Ediz Tutar

A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve

Plexiform neurofibroma is generally considered as a component of neurofibromatosis 1, and a great majority of the cases with plexiform neurofibroma display other symptoms related to neurofibromatosis. Plexiform neurofibromas occur frequently in the head and neck region due to the rich innervation of the area, however appear rarely in the extremities. We report here an isolated giant plexiform neurofibroma involving the common peroneal nerve branches without symptoms related to neurofibromatosis in a 5-year-old case. Surgical excision was performed due to pain and numbness in the leg, and against the possibility of malignant transformation due to sudden growth observed in the tumor. One month following the excision of all the reachable tumoral tissues, tendon transfer surgery was performed for the ankle and toe extensions. Our case stands as the only reported case of isolated giant plexiform neurofibroma involving the common peroneal nerve in the pediatric age.

Protective Effects of Dexpanthenol and Y-27632 on Stricture Formation in a Rat Model of Caustic Esophageal Injury

BACKGROUND This experimental study was conducted to investigate the effect of dexpanthenol (converted in the body to pantothenic acid) and Y-27632 (a selective Rho-kinase inhibitor) on stricture formation after caustic (alkaline) esophageal injury in rats. MATERIALS AND METHODS Sixty male Wistar albino rats were randomly allocated into six groups. In group 1 (sham) the distal esophagus was isolated and cannulated but no caustic injury was induced. In all remaining groups, a caustic esophageal burn was induced with 50% sodium hydroxide solution for 90 s and drug treatment was given by daily intraperitoneal injection, beginning 24 h after injury and continuing for 21 d. In group 2 (controls), animals were treated with 0.9% saline; in groups 3 and 4, with 50 and 500 mg/kg/d of dexpanthenol, respectively; and in groups 5 and 6, with 0.3 and 3 mg/kg/d of Y-27632, respectively. Rats were sacrificed 22 d after caustic injury and the distal esophagus was isolated for histopathology and biochemical investigation. RESULTS Stenosis index and collagen deposition scores were significantly lower in both the dexpanthenol and Y-27632 treated groups (P<0.05). Dexpanthenol and Y-27632 treatment markedly depressed esophageal tissue malondialdehyde and hydroxyproline levels. CONCLUSION In this experimental model of caustic esophageal stricture, dexpanthenol and Y-27632 significantly attenuated esophageal stricture formation. These findings indicate that inhibition of Rho-kinase or dexpanthenol administration may offer novel therapeutic approaches in the treatment of caustic esophageal injury.

Unusual presentation: Pulmonary hemosiderosis with celiac disease and retinitis pigmentosa in a child†

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9‐year‐old boy with Lane‐Hamilton syndrome, co‐occurrence of pulmonary hemosiderosis with CD. This presentation is unique presentation because he has also retinal pigmentation. Pediatr. Pulmonol. 2011; 46:820–823.

Urotensin Inhibition with Palosuran Could Be a Promising Alternative in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive and a life-threatening disease with its high morbidity and mortality ratios. On searching for new shining targets in pathogenesis, we noticed, in our previous studies, urotensin-II (UII) in systemic sclerosis with potent angiogenic and pro-fibrotic features. Owing to the mimicking properties of UII with endothelin-1 (ET1), we attempted to investigate the effect of palosuran in a PAH rat model. Thirty rats were randomly divided into three groups, with each group comprising 10 rats: group 1 (control group) received the vehicle subcutaneously, instead of monocrotaline (MCT) and vehicle; group 2 (MCT group) received subcutaneous MCT and vehicle; and group 3 (MCT + palosuran group) received subcutaneous MCT and palosuran. Serum UII, ET1, transforming growth factor-β1 (TGF-β1) levels, pulmonary arteriolar pathology of different diameter vessels, and cardiac indices were evaluated. The ET1, TGF-β1, and UII levels were significantly diminished in the treatment group, similar to the controls (p < 0.001). Right ventricular hypertrophy index and mean pulmonary arterial pressure scores were also significantly reduced in the treatment group (p = 0.001). Finally, in the 50–125-μm diameter arterioles, in contrast to Groups 3 and 1, there was a statistically significant thickness (p < 0.01) in the arteriolar walls of rats in Group 2. The treatment effect on arteries of more than 125-μm diameters was found to be valuable but not significant. Owing to its healing effect on hemodynamic, histological, and biochemical parameters of MCT-induced PAH, palosuran as an antagonist of UII might be an optional treatment alternative for PAH.

The Efficiency of a Urotensin II Antagonist in an Experimental Lung Fibrosis Model

Pulmonary fibrosis is a chronic disease. Urotensin II (U-II) is a new peptide with angiogenic and profibrotic features. Therefore, we aim to evaluate the antagonism of U-II with palosuran in an animal model and plan to measure U-II, endothelin-1 (ET-1), and transforming growth factor-β1 (TGF-β1) and their association with lung fibrosis. Thirty Wistar male rats were used in the study and were divided into three groups: group 1, control; group 2, bleomycin-induced lung fibrosis group; and group 3, bleomycin-induced lung fibrosis with treatment palosuran group. U-II level (nanograms per milliliter) was 2.957 ± 0.159 in group1, 3.188 ± 0.122 in group 2, and 2.970 ± 0.165 in group 3 (p = 0.002). The ET-1 level (picograms per milliliter) was 4.486 ± 0.376 in group 1, 9.086 ± 1.850 in group 2, and 4.486 ± 0.376 in group 3 (p < 0.001). The TGF-β1 (nanograms per milliliter) level was 73.143 ± 9.96 in group 1, 84.81 ± 4.73 in group 2, and 77.86 ± 5.77 in group 3 (p = 0.006). Finally, the fibrosis score was 0.7 ± 0.48 in group 1, 4.4 ± 1.34 in group 2, and 3.2 ± 0.63 in group 3 (p < 0.001). There is a statistically significant positive relationship between fibrosis scores and the UT-II, ET-1, and TGF-β1 levels of the experimental lung fibrosis model. We believe U-II is an important mediator in lung fibrosis models, and its antagonism with palosuran could be a new treatment choice for interstitial lung fibrosis, but further studies need to be conducted to verify the findings of the current study.

