A rare cause of neonatal seizure: incontinentia pigmenti.

Incontinentia pigmenti (IP) is a rare genetic multisystem disorder that may affect many organs including the skin, bone, eyes and the central nervous system. Central nervous system manifestations are seen in 30% of cases with seizures and mental retardation. Seizures occurring as the presenting sign of IP are rarely reported. We report a case of a female newborn with IP who had seizures on day 4 of life, which were followed in her second month by the development of the characteristic cutaneous changes for IP. With this case report, we would like to emphasize the need for inclusion of IP in the differential diagnosis of neonatal seizures.

Early and late effects of capsaicin pretreatment in otitis media with effusion.

PURPOSE: Discovery of the role of the neurogenic inflammation in the formation of otitis media with effusion has led to the investigation of the place of some neuropeptide antagonists in the treatment. In the current study, we investigated the effect of capsaicin (CP) pretreatment on the inflammation and proliferation in the middle ear mucosa and on the nerve fibers containing substance P, vasoactive intestinal polypeptide, and calcitonin gene-related peptide. METHODS: Seventeen Wistar rats were used in the study. Ten rats were given CP on 3 consecutive days, and seven rats were given isotonic saline solution. Seven days after the third injection, animals were operated on, and their eustachian tubes were occluded. On the seventh day after the operation, five rats from the test group and three from the control group were killed. The others were killed 21 days after the operation. In the histopathologic examination of the sections, acute inflammation and proliferation scores were determined. Gland degeneration, goblet cell hyperplasia, and the density of mast cells were evaluated. Neural elements were stained immunohistochemically. RESULTS: The acute inflammation score in the test group was lower, but the difference was insignificant (p > 0.05). The proliferation score in the test group was lower, and the difference was significant (p = 0.02). In the control group, gland degeneration was significantly higher (p = 0.044). Goblet cell hyperplasia demonstrated no difference between two groups (p > 0.05). Mast cell density was higher in the control group, but the difference was not significant (p > 0.05). Substance P immunoreactivity (IR) was significantly higher in the control group (p = 0.015). calcitonin gene-related peptide-IR and vasoactive intestinal polypeptide-IR were limited in both groups. CONCLUSIONS: That CP pretreatment reduces inflammatory proliferative findings, and gland degeneration leads us to consider that it could be effective in both treatment of experimental otitis media with effusion and prevention of its complications.

Elevated expression of MMP-13 and TIMP-1 in head and neck squamous cell carcinomas may reflect increased tumor invasiveness.

BACKGROUND: Matrix metalloproteinases [MMPs], which degrade the extracellular matrix, play an important role in the invasion and metastasis of squamous cell carcinomas. One MMP, MMP-13, is thought to play a central role in MMP activation. The purpose of this study was to investigate MMP-13 and TIMP-1 expression in squamous cell carcinomas of the head and neck and to relate these levels of expression to histologic patterns of invasion. METHODS: This study included T1 lesions obtained via biopsy from the larynx, tongue, and skin/mucosa of 78 patients with head and neck squamous cell carcinomas. The relationship between expression of MMP-13 and TIMP-1 and the mode of tumor invasion [MI] was evaluated immunohistochemically, using breast carcinoma tissue as a positive control. RESULTS: Increased expression was observed in highly invasive tumors, as reflected by the significant correlation between the degree of staining for MMP-13 or TIMP-1 and MI grade [p < 0.05]. There was no significant relationship between the degree of staining for MMP-13 or TIMP-1 and patient age, sex, tumor site, or tumor histologic grade. In addition, levels of staining for MMP-13 did not correlate with levels of staining for TIMP-1. CONCLUSION: The expression of MMP-13 and TIMP-1 appears to play an important role in determining the invasive capacity of squamous cell carcinomas of the head and neck. Whereas additional studies are needed to confirm these findings, evaluating expression of these MMPs in small biopsy samples may be useful in determining the invasive capacity of these tumors at an earlier stage.

Reproducibility of the 1998 World Health Organization/International Society of Urologic Pathology classification of papillary urothelial neoplasms of the urinary bladder.

OBJECTIVES: This study assessed the diagnostic agreement and intra- and inter-observer reproducibility of the World Health Organization/International Society of Urologic Pathology Consensus Classification of Urothelial Neoplasms (1998 WHO/ISUP classification) and the 1973 WHO classification. METHODS: A teaching set with 5 slides of each papillary neoplasm of low malignant potential, low-grade papillary carcinoma, high-grade papillary carcinoma, and a guideline, as well as a study set of 30 slides containing ten cases of each category, were sent to participants. Six pathologists expert in urological pathology reviewed the 30 slides of non-invasive papillary urothelial tumors in the study set. Diagnostic accuracy and reproducibility were evaluated using intra- and inter-rater techniques (kappa statistic). RESULTS: A moderate to substantial intra- and inter-observer reproducibility was achieved for both the 1998 WHO/ISUP and 1973 WHO classification. The results of the two classification systems were not different statistically ( P>0.05). Reproducibility was lower in low-grade tumors for both classifications. CONCLUSIONS: The new proposed classification system for non-invasive urothelial neoplasms does not increase the reproducibility. There is still a need for uniformity in grading in order to compare the different studies and therapies and to provide more accurate information for management.

