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.     

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.     

Laminin-dependent and laminin-independent adhesion of human melanoma cells to sulfatides

Sulfatides (galactosylceramide-I3-sulfate) but not neutral glycolipids or gangliosides adsorbed on plastic promote adhesion of the human melanoma cell line G361. Direct adhesion of G361 cells requires densities of sulfatide greater than 1 pmol/mm2. In the presence of laminin, however, specific adhesion of G361 cells to sulfatide or seminolipid (galactosylalkylacyl-glycerol-I3-sulfate) but not to other lipids is strongly stimulated and requires only 25 fmol/mm2 of adsorbed lipid. The effects of laminin and sulfatide on adhesion are synergistic, suggesting that laminin is mediating adhesion by cross-linking receptors on the melanoma cell surface to sulfatide adsorbed on the plastic. Although thrombospondin binds to sulfatides and G361 cells, it does not enhance, but rather inhibits direct and laminin-dependent G361 cell adhesion to sulfatide. In contrast, C32 melanoma cells also adhere specifically to sulfatide, but adhesion of these cells is not enhanced by laminin or inhibited by antibodies to laminin that block laminin-dependent adhesion of G361 cells. Thrombospondin is a potent inhibitor of C32 cell adhesion to sulfatide. Fucoidan, which inhibits laminin binding to sulfatide, inhibits laminin-dependent adhesion of G361 cells by 50% at 0.2 micrograms/ml. Several other tumor cell lines also attach directly on sulfatide-coated surfaces. Laminin stimulates adhesion to sulfatide of three of the six cell lines tested. The ability of laminin to promote adhesion of tumor cells to sulfatide suggests that binding to sulfatide could participate in laminin-mediated cell-cell adhesion. Thus, many tumor cell lines can attach on sulfatide substrates using endogenous sulfatide binding proteins, and in some cells laminin but not thrombospondin can promote tumor cell adhesion to sulfatide.     

Active transport of carnitine into skeletal muscle

Skeletal muscle carnitine concentration exceeds plasma carnitine concentration. To determine whether this concentration gradient is maintained by active transport we studied rat soleus and extensor digitorum longus muscles. Observations consistent with the existence of an active transport mechanism were that the soleus accumulated carnitine linearly for 3 hours of incubation to exceed a distribution ratio of 1; the temperature coefficient for carnitine accumulation between 33 degrees C and 43 degrees C was 2.0; anaerobic incubation reduced carnitine accumulation by 30 percent; and the rate of carnitine accumulation was saturated at high substrate concentrations and competitively inhibited by gamma-butyrobetaine. The Km for carnitine of the carnitine transport mechanism of the soleus muscle was 0.259 mM and of the extensor digitorum longus muscle, 0.585 mM. The greater affinity of the soleus transport mechanism may explain the difference in carnitine transport by red and white muscle in intact animals. A defect in active transport of carnitine may be involved in the pathogenesis of some human myopathies characterized by excessive lipid storage and in diphtheritic cardiomyopathy.