Primary angioplasty or stenting for hepatic artery stenosis treatment after liver transplantation.

BACKGROUND: Endovascular treatment (EVT) by percutaneous transluminal angioplasty (PTA) or stent is the first-line treatment for hepatic artery stenosis (HAS) after liver transplantation, but there are no guidelines to help choose between PTA and stent. METHODS: Retrospective review of HAS EVT after liver transplantation, between 1999 and 2017. HAS was treated by PTA or stent. We report EVT primary effectiveness, arterial patency after 1 year of follow-up, complications, HAS recurrence rate; comparing PTA to stent. RESULTS: Fifty-two HAS were diagnosed in 42 patients. We performed 51 EVT; 34 PTA (66.7%) and 16 stents (31.4%). Global primary EVT effectiveness was 86.3%: 82.3% after PTA and 100% after stent (P = 1.00 after propensity score matching). Recurrent HAS was found in 22.0% of cases: 29.4% after PTA and 6.2% after stenting, (P = .053 after propensity score matching). Patency rate without recurrent HAS or HAT at 12 months was 73.5% with PTA and 93.8% with stent (P = .09), and globally this was 92.8%. There were 7.8% complications: 2.9% after PTA, 12.5% after stenting (P = .23). CONCLUSION: Primary effectiveness was the same for PTA and stenting. There was a strong trend toward more HAS recurrence after PTA than after stenting suggesting that HAS should benefit from primary stenting.

Digital breast tomosynthesis versus mammography and breast ultrasound: a multireader performance study.

OBJECTIVES: To compare the diagnostic performance of single-view breast tomosynthesis (BT) with that of dual-view mammography (MX); to assess the benefit of adding the craniocaudal (CC) mammographic view to BT, and of adding BT to MX plus breast ultrasound, considered to be the reference work-up. METHODS: One hundred and fifty-five consenting patients with unresolved mammographic and/or ultrasound findings or breast symptoms underwent conventional work-up plus mediolateral oblique-view BT of the affected breast. The final study set in 130 patients resulted in 55 malignant and 76 benign and normal cases. Seven breast radiologists rated the cases through five sequential techniques using a BIRADS-based scale: MX, MX + ultrasound, MX + ultrasound + BT, BT, BT + MX(CC). Multireader, multicase receiver operating characteristic (ROC) analysis was performed and performance of the techniques was assessed from the areas under ROC curves. The performance of BT and of BT + MX(CC) was tested versus MX; the performance of MX + ultrasound + BT tested versus MX + ultrasound. RESULTS: Tomosynthesis was found to be non-inferior to mammography. BT + MX(CC) did not appear to be superior to MX, and MX + ultrasound + BT not superior to MX + ultrasound. CONCLUSIONS: Overall, none of the five techniques tested outperformed the others. Further clinical studies are needed to clarify the role of BT as a substitute for traditional work-up in the diagnostic environment. KEY POINTS: • Digital breast tomosynthesis is a new adjunct to mammography and breast ultrasound. • We compared the diagnostic performance of these investigations in an experimental observer study. • Single-view breast tomosynthesis was confirmed as non-inferior to dual-view mammography. • None of the investigations (or combinations) tested outperformed the others. • Further prospective studies are needed to clarify precise role of tomosynthesis for diagnostic application.

Disease and patient characteristics in NP-C patients: findings from an international disease registry.

BACKGROUND: Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterized by progressive neurodegeneration and premature death. We report data recorded at enrolment in an ongoing international NP-C registry initiated in September 2009 to describe disease natural history, clinical course and treatment experience of NP-C patients in clinical practice settings. METHODS: The NPC Registry is a prospective observational cohort study. Participating sites are encouraged to evaluate all consecutive patients with a confirmed diagnosis of NP-C, regardless of their treatment status. All patients undergo clinical assessments and medical care as determined by their physicians. Data are collected through a secure internet-based data collection system. RESULTS: As of 19th March, 2012, 163 patients have been enrolled in centres across 14 European countries, Australia, Brazil and Canada. The mean (SD) age at enrolment was 19.6 (13.0) years. In general there was a long lag time between the mean (SD) age at neurological onset (10.9 (9.8) years) and age at diagnosis (15.0 (12.2) years). Among all enrolled patients, 107 were diagnosed based on combined genetic testing and filipin staining. Sixteen (11%) out of 145 patients with available age-at-neurological-onset data had early-infantile neurological onset, 45 (31%) had late-infantile onset; 45 (31%) had juvenile onset and 39 (27%) had adolescent/adult onset. The frequencies of neonatal jaundice, hepatomegaly and/or splenomegaly during infancy were greatest among early-infantile patients, and decreased with increasing age at neurological onset. The most frequent neurological manifestations were: ataxia (70%), vertical supranuclear gaze palsy (VSGP; 70%), dysarthria (66%), cognitive impairment (62%), dysphagia (52%). There were no notable differences in composite NP-C disability scores between age-at-neurological-onset groups. Miglustat therapy at enrolment was recorded in 117/163 (72%) patients. CONCLUSIONS: Approximately two-thirds of this NP-C cohort had infantile or juvenile onset of neurological manifestations, while the remaining third presented in adolescence or adulthood. While systemic symptoms were most common among patients with early-childhood onset disease, they were also common among patients with adolescent/adult onset. The profiles of neurological manifestations in this Registry were in line with previous publications.