[Doxorubicin-induced chemomyectomy effects in rat skeletal muscle]. / Doksorubisinin siçan iskelet kasinda kemomiyektomi etkisi.

OBJECTIVES: This study was based on the assumption that decreased number of active muscle fibres in the spastic muscle may be associated with restoration of the balance. Hence, the effect of doxorubicin was evaluated in inducing permanent cell necrosis on skeletal muscle (chemomyectomy). METHODS: Eighteen Wistar rats were divided into two groups, equal in size, to receive doxorubicin injections (0.02 and 0.05 mg/0.5 ml) into the left quadriceps muscles under general anaesthesia. The right quadriceps muscles (controls) were assigned to receive 0.5 ml of physiologic saline solution. Three rats in each group were sacrificed on the 1st, 6th, and 10th post-injection weeks, after which all the quadriceps muscles were histologically evaluated. RESULTS: Histopathologic findings were normal in the control muscles. Dose-dependent acute inflammatory reactions were observed in both groups at the end of the first week, with a higher severity at 0.05 mg doses. The size of the inflammation areas were 2.5% and 10% at 0.02 mg and 0.05 mg doses, respectively. At the end of six weeks, acute inflammatory reactions were replaced by the development of regeneration and fibrosis accounting to 0.8% of all the areas, regardless of the dose given. Specimens of the 10th week exhibited no regeneration; there was a mean of 2.5% fragmentation within the impaired areas, with atrophic fibrils and marked fibrosis showing no dose-dependence. CONCLUSION: Doxorubicin induces a dose-dependent inflammatory reaction during the early postoperative period. In the long-term, permanent effects, which are not dose-dependent, are observed characterized by fragmentation, atrophy, fibrosis of muscle fibres, and losses in contractile muscle elements. These doxorubicin-induced chemomyectomy effects may prove therapeutic in the treatment of spasticity-associated diseases.

Nature of cell kinetics in psoriatic epidermis.

BACKGROUND: Psoriasis vulgaris is a common chronic inflammatory dermatosis. Disorders in keratinocyte proliferation, differentiation, inflammation and immune dysregulation are the major factors implicated in the pathogenesis of psoriasis vulgaris. METHODS: The study was performed in skin specimens of 25 patients with psoriasis vulgaris and a control group of 10 individuals without a skin disease. Biopsy specimens from lesional and normal skin were analyzed by immunohistochemical method for expressions of Ki-67, Bcl-2, terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labeling (TUNEL), tumor necrosis factor alpha (TNF-alpha) and nuclear factor kappa B (NF-kappaB). In addition, densities of mast cell infiltration were also investigated. RESULTS: Ki-67 and TUNEL indexes and TNF-alpha and NF-kappaB expressions were significantly higher in psoriatic epidermis than in normal epidermis (p < 0.05). There was no significant difference at Bcl-2 reactivity between the normal and the psoriatic epidermis (p > 0.05); however, Bcl-2 staining intensity of lymphocytes was higher in psoriatic lesions than in normal dermis (p < 0.05). Additionally, the number of mast cells was significantly higher in psoriatic dermis than in normal skin (p < 0.05). CONCLUSIONS: There were several complex factors involved in the pathogenesis of psoriasis. We conclude that cellular damage and apoptosis temporarily coincide with epidermal proliferation during the course of psoriatic hyperplasia.

Effects of cyclosporin a on neurological outcome and serum biomarkers in the same setting of spinal cord ischemia model.

Spinal cord ischemic injury is one of the feared complications during aortic cross-clamping. The aim of this study was to investigate whether cyclosporin A (CsA) has a protective effect on spinal cord during ischemia in a rabbit model. A total of 22 New Zealand white rabbits were studied in three groups. One of the groups served as a sham group (n=7), in which only laparatomy was performed and closed. One group served as a control group (n=7), in which rabbits had their abdominal aortas cross-clamped for 40 min following median laparatomy. The last group was the CsA group (n=8), in which rabbits underwent the same procedure as the control group as well as CsA infusion at 20 mg/(kg . hr) over 60 min starting with aortic cross-clamping and continuing in the first 20 min of reperfusion. Neurological outcome of rabbits was evaluated according to Johnson's scale at postoperative hours 24 and 48 in all groups, and then they were killed. Their spinal cords were harvested, and segments corresponding to L4-L6 were prepared for pathological examination. Serum neuron-specific enolase (NSE) and nitric oxide (NO) levels were measured prior to and following aortic occlusion, and comparisons were made. Physiological data were similar in all groups. Rabbits in the sham group did not have any neurological deficit. However, all rabbits in the control group showed severe neurological deficits, including total paraplegia in five. According to Johnson's scale, neurological status of the rabbits at postoperative hour 48 was better in the CsA group compared to controls (p<0.01). Pathological examination of spinal cord specimens revealed a higher viability index in the CsA group compared to controls (p<0.01). Serum NSE and NO levels were lower in CsA-treated animals compared to controls. Our results demonstrate that CsA, when administered during ischemia and in the early period of reperfusion, may reduce neuronal damage in the spinal cord in a rabbit model of transient spinal cord ischemia.

