Atlanto-axial rotatory fixation: Improved demonstration using spiral CT

Atlanto-axial rotatory fixation (AARF) is an uncommon condition which is often missed at presentation because of its rarity and the relative subtlety of plain film X-ray findings, but early detection and appropriate management are vital for a cure. We describe three cases in which the use of spiral computed tomography scanning with 3D and sagittal reconstructions greatly aided diagnosis and management. The 3D images gave a more graphic picture of the overall alignment of the upper cervical spine and the skull base, while the sagittal reconstructions demonstrated the presence or absence of compensatory atlanto-occipital subluxation. The literature is briefly reviewed.     

Epidemiology and treatment of tinea capitis: ketoconazole vs. griseofulvin

We studied 80 children with tinea capitis without kerion to define the epidemiology and clinical characteristics of tinea capitis and to compare the therapeutic efficacy of griseofulvin and ketoconazole for treatment of this disorder. Patients ranged in age from 2.1 to 11 years (median, 5.2 years). Trichophyton tonsurans (74%), Microsporum canis (13.5%) and Trichophyton mentagrophytes (2.7%) accounted for 90% of the infections. Pretreatment KOH slide preparations were positive in 69% of patients with alopecia and in only 29% of those with diffuse scale with little hair loss. Sixty-three patients were randomly assigned to receive ketoconazole (5 mg/kg/day) or griseofulvin (15 mg/kg/day). The treatment groups were comparable with regard to age, sex, duration of lesions prior to treatment and type of lesions. The percent of patients with positive cultures on therapy at 4, 6, 8, and 10 weeks and the mean time to a sterile culture were significantly larger (P less than 0.01) in ketoconazole (8 weeks) than in griseofulvin-treated (4 weeks) patients. The time for complete scalp clearing was significantly longer in patients who received ketoconazole (median, 108 days) compared with those who were treated with griseofulvin (median, 60 days) (P = 0.01).     

A pilot study of thalidomide for patients with symptomatic mesenteric panniculitis

BACKGROUND: Mesenteric panniculitis is a rare condition with no standard therapy. AIM: To assess the safety and efficacy of thalidomide for the treatment of patients with symptomatic mesenteric panniculitis using a newly established clinical disease activity index (Mesenteric Panniculitis Subjective Assessment Score). METHODS: In an open-label pilot study, five patients with symptomatic mesenteric panniculitis received oral thalidomide, 200 mg nightly, for 12 weeks. The primary end-point was a reduction in the Mesenteric Panniculitis Subjective Assessment Score by > or = 20% at 12 weeks or complete remission (absence of symptoms). RESULTS: Four (80%) of the five patients responded. The median Mesenteric Panniculitis Subjective Assessment Score at baseline was 39 and at week 12 was 25 (average decrease of 44%). One patient achieved complete remission by week 4, which was sustained. At 12 weeks, three (75%) patients experienced a global response, five (100%) patients had a > or = 20% (range, 29-98%) decrease in erythrocyte sedimentation rate and three (75%) patients had a > or = 20% (range, 61-93%) decrease in C-reactive protein. Abdomino-pelvic computed tomography scans were unchanged in all five patients. There were no serious adverse events. CONCLUSIONS: Thalidomide is safe, well tolerated and efficacious in the treatment of some patients with symptomatic mesenteric panniculitis. Further study is indicated.     

Sialyltransferase ST3Gal-IV operates as a dominant modifier of hemostasis by concealing asialoglycoprotein receptor ligands

A number of poorly characterized genetic modifiers contribute to the extensive variability of von Willebrand disease, the most prevalent bleeding disorder in humans. We find that a genetic lesion inactivating the murine ST3Gal-IV sialyltransferase causes a bleeding disorder associated with an autosomal dominant reduction in plasma von Willebrand factor (VWF) and an autosomal recessive thrombocytopenia. Although both ST3Gal-IV and ST6Gal-I sialyltransferases mask galactose linkages implicated as asialoglycoprotein receptor ligands, only ST3Gal-IV deficiency promotes asialoglycoprotein clearance mechanisms with a reduction in plasma levels of VWF and platelets. Exposed galactose on VWF was also found in a subpopulation of humans with abnormally low VWF levels. Oligosaccharide branch-specific sialylation by the ST3Gal-IV sialyltransferase is required to sustain the physiologic half-life of murine hemostatic components and may be an important modifier of plasma VWF level in humans.     

Extended Indications for Lung Volume Reduction Surgery in Advanced Emphysema

Background. Lung volume reduction surgery has shown early promise as a palliative therapy in severe emphysema. Selection of potential candidates has been based on certain functional and anatomic criteria, and a variety of operative contraindications have been proposed.

Methods. Over 15 months, we performed lung volume reduction surgery in 85 patients selected on the basis of severe hyperinflation with air trapping, diaphragmatic dysfunction, and disease heterogeneity. Patients were not excluded on the basis of severe hypercapnia, steroid dependence, profound pulmonary dysfunction, or inability to complete preoperative rehabilitation.

Results. We observed significant improvements in pulmonary function, exercise capacity, and dyspnea, with an acceptable 30-day perioperative mortality of 7% and actuarial survival of 90% and 83% at 6 and 12 months, respectively. In each “high-risk” group, perioperative mortality, actuarial survival to 1 year, and functional results were equivalent, and in some cases superior, to those in the corresponding “low-risk” patients.

Conclusions. Severe hypercapnia, steroid dependence, profound pulmonary dysfunction, and inability to complete preoperative rehabilitation do not preclude successful lung volume reduction surgery and should not be regarded as absolute exclusionary criteria.