scholarly journals Point Prevalence and Associated Factors of Hip Displacement in Pediatric Patients With Mitochondrial Disease

2021 ◽  
Vol 9 ◽  
Author(s):  
Sungmin Kim ◽  
Young-Mock Lee ◽  
Kun-Bo Park ◽  
Minsu Lee ◽  
Hoon Park

Objective: Mitochondrial disease is a multisystem disorder resulting from mitochondrial dysfunction. Although musculoskeletal system is vulnerable to mitochondrial dysfunction, little information is available on orthopedic issues such as hip displacement and scoliosis in patients with mitochondrial disease. We aimed to examine the point prevalence of hip displacement and investigate the associated factors in patients with mitochondrial disease.Methods: We retrospectively reviewed the medical records and plain radiographs of patients diagnosed with mitochondrial disease between January 2006 and January 2019 at a single institution. Data, including patient age, sex, follow-up duration, syndromic diagnosis, and gross motor function were collected. Migration percentage was measured on the radiographs. The clinical and radiologic variables were compared between patients classified according to the presence of hip displacement and motor function level.Results: We included 225 patients (135 men, 90 women). The mean age at the latest follow-up was 11.1 years, and the mean follow-up duration was 7.0 years. Hip displacement was noted in 70 (31.1%) patients. The proportion of patients with Leigh disease (p = 0.007) and the ratio of non-ambulators (p < 0.001) were higher among patients with hip displacement. The proportion of patients with Leigh disease was higher in the non-ambulators than the ambulators.Conclusion: One-third of patients with mitochondrial disease developed hip displacement. Hip displacement was more common in non-ambulators or patients with hypertonia. Careful and serial monitoring for hip problems is required for non-ambulatory patients with mitochondrial disease who have increased muscle tone.

2019 ◽  
Vol 36 (9) ◽  
pp. 1935-1943
Author(s):  
Qijia Zhan ◽  
Xidan Yu ◽  
Wenbin Jiang ◽  
Min Shen ◽  
Shuyun Jiang ◽  
...  

Abstract Purpose Our aim was to test whether the newly modified rhizotomy protocol which could be effectively used to guide single-level approach selective dorsal rhizotomy (SL-SDR) to treat spastic hemiplegic cases by mainly releasing those spastic muscles (target muscles) marked pre-operatively in their lower limbs was still applicable in spastic quadriplegic or diplegic cerebral palsy (CP) cases in pediatric population. Methods In the current study, we retrospectively conducted a cohort review of cases younger than 14 years of age diagnosed with spastic quadriplegic or diplegic CP who undergone our modified protocol-guided SL-SDR in the Department of Neurosurgery, Children’s Hospital of Shanghai since July 2016 to November 2017 with at least 12 months post-op intensive rehabilitation program (pre-op GMFCS level-based). Clinical data including demographics, intra-operative EMG responses interpretation, and relevant assessment of included cases were taken from the database. Inclusion and exclusion criteria were set for the selection of patients in the current study. Muscle tone (modified Ashworth scale) and strength of those spastic muscles (muscle strength grading scale), range of motion (ROM) of those joints involved, the level of Gross Motor Function Classification System (GMFCS), and Gross Motor Function Measure 66 items (GMFM-66) score of those cases were our focus. Results A total of 86 eligible cases were included in our study (62 boys). Among these patients, 61.6% were quadriplegic. Pre-operatively, almost 2/3 of our cases were with GMFCS levels II and III. Mean age at the time of surgery in these cases was 6.2 (3.5–12) years. Pre-op assessment marked 582 target muscles in these patients. Numbers of nerve rootlets tested during SDR procedure were between 52 and 84 across our cases, with a mean of 66.5 ± 6.7/case. Among those tested (5721 in 86 cases), 47.9% (2740) were identified as lower limb-related sensory rootlets. Our protocol successfully differentiated sensory rootlets which were considered to be associated with spasticity of target muscles across all our 86 cases (ranged from 3 to 21). Based on our protocol, 871 dorsal nerve rootlets were sectioned 50%, and 78 were cut 75%. Muscle tone of those target muscles reduced significantly right after SL-SDR procedure (3 weeks post- vs. pre-op, 1.7 ± 0.5 vs. 2.6 ± 0.7). After an intensive rehabilitation program for 19.9 ± 6.0 months, muscle tone continued to decrease to 1.4 ± 0.5. With the reduction of muscle tone, strength of those target muscles in our cases improved dramatically with statistical significance achieved (3.9 ± 1.0 at the time of last follow-up vs. 3.3 ± 0.8 pre-op), and as well as ROM. Increase in GMFCS level and GMFM-66 score was observed at the time of last follow-up with a mean of 0.4 ± 0.6 and 6.1 ± 3.2, respectively, when compared with that at pre-op. In 81 cases with their pre-op GMFCS levels II to V, 27 (33.3%) presented improvement with regard to GMFCS level upgrade, among which 4 (4.9%) even upgraded over 2 levels. Better results with regard to upgrading in level of GMFCS were observed in cases with pre-op levels II and III when compared with those with levels IV and V (24/57 vs. 3/24). Upgrading percentage in cases younger than 6 years at surgery was significantly greater than in those older (23/56 vs. 4/25). Cases with their pre-op GMFM-66 score ≥ 50 had greater score increase of GMFM-66 when compared with those less (7.1 ± 3.4 vs. 5.1 ± 2.8). In the meanwhile, better score improvement was revealed in cases when SDR performed at younger age (6.9 ± 3.3 in case ≤ 6 years vs. 4.7 ± 2.7 in case > 6 years). No permanent surgery-related complications were recorded in the current study. Conclusion SL-SDR when guided by our newly modified rhizotomy protocol was still feasible to treat pediatric CP cases with spastic quadriplegia and diplegia. Cases in this condition could benefit from such a procedure when followed by our intensive rehabilitation program with regard to their motor function.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Dhanach Dhirachaikulpanich ◽  
Kunravitch Soraprajum ◽  
Sutasinee Boonsopon ◽  
Warinyupa Pinitpuwadol ◽  
Preeyachan Lourthai ◽  
...  

