Long-term Clinical Study Research Articles

Long-term clinical study and multiscale analysis of in vivo biodegradation mechanism of Mg alloy

There has been a tremendous amount of research in the past decade to optimize the mechanical properties and degradation behavior of the biodegradable Mg alloy for orthopedic implant. Despite the feasibility of degrading implant, the lack of fundamental understanding about biocompatibility and underlying bone formation mechanism is currently limiting the use in clinical applications. Herein, we report the result of long-term clinical study and systematic investigation of bone formation mechanism of the biodegradable Mg-5wt%Ca-1wt%Zn alloy implant through simultaneous observation of changes in element composition and crystallinity within degrading interface at hierarchical levels. Controlled degradation of Mg-5wt%Ca-1wt%Zn alloy results in the formation of biomimicking calcification matrix at the degrading interface to initiate the bone formation process. This process facilitates early bone healing and allows the complete replacement of biodegradable Mg implant by the new bone within 1 y of implantation, as demonstrated in 53 cases of successful long-term clinical study.

A RANDOMIZED ACTIVE CONTROLLED CLINICAL STUDY TO EVALUATE EFFICACY AND SAFETY OF RESVERATROL AS AN ADJUVANT THERAPY IN PATIENTS WITH HYPERTENSION

ABSTRACTObjectives: No long-term clinical efficacy and safety study of resveratrol as adjuvant therapy along with gold standard therapy has been conductedin patients with essential hypertension. The aim of this study was to investigate the efficacy and safety of resveratrol as an adjuvant therapy in newlydiagnosed hypertensive patients.Methods: In this randomized active-controlled study, hypertensive patients (male/female) of 20-65 years aged were randomized (1:1) to receivetelmisartan 20 mg or telmisartan 20 mg plus resveratrol 1 g daily for 12 months. Efficacy variables included a change in systolic and diastolic bloodpressure (DBP) from baseline and were followed up for every 3 months. Treatment-emergent adverse events were assessed.Results: A total of 60 hypertensive patients were analyzed (telmisartan [n=30] and telmisartan plus resveratrol [n=30]). Resveratrol as an adjuvantwith telmisartan significantly reduced in systolic (p<0.001) and DBP (p<0.001) as compared to telmisartan monotherapy. Change in systolic and DBPfrom baseline was significantly higher in telmisartan plus resveratrol group than telmisartan (p<0.001) at all followed visits. Both the study drugshave similar safety profile and found well tolerable.Conclusions: Resveratrol plus Telmisartan was found to be superior over telmisartan monotherapy in reducing systolic and DBP in newly diagnosedhypertensive patients. Both the study drugs were effective with comparable safety profile. Our study supports the long-term clinical efficacy andsafety study of resveratrol along with gold standard therapy in essential hypertension.Keywords: Essential hypertension, Resveratrol, Systolic and diastolic blood pressure, Telmisartan.

Efficacy and Safety of Metreleptin in Patients with Partial Lipodystrophy: Lessons from an Expanded Access Program.

ObjectivePatients with lipodystrophy have severe metabolic abnormalities (insulin resistance, diabetes, and hypertriglyceridemia) that may increase morbidity and mortality. Metreleptin is approved by the United States Food and Drug Administration for treatment of generalized forms of lipodystrophy. We aimed to determine the efficacy and safety of metreleptin among patients with partial lipodystrophy using an expanded-access model.MethodsStudy FHA101 (ClinicalTrials.gov identifier: NCT00677313) was an open-label, expanded-access, long-term clinical effectiveness and safety study in 23 patients with partial lipodystrophy and diabetes and/or hypertriglyceridemia with no prespecified leptin level. Metreleptin was administered subcutaneously at 0.02 mg/kg twice daily (BID) at Week 1, followed by 0.04 mg/kg BID at Week 2. Dose adjustments thereafter were based on patient response (maximum dose of 0.08 mg/kg BID). One-year changes in glycated hemoglobin (HbA1c), fasting plasma glucose, triglycerides, alanine and aspartate aminotransferases, and treatment-emergent adverse events (TEAEs) were evaluated.ResultsHbA1c, fasting plasma glucose, and triglycerides were numerically decreased throughout 1 year, with mean (standard error) changes from baseline of −0.88 (0.62)%, −42.0 (22.4) mg/dL, and −119.8 (84.1) mg/dL, respectively, which were greater among patients with higher baseline abnormalities. Liver enzymes did not worsen, and the most frequently observed TEAEs (≥ 10% incidence) were mild to moderate and included nausea (39.1%), hypoglycemia (26.1%), and urinary tract infections (26.1%)—all reported previously. There were no reports of clinically significant immune-related adverse events or new safety signals.ConclusionsOur clinical observations document the large heterogeneity and disease burden of partial lipodystrophy syndromes and suggest that metreleptin treatment benefits may extend to patients with partial lipodystrophy. Additional studies are needed to confirm these preliminary findings.

