scholarly journals Over 20-Year Follow-up of Patients with Hepatic Glycogen Storage Diseases: Single-Center Experience

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 297
Author(s):  
Edyta Szymańska ◽  
Patryk Lipiński ◽  
Dariusz Rokicki ◽  
Janusz Książyk ◽  
Anna Tylki-Szymańska
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Published Data ◽  
Type I ◽  
Long Term Outcomes ◽  
Normal Height ◽  
Metabolic Condition ◽  
Glycogen Storage Diseases

Background: The published data on the long-term outcomes of glycogen storage disease (GSD) patients is sparse in the literature. The aim of this study was to analyze the long-term (over 20 years) follow-up of patients with hepatic types of GSD-I, III, VI, and IX—from childhood to adulthood, managed by one referral center. Patients and methods: Thirty adult patients with hepatic GSD were included in the study. A retrospective chart review of patients’ medical records has been performed. Results: During the long-term follow-up, the most frequent complications observed in a group of 14 GSD I patients were nephropathy with blood hypertension (10/14), hyperuricemia (8/14), and development of hepatocellular adenomas (HCA; 5/14). All individuals but four presented with normal height. Two patients with GSD Ib suffered from inflammatory bowel disease (IBD). Nine (64%) GSD I patients were in balanced metabolic condition at the age of 18. Regarding GSD III/VI/IX, the most frequent complication was short stature observed in 5 out of 16 patients. All patients but one with GSD VI were in balanced metabolic condition at the age of 18. Conclusion: The long-term outcomes of patients with GSD depend mainly on proper (adjusted to each type of GSD) dietary management and patient compliance. However, in GSD type I, even proper management does not eliminate all long-term complications in adulthood.

scholarly journals Current status of hepatic glycogen storage disease in Japan: clinical manifestations, treatments and long-term outcomes

2013 ◽  
Vol 58 (5) ◽  
pp. 285-292 ◽  
Author(s):  
Jun Kido ◽  
Kimitoshi Nakamura ◽  
Shirou Matsumoto ◽  
Hiroshi Mitsubuchi ◽  
Toshihiro Ohura ◽  
...  
Keyword(s):  
Glycogen Storage Disease ◽  
Storage Disease ◽  
Clinical Manifestations ◽  
Glycogen Storage ◽  
Current Status ◽  
Hepatic Glycogen ◽  
Long Term Outcomes

Long-term outcomes after laparoscopic splenectomy for splenomegaly

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 17527-17527 ◽  
Author(s):  
S. A. Brethauer ◽  
S. Chalikonda ◽  
A. Dan ◽  
V. Nfonsam ◽  
C. Goncalves ◽  
...  
Keyword(s):  
Laparoscopic Splenectomy ◽  
Postoperative Hemorrhage ◽  
Published Data ◽  
Long Term Outcomes ◽  
Hematological Indices ◽  
Progression Of Disease ◽  
Splenic Involvement ◽  
Operative Indications

17527 Background: Numerous studies have demonstrated the feasibility of laparoscopic splenectomy (LS) for splenomegaly. There is little published data on the long-term efficacy of LS. The aim of this study is to determine long-term outcomes following LS for splenomegaly. Methods: Retrospective review of patients undergoing LS between 8/95 and 5/05. Splenomegaly was defined by CT criteria of craniocaudal length > 17 cm. Preoperative diagnoses included lymphoma in 13 patients (20%), leukemia in 15 (23%), autoimmune hemolytic anemia in 3 (5%) and other hematologic disorders in 8 (12%). 26 patients (40%) had no definitive diagnosis prior to splenectomy. Patients with ITP were excluded. Patient demographics, operative indications, operative morbidity and mortality, pathology, pre- and postoperative hematological indices were collected. Follow-up was obtained from patient records and telephone interviews. Results: 311 patients underwent LS during the study period. 65 patients underwent LS for splenomegaly. The mean age was 59 and 62% were male. There were no operative deaths. 7 patients (11%) were converted from LS to open. There were 9 (14%) major complications including 4 re-operations (3 for bleeding), 3 portal vein thromboses, 1 intra-abdominal abscess and 1 postoperative hemorrhage managed non-operatively. Thirty-two patients underwent LS primarily for diagnostic purposes. Nine had an existing hematologic disease and LS was performed to evaluate splenic involvement. LS confirmed a diagnosis in 19 (59%) patients including 11 lymphomas (34%). Thirty-three patients underwent LS primarily for treatment of either symptoms (33%) or cytopenias (66%). LS effectively treated mass symptoms in all patients. Among all patients 33 (51%) had associated cytopenias. At median f/u of 20 months 15 (45%) patients had no recurrence of cytopenias, 15 (45%) patients required further treatment and 3 were lost to follow up. One responder (7%) died of lymphoma 3 months after surgery without recurrence of cytopenia. The 15 non-responders had progression of disease requiring chemotherapy and transfusions and 9 (60%) died a median of 6 months after LS. Conclusions: LS is a valuable diagnostic and therapeutic tool for patients with splenomegaly and provides durable treatment for splenomegaly-associated cytopenias. No significant financial relationships to disclose.

