Rituximab as a first-line agent for the treatment of dermatomyositis

B cells may play a pivotal role in the pathophysiology of DM, and reports have claimed that targeting B cells is a viable treatment option in patients with dermatomyositis. A 20-year-old girl presented in October 2007, with few weeks’ history of proximal muscle weakness. Gottron’s papules were noted on her knuckles. She had normal inflammatory markers and negative autoantibody screen. Her CPK was 7,000 U/L (normal range 0–170) with an LDH of 1,300 U/L (normal range 266–500). EMG and muscle biopsy was consistent with active myositis. She had normal pulmonary function tests. HRCT showed no interstitial lung disease. She was started with 60 mg glucocorticoids (1 mg/kg), with a good clinical response. However, any attempt to taper down the steroid dose led to recurrence of her symptoms. The options of available immunosuppressive therapies, including the experimental usage of rituximab, were discussed with her; averse to long-term systemic treatments, she opted to try a course of rituximab. She had rituximab 1,000 mg on days 0 and 14, and her glucocorticoids were tapered in next few weeks. Now, 24 months since her rituximab infusions, she remains in complete clinical and biochemical remission and is na?ve to other immunosuppressive agents apart from glucocorticoids and rituximab. Depleting peripheral B cells with rituximab (one course) in our patient has led not only to complete resolution of muscle and skin disease (induction) but also remains off all immunosuppressives including glucocorticoids.     

Increased use of multidisciplinary treatment modalities adds little to the outcome of rectal cancer treated by optimal total mesorectal excision

Purpose

Total mesorectal excision (TME) is the standard surgical treatment for rectal cancer. The roles of chemotherapy and radiotherapy have become more defined, accompanied by improvements in preoperative staging and histopathological assessment. We analyse our ongoing results in the light of changing patterns of treatment over consecutive time periods.

Methods

In total, 151 consecutive patients underwent potentially curative rectal excision for cancer in a single institution. Management and outcomes were compared between 1993–1999 and 2000–2007 which corresponded with the restructuring of the regional oncological services.

Results

We found an increase in patients treated with neoadjuvant chemoradiotherapy after 1999 (20/89 vs 1/62, p?p?=?0.037). The locoregional recurrence rate was 5.3%. The rates were not significantly different between the two study periods [4/89 (4.5%) 1999–2007 vs 4/62 (6.5%) 1993–1999, p?=?0.597]. There was no statistical difference in overall or disease-free survival in the time periods examined.

Conclusions

Increasing use of neoadjuvant therapy and concomitant improvement in lymph node assessment did not translate into a concurrent reduction in the local recurrence, disease-free and overall survival rates. Our results demonstrate the enduring benefit of specialist training in TME in the outcome of rectal cancer surgery. This observational study suggests that low local recurrence rates are surrogate markers for improved overall and disease-free survival. Multidisciplinary team practice should be examined and made cost effective according to the individual unit’s local recurrence rate in the light of this and other reports.     

The “Forensication” of Public Sector Mental Health

Abstract

The Kansas vs. Hendricks U. S. Supreme Court decision of 1996 has been widely criticized by a variety of mental health professionals. Concerns have been expressed that its provisions for the indefinite involuntary commitment of persons who have committed sex offenses will weaken the protection of patients' rights and lead to a huge increase in forensic inpatient treatment for individuals who historically not responded well to treatment. This paper will review how states have begun to respond to the Hendricks decision. It will also examine how the Hendricks decision is part of a larger trend towards the “forensication” of public sector mental health services. Forensication refers to the ongoing process though which ever-increasing public sector resources are applied to forensic populations. Increasing tensions between the need to protect public safety and the need to attend to patient rights within a consumer-based approach will also be examined. Finally, recommendations for coping with changing expectations for mental health professionals will also be presented.     

A standardised investigative strategy prior to revisional oesophageal surgery in children: High incidence of unexpected findings

Background/Purpose

Revisional oesophageal reconstructive surgery carries uncommon and unusual risks related to previous surgery. To provide maximum anatomical detail and facilitate successful outcome, we report a standardised pre-operative investigative strategy for all such patients.

Methods

Prospective 8-month cohort study following the introduction of this strategy. All patients underwent high resolution thoracic contrast CT scan and micro-laryngo-bronchoscopy by a paediatric ENT surgeon in addition to upper gastrointestinal contrast study, oesophagoscopy, and echocardiogram.

Results

Seven children (median age 5.6 months [range 2.2–60]) completed the pathway. Four were referred with recurrence of a previously divided tracheo-oesophageal fistula (3 congenital, 1 acquired) and 3 (all with oesophagostomy) for oesophageal replacement for congenital isolated oesophageal atresia (OA, n = 1) and failed repair of OA with distal TOF with wide gap (n = 2). Overall, unanticipated findings were demonstrated in 6/7 children and comprised severe tracheomalacia and right main bronchus stenosis requiring aortopexy (n = 1), vocal cord palsy (n = 2), extensive mediastinal rotation (n = 1), proximal tracheal diverticulum (n = 1), severe subglottic stenosis requiring airway reconstruction (n = 1), proximal tracheal diverticulum (n = 1), right sided aortic arch (n = 1) and left sided aortic arch (previously reported to be right sided, n = 1).

Conclusions

This standardised approach for this complex group of patients reveals a high incidence of unexpected anatomical and functional anomalies with significant surgical and possible medico-legal implications. We recommend these investigations during the pre-operative work-up prior to all revisional oesophageal surgery.