AbstractTo demonstrate the demographics, associated factors, clinical presentations, microbiology, management, visual outcome and complications of keratitis/scleritis-related endophthalmitis (KSE). A retrospective chart of all endophthalmitis patients diagnosed between September 2001 and August 2011 was reviewed. Only endophthalmitis cases with previous corneal or scleral infection were included in the study. The patients were followed until losing vision or eyeball, becoming phthisis, or the end of 2018. Eighty-seven patients with KSE were identified, all unilateral. The mean age was 56.4 ± 21.4 years. There was a slight male predilection (55 patients, 63.2%). The mean follow-up time was 50 ± 149 weeks. The causative pathogens were identified in 35 patients (40.2%), with the highest frequency being bacteria. The most common bacterium was Pseudomonas aeruginosa (n = 13), and the most common fungus was Aspergillus sp. (n = 5). Fifteen patients achieved (17.2%) final visual acuity (VA) of hand motion or better after treatment. Eyeball removal was performed in 61 (70.1%) patients. From multivariate analysis, the only prognostic factor for poor final VA (worse than hand motion, HM) was poor VA (worse than HM) at the initial visit (relative risk 1.97, 95% confidence interval 1.15–3.36, p = 0.013). KSE is uncommon but has a devastating outcome. We found that the patient’s initial VA was the only predictor for their final vision. P. aeruginosa was the most common identifiable organism in this study. However, several fungal infections were recognised. These findings should raise awareness for treatment of KSE in the tropics.


2021 ◽  
Vol 10 (11) ◽  
pp. 2376
Author(s):  
Dayoung Ko ◽  
Hee-Beom Yang ◽  
Joong Youn ◽  
Hyun-Young Kim

Chronic intestinal pseudo-obstruction (CIPO) is an extremely rare condition with symptoms of recurrent intestinal obstruction without any lesions. The outcomes of pediatric CIPO and predictors for the outcomes have not yet been well established. We analyzed the clinical outcomes and associated factors for the outcomes of pediatric CIPO. We retrospectively reviewed 66 primary CIPO patients diagnosed between January 1985 and December 2017. We evaluated parenteral nutrition (PN) factors such as PN duration, PN use over 6 months, home PN, and mortality as outcomes. We selected onset age, presence of urologic symptoms, pathologic type, and involvement extent as predictors. The early-onset CIPO was found in 63.6%, and 21.2% of the patients presenting with urologic symptoms. Of the 66 patients, 47 and 11 had neuropathy and myopathy, respectively. The generalized involvement type accounted for 83.3% of the cases. At the last follow-up, 24.2% of the patients required home PN management. The mean duration of PN was 11.8 ± 21.0 months. The overall mortality rate of primary CIPO was 18.2%. PN factors were predicted by the urologic symptoms and extent of involvement. However, mortality was predicted by pathologic type. The onset age was not significantly associated with the outcomes. CIPO with urologic symptoms and generalized CIPO had poor PN outcomes. Myopathy is suggested as a predictor of mortality in children with primary CIPO.