Breast carcinogenesis. Transition from hyperplasia to invasive lesions.

OBJECTIVES: To examine the balance loss between proliferation and apoptosis that play a role in breast cancer development, and to explore the places of various genes and molecules within this process in this supposed multistep process. METHODS: We obtained the specimens from 40 patients between 2002 and 2004 at the Department of Pathology, Medical Faculty, Adnan Menderes University, Aydin, Turkey. We categorized the lesions ductal hyperplasia (DH), atypical ductal hyperplasia (ADH), in situ ductal carcinoma (DCIS), and invasive ductal carcinoma (IDC). We determined the tumor size, histological grade and lymph node status of invasive cases and we used nottingham prognostic index (NPI). We applied ER, PR, c-erbB2, p53, Ki-67, bcl-2, dUTP nick end labeling (TUNEL), breast cancer gene-1, matrix metalloproteinases-1 and tissue inhibitor matrix metalloproteinases-1 stains to each lesion using the immunohistochemical method. RESULTS: We observed that ER and PR decreased in ADH when compared with DH (p=0.0001 and p=0.019). However, we determined that in DCIS as c-erbB2 (p=0.005) and Ki-67 (p=0.004) increase, TUNEL (p=0.04) and bcl-2 (p=0.005) decrease, when compared with ADH. When compared with DCIS lesions, we observed the existence of a higher c-erbB2 (p=0.003) and a lower TUNEL (p=0.012) in invasive tumors. Furthermore, we found that there is a higher MMP-1 (p=0.04) in invasive lesions, when compared with non-invasive lesions. We detected higher PR (p=0.049), lower TUNEL and c-erbB2 (p=0.017) in low grade group of NPI, when compared with high grade group of NPI. CONCLUSION: As a result, it has been shown that together with increase in proliferation, decrease in apoptosis, too, contributes to the proliferation/apoptosis imbalance that occurs in breast carcinogenesis. Increase in proliferation and decrease in apoptosis are parallel with the progression of lesions. We also showed that the changes, beginning with loss of ER and PR in ADH step, can cause malign transformation, which is especially notable both in DCIS step due to Ki-67 and c-erbB2 increase, and also with bcl-2 and TUNEL decrease.

Multiple melanotic schwannoma.

Melanotic schwannoma is a rare pigmented neural tumor most commonly occurring in the paraspinal region. In a small minority of instances, melanotic schwannoma may have multiple nodules. Here, a 52-year-old woman is presented with multiple melanotic schwannomas of paraspinal region.

Subcutaneous nodules on the face: acantholytic in-transit cutaneous metastasis.

BACKGROUND: Although skin is the biggest organ of the body, cutaneous or subcutaneous metastasis from internal malignancies or primary skin cancers is very uncommon. OBJECTIVE: In this report, we present two cases of in-transit cutaneous metastasis in acantholytic pattern from a skin tumor on the face. METHODS: Both patients presented with the subcutaneous nodule and had history of multiple squamous cell carcinomas on the face. The epidermis was intact above the nodule. Computed tomography scan was performed, and subcutaneous located cystic lesions were seen. RESULTS: Nodules were excised and reported as acantholytic squamous cell carcinoma. There was no recurrence or distant metastasis. There were no early or late complications. CONCLUSIONS: These nodules were in-transit metastasis of the previous multiple squamous cell carcinomas of the face. Although patients with squamous cell carcinomas presenting as subcutaneous nodules of the head were presented in the literature, to our knowledge, this is the first report to present in-transit metastasis of acantholytic squamous cell carcinoma.

The reliability of long-term storage of direct immunofluorescent staining slides at room temperature.

BACKGROUND: Long-term preservation of immunofluorescence is important for re-examinations. We investigated whether the storage of direct immunofluorescent (DIF)-positive slides at room temperature was reliable in daily practice. METHODS: One hundred and twenty-five DIF-positive slides from the skin of 52 patients were evaluated. Sections were examined for the presence of immunoglobulin A (IgA), IgG, IgM, C3, and fibrinogen using fluorescein isothiocyanate-conjugated antisera and mounted with a ready-to-use permanent mounting medium containing an antifading reagent and sodium azide (DAKO, Glostrup, Denmark, S3023). The slides were stored at room temperature for 16-24 months. Changes in diagnostic pattern, fluorescence intensity, and the form and location of accumulation of immunoreactants and technical deformation were investigated. RESULTS: Over the entire observation period, 49.6% of the slides faded away; the median length of survival was 16 months. Before 12 months, the survival rate of slides was 92.0%, whereas after 20 months it was 28.0%. In the early faded slides, which faded away before 16 months following the first examination, C3 and IgA were the most frequently observed immunoreactants. The technical deformations did not prevent the diagnosis. CONCLUSIONS: The preservation of fluorescence in DIF-positive slides using mounting media with an antifading reagent is possible for 2 years at room temperature. However, in daily practice, storage for longer than 11 months prevents a reliable diagnosis.