Rituximab seems a promising therapeutic option in granulomatosis with polyangiitis with intestinal perforation: a case report and literature review

Granulomatosis with polyangiitis (Wegeners) (GPA) is a chronic disease of unknown aetiology that leads to necrotising vasculitis in small and medium-sized vessels characterised by respiratory system and kidney involvement. Intestinal involvement is rare and perforation is even rarer in GPA. In this study, we are presenting a literature review of related cases, and a 29-year-old man referred from the emergency department with a multiple distal ileal perforation that was diagnosed with GPA, and successfully treated with rituximab.

Protective effects of Y-27632 on hypoxia/reoxygenation-induced intestinal injury in newborn rats

BACKGROUND/PURPOSE Necrotizing enterocolitis (NEC) is a major cause of mortality in neonates and is associated with a disruption in the protective intestinal barrier. The precise cause of NEC is elusive. However, ischemia/reperfusion injury of the intestine has been considered a major contributing factor. We examined the role of Y-27632, a selective Rho-kinase inhibitor, on a hypoxia/reoxygenation (H/R)-induced intestinal injury of newborn rat pups. METHODS Hypoxia/reoxygenation was achieved by placing rat pups in an airtight chamber aerated with 95% N(2) + 5% CO(2) for 10 minutes followed by 10-minute 100% oxygen. Forty newborn rat pups were randomly allocated into 4 groups. Group 1 served as untreated controls. The pups in group 2 were subjected to H/R only. In groups 3 and 4, the rats were treated with intraperitoneal injection of 0.3 and 3 mg kg(-1) day(-1) of Y-27632 for 5 days following H/R, respectively. The pups were killed 6 days following the H/R injury. Intestine specimens were evaluated for histopathology and biochemical investigation. RESULTS The microscopic lesions in H/R rat pups were virtually the same as those seen in neonatal NEC, with severe destruction of villi and crypts. Hypoxia/reoxygenation resulted in significant elevation in malondialdehyde levels, but decreased tissue nitric oxide levels (P < .05). Protective effects of Y-27632 on H/R-induced intestinal injury of newborn rat pups were observed with a significant decrease in the intestinal injury score, suppression in malondialdehyde levels, and increase in nitric oxide levels (P < .05). CONCLUSIONS In this experimental study, Y-27632 significantly attenuated H/R-induced intestinal injury. These findings indicate that inhibition of Rho-kinase may offer a novel therapeutic approach in the treatment of NEC.

Role of fragile histidine triad protein expression in pathogenesis of malignant pleural mesothelioma.

Aim: To investigate the relationship of fragile histidine triad (FHIT) and Ki‐67 expression with clinicopathological variables of patients with malignant pleural mesothelioma (MPM). Methods: Formalin‐fixed, paraffin‐embedded tissue sections of 30 asbestos induced MPM (epithelial and biphasic) patients were examined for FHIT and Ki‐67 expression using immunohistochemical techniques and results were compared with clinicopathological variables. Results: Immunohistochemical study results were as follows: 12 (40%) cases showed low FHIT expression and 18 (60%) showed high expression. There was no significant relationship between FHIT and age, gender or histological subtypes (p > 0.05). Ki‐67 expression was ‘low’ in 13 (43.3%) cases and ‘high’ in 17 (56.7%) cases. No correlation could be demonstrated between Ki‐67 expression and age, gender or histological subtypes (p > 0.05). No significant association was observed between FHIT and Ki‐67 expression in MPM. Conclusion: The results support the role of FHIT as a tumour suppressor gene in asbestos induced MPM. There is no significant correlation between FHIT and cell proliferation marker expressions in malignant pleural mesothelioma. Therefore, it can be concluded that loss of FHIT does not interfere with tumour proliferation. This can be accepted as evidence for an early role of FHIT loss in carcinogenesis; however, it needs to be strengthened by further studies.

Biopsy of vertebral tumour metastasis for diagnosing unknown primaries

Purpose. To examine patients with vertebral tumour metastasis using transpedicular biopsy for diagnosing unknown primary tumours. Methods. 13 men and 8 women aged 41 to 80 (mean, 61) years with vertebral tumour metastasis of unknown primary origin underwent transpedicular biopsy of the affected vertebra. Results. The origins of the primary tumours were lung cancer (n=6), prostate cancer (n=5), colorectal cancer (n=5), kidney cancer (n=4) and lymphoma (n=1). All the specimens matched pathological characteristics of their corresponding primary tumours, except in one patient. This 42-year-old man had stage-4 colon cancer, in whom the pathologic findings could not enable differentiation between colon and prostate cancer. Conclusion. Transpedicular biopsy of the vertebra is a cost-effective diagnostic tool for evaluating unknown primary tumours.

Amyloid tumor of the breast mimicking breast carcinoma.

Amyloid tumors of the breast are extremely rare. Few isolated cases have been reported to date. Amyloid involvement of the breast has no specific diagnostic features on mammography; on occasion, this causes diagnostic challenges. In this paper, the case of a 58-year-old woman with an amyloid tumor of the breast, which developed secondary to long-standing rheumatoid arthritis, is presented. She presented with a palpable mass in her right breast, which led to an erroneous clinical diagnosis of breast cancer. The unusual mammography findings are demonstrated and the differential diagnosis, in light of the radiological images and the clinical and pathologic features, is discussed.