Biologics in oral medicine: Sjogren syndrome

Oral Diseases (2012) doi:10.1111/j.1601‐0825.2012.01932.x Biologic therapy has a potential to benefit patients with orofacial manifestations of Sjogren syndrome (SS). The most appropriate use of biologics would appear to be in patients with severe or multisystem features of SS, but their use early in the pathogenesis has the potential to prevent disease progression. Tumour necrosis factor‐alpha blockade has not proven effective in SS. B‐cell depletion using rituximab has been of benefit, mainly in relation to extraglandular features, and to some extent in relation to hyposalivation where there is still residual salivary function. Rituximab is also effective in the treatment of SS‐associated (extrasalivary) lymphomas, although the therapeutic response in salivary lymphoma is poorer. Rituximab is given as a single or periodic intravenous infusion. Potential adverse effects exist, notably infusion reactions and infection, and so a full risk/benefit analysis is indicated for prospective patients. This and clinical use is best performed and monitored in conjunction with rheumatologists with appropriate training and experience in biologic therapies. Further studies of rituximab in SS are ongoing, and newer agents under trial include belimumab.     

Myeloid-associated differentiation antigens on stem cells and their progeny identified by monoclonal antibodies

Within the hematopoietic system, monoclonal antibodies reactive with antigenic determinants, expressed in a lineage- and stage-restricted fashion, can be used to map myeloid differentiation. We have generated a series of monoclonal antibodies that reacts with myeloid-associated determinants on committed myeloid stem cells and their progeny. Their reactivity with peripheral blood cells was identified by immunofluorescence assays, with bone marrow cells by fluorescence- activated cell sorting, and with committed hematopoietic progenitor cells by both cytotoxic assays and fluorescence-activated cell sorting. Antibody 1G10, which has previously been reported to react with cells of the granulocytic lineage and with a minor subset of mature monocytes, was shown to react with granulocyte-macrophage colony- forming units (CFU-GM). Three antibodies not previously characterized (T5A7, L4F3, L1B2) were shown to react with both granulocytic and monocytic cells and in fluorescence-activated cell sorting studies to detectably stain granulocytic cells at different stages of maturation. These three antibodies also react with CFU-GM, two (L4F3 and L1B2) reacting with all CFU-GM, while T5A7 reacts with only a portion of the day 7 CFU-GM. Antibody L4F3 also reacts with a portion of erythroid burst-forming units (BFU-E). In contrast, the previously reported antibody 5F1, which reacts with monocytic cells, nucleated erythroid cells, and platelets, was shown to react with erythroid colony-forming units (CFU-E). Potential applications of these antibodies to studies of normal and malignant hematopoiesis are discussed.     

Current Concepts in Laparoscopic Liver Surgery

Laparoscopic liver surgery is currently acknowledged to be a safe and effective approach for the treatment of a variety of benign and malignant hepatic lesions. Its wider adoption was delayed by lack of experience, unavailability of suitable laparoscopic equipment, fear of uncontrollable intraoperative adverse events and the perceived oncological inadequacy of the approach. Over the years all these obstacles were overcome, and increasing numbers of studies documented the safety and efficacy of the procedure, with beneficial short-term results and long-term outcome comparable with that of the traditional open approach. This review presents the current knowledge on the indications, advantages, and short- and long-term outcomes of laparoscopic liver resection for a variety of liver lesions.     

Glycogen Storage Disease Diagnosed in Adults

Glycogen storage diseases are usually identified in childhood.We present the clinical, biochemical and histological featuresof 10 patients first diagnosed in adult life. Five had glycogenstorage disease type 1a, one type 1e, two type IX, and in twopatients there were previously unreported abnormalities of hepaticglucose-6-phosphatase system activity. Of the latter, one patienthad an inhibitor of liver glucose-6-phosphatase (pseudo-1b glycogenstorage disease) the other having abnormal glucose-6-phosphataseactivity and microsomal pyrophosphate transport. A glucagontest is suggested as a useful screening procedure. Glycogenstorage disease should be considered in adults with symptomssuggesting hypoglycaemia.     

