Characteristics of the patients followed with the diagnosis of common variable immunodeficiency and the complications.

INTRODUCTION: In this study, we aimed to retrospectively evaluate the clinical and laboratory findings and complications of 28 common variable immunodeficiency (CVID) patients. MATERIAL AND METHODS: The clinical features and laboratory data of 28 CVID patients were evaluated. RESULTS: Nineteen patients were male. In 53.5% of the cases, complications included inflammatory bowel disease, cytopenia, bronchiectasis, granulomatous lymphocytic interstitial lung disease (ILD) and asthma. In their immunological evaluations, IgG, IgM, and IgA mean values were 474.8 ±214.1 mg/dl; 56.7 ±41.9 mg/dl; 35.3 ±58.2 mg/dl, respectively, and the vaccine response was positive in 64.2% of the cases. In all age groups, absolute lymphocyte counts, naive (CD19+IgD+27-), nonswitch (CD19+IgD-27+) memory B cells were numerically higher when compared to the data of healthy children; however, although switch memory (CD19+IgD+27+) B cells were proportionally low in the 4-8 and 12-18 age groups, they were low both numerically and proportionally in the 8-12 age group. No statistically significant difference was found between the cases with complications and without complications. But the cases with pulmonary complications were compared within the group, the CD8 ratio was high but the IgA level was low in patients with bronchiectasis and CD3 was numerically and proportionally low in the cases with ILD compared to others. According to the Paris classification, 11/27 (40.7%) of the cases, 3/27 (11.1%) of them and 13/27 (48.2%) of them were evaluated as MB0, MB1, and MB2, respectively. CONCLUSIONS: In genetic studies, TACI (trans-membrane activator and calcium-modulating cyclophilin ligand interactor - TNFRSF13B) mutation was found positive in 25% of the cases.

Marcadores de inflamación y desarrollo de tolerancia en la proctocolitis alérgica / Markers of inflammation and tolerance development in allergic proctocolitis

Antecedentes: Con el incremento de la proctocolitis alérgica inducida por proteínas de la dieta (PAIPD), son necesarios estudios que aclaren su fisiopatología y determinar marcadores no invasivos y sencillos para el diagnóstico y la evaluación del desarrollo de tolerancia. No hallamos estudios publicados sobre la función del índice de neutrófilos/linfocitos (INL) y el volumen plaquetario medio (VPM), que son marcadores no invasivos fácilmente medibles, en pacientes con PAIPD. Objetivos: Determinar la relación entre el INL y el VPM con el diagnóstico y desarrollo de tolerancia en niños con PAIPD. Métodos: Estudio transversal retrospectivo, los datos se obtuvieron del sistema de registros médicos, los síntomas y los resultados de laboratorio de los pacientes con diagnóstico de PAIPD fueron controlados en los consultorios de alergia y gastroenterología. Se compararon valores del hemograma al momento del diagnóstico con el grupo de niños sanos de edad y sexo similares. Resultados: Entre los 59 pacientes con diagnóstico de PAIPD, los varones representaron el 47,4% y las niñas, el 52,6%. El VPM y el volumen plaquetario relativo (VPR) eran significativamente más altos entre los pacientes con PAIPD en comparación con el grupo de referencia (n: 67) (p < 0,001). Asimismo, VPM y el VPR fueron significativamente elevados en pacientes que no desarrollaron tolerancia comparados con los que la desarrollaron (p= 0,01). Con el INL no hubo diferencias entre los grupos. Conclusiones: El VPM y el VPR se consideraron marcadores adecuados para predecir el pronóstico de los pacientes con PAIPD dado que son rápidos, costo-efectivos y fáciles de medir.

Background. Today, as a result of an increase in the frequency of food protein-induced allergic proctocolitis (FPIAP), there is a need for studies not only to enlighten the pathophysiology of the disease but also to determine simple, non-invasive markers in both diagnosis, and evaluation of the development of tolerance. No study has been found in the literature about the place of neutrophil/lymphocyte ratio (NLR) and mean platelet volume (MPV), which are easy to calculate and non-invasive markers. Objectives. The purpose is to determine the relation between NLR and MPV with the diagnosis and development of tolerance in children with FPIAP. Methods. In this retrospective cross-sectional study, clinical, demographic symptoms and laboratory findings of patients, monitored with FPIAP diagnosis in allergy and gastroenterology clinics, were acquired from the patient record system. Hemogram values at the time of diagnosis were compared with the values of healthy children of the same age and gender. Results. Among 59 patients diagnosed with FPIAP, males constitute 47.4% and females constitute 52.6%. MPV and platelet crit (PCT) values were significantly high when compared to the control group (n: 67) in FPIAP cases (p <0.001). Also, MPV and PCT values were significantly high in non-tolerance developing cases when compared to developing ones (p= 0.01). Conclusions. Contrary to NLR, MPV and PCT values have been considered to be good markers in predicting prognosis in cases with FPIAP since they are quick, cost effective and easy to calculate.

