Orbital pseudotumor: clinical features and outcomes

To describe the clinical characteristics of orbital pseudotumor, a retrospective analysis was performed on patients with orbital pseudotumor at Siriraj Hospital for ten years. Forty-nine patients (24 males and 25 females; 62 eyes) with a mean age of 43.75 years were included (a mean follow-up of 25 months). Thirty-six patients (73.5%) had unilateral disease. The clinical features were proptosis (79.6%), ocular motor deficit (61.2%), pain (51%), lid swelling or lid mass (44.9%), ptosis (24.5%), and chemosis (18.4%). The most common presenting sign was proptosis (49%). All were treated with corticosteroids with clinical improvement in 40 (81.6%) patients. Ten (83.3%) of 12 patients with visual loss improved with mean recovery time of 10.3 days. Ocular motility recovered in 24 (80%) patients, occurring an average of 17.8 days after initiation of therapy. It is concluded that the clinical features of orbital pseudotumor are varied. Most patients were improved with corticosteroids treatment.     

RUBINSTEIN-TAYBI'S SYNDROME—A CASE REPORT

An autopsy case of Rubinstein-Taybi's syndrome is described. Since the mental retardation is considered to be a part of the syndrome, central nervous system was carefully examined, illustrating a moderate degree of the cerebral cortical and callosal hypoplasia and diffusely distrlbuted neuronal anoxic changes possibly associated with cardio-pulmonary insufficiency. As far as the pathognomony of the facio-osseal deformities, no convinciable causative factors have been obtained.     

Isolated optic neuritis from an identified Gnathostoma spinigerum

PURPOSE: To describe a patient with isolated monocular optic neuritis caused by an identified Gnathostoma spinigerum infestation. CASE REPORT: A 21-year-old man developed a swollen eyelid and painful monocular visual loss of his left eye which did not improve after treatment by intravenous steroid and albendazole. A remarkable eosinophilia in his peripheral blood count was demonstrated. The patient subsequently found a live parasite emerged from his lower eyelid and it was successfully removed by himself. Gross and histopathology examinations of the obtained parasite was undertaken. The parasite was identified as Gnathostoma spinigerum. His blood test for Gnathostoma antibody was positive. DISCUSSION: The etiology of isolated optic neuritis in this patient was Gnathostoma spinigerum which was confirmed by the histopathology of the obtained parasite and the positive serologic test. CONCLUSIONS: We could identify the exact parasite that was proven to cause an isolated optic neuritis. The immediate removal of a causative parasite maynot result in an improvement of the injured tissue but is beneficial in preventing further destruction as well as future complications.     

An unusual family with Leber''s hereditary optic neuropathy and facioscapulohumeral muscular dystrophy

We performed an observational prospective analysis to study the clinical characteristics as well as a molecular genetic analysis of 17 members of a Thai family who had visual loss and/or muscle weakness. Their blood mitochondrial DNA were examined for the presence of the G11778A Leber's hereditary optic neuropathy (LHON) mutation. Facioscapulohumeral muscular dystrophy (FSHD) DNA analysis was performed in four members who had visual loss. Of 17 family members, the eight members who had the 11778 LHON mutation were all from branch 'a'. Three of these eight members had FSHD with a 17-27-kb deletion of a tandem repeat in the 4q35 subtelomere, and two had been clinically diagnosed as FSHD. Four of six examined members in branch 'b' showed muscular dystrophy clinically diagnosed as FSHD. No correlation of blood DNA analysis between LHON and FSHD in affected members was found. We describe the first family with FSHD and G11778A LHON in which a mutation in mitochondrial DNA at nucleotide position 11778 of branch 'a' was found to be the origin of the mutation.     

Expression of CD24 in cholangiocarcinoma cells is associated with disease progression and reduced patient survival

Cholangiocarcinoma is frequently found to invade local tissues and metastasize to distal organs. We investigated the expression of CD24 in cholangiocarcinoma samples and its prognostic significance. In addition, the cellular function of CD24 was studied in the RMCCA1 cholangiocarcinoma cell line. High CD24 expression significantly correlated with lymph node metastasis and positive surgical margins in cholangiocarcinoma patients. Univariate and multivariate analyses further demonstrated that CD24 expression was significantly associated with the overall survival of these patients (p=0.007 and p=0.040, respectively). For in vitro studies, the magnetic-activated cell sorting (MACS) system was used to isolate CD24+ and CD24- cell populations from RMCCA1 cells. CD24+ RMCCA1 cells had increased chemoresistance, adhesion (p=0.004), motility (p<0.001), migration (p<0.001) and invasion (p<0.001) capabilities when compared to CD24- cells. The matrix metalloproteinase (MMP)-7 was significantly elevated in CD24+ RMCCA1 cells (p=0.01). We found that inhibition of CD24 using siRNA silencing significantly decreased the invasive capacity of RMCCA1 cells. Both clinical and in vitro studies suggest that expression of CD24 is associated with cholangiocarcinoma disease progression. CD24 may thus serve as a new target for directed molecular therapy of cholangiocarcinoma.     

Hematologic and clinical indices of malaria in a semi-immune population of western Thailand

This study examines hematologic profiles of persons with acute Plasmodium falciparum or P. vivax infection in Maesod on Thailand's western border with Myanmar compared with febrile, non-parasitemic persons also reporting to malaria clinics. Nine hundred seventy-nine subjects were malaria-negative, 414 were infected with P. falciparum, and 646 were infected with P. vivax. Persons with patent parasitemia tended to have significantly lower white blood cell, red blood cell, platelet, and hemoglobin levels than those who were malaria-negative. For the first time, a parallel trend in thrombocytopenia with parasitemia was found to be associated with both P. falciparum, and P. vivax infection. Using logistic regression, persons with platelet counts < 150,000/microL were 12-15 times more likely to have malaria than persons with platelet counts > or = 150,000/microL. This study supplements previous literature on the hematologic effects of malaria and helps define those alterations for a semi-immune population. Thrombocytopenia is identified as a key indicator of malaria in these febrile patients.