Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure

HLA-B SNA antigen: A BW6-associated B locus antigen belonging to the B5 CREG

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HLA‐B POT: a new HLA‐antigen within the B7‐cross reactive group

In this report, a new HLA‐B locus antigen is described (tentatively called POT). The antigen has been identified with antisera against the antigens that belong to the B7‐cross reacting group. In a collaborative study, based on exchange of cells and sera, it was confirmed by population and family studies that the antigen is distinct from B7, Bw42 or Bw73 and is associated with Bw6. In absorption experiments with EBV‐transformed cell lines, the POT‐antigens removes preferentially anti‐B7, Bw42 and Bw73 activity and to a lesser extent antibodies reactive with the B40 CREG‐antigens. Copyright © 1986, Wiley Blackwell. All rights reservedSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Embolization for hemoptysis in chronic thromboembolic pulmonary hypertension: report of two cases and a review of the literature

Hemoptysis is a known complication in patients with bronchial artery hypertrophy due to a variety of chronic pulmonary disorders. Bronchial artery hypertrophy is observed in most patients with chronic thromboembolic pulmonary hypertension (CTEPH), but surprisingly little is known about the incidence of hemoptysis in these patients. In this paper, we report on 2 patients with CTEPH and recurrent severe hemoptysis, who were treated by bronchial artery embolization. One patient recovered and 1 patient died as a consequence of the bleeding. A systematic review revealed 21 studies on the underlying pathology in 1,844 patients with moderate to severe hemoptysis. CTEPH was reported to be the cause of bleeding in 0.1% (n = 2), pulmonary arterial hypertension without chronic thromboembolic disease in 0.2% (n = 4), and acute pulmonary embolism in 0.7% (n = 12) of the patients. In contrast to this, 5 patients (6%) in our own series of 79 CTEPH patients suffered from moderate to severe hemoptysis requiring medical intervention. Severe hemoptysis appears to be an uncommon, but possibly underreported, life-threatening complication in CTEPH patients. As most CTEPH patients require life-long anticoagulants a therapeutic dilemma may ensue. Therefore, we propose that even mild hemoptysis in CTEPH patients warrants prompt evaluation, and treatment by embolization should be offered as first choice in CTEPH patient

Renal denervation of the native kidneys for drug-resistant hypertension after kidney transplantation

There is a strong rationale for renal denervation (RDN) of the native kidneys in kidney transplant recipients with treatment-resistant hypertension. We present a patient with a stable graft function, who underwent RDN for posttransplant therapy-resistant hypertension (24-h ambulatory blood pressure measurement (ABPM) 143/89 mmHg, while compliantly using five different antihypertensive agents). After RDN, BP measurements and orthostatic complaints required withdrawal of two antihypertensive agents and halving a third. At 6 months, ABPM was 134/84 mmHg and allograft function remained unchanged. This case calls for designing well-designed prospective studies on RDN in kidney transplant recipient

Renal sympathetic nerve activity after catheter-based renal denervation

Abstract Background Catheter-based renal sympathetic denervation (RDN) has been considered a potential treatment for therapy resistant hypertension (RHT). However, in a randomized placebo-controlled trial, RDN did not lead to a substantial blood pressure (BP) reduction. We hypothesized that variation in the reported RDN efficacy might be explained by incomplete nerve disruption as assessed by renal 123I–meta-iodobenzylguanidine (123I–mIBG) scintigraphy. Methods In 21 RHT patients (median age 60 years), we performed 123I–mIBG scintigraphy before and 6 weeks after RDN. Additionally, we assessed changes in BP (24 h day, night, and average), plasma- and urinary-catecholamines and plasma renin activity (PRA) before and after RDN. Planar scintigraphy was performed at 15 min and 4 h after 123I–mIBG administration. The ratio of the mean renal (specific) counts vs. muscle (non-specific) counts represented 123I–mIBG uptake. Renal 123I–mIBG washout was calculated between 15 min and 4 h. Results After RDN office-based systolic BP decreased from 172 to 153 mmHg (p = 0.036), while diastolic office BP (p = 0.531), mean 24 h systolic and diastolic BP (p = 0.602, p = 0.369, respectively), PRA (p = 0.409) and plasma catecholamines (p = 0.324) did not significantly change post-RDN. Following RDN, 123I–mIBG renal uptake at 15 min was 3.47 (IQR 2.26–5.53) compared to 3.08 (IQR 2.79–4.95) before RDN (p = 0.289). Renal 123I–mIBG washout did not change post-RDN (p = 0.230). In addition, there was no significant correlation between the number of denervations and the renal 123I–mIBG parameters. Conclusions No changes were observed in renal 123I–mIBG uptake or washout at 6 weeks post-RDN. These observations support incomplete renal denervation as a possible explanation for the lack of RDN efficacy