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Taklo Simeneh Yazie

Hepatotoxicity was found in different case reports and studies in tenofovir disoproxil fumarate- (TDF-) based regimen. However, there was no data regarding liver enzymes, glucose, and hemoglobin in Ethiopian patients receiving TDF-based regimen. The aim of this study was to determine elevated liver enzymes and its associated factors as well as elevated fasting plasma glucose and anemia. A hospital-based observational prospective cohort study was conducted on conveniently selected 63 patients in Tikur Anbessa Specialized Hospital (TASH) from January to September 2019. Laboratory values were determined at pre-TDF-based regimen baseline and six-month follow-up. The data was analyzed by using SPSS version 21.0, and multivariate logistic regression was used to determine associated factors with elevated liver enzymes. The overall elevated liver enzymes were found in 26 (41.3%) participants. From this, elevated alanine aminotransferase (ALT), aspartate aminotransferase (AST), and alkaline phosphatase (ALP) comprise 3 (4.8%), 3 (4.8%), and 20 (31.8%), respectively. Elevated fasting plasma glucose (FPG) was found in 9 (14.3%) and 14 (22.2%) of participants at baseline and six-month visit, respectively. At six-month visit, 4 (6.4%) of participants experienced anemia. The mean value of ALP and FPG at six months was significantly higher than their respective baseline mean values ( mean   difference   MD = + 63.38 , 95% CI (39.84, 86.92), p = 0.0001 ; MD = + 6.64 , 95% CI (2.63, 10.64), p = 0.002 , respectively). The mean value of ALT, AST, and Hg at six months was slightly increased compared to their respective baseline mean values, but the difference was not significant. In multivariate analysis, only female sex was significantly associated with elevated ALP ( AOR = 4.5 , 95% CI (1.03, 19.6), p = 0.045 ). Overall mild and moderate hepatotoxicity was found to be high (26, 41.3%) in the present study, and from this, the majority was comprised by elevated ALP (20, 31.8%). The proportion of participants with hyperglycemia was increased at the end of follow-up compared to its baseline value, but anemia was not. Female sex was significantly associated with elevated ALP. This study warrants monitoring of liver enzymes and glucose in TDF-based regimen.


Pain Medicine ◽  
2020 ◽  
Author(s):  
César Fernández-de-las-Peñas ◽  
Albert Pérez-Bellmunt ◽  
Luis Llurda-Almuzara ◽  
Gustavo Plaza-Manzano ◽  
Ana I De-la-Llave-Rincón ◽  
...  

Abstract Objective To evaluate the effects of muscle dry needling alone or combined with other interventions on post-stroke spasticity (muscle tone), related pain, motor function, and pressure sensitivity. Databases and Data Treatment Electronic databases were searched for randomized controlled trials including post-stroke patients where at least one group received dry needling and outcomes were collected on spasticity and related pain. Secondary outcomes included motor function and pressure pain sensitivity. Data were extracted by two reviewers. The risk of bias was assessed with the Cochrane Risk of Bias tool, methodological quality was assessed with the Physiotherapy Evidence Database score, and the quality of evidence was assessed by the Grading of Recommendations Assessment, Development, and Evaluation approach. Between-groups mean differences (MDs) and standardized mean differences (SMDs) were calculated. Results Seven studies (three within the lower extremity, four in the upper extremity) were included. The meta-analysis found significantly large effect sizes of dry needling for reducing spasticity (SMD: -1.01, 95%confidence interval [CI] -1.68 to -0.34), post-stroke pain (SMD -1.01, 95%CI -1.73 to -0.30), and pressure pain sensitivity (SMD 1.21, 95% CI: 0.62 to 1.80) as compared with a comparative group at short-term follow-up. The effect on spasticity was found mainly in the lower extremity (MD -1.05, 95% CI: -1.32 to -0.78) at short-term follow-up. No effect on spasticity was seen at 4 weeks. No significant effect on motor function (SMD 0.16, 95% CI: -0.13 to 0.44) was observed. The risk of bias was generally low, but the imprecision of the results downgraded the level of evidence. Conclusion Moderate evidence suggests a positive effect of dry needling on spasticity (muscle tone) in the lower extremity in post-stroke patients. The effects on related pain and motor function are inconclusive.


