A review of the orthopedic interventions and functional outcomes among a cohort of 114 children with arthrogryposis multiplex congenita

2020 ◽  
Vol 13 (3) ◽  
pp. 263-271
Author(s):  
Delphine Hansen-Jaumard ◽  
Caroline Elfassy ◽  
Kathleen Montpetit ◽  
Bayan Ghalimah ◽  
Reggie Hamdy ◽  
...  

PURPOSE: Arthrogryposis multiplex congenita (AMC) refers to a large heterogeneous group of conditions involving joint contractures in two or more different areas of the body. Contractures can lead to decreased range of motion and strength, and affect ambulation and autonomy. The aim of this study was to describe the orthopedic interventions and functional outcomes of a large cohort of children with AMC followed in a pediatric orthopedic center. METHODS: A retrospective chart review of all children diagnosed with AMC followed at Shriners Hospital for Children – Canada (SHC) between January 1979 and July 2016 was conducted. One hundred twenty patients were identified, of whom six were excluded due to misdiagnosis or insufficient chart information. One hundred fourteen were retained. Patient demographics, AMC classification, comorbidities, operative and non-operative treatments received as well as community ambulation status, level of autonomy in self-care and transfers at latest follow-up were recorded. RESULTS: There were 54 males and 60 females with a mean age at last clinic visit of 10 years 3 months. Amyoplasia and distal arthrogryposis (DA) were equally represented in our sample, 47 (41.2%) and 49 (43.0%) participants respectively, with the category Other comprising the remaining 18 (15.8%) participants. Children with DA had less involvement of the proximal joints than those in the two other groups. Contractures and deformities of the foot and ankle were the most prevalent, affecting 91.5% with Amyoplasia, 85.7% with DA and 83.3% in the Other category. Contractures of the shoulder and elbow were more common among individuals with Amyoplasia and those categorized Other than those with DA. In terms of walking ability, 98% of participants with DA were independent ambulators. Walking ability varied among the Other participants. Similarly, most children with DA were independent in self-care and transfers at the most recent follow-up. CONCLUSION: The relatively large sample size of this study allowed for a better insight into the challenges associated with AMC management. These findings demonstrated the need for genetic testing to provide accurate diagnosis and classification, along with the use of standardized outcome tools to measure effectiveness of interventions. As AMC is rare, multi-site prospective studies are needed to improve research opportunities, develop functional measures specific to AMC and disseminate findings on a wider scale.

Author(s):  
Alun Ackery ◽  
Christine Provvidenza ◽  
Charles H. Tutor

Objectives:To determine the compliance rate among hockey players with concussion or other head injuries who were advised by a physician about return to play. To assess compliance of hockey players with return to play advice and to assess the incidence of long-term post-concussion symptoms.Methods:A retrospective chart review, telephone questionnaire and follow-up analysis of income, level of education and professional aspirations. The study examined 40 hockey players with concussion or other head injury treated at a neurosurgical ambulatory clinic, who had initial visits between 1995 and 2003, and had been seen at least two years prior to completing the questionnaire.Results:There was a 58% (23 of 40) participation rate in the study. Fifteen (65%) of the 23 participants were advised to never return to play, and 5 (33%) were non-compliant and returned to play. Four (80%) of the five non-compliant players continued to suffer from post concussion symptoms. Overall, 15 (65%) of the 23 players participating in the study continued to suffer post concussion symptoms at least two years after the clinic visit.Conclusions:Five (33%) of 15 hockey players advised to never return to play were non-compliant and returned to play, and four continued to suffer from post concussion symptoms two or more years later. After repeated concussions, 65% of hockey players had long-term sequelae that prevented return to play and produced long-term post-concussion symptoms.


Author(s):  
Sadaf Ilias ◽  
Jill Barber

This case study describes a recent learning activity involving pharmacy undergraduate students in which a final (fourth) year student trained 21 second year students to administer questionnaires about antibiotic resistance to over 700 student users of a large university building. The aim was to raise awareness of the problem of antibiotic resistance.  The second year students were also trained to correct any misapprehensions held by the questionnaire participants about antibiotic resistance, and to encourage them to become "Antibiotic Guardians". Finally the 22 students analysed the data to give a picture of what the other students understood about antibiotic resistance.  Peer-assisted learning therefore cascaded from a single fourth year student to 21 second year students and then to 700 students from various disciplines and year groups.  The first stage of the cascade was evaluated and the 21 second year students overwhelmingly believed that their knowledge of antibiotic resistance was enhanced.  A follow-up study using the same questionnaire will be used to determine whether the exercise was effective in raising awareness of antibiotic resistance among the body of students surveyed.


