Immunologic Heterogeneity in 2 Cartilage-Hair Hypoplasia Patients With a Distinct Clinical Course

Introduction: Cartilage-hair hypoplasia (CHH) syndrome is a rare autosomal recessive syndrome associated with skeletal dysplasia, varying degrees of combined immunodeficiency (CID), short stature, hair hypoplasia, macrocytic anemia, increased risk of malignancies, and Hirschsprung disease. Purpose: To provide clinical and immunological insights obtained from 2 unrelated patients who displayed clinical characteristics of CHH. Methods: Two patients with suspected CHH syndrome due to skeletal dysplasia and immunodeficiency underwent an immunological and genetic work-up using flow cytometry, next-generation sequencing (NGS) of the immune repertoire, and Sanger sequencing to identify the underlying defects. Results: Patient 1 presented with low birth weight and skeletal dysplasia. Newborn screening was suggestive of T-cell immunodeficiency, as T-cell receptor excision circle levels were undetectable. Both the T-cell receptor (TCR) Vß and TCR-g (TRG) repertoires were restricted, with evidence of clonal expansion. Genetic analysis identified compound heterozygous RMRP variants inherited from both parents. Patient 2 presented with recurrent lung and gastrointestinal infections, skeletal dysplasia, failure to thrive, and hepatomegaly. The polyclonal pattern of the TCRß repertoire was normal, with only slight overexpression of TCR-ßV20 and restricted expression of Vßs. TRG expressed a normal diverse repertoire, similar to that of the healthy control sample. Genetic analysis identified biallelic novel regulatory variants in RMRP. Both parents are carriers of this mutation. Conclusion: Our findings demonstrate how the immunological work-up, supported by genetic findings, can dramatically change treatment and future outcome in patients with the same clinical syndrome (AU)

Diagnosis of primary immunodeficiency diseases in pediatric patients hospitalized for recurrent, severe, or unusual infections

Background: Primary immunodeficiency diseases (PID) usually presents itself with recurrent, severe, and unusual infections, along with autoimmunity and various other malignancies. But, the diversity of PID often makes the diagnosis of patients difficult for physicians other than clinical immunologists. This study aimed to describe the characteristics of patients diagnosed with PIDs during the inpatient treatment for infectious diseases, and to highlight the cases in which a PID diagnosis should be considered.Methods: The clinical, immunological, and molecular features of 81 pediatric patients treated for infectious diseases, who were diagnosed with a PID during hospitalization was retrospec-tively analyzed. The diagnosis was based on the PID criteria of the International Union of Immunological Societies.Results: The five main PID sub-types were identified. Predominantly, antibody deficiencies were the most common (61.7%) group. The average delay in diagnosis was 34.6 months, and the positive family history rate was 24.7%, while the consanguineous marriage rate was 45.7%. Around thirty-five (43%) patients were found to have mutated PID-related genes. While lower respiratory tract infections were the most common symptom, a fever of unknown origin was another remarkable diagnosis. Eight (9.9%) patients underwent allogeneic hematopoietic stem cell transplantation.Conclusions: Clinicians should consider a PID diagnosis, especially in the cases of recurrent, severe, or atypical infections. Increased knowledge of the alarm features of PID can promote early diagnosis (AU)

Niveles séricos de inmunoglobulinas, componentes 3 y 4 del complemento, alelo HLA-B27 y espondiloartropatía en pacientes con uveítis anterior no infecciosa / Serum Immunoglobulin Levels, Complement Components 3 and 4, HLA-B27 Allele and Spondyloarthropathy in Patients with Non-Infectious Anterior Uveites

