Myostatin blockade improves function but not histopathology in a murine model of limb-girdle muscular dystrophy 2C

Myostatin is a negative regulator of skeletal muscle growth. Myostatin mutations and pharmacological strategies increase muscle mass in vivo, suggesting that myostatin blockade may prove useful in diseases characterized by muscle wasting, such as the muscular dystrophies. We subjected the gamma-sarcoglycan-deficient (Sgcg(-/-)) mouse model of limb-girdle muscular dystrophy (LGMD) 2C to antibody-mediated myostatin blockade in vivo. Myostatin inhibition led to increased fiber size, muscle mass, and absolute force. However, no clear improvement in muscle histopathology was evident, demonstrating discordance between physiological and histological improvement. These results and previous studies on the dyw/dyw mouse model of congenital muscular dystrophy and in the late-stage delta-sarcoglycan-deficient (Sgcd(-/-)) mouse model of LGMD2F document disease-specific limitations to therapeutic strategies based on myostatin blockade in the more severe mouse models of different muscular dystrophies.     

CT patterns of nodal disease in pediatric chest tuberculosis

AIM:To highlight various patterns of nodal involvement and post treatment changes in pediatric chest tuberculosis based on contrast enhanced computed tomography(CECT) scans of chest.METHODS:This was a retrospective study consisting of 91 patients aged less than 17 years,who attended Paediatrics OPD of All India Institute of Medical Sciences with clinically diagnosed tuberculosis or with chest radiographs suggestive of chest tuberculosis.These patients had an initial chest radiograph as well as CECT of the chest and follow up imaging after 6 mo,and in some cases 9 mo,of completion of anti-tubercular treatment(ATT).CECT of these patients was reviewed for the location and extent of nodal involvement along with determination of site,size,enhancement pattern and calcification.RESULTS:Enlargement of mediastinal or hilar lymph nodes was found in 88/91 patients(96.7%),with the most common locations being paratracheal(84.1%),and subcarinal(76.1%).The most common pattern of enhancement was found to be inhomogenous.The nodes were conglomerate in 56.8% and discrete in 43.2%.In addition,perinodal fat was obscured in 84.1% of patients.In the post-treatment scan,there was 87.4% reduction in the size of the nodes.All nodes post-treatment were discrete and homogenous with perinodal fat present.Calcification was found both pre-and post-treatment,but there was an increase in incidence after treatment(41.7%).There was hence a reduction in size,change in enhancement pattern,and appearance of perinodal fat with treatment.CONCLUSION:Tubercular nodes have varied appearance and enhancement pattern.Conglomeration and obscuration of perinodal fat suggest activity.In residual nodes decision to continue ATT requires clinical correlation.     

Osteomyelitis of the lower extremity: pathophysiology,imaging, and classification,with an emphasis on diabetic foot infection

Osteomyelitis is inflammation of the bone caused by an infectious organism, and is a difficult clinical problem. The pathophysiology, imaging, and classification of osteomyelitis are challenging, varying with the age of the patient (child versus adult), the chronicity of the infection (acute versus chronic), and the route of spread (hematogenous versus contiguous focus), as well as the immune and vascular status of the patient and affected region. The two most common classification schemes are those of Lew and Waldvogel, and Cierny and Mader. Brodie’s abscess is seen in subacute osteomyelitis, while sequestrum, involucrum, and cloaca are inter-related entities of chronic osteomyelitis. Imaging workup of suspected osteomyelitis should begin with radiographs, although MRI is the most accurate imaging test. Three patterns of T1 signal change have been described in the setting of suspected osteomyelitis including confluent intramedullary, hazy reticular, and subcortical. The confluent intramedullary pattern is most associated with osteomyelitis, while hazy reticular is rarely associated with hematogenous osteomyelitis, and subcortical is not associated with osteomyelitis. It can be challenging to differentiate neuropathic arthropathy from osteomyelitis. Osteomyelitis tends to involve a single bone subjacent to an ulcer or sinus tract. In contrast, neuropathic arthropathy tends to involve multiple bones of the midfoot. Subchondral cystic change, thin rim enhancement of a joint effusion, and the presence of intra-articular bodies are more indicative of a neuropathic joint without infection. Biopsy can play an important role in diagnosis and treatment of osteomyelitis.     