2011 ◽  
Vol 1 (2) ◽  
pp. 34
Author(s):  
Ninad Gaikwad ◽  
Nilam Sathe ◽  
Abhijeet Bhatia ◽  
Dhanashree Chiplunkar ◽  
Manoj Patil

A series of 80 patients with histopathologically confirmed juvenile nasopharyngeal angiofibroma were treated surgically over a period of ten years (1995-2004). The lateral rhinotomy approach was used to expose the tumor and its extensions. Lateral rhinotomy with its extensions provides wide exposure of and access to the nose, nasopharynx, paranasal sinuses, pterygopalatine fossa, infratemporal fossa and temporal fossa. Most intracranial, extradural extensions can also be approached. There were no major operative or post-operative complications. Longterm follow up from one to ten years showed only 8 recurrences. The added advantage of this approach is that it can be combined with all the other anterior and lateral skull base approaches. The cosmetic outcome is satisfactory if nasal aesthetic subunits are taken into considerations while making the incision. The lateral rhinotomy approach is the most direct route to the body of the tumor and can be used to approach all the possible extensions of the tumor.


2019 ◽  
pp. 014556131989315 ◽  
Author(s):  
Brett T. Comer ◽  
Lauren E. Harris ◽  
Caitlin E. Fiorillo ◽  
Thomas J. Gal ◽  
Allyson Hughes

Objective: To report baseline no-show rates in the hospital-employed otolaryngology practice setting and to identify factors that may affect clinic show rates that are targets for potential improvement. Study Design: Retrospective chart review. Methods: Electronic medical records from October 2012 through July 2014 of a hospital-employed otolaryngology practice were reviewed. Patients were classified by insurance type: commercial, Medicare, Medicaid, and self-pay. Clinic visits were classified as new patient, follow-up, or postoperative. No-show rates were tabulated for each type of clinic visit and compared. Factors to improve no-show rates are discussed. Results: There was an overall no-show rate of 8.3% for 5817 scheduled clinic visits. Among visit types, follow-up visits had the highest no-show rates. Among insurance types, Medicaid had the highest no-show rates. New patient Medicaid patients, follow-up Medicaid patients, and follow-up commercial insurance patients had the highest rate of no-shows among visit/insurance type combinations. Persistent reminders are a key factor in improving rate of clinic visit adherence. Conclusion: A previously unreported baseline no-show rate was established for hospital-employed otolaryngology clinics. The utilization of repeated, live-person reminders to mitigate the impact of clinic no-show rates needs to be further investigated.


2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S443-S444
Author(s):  
B Bressler ◽  
B Sattin ◽  
M A Williamson ◽  
R Khanna ◽  
C H Seow ◽  
...  

Abstract Background Therapeutic drug monitoring is an important adjunct in optimising IBD management in patients treated with anti-TNF therapies. However, it remains unclear whether these principles apply to non-anti-TNF biologic therapies, such as the anti-IL12/23 agent, ustekinumab (UST). A follow-up analysis of the ‘mUST-DECIDE’ study explores the correlation between UST drug level and clinical outcomes. Methods 111 consecutive UST-treated adult CD patients across 11 sites in Canada from April 2017 to January 2018 were evaluated. Clinical decisions were recorded for all subjects at the single study visit and blood was drawn for TDM (Sanquin, radioimmunoassay). A retrospective chart review was completed in patients who had a follow-up visit ≥ 30 days after the baseline visit. Improved disease control (primary outcome) was defined as a composite assessment outcome meeting ≥ 1 disease control criterion (symptomatic, endoscopic/imaging, biochemical) without any of the non-response criteria (inadequate or loss of response, worsening of any disease control criteria, initiation of any CD-related medications and adverse events). Serum [UST] were categorised as therapeutic, subtherapeutic or uninterpretable based on their position in a 2 compartment PK model [subjects on Q8W dosing were assessed as per the log-linear model which projected a therapeutic level of ≥4.5 µg/ml at 4 weeks and ≥1.0 µg/ml at 8 weeks]. Results 53 patients had a clinical follow-up visit and an interpretable serum [UST]. Patients had refractory disease (89% anti-TNF-exposed, median 16.4 years since diagnosis), but clinically well (66% had baseline HBI <5) on treatment with UST for a median [IQR] of 16.5 [10.4–26.8] months. At baseline, the majority had serum [UST] in the therapeutic range (n = 44/53, 83%). After a median [IQR] of 148 [104–193] days, improved disease control was achieved by 51% of patients at next clinic visit. The outcome appeared independent of achieving therapeutic [UST] at baseline (OR[95% CI] = 0.80[0.19–3.38]). Dose-adjustment occurred in eight (15%) patients and improvements were observed in 75% of these patients. Subgroup analyses failed to detect clinical outcomes that were dependent on therapeutic serum [UST] (Table 1). No new safety signal was observed, no patient samples were positive for antibodies to UST. Conclusion The sub-study did not detect any association between routine serum [UST] and short-term clinical outcomes at the next patient visit. These data broadly suggests that the routine measurement of serum UST in CD patients does not aid in disease management, though further studies across different clinical scenarios (e.g. loss of response) are warranted.