Objetivo: Identificar la relación entre los niveles séricos de inmunoglobulinas, los componentes 3 y 4 del complemento, la presencia del alelo HLA-B27 y el diagnóstico de espondiloartropatía en pacientes con uveítis anterior no infecciosa. Materiales y métodos: Se incluyeron 197 pacientes con diagnóstico de uveítis anterior no infecciosa. Se determinaron las concentraciones de inmunoglobulinas séricas y proteínas C3 y C4 del complemento mediante turbidimetría. Se recogieron los antecedentes personales de sospecha de inmunodeficiencia, complicaciones oftalmológicas, de artralgias, antecedentes familiares de espondiloartropatías y la presencia del alelo HLA-B27. Resultados: Los antecedentes familiares de espondiloartropatías, artralgias axiales y complicaciones oftalmológicas fueron más frecuentes en los pacientes positivos a HLA-B27 (p=0,0005, p≤0,0001 y p≤0,0001, respectivamente) y en aquellos con diagnóstico de espondiloartropatías (p≤0,0001, p≤0,0001 y p≤0,0001, respectivamente). Los antecedentes personales de sospecha de inmunodeficiencia, sepsis recurrentes y alteraciones gastrointestinales, se asociaron a la presencia del alelo HLA-B27 (p≤0,0001 y p=0,0240, respectivamente) y al diagnóstico de espondiloartropatía (p=0,0492 y p=0,0017, respectivamente). Se observó disminución de las IgG (χ2=18,5; OR 5,03; IC 95% 2,32-10,89; p=0,0001) e IgM (OR 7,13; IC 95% 1,40-36,4; p=0,0128) en pacientes positivos para el alelo HLA-B27 y en aquellos con diagnóstico de espondiloartropatías (p=0,0364 y p=0,0028, respectivamente). La disminución de las proteínas C3 (OR 4,82; IC 95% 1,35-17,11; p=0,0328) y C4 (OR 9,09; IC 95% 2,13-38,88; p=0,0074) se asoció al diagnóstico de espondiloartropatías.(AU)

Objective: To identify the relationship between serum immunoglobulin levels, complement components 3 and 4, the presence of the HLA-B27 allele and diagnosis of spondyloarthropathies in patients with non-infectious anterior uveitis. Materials and methods: The participants were 197 patients with a non-infectious anterior uveitis. The concentrations of serum immunoglobulins, and C3 and C4 proteins of the complement were determined by turbidimetry. The personal history of suspected immunodeficiency, ophthalmological complications, arthralgia, family history of spondyloarthropathies and the presence of the HLA-B27 allele were collected. Results: A family history of spondyloarthropathy, axial arthralgias, and ophthalmological complications were more frequent in HLA-B27 positive patients (P=.0005, P≤.0001, and P≤.0001, respectively) and in patients with spondyloarthropathy diagnoses (P≤.0001, P≤.0001, and P≤.0001, respectively). A personal history of recurrent sepsis, and gastrointestinal abnormalities was associated with the presence of the HLA-B27 allele (P≤.0001, and P=.0240, respectively) and with the diagnosis of spondyloarthropathy (P=.0492, and P=.0017, respectively). IgG decrease was observed (χ2=18.5, OR 5.03, 95% CI 2.32-10.89; P=.0001) and IgM (OR 7.13, 95% CI 1.40-36.4; P=.0128) in patients positive for the HLA-B27 allele and in patients with a diagnosis of spondyloarthropathies (P=.0364 and P=.0028, respectively). The decrease of C3 proteins (OR 4.82; CI 95% 1.35-17.11; P=.0328) and C4 (OR 9.09; CI 95% 2.13-38.88; P=.0074) were associated with a spondyloarthropathies diagnosis. Conclusions: Patients with non-infectious anterior uveitis, positive for the HLA-B27 allele and diagnosed with spondyloarthropathies have alterations in serum immunoglobulin levels and complement components 3 and 4, which could contribute to the perpetuation and worse clinical course of this disease.(AU)

Experience of Autoimmune and autoinflammatory diseases in a Turkish pediatric cohort with primary immunodeficiencies

