Orthotopic Liver Transplantation for Hereditary Hemorrhagic Telangiectasia and MEN Type I Syndrome - Case Report and Review of Literature

Abstract

M.I. Ionescu et al în literatură. Mai mult decât atât, reprezintă de asemenea primul caz de anastomoză arterială latero-terminală efectuată în cursul unui TH la un pacient cu THE. Lucrarea noastră demonstrează că această abordare este atât fezabilă din punct de vedere tehnic, cât şi sigură în context clinic. Cuvinte cheie: telangiectazie hemoragică ereditară, sindromul neoplaziei endocrine multiple tip 1, transplant hepatic ortotopic

Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review

“…Two studies were identified by hand-searching in the bibliography of retrieved articles [ 24 , 25 ]. Therefore 45 studies were finally included for analysis [ 10 , 11 , 13 , 15 , 16 , 17 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 ]. The PRISMA flow diagram is depicted in Figure 1 .…”

“…Interestingly, HHT relapse in the transplanted allograft was detected in 13 (17.1%) among 76 discharged patients between 1 and 19 years post-transplant, including two patients that died due to recurrence complications (Case 2, [ 12 , 19 , 20 , 21 , 22 , 23 , 42 ]). Three patients died during follow-up due to myocardial infarction (at day 34), ischemic cholangitis due to recurrence of hepatic VM (8 years later), and portal hypertension due to recurrence of hepatic VM (6 years later) (Case 2, [ 32 , 42 ]). Thus, overall mortality was 12%.…”

“… P3, P4 & P5: Shortness of breath and volume overload signs + PHTN P1: 10.2/6 P2: 8.6/4.7 P3: 9.1/4.1 P4: 5.3/5.7 P5: 8.5/4.3 Some received BVZ (not identified) Dumortier, 2019 [ 29 ] 1 (F) 60 - 2 * (V, FH) HOCF + Hepatic encephalopathy Dyspnea, fluctuating confusion and somnolence. -/4.9 BVZ Álamo, 2019 [ 30 ] 1 (F) 62 - 1 * (V) HOCF Severe heart failure 7.1/- BVZ Sildenafil Ejiri, 2019 [ 31 ] 1 (F) 52 - 4 HOCF and PHTN secondary to hepatic AVMs WHO class IV (dyspnea at rest) 9/- - Ionescu, 2018 [ 32 ] 1 (M) 59 2 * (E, V) HOCF MEN-I, transitory ischemic attack, and AF. 15.3/7.6 - Barajas, 2018 [ 33 ] 1 (F) …”

Living donor liver transplantation for Wilson disease: A collective review

“…Conversely, patients with liver conditions other than ALF can remain asymptomatic with proper treatment. However, without adequate treatment, these patients may develop decompensated CLD with common CNS complications, making LT a potentially curative option [10][11][12].…”

The advantage of early liver transplantation for wilson’s disease using living donors

“…On the other hand, in patients who have not been treated properly, decompensated CLD can develop, and CNS findings are common. Thus, a liver transplant should be considered as a curative therapy in such patients [ 10 – 12 ].…”