Clinical evaluation of ceramic inlays and onlays fabricated with two systems: 12-year follow-up.

The aim of this study was to evaluate the 12-year clinical performance of sintered (Duceram, Dentsply-Degussa, Dentsply International Inc., PA, USA)-D and pressable (IPS Empress, Ivoclar-Vivadent, Schaan, Leichtenstein)-IPS ceramic inlay and onlay restorations. Eighty-six restorations were placed in 35 patients with a median age of 33years by a single operator. The restorations were luted with dual-cured resin luting material (Variolink II, Ivoclar-Vivadent, Schaan, Leichtenstein) and Syntac Classic adhesive under rubber dam. The evaluations were conducted by two independent investigators at the baseline, 1, 2, 3, 5, and 12years using the modified USPHS criteria. At the 12-year recall, 22 patients were evaluated (62.86%), totalling 48 (55.81%) restorations. Seven restorations were fractured (one from Duceram and six from IPS), eight restorations presented secondary caries (three from Duceram and five from IPS), nine restorations showed unacceptable defects at the restoration margin and needed repair or replacement (two from Duceram and seven from IPS), and four IPS restorations presented pulp sensitivity. Chi-square and Mann-Whitney tests revealed that IPS Empress system showed the best results for color match and surface texture (p<0.05) but a significant worse result for fracture (p=0.05). Wilcoxon test showed significant differences in relation to color match, surface texture, marginal discoloration, and marginal integrity between the baseline and 12-year recall for both ceramic systems. This long-term clinical study observed that the main reasons for failure of ceramic restorations were related to fracture, recurrent caries, and decreased marginal integrity over time. Carefully monitoring of the ceramic-tooth interface may extend their clinical longevity.

소아근시에 대한 눈 주위혈 지압의 유효성 및 안전성 평가 연구

Objectives Myopia has a higher prevalence rate in eastern countries, which also have a higher rate of educational fever compared to western countries. Considering this, social costs paid for myopia will increase rapidly in Korea. Although the development of myopia treatment is necessary, there has been a lack of relevant studies. Thus, this study aims to produce data to prevent unnecessary treatments and medical expenses. Methods The objective is to evaluate the effect of periocular acupressure exerted by a medical massager for myopia. This is an open-label, prospective, single-arm, and pre and post superiority study. The subjects are 7~12-year-old myopia patients with under 5 D (diopter) of spherical equivalent. A total of 56 subjects were enrolled. The selected subjects will wear the massager for 15 minutes twice a day for 24 weeks. The primary endpoint is the refraction change. The secondary endpoint is the axial length change. Statistical analysis was performed at a significant level of 0.05, using a two-tailed test. The criterion for significantly improved refraction was <TEX>$-0.17{\pm}0.50$</TEX> D/6 months and that of axial length change was 0.126 mm. Results and Conclusions This study did not include a control group because children represent a vulnerable group. This objective study will bring some impact on Korean medical myopia treatment. A long-term confirmatory clinical study may be necessary in future.

