Bilateral giant full thickness macular holes: an infrequent manifestation of alport syndrome

Purpose: To report a case of Alport syndrome presenting with bilateral giant full-thickness macular holes, hypertensive chorioretinopathy, and exudative retinal detachment. Case Report: A 20 year-old man, a known case of Alport syndrome on hemodialysis, was referred to our clinic with bilateral vision loss initiated about 10 years prior to presentation, which exacerbated in the month prior to our visit. Bilateral large full-thickness macular holes, hypertensive chorioretinopathy, and exudative retinal detachment were detected in fundus examination. The patient had previous genetic counseling confirming the diagnosis of Alport syndrome. During follow-up, macular holes were covered with a thick epiretinal membrane and visual acuity decreased progressively in two weeks. Pars plana vitrectomy was performed in the right eye. Two weeks following surgery, the macular hole was closed and visual acuity improved significantly. Conclusion: Bilateral giant full-thickness macular holes are uncommon presentations of Alport syndrome. The retinal findings may be caused by an inefficient type IV collagen presenting in the Bruch's membrane and in the internal limiting membrane. Pars plana vitrectomy can be considered to repair macular holes in these patients.

Effects of bone marrow mesenchymal stem cells-conditioned medium on tibial partial osteotomy model of fracture healing in hypothyroidism rats

Background: Hypothyroidism is associated with dysfunction of the bone turnover with reduced osteoblastic bone formation and osteoclastic bone resorption. Mesenchymal stem cells [MSCs] secrete various factors and cytokines that may stimulate bone regeneration. The aim of this study was to determine the effects of MSCs-conditioned medium [CM] in hypothyroidism male rats after inducing bone defect

Methods: In this study, 24 male rats were randomly assigned to three groups: [I] hypothyroidism + bone defect [HYPO], [II] hypothyroidism + bone defect + CM [HYPO + CM], and [III] no hypothyroidism + bone defect [control]. Four weeks after surgery, the right tibia was removed, and immediately, biomechanical and histological examinations were performed

Results: The results showed a significant reduction in bending stiffness [32.64 +/- 3.99], maximum force [14.63 +/- 1.89], high stress load [7.59 +/- 2.31], and energy absorption [12.68 +/- 2.12] at the osteotomy site in hypothyroidism rats in comparison to the control and hypothyroidism + condition medium groups [p < 0.05]. There was also a significant decrease in the trabecular bone volume [3.86 +/- 3.88] and the number of osteocytes [5800 +/- 859.8] at the osteotomy site in hypothyroidism rats compared to the control and hypothyroidism + condition medium groups [p < 0.01 and p < 0.02, respectively]

Conclusion: The present study suggests that the use of the CM can improve the fracture regeneration and accelerates bone healing at the osteotomy site in hypothyroidism rats

[Assessment of quality of life in patients with irritable bowel syndrome: a cross-sectional study in south-east Iran]

Background: Improving life is the main goal of therapeutic interventions for chronic diseases. Although we needed to determine quality of life by various variables. This study aimed to determine quality of life in patients with Irritable Bowel Syndrome [IBS] in Kerman

Materials and Methods: This is a cross sectional study of 143 patients with IBS referred to a specialist, assessed by IBS-QOL.34. Data analyzed by descriptive statistics, Pearson correlation coefficients, t-test and ANOVA, using SPSS software. p.Value <0.05 was considered as statistically significant

Results: In this study from 143 samples, 82 were females [57.3%] and 61 males [42.7%]. Quality of life score in patients was 37.16 +/- 16.72, and there was significant difference in terms of employment and education level [p.value<0.05]

Conclusion: Among patients with IBS, quality of life is far from desired. Therefore, health care providers should consider psychosocial support alongside medical management for achieving better quality of life in these patients

Nonadherence to medication in inflammatory bowel disease: rate and reasons

Background: This study is the first study to evaluate the nonadherence rate and reasons of same patient with inflammatory bowel disease [IBD] in Iran

Method: During 9 months, 500 patients with IBD were enrolled in the study. Patients were interviewed about their nonadherence behaviors. Factor analysis was used to analyze the collected answers

Results: The overall rate of nonadherence was 33.3% [27.6% intentional nonadherence and 5.7% unintentional nonadherence]. 33.6% of the patients had at least one relapse after discontinuing treatment. The most frequent reason for intentional nonadherence was discontinuing the treatment after recovering from symptoms [42.7%]. The most frequent reason for unintentional nonadherence was forgetfulness [5.2%]. 19.8% of the patients did not visit their gastroenterologist on time and they purchased drugs from the drugstore. These patients reported that their clinics were too far and difficult to access. There was no significant relationship between nonadherence and demographic variables