Background Primary immunodeficiency diseases (PID) are the diseases characterized by a dysfunction of the immune system. Affected patients share a different phenotype such as chronic infections, allergy, autoimmunity, and autoinflammation. Methods In all, 433 children with PID were enrolled in this study. Clinical, laboratory, and demographic data of patients were reviewed retrospectively to investigate autoimmune and autoinflammatory complications. Autoinflammation in all patients with inflammation was confirmed by genetic analysis after excluding infectious etiology. Results Clinical features of 433 PID patients were evaluated retrospectively with long-term follow-up. Autoimmune disorders were identified in 69 (15.9%) patients with PID; 31 (45%) patients had a history of autoimmune disease before diagnosis of PID. The frequency of autoimmunity in immune dysregulation subgroup (76.6%) was higher than other forms of PID. The most common autoimmune manifestations were reported to be Addison’s disease, hypoparathyroidism, and autoimmune hemolytic anemia. Autoinflammation were identified in 22 of the 433 (5.1%) patients with PID, including hyper immunoglobulin D syndrome (n = 9), Aicardi–Goutieres syndrome 1 (n = 6), adenosine deaminase 2 deficiency (n = 3), Blau syndrome (n = 2), tumor necrosis factor (TNF) receptor-associated periodic syndrome (n = 1), and auto-inflammation and phospholipase Cγ2-associated antibody deficiency and immune dysregulation syndrome (n = 1). Conclusions It is important to recognize association between autoimmunity, autoinflammation, and PID, which in the future could be useful for increased awareness and early diagnosis for these diseases (AU)

Syndromic immunodeficiencies: a pediatrician’s perspective on selected diseases

Background: Syndromic immunodeficiencies are a genetically and pathophysiologically heterogeneous group of inborn errors of immunity. These are characterized by multiple extra immune clinical symptoms and a wide range of immunological phenotypes with increased susceptibility to infections, autoimmune phenomena, immune dysregulation, organ-specific pathology, and malignancy. Objective: To increase the pediatricians’ awareness of this multifaceted group of primary immunodeficiencies in children. Methods: A comprehensive review of genetic background and clinical symptomatology of syndromic immunodeficiencies as well as current diagnostic approach and treatment modalities. Results: From the pediatrician’s perspective, an early-life diagnosis of syndromic immunodeficiencies, which is frequently indispensable for successful life-saving immunocorrection, poses a diagnostic challenge. Increased pediatricians’ awareness to recognize signs and symptoms of these diseases in affected children is of paramount importance. Current advances in molecular biotechnology and immunogenetics, resulting in the implementation of newborn screening and new-generation sequencing, provide informative tools for definitive diagnosis and, in many new disease entities, for their definition and genotype–phenotype delineation and correlation. Conclusions: A broad spectrum of clinical phenotypes in children with syndromic primary immunodeficiencies requires pediatrician’s special attention, that is, individualized multidisciplinary approach under the supervision of a clinical immunologist (AU)

Acute pericarditis: A peculiar manifestation of common variable immune deficiency

Common variable immune deficiency (CVID) is known as the most prevalent symptomatic inborn error of immunity associated with autoimmune and inflammatory complications in addition to recurrent infections. In this study, we investigated the prevalence of acute pericarditis as a complication in the past medical history of 337 CVID patients. We found five patients (1.5%) that had experienced acute pericarditis, and described the medical history of three patients (AU)

22q11.2 deletion syndrome: 20 years of experience from two pediatric immunology units and review of clues for diagnosis and disease management

INTRODUCTION AND OBJECTIVES: The purpose of this study was to evaluate patients diagnosed with 22q11.2 deletion syndrome and determine the clues directing to diagnosis and evaluation of immunological findings for excellent management of the disease. MATERIAL AND METHODS: Thirty-three pediatric patients with 22q11.2 deletion syndrome diag­nosed between 1998 and 2019 at Pediatric Immunology Division of Ege University Faculty of Medicine and SBU Izmir Dr Behcet Uz Children's Education and Research Hospital were evaluated. RESULTS: This study includes the largest case series reported from Turkey. Congenital car­diac anomalies were the most common pathology associated with the syndrome (90.9%). Hypocalcemic symptoms were observed in 13 patients (40%). Twenty-two of the 33 (66.6%) patients were diagnosed before two years of age. Autoimmune diseases, dysmorphic facial findings, recurrent infections, growth retardation, and speech impairment were other clues for diagnosis in older patients. Clinical spectrum and immunological abnormalities of this syn­drome are quite variable. All T-cell subset counts were less than 5th percentile below median by age in one patient (3%) and 10 patients had normal all T-cell subset counts (30.3%). Overall, 69.6% of the patients had normal IgG, IgA, and IgM levels and two patients had panhypogam­maglobulinemia. Recurrent infections were revealed in 75.7% of the patients during follow-up. CONCLUSIONS: Presence of cardiac anomaly is more helpful in the diagnosis, especially under two years of age. Patients with immunologically high or standard risk did not show any differ­ence in terms of numbers and severity of infections and autoimmunity

