Dataset Information

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

ABSTRACT: Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

SUBMITTER: Gera DN

PROVIDER: S-EPMC3841517 | biostudies-literature | 2013 Nov

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

Gera D N DN Ghuge P P PP Gandhi S S Vanikar A V AV Shrimali J D JD Kute V B VB Trivedi H L HL

Indian journal of nephrology 20131101 6

Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated ge ...[more]

PMID: 24339527

Similar Datasets

Project description:BackgroundThoracic endovascular aortic repair (TEVAR) has been chosen as a less invasive alternative for type B aortic dissections (TBADs). However, the therapeutic effect of TEVAR has been challenged by postoperative adverse events, which were induced by inflammatory response. Glucocorticoids have been widely used because of the powerful and effective anti-inflammatory properties. Nevertheless, the prognostic effect of glucocorticoids after TBAD patients underwent TEVAR remains unclear. The objective of this study was to assess the potential effect of postoperative glucocorticoids on the prognosis of TEVAR for TBADs.MethodsA total of 92 chronic TBADs patients underwent TEVAR with epidural anesthesia between June 2012 and June 2014 was retrospectively reviewed. The patients were stratified into dexamethasone (DXM) and non-dexamethasone group (N-DXM). The indications for TEVAR were as following: malperfusion (n = 28); contained or impending rupture (n = 17); persistent intractable chest/back pain (n = 32); refractory hypertension (n = 15).ResultsNo 30-day mortality and incision infection occurred in each group. The postoperative pain score on the second day was significantly higher in N-DXM group (3.60 ± 0.21 versus 4.83 ± 0.32, P = 0.001). The differences of white blood cell, body temperature and heart rate were pronounced in both groups judged by the peak values (13.01 ± 0.58 × 10(9)/L versus 10.04 ± 0.61 × 10(9)/L, 37.67 ± 0.08 °C versus 37.92 ± 0.09 °C and 89.06 ± 1.21 bpm versus 95.95 ± 1.70 bpm, P = 0.002, 0.04 and 0.001, respectively). The white blood cells in DXM group significantly increased on the second and third postoperative day (P = 0.009 and 0.023), while the body temperature and heart rate showed an apparent decline on the second (P = 0.001 and 0.028), third (P = 0.007 and 0.005) and fourth postoperative days (P = 0.024 and 0.018). However, the changes of false lumen volumes and the endoleak incidence at 3-month follow-up were comparable in the two groups. No significant difference of post-implantation syndrome was observed either.ConclusionsAlthough postoperative prophylactic glucocorticoids administration was unable to influence mortality, incision infection or the change of false lumen volumes, it enabled to enhance the recovery of vital signs and alleviate the postoperative pain. A prospective, randomized controlled trial has been registered (NCT02523300), which will be warranted before prophylactic administration of glucocorticoids after TEVAR procedure could be recommended in the clinical work.

Project description:BackgroundRetrograde type A aortic dissection (RTAD) is a rare but life-threatening complication after thoracic endovascular aortic repair (TEVAR) for type B aortic dissection (TBAD). A graft inversion technique was applied to distal anastomosis in total arch replacement for this complicated dissection. We reviewed our results of the processing for this serious complication. The aim is to evaluate the feasibility of this technology.MethodsFrom January 2013 to December 2017, 20 patients (80% male, mean age 50.9 ± 9.5 years) with retrograde type A aortic dissection after thoracic endovascular aortic repair for type B aortic dissection were scheduled for surgical treatment at our center. All patients underwent an ascending aorta and total aortic arch replacement procedure. The 20 patients were divided into two groups, 1 group involved 9 patients underwent surgery using stepwise technique; the graft inversion technique was performed in the other group containing the remaining 11 patients. The postoperative variables, including cardiopulmonary bypass time, the circulatory arrest time, the aortic cross clamp time, were analyzed. Meanwhile we also analyzed the postoperative mortality and complications to evaluate the early and mid-term outcomes of surgical treatment for RTAD after TEVAR.ResultsIn-hospital mortality was 10% (2 of 20 patients). No patient developed postoperative paraplegia, renal failure, stroke, or distal anastomotic bleeding. Two patients developed renal insufficiency, one developed neurologic insufficiency, and one developed pulmonary infection, all of which were managed accordingly. Cardiopulmonary bypass (CPB) time, and circulatory arrest time were significantly shorter in the graft inversion group than in the stepwise group (165.8 ± 37.9 min versus 206.1 ± 46.8 min, p<0.05; 34.5 ± 5.6 min versus 42.4 ± 9.5 min, p<0.05, respectively). The 18 survivors had a mean follow-up of 25.8 ± 18.2 months, and all patients remained alive and well.ConclusionGraft inversion can enable a secure distal anastomosis under good surgical exposure, resulting in reduced durations of CPB, and circulatory arrest for RTAD after TEVAR. Surgical treatment could be a safe alternative for treatment of this patients.

Dataset Information

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

Publications

Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets