scholarly journals NEUROENDOCRINE TUMOR IN A CHILD WITH COMMON VARIABLE IMMUNODEFICIENCY

2020 ◽  
Vol 38 ◽  
Author(s):  
Pedro de Souza Lucarelli Antunes ◽  
Heloísa Gabriel Tersariol ◽  
Mainã Marques Belém Veiga ◽  
Maria Conceição Santos de Menezes ◽  
Fabíola Del Carlo Bernardi ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Common Variable Immunodeficiency ◽  
Pediatric Patients ◽  
Medical Literature ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Immunological Investigation ◽  
One Year ◽  
To Receive ◽  
Common Variable

ABSTRACT Objective: To report a case of a child with primary immunodeficiency who at eight years developed digestive symptoms, culminating with the diagnosis of a neuroendocrine tumor at ten years of age. Case description: One-year-old boy began to present recurrent pneumonias in different pulmonary lobes. At four years of age, an immunological investigation showed a decrease in IgG and IgA serum levels. After the exclusion of other causes of hypogammaglobinemia, he was diagnosed with a Common Variable Immunodeficiency and started to receive monthly replacement of human immunoglobulin. The patient evolved well, but at 8 years of age began with epigastrium pain and, at 10 years, chronic persistent diarrhea and weight loss. After investigation, a neuroendocrine tumor was diagnosed, which had a rapid progressive evolution to death. Comments: Medical literature has highlighted the presence of gastric tumors in adults with Common Variable Immunodeficiency, emphasizing the importance of early diagnosis and the investigation of digestive neoplasms. Up to now there is no description of neuroendocrine tumor in pediatric patients with Common Variable Immunodeficiency. We believe that the hypothesis of digestive neoplasm is important in children with Common Variable Immunodeficiency and with clinical manifestations similar to the case described here in the attempt to improve the prognosis for pediatric patients.

scholarly journals The many faces of common variable immunodeficiency

Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 301-305 ◽  
Author(s):  
Charlotte Cunningham-Rundles
Keyword(s):  
Common Variable Immunodeficiency ◽  
Clinical Manifestations ◽  
Chronic Infections ◽  
Patient Registries ◽  
Biologically Relevant ◽  
Inflammatory Conditions ◽  
Large Patient ◽  
Immune Defect ◽  
Autoimmune Cytopenias ◽  
Common Variable

Abstract Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune diseases, and an increased incidence of cancer and lymphoma. For all of these reasons, the disease phenotype is both heterogeneous and complex. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in separating groups of subjects into biologically relevant categories. CVID consists of 2 phenotypes, 1 in which infections are the characteristic and another in which impressive inflammatory and/or hematologic complications also develop, including lymphadenopathy, splenomegaly, autoimmune cytopenias, enteropathy, and/or and granulomatous disease. These phenotypes appear to be stable, are related to immunologic and inflammatory markers, and are predictive of outcomes. This review outlines current understanding about this syndrome based on studies of large cohorts, highlighting the evaluation and treatment of complications and, in particular, the autoimmune and inflammatory conditions that affect these patients.

Increased Serum Levels of Soluble CD30 in Patients with Common Variable Immunodeficiency and Its Clinical Implications

2007 ◽  
Vol 28 (1) ◽  
pp. 78-84 ◽  
Author(s):  
Nima Rezaei ◽  
Mostafa Haji-Molla-Hoseini ◽  
Asghar Aghamohammadi ◽  
Ali Akbar Pourfathollah ◽  
Mina Moghtadaie ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Serum Levels ◽  
Clinical Implications ◽  
Soluble Cd30 ◽  
Common Variable

scholarly journals Proteomic Analysis of Sera from Common Variable Immunodeficiency Patients Undergoing Replacement Intravenous Immunoglobulin Therapy

2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
Giuseppe Spadaro ◽  
Concetta D'Orio ◽  
Arturo Genovese ◽  
Antonella Galeotafiore ◽  
Chiara D'Ambrosio ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Serum Proteins ◽  
Immunoglobulin Therapy ◽  
Normal Subjects ◽  
Serum Levels ◽  
Serum Samples ◽  
Intravenous Immunoglobulin Therapy ◽  
Proteomic Approach ◽  
Adults And Children ◽  
Common Variable

