Iranian Journal Of Pathology
Volume:5 Issue: 1, winter2010

Becker Muscular Dystrophy (BMD) is a subtype of dystrophinopathies and designated as “mild form of dystrophinopathy”. The frequency rate of the disease is 1:18000 to 1:30000 in different populations and the symptoms are presented at about 5-6 years of age. The diagnostic panel composed of Serum Ceratin Kinase (SCK) measurement, Electromyography (EMG), and as a major component, muscle biopsy and immunohistochemistry for dystrophines; finally the results should be confirmed by Western blot (WB) analysis, which is a sensitive method for protein detection. The aim of this study was using utrophin, an autosomal homologue for dystrophin, which is upregulated in dystrophinopathies, parallel to WB in order to evaluate its diagnostic value. In this case-control study, fifteen clinically suspected cases of BMD were examined from 2006 to 2008. After muscle biopsy and dystrophin IHC, the muscle samples were immunostained for utrophin and the tissue extract were analyzed for protein components. In all of the cases, the results revealed partial staining for utrophin in the sarcolemma and pale or distorted band of dystrophin in WBA. Utrophin immunostain could be considered as an important component of BMD diagnostic panel and may be substituted for WBA, which is an expensive and time-consuming method.

Esophageal cancer especially squamous cell carcinoma (SCC) is one of the most common gastro intestinal malignancies in north part of Iran (Khorasan). The standard treatment for esophageal cancer is surgical resection, but its outcome remains poor. Then, the oncologists try to treat this cancer with sandwich protocols especially neo-adjuvant chemoradiotherapy. Several studies have reported that over expression of Cyclin D1 is a negative prognostic factor and is correlated with poor response to chemo-radiotherapy and decrease of survival. For this reason we evaluated Cyclin D1 expression in patients with esophageal SCC and its effect on response rate to neo- adjuvant chemo-radiotherapy in north-east Iran. We analyzed Cyclin D1 expression by immunohistochemistry in 37 endoscopic biopsies of esophageal SCC from April 2004 to March 2005 in Mashhad University of Medical Science, Iran and compared it with clinical and pathologic response rate to neo adjuvant chemo radiotherapy. Cyclin D1 over expression was detected in 24 patients (64.9%). Nine patients with Cyclin D1 over expression had pathologic complete response (37.5%) as compared with 9 patients with negative cyclin D1 expression (69.2%) (P=0.09). Cyclin D1 is a useful tumor marker to select patients may not be suitable for neoadjuvant chemo-radiotherapy and it is better to refer them for surgery or definitive radiotherapy.

Sinusitis is one of the most common hygienic problems and a large part of the budget has been allocated to its diagnosis and treatment yearly. In this study، the presence of cytomegalovirus in sinuses of patients، undergone operation for treatment، with or without sinusitis، was studied. This study was an applied and case-control study، which had been performed on 44 HIV negative patients in ENT clinic of Shahid Mostafa Khomeini Hospital in Tehran during 4 months in 2005. Biopsy specimens were taken from left and right of uncinet process mucosa of middle meatus of 22 patients with chronic sinusitis and 22 patients without sinusitis undergo operation for nasal septal deviation. After purification of DNA، PCR test was done for replication of early gene in cytomegalovirus DNA by two kits، which was purchased from Cinnagen Co. and Gen Fanavaran Co. After the electrophoresis of PCR product on agarose gel، neither of samples has shown DNA band same the positive control enclosed in kits. Therefore، all specimens were considered negative for cytomegalovirus DNA. Cytomegalovirus has not been detected in sinunasal mucosa of patients with chronic sinusitis and without sinusitis. Test with more specimens and other diagnostic procedure are recommended for prove of absence of cytomegalovirus in sinunasal mucosa.

Apoptosis of eosinophils is of significant value in assessing the airway inflammation in patients with asthma. Our purpose was to investigate the degree of expression of the Bcl-2 protein in sputum eosinophils during acute asthma exacerbation and its relationship with exacerbation severity. The study was carried out in Mostafa Khomeini Hospital, Tehran, Iran in March 2008. Sputum was obtained from 15 asthmatic patients and 13 healthy subjects as a control group. Number of eosinophils was counted and Bcl-2+ eosinophils were stained using immunocytochemistry. Sputum eosinophils and Bcl-2+ eosinophils were significantly higher in patients with acute exacerbation than controls (P<0.05). Bcl-2 prolongs survival and decreases apoptosis of airway eosinophils during acute asthma exacerbation. Eosinophil apoptosis and inhibition of Bcl-2 represent a target for new and effective therapeutic strategies of asthma.