scholarly journals Long-term outcomes of per-oral endoscopic myotomy in achalasia patients with a minimum follow-up of 4 years: a multicenter study

2020 ◽  
Vol 08 (05) ◽  
pp. E650-E655 ◽  
Author(s):  
Olaya I. Brewer Gutierrez ◽  
Robert A. Moran ◽  
Pietro Familiari ◽  
Mohamad H. Dbouk ◽  
Guido Costamagna ◽  
...  
Keyword(s):  
Clinical Response ◽  
Adverse Event Rate ◽  
Type I ◽  
Safe Procedure ◽  
Long Term Outcomes ◽  
Per Oral Endoscopic Myotomy ◽  
Endoscopic Myotomy ◽  
Per Oral

Abstract Background and study aims Per-oral endoscopic myotomy (POEM) is associated with a short-term clinical response of 82 % to 100 % in treatment of patients with achalasia. Data are limited on the long-term durability of the clinical response in these patients. The aim of this study was to determine the long-term outcomes of patients undergoing POEM for management of achalasia. Methods This was a retrospective multicenter cohort study of consecutive patients who underwent POEM for management of achalasia. Patients had a minimum of 4 years follow-up. Clinical response was defined by an Eckardt score ≤ 3. Results A total of 146 patients were included from 11 academic medical centers. Mean (± SD) age was 49.8 (± 16) years and 79 (54 %) were female. The most common type of achalasia was type II, seen in 70 (47.9 %) patients, followed by type I seen in 41 (28.1 %) patients. Prior treatments included: pneumatic dilation in 29 (19.9 %), botulinum toxin injection in 13 (8.9 %) and Heller myotomy in seven patients (4.8 %). Eight adverse events occurred (6 mucosotomies, 2 pneumothorax) in eight patients (5.5 %). Median follow-up duration was 55 months (IQR 49.9–60.6). Clinical response was observed in 139 (95.2 %) patients at follow-up of ≥ 48 months. Symptomatic reflux after POEM was seen in 45 (32.1 %) patients, while 35.3 % of patients were using daily PPI at 48 months post POEM. Reflux esophagitis was noted in 16.8 % of patients who underwent endoscopy. Conclusion POEM is a durable and safe procedure with an acceptably low adverse event rate and an excellent long-term clinical response.

scholarly journals Does Type I Truly Dominate Hepatic Glycogen Storage Diseases in Korea?: A Single Center Study

2014 ◽  
Vol 17 (4) ◽  
pp. 239 ◽  
Author(s):  
Yu Ju Jeong ◽  
Ben Kang ◽  
So Yoon Choi ◽  
Chang-Seok Ki ◽  
Soo-Youn Lee ◽  
...  
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Type I ◽  
Single Center ◽  
Storage Diseases ◽  
Glycogen Storage Diseases ◽  
Single Center Study ◽  
Center Study

Periprocedural risk and long-term outcome of intracranial angioplasty based on a single-centre experience

VASA ◽  
2013 ◽  
Vol 42 (4) ◽  
pp. 264-274
Author(s):  
Dagmar Krajíčková ◽  
Antonín Krajina ◽  
Miroslav Lojík ◽  
Martina Mulačová ◽  
Martin Vališ
Keyword(s):  
Death Rate ◽  
Long Term Outcomes ◽  
Single Centre ◽  
Intracranial Atherosclerotic Stenosis ◽  
Atherosclerotic Stenosis ◽  
Angioplasty And Stenting ◽  
Aggressive Medical Management ◽  
Stroke Death