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Activated PI3K-delta syndrome in an Egyptian pediatric cohort with primary immune deficiency

BACKGROUND: Activated Phospho-Inositide 3 (PI3) Kinases Delta syndrome (APDS) can underlie primary immune deficiency. The prevalence and phenotypic characterization of these patients are not well described in Egypt. OBJECTIVES: To describe patients with APDS in hospitalized children with recurrent respiratory tract infections with suspected primary immune deficiency. METHODS: 79 patients were included in the study. E1021K and E525K mutations of PI3K δ chain gene were screened by Sanger sequencing technique. RESULTS: one patient was heterozygous to E1021K mutation; a female child was diagnosed clinically as Combined Immune Deficiency with CD4 and B lymphopenia and markedly deficient IgG and increased IgM. The E525K mutation was not detected in our cohort. CONCLUSIONS: Screening for APDS in patients with recurrent respiratory tract infections with undefined antibody deficiency or combined immune deficiency with or without bronchiectasis is required. These patients need great attention to benefit from the available treatment. Further studies on the Egyptian population are recommended to increase the knowledge about the prevalence and phenotypic characterization of this disease in Egypt

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Autoimmune and inflammatory manifestations in pediatric patients with primary immunodeficiencies and their importance as a warning sign

INTRODUCTION AND OBJECTIVES: As well as increased susceptibility to infections, autoimmune and inflammatory manifestations also eventuate due to dysregulation of immune system in a substantial proportion of patients with primary immunodeficiency (PID). Autoimmune and inflammatory manifestations can occur prior or after diagnosis of PID. This study aimed to evaluate autoimmune and inflammatory complications among all types of PID patients in childhood and to emphasize the importance of these findings as a warning sign to diagnose PIDs. METHODS: Medical records of 1036 patients with PID, followed up between 2003 and 2019, were retrospectively screened for occurrence of autoimmunity and inflammation. During this time, demographic features, autoimmune/inflammatory findings and initial time, genetic mutations, laboratory and clinical follow up findings, treatment regimens and outcomes were recorded. RESULTS: Autoimmune and inflammatory manifestations were observed in 83 patients (10.1%). The median age of autoimmunity initial time was 61.3 ± 53 months. Sixty-seven (80.7%) patients presented with autoimmune and inflammatory manifestations, and these findings had occurred during 16 patients' (19.3%) follow-up. The most common autoimmune manifestations were autoimmune hematologic (51.8%) and endocrine diseases (26.5%). Fifty patients (60.2%) had a single autoimmune/inflammatory manifestation, however 23 patients (27.7%) had two, eight patients (9.6%) had three and two patients (2.4%) had four different types of autoimmune/inflammatory manifestations. The frequency of autoimmune and inflammatory manifestations in phagocyte defects (56%), combined immune deficiencies (53%) and immune dysregulation diseases (52%) were observed higher than other forms of PIDs. During follow-up 13 (15.7%) patients died. CONCLUSION: Autoimmune/inflammatory manifestations are associated with high morbidity in patients with PIDs and may precede the diagnosis of PID in childhood. Therefore, physicians must be aware of underlying possible immune deficiency and patients with known PIDs should be evaluated for autoimmune and inflammatory complications

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Evaluation of the frequency and diagnostic delay of primary immunodeficiency disorders among suspected patients based on the 10 warning sign criteria: A cross-sectional study in Iran

