Patients with acute, fulminant form of SSPE

Subacute sclerosing panencephalitis (SSPE) usually begins insidiously and follows a subacute course with relentless but slow progression to death. In recent years, however, patients with acute or fulminant course were reported. In this article, we report on three patients (2 girls, 1 boy) with SSPE who developed an acute and fulminant course. Subacute sclerosing panencephalitis may be seen with more atypical symptoms and more acute and fulminant courses due to various undetermined reasons. Early diagnosis is very important for the effectiveness of treatment. Children presenting with acute or subacute neurologic symptoms should be examined for SSPE, especially if they have no risk factors for hereditary neurodegenerative/ neurometabolic diseases, and it is more important if those children were not vaccinated or were infected with measles.     

The mucocutaneous manifestations associated with celiac disease in childhood and adolescence

This study was performed to detect the prevalence of mucocutaneous manifestations of celiac disease in childhood and adolescence and to investigate the relationship between these findings and duration of disease and a gluten-free diet. The study included 55 children and adolescents with a mean age of 10.0 +/- 4.61 years (2-19 years). Mucocutaneous manifestations were evaluated with respect to age, gender, duration of illness, and of gluten-free diet and compliance with this diet. Cutaneous, mucosal, nail, and hair findings were detected in 74.5%, 27.3%, 20.0%, and 7.3% of patients, respectively. The most prevalent dermatologic diagnosis was xerosis (69.1%). No significant relationship was detected between the cutaneous findings and the duration of illness (p > 0.05). However, the duration was longer in patients with mucosal findings compared to those without mucosal findings (p < 0.05). It was found that all patients without cutaneous findings were on a strict gluten-free diet (p < 0.05). Ours is one of the few studies describing skin findings in children with celiac disease. We believe that both pediatricians and dermatologists should be aware of the various mucocutaneous manifestations of celiac disease, because a careful skin, mucosa, nail, and hair examination may lead to a diagnosis of celiac disease, particularly in atypical presentations.     

Circulating levels of vascular endothelial growth factor (VEGF), matrix metalloproteinase-3 (MMP-3), and BCL-2 in malignant melanoma

Vascular endothelial growth factor (VEGF) is a critical regulator of angiogenesis that stimulates proliferation, migration, and metastasis of melanoma. In literature, all studies concerning influences of matrix metalloproteinases (MMPs) and antiapoptotic proteins on VEGF-induced angiogenesis in melanoma patients have been performed in tissue scale in melanoma. The objective of this study was to determine the value of circulating serum VEGF and its possible mechanisms of angiogenesis by circulating VEGF, MMP-3, and Bcl-2 in patients with melanoma. Fifty-one patients with cutaneous melanoma pathologically verified at different stages, and eighteen healthy controls were investigated. Serum VEGF, MMP-3, and Bcl-2 levels were quantitatively analyzed by ELISA. The serum VEGF (P = 0.034) and Bcl-2 (P = 0.005) levels were significantly higher in patients with melanoma than in the control group. However, there was no significant difference in the serum MMP-3 level between melanoma patients and controls (P = 0.51). The serum levels of VEGF were significantly influenced only by Breslow thickness (P = 0.045) and mitosis (0.039) and were not positively correlated with the stage of the disease. Among serum parameters, a significant relationship was found only between serum levels of VEGF and MMP-3 (r = 0.32, P = 0.023). In conclusion, our study demonstrates increased concentrations of VEGF and Bcl-2, but not MMP-3, in serum of melanoma patients regardless of the stage of the disease. VEGF may be a potential endothelial cell growth and survival factor. The mechanism of VEGF regulation of angiogenesis may be in part due to enhanced proliferation and survival of endothelial cells by differential expression of antiapoptotic genes and in part by activation of MMPs.     

Serum levels of intercellular adhesion molecule ICAM-1 and E-selectin in advanced stage non-small cell lung cancer

