Congenital hyperinsulinism due to SUR1 (ABCC8) mutation in newborn twins: improvement of clinical outcome after eight years follow-up

2015 ◽  
Author(s):  
Betul Ersoy ◽  
Nermin Tansug ◽  
Abdulkadir Genc ◽  
Deniz Kizilay ◽  
Semiha Kiremitci ◽  
...  
Author(s):  
Jung-Won Lim ◽  
Yong-Beom Park ◽  
Dong-Hoon Lee ◽  
Han-Jun Lee

AbstractThis study aimed to evaluate whether manipulation under anesthesia (MUA) affect clinical outcome including range of motion (ROM) and patient satisfaction after total knee arthroplasty (TKA). It is hypothesized that MUA improves clinical outcomes and patient satisfaction after primary TKA. This retrospective study analyzed 97 patients who underwent staged bilateral primary TKA. MUA of knee flexion more than 120 degrees was performed a week after index surgery just before operation of the opposite site. The first knees with MUA were classified as the MUA group and the second knees without MUA as the control group. ROM, Knee Society Knee Score, Knee Society Functional Score, Western Ontario and McMaster Universities (WOMAC) score, and patient satisfaction were assessed. Postoperative flexion was significantly greater in the MUA group during 6 months follow-up (6 weeks: 111.6 vs. 99.8 degrees, p < 0.001; 3 months: 115.9 vs. 110.2 degrees, p = 0.001; 6 months: 120.2 vs. 117.0 degrees, p = 0.019). Clinical outcomes also showed similar results with knee flexion during 2 years follow-up. Patient satisfaction was significantly high in the MUA group during 12 months (3 months: 80.2 vs. 71.5, p < 0.001; 6 months: 85.8 vs. 79.8, p < 0.001; 12 months: 86.1 vs. 83.9, p < 0.001; 24 months: 86.6 vs. 85.5, p = 0.013). MUA yielded improvement of clinical outcomes including ROM, and patient satisfaction, especially in the early period after TKA. MUA in the first knee could be taken into account to obtain early recovery and to improve patient satisfaction in staged bilateral TKA.


2018 ◽  
Vol 4 (3) ◽  
pp. 492-496
Author(s):  
Yousef Ahmed Alomi ◽  
Hussam Saad Almalki ◽  
Aisha Omar Fallatah ◽  
Awatif Faraj Alshammari ◽  
Nesreen Al-Shubbar

The national total parental nutrition program with an emphasis on pediatrics started before several ago at Ministry of health hospitals In Kingdom of Saudi Arabia. The program covered several regions and consisted from the foundation of Intravenous Admixture and preparation of pediatric parenteral nutrition to administration and follow up of patients outcomes. In addition to the prior system, the new initiative project with the standardized formulation of pediatric’s parenteral nutrition is the complementary project of the parental nutrition for pediatrics. The project initiated to prevent drug-related problems of parental nutrition, improve patient clinical outcome and reduce the unnecessary economic burden on the healthcare system. It is the new system in the Middle East and Gulf counties in additional to Saudi Arabia. The initiatives are the systemic implementation of standardized pediatrics formulation using management project tools of starting new idea until finding in the ground.


2021 ◽  
pp. 219256822098827
Author(s):  
Giorgio Lofrese ◽  
Alba Scerrati ◽  
Massimo Balsano ◽  
Roberto Bassani ◽  
Michele Cappuccio ◽  
...  

Study Design: Retrospective multicenter. Objectives: diffuse idiopathic skeletal hyperostosis (DISH) involving the cervical spine is a rare condition determining disabling aero-digestive symptoms. We analyzed impact of preoperative settings and intraoperative techniques on outcome of patients undergoing surgery for DISH. Methods: Patients with DISH needing for anterior cervical osteophytectomy were collected. Swallow studies and endoscopy supported imaging in targeting bone decompression. Patients characteristics, clinico-radiological presentation, outcome and surgical strategies were recorded. Impact on clinical outcome of duration and time to surgery and different surgical techniques was evaluated through ANOVA. Results: 24 patients underwent surgery. No correlation was noted between specific spinal levels affected by DISH and severity of pre-operative dysphagia. A trend toward a full clinical improvement was noted preferring the chisel ( P = 0.12) to the burr ( P = 0.65), and whenever C2-C3 was decompressed, whether hyperostosis included that level ( P = 0.15). Use of curved chisel reduced the surgical times ( P = 0.02) and, together with the nasogastric tube, the risk of complications, while bone removal involving 3 levels or more ( P = 0.04) and shorter waiting times for surgery ( P < 0.001) positively influenced a complete swallowing recovery. Early decompressions were preferred, resulting in 66.6% of patients reporting disappearance of symptoms within 7 days. One and two recurrences respectively at clinical and radiological follow-up were registered 18-30 months after surgery. Conclusion: The “age of DISH” counts more than patients’ age with timeliness of decompression being crucial in determining clinical outcome even with a preoperative mild dysphagia. Targeted bone resections could be reasonable in elderly patients, while in younger ones more extended decompressions should be preferred.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 807.3-807
Author(s):  
I. Moriyama