Common variable immunodeficiency is the most common form of symptomatic primary antibody failure in adults and children. Replacement immunoglobulin is the standard treatment of these patients. By using a differential proteomic approach based on 2D-DIGE, we examined serum samples from normal donors and from matched, naive, and immunoglobulin-treated patients. The results highlighted regulated expression of serum proteins in naive patients. Among the identified proteins, clusterin/ApoJ serum levels were lower in naive patients, compared to normal subjects. This finding was validated in a wider collection of samples from newly enrolled patients. The establishment of a cellular system, based on a human hepatocyte cell line HuH7, allowed to ascertain a potential role in the regulation ofCLUgene expression by immunoglobulins.

scholarly journals Association of clinical manifestations and B cell subsets with Freiburg and Paris classifications in patients with Common Variable Immunodeficiency (CVID).

2015 ◽  
Vol 6 ◽  
Author(s):  
Berrón-Ruiz Laura ◽  
Vargas-Hernández Alexander ◽  
Segura-Méndez Nora ◽  
López-Herrera Gabriela ◽  
Mogica-Martínez Dolores ◽  
...  
Keyword(s):  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Clinical Manifestations ◽  
B Cell Subsets ◽  
Common Variable

scholarly journals Major approaches in early diagnostics of common variable immunodeficiency in adults in Moscow

F1000Research ◽  
2012 ◽  
Vol 1 ◽  
pp. 46 ◽  
Author(s):  
Alexander V Karaulov ◽  
Irina V Sidorenko ◽  
Anna S Kapustina
Keyword(s):  
Common Variable Immunodeficiency ◽  
Structural Damage ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Immunoglobulin Therapy ◽  
Early Diagnostics ◽  
Intravenous Immunoglobulin Therapy ◽  
Early Start ◽  
Treatment Concepts ◽  
Common Variable

Common variable immunodeficiency (CVID) is a primary immunological disease characterized predominantly by hypogammaglobulinemia. The main clinical manifestations are severe recurrent infections that often lead to structural damage of affected organs. The early start of adequate intravenous immunoglobulin therapy has significantly improved the prognosis of this serious disorder. Patients with CVID are also predisposed to autoimmune and lymphoproliferative complications. This article deals with the features of this primary immunodeficiency in adults. Clinical manifestations, immunological features and treatment concepts were gathered during 21 years of observation of such patients in Moscow. The authors suggest early predictive clinical signs of CVID in adults.

scholarly journals Profil Klinis dan Luaran Syok Sepsis pada Pasien Anak yang Dirawat di PICU RSUP Dr. M. Djamil Padang

2021 ◽  
Vol 1 (3) ◽  
pp. 234-238
Author(s):  
Yoga Gandha Prasetya ◽  
Indra Ihsan ◽  
Amirah Zatil Izzah
Keyword(s):  
Septic Shock ◽  
Mortality Rate ◽  
General Hospital ◽  
High Mortality ◽  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Sampling Technique ◽  
Target Population ◽  
Pediatric Emergency ◽  
One Year

Background. Septic shock is a common pediatric emergency with a high mortality rate. Objective. The purpose of this study was to determine the clinical profile and outcome of septic shock in pediatric patients treated at PICU of DR. M. Djamil Padang State General Hospital. Method. This descriptive study used data from medical records, conducted in the Medical Record Department of DR. M. Djamil Padang State General Hospital. The target population was pediatric patients which was diagnosed with septic shock in PICU through 2015 - 2017. Samples were collected by the total sampling technique. Result. During the following study of ninety-one patients septic and forty-eight having septic shock. Children having septic shock were commonly under one year of age, female, low nutritional status, having central neuron system infection of Klebsiella sp, having less than two organ dysfunctions with clinical manifestations of Hyperthermia, Tachycardia, and Tachypnea. Hematological profile found that patients commonly had anemia, leukocytosis, thrombocytopenia, and normal I/T ratio. Shock septic outcomes were found high mortality rate, almost some patients use a ventilator, high inotropic usage, and length of stay in living patients compared to dead. Conclusion. The study concluded that prevalence Septic Shock pediatric still high with higher mortality and also morbidity

scholarly journals Serum B cell activating factor and interleukin 10 levels in common variable immunodeficiency: Relationship with clinical findings