Background: Intracranial atherosclerotic stenosis is a major cause of stroke and yet there are currently no proven effective treatments for it. The SAMMPRIS trial, comparing aggressive medical management alone with aggressive medical management combined with intracranial angioplasty and stenting, was prematurely halted when an unexpectedly high rate of periprocedural events was found in the endovascular arm. The goal of our study is to report the immediate and long-term outcomes of patients with ≥ 70 % symptomatic intracranial atherosclerotic stenosis treated with balloon angioplasty and stent placement in a single centre. Patients and methods: This is a retrospective review of 37 consecutive patients with 42 procedures of ballon angioplasty and stenting for intracranial atherosclerotic stenosis (≥ 70 % stenosis) treated between 1999 and 2012. Technical success (residual stenosis ≤ 50 %), periprocedural success (no vascular complications within 72 hours), and long-term outcomes are reported. Results: Technical and periprocedural success was achieved in 90.5 % of patients. The within 72 hours periprocedural stroke/death rate was 7.1 % (4.8 % intracranial haemorrhage), and the 30-day stroke/death rate was 9.5 %. Thirty patients (81 %) had clinical follow-up at ≥ 6 months. During follow-up, 5 patients developed 6 ischemic events; 5 of them (17 %) were ipsilateral. The restenosis rate was 27 %, and the retreatment rate was 12 %. Conclusions: Our outcomes of the balloon angioplasty/stent placement for intracranial atherosclerotic stenosis are better than those in the SAMMPRIS study and compare favourably with those in large registries and observational studies.

Risk scoring by CHADS2 and CHA2DS2-VASc as predictors for long-term outcomes after surgical ablation for atrial fibrillation

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Lauritzen ◽  
H.J Vodstrup ◽  
T.D Christensen ◽  
M Onat ◽  
R Christensen ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Cardiac Surgery ◽  
Isolation Procedure ◽  
Survival Times ◽  
Long Term Outcomes ◽  
Surgical Ablation ◽  
Follow Up Study ◽  
Risk Scoring

Abstract Background Following catheter ablation for atrial fibrillation (AF), CHADS2 and CHA2DS2-VASc have utility in predicting long-term outcomes. However, it is currently unknown if the same holds for patients undergoing surgical ablation. Purpose To determine whether CHADS2 and CHA2DS2-VASc predict long-term outcomes after surgical ablation in concomitance with other cardiac surgery. Methods In this prospective, follow-up study, we included patients who underwent biatrial ablation - or pulmonary vein isolation procedure concomitantly with other cardiac surgery between 2004 and 2018. CHADS2 and CHA2DS2-VASc scores were assessed prior to surgery and categorized in groups as 0–1, 2–4 or ≥5. Outcomes were death, AF, and AF-related death. Follow-up was ended in April 2019. Results A total of 587 patients with a mean age of 68.7±0.4 years were included. Both CHADS2 and CHA2DS2-VASc scores were predictors of survival p=0.005 and p<0.001, respectively (Figure). For CHADS2, mean survival times were 5.9±3.7 years for scores 0–1, 5.0±3.0 years for scores 2–4 and 4.3±2.6 years for scores ≥5. For CHA2DS2-VASc mean survival times were 7.3±4.0 years for scores 0–1, 5.6±2.9 years for scores 2–4 and 4.8±2.1 years for scores ≥5. The incidence of death was 20.1% for CHADS2 0–1, 24.8% for CHADS2 2–4, and 35.3% for CHADS2 ≥5, p=0.186. The incidence of AF was 50.2% for CHADS2 0–1, 47.9% for CHADS2 2–4, and 76.5% for CHADS2 ≥5, p=0.073. The incidence of AF related death was 13.0% for CHADS2 0–1, 16.8% for CHADS2 2–4, and 35.3% for CHADS2 ≥5, p=0.031. The incidence of death was 16.8% for CHA2DS2-VASc 0–1, 26.2% for CHA2DS2-VASc 2–4, and 45.0% for CHA2DS2-VASc ≥5, p=0.001. The incidence of AF was 49.6% for CHA2DS2-VASc 0–1, 52.5% for CHA2DS2-VASc 2–4, and 72.5% for CHA2DS2-VASc ≥5, p=0.035. The incidence of AF related death was 12.2% for CHA2DS2-VASc 0–1, 16.0% for CHA2DS2-VASc 2–4, and 42.5% for CHA2DS2-VASc ≥5, p<0.001. Conclusion Both CHADS2 and CHA2DS2-VASc scores predict long-term outcomes after surgical ablation for AF. However, CHA2DS2-VASc was superior in predicting death, AF, and AF-related death. Survival curves Funding Acknowledgement Type of funding source: None

Sign in / Sign up

Export Citation Format

Share Document