Background:No widely accepted view or criteria currently exist concerning whether or not patellar replacement (resurfacing) should accompany total knee arthroplasty for osteoarthritis of the knee.1)2)3)Objectives:We recently devised our own criteria for application of patellar replacement and performed selective patellar replacement in accordance with this set of criteria. The clinical outcome was analyzed.Methods:The study involved 1150 knees on which total knee arthroplasty was performed between 2005 and 2019 because of osteoarthritis of the knee. The mean age at operation was 73, and the mean postoperative follow-up period was 91 months. Our criteria for application of patellar replacement are given below. Criterion A pertains to evaluation of preoperative clinical symptoms related to the patellofemoral joint: (a) interview regarding presence/absence of pain around the patella, (b) cracking or pain heard or felt when standing up from a low chair, (c) pain when going upstairs/downstairs. Because it is difficult for individual patients to identify the origin of pain (patellofemoral joint or femorotibial joint), the examiner advised each patient about the location of the patellofemoral joint when checking for these symptoms. Criterion B pertains to intense narrowing or disappearance of the patellofemoral joint space on preoperative X-ray of the knee. Criterion C pertains to the intraoperatively assessed extent of patellar cartilage degeneration corresponding to class 4 of the Outerbridge classification. Patellar replacement was applied to cases satisfying at least one of these sets of criteria (A-a,-b,-c, B and C). Postoperatively, pain of the patellofemoral joint was evaluated again at the time of the last observation, using Criterion A-a,-b,-c.Results:Patellar replacement was applied to 110 knees in accordance with the criteria mentioned above. There were 82 knees satisfying at least one of the Criterion sets A-a,-b,-c, 39 knees satisfying Criterion B and 70 knees satisfying Criterion C. (Some knees satisfied 2 or 3 of Criteria A, B and C).When the pain originating from patellofemoral joint (Criterion A) was clinically assessed at the time of last observation, pain was not seen in any knee of the replacement group and the non-replacement group.Conclusion:Whether or not patellar replacement is needed should be determined on the basis of the symptoms or findings related to the patellofemoral joint, and we see no necessity of patellar replacement in cases free of such symptoms/findings. When surgery was performed in accordance with the criteria on patellar replacement as devised by us, the clinical outcome of the operated patellofemoral joint was favorable, although the follow-up period was not long. Although further follow-up is needed, the results obtained indicate that selective patellar replacement yields favorable outcome if applied to cases judged indicated with appropriate criteria.References:[1]The Effect of Surgeon Preference for Selective Patellar Resurfacing on Revision Risk in Total Knee Replacement: An Instrumental Variable Analysis of 136,116 Procedures from the Australian Orthopaedic Association National Joint Replacement Registry.Vertullo CJ, Graves SE, Cuthbert AR, Lewis PL J Bone Joint Surg Am. 2019 Jul 17;101(14):1261-1270[2]Resurfaced versus Non-Resurfaced Patella in Total Knee Arthroplasty.Allen W1, Eichinger J, Friedman R. Indian J Orthop. 2018 Jul-Aug;52(4):393-398.[3]Is Selectively Not Resurfacing the Patella an Acceptable Practice in Primary Total Knee Arthroplasty?Maradit-Kremers H, Haque OJ, Kremers WK, Berry DJ, Lewallen DG, Trousdale RT, Sierra RJ. J Arthroplasty. 2017 Apr;32(4):1143-1147.Disclosure of Interests:None declared


Author(s):  
Mohamed S. Alwarraky ◽  
Hasan A. Elzohary ◽  
Mohamed A. Melegy ◽  
Anwar Mohamed

Abstract Background Our purpose is to compare the stent patency and clinical outcome of trans-jugular intra-hepatic porto-systemic shunt (TIPS) through the left branch portal vein (TIPS-LPV) to the standard TIPS through the right branch (TIPS-RPV). We retrospectively reviewed all patients (n = 54) with refractory portal hypertension who were subjected to TIPS-LPV at our institute (TIPS-LPV) between 2016 and 2018. These patients were matched with 56 control patients treated with the standard TIPS-RPV (TIPS-RPV). The 2 groups were compared regarding the stent patency rate, encephalopathy, and re-interventions for 1 year after the procedure. Results TIPS-LPV group showed 12 months higher patency rate (90.7% compared to 73.2%) (P < 0.005). The number of the encephalopathy attacks in the TIPS-LPV group was significantly lower than that of the TIPS-RPV group at 6 and 12 months of follow-up [P = 0.012 and 0.036, respectively]. Re-bleeding and improvement of ascites were the same in the two groups [P > 0.05]. Patients underwent TIPS-LPV needed less re-interventions and required less hospitalizations than those with TIPS-RPV [P = 0.039 and P = 0.03, respectively]. Conclusion The new TIPS approach is to extend the stent to LPV. This new TIPS-LPV approach showed the same clinical efficiency as the standard TIPS-RPV in treating variceal bleeding and ascites. However, it proved a better stent patency with lower rates of re-interventions, encephalopathy, and hospital admissions than TIPS through the right branch.