Cell adhesion is a basic count in inter- and intra-cellular communication and plays an important role in tumor progression. This study was conducted to investigate the serum levels of intercellular adhesion molecule (ICAM-1) and E-selectin in patients with advanced stage non-small cell lung cancer (NSCLC) and the relationships with known prognostic parameters and therapy. These serum factors were measured of 57 NSCLC patients pathologically verified before and after chemotherapy in comparison with 24 healthy controls by using ELISA method. Serum levels of ICAM-1 were increased significantly in NSCLC patients compared with the healthy controls (P = 0.006). However, serum E-selectin levels were not significantly different from healthy control groups (0.643). No statistically significant relationships were found between investigated all serum parameters and various characteristics of patients, and the diseases such as stage and tumor burden. Likewise, we also found no correlation between serum ICAM-1 and E-selectin (P = 0.78). We found that serum ICAM-1 levels were decreased owing to the chemotherapy effect, independently from chemotherapy response. However, serum E-selectin levels were not changed by the chemotherapy effect. The median survival of all patients was 11.9 months and 1-year survival rate was 47.6%. We found that patients performance status (P = 0.013), age (P = 0.015), and weight loss (P = 0.007) were prognostic factors for survival. Serum E-selectin levels showed a trend (P = 0.08) related to worse prognosis, however serum ICAM-1 levels were determined as ineffective on survival (P = 0.11). Multivariate analysis revealed that only weight loss (P = 0.005) and E-selectin levels (P = 0.002) remained as an independent prognostic factor for survival in patients with advanced NSCLC. In conclusion, our data suggest that higher serum ICAM-1 can be useful for diagnosis while E-selectin levels have prognostic significance and could be a potential prognostic factor in NSCLC patients.     

Acrodermatitis enteropathica-like eruptions in a child with Hartnup disease

Acrodermatitis enteropathica-like eruptions, not related to zinc deficiency, have been rarely reported in some metabolic disorders. Reported patients usually had low levels of essential amino acids, particularly isoleucine. Here we report a girl who first presented with an acrodermatitis enteropathica-like eruption and eventually had the diagnosis of Hartnup disease with a normal isoleucine level. We discuss the probable cause of her skin lesions and the differential diagnosis with pellagra.     

In vitro–in vivo evaluation of tetrahydrozoline-loaded ocular in situ gels on rabbits for allergic conjunctivitis management

Ocular allergy is one of the most common disorders of the eye surface. The conventional eye drops lack of therapeutic efficacy due to low ocular bioavailability and decreased drug residence time on eye surface. Hence, the present research work aimed to formulate, optimize, and evaluate the in situ gel for ophthalmic drug delivery. The prepared in situ gel formulations were evaluated for clarity, pH, gelling capacity, viscosity, osmolality, in vitro release study, and kinetic evaluation. ex vivo corneal permeation/penetration study using goat and in vivo studies on rabbits were also performed. Fourier-transformed infrared spectroscopy was also applied to study possible interactions between drug and polymers. The formulations found to be stable, nonirritant, and showed sustained release of the drug for a period of up to 24 hr with no ocular damage. The developed in situ gels loaded with tetrahydrozoline are alternative and promising ocular candidates for the treatment of allergic conjunctivitis.     

Metastatizing low-grade clear cell leiomyosarcoma of the uterus

An unusual case of uterine clear cell tumor is reported In a 56-year-old woman. The neoplasm was Identified as a peculiar low-grade leiomyosarcoma, composed mainly of watery, clear large cells with round, fairly regular nuclei. No appreciable pleomorphlsm or high mitotlc activity was noted. There was no necrosis. The presence of occasional areas of transition between these cells and typical spindle leiomyosarcoma cells, together with the immunohisto-chemical results, allowed the recognition of a smooth muscle origin of this clear cell tumor. The patient was treated by hysterectomy. Six years later she developed an Inguinal metastasis resembling an hibernoma, and then later developed a large retroperitoneal metastasis, which was composed entirely of clear cells and was distinguished from a Ilposarcoma by Immunohlstochemlstry.     

Circulating serum levels of angiopoietin-1 and angiopoietin-2 in nasopharynx and larynx carcinoma patients

Tumor Biology - We aimed to determine the serum levels of angiogenic factors, namely angiopoietins, in nasopharyngeal and laryngeal carcinoma patients. We also aimed to seek the relation of these...     

Camptocormia in a patient with Parkinson disease and a myopathy with nemaline rods

Camptocormia, also referred to as bent spine, is a gait disorder characterized by hyperflexion of the thoracolumbar spine that develops in recumbent position while walking and that disappears in supine position. Myopathy is one of the frequent causes of camptocormia. A 77-yr-old male patient who was followed up with the diagnosis of rheumatoid arthritis for 2 yrs was admitted with progressive gait deterioration. Hyperflexion of trunk, disappearing in supine position, was detected and diagnosed as camptocormia. He also exhibited the signs of parkinsonism. A paraspinal muscle biopsy showed myopathy with rods in many muscle fibers. Camptocormia in this patient may be attributable to the myopathic weakness of thoracolumbar paraspinal muscles. The normal biceps brachii muscle biopsy refers to the isolated affection of paraspinal muscles in this patient. A camptocormia (bent spine) case of myopathy with nemaline rods associated with Parkinson disease is presented.