2015 ◽  
Vol 10 (2) ◽  
pp. 165-176 ◽  
Author(s):  
Éva Brantmüller ◽  
Monika Gyuró ◽  
Ilona Karácsony

Abstract Development of the walking ability and self-care of patients with Down syndrome is affected by their body weight determining their lifestyle to a great extent. Objectives: The study aimed at the determination of body mass index for persons living in residential institutions and families, exploration its impact on walking and self-care as two, objective factors of life quality. Method: Data collection of persons aged 3-35 with Down syndrome living in families covered seven counties, while those of living in residential institutions covered thirteen counties in Hungary. In the 183 cases studied 76 people in residential institutions, 107 people lived in families. The cross-sectional study was processed by non-random sample selection. The questionnaires were filled out by health visitors and care takers edited by their own. Results: 50.6% of adults and 26.1% of children belonged to the overweight or obese category. Their residence showed a significant correlation with the body mass index (p< 0.001). Overweight and obese persons in families, while thin ones were more prevalent in institutions. Regarding the walking ability and self-care of the persons living in families a significantly higher level of development was achieved (p< 0.001). Walking ability (p = 0.001) and self-care (p = 0,008) were worsened by less body weight significantly, while overweight or obesity influenced it less negatively. Discussion: The claim is not further acceptable whereas persons with Down syndrome are more prone to obesity than average people. However unfavourable weight gain in adults draws attention to the necessity to a healthy diet and regular exercise. The people living in residential institutions with significantly lower body mass index and the associated low development of walking ability and self-care envisages an urgent reform of residential institutions. Life in the institutions negatively affects the walking ability and self-care, and thus significantly reduces the quality of life of persons with Down's syndrome.


Author(s):  
S Ahmed ◽  
K Waterhouse ◽  
A Vitali

Background: Studies have placed the rate of infection associated with neuromodulation units to be up to 20%. We present our experience with the TYRX absorbable antibiotic envelope. Our length of follow-up adds to the body of evidence around the use of antibiotic envelops. Methods: We conducted a retrospective chart review of patients referred to our center for either new implantation or revision of neuromodulation units between July 2014 and September 2016. Consecutive cases were included for analysis. We included a control group of consecutive patients with neuromodulation units placed immediately prior to our experience with the TYRX envelopes for comparison Results: Between July 2014 and September 2016, 76 patients had 81 instances of neuromodulation unit insertion. All patients received the TYRX antibiotic envelope. There were no incidences of infection involving antibiotic envelope-containing implants over an average follow-up period of 11 months. In 77 consecutive cases of neuromodulation unit implantation prior to usage of the antibiotic pouch, there were 4 instances of infection (5.2%). Conclusions: Our single center experience demonstrates a significant drop in the rate of infections with the use of an antibiotic envelope for neuromodulation unit implantation. We consider the routine use of the envelope to be a cost-effective method of infection avoidance.


Author(s):  
Anja Wegner ◽  
Elke Doberentz ◽  
Burkhard Madea

AbstractIn sauna-associated deaths, the vitality of heat exposure is of great importance. Two case reports address this. First, we present the case of a 77-year-old man who was found dead in the sauna of his family home. When found, the sauna door was closed, and the sauna indicated a temperature of 78 °C. The body had already begun to decay and was partially mummified when it was found. In the other case, a 73-year-old woman was found dead in the sauna by her husband. In this case, the sauna door was also closed. The sauna was still in operation at a temperature of approximately 70 °C. Epidermal detachments were found. In both autopsies and their follow-up examinations, there were no indications of a cause of death competing with heat shock. The expression of heat shock proteins in kidneys and lungs and the expression of aquaporin 3 in skin were investigated to detect pre-mortal temperature influences.


2021 ◽  
Vol 11 (4-S) ◽  
pp. 258-262
Author(s):  
Hina V Kouser ◽  
Mohd Nayab ◽  
Ayesha Tehseen ◽  
Shaista Mahfooz ◽  
Baig Ruqaiyya ◽  
...  

Ilaj bi’l tadbeer (regimenal therapy) is one of the preferred treatment methods used in Unani system of medicine since ancient times. Ḥijāma is one of the effective modalities of treatment in Ilaj bi’l tadbeer. It is a unique therapeutic procedure in which cup like vessels are placed on the body surface by creating a vacuum to either let out blood from below the skin surface or just plain suction without bloodletting. The objective of this comprehensive review paper is to address the claims of Unāni physicians and clinical studies conducted on the efficacy of Ḥijāma. Various published clinical trials showed the efficacy of Ḥijāma in the management of a number of diseases. Though, the effect showed by some clinical trials was short term. Hence, it is suggested that rigorous, well-designed, controlled, randomized and long duration follow up clinical trials on large sample size are to be conducted by trained clinicians or researchers to establish the efficacy of Ḥijāma in the management of various diseases. Keywords: Ilaj bi’l tadbeer, Ḥijāma, Therapeutic Benefits


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