Conclusion: Multiple reasons are suggested as factors of medication nonadherence and they seem to be different among different populations. Determining these possible reasons, could lead to finding suitable strategies to overcome or reduce them

Assessment of vertical root fracture using cone-beam computed tomography

PURPOSE: The aim of this study was to investigate the accuracy of cone-beam computed tomography (CBCT) in the diagnosis of vertical root fractures in a tooth with gutta-percha and prefabricated posts. MATERIALS AND METHODS: This study selected 96 extracted molar and premolar teeth of the mandible. These teeth were divided into six groups as follows: Groups A, B, and C consisted of teeth with vertical root fractures, and groups D, E, and F had teeth without vertical root fractures; groups A and D had teeth with gutta-percha and prefabricated posts; groups B and E had teeth with gutta-percha but without prefabricated posts, and groups C and F had teeth without gutta-percha or prefabricated posts. Then, the CBCT scans were obtained and examined by three oral and maxillofacial radiologists in order to determine the presence of vertical root fractures. The data were analyzed using IBM SPSS 20.0 (IBM Corp., Armonk, NY, USA). RESULTS: The kappa coefficient was 0.875+/-0.049. Groups A and D showed a sensitivity of 81% and a specificity of 100%; groups E and B, a sensitivity of 94% and a specificity of 100%; and groups C and F, a sensitivity of 88% and a specificity of 100%. CONCLUSION: The CBCT scans revealed a high accuracy in the diagnosis of vertical root fractures; the accuracy did not decrease in the presence of gutta-percha. The presence of prefabricated posts also had little effect on the accuracy of the system, which was, of course, not statistically significant.

Surveillance for hepatocellular carcinoma after autologous stem cell transplantation in Cirrhosis

During the resent years there has been interest in using bone marrow stem cells to treat liver cirrhosis. However, there is a potential concern for malignant transformation after stem cell therapy. The aim of this study was to evaluate the development of hepatocellular carcinoma [HCC] after autologous bone marrow stem cell transplantation for liver cirrhosis. All the patients who underwent autologous stem cell transplantation for liver cirrhosis between 2005 and 2011 at our center were enrolled. Cellular infusion was made through peripheral vein, portal vein, or hepatic artery.The patients were invited to undergo screening for hepatocellular carcinoma. The screening was made with ultrasonography and alpha-feto protein [AFP] measurement. Thirty two patients [18 males] were included in the study. Mean age of patients was 45.7 years. Fifteen patients [47%] received mesenchymal stem cell [MSC], 9 [28%] received bone marrow mononuclear cells, 5 [16%] were given CD 133-positive bone marrow cells, and 3 [9%] patients received CD 34-positive bone marrow cells. Mean duration of follow up was 20.5months. Mean serum level of AFP was 2.8 ng/ml at baseline and 3.4ng/ml at the end of follow up [p= 0.3]. One patient was found to have hepatocellular carcinoma three months after infusion of bone marrow mononuclear cells. The incidence rate for HCC was 1.8 cases per 100 person-years in this study. Autologous bone marrow stem cell infusion does not appear to increase the risk of hepatocellular carcinoma. The incidence rate of HCC in this study is comparable or even less than the reported rates of HCC in cohort studies of cirrhotic patients

In vitro differentiation of human bone marrow mesenchy-mal stem cells into hepatocyte-like cells

Orthotropic liver transplantation [OLT] is the final procedure of both end stage and metabolic liver diseases. Hepatocyte transplantation is an alternative for OLT, but the sources of hepatocytes are limited. Bone marrow mesenchymal stem cells [BM-MSCs] can differentiate into hepatocyte-like cells and are a potential alternative source for hepatocytes. We aimed to investigate the differentiation potential of BM-MSCs into hepatocyte-like cells. Human BM-MSCs from a healthy donor were cultured and differentiated into hepatocyte-like cells. We investigated the expression of hepatocyte-specific markers in MSC-derived hepatocyte-like cells [MSC-HLC[s]] and evaluated their functionality using metabolic assays. MSC-HLCs expressed hepatocyte-specific markers at both mRNAand protein levels. In addition, the cells had the ability to uptake low density lipoprotein [LDL], clear ammonia, secrete albumin, and store glycogen. MSC-HLCs were transplanted into a familial hypercholesteromia patient. Human MSCs can be differentiated into partially functional hepatocyte-like cells. Thus, they could be a potential source for cell therapy in liver disorders

