidência de infecções foi de 0,28 episódios/pacientes-ano. Conclusão: A densidade de incidência de infecções observada no presente estudo foi significativamente menor em comparação ao que se tem registro na literatura, sugerindo importante papel da IgIV na prevenção de infecções em pacientes com MM.

Introduction: Multiple myeloma (MM) is a hematologic malignancy that leads to hypogammaglobulinemia and consequent secondary immunodeficiency. Infections are a major cause of morbidity and mortality in these patients. The objective of this study was to evaluate the impact of intravenous immunoglobulin (IVIg) replacement on the rate of infections in patients with MM. Methods: This document analysis study used qualitative and quantitative variables to perform a retrospective analysis of the medical records of patients with MM who were treated with human IVIg in a private hospital in the city of Patos de Minas, MG, Brazil, from May 1, 2016 to December 31, 2020. Epidemiological data, test results, episodes of infections, adverse medication events, and patient outcomes were collected from the medical records. Results: Ten patients diagnosed with MM were identified, and they all received IVIg at a dose of 300 to 400 mg/kg/month. No adverse reactions related to the use of IVIg were recorded. Six infections that occurred in 4 patients were identified. No diagnosis of sepsis was recorded. The incidence density of infections was 0.28 episodes/patient-years. Conclusion: The incidence density of infections was significantly smaller in this study in comparison with previous literature findings, which suggests a significant role of IVIg in the prevention of infections in patients with MM.

Repercussions of inborn errors of immunity on growth / Repercussões dos erros inatos da imunidade sobre o crescimento

Abstract Objectives: This study aimed to review the literature on the repercussions of the different inborn errors of immunity on growth, drawing attention to the diagnosis of this group of diseases in patients with growth disorders, as well as to enable the identification of the different causes of growth disorders in patients with inborn errors of immunity, which can help in their treatment. Data sources: Non-systematic review of the literature, searching articles since 2000 in PubMed with the terms "growth", "growth disorders", "failure to thrive", or "short stature" AND "immunologic deficiency syndromes", "immune deficiency disease", or "immune deficiency" NOT HIV. The Online Mendelian Inheritance in Man (OMIN) database was searched for immunodeficiencies and short stature or failure to thrive. Data summary: Inborn errors of immunity can affect growth in different ways, and some of them can change growth through multiple simultaneous mechanisms: genetic syndromes; disorders of the osteoarticular system; disorders of the endocrine system; reduction in caloric intake; catabolic processes; loss of nutrients; and inflammatory and/or infectious conditions. Conclusions: The type of inborn errors of immunity allows anticipating what type of growth disorder can be expected. The type of growth disorder can help in the diagnosis of clinical conditions related to inborn errors of immunity. In many inborn errors of immunity, the causes of poor growth are mixed, involving more than one factor. In many cases, impaired growth can be adjusted with proper inborn errors of immunity treatment or proper approach to the mechanism of growth impairment.

Resumo Objetivos: Revisão da literatura sobre as repercussões dos diferentes erros inatos da imunidade sobre o crescimento, chamar a atenção para o diagnóstico desse grupo de doenças em pacientes que apresentem desordens do crescimento, assim como permitir que se identifiquem as diferentes causas de alterações do crescimento em pacientes com erros inatos da imunidade, o que pode auxiliar em seu manejo. Fonte dos dados: Revisão não sistemática da literatura, com busca de artigos desde 2000 no Pubmed com os termos "growth" ou "growth disorders" ou "failure to thrive" ou "short stature" AND "immunologic deficiency syndromes" ou "immune deficiency disease" ou "imune deficiency" NOT HIV. E buscas na base OMIN (Online Mendelian Inheritance in Man) por imunodeficiências e baixa estatura ou falha no crescimento ("failure to thrive"). Síntese dos dados: Há diferentes modos pelos quais os erros inatos da imunidade podem afetar o crescimento e alguns deles podem alterar o crescimento por múltiplos mecanismos simultâneos: síndromes genéticas; afecções do aparelho osteoarticular; afecções do sistema endócrino; redução de aporte calórico; processos catabólicos: perda de nutrientes, assim como afecções inflamatórias e/ou infecciosas. Conclusões: O tipo de erros inatos da imunidade permite prever que tipo de alteração no crescimento devemos esperar. O tipo de alteração no crescimento pode auxiliar no diagnóstico de condições clínicas associadas aos erros inatos da imunidade. Em muitos erros inatos da imunidade, as causas do crescimento deficiente são mistas, envolvem mais de um fator. Em muitos casos, o prejuízo do crescimento pode ser corrigido com o adequado tratamento dos erros inatos da imunidade ou adequada abordagem do mecanismo que causa o prejuízo do crescimento.

As percepções das mulheres portadoras de HIV/AIDS perante a impossibilidade de amamentação / Percepciones mujeres portadoras de VIH / SIDA ante la imposibilidad de lactancia materna / Perceptions of the bearers of HIV/AIDS before the inability to breastfeeding

Objective: To understand the perceptions of living with HIV / AIDS face impossibility of breastfeeding. Method: This is a descriptive, exploratory qualitative study conducted in a Day Hospital, reference the state of Santa Catarina in 2012. The sampling technique used was the semi-structured interview as open Minayo1. Results: The study subjects showed predominant age group was between 27-38 years. The experience of not breastfeeding was for women experience painful and emotionally draining, and created a way to satisfy the idealized symbolic breastfeeding by them during the act of breastfeeding, replacing the physiological significance of breastfeeding. Conclusion: Thus, the nurse needs beyond compliance with protocols regarding the inhibition of lactation, understand and encourage breastfeeding symbolic, created by women, in addition to the biological, the emotional, social and cultural conditions that surround the woman.

Objetivo: Conhecer as percepções das portadoras de HIV/AIDS perante impossibilidade de amamentação. Método: Trata-se de estudo qualitativo descritivo e exploratório realizado em um Hospital Dia, de referência no estado de Santa Catarina, em 2012. A técnica de coleta utilizada foi à entrevista semi-estruturada aberta conforme Minayo1. Resultados: Os sujeitos de pesquisa apresentaram faixa etária predominante foi entre 27 a 38 anos. A experiência de não amamentar, foi para as mulheres uma experiência penosa e emocionalmente desgastante, e criaram um modo de satisfazer a amamentação simbólica idealiza da por elas durante o ato de amamentar, substituindo o significado da amamentação fisiológica. Conclusão: Deste modo, a enfermagem precisa além do cumprimento de protocolos a respeito da inibição da lactação, compreender e estimular a amamentação simbólica, criada pelas mulheres, além dos aspectos biológicos, os emocionais, sociais e culturais que circundam a mulher.

Objetivo: Conocer las percepciones de los que viven con el VIH/SIDA imposibilidad frente a la lactancia materna. Método: Se trata de un estudio cualitativo, descriptivo, exploratorio realizado en un Hospital de Día, referencia al estado de Santa Catarina en 2012. La técnica de muestreo utilizada fue la entrevista semi-estructurada como abierto Minayo1. Resultados: Los sujetos del estudio mostraron grupo de edad predominante fue entre 27 a 38 años. La experiencia de la lactancia materna no fuera por las mujeres experimentan dolor y emocionalmente agotador, y creó una forma de satisfacer la lactancia simbólica idealizada por ellos, en sustitución de la importancia fisiológica de la lactancia materna. Conclusión: La enfermera necesita más allá de cumplimiento de los protocolos con respecto a la inhibición de la lactancia, entender y fomentar la lactancia materna simbólica, creado por las mujeres, además de los, las condiciones emocionales, sociales y culturales biológicos que rodean la mujer.