Author(s):  
Adnan I Qureshi ◽  
William I Baskett ◽  
Wei Huang ◽  
Iryna Lobanova ◽  
S Hasan Naqvi ◽  
...  

Abstract Background A better understanding of re-infection after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has become one of the healthcare priorities in the current pandemic. We determined the rate of re-infection, associated factors and mortality during follow up in a cohort of patients with SARS-CoV-2 infection. Methods We analyzed 9,119 patients with SARS-CoV-2 infection who received serial tests in total of 62 healthcare facilities in United States between December 1, 2019 to November 13, 2020. Re-infection was defined by two positive tests separated by interval of greater than 90 days two after resolution of first infection was confirmed by two or more consecutive negative tests. We performed logistic regression analysis to identify demographic and clinical characteristics associated with re-infection. Results Re-infection was identified in 0.7% (n=63, 95% confidence interval [CI] 0.5%-0.9%) during follow up of 9,119 patients with SARS-CoV-2 infection. The mean period (±standard deviation [SD]) between two positive tests was 116 ± 21 days. A logistic regression analysis identified that asthma (odds ratio [OR] 1.9, 95% CI 1.1-3.2) and nicotine dependence/tobacco use (OR 2.7, 95% CI 1.6-4.5) were associated with re-infection. There was a significantly lower rate of pneumonia, heart failure, and acute kidney injury observed with re-infection compared with primary infection among the 63 patients with re-infection There were two deaths (3.2%) associated with re-infection. Conclusions We identified a low rate of re-infection confirmed by laboratory tests in a large cohort of patients with SARS-CoV-2 infection. Although re-infection appeared to be milder than primary infection, there was associated mortality.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 458.1-459
Author(s):  
H. J. Choi ◽  
K. W. Moon ◽  
H. O. Kim ◽  
Y. A. Lee ◽  
S. J. Hong ◽  
...  

Background:Gout shows a seasonal variation that widely differs among geographic areas, and we previously reported a seasonal effect on gout in Korea. However, we had no information regarding changes in diet and had only limited laboratory data because this was a retrospective study in patients receiving a urate-lowering therapy. Therefore, we designed this prospective study to elucidate the seasonality and associated factors of gout attacks in Korea.Objectives:To evaluate the seasonality and associated factors of the incidence of gout attacks in Korea.Methods:We prospectively enrolled patients with gout attacks who were treated at nine rheumatology clinics between January 2015 and July 2018 and followed them for 1-year. Demographic data, clinical and laboratory features, and meteorological data including seasonality were collected.Results:Two hundred-five patients (males, 94.1%) were enrolled. The proportion of patients with initial gout attacks was 46.8% (n = 96). The mean age, body mass index, attack duration, and serum uric acid level at enrollment were 50.5 years, 26.1, 10.2 days, and 7.3 mg/dL, respectively. Gout attacks were most common during spring (43.4%,P< 0.001) and in March (23.4 %,P< 0.001). A similar pattern of seasonality was observed in the group with initial gout attacks. Alcohol was the most common provoking factor (39.0%), particularly during summer (50.0%). The mean diurnal temperature change on the day of the attack was highest in the spring (10.3°C), followed by winter (9.1°C), summer (8.1°C), and fall (8.0°C) (P= 0.027). The mean change in humidity between the 2 consecutive days (the day before and the day of the attack) was significantly different among the seasons (3.4%, spring; 0.2%, summer; 0.4%, fall; -3.9%, winter;P= 0.015). One hundred twenty-five (61%) patients completed 1-year follow-up (51% in the initial attack group). During the follow-up period, 51 gout flares developed (18 in the initial attack group). No significant seasonal variation in the follow-up flares was found.Conclusion:In this prospective study, the most common season and month of gout attacks in Korea were spring and March, respectively. Alcohol was the most common provoking factor, particularly during summer. Diurnal temperature changes on the day of the attack and humidity changes from the day before the attack to the day of the attack were associated with gout attack in our cohort.References:[1]Choi HJ, Lee CH, Lee JH et al. Seasonality of gout in Korea: a multicenter study. J Korean Med Sci 2015;30:240-4.Disclosure of Interests: :None declared


