Changing scenario of therapeutic apheresis in India in the last 14 years.

There has been a remarkable change in the scenario of therapeutic apheresis in the last 14 years in India. The crude method of manual removal of blood followed by separation of plasma by gravity, keeping it in the bottle for a long time, has now been totally replaced by plasmapheresis, centrifugation, membrane filtration, and immunoadsorption techniques. The indications for use have also changed from a list of limited indications in the beginning to include all immune complex disorders. The clinical beneficiaries have also increased from blood bankers to nephrologists and immunologists in addition to oncologists. Efforts are now underway with the help of the Indian Society for Apheresis (founded in 1985) to popularize the newer methods of cryofiltration, photopheresis and heparin extracorporeal low-density lipoprotein (HELP) and DALI apheresis systems besides the specialized techniques of immunoadsorption using filters, columns, or ligands. This is suggestive of a positive trend for the treatment of immune complex disorders.     

Double skin island pectoralis major myocutaneous flap with nipple-areola complex preservation: a case report.

A horizontal double skin island pectoralis major myocutaneous flap with preservation of nipple-areola complex is described, a different modification used to reconstruct a complex defect following radical resection of a locally advanced gingival carcinoma. The skin islands are horizontally separated by a distance of 2.5 cm but rely on one vascular pedicle for their blood supply. The muscle between the skin islands provides the soft tissue bulk between the internal mucosal and external skin lining. The need for a separate segmental flap or a split-thickness skin graft for mucosal lining was eliminated.     

Chromosome 2 (2p16) abnormalities in Carney complex tumours

Carney complex (CNC) is an autosomal dominant multiple endocrine neoplasia and lentiginosis syndrome characterised by spotty skin pigmentation, cardiac, skin, and breast myxomas, and a variety of endocrine and other tumours. The disease is genetically heterogeneous; two loci have been mapped to chromosomes 17q22–24 (the CNC1 locus) and 2p16 (CNC2). Mutations in the PRKAR1A tumour suppressor gene were recently found in CNC1 mapping kindreds, while the CNC2 and perhaps other genes remain unidentified. Analysis of tumour chromosome rearrangements is a useful tool for uncovering genes with a role in tumorigenesis and/or tumour progression. CGH analysis showed a low level 2p amplification recurrently in four of eight CNC tumours; one tumour showed specific amplification of the 2p16-p23 region only. To define more precisely the 2p amplicon in these and other tumours, we completed the genomic mapping of the CNC2 region, and analysed 46 tumour samples from CNC patients with and without PRKAR1A mutations by fluorescence in situ hybridisation (FISH) using bacterial artificial chromosomes (BACs). Consistent cytogenetic changes of the region were detected in 40 (87%) of the samples analysed. Twenty-four samples (60%) showed amplification of the region represented as homogeneously stained regions (HSRs). The size of the amplicon varied from case to case, and frequently from cell to cell in the same tumour. Three tumours (8%) showed both amplification and deletion of the region in their cells. Thirteen tumours (32%) showed deletions only. These molecular cytogenetic changes included the region that is covered by BACs 400-P-14 and 514-O-11 and, in the genetic map, corresponds to an area flanked by polymorphic markers D2S2251 and D2S2292; other BACs on the centromeric and telomeric end of this region were included in varying degrees. We conclude that cytogenetic changes of the 2p16 chromosomal region that harbours the CNC2 locus are frequently observed in tumours from CNC patients, including those with germline, inactivating PRKAR1A mutations. These changes are mostly amplifications of the 2p16 region, that overlap with a previously identified amplicon in sporadic thyroid cancer, and an area often deleted in sporadic adrenal tumours. Both thyroid and adrenal tumours constitute part of CNC indicating that the responsible gene(s) in this area may indeed be involved in both inherited and sporadic endocrine tumour pathogenesis and/or progression.     

Chronic progressive multiple sclerosis: double-blind controlled study of plasmapheresis in patients taking immunosuppressive drugs

Fifty-four patients with chronic progressive multiple sclerosis received prednisone plus oral low-dose cyclophosphamide and either true plasmapheresis (PP) or "sham" PP weekly for 20 weeks in a double-blind controlled study. Immunosuppressive drug therapy alone (sham PP group, n = 29) was associated with improvement (greater than or equal to one step in Kurtzke Disability Status Scale [DSS]; mean change of 1.5) in 8 and stabilization of MS in 18 patients, with this status sustained in 23 patients at follow-up, 11 months after entry. In contrast, 14 of 26 patients who received "true" PP improved (greater than or equal to one step in DSS; mean change of 2.6), and 11 more were stable, with these changes sustained in 23 of 26 patients at follow-up. These differences, overall, between the PP and sham PP groups were significant at p less than 0.007.     

High Velocity Gunshot Fractures of Humerus: Results of Primary Plate Osteosynthesis

BackgroundHigh velocity gunshot fractures usually seen in conflict zones, often mandate external fixation due to delayed presentation and associated contamination. In the presented observational study, we managed high velocity gunshot fractures of humerus with adequate debridement and primary plate osteosynthesis under controlled conditions with gratifying results.Material and MethodsEighteen consecutive cases of fracture of the humerus secondary to high velocity gunshot wounds with ages ranging from 28 to 45 years reporting within 6 h of the initial injury formed our study group. Immediate debridement, lavage and primary plate osteosynthesis was carried out following hemodynamic stabilisation and intravenous antibiotics. All the cases were followed up at 2, 4, 6 months, 1 year and then annual follow-up including telephonic follow-up for six patients.ResultsFifteen cases of Gustillo Anderson type III A, two of type III B and one of type III C were managed with primary plate osteosynthesis. Brachial artery injury was addressed immediately, however injured radial nerve ends were tagged. Five cases showed delayed/non-union and were managed with decortication and autologous bone grafting. Two cases of deep infection could be managed with implant retention till union. The implants were removed following fracture consolidation. All the fractures united and no patient was left with sequelae of chronic infection.ConclusionTimely presentation of high velocity gunshot fractures of humerus teamed up with adequate debridement, soft tissue management and primary plate osteosynthesis can offer satisfactory outcomes. Associated vascular injury needs immediate attention. Nerve injuries can be addressed in a staged procedure. Our results have been satisfactory and add to the scant literature available on the subject, however further studies are warranted.     

Cowden's disease

A 24-year old male patient developed multiple lesions of keratoacanthoma in the epidermal verrucous naevus. He also had multiple papillomatous lesions on the lips, buccal mucosa, gingiva and tongue, with positive family history of similar lesions. He also had multiple skin tags and patchy palmoplantar keratoderma and minimal kyphoscoliosis.