Publications by authors named "Dino Pietropaolo Cienfuegos"

Introduction: Velocardiofacial syndrome (VCFS) is the most common microdeletion syndrome with an incidence of 1:4000 live births. Its phenotype is highly variable with facial, velopharyngeal, cardiac, endocrine, immunologic and psychiatric abnormalities. It is caused by a microdeletion in chromosome 22q11.

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Background: Although we have epidemiological information on primary immunodeficiencies (PID), the available information is meager in Mexico.

Objective: To provide epidemiological information on the delay in the diagnosis of PID and its correlation to chronic lung damage.

Material And Method: A retrospective, analytical study was done in patients 0-18 year old age diagnosed with PID for 11 years at the HIMFG (Hospital Infantil de Mexico Federico Gomez).

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Article Synopsis
  • Analyzed data from 71 patients with chronic granulomatous disease (CGD) in Latin America, focusing on their clinical presentations, genetic mutations, and adverse reactions to BCG vaccination.
  • Patients experienced severe recurrent infections, especially pneumonia (76.8%) and lymphadenopathy (59.4%), with a mean onset age of 23.9 months and diagnosis at 52.7 months.
  • The majority had mutations in the CYBB gene, with 30% showing adverse reactions to BCG vaccination, suggesting it should be avoided for CGD patients.
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BCG vaccine contains low virulence Mycobacterium bovis bacillus. In Mexico it is given at birth with few reported adverse reactions in immunocompetent children; however, in immuno-compromised patients it can cause serious local or systemic adverse reactions. The most frequent related diseases include chronic granulomatous disease (CGD), human immunodeficiency virus infection (HIV), severe combined immunodeficiency (SCID) and mendelian susceptibility to mycobacterial disease (MSMD).

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Objective: To update the scientific evidence of subcutaneous immunotherapy (SCIT) in children.

Data Sources: PubMed, EMBASE, and known articles.

Study Selection: All publications on SCIT in pediatric patients from January 2006 to April 2011.

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Background: Several international guidelines on immunotherapy exist, but they only apply partially in Mexico. The Mexican guideline of immunotherapy dates from 1998.

Objectives: To establish clinical recommendations and suggestions for Allergy residents and specialists for skin testing and allergen immunotherapy based on evidence and Mexican expert opinion, according to the GRADE system.

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The epidemiology of allergic diseases has not been studied extensively in Mexico. The present study, based on the International Study of Asthma and Allergies in Childhood Phase IIIB survey, reports the prevalence of allergic rhinitis and the associated risk factors in the pediatric population in four cities in northern Mexico. Children (6-7 years old) and adolescents (13-14 years old) in public elementary and secondary schools were surveyed in 2002 and 2003.

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Allergic bronchopulmonary aspergillosis is a world rare disease with a prevalence between 1 and 2%. It presents in moderate-severe asthma and cistic fibrosis patients. The diagnosis is made in the basis of Rossenberg and Greenberg criteria that can be essential or non essential.

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Background: Chronic granulomatous disease (CGD) is a primary phagocyte immunodeficiency. It is often accompanied by an exuberant and aberrant inflammatory response, with granulomata and obstruction of the gastrointestinal and genitourinary tracts and inflammatory bowel disease. Although corticosteroids are successful in managing the obstructive and inflammatory disorders of CGD, they are not ordinarily used for the management of infection because of the possibility of further compromising the patient's immune system.

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