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We report the case of a 68-year-old man with right intermittent claudication by adventitial cystic disease. We performed resection of the cyst and affected popliteal artery with interposing an autologous vein graft. Intraoperative findings revealed an intimal tear between the cyst and the compressed artery. His symptoms resolved after surgery, and the postoperative course was uneventful. Although adventitial cystic disease with intimal tear is rare, we consider that conventional surgical intervention remains the favorable treatment option for adventitial cystic disease.

To clarify clinical and genetic features of Japanese children with congenital chloride diarrhea (CCD).This was a multi-institutional, retrospective survey of 616 pediatric centers in Japan with identified patients with CCD between 2014 and 2018. Mutations involving SLC26A3 were detected by Sanger sequencing.Thirteen patients met all entry criteria including mutations in SLC26A3, and 14 patients satisfied clinical diagnostic criteria. Homozygous or compound heterozygous mutations in SLC26A3, including 6 novel mutations, were identified in 13 of these 14 patients (93%). The most common (detected in 7 of 13) was c.2063-1gt. Median age at diagnosis was 1 day. Nine of the patients meeting all criteria were diagnosed as neonates (69%). Median follow-up duration was 10 years. When studied, 8 patients had5 stools daily (62%), and all had fewer than in infancy. Only 1 patient had nephrocalcinosis, and 3 (23%) had mild chronic kidney disease. Neurodevelopment was generally good; only 1 patient required special education. Five patients (38%) received long-term sodium, potassium, and chloride supplementation.Early fetal ultrasound diagnosis and prompt long-term sodium, potassium, and chloride supplementation were common management features. Genetic analysis of SLC26A3 provided definitive diagnosis of CCD. In contrast with previously reported localities, c.2063-1gt might be a founder mutation in East Asia.

Purpose To report the early results of use of the Endurant stent graft in the treatment of ruptured abdominal aortic aneurysms (AAAs). Materials and Methods Nine consecutive patients (seven men and two women; mean age, 76 y; range, 65–87 y) underwent endovascular aneurysm repair (EVAR) for a ruptured AAA with the Endurant stent graft between April and December 2012. EVAR was emergent in all cases. Early technical success, clinical success, major complication, and mortality rates were analyzed. Results Intraoperative immediate technical success was achieved in all nine patients. The 30-day clinical success rate was 67% (six of nine patients). The 30-day mortality rate was 33% (three of nine patients). During a mean follow-up of 6 months (range, 3–10 mo), none of the cases required reintervention; there was one late death attributed to probable endograft infection. Conclusions The short-term results of EVAR with the Endurant stent graft in patients with ruptured AAAs are encouraging.

Nephrin is an essential structural component of the glomerular slit diaphragm (SD), a highly organized intercellular junction that constitutes the ultrafiltration barrier of the kidney. Recent studies have identified two additional nephrin-interacting SD proteins (NEPH1 and NEPH2), suggesting that the zipper-like pattern of the SD is formed through complex intra- and intermolecular interactions of these proteins. However, the complexity of the SD structure suggests that additional SD components remain to be discovered. In this study, we identified galectin-1 (Gal-1) as a new component of the SD, binding to the ectodomain of nephrin. Using dual-immunofluorescence and confocal microscopy and dual-immunoelectron microscopy, we found Gal-1 co-localizing with the ectodomain of nephrin at the SD in normal human kidney. By immunoprecipitation and surface plasmon resonance, we confirmed a direct molecular interaction between Gal-1 and nephrin. Moreover, recombinant Gal-1 induced tyrosine phosphorylation of the cytoplasmic domain of nephrin and activation of the extracellular signal-regulated kinase 1/2 in podocytes. We also showed that podocytes are a major site of biosynthesis of Gal-1 in the glomerulus and that the normal expression patterns and levels of Gal-1 are altered in patients with minimal change nephrotic syndrome. Finally, in puromycin aminonucleoside-induced rat nephrosis, an apparent reduction in the levels of Gal-1 and nephrin around the onset of heavy proteinuria was also revealed. Our data present Gal-1 as a new extracellular ligand of nephrin localized at the glomerular SD, and provide further insight into the complex molecular organization, interaction, and structure of the SD, which is an active site of intracellular signaling necessary for podocyte function.