2020 ◽  
Vol 5 (4) ◽  
pp. 2473011420S0031
Author(s):  
Hong S. Lee ◽  
Kiwon Young ◽  
Tae-Hoon Park ◽  
Hong Seop Lee

Category: Ankle; Arthroscopy Introduction/Purpose: The purpose of the present study was to evaluate the outcomes of arthroscopic microfracture for osteochondral lesions of the talus, in patients of older than 60 years old. Methods: Sixteen patients (16 ankles) with osteochondral lesions of the talus were treated by arthroscopic microfracture from October 2012 to June 2019. As two patients were lost to follow-up, Fourteen patients (14 ankles) participated in the study. There were 6 men (42.9%) and 8 women (57.1%) of average age 67.4years (range 60-77) at the time of surgery. Clinical outcome evaluations were performed using Foot and Ankle Outcome Score (FAOS) and patient’s satisfaction after surgery at a mean follow-up of 50.8 months. Results: Mean FAOS scores improved from SYMPTOM 68.2 points (range 39-86), PAIN 65.1 points (range 36-94), ADL 69.4 points (range 32-99), SPORTS 45.4 points (range 25-80), QOL 47.1 points (range 13-94) preoperatively to SYMPTOM 94.8 points (range 68-100), PAIN 91.1 points (range 48-100), ADL 93.3 points (range 59-100), SPORTS 71.8 points (range 30-100), QOL 79.8 points (range 25-100) at final follow up. Very satisfaction in 4 (29%), Satisfaction in 7 (50%), Fair in 2 (14%), Dissatisfaction in 1 (7%), and reason for dissatisfaction was persistent pain after surgery. Conclusion: Arthroscopic microfracture for osteochondral lesions of the talus is a safe and effective procedure for old age patient.


2021 ◽  
Vol 11 (4) ◽  
pp. 416
Author(s):  
Carla Piano ◽  
Francesco Bove ◽  
Delia Mulas ◽  
Enrico Di Stasio ◽  
Alfonso Fasano ◽  
...  

Previous investigations have reported on the motor benefits and safety of chronic extradural motor cortex stimulation (EMCS) for patients with Parkinson’s disease (PD), but studies addressing the long-term clinical outcome are still lacking. In this study, nine consecutive PD patients who underwent EMCS were prospectively recruited, with a mean follow-up time of 5.1 ± 2.5 years. As compared to the preoperatory baseline, the Unified Parkinson’s Disease Rating Scale (UPDRS)-III in the off-medication condition significantly decreased by 13.8% at 12 months, 16.1% at 18 months, 18.4% at 24 months, 21% at 36 months, 15.6% at 60 months, and 8.6% at 72 months. The UPDRS-IV decreased by 30.8% at 12 months, 22.1% at 24 months, 25% at 60 months, and 36.5% at 72 months. Dopaminergic therapy showed a progressive reduction, significant at 60 months (11.8%). Quality of life improved by 18.0% at 12 months, and 22.4% at 60 months. No surgical complication, cognitive or behavioral change occurred. The only adverse event reported was an infection of the implantable pulse generator pocket. Even in the long-term follow-up, EMCS was shown to be a safe and effective treatment option in PD patients, resulting in improvements in motor symptoms and quality of life, and reductions in motor complications and dopaminergic therapy.


Author(s):  
Silvan Hess ◽  
Timo Fromm ◽  
Filippo Schiapparelli ◽  
Lukas B. Moser ◽  
Emma Robertson ◽  
...  

Abstract Purpose The main purpose of this study was to determine whether there is a correlation between the change of tibial tuberosity-trochlear groove (TT-TG) distance and clinical outcomes after total knee arthroplasty (TKA). Methods A total of 52 knees undergoing TKA due to primary osteoarthritis were included in this retrospective study. All patients had pre- and postoperative CT scans. TT-TG distance was measured by two independent observers and the following alignment parameters were measured: hip-knee ankle angle (HKA), femoral mechanical angle (FMA), tibial mechanical angle (TMA), and posterior condylar angle (PCA). Clinical outcome was assessed using Knee Society Score (KSS) pre- and post-operatively and at a minimum of 12-month follow-up. Evidence of AKP was noted from follow-up reports. Pre- and postoperative scores were compared using a paired Student t-test. Pearson correlations were calculated to assess the influence of TT-TG on clinical outcome and of alignment parameters on the change in TT-TG. TT-TG between patients with and without AKP was compared using unpaired Student’s t-test (p < 0.05). Results Neither the absolute postoperative TT-TG nor the amount of change in TT-TG correlated with the post-operative KSS or the change in KSS. Post-operative TT-TG and change in TT-TG did not differ significantly between patients with and patients without AKP. Only the change in FMA showed a correlation with the change in TT-TG (p = 0.01, r = 0.36). Conclusion Despite a missing correlation between outcomes and TT-TG distance in this study, excessive TT-TG distance should be avoided. Furthermore, surgeons need to be aware that changes in femoral joint line orientation might affect TT-TG distance.


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