Dissolution of alkaline earth silicate wool and polycrystalline wool in saline solution

Tobacco Heating Products (THPs) are tobacco products that heat rather than burn tobacco with temperatures less than 350 °C. Because of this operating principle, they produce substantially fewer and lower levels of tobacco smoke toxicants than combustible cigarette smoke produced when tobacco is burnt, which occurs at much higher temperatures of around 900 °C. This paper analyses data on a THP, glo™, and assesses whether its use would result in reduced health risks compared to the health risks of smoking cigarettes. It also looks at the possibility of bridging datasets across the different variants of the glo™ product.The approach is to consider whether datasets from behavioural, chemical, toxicological and clinical studies provide consistent findings of reductions in toxicant exposure with glo™ use by subjects who switch completely from smoking cigarettes to using glo™ and whether these reductions are similar to those who stop smoking cigarettes without switching to glo™ or any other tobacco or nicotine product. We also examine the similarities and differences of different versions of the glo™ product and benchmark it against a THP from another manufacturer.The studies indicate that the use of the glo™ results in substantial and prolonged reductions in toxicant exposure for smokers who switch to glo™ completely. A long-term clinical study shows substantial reductions in toxicant exposure over a period of time, similar to reduction of some biomarkers of exposure found following smoking cessation without switching to glo™ or any other tobacco product, and biomarkers of potential harm trending in a favourable manner for both groups that switch to glo™ and that quit all tobacco and nicotine use. Data suggests that all iterations of glo™ result in substantial reductions in toxicant exposure compared to smoking cigarettes and that bridging across datasets is feasible.Given the accumulated scientific data summarised in this paper, and particularly the findings from a long-term clinical study, the data demonstrate that glo™ is a reduced exposure product compared to combustible cigarettes and is reasonably deemed to reduce the risk of smoking-related diseases and supports the conclusion that smokers who would have otherwise continued to smoke and instead switch entirely to THP glo™ use, will reduce their relative risk of developing smoking-related diseases as compared to continued smoking. The extent of reduction in risk compared to continuing to smoke is likely to vary by smoking-related disease and by an individuals’ smoking history, other risk factors and an individual’s susceptibility to disease. Use of the THP will present some level of increased health risk as compared to cessation of tobacco and nicotine products and will cause dependence. As long as the principles of heat-not-burn are maintained, THP use will result in substantially reduced exposure to smoke toxicants as compared to continued conventional cigarette smoking. It is possible to use bridging or read across to apply these conclusions to new iterations of the glo™ product, extending the utility and validity of the evidence generated through study of prior iterations.

Renoprotective effects of incretin-based drugs: A novel pleiotropic effect of dipeptidyl peptidase-4 inhibitor.

Renoprotective effects of incretin-based drugs: A novel pleiotropic effect of dipeptidyl peptidase-4 inhibitor.

A Long-Term Clinical and Cephalometric Study of Cleft Lip and Palate Patients following Intraoral Maxillary Quadrangular Le Fort I Osteotomy

To evaluate the horizontal and vertical stability of the quadrangular Le Fort I in patients with congenital cleft lip and palate. Prospective longitudinal study. A total of 15 congenital cleft lip and palate patients treated with the maxillary quadrangular Le Fort I were enrolled. Lateral cephalometric radiographic examinations were obtained preoperatively, early postoperatively, and late postoperatively for four dental and skeletal landmarks. A questionnaire regarding patients' satisfaction with treatment and functional/cosmetic outcomes (airway, speech, mastication) was administered. Surgical horizontal and vertical movement, late postsurgical horizontal and vertical movement, and surgical and postsurgical movement in relation to age and cleft type were evaluated using Spearman correlation coefficients, Wilcoxon signed rank tests, and Mann-Whitney tests. Surgical horizontal movements of all measured points showed significant changes. Significant differences of postsurgical horizontal movement were observed in younger patients versus adult patients. Significant differences of postsurgical horizontal movement were observed in unilateral cleft patients versus bilateral cleft patients. A high percentage of patients showed significant functional improvement in nasal airflow, speech, mastication, temporomandibular joint function, and mouth versus nose breathing. The quadrangular Le Fort I is a functionally stable and a surgically predictable procedure for cleft lip and palate patients who present with midface deficiency. Patients under the age of 18 at the time of the osteotomy had a higher relapse rate than patients over 18 years of age. Younger patients who need surgery should be advised regarding the increased risk of skeletal relapse. Patients' satisfaction was high in all aesthetic- and function-related items on the questionnaire.

