Interferon-alpha 2a treatment of neuro-Behcet disease.

Behcet disease is a multisystemic, chronic, recurrent inflammatory disorder characterized as a triad of hypopyon uveitis, oral aphthae, and genital ulcers. Neurologic involvement in Behcet disease (neuro-Behcet) is common. Neuro-Behcet disease typically manifests late after disease onset, rarely coincides with ocular involvement, and often heralds a poor prognosis for final vision and survival. We present a case of neuro-Behcet disease with neurologic onset concomitant with systemic and ocular involvement that was responsive to treatment with interferon-alpha 2a.

Cryoglobulinemia associated with Purtscher-like retinopathy.

PURPOSE: To report a case of Purtscher-like retinopathy in a young man with chronic hepatitis C--associated cryoglobulinemia. METHODS: Case report. RESULTS: A 44-year-old Caucasian man with chronic hepatitis C developed sudden loss of vision in the left eye and abdominal pain. Ocular fundus examination revealed peripapillary cotton-wool spots and superficial retinal whitening in the macula. Systemic evaluation revealed markedly elevated rheumatoid factor, hypertension, and acute renal insufficiency. A renal biopsy demonstrated intravascular deposition of immunoglobulins IgG and IgM and complement consistent with type II mixed cryoglobulinemia. CONCLUSION: Clinicians should consider cryoglobulinemia in the differential diagnosis of a patient with Purtscher-like retinopathy and history of infection with hepatitis C.

Ultrasonographic evidence of a mushroom-shaped choroidal metastasis.

PURPOSE: To describe the evolution of a "collar-button" or mushroom-shaped choroidal metastasis. METHODS: Case report. RESULTS: A 52-year-old woman with known primary colon cancer and widespread extraocular metastases developed bilateral choroidal masses with neurosensory retinal detachments. Clinical and ultrasound evaluation demonstrated evolution of a choroidal metastasis with a "collar-button" configuration in the right eye. CONCLUSION: A choroidal metastasis may develop a mushroom-shaped configuration. This "collar-button" configuration is not pathognomonic for choroidal melanoma.

Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive amblyopia therapy with low-dose external beam irradiation.

Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma.(1-3) Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy.

Choroidal neovascularization with granulomatous inflammation in ocular histoplasmosis syndrome.

PURPOSE: To report pathologic examination of an excised choroidal neovascular membrane in a patient with ocular histoplasmosis syndrome that demonstrated granulomatous inflammation. METHOD: Case report. A 50-year-old woman with sudden vision loss in her left eye demonstrated clinical and fluorescein angiographic findings characteristic of choroidal neovascularization secondary to ocular histoplasmosis syndrome. RESULTS: Histopathologic examination of the surgically excised choroidal neovascular membrane disclosed granulomatous inflammation. CONCLUSIONS: This case suggests an important role of mononuclear phagocytic cells as primary mediators of angiogenesis or modifiers of choroidal neovascularization. This association of choroidal neovascularization with granulomatous inflammation did not respond to treatment with systemic corticosteroids.

Pilomatrical carcinosarcoma of the cheek with subsequent pulmonary metastases. A case report.

We report a case of malignant pilomatrixoma with a pronounced biphenotypic morphology. The lesion, which was excised from the cheek of a 36-year-old man, was composed of a large pilomatrixoma lying within a spindled, sarcomatoid stroma. Fourteen months later, the tumor metastasized to the right upper lobe of the lung. We describe the tumor's pathology, histology, and immunochemistry and discuss the differential diagnosis. We also speculate on its histogenesis.

Reconceptualizing migration as a household phenomenon: outmigration from New York City by race and Hispanic origin.

"This paper proposes a conceptual framework for analyzing migration as a household event and presents two public use microdata applications of this approach for out-migrants from New York City. The distribution of single- and multi-origin households by race and Hispanic origin permits a more rigorous analysis of household migration differentials....and the disaggregation of household members by migration status provides insight into the household outcomes of the migration process...." (SUMMARY IN FRE AND SPA)

Length distributions of hemoglobin S fibers.

Electron microscopy of sickle cell hemoglobin fibers fixed at different times during gelation shows an exponential distribution of fiber lengths, with many short fibers and few long ones. The distribution does not change significantly with time as polymerization progresses. If this distribution of lengths reflects kinetic mechanism of fiber assembly, it complements information from studies of the progress of average properties of the polymers and, as has been done for other rod-like polymerizing systems, permits testing of models for the mechanism of fiber assembly. In this case, the results are consistent with the double nucleation model of Ferrone et al. or with a related alternative model based on fiber breakage. However, other possible causes of this microheterogeneity exist, including: breakage due to solution shearing of the long, rod-like, fibers; the presence of residual nuclei; equilibrium relations governing polymerization; and breakage of solid-like but weak gels that develop early and adhere to the grid. The arguments against the first three of these possibilities suggest that they are not responsible. However, breakage of entanglements or cross-links in a solid-like and adherent gel is consistent with the distributions.

Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa.

Successful anaesthetic management of two patients with severe epidermolysis bullosa was accomplished using subarachnoid and epidural blockade. In order to avoid complications of regional anaesthesia, antiseptic preparation of the skin should not involve scrubbing nor should adhesive tape be used to secure catheters. In addition local infiltration of the skin is best avoided. The potential complications of skin and oral mucosal damage associated with mask or endotracheal inhalation anaesthesia was avoided.