The spur occasionally seen in a left common iliac vein was investigated by anatomical and histological examination of cadavers so the occurrence mechanism could be discussed. Spurs were found in six cases of the 28 cadavers (21.4%) and they were classified into few different kinds of composition of endosporia, tunica media and adventitia. It is considered that there may be different formation mechanisms and stages even in cases of similar anatomical finding. (English translation of J Jpn Coll Angiol 2013; 53: 43-47).
McArdle disease (glycogen storage disease type V) is a rare hereditary metabolic myopathy. It can be overlooked clinically because it often presents as chronic asymptomatic hypercreatine phosphokinasemia (hyperCKemia). However, vigorous exercise or infections can trigger severe rhabdomyolysis. We present the case of a patient with long-term idiopathic hyperCKemia who, after contracting an upper respiratory tract infection, developed severe rhabdomyolysis and acute kidney injury. Upon hemodialysis, his renal function recovered and CK levels fell to below baseline, and maintenance therapy with vitamin B6 was also started. A molecular diagnosis of McArdle disease was subsequently made. Whole-exome sequencing revealed homozygous c1538delG (p.Asp511Thr fs*28) mutations in the PYGM gene, which was a novel mutation. Therefore, when investigating idiopathic hyperCKemia, glycogen storage disorders should also be considered.
Objectives This study aimed to clarify the positional relationship between the left brachiocephalic vein and its surrounding vessels and to analyse the association between this positional relationship and ageing. Method Chest contrast-enhanced computed tomography was performed for 100 adults. The contact number between left brachiocephalic vein and surrounding vessels (aorta, brachiocephalic artery, left common carotid artery and left subclavian artery) was determined. The correlations of ageing with the cross-sectional areas of left brachiocephalic vein crossing brachiocephalic artery and left common carotid artery and peripheral end of left brachiocephalic vein were analysed. Results LBV was in contact with aorta in 19, brachiocephalic artery in 97, left common carotid artery in 90 and left subclavian artery in 21 patients. There were significant negative correlations of ageing with the cross-sectional areas of left brachiocephalic vein crossing brachiocephalic artery and left common carotid artery and peripheral end of left brachiocephalic vein. Conclusions Brachiocephalic artery and left common carotid artery have easy contact with left brachiocephalic vein. There was a negative relationship between the cross-sectional area of left brachiocephalic vein and age.
BackgroundComplement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance.Case presentationWe herein report a case of monoclonal gammopathy-induced DDD that improved histologically and clinically with chemotherapy including bortezomib. Our case is the first in which treatment response can be linked to the histological response. Potential pathological insights are also discussed.ConclusionsRapid and efficient chemotherapy has the potential to limit renal damage in monoclonal gammopathy-associated DDD.
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