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1.
Allergol Immunopathol (Madr) ; 47(5): 499-505, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31377030

RESUMO

INTRODUCTION AND OBJECTIVES: Vitamin D plays a role in the immune system, however studies regarding this are scarce. This study aimed to evaluate the nutritional status of vitamin D in patients with Common Variable Immunodeficiency (CVID) or Ataxia-Telangiectasia (A-T) and to relate it to body composition, inflammatory and bone metabolism markers. PATIENTS AND METHODS: This is a cross-sectional and controlled study involving 24 patients of both sexes (59.3% male), aged 8-56 years, with CVID (n=15) or A-T (n=9). The following variables were evaluated: body mass index (BMI), 25-hydroxyvitamin D (25 (OH) D), hepatic profile, parathormone, calcium, phosphorus, alkaline phosphatase, interleukin 6 and high-sensitivity C-reactive protein. RESULTS: The median age was 26.0 years. A deficiency of 25 (OH) D was found in four A-T patients (44%) and two CVID patients (13%). Nine patients with CVI (60%) and six with A-T (66.7%) were overweight and underweight, respectively. There was a negative correlation between vitamin D and fat mass in the CVID group, and vitamin D and BMI in the A-T group. Vitamin D was negatively associated with the percentage of total fat among the patients (ß - 0.842, 95% CI: -1.5-0.17, p=0.015), R2=0.21, after adjusting for sex and age. CONCLUSION: Vitamin D deficiency occurred in a quarter of the patients although there was no difference between the patient and the control group; without association with bone and inflammation biomarkers. The percentage of fat and BMI were negatively associated with the concentrations of 25 (OH) D.


Assuntos
Ataxia Telangiectasia/metabolismo , Imunodeficiência de Variável Comum/metabolismo , Vitamina D/metabolismo , Adolescente , Adulto , Biomarcadores/metabolismo , Índice de Massa Corporal , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Hormônio Paratireóideo/metabolismo , Adulto Jovem
2.
Orphanet J Rare Dis ; 12(1): 136, 2017 08 04.
Artigo em Inglês | MEDLINE | ID: mdl-28778179

RESUMO

BACKGROUND: Ataxia telangiectasia (A-T) is a neurodegenerative disease that leads to mitochondrial dysfunction and oxidative stress. Insulin resistance (IR), type 2 diabetes and the risk for development of cardiovascular disease was recently associated as an extended phenotype of the disease. We aimed to assess IR; liver involvement; carotid intima-media thickness (cIMT) and metabolic alterations associated to cardiovascular risk in A-T patients, and relate them with age. RESULTS: Glucose metabolism alterations were found in 54.6% of the patients. Hepatic steatosis was diagnosed in 11/17 (64.7%) A-T patients. AST/ALT ratio > 1 was observed in 10/17 (58.8%). A strong positive correlation was observed between insulin sum concentrations with ALT (r = 0.782, p < 0.004) and age (r = 0.818, p = 0.002). Dyslipidemia was observed in 55.5% of the patients. The apolipoprotein (Apo-B)/ApoA-I ratio (r = 0.619; p < 0.01), LDL/HDL-c (r = 0.490; p < 0.05) and the Apo-B levels (r = 0.545; p < 0.05) were positively correlated to cIMT. CONCLUSIONS: Metabolic disorders implicated in cardiovascular and liver diseases are frequently observed in adolescent A-T patients and those tend to get worse as they become older. Therefore, nutritional intervention and the use of drugs may be necessary.


Assuntos
Ataxia Telangiectasia/metabolismo , Fígado Gorduroso/metabolismo , Hepatopatias/metabolismo , Adolescente , Adulto , Fatores Etários , Ataxia Telangiectasia/fisiopatologia , Aterosclerose/metabolismo , Aterosclerose/fisiopatologia , Índice de Massa Corporal , Espessura Intima-Media Carotídea , Criança , Pré-Escolar , Dislipidemias/metabolismo , Dislipidemias/fisiopatologia , Feminino , Humanos , Resistência à Insulina/fisiologia , Hepatopatias/fisiopatologia , Masculino , Fatores de Risco , Adulto Jovem
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