2019 ◽  
Vol 76 (1) ◽  
pp. 42-49
Author(s):  
Radovan Mijanovic ◽  
Sladjana Andrejevic ◽  
Vladimir Jurisic ◽  
Branka Bonaci-Nikolic
Keyword(s):  
Autoimmune Diseases ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Healthy Subjects ◽  
Serum Levels ◽  
Pulmonary Diseases ◽  
Recurrent Infections ◽  
B Cell Activating Factor ◽  
Chronic Pulmonary Diseases ◽  
Common Variable

Background/Aim. Common variable immunodeficiency (CVID) is an immunologically and clinically heterogeneous disorder. Disturbed cytokine production is implicated in dysfunctional immune response. The aim of this study was to investigated B-cell activating factor (BAFF) and interleukin (IL)- 10 levels in CVID patients. Methods. The study included 28 CVID patients diagnosed and followed during a 20-year period (mean follow-up 14.5 years). Control groups consisted of 4 patients with X-linked agammaglobulinemia (XLA) and 21 healthy subjects. According to clinical characteristics, the CVID patients were divided into four groups which partly overlap: chronic pulmonary diseases (n = 21), splenomegaly (n = 13), autoimmune diseases (n = 9) and patients with recurrent infections despite regular intravenous immunoglobulin (IVIg) substitution (n = 4). The serum levels of BAFF and IL-10 were measured by commercial ELISA. Results. The BAFF levels were found to be higher in all CVID patients compared to the healthy controls (p < 0.01). The most significant differences were observed in the patients with pulmonary diseases and splenomegaly (p < 0.0001). Also, concentrations of IL-10 were higher in all CVID patients in comparison with the XLA patients (p < 0.05) and healthy subjects (p < 0.01). A statistically significant positive correlation (r = 0.86; p < 0.01) was found between the levels of BAFF and IL-10 in the CVID patients with autoimmune diseases. We demonstrated that the CVID patients with chronic pulmonary diseases had higher levels of IL-10, while the CVID patients with recurrent infections had higher BAFF concentrations in comparison to the patients without these features (p < 0.05). Conclusion. In spite of the limited number of patients, this is the first report from Serbia, examining the serum levels of BAFF and IL-10 in the CVID patients. Our study showed significantly increased concentrations of serum BAFF and IL-10 in the patients with CVID compared to the healthy subjects. Further studies are needed to confirm our findings that the BAFF levels are more pronounced in patients with recurrent infections while IL-10 levels are higher in patients with chronic pulmonary diseases.

scholarly journals How I treat common variable immune deficiency

Blood ◽  
2010 ◽  
Vol 116 (1) ◽  
pp. 7-15 ◽  
Author(s):  
Charlotte Cunningham-Rundles
Keyword(s):  
Immune Deficiency ◽  
Common Variable Immunodeficiency ◽  
Clinical Manifestations ◽  
Chronic Infections ◽  
Patient Registries ◽  
Biologically Relevant ◽  
Large Patient ◽  
Immune Defect ◽  
Laboratory Biomarkers ◽  
Common Variable

Abstract Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune disease, and an increased incidence of cancer and lymphoma. For all these reasons, the disease phenotype is both heterogeneous and complex. Contributing to the complexity is that patient cohorts are generally small, criteria used for diagnosis vary, and the doses of replacement immune globulin differ. In addition, routines for monitoring patients over the years and protocols for the use of other biologic agents for complications have not been clarified or standardized. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in dissecting groups of subjects into biologically relevant categories. This review presents my approach to the diagnosis and treatment of patients with common variable immunodeficiency, with suggestions for the use of laboratory biomarkers and means of monitoring patients.

Sign in / Sign up

Export Citation Format

Share Document