Author(s):  
Julie L. Wambaugh ◽  
Lydia Kallhoff ◽  
Christina Nessler

Purpose This study was designed to examine the association of dosage and effects of Sound Production Treatment (SPT) for acquired apraxia of speech. Method Treatment logs and probe data from 20 speakers with apraxia of speech and aphasia were submitted to a retrospective analysis. The number of treatment sessions and teaching episodes was examined relative to (a) change in articulation accuracy above baseline performance, (b) mastery of production, and (c) maintenance. The impact of practice schedule (SPT-Blocked vs. SPT-Random) was also examined. Results The average number of treatment sessions conducted prior to change was 5.4 for SPT-Blocked and 3.9 for SPT-Random. The mean number of teaching episodes preceding change was 334 for SPT-Blocked and 179 for SPT-Random. Mastery occurred within an average of 13.7 sessions (1,252 teaching episodes) and 12.4 sessions (1,082 teaching episodes) for SPT-Blocked and SPT-Random, respectively. Comparisons of dosage metric values across practice schedules did not reveal substantial differences. Significant negative correlations were found between follow-up probe performance and the dosage metrics. Conclusions Only a few treatment sessions were needed to achieve initial positive changes in articulation, with mastery occurring within 12–14 sessions for the majority of participants. Earlier occurrence of change or mastery was associated with better follow-up performance. Supplemental Material https://doi.org/10.23641/asha.12592190


VASA ◽  
2012 ◽  
Vol 41 (2) ◽  
pp. 90-95 ◽  
Author(s):  
Rastan ◽  
Noory ◽  
Zeller

We have investigated the role of drug-eluting stents on patency rates after treatment of focal infrapopliteal lesions in patients with intermittent claudication and critical limb ischemia. Reports indicate that drug-eluting stents reduce the risk of restenosis after percutaneous infrapopliteal artery revascularization. A Pub Med, EMBASE, Cochrane database review search of non-randomized studies investigating patency rates, target lesion revascularisation rates, limb salvage rates and mortality rates in an up to 3-year follow-up period after drug-eluting stent placement was conducted. In addition, preliminary results of randomized studies comparing drug-eluting stents with bare-metal stents and plain balloon angioplasty in treatment of focal infrapopliteal lesions were included in this review. A total of 1039 patients from 10 non-randomized and randomized studies were included. Most commonly used drug-eluting stents were sirolimus-eluting. The mean follow-up period was 12.6 (range 8 - 24). The mean 1-year primary patency rate was 86 ± 5 %. The mean target lesion revascularization rate and limb salvage rate was 9.9 ± 5 % and 96.6 %±4 %, respectively. Results from non-randomized and preliminary results from prospective, randomized trials show a significant advantage for drug-eluting stents in comparison to plain balloon angioplasty and bare-metal stents concerning target lesion patency and in parts target lesion revascularisation. No trial reveals an advantage for drug-eluting stents with regard to limb salvage and mortality.


Swiss Surgery ◽  
2002 ◽  
Vol 8 (6) ◽  
pp. 255-258 ◽  
Author(s):  
Perruchoud ◽  
Vuilleumier ◽  
Givel

Aims: The purpose of this study was to evaluate excision and open granulation versus excision and primary closure as treatments for pilonidal sinus. Subjects and methods: We evaluated a group of 141 patients operated on for a pilonidal sinus between 1991 and 1995. Ninety patients were treated by excision and open granulation, 34 patients by excision and primary closure and 17 patients by incision and drainage, as a unique treatment of an infected pilonidal sinus. Results: The first group, receiving treatment of excision and open granulation, experienced the following outcomes: average length of hospital stay, four days; average healing time; 72 days; average number of post-operative ambulatory visits, 40; average off-work delay, 38 days; and average follow-up time, 43 months. There were five recurrences (6%) in this group during the follow-up period. For the second group treated by excision and primary closure, the corresponding outcome measurements were as follows: average length of hospital stay, four days; average healing time, 23 days; primary healing failure rate, 9%; average number of post-operative ambulatory visits, 6; average off-work delay, 21 days. The average follow-up time was 34 months, and two recurrences (6%) were observed during the follow-up period. In the third group, seventeen patients benefited from an incision and drainage as unique treatment. The mean follow-up was 37 months. Five recurrences (29%) were noticed, requiring a new operation in all the cases. Discussion and conclusion: This series of 141 patients is too limited to permit final conclusions to be drawn concerning significant advantages of one form of treatment compared to the other. Nevertheless, primary closure offers the advantages of quicker healing time, fewer post-operative visits and shorter time off work. When a primary closure can be carried out, it should be routinely considered for socio-economical and comfort reasons.


Sign in / Sign up

Export Citation Format

Share Document