Inline Balloon Occlusion–Assisted Delivery of Ethylene Vinyl Alcohol Copolymer for Peripheral Arterial Applications: A Multicenter Case Series

PurposeTo assess the feasibility, safety, and efficacy of balloon-assisted delivery of ethylene vinyl alcohol copolymer (EVOH) for a range of peripheral arterial applications.Materials and MethodsSix academic medical centers entered retrospective data on 46 consecutive patients (27 men, 19 women; ages, 11–94 y; mean age, 50.3 y) who underwent 60 balloon-assisted EVOH procedures. The cohort was restricted to procedures involving peripheral, nonneural arteries 1–5.5 mm in diameter. Clinical indications included a wide range of vascular pathologic conditions (most commonly arteriovenous malformations [n = 20], renal angiomyolipomas [n = 8], and acute hemorrhage [n = 9]) and targeted visceral and musculoskeletal peripheral arteries. Data collected included sex, age, clinical indication, arterial pathology, arteries embolized, type of occlusion balloon microcatheter, type and concentration of EVOH agent, effectiveness as an embolic backstop, vessels protected, adequacy of EVOH cast penetration, catheter extraction, nontarget embolization, and complications.ResultsBalloon occlusion prevented EVOH reflux in 59 of 60 procedures (98.3%). Nontarget EVOH embolization occurred in 2 procedures (3.3%). Adequate EVOH cast penetration and complete filling of the target pathologic structure were seen in 57 of 60 procedures (95%). Balloon deflation and uneventful extraction occurred in all procedures; small EVOH fragments detached into target arteries in 2 cases. One major (1.7%) and 2 minor (3.3%) complications occurred.ConclusionsBalloon-assisted EVOH embolization of peripheral arteries is feasible, safe, effective, and versatile. The primary advantage of balloon-assisted EVOH embolization is the ability to apply more injection pressure to advance the EVOH cast assertively into the pathologic structure(s).     

Bronchial Artery Embolization in Pediatric Pulmonary Hemorrhage: A Single-Center Experience

PurposeTo explore the safety and effectiveness of bronchial artery (BA) embolization (BAE) in children with pulmonary hemorrhage.Materials and MethodsBetween February 2016 and February 2019, 41 patients (median age, 4 y; interquartile range, 2.3-8 y; median weight, 17.6 kg; interquartile range, 12.3–23.6 kg) underwent BAE. The indication of BAE included massive hemoptysis in 10 patients (24.4%), recurrent hemoptysis in 18 patients (43.9%), and refractory anemia in 13 patients (31.7%). The main etiology of pulmonary hemorrhage included pulmonary hemosiderosis (58.5%), congenital heart disease (17.1%), and infection (14.6%). A retrospective review was conducted of clinical outcomes of BAE.ResultsThere were 44 embolization sessions, with a total of 137 embolized vessels. Pulmonary hemorrhage was caused by BAs in 30 cases, nonbronchial systemic arteries plus BAs in 10, and nonbronchial systemic arteries in 1. Embolic particles were used in 30 cases (24 polyvinyl alcohol [PVA] and 6 microsphere), coils in 9 cases, and particles plus coils in 5 cases (4 PVA and 1 microsphere). Technical success (ability to embolize abnormal vessel) was achieved in 97.6% of patients (40 of 41), and clinical success (complete or partial resolution of hemoptysis within 30 days of embolization) was achieved in 90.2% (37 of 41). There was 1 procedure-related complication (2.4%) of cerebral infarction and 1 death from multiple-organ dysfunction (2.4%). Bleeding-free survival rates at 6, 12, 24, and 36 months were 92.5%, 83.9%, 83.9%, and 70.8%, respectively.ConclusionsBAE is a safe and effective procedure in children with pulmonary hemorrhage.     

First-in-human in vivo genome editing via AAV-zinc-finger nucleases for mucopolysaccharidosis I/II and hemophilia B

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The in vitro pharmacological profile of KR31080, a nonpeptide AT1 receptor antagonist

Summary— KR31080 (2-butyl-5-methyl-6-(1-oxopyridin-2-yl)-3-[[2'-(1H-tetrazol-5-yl) biphenyl-4-yl]methyl]-3H-imidazo[4,5-b] pyridine) is a potent inhibitor of angiotensin type 1 (AT₁) receptors in rabbit aorta and human recombinant AT₁ receptors. In the isolated rabbit thoracic aorta, KR31080 caused a nonparallel shift to the right of the concentration-response curves to angiotensin II (All) with decreased maximal response (pD'₂ = 10.1 ± 0.1), but had no effect on the contractile response induced by norepinephrine. KR31080 inhibited specific [¹²⁵I]AII binding to rabbit aortic membranes (AT, receptors) and [¹²⁵I][Sar¹, Ile⁸]AII binding to human recombinant AT₁ receptors in a concentration-dependent manner with IC₅₀ values of 0.84 ± 0.08 nM and 1.92 ± 0.15 nM, respectively, but did not inhibit specific [¹²⁵I)AII binding to bovine cerebellum membranes (ÀT₂ receptors). In the Scatchard analysis, KR31080 interacted with rabbit aortic AT₁ receptors in a competitive manner, similar to losartan. These results demonstrate that KR31080 is a potent and AT₁ selective angiotensin receptor antagonist which exerts a competitive antagonism in the [¹²⁵I]AII binding assay and insurmountable AT₁ receptor antagonism in the functional study.     

Cor triatriatum associated with partial anomalous pulmonary venous connection to the coronary sinus: Echocardiographic and angiocardiographic features

Summary An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.     

Cervical intramedullary cavernous angioma with MRI-proven haemorrhages

Summary Two contrasting cases of cervical intramedullary cavernous angioma in young female patients are reported. One patient had a 3-year course of step-wise progressive tetraparesis; at each of the five events intramedullary bleeding from a cryptic vascular malformation at C6–7 level was diagnosed by MRI. The other patient presented with one episode which led to MRI diagnosis of a vascular malformation at the C2 level. Both patients eventually underwent complete surgical excision of the angioma with subsequent steady improvement of the neurological deficit.     

Mutational analysis of HOXD13 and HOXA13 genes in the triphalangeal thumb-brachyectrodactyly syndrome.

The triphalangeal thumb-brachyectrodactyly syndrome is a very rare autosomal dominant disorder of unknown etiology characterized by an unusual pattern of limb malformations: triphalangeal thumbs and brachyectrodactyly in the hands, and ectrodactyly in the feet. In a previous report, we described the clinical and radiographical features of three related subjects with the disease and suggest that due to the unusual combination of limb defects and to its phenotypic similarity with the limb malformative pattern induced by disrupting the Hoxd13 gene in mouse, the triphalangeal thumb-brachyectrodactyly syndrome might be caused by mutations in a HOX gene. After sequencing the entire coding region of HOXD13 and the highly conserved homeodomain encoding region of HOXA13, we do not detect any deleterious mutation in any of the patients excluding that alterations at these sequences are responsible for the disease. Mutations in regulatory regions of these genes or in other genes involved in limb development might be responsible for the disease.