Postoperative rhabdomyolysis following laparoscopic gastric bypass in the morbidly obese

HYPOTHESIS: Laparoscopic approaches for weight reduction in the morbidly obese have become common with more than 50,000 bariatric surgical procedures being performed in 2001. The objective of this article is to raise awareness among surgeons of a new complication of rhabdomyolysis from this frequent procedure. DESIGN: Case series extracted from surgical database from January 2, 2001, through December 31, 2002. PATIENTS AND METHODS: We identified 5 cases of postoperative rhabdomyolysis in morbidly obese patients who underwent laparoscopic duodenal switch procedures with parietal gastrectomy. The cause, pathogenesis, and clinical features are reviewed and discussed. RESULTS: Postoperative rhabdomyolysis developed in 5 of 353 morbidly obese patients who underwent consecutive laparoscopic duodenal switch procedures, an incidence of 1.4%. All 5 patients were male, had a mean peak serum creatine kinase level of 19 680 U/L, and reported muscle pain in either the buttock, hip, or shoulder regions during the early postoperative period. CONCLUSIONS: We hypothesized that morbidly obese patients develop critical surface and deep tissue pressures during bariatric surgery, increasing their risk for tissue injury and rhabdomyolysis. Unexplained elevations in the serum creatinine level or reports of buttock, hip, or shoulder pain in the postoperative period should raise the possibility of rhabdomyolysis and prompt clinical investigation. We recommend routine preoperative and postoperative measurements of the serum creatine kinase and serum creatinine levels to aid detection. Surgeons need to keep a low index of suspicion because early diagnosis and treatment are the cornerstones of successful management of rhabdomyolysis.     

Weil osteotomy: Assessment of medium term results and predictive factors in recurrent metatarsalgia

Background

The angle of the Weil osteotomy is usually referenced relative to the floor irrespective of the plantar angulation of the metatarsal. This study aims to analyse the long term results following the Weil osteotomy and identify the cause of poor outcome.

Methods

This study presents a retrospective review of 61 patients (86 feet), with mean follow-up of 31 months. Each patient underwent clinical, pedobarographic and radiological examination. The radiographs obtained included ‘Metatarsal Skyline Views’ (MSV), to assess the plantar declination of the metatarsal heads following the osteotomy. The functional scoring was performed using AOFAS and Foot Function Index.

Results

Fifty-five patients (80 feet) showed good to excellent results clinically. Six patients had persistent metatarsalgia. All these 6 patients had callosities beneath metatarsal heads. Pedobarography showed peak pressures in the same distribution as callosities and the MSV showed increased plantar declination of the metatarsal heads. This correlation was found to be significant (p < 0.05).

Conclusion

The Weil osteotomy is a safe and effective treatment for metatarsalgia. An MSV radiograph is helpful to identify the plantar prominence of metatarsal which can be associated with poor clinical outcomes.     

Efficacy and safety of zonisamide monotherapy in a cohort of children with epilepsy

The aim of this study was to evaluate the efficacy and safety of zonisamide monotherapy in a cohort of children and adolescents with various types of epilepsy. Retrospective review of charts of our institution from 2001 through 2004 identified 69 children (19 males and 50 females, mean age 13.2 years) with epilepsy on zonisamide monotherapy. Seizure count and side effect profile were maintained during therapy. Sixty-one percent had idiopathic generalized epilepsy, 4% symptomatic generalized epilepsy, and 35% partial-onset epilepsy. Zonisamide was the first-line and second-line monotherapy for 32% and 68% of patients, respectively. The mean duration of follow-up on treatment was 22 months (range 3-48 months). The overall efficacy of zonisamide was 75.4% (> or = 50% seizure frequency reduction: good responders). Sixty-seven percent of good responders became seizure-free. Seventy-nine percent of patients with partial epilepsy and 71% with generalized epilepsy were good responders, of whom 79% and 63% were free of seizure, respectively. Eighteen (26%) patients developed side effects: weight loss (9), cognitive impairment (3), sleepiness (3), dizziness (2), and decreased appetite (1). In seven patients (10%), zonisamide had to be discontinued: four due to side effects and three because of poor seizure control. Zonisamide was demonstrated to be effective as monotherapy in children with epilepsy.     

Radiographic Outcomes in Treatment of Thumb Basal Joint Arthritis: Does Interposition of Acellular Dermal Allograft Prevent Metacarpal Settling?

Background  Osteoarthritis at the thumb carpometacarpal joint can have a profound impact on quality of life. Here, we evaluate radiographic outcomes in patients who have had open complete trapeziectomy, ligament reconstruction with tendon interposition, and acellular dermal matrix (GraftJacket) interposition—Group A, and compare them with those without GraftJacket interposition—Group B. Materials and Methods  Thirty patients who had undergone operative treatment for thumb basal joint arthritis by a single surgeon from 2009 to 2016 were identified, and charts were retrospectively reviewed for demographic data, surgical and radiographic outcomes, and complications. Results  There was no significant difference in pre- and postoperative radial abduction or pre- and postoperative palmar abduction. The difference in intraoperative joint space was significant ( p = 0.006), but the difference in postoperative joint space was not ( p = 0.310). The average amount of metacarpal settling was 6.9 versus 3.7 mm ( p = 0.035) (Groups A and B, respectively). Three patients in Group A developed an inflammatory reaction to the GraftJacket, and one required reoperation for allograft removal. Conclusion  This study suggests that thumb basal joint arthroplasty with GraftJacket interposition does not lead to more favorable radiographic outcomes at long-term follow-up. The increased costs associated with GraftJacket use may not be justified in light of these outcomes.