BACKGROUND: Common Variable Immunodeficiency (CVID) is characterized by an impaired antibody production and a higher susceptibility to encapsulated bacterial infections. Lung disease is considered to be the most important cause of morbidity and mortality. METHODS: We analyzed clinical, radiological and functional characteristics in 80 patients with CVID assisted in the Unidad Inmunologia e Histocompatibilidad at Durand Hospital from 1982 to 2018. RESULTS: Of the 80 patients, 55 showed pathologic lung Computed Tomography (CT). Twenty of them (36.4%) showed bronchiectasis; 26 (47.3%) interstitial involvement associated with nodules and adenopathies called GLILD (granulomatous-lymphocytic interstitial lung disease); and nine patients (16.3%) showed other lesions. Nine percent of patients with lung disease showed CT progression; none of them had spirometry worsening. GLILD patients had normal and restrictive patterns in lung function tests, in equal proportions. Two patients - one with GLILD and the other one with bronchiectasis - had an increase in spirometric pattern severity without CT progression. Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP). CONCLUSIONS: GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression

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Evaluation of patients with primary immunodeficiency associated with Bacille Calmette-Guerin (BCG)-vaccine-derived complications

BACKGROUND: Bacille Calmette-Guerin (BCG) vaccination has a great impact on the prevention of severe complications of tuberculosis. However, in patients with primary immunodeficiencies (PID), it can lead to severe complications such as severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial disease. This study highlights the demographics, clinical complications and laboratory parameters among PID patients associated with BCG vaccination side effects. METHODS: One hundred and thirty-seven PID patients with BCGosis were evaluated in this study, based on the complications following BCG vaccination. RESULTS: The mean age of the patients with BCG complications at the time of the first visit was five years. The within-group comparison of patients showed a highly significant incidence of pneumonia and hepatomegaly in severe combined immunodeficiency patients. Furthermore, the immunologic data showed an increase in the overall rates of lymphocytes such as CD3+, CD4+ and CD8 + T cells in Mendelian susceptibility to mycobacterial disease patients. The level of immunoglobulins has also increased in chronic granulomatous disease patients. CONCLUSION: The high rate of undiagnosed PIDs predisposes individuals to a high risk of severe side effects as a result of BCG vaccination, as well as infants that are less than one month of age. Therefore, there is a need for early screening and diagnosis of PIDs before exposing unknown PID status patients to BCG vaccination. The benefits of screening and early diagnosis of PID cannot be overemphasized, especially in patients with a previous family history of immunodeficiency

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Adverse reactions and influencing factors in children with primary immunodeficiencies receiving intravenous immunglobulin replacement

OBJECTIVES: We aimed to determine adverse reactions and influencing factors, within the scope of the number of patients and total infusions, in patients with primary immunodeficiencies receiving intravenous immunoglobulin (IVIG) replacement. MATERIALS AND METHODS: Children with primary immunodeficiencies receiving IVIG replacement in Izmir Dr Behcet Uz Children's Hospital, between June 2014 and June 2016, were included in our study. RESULTS: The total number of the patients receiving IVIG replacement was 145 (37 female, 108 male). The number of total IVIG infusions was 1214. Adverse reactions were observed in 44.8% of the patients and 14.2% of the infusions. Common variable immunodeficiency was the most common diagnosis of the patients and adverse reactions most commonly developed in this group (24.2%). In all infusions the most frequent adverse reaction was headache (7.8%); fever was the most frequent immediate side effect (3.9%), whereas headache was the most common delayed adverse effect (5.1%). By logistic regression analyses, history of adverse reaction to IVIG in previous infusions, existence of concomitant infectious disease, past or family history of atopic disease, to receive IVIG infusion at the first time, or being under 10 years old were found associated with adverse reactions. There was no correlation between the concentration of IVIG preparations and the rate of side-effect development. CONCLUSIONS: In our study no severe adverse reaction to IVIG was observed, but many mild or moderate side effects occurred. Therefore, IVIG indications must be well identified. Patients, family of the patients and health care workers must be informed for adverse reactions

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Executive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies / Resumen ejecutivo del Documento de consenso sobre diagnóstico y manejo de pacientes con inmunodeficiencias primarias

Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and children with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available