Chronic manganism: A long-term follow-up study with a new dopamine terminal biomarker of 18F-FP-(+)-DTBZ (18F-AV-133) brain PET scan

Recent experimental studies revealed that dopamine neuron dysfunction in chronic manganism may be due to a reduced capacity of dopamine release in the striatum. The findings imposed further difficulty in the differential diagnosis between manganism and IPD. We conducted a long-term clinical follow-up study of 4 manganism patients, applying a new tracer 18F-9-fluoropropyl-(+)-dihydrotetrabenazine (18F-AV-133) with positron emission tomography (PET). Twenty age-matched subjects including 4 manganism patients, 8 idiopathic Parkinson's disease (IPD) patients, and 8 healthy controls were enrolled for comparison. Volumes of interest of the bilateral putamen, caudate nuclei and occipital cortex as the reference region were delineated from individual magnetic resonance images. The clinical features of the manganism patients still progressed, with increased scores on the Unified Parkinson Disease Rating Scale. The 18F-AV-133 uptake in the IPD patients decreased at the bilateral striatum, compared with the healthy controls. In the manganism patients, there was no decreased uptake of radioactivity involving the bilateral striatum, except Patient 4, who had a stroke with decreased uptake in the right posterior putamen. The 18F-AV-133 PET finding reveals that nigrostriatum neurons are not degenerated in chronic manganism and can provide a useful neuroimage biomarker in the differential diagnosis.

Schizophrenia in adolescence

Schizophrenia with childhood and adolescent onset is a serious clinical/social problem. It has been attempted to specify main clinical/dynamic sex-related characteristics of early onset schizophrenia, prognostic criteria and parameters of outcome. The results of a long-term (1995-2014) clinical follow-up study of outpatients of a psychoneurological dispensary and inpatients of a Moscow Psychiatric Hospital №15, aged 15-17 years, were analyzed. There was the predominance of men over women (4:1). The evidence of poor outcome of childhood and adolescent onset schizophrenia was obtained. At the age of 18 years, no one patient had stable remission, more than 40% of the patients were disabled since childhood. Most of adolescents were disabled in one or another activity. At the same time, there was a relative stabilization of the early malignant variants of schizophrenia resulted in oligophrenia-like deficit. The delayed (for several years) and frequently mistaken diagnosis and the absence of adequate treatment are the most robust premises of the development of early disability, along with the severity of destructive potential of the disease. The more distinct qualification of early symptoms of schizophrenia with childhood and adolescent onset, search for social/therapeutic approaches directed to the prevention of exacerbations and prophylaxis of social incompetence of adolescents are needed.

More Effective Strategies are Needed for Elderly Asthmatics in Real-World Practice.

More Effective Strategies are Needed for Elderly Asthmatics in Real-World Practice.

Progression of early retinal dysfunction in diabetes over time: results of a long-term prospective clinical study.

We explored signs of retinal dysfunction over time in diabetic subjects before or early in the course of retinopathy. Patients with no, mild, or moderate retinopathy were consecutively recruited and underwent standard automated perimetry, visual acuity measurement, and fundus photography. These examinations and measurements of HbA1c and blood pressure were repeated for up to 5 years from baseline. Visual field improvement/deterioration in diabetic subjects was evaluated using significance limits for change. Progression or regression of retinopathy was defined as a two-step change on the Early Treatment Diabetic Retinopathy Study final severity scale. Seventy-four subjects completed at least 3 years of follow-up, and 22% showed visual field worsening, defined as repeated significant deterioration at ≥10% of the test points, whereas only 1% showed field improvement. Worsening occurred in subjects both with and without vascular lesions. The degree of retinopathy was stable throughout the observation period in 68 of 74 eyes, improved in 4, and worsened in 2. Visual field deterioration was not correlated with a change in retinopathy. By using perimetry with an analysis tailored for monitoring diabetic subjects, we were able to demonstrate progression of retinal dysfunction over time, which may represent early signs of retinal neurodegeneration.

