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Biomedical subjects

Steven R Cohen

Publications and source records attributed to Steven R Cohen.

27 records · Page 2Linked to original sources

Pemphigus erythematosus associated with anti-DNA antibodies and multiple anti-ENA antibodies: a case report.

Pemphigus erythematosus (PE) is an autoimmune blistering disease combining features of pemphigus foliaceus (PF) and systemic lupus erythematosus (SLE). We report a case of PE associated with anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), anti-Ro (anti-SSA), and antiribonucleoprotein (anti-RNP) antibodies. This case required extensive immunosuppressive therapy. We treated our patient with a combination of systemic steroids, intramuscular gold injections, azathioprine, and hydroxychloroquine. The patient's response was complete remission--evaluated clinically, serologically, and immunohistochemically.

Adult↗

Reticulate and stellate acral pigmentation associated with systemic lupus erythematosus and high titers of circulating anticardiolipin antibodies: a possible association with acral microlivedo.

A Hispanic man with a twenty-eight year history of systemic lupus erythematosus (SLE) and a high titer of anticardiolipin IgG antibodies was noted to have reticulate and stellate acral pigmentation. The patient reported that hand swelling and erythema developed soon after the diagnosis of SLE was established. This episode resolved quickly without recurrence or immediate sequelae. We postulate that this eruption was related to SLE and anticardiolipin antibodies. Reticulate and stellate acral pigmentation should be considered a possible manifestation of SLE and high titers of anticardiolipin antibodies, or a consequence of therapy.

Antibodies, Anticardiolipin↗

Surgical strategies in the treatment of complex obstructive sleep apnoea in children.

Complex OSA in children is a challenging problem that requires careful team management. Evaluation includes history, physical examination and appropriate radiological and endoscopic studies. Twelve-channel polysomnography, with or without a pH probe, is mandatory in children with OSA to determine the severity and to document the efficacy of treatment. A variety of individual problems and nuances arise and will continue to do so. Clinical experience and a high degree of suspicion in the craniofacial population is necessary to rule out the existence of OSA in a given patient. Newer treatment modalities, such as distraction osteogenesis of the mandible and midface, have revolutionised treatment of OSA during childhood. Although still the gold standard of care, tracheostomy is not a benign procedure and the toll on both the patient and family can be devastating.

Cerebral Palsy↗

Midface distraction following Le Fort III and monobloc osteotomies: problems and solutions.

Distraction osteogenesis has been used increasingly for midfacial advancement in patients with syndromic craniosynostosis and in severe developmental hypoplasia of the midface. In these patients, the degree of advancement required is often so great that restriction of the adjacent soft tissues may preclude stable advancement in one stage. Whereas distraction is an ideal solution by which to gradually lengthen both the bones and the soft tissues, potential problems remain in translating the distraction forces to the midface. In these patients, severe developmental hypoplasia may be associated with weak union between the zygoma and the maxilla, increasing the chance of zygomaticomaxillary dysjunction when using internal devices that translate distraction force to the maxilla through the zygoma. Eight cases are reported in which either internal or external distraction systems were used for midface advancement following Le Fort III (n = 7) or monobloc (n = 1) osteotomies. Cases of patients in whom hypoplasia at the zygomaticomaxillary junction altered or impaired plans for midface distraction were reported from three host institutions. Seven patients had midface hypoplasia associated with syndromic craniosynostosis, and one patient had severe developmental midface hypoplasia. The distraction protocol was modified to successfully complete midface advancement in light of weakness at the zygomaticomaxillary junction in seven patients. Modifications included change from an internal to an external distraction system in two patients, rigid fixation and bone graft stabilization of the midface in one patient, and plate stabilization of a fractured or unstable zygomaticomaxillary junction followed by resumption of internal distraction in four patients. Previous infection and bone loss involving both malar complexes precluded one patient from being a candidate for an internal distraction system. Using a problem-based approach, successful advancement of the midface ranging from 9 to 26 mm at the occlusal level as measured by preoperative and postoperative cephalograms was undergone by all patients. Advantages and disadvantages of the respective distraction systems are reviewed to better understand unique patient characteristics leading to the successful use of these devices for correction of severe midface hypoplasia.

Child↗

Reconstruction of complex orbitocranial deformities using bioresorbable mesh, sterilized orbital models, and in situ contouring.

PURPOSE: To assess the efficacy of bioresorbable mesh in reconstruction of orbitocranial deformities. METHODS: A retrospective case series evaluating 6 orbits of 6 patients, ages 12 to 70 years old, with large multicontoured orbitocranial defects that were repaired using bioresorbable macroporous mesh. Both functional (presence of diplopia, ocular motility, exophthalmometry, and vertical globe position) and cosmetic outcomes were evaluated. RESULTS: Follow-up time ranged from 20 to 48 months (mean, 29.1 months). After surgery, all patients reported cosmetically pleasing results. Diplopia resolved in all patients. Ocular motility, exophthalmos, enophthalmos, and vertical globe positioning improved in all patients. One patient had new onset of mild enophthalmos and hypoglobus after surgery. No cases of implant exposure or instability occurred. Complications included one patient with skin breakdown over a previous site of irradiation and one patient with ptosis requiring surgical correction. CONCLUSIONS: Bioresorbable macroporous mesh combined with bone grafts is a useful and accurate method to reconstruct complex multicontoured orbitocranial defects.

Absorbable Implants↗