Las inmunodeficiencias primarias (IDP) son unas enfermedades raras, frecuentemente infradiagnosticadas y potencialmente fatales. Las manifestaciones clínicas de las IDP pueden ser muy graves y ocasionar secuelas que empeoran la calidad de vida de los pacientes. Tradicionalmente, el tratamiento de las IDP ha sido fundamentalmente de soporte, con excepción del trasplante de progenitores hematopoyéticos y, más recientemente, la terapia génica. El descubrimiento de nuevos mecanismos patogénicos, el desarrollo de nuevas moléculas y fármacos biológicos y los avances en el conocimiento de las bases moleculares de estas enfermedades han abierto oportunidades para el tratamiento de esta afección. El objetivo de este documento es revisar el conocimiento actual y aportar recomendaciones para el diagnóstico y el tratamiento clínico de los pacientes adultos y pediátricos con IDP basado en la evidencia científica disponible y teniendo en cuenta la actual práctica y los retos futuros. Se realizó una revisión sistemática, que justifica los niveles de evidencia para cada recomendación

Documento de consenso para mejorar la adherencia a la farmacoterapia en pacientes con infección por el virus de la inmunodeficiencia humana en tratamiento antirretroviral / Consensus document on enhancing medication adherence in patients with the human immunodeficiency virus receiving antiretroviral therapy

La adherencia al tratamiento en el paciente con infección por el virus de la inmunodeficiencia humana sigue siendo foco de atención de profesionales sanitarios e investigadores. Sin embargo, el perfil del paciente y el arsenal terapéutico disponible han cambiado enormemente en la última década. La adherencia inadecuada, no solo al tratamiento antirretroviral sino también a otros fármacos prescritos, sigue siendo la principal causa de fracaso terapéutico. Existen diversos factores asociados a la mala adherencia y otros que facilitan la misma, de ahí la importancia de identificar y manejar las situaciones que puedan dificultar la adherencia e intentar corregirlas. Asimismo, se debe reevaluar periódicamente la adherencia durante el seguimiento del tratamiento antirretroviral y del resto de los fármacos prescritos. En la actualidad no existe un método único para medir la adherencia de forma fiable. Por ello se hace necesario utilizar varios métodos combinados de fácil realización. Adicionalmente, una buena relación entre el personal sanitario y los pacientes facilita la obtención de una adecuada información sobre la adherencia. Las intervenciones para mejorar la adherencia deben ser multidisciplinares, individualizadas y ajustadas a los nuevos patrones de transmisión de la infección, y es fundamental incluir el control de la adherencia a otros fármacos prescritos al paciente con el virus de la inmunodeficiencia humana. El presente documento actualiza las recomendaciones publicadas en 2008 tras una revisión de la literatura científica, lo que ha permitido emitir unas recomendaciones consensuadas para la mejora de la adherencia al tratamiento. El objetivo principal es ayudar a todos los profesionales sanitarios dedicados al control clínico y terapéutico de los pacientes con el virus de la inmunodeficiencia humana (médicos, farmacéuticos, enfermeras, psicólogos y trabajadores sociales) a mejorar la adherencia a toda la farmacoterapia que tengan prescrita

Adherence to treatment in patients living with HIV remains the focus of attention of health professionals and researchers. However, patient pro-files and the available therapeutic arsenal have changed greatly over the last decade. Inadequate adherence not only to antiretroviral therapy but also to other prescribed drugs remains the main cause of therapeutic failure. There are several factors associated with poor adherence and others that facilitate it, hence the importance of identifying, managing and correcting situations that may hinder adherence. Likewise, adherence should be periodically reassessed during the follow-up of ART and other prescribed drugs. It has so far proved impossible to find a single method capable of providing a reliable measurement of adherence. That is why it is necessary to use a combination of multiple easy-to-implement methods. Additionally a good relationship with the patient facilitates the conveyance of adequate information on adherence. It is currently considered that interventions to improve adherence should be multidisciplinary, individualized and adjusted to the new patterns of infection transmission, and that controlling adherence to other drugs prescribed to patients with HIV should be part of such interventions. This document provides an update on the recommendations published in 2008 based on a review of the scientific literature. The main goal is to help healthcare professionals dedicated to the clinical and therapeutic management of HIV patients (doctors, pharmacists, nurses, psychologists and social workers) improve adherence of such patients to all the drugs prescribed to them as treatment for their HIV infection