Assessment of vascular damage in children and young adults with Familial Mediterranean Fever.

Familial Mediterranean Fever (FMF) is the most frequent autoinflammatory disease. This study aimed to evaluate the risk of subclinical vascular damage in FMF children, and young adults, using both imaging and laboratory tests. Forty-five FMF patients (mean age 14.3 ± 9.5 years, 33 children) and 44 healthy controls(mean age 13.3 ± 8.6 years, 36 children) were included in the study. The patients were diagnosed according to Tel-Hashomer criteria, were positive for MEFV gene mutation, were treated with colchicine and were evaluated during an attack free-period. The arterial stiffness parameters studied were carotid-femoral pulse wave velocity (PWV), Augmentation Index (Aix), subendocardial viability ratio (SEVR) and carotid intima-media thickness (cIMT). Laboratory parameters, inflammation markers and lipid profile were also evaluated for all participants. There were no significant differences between patients and healthy individuals, as well as in our children population regarding PWV, SEVR, Aix and cIMT. However, significantly higher ESR, CRP and fibrinogen levels were detected in the total population of FMF patients and higher amyloid levels in FMF children, compared to controls. Atherogenic Index of Plasma was significantly higher both in the total patient population and in the subgroup of children, compared to controls. Furthermore, a significant positive correlation between Aix and CRP and a negative correlation between SEVR and ESR became apparent in the pediatric subgroup. Our study demonstrated no significant differences in vascular measurements between FMF patients and controls. The above could be attributed to the regular colchicine treatment, which seems to have a cardioprotective role against vascular damage.

Similar Myocardial Perfusion and Vascular Stiffness in Children and Adolescents with High Lipoprotein (a) Levels, in Comparison with Healthy Controls.

BACKGROUND AND AIMS: This study investigated the possible correlation between elevated lipoprotein (a) (Lp(a)) levels and early vascular aging biomarkers in healthy children and adolescents. METHODS: Twenty-seven healthy children/adolescents, mean age 9.9 ± 3.7 years, with high Lp(a) levels without other lipid abnormalities and 27 age- and sex-matched controls with normal Lp(a) levels, were included in the study. The investigation of possible early vascular aging was assessed by measuring vascular function indices: carotid intima-media thickness (c-IMT), pulse wave velocity (PWV), augmentation index (AIx), and subendocardial viability ratio (SEVR). RESULTS: Although serum lipid values were within normal levels, mean values of total cholesterol and apolipoprotein B were higher in the group of children with high Lp(a) levels than controls (p = 0.006 and p < 0.001, respectively). Vascular function indices did not show significant differences, neither between the 2 groups nor in the subgroups of children with increased Lp(a) levels. These subgroups were defined by the presence or absence of family history of premature coronary artery disease. Lp(a) levels did not show a significant correlation with the other parameters studied, both regarding the whole sample (patients and controls), as well as in the subgroups of elevated Lp(a) levels. However, in the group of children with high Lp(a) levels, c-IMT and PWV were positively correlated with diastolic blood pressure (r = 0.427, p = 0.026 and r = 0.425, p = 0.030, respectively), while SEVR was negatively correlated with AIx (r = -0.455, p = 0.017). CONCLUSIONS: Healthy children and adolescents with high Lp(a) levels do not yet have impaired vascular indices, compared to controls. However, in order to prevent early atherosclerosis, it is crucial to early identify and follow up children with high Lp(a) levels and positive family history of premature coronary disease or other cardiovascular risk factors.

Familial Mediterranean fever and atherosclerosis in childhood and adolescence.

Familial Mediterranean fever is a chronic inflammatory disease characterized by periodic and self-limited episodes of fever and aseptic polyserositis. Although colchicine treatment has altered the course of the disease, it is believed that subclinical inflammation is still present, leading to endothelial dysfunction and atherosclerosis in the course of time. In this review, following the published recommendations, we queried online databases such as MEDLINE Pubmed, Scopus, and Web of science for peer-reviewed studies and reviews written in English language, using the following keywords: familial Mediterranean fever, children, endothelial dysfunction, atherosclerosis, cardiovascular disease. The objective of this review is to highlight the correlation between familial Mediterranean fever and atherosclerosis, and moreover to describe new serum inflammatory markers and non-invasive methods of endothelial dysfunction, to detect the atherosclerosis process early starting from childhood.

Comorbid presentation of syringomyelia and Guillain-Barre syndrome, attributed to mycoplasma, in a 6-year-old female patient.

Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord that can cause loss of sensation and muscle spasticity. Guillain-Barre syndrome (GBS) is a postinfection autoimmune disease, classified as an acute polyneuropathy. This report describes the emergency admission of a 6-year-old girl presenting with sudden pallor and pain in both lower limbs. The patient's reflexes were normal, as were the results of her sonography, radiography and biochemical tests; however, spinal MRI revealed extensive compartmentalised syringomyelia extending from C2 to T3. A sensory and motor nerve conduction study revealed a demyelinating type motor polyneuropathy which, along with positive Mycoplasma pneumoniae test, was suggestive of GBS. Intravenous immunoglobulin infusion showed excellent results. In conclusion, we report a rare paediatric case of syringomyelia coexisting with GBS. It is important to bear in mind the possibility of other coexisting diseases even if MRI reveals definitive characteristics of another condition.