Síndrome de Berardinelli-Seip: identificação de anormalidades imunológicas e a relação com hipoleptinemia
The Berardinelli-Seip syndrome (SBS) is a rare autosomal recessive disease characterized by a near complete absence of adipose tissue and metabolic disorders such as hypertriglyceridemia, hyperlipidemia and diabetes mellitus. The reduction of serum leptin concentration is another important finding i...
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| Formato: | Dissertação |
| Idioma: | pt_BR |
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Universidade Federal do Rio Grande do Norte
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| Endereço do item: | https://repositorio.ufrn.br/handle/123456789/44817 |
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| Resumo: | The Berardinelli-Seip syndrome (SBS) is a rare autosomal recessive disease
characterized by a near complete absence of adipose tissue and metabolic disorders
such as hypertriglyceridemia, hyperlipidemia and diabetes mellitus. The reduction of
serum leptin concentration is another important finding in the SBS and is related to
changes in adipocyte function. Leptin is an adipocytokine that plays an important role
in the regulation of various systems, including the immune system, acting directly or
indirectly on immunity. This study aimed to identify the presence of immunological
abnormalities and evaluate the relationship between changes found and leptin
deficiency. The study was approved by the Ethics Committee and were included 28
subjects, 10 males and 18 females, all diagnosed with SBS. Analyses were
performed biochemical serum and leptin, in addition to evaluation of hematologic
parameters, immunoglobulin dosages (IgA, IgG, IgM and IgE), complement proteins
(C3 and C4) and the count of lymphocyte subpopulations (T lymphocytes, B and
natural killer cells) using immunophenotyping by flow cytometry. All study subjects
had significantly lower leptin averaging 1.00±0,45ng/mL. Immunological evaluation
showed an increase in the concentrations of immunoglobulins and complement
proteins compared with serum leptin was no statistically significant difference in IgA,
IgM, C3 and C4. In the assessment of immunophenotype was no statistically
significant difference with B lymphocyte count (CD19+) (p<0,05) compared with
leptin. The results showed alterations in innate and adaptive immune response. The
changes found may involve the development of various diseases, contributing mainly
to the increased risk of infections. Additional studies are needed to confirm and assist
in the investigation of the effect of leptin on immunity. |
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