Risk Stratification and Clinical Pathways to Optimize Length of Stay After Transcatheter Aortic Valve Replacement

BackgroundTranscatheter aortic valve replacement (TAVR) program experience and advances present opportunities to introduce minimalist clinical pathways. The purpose of this study was to determine the safety and feasibility of preprocedural individualized risk stratification for general anaesthesia and transesophageal echocardiography (GA/TEE) or awake TAVR and the postprocedural standard or rapid discharge TAVR clinical pathways. MethodsStandardized screening and multidisciplinary heart team consensus was used to evaluate individual periprocedural risk and requirements. Postprocedural clinical status and criteria guided the timing of discharge. We evaluated standardized TAVR outcomes and length of stay according to periprocedural practice and postprocedural trajectory. ResultsIn 144 consecutive patients who underwent TAVR in 2013 (mean age, 82.0 ± 7.1 years; 38.2% women; mean Society of Thoracic Surgeons score, 6.5% ± 4.1%), 101 (69.1%) were assigned to the GA/TEE protocol, whereas 43 (29.9%) were assigned to the minimalist awake TAVR protocol. Irrespective of mode of anaesthesia, 94 (65.3%) patients were discharged within the standard time, whereas 50 (34.7%) patients were suitable for rapid discharge. Overall outcomes at 30 days were 2.1% mortality, 1.4% stroke, and 2.1% life-threatening bleeding. Median length of stay was shortest in the awake TAVR group (2 days; interquartile range [IQR], 1-3 days) and rapid discharge group (2 days; IQR, 1-2 days) and longer in the GA/TEE and standard discharge (3 days, IQR, 3-4 days) groups. ConclusionsExcellent outcomes and decreased length of stay can be achieved with individualized risk stratification to select the optimal periprocedural practice and determine the timing of discharge. These findings should be further evaluated in a large long-term clinical study.

Long-term clinical study of thulium laser resection of prostate-tangerine technique in the treatment of begin prostatic hyperplasia

To evaluate the long-term efficacies and complications of thulium laser resection of prostate-tangerine technique (TmLRP-TT) in the treatment of benign prostatic hyperplasia (BPH). From November 2004 to December 2009, a total of 348 BPH patients undergoing TmLRP-TT at our hospital were evaluated retrospectively for long-term efficacies and complications. The follow-up data included international prostate symptom score (IPSS), quality of life score (QOL), maximum urinary flow (Qmax) and post void urinary residual (PVR). After 4 years, IPSS decreased 70% (22.7 ± 7.7 vs 6.8 ± 5.1) , QOL decreased 65% (4.3 ± 0.7 vs 1.5 ± 1.0), Qmax increased 212% (6.0 ± 2.6 ml/s vs 18.7 ± 4.6 ml/s) and PVR decreased 83% (104.7 ± 34.3 ml vs 17.7 ± 10.7 ml). Cumulative incidences of long-term complications was 6.0% (n = 21), including a second TmLRP-TT due to BPH recurrence (n = 4, 1.2%), urethral stricture (n = 8, 2.3%) and bladder neck contracture (n = 9, 2.6%). Overall, 93% were satisfied with surgical outcomes. TmLRP-TT has excellent efficacies with a low rate of long-term complications. Most patients are satisfied with surgical outcomes.

Is commissural closure associated with mitral annuloplasty a durable technique for the treatment of mitral regurgitation? A long-term (≤15 years) clinical and echocardiographic study

Mitral regurgitation (MR) due to commissural prolapse/flail can be corrected by suturing the margins of the anterior and posterior leaflets in the commissural area (commissural closure). The long-term results of this type of repair are unknown. Our aim was to assess the clinical and echocardiographic outcomes of this technique up to 15 years after surgery. From 1997 to 2007, 125 patients (age, 56.8 ± 15.7 years; left ventricular ejection fraction, 58.1% ± 7.1%) with MR due to pure commissural prolapse/flail of 1 or both leaflets underwent commissural closure combined with annuloplasty. The etiology of the disease was degenerative in 88.8% and endocarditis in 11.2%. The commissural region involved was posteromedial in 96 patients (76.8%) and anterolateral in 29 (23.2%). Hospital mortality was 1.6%. At discharge, MR was absent or mild in 120 patients (97.5%) and moderate (2+/4+) in 3 (2.4%). Clinical and echocardiographic follow-up was 98.4% complete (mean length, 7.1 ± 3.0 years; median, 6.7; longest follow-up, 15). At 11 years, the actuarial survival, freedom from cardiac death, and freedom from reoperation was 78.8% ± 6.2%, 95.2% ± 3.3%, and 97.4% ± 1.4%, respectively. At the last echocardiographic examination, MR 3+ or greater was demonstrated in 4 patients (3.3%). Freedom from MR 3+ or greater at 11 years was 96.3% ± 1.7%. No predictors for recurrence of MR 3+ or greater were identified. The mean mitral valve area and gradient was 2.9 ± 0.4 cm(2) and 3.4 ± 1.1 mm Hg, respectively. New York Heart Association class I to II was documented in all cases. Commissural closure repair combined with annuloplasty provides excellent clinical and echocardiographic long-term results in patients with MR due to commissural lesions.