Familial hypercholesterolemia (FH) is an autosomal trait, which is caused by mutations in Low Density Lipoprotein Receptor (LDLR) gene. FH penetrance is about 100% and worldwide prevalence for heterozygous subjects is almost 1 in 500 and for homozygous 1 in 1,000,000. The patients are at risk of premature coronary heart disease (CHD) due to defective LDLR and hence cholesterol metabolism disorder. The aim of this study was identifying genotype of possible mutation in an Iranian FH patient. Promoter and all 18 exons including exon-intron boundaries of LDLR gene were scanned. Polymerase chain reaction - single strand conformation polymorphism (PCRSSCP) was used as mutation scanning method. DNA sequencing was used to identify any nucleotide change(s). A new frameshift mutation (660-661InsCC) was found in proband. This mutation causes a truncated, non-functional protein, which results in hypercholesterolemia. The mutation can be screened in proband''s relatives to find other FH patients.

There is well established the beneficial effects of Crocus sativus extract in learning and memory improvement. In the present study the effect of this plant in memory behavioral impairment and forbrain histological damage induced by STZ-icv model of Alzheimer disease were investigated. This study was conducted at Shahed University (Tehran) in 2007. Forty five male rats were divided into three 15 number groups: 1- Control which received CSF bilaterally two times in 1 and 3 days (10 μl in each injection) 2- STZ-icv, streptozotocin (3 mg/kg) dissolved in CSF was injected (icv) to the animals. 3- STZ+CSE, the STZ-icv animals received the plant extract (30 mg/kg; i.p) one other day as treatment ones. All of the animal groups were weighted and subjected to memory behavioral passive avoidance test and brain histological damage analysis. STZ caused selective injury to the fornix and hippocampus and an enlargement as well as loss of ependymal cell in third ventricle. However, STZ-icv treated animals with CSE (30 mg/ kg, i.p) one other day starting one day pre-surgery for three weeks show higher correct choice and lower errors in shuttle box test than vehicle-treated STZ-injected rats. But the same CSE treatment rats did not show any antagonizing effects on STZ-icv induced histological impairment. Our findings provide an explanation for effectiveness of CSE in preventing the cognitive deficits caused by STZ-icv in rats, which mediated by enzymes, metabolisms (glucose utilization) and other biochemical pathways, but not via histological injury repair.

To evaluate extended spectrum betalactamase (ESBL) positive strains of Klebsiella pneumonia and Escherichia coli in positive bacterial cultures. In this analytical cross-sectional study, between March 2006 and March 2007, 170 bacterial isolates including 133 cases of E. coli and 37cases of K. pneumonia were examined. All cases underwent double disk diffusion for ESBL. Demographic data were assessed and all data analyzed accordingly. Patients’ mean age was 55 ±26.63 yr. Ninety six cases (56.5%) were female and 74 cases (43.5%) were male. Clinical presentation of infection were 118 cases UTI (96.4%), 15 cases septicemia (8.8%), 16cases wound infection (9.4%), 7 cases pneumonia (4.1%), 1 case meningitis (0.6%) and 13 cases other presentations (7.6%). Frequency of ESBL positive in E. coli isolates was 38 cases (28.6%) and in K. pneumonia isolates was 10 cases (27%). There was no significant correlation between ESBL positivity and age, gender, ward or clinical presentation of infection. Incidence of ESBL positive isolates of E. coli and K. pneumonia was high. These results should be considered in administration of broad-spectrum antibiotics by clinicians.

Lipomas of the gasterointestinal Tract are relatively uncommon in clinical practice. Most cases are asymptomatic with small tumor size and do not need any special treatment but the large ones are known to cause symptoms such as abdominal pain, obstruction, intussusceptions, and bleeding. The majority (90%) of these lesions are submucosal with predominantly right sided with a slight preponderance in females. Recurrence or malignant transformation has not documented in these lesions. We present a case of giant submucosal lipoma in a 33 years old male in sigmoid colon presenting with abdominal pain and rectal bleeding from Imam Hospitals’ complex, Tehran-Iran. In patients with gastrointestinal symptoms and large mass in sigmoid and with non-specific findings in colonoscopy, this disease has to be taken into consideration.

Although most breast cancers are adenocarcinomas of the mammary gland, primary breast sarcomas may also arise from mammary gland mesenchymal tissue. Sarcomas represent less than 1% of primary breast neoplasms. These tumors are at high risk of recurrence and are known to have poor prognosis. Surgical resection is the primary treatment for these lesions and axillary lymph node dissection is not routinely indicated in the setting of a clinically negative axilla. In this report, we introduce a patient with breast stromal sarcoma based on surgical pathology that has experienced axillary lymph node recurrence later during follow up period.

Human echinococcosis remains a complex problem that affects several organs. Hydatid disease mainly (85%) affects liver as well as lung, and 10% the rest of the body. Renal involvement is about 2% while skin hydatidosis is nearly 1%. Coincidental involvement of kidney and skin is very rare. We report a 51 year-old female patient with renal and skin (chest wall) hydatid lesions that were excised radically and the diagnosis of hydatidosis was confirmed histologically in EMAM REZA Hospital, Tehran in 2007. For treatment albendazol 400 mg BID was chosen, followed by monthly inspection of liver and CBC control up to six months. The case did not show any sign of recurrence in 24 months of following up.