Eosinophilic gastroenteritis: a case series from Iran

Eosinophilic gastroenteritis [EG] is a rare inflammatory disorder of the gastrointestinal [GI] tract. There have been several case series of patients with EG from the western world and East Asia. However, there has not been a report of patients with EG from the Middle East region. The aim of this study is to describe clinical characteristics and treatment response in a series of EG patients from Iran. We retrospectively reviewed charts with a diagnosis of EG from 1997 to 2010 at Shariati Hospital and the private clinics of the authors. Clinical characteristics of the patients were evaluated, and the treatment response and relapse rate were assessed. Twenty-two patients [9 male] with EG were identified. Mean age of the patients was 45.1 +/- 15.5 [range: 27-75] years. Median duration between symptom onset and diagnosis was 12 [range 1- 48] months. Twenty [90%] patients had mucosal involvement, one [5%] had muscular involvement and one [5%] had subserosal involvement. Patients were followed for a median duration of 36.5 [range 4- 123] months. Two [90%] patients had mucosal involvement, one [5%] had muscular involvement and one [5%] had subserosal involvement. Patients were followed for a median duration of 36.5 [range 4-123] months. Two patients had spontaneous remission with supportive care. The remaining 20 patients responded well to oral corticosteroid treatments. The relapse rate was 33%. Episodes of relapse were successfully controlled with a repeat course of corticosteroids. Two patients with several relapses required maintenance treatment with azathioprine. The clinical characteristics and treatment responses of EG patients from Iran are similar to reports from other parts of the world. Patients need to undergo close follow up after treatment to detect early signs of relapse

effect of silver nitrate, chloroformic garlic extract, and normal saline in induction of sclerosing cholangitis in rabbits

To evaluate the effects of 0.5% silver nitrate, 20% chloroformic garlic extract, and 0.9% normal saline in induction of sclerosing cholangitis in the bile ducts of rabbits. During a-6-months period from April to September 2006 in Shiraz University Laboratory Animal Research Center, we selected 3 equal groups of rabbits. We injected 0.5% silver nitrate, 20% chloroformic garlic extract, and 0.9% normal saline into the bile ducts of each group. The animals were euthanized, and autopsied after 4 months and the liver and bile ducts were removed and studied histopathologically. Cholangiography was undertaken to evaluate the presence and extent of any sclerosing cholangitis. Animals showed sclerosing cholangitis in silver nitrate group [7 [58%]], one [8%] in chloroformic garlic extract group and one [7%] in normal saline group. The difference between silver nitrate and chloroformic garlic extract groups were statistically significant and similar results were noticed between chloroformic garlic extract and normal saline groups. Twenty percent of chloroformic garlic extract had fewer complications such as sclerosing cholangitis, compared to other materials

[ first experience of stem cell labeling in iran using 111In- oxine]

The field of stem cell biology and regenerative medicine is rapidly moving toward translation to clinical practice. Stem cell therapy seems to be a new treatment option for some diseases. So, tracking the distribution of stem cells is crucial to their therapeutic use. Based on this fact we labeled human mesenchymal stem cells [MSCs] with 111In-oxine for the first time in our country. The aim of this study was to investigate the possibility of stem cell labeling in Iran. In addition the researchers assessed the cell viability, specific activity and labeling efficiency after labeling. After culturing mesenchymal stem cells [MSCs], for radio-labeling, the sample [which contained 1x106MSCs] were mixed, and suspended with 50 micro Ci 111In-oxine, and then incubated for 20 min at the room temperature. The cells were then washed with normal saline twice to remove the free 111In. The labeling efficiency, specific activity and cell viability was 70.6%, 31.70 micro Ci/106 and 100%, respectively. It seems that, this method is practical and easily applicable with acceptable efficiency and specific activity in our laboratory settings using pharmaceutical produced in Iran

Study of clinical features of Wegener's in head and neck structures

To study the clinical features of Wegener's in head and neck structures. Method: We reviewed all selected patients with diagnosis of Wegener's granulomatosis according to ACR criteria from two academic hospitals in Mashhad. 12 patients with diagnosis of WG during the past 10 years were studied. M/F ratio was 2/1 and the majority of them were in the fifth decade of life. 75% of our patients had head and neck related complaints and 65% had lower respiratory tract involvement. Recurrent sinusitis in 45%, nose structure involvement in 72%, eye complaints in 27% and ear involvements were found in 45%. We had one case of eye proptosis and 15% nose deformity. Eight patients had biopsy samples, and granulomatos vasculitis with necrosis were found in 25%. Wegener's granulomatosis is a rare systemic vasculitis in our patients. Ulcer with giant crust, sensory neural hearing loss in association with systemic findings such as low grade fever, fatigue and arthralgia