Pilot study: Hypoglycemic and antiglycation activities of bitter melon (Momordica charantia L.) in type 2 diabetic patients

Background/ObjectivesBitter melon (Momordica charantia L., MC) has been used as a traditional remedy in diabetics due to its hypoglycemic activity. However, its anti-hyperglycemic effect and antiglycation activity have been demonstrated in vitro and in animal experiments, but not in a long-term clinical study. The aim of this study was to investigate the effect of bitter melon on long-term glycemic control and glycation status in type 2 diabetic patients. MethodsThis study was a two-arm, parallel, randomized, double-blinded, placebo-controlled trial in which type 2 diabetic patients were randomized to continuously take either 6 g/day of MC dried-fruit pulp containing 6.26 ± 0.28 mg of charantin (N = 19) or placebo (N = 19) for 16 weeks. ResultsAfter 8 and 16 weeks of the treatment, the reduction of A1C from baseline in the MC group was greater than that of the placebo group (0.25 ± 0.12%, P = 0.042 and 0.31 ± 0.15%, P = 0.044, respectively). In addition, the MC group showed a significant decline of total advanced glycation endproducts (AGEs) in serum after 16 weeks of the intervention. The mean difference between both groups was 8.22 ± 3.58 × 103 AU/g protein (P = 0.028). The levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST) and serum creatinine (Cr) did not change from baseline in each group and were not different between the two groups. None of participants experienced serious adverse events. ConclusionsIt is possible that this herb is beneficial not only on glycemic control, but also on potential systemic complications of type 2 diabetes mellitus.

Clinical study on psoriasis patients for past 30 years (1982–2012) in Seoul National University Hospital Psoriasis Clinic

Psoriasis is a chronic relapsing disorder which shows variable clinical features. The long-term clinical study with many patients is important to elucidate the epidemiologic characteristics and clinical features of psoriasis. The purpose of this study was to analyze the epidemiologic characteristics and clinical features of psoriasis in Korean patients. Epidemiologic and clinic data and assessments for severity of extent and activity of psoriasis were collected from the medical records of 5084 patients, who had been newly diagnosed with psoriasis in the Psoriasis Clinic of Seoul National University Hospital between 1982 and 2012. The sex ratio of the psoriasis patients was 1.2:1 (male 54.6%, female 45.4%). The peak age of onset in males was 20s, while it was the teenage years in females. A total of 63.5% of patients developed psoriasis before 30years of age. Family history of psoriasis was observed in 26.0% of patients. Moderate to severe extent of involvement were more frequently observed in male patients and patients under 30years of onset age than in females and patients 30years or over of onset age, respectively. Moderate to severe disease activity were also more frequently presented in male patients, but not in patients under 30years of onset age. The most common morphological type was nummular (56.7%), followed by large plaque (28.5%) and guttate (8.5%). Nail involvement accompanied in 26.4% of patients. We demonstrated the epidemiologic and clinical characteristics of psoriasis in Korean patients.

Antiphospholipid antibodies in children with systemic lupus erythematosus: a long-term clinical and laboratory follow-up status study from northwest India