Factors affecting mortality and reoperations in high-energy pelvic fractures

Aim

Factors affecting mortality during the first year following high-energy pelvic fractures has not been reported previously. Nor has surgical complications leading to reoperations been reported in a cohort with only high-energy pelvic trauma patients.

Objectives

The aim of this study was to report and analyse factors affecting outcome, in terms of mortality and reoperations, up to 1 year after the injury in patients with a traumatic pelvic ring injury due to a high-energy trauma.

Materials and methods

Data from the SweTrau (Swedish National Trauma Registry) on patients admitted to the Trauma Centre Karolinska in Stockholm, Sweden, were collected. Inclusion criteria were adults (age?≥?18), trauma with a high-energy mechanism, alive on arrival, Swedish personal identification number, reported pelvic fracture on CT scan. Patient records and radiographies were reviewed. The study period was 2011–2015 with 1-year follow-up time. Univariate and regression analysis on factors affecting mortality was performed. Risk of reoperation was analysed using univariate and case-by-case analysis.

Results

We included 385 cases with mean age 47.5?±?20.6 years (38% females): 317 pelvic fractures, 48 acetabular fractures and 20 combined injuries. Thirty-day mortality was 8% (30/385), and 1-year mortality was 9% (36/385). The main cause of death at 1 year was traumatic brain injury (14/36) followed by high age (>?70) with extensive comorbidities (8/36). Intentional fall from high altitude (OR 6, CI 2–17), GCS?<?8 (OR 12, CI 5–33) and age?>?70 (OR 17, CI 6–51) were factors predicting mortality. Thirty patients (22%, 30/134) were further reoperated due to hardware-related (n?=?18) or non-hardware-related complications (n?=?12). Hardware-related complications included: mal-placed screws (n?=?7), mal-placed plate (n?=?1), implant failure (n?=?6), or mechanical irritation from the implant (n?=?4). Non-hardware-related reasons for reoperations were: infection (n?=?10), skin necrosis (n?=?1), or THR due to post-traumatic osteoarthritis (n?=?1).

Conclusion

Non-survivors in our study died mainly because of traumatic brain injury or high age with extensive comorbidities. Most of the mortalities occurred early. Intentional injuries and especially intentional falls from high altitude had high mortality rate. Reoperation frequency was high, and several of the hardware-related complications could potentially have been avoided.

     

Distraction Osteogenesis for Management of Severe OSA in Pierre Robin Sequence: An Approach to Elude Tracheostomy in Infants

Background

Severe obstructive sleep apnoea (OSA) is a life threatening condition associated with Pierre Robin sequence (PRS) due to mandibular micrognathia and glossoptosis. Often these patients require tracheostomy at an early age which has high morbidity. Distraction osteogenesis (DO) is an accepted method of treatment for patients with hypoplastic mandible to achieve mandibular lengthening without need for a bone graft. It has also been used in respiratory distressed neonates and infants to avoid tracheostomy.

Case report

An eight month old baby, a diagnosed case of PRS with severe OSA and recurrent episodes of aspiration pneumonia and on nasogastric tube feeding since birth was referred to us for evaluation and possibility of therapeutic augmentation of the mandible by DO. After a thorough clinico-radiological assessment the child was operated for bilateral extraoral placement of horizontal corpus distractor. A total distraction of 12 mm was carried out and consolidation of callus was monitored by USG. Postoperatively the patient was followed up for 12 months. Presently she has normal respiratory and feeding function without any episode of aspiration pneumonia.

Conclusion

Mandibular corpus DO is a safe and effective technique that can be applied to predictably relieve severe upper airway obstruction in selected PRS cases. In order to avoid the limitations of alternative surgical procedures and the tracheostomy-associated morbidity, DO should be considered among the routine treatment modalities.