Long-term Follow-up of Patients with Congenital Hyperinsulinism in Austria

2008 ◽  
Vol 21 (6) ◽  
pp. 523-532
Author(s):  
Birgit Rami ◽  
Saadet Mercimek-Mahmutoglu ◽  
Martha Feucht ◽  
Marion Herle ◽  
Olaf Rittinger ◽  
...  

Abstract Aim: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria. Patients and Methods: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2- 25.5 years) in a follow-up study in the year 2004. Results: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up. Conclusion: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

2008 ◽  
Vol 102 (1-3) ◽  
pp. 213
Author(s):  
Lisa Henry ◽  
G.P. Amminger ◽  
M. Harris ◽  
H.P. Yuen ◽  
S. Harrigan ◽  
...  

Crisis ◽  
1999 ◽  
Vol 20 (3) ◽  
pp. 115-120 ◽  
Author(s):  
Stephen Curran ◽  
Michael Fitzgerald ◽  
Vincent T Greene

There are few long-term follow-up studies of parasuicides incorporating face-to-face interviews. To date no study has evaluated the prevalence of psychiatric morbidity at long-term follow-up of parasuicides using diagnostic rating scales, nor has any study examined parental bonding issues in this population. We attempted a prospective follow-up of 85 parasuicide cases an average of 8½ years later. Psychiatric morbidity, social functioning, and recollections of the parenting style of their parents were assessed using the Clinical Interview Schedule, the Social Maladjustment Scale, and the Parental Bonding Instrument, respectively. Thirty-nine persons in total were interviewed, 19 of whom were well and 20 of whom had psychiatric morbidity. Five had died during the follow-up period, 3 by suicide. Migration, refusals, and untraceability were common. Parasuicide was associated with parental overprotection during childhood. Long-term outcome is poor, especially among those who engaged in repeated parasuicides.


Rheumatology ◽  
2017 ◽  
Vol 56 (11) ◽  
pp. 1928-1938 ◽  
Author(s):  
Despoina Dimopoulou ◽  
Maria Trachana ◽  
Polyxeni Pratsidou-Gertsi ◽  
Prodromos Sidiropoulos ◽  
Florentia Kanakoudi-Tsakalidou ◽  
...  

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Xue Zhao ◽  
Jianqiang Hu ◽  
Yan Huang ◽  
Yawei Xu ◽  
Yanzhou Zhang ◽  
...  

Objectives: The aim of this study was to determine the mechanisms and effectiveness of pulmonary antrum radial-linear (PAR) ablation in comparison with pulmonary vein isolation (PVI) in patients with paroxysmal atrial fibrillation (AF) after a long-term follow-up. Background: The one-year follow up data suggested that PAR ablation appeared to have a better outcome over the conventional PVI for paroxysmal AF. Methods: The enrollment occurred between March, 2011, and August, 2011, with the last follow-up in May, 2014. A total of 133 patients with documented paroxysmal AF were enrolled from 5 centers and randomized to PAR group or PVI group. Event ECG recorder and Holter monitoring were conductedduring the follow-up for all patients. Results: The average procedure time was 151±23 min in PAR group and 178±43 min in PVI group ( P <0.001). The average fluoroscopy time was 21±7 min in PAR group and 27±11 min in PVI group ( P= 0.002). AF triggering foci were eliminated in 59 patients (89.4%) in PAR group, whereas, only 4 patients (6.0%) in PVI group (P<0.001).At median 36 (37-35) months of follow-up after single ablation procedure, 43 of 66 patients in PAR group (65%) and 28 of 67 patients in PVI group (42%) had no recurrence of AF off antiarrhythmic drug (AAD) (P=0.007); and 47 of 66 patients in PAR group (71%) and 32 of 67 patients in PVI group (48%) had no recurrence of AF with AAD (P=0.006). At the last follow-up, the burden of AF was significantly lower in PAR group than in PVI group (0.9% ± 2.3% vs 4.9% ± 9.9%;90th percentile, 5.5% vs 19.6%; P=0.008). No major adverse event (death, stroke, PV stenosis) was observed in all the patients except one case of pericardial tamponade. Conclusions: PAR ablation is a simple, safe, and effective strategy for the treatment of paroxysmal AF with better long-term outcome than PVI. PAR ablation might exhibit the beneficial effect on AF management through multiple mechanisms. Registration: ChiCTR-TRC-11001191


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Erica S Zado ◽  
Pasquale Santangeli ◽  
Francis E Marchlinski

Introduction: Endo-epicardial catheter ablation of ventricular tachycardia (VT) in patients (pts) with nonischemic cardiomyopathy (NICM) has been reported to have satisfactory results at the short- and mid-term follow-up. We sought to determine the outcomes at the long-term follow-up of endo-epicardial ablation of VT in NICM. Hypothesis: Catheter ablation provides satisfactory long term outcome Methods: We prospectively enrolled 128 pts (age 59±13 years, 116 [91%] males) with NICM who underwent endo-epicardial radiofrequency catheter ablation at our Institution. After substrate mapping, all critical sites for the clinical or induced VT(s), identified with activation, entrainment or pace-mapping, together with late, split and fractionated potentials were targeted with focal and/or linear ablation. The procedural endpoint was noninducibility of sustained monomorphic VT. Pts were followed with ICD interrogation. Results: A total of 108 (73%) pts had idiopathic dilated NICM. The remaining 20 (14%) pts had hypertrophic CM (n=11), suspected inflammatory CM (n=6), or valvular CM (n=3). The mean LV ejection fraction was 33±15%. After a mean follow-up of 19 months (max 97 months), a total of 36 (28%) pts died and 17 (13%) underwent heart transplant. Cumulative survival free from any recurrent VT was 53% (68/128 patients) (Figure A). In the remaining 60 (47%) patients with VT recurrences, catheter ablation still resulted in a significant beneficial clinical impact on VT burden, with 25/60 (42%) having only isolated (1-2) VT episodes over follow-up, and a striking reduction of VT storm in the remaining pts (Figure B). Conclusions: In patients with NICM and VT, endo-epicardial substrate-based ablation is effective in achieving long-term freedom from any VT in 53% of patients, with a substantial improvement in VT burden in many of the remaining patients.


2018 ◽  
Vol 38 (6) ◽  
pp. 1763-1771 ◽  
Author(s):  
Akiko Nakagawa ◽  
Nora Choque Olsson ◽  
Yoko Hiraoka ◽  
Hirofumi Nishinaka ◽  
Tetsuji Miyazaki ◽  
...  

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