We have previously shown that children with pediatric systemic lupus erythematosus (pSLE) have high incidence of anti-phospholipid antibodies (APLA) and reports suggest that presence of APLA can modify disease expression. While the frequency of APLA has been studied previously in adults with SLE, there is paucity of literature in children especially with regard to long-term follow-up. In the earlier study, we analyzed 27 pSLE patients for the prevalence of APLA; in the present study, we further reviewed the APLA status and its relation with clinical outcome of this cohort of patients over a further 7year follow-up period. Out of the initial cohort of 27 patients, follow-up APLA testing was available in 21 patients who were tested for APLA at least once during this time. Seven (33.3%) of these 21 patients were never positive for any of the APLA; 1 (4.8%) was persistently positive; and 13 (61.9%) were positive for APLA intermittently or at least once. Overall, APLA positivity for IgG, IgM anticardiolipin antibodies (ACA) and lupus anticoagulant (LA) was comparable, with positivity seen in 10 (47.6%), 9 (42.9%) and 9 (42.9%) cases, respectively. Anti-β2 GP1 antibodies were tested in 11 patients on follow-up, of which 3 (27.3%) showed positivity. In all, 10 (47.6%) patients showed positivity for more than 1 APLA. Two (9.5%) patients showed varying degrees of positivity for LA, ACA, and anti-β2 GP1 antibodies at different times, thereby showing the importance of checking for all APLAs at each time of testing. Out of these 21 patients, 3 (14.3%) patients had thrombosis, and all 3 patients were positive for APLA. There were 2 (9.5%) fatalities-both of these had thrombosis and were positive for APLA. Our study shows that pSLE patients on treatment frequently test positive for APLA. Thrombosis was infrequent in this cohort. However, when present it was associated with APLA positivity and high fatality in our experience. On the other hand, presence or persistence of these antibodies was not always associated with thrombosis. Our study suggests that pSLE children should be tested routinely for APLA, as this would identify patients with an increased risk of thrombotic complications. However, the frequency of repeat testing for APLA in those who test negative initially needs to be determined on a case-to-case basis.

Long‐term follow‐up study on patients with Miyoshi phenotype of distal muscular dystrophy

To describe the long-term follow-up of a cohort of 22 patients with the Miyoshi phenotype of distal muscular dystrophy (MMD). A long-term clinical follow-up study was conducted. Patients were genotyped for dysferlin (MMD1) or anoctamin 5 (MMD3) mutations. Patients also underwent cardiological evaluation. There were 10 patients with MMD1, eight patients with MMD3 and four patients with linkage to chromosome 10 (MMD2). All patients deteriorated over 5.7 (range: 4.2-6.6) years of follow-up. Weakness increased significantly (P < 0.035) in all but the neck extensor, serratus anterior, and wrist flexor and extensor muscles. The decrease of strength was most pronounced in the iliopsoas (15%), toe extensors (15%), anterior tibial and peroneal muscles (10%). Patients with MMD1 showed early onset of the disease (mean 22 years) with typically symmetrical distribution of weakness starting in the calf muscles. Patients with MMD1 had a worse clinical course compared with patients with MMD3. Ninety percent of the former had to make use of a wheelchair within 15 years after onset of the disease, whereas patients with MMD3, who have a significantly later onset (mean 35 years) of asymmetrical calf muscle weakness and atrophy, remained ambulant during the first 15 years of their disease. None of the patients with MMD2 became fully confined to the wheelchair. None of the 22 MMD phenotype patients had heart disease. Patients with MMD1 have a worse clinical course compared with patients with MMD3. There are no cardiological abnormalities in all MMD categories.

Preservation of condyle and disc in the surgical treatment of type III temporomandibular joint ankylosis: a long-term follow-up clinical study of 111 joints

The purpose of this study was to establish the role of retaining the condyle and disc in the treatment of type III ankylosis, by clinical and computed tomography (CT) evaluation. A total of 90 patients with type III ankylosis met the inclusion criteria; 42 patients had left temporomandibular joint (TMJ) ankylosis, 27 patients had right TMJ ankylosis, and 21 had bilateral TMJ ankylosis, thus a total 111 joints were treated. Considerable improvements in mandibular movement and maximum mouth opening were noted in all patients. At the end of a minimum follow-up of 2 years, the mean inter-incisal mouth opening was 30.7mm. Postoperative occlusion was normal in all patients, and open bite did not occur in any case because the ramus height was maintained through preservation of the pseudo-joint. Only three patients had recurrence of ankylosis, which was due to a lack of postoperative physiotherapy. The advantages of condyle and disc preservation in type III ankylosis are: (1) surgery is relatively safe; (2) the disc helps to prevent recurrence of ankylosis; (3) the existing ramus height is maintained; (4) the growth site is preserved; and (5) there is no need to reconstruct the joint with autogenous or alloplastic material. It is recommended that the disc and condyle are preserved in type III TMJ ankylosis.