Markers of inflammation and tolerance development in allergic proctocolitis. / Marcadores de inflamación y desarrollo de tolerancia en la proctocolitis alérgica.

BACKGROUND: Today, as a result of an increase in the frequency of food protein-induced allergic proctocolitis (FPIAP), there is a need for studies not only to enlighten the pathophysiology of the disease but also to determine simple, non-invasive markers in both diagnosis, and evaluation of the development of tolerance. No study has been found in the literature about the place of neutrophil/lymphocyte ratio (NLR) and mean platelet volume (MPV), which are easy to calculate and non-invasive markers. OBJECTIVES: The purpose is to determine the relation between NLR and MPV with the diagnosis and development of tolerance in children with FPIAP. METHODS: In this retrospective cross-sectional study, clinical, demographic symptoms and laboratory findings of patients, monitored with FPIAP diagnosis in allergy and gastroenterology clinics, were acquired from the patient record system. Hemogram values at the time of diagnosis were compared with the values of healthy children of the same age and gender. RESULTS: Among 59 patients diagnosed with FPIAP, males constitute 47.4% and females constitute 52.6%. MPV and platelet crit (PCT) values were significantly high when compared to the control group (n: 67) in FPIAP cases (p < 0.001). Also, MPV and PCT values were significantly high in non-tolerance developing cases when compared to developing ones (p= 0.01). CONCLUSIONS: Contrary to NLR, MPV and PCT values have been considered to be good markers in predicting prognosis in cases with FPIAP since they are quick, cost effective and easy to calculate.

ANTECEDENTES: Con el incremento de la proctocolitis alérgica inducida por proteínas de la dieta (PAIPD), son necesarios estudios que aclaren su fisiopatología y determinar marcadores no invasivos y sencillos para el diagnóstico y la evaluación del desarrollo de tolerancia. No hallamos estudios publicados sobre la función del índice de neutrófilos/linfocitos (INL) y el volumen plaquetario medio (VPM), que son marcadores no invasivos fácilmente medibles, en pacientes con PAIPD. OBJETIVOS: Determinar la relación entre el INL y el VPM con el diagnóstico y desarrollo de tolerancia en niños con PAIPD. MÉTODOS: Estudio transversal retrospectivo, los datos se obtuvieron del sistema de registros médicos, los síntomas y los resultados de laboratorio de los pacientes con diagnóstico de PAIPD fueron controlados en los consultorios de alergia y gastroenterología. Se compararon valores del hemograma al momento del diagnóstico con el grupo de niños sanos de edad y sexo similares. RESULTADOS: Entre los 59 pacientes con diagnóstico de PAIPD, los varones representaron el 47,4% y las niñas, el 52,6%. El VPM y el volumen plaquetario relativo (VPR) eran significativamente más altos entre los pacientes con PAIPD en comparación con el grupo de referencia (n: 67) (p < 0,001). Asimismo, VPM y el VPR fueron significativamente elevados en pacientes que no desarrollaron tolerancia comparados con los que la desarrollaron (p= 0,01). Con el INL no hubo diferencias entre los grupos. CONCLUSIONES: El VPM y el VPR se consideraron marcadores adecuados para predecir el pronóstico de los pacientes con PAIPD dado que son rápidos, costo-efectivos y fáciles de medir.

Can mean platelet volume and neutrophil-to-lymphocyte ratio be biomarkers of acute exacerbation of bronchiectasis in children?

INTRODUCTION: Bronchiectasis (BE) is a parenchymal lung disease evolving as a result of recurrent lung infections and chronic inflammation. Although it has been shown in adult studies that mean platelet volume (MPV) and neutrophil-to-lymphocyte ratio (NLR) can be used as biomarkers of airway inflammation, knowledge is limited in the paediatric age group. The aim of our study is to investigate the potential of MPV and NLR as biomarkers that may indicate acute exacerbations of non-cystic fibrosis BE in children. MATERIAL AND METHODS: Children with non-cystic fibrosis BE (n = 50), who were followed in the division of Paediatric Pulmonology of our hospital between June 2010 and July 2015, were involved in the present retrospective cross-sectional study. Haemogram values during acute exacerbations and non-exacerbation periods, and a control group were compared. RESULTS: In children with bronchiectasis, the average leukocyte count (p < 0.001), platelet count (p = 0.018), absolute neutrophil count (p < 0.001), and NLR (p < 0.001) were higher, as expected, when compared with the control group. NLR values, in the period of acute exacerbation were significantly higher than the values of both the non-exacerbation periods (p = 0.02) and the control group (p < 0.001). In contrast, MPV values in the period of acute exacerbation did not exhibit a significant difference from those of non-exacerbation periods (p = 0.530) and the control group (p = 0.103). CONCLUSIONS: It was concluded that leukocyte count, platelet count, absolute neutrophil count, and NLR can be used to show chronic inflammation in BE, but only NLR and absolute neutrophil count can be used as biomarkers to show acute exacerbations.

