Introduction: Given the increasing incidence of chronic degenerative diseases related to changes in tissues, the availability of diagnostic tools with greater accuracy in the estimation of body composition (BC) has become necessary. Interpreting the BC values of individuals requires reference data obtained from a healthy population with the same ethnicity, to identify individuals at risk for the development of negative health outcomes.
Objective: Generate reference values (RV) of body composition (BC) for Mexican children and adolescents.
The relevance of molecular mechanisms governing mitochondrial proteostasis to the differentiation and function of hematopoietic and immune cells is largely elusive. Through dissection of the network of proteins related to HCLS1-associated protein X-1, we defined a potentially novel functional CLPB/HAX1/(PRKD2)/HSP27 axis with critical importance for the differentiation of neutrophil granulocytes and, thus, elucidated molecular and metabolic mechanisms underlying congenital neutropenia in patients with HAX1 deficiency as well as bi- and monoallelic mutations in CLPB. As shown by stable isotope labeling by amino acids in cell culture (SILAC) proteomics, CLPB and HAX1 control the balance of mitochondrial protein synthesis and persistence crucial for proper mitochondrial function.
View Article and Find Full Text PDFIntroduction: Clinical assessment of bone health by Dual-Energy X-ray Absorptiometry (DXA) in the paediatric population requires robust reference values. The International Society for Clinical Densitometry (ISCD) recommends that country/regional reference values ideally should be used to improve precision in bone health assessment.
Objective: The aim of this study was to provide reference values for relevant bone health variables for healthy Mexican children and adolescents aged 5 to 18 years.
Background: Allergen immunotherapy (AIT) has a longstanding history and still remains the only disease-changing treatment for allergic rhinitis and asthma. Over the years 2 different schools have developed their strategies: the United States (US) and the European. Allergen extracts available in these regions are adapted to local practice.
View Article and Find Full Text PDFPompe disease is a lysosomal storage disorder caused by malfunctions of the acid alpha-glucosidase (GAA) enzyme with a consequent toxic accumulation of glycogen in cells. Muscle wasting and hypertrophic cardiomyopathy are the most common clinical signs that can lead to cardiac and respiratory failure within the first year of age in the more severe infantile forms. Currently available treatments have significant limitations and are not curative, highlighting a need for the development of alternative therapies.
View Article and Find Full Text PDFOverweight (OW) and obesity (OB) during childhood/adolescence are major public health problems in Mexico. Several obesogenic lifestyle (OL) risk factors have been identified, but the burden and consequences of them in Mexican children/adolescents remain unclear. The objective of this study was to estimate the prevalence of OL components and describe their relationships with adiposity, and OW/OB.
View Article and Find Full Text PDFSevere combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are the only curative treatments available, and the best-known prognostic factors for success are age at diagnosis, age at hematopoietic stem cell transplantation, and the comorbidities that develop in between. There are no evidence-based guidelines for standardized clinical care for patients with SCID during the time between diagnosis and definitive treatment, and we aim to generate a consensus management strategy on the supportive care of patients with SCID.
View Article and Find Full Text PDFBackground: In Mexico, allergen immunotherapy (AIT) and immunotherapy with hymenoptera venom (VIT) is traditionally practiced combining aspects of the European and American school. In addition, both types of extracts (European and American) are commercially available in Mexico. Moreover, for an adequate AIT/VIT a timely diagnosis is crucial.
View Article and Find Full Text PDFBackground: Peritoneal dialysis is the most frequent dialysis method in children, and peritonitis is a frequent complication. The responsible organisms differ between nosocomial and community acquired peritonitis, they cause longer hospitalization time, and can lead to dialysis failure.
Objective: The aim of the study was to describe the risk factors associated with nosocomial peritonitis in children with end-stage renal disease undergoing dialysis treatment.