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Co‐existence of renovascular hypertension, polyarteritis nodosa, antiphospholipid syndrome and methylenetetrahydrofolate reductase mutation
Author(s) -
Yildiz Bilal,
Cetin Nuran,
Kural Nurdan,
Kaya Tamer,
Akcar Nevbahar
Publication year - 2013
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12135
Subject(s) - medicine , methylenetetrahydrofolate reductase , polyarteritis nodosa , vasculitis , gastroenterology , magnetic resonance angiography , renal artery stenosis , thrombosis , hypertensive encephalopathy , cardiology , magnetic resonance imaging , renal artery , kidney , radiology , biochemistry , chemistry , disease , genotype , blood pressure , gene
We present a hypertensive child with a co‐existence of polyarteritis nodosa, anti‐phospholipid antibodies (a PL ), methylenetetrahydrofolate reductase ( MTHFR ) mutation and increased lipoprotein a level. Elevated renin, aldosterone and a PL levels, micro‐aneurysms, occlusion and thrombosis at left and right renal artery were found. Anti‐hypertensive agents, prednisolone and pulse cyclophosphamide therapy were started and a stent was inserted in the left renal artery. Two months later, brain magnetic resonance imaging/magnetic resonance imaging angiography showed acute infarct area of the left parietofrontal lobe and middle cerebral artery stenosis. We found bilateral peripheral neuropathy, persistent a PL and elevated L p(a) level and heterozygous A 1298 C / MTHFR mutation. Intravenous immunoglobulin and low‐molecular‐weight heparin treatment was added. In conclusion, our observation suggests that in patients with systemic vasculitis, such as polyarteritis nodosa, a PL are probably associated with greater thrombotic risks. The investigation of the LP (a) levels and MTHFR mutations as a synergic pro‐coagulant effect might also be considered for determining patients with vasculitis at risk for severe thrombotic events.

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