Complicaciones pulmonares de la neumonía química: A propósito de un caso / Pulmonary complications of chemical pneumonía; a case report

La aspiración de hidrocarburos puede causar un daño significativo a los pulmones al inducir una respuesta inflamatoria, alveolitis exudativa hemorrágica y pérdida de la función del tensioactivo pulmonar. El efecto secundario más grave de la aspiración de hidrocarburos es la neumonía por aspiración. Anteriormente se han notificado casos de neumotórax, neumatocele, síndrome de dificultad respiratoria aguda (SDRA), absceso pulmonar, fístula broncopleural, derrame pleural bilateral hemorrágico y pioneumotórax. En este artículo presentamos el caso de un paciente hospitalizado debido a neumonía por aspiración que desarrolló pleuritis y neumotórax después de ingerir disolvente para pintura. Se presenta este caso ya que raramente se ha informado en niños como causa de complicaciones pulmonares diferentes. Es necesario evaluar integralmente a los pacientes con complicaciones asociadas a la intoxicación por hidrocarburos. Debe evitarse el alta hospitalaria temprana de los pacientes, quienes deben ser controlados durante, al menos, 48 horas, aunque no tengan síntomas respiratorios. Debe considerarse que los pacientes con neumonía química pueden tener complicaciones pulmonares graves.

Hydrocarbon aspiration (HA) can cause significant lung disease by inducing an inflammatory response, hemorrhagic exudative alveolitis, and loss of surfactant function. The most serious side effect of HA is aspiration pneumonia. Pneumothorax, pneumatocele, acute respiratory distress syndrome (ARDS), pulmonary abscess, bronchopleural fistula, bilateral hemorrhagic pleural effusion and pyopneumothorax were previously reported. Hereby we report a patient hospitalized due to aspiration pneumonia who developed pleurisy and pneumothorax after drinking paint thinner. It is presented as it was seldom reported in children to cause distinct pulmonary complications. Patients with complaints associated withhydrocarbon poisoning must be fully evaluated. They must not be discharged from the hospital early and must be followed for at least 48 hours even if they don't have respiratory symptoms. It should be kept in mind that severe pulmonary complications can develop in patients with chemical pneumonia.

Pulmonary complications of chemical pneumonía: a case report. / Complicaciones pulmonares de la neumonía química: a propósito de un caso.

Hydrocarbon aspiration (HA) can cause significant lung disease by inducing an inflammatory response, hemorrhagic exudative alveolitis, and loss of surfactant function. The most serious side effect of HA is aspiration pneumonia. Pneumothorax, pneumatocele, acute respiratory distress syndrome (ARDS), pulmonary abscess, bronchopleural fistula, bilateral hemorrhagic pleural effusion and pyopneumothorax were previously reported. Hereby we report a patient hospitalized due to aspiration pneumonia who developed pleurisy and pneumothorax after drinking paint thinner. It is presented as it was seldom reported in children to cause distinct pulmonary complications. Patients with complaints associated withhydrocarbon poisoning must be fully evaluated. They must not be discharged from the hospital early and must be followed for at least 48 hours even if they don't have respiratory symptoms. It should be kept in mind that severe pulmonary complications can develop in patients with chemical pneumonia.

La aspiración de hidrocarburos puede causar un daño significativo a los pulmones al inducir una respuesta inflamatoria, alveolitis exudativa hemorrágica y pérdida de la función del tensioactivo pulmonar. El efecto secundario más grave de la aspiración de hidrocarburos es la neumonía por aspiración. Anteriormente se han notificado casos de neumotórax, neumatocele, síndrome de dificultad respiratoria aguda (SDRA), absceso pulmonar, fístula broncopleural, derrame pleural bilateral hemorrágico y pioneumotórax. En este artículo presentamos el caso de un paciente hospitalizado debido a neumonía por aspiración que desarrolló pleuritis y neumotórax después de ingerir disolvente para pintura. Se presenta este caso ya que raramente se ha informado en niños como causa de complicaciones pulmonares diferentes. Es necesario evaluar integralmente a los pacientes con complicaciones asociadas a la intoxicación por hidrocarburos. Debe evitarse el alta hospitalaria temprana de los pacientes, quienes deben ser controlados durante, al menos, 48 horas, aunque no tengan síntomas respiratorios. Debe considerarse que los pacientes con neumonía química pueden tener complicaciones pulmonares graves.

Coexistence of hereditary angioedema in a case of familial Mediterranean fever with partial response to colchicine.

Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic disease characterised by episodes of edema in various parts of the body, including the extremities, face, and airway. The disease is usually associated with attacks of abdominal pain. On the other hand, familial Mediterranean fever (FMF) is an inherited condition characterised by recurrent episodes of painful inflammation in the abdomen, chest, or joints. In this report, we present a child with FMF and undiagnosed HAE, which made him a partial responder to colchicine treatment. Consequently, HAE must be considered in differential diagnosis of cases in which a partial response is obtained from FMF treatment, particularly in countries where FMF is frequently encountered, because early diagnosis of HAE can facilitate prevention of life-threatening complications, such as upper airway obstruction. To our knowledge, our patient is the first patient reported in the literature with the diagnosis of HAE and FMF together.