Adult onset still disease associated with endogenous lipoid pneumonia.

Cholesterol pneumonia or endogenous lipid pneumonia (ELP) is a rare disease that can occur in the context of a systemic disease or following a bronchial obstruction. It is characterized by a wide range of diverse symptoms and various disease course. The present report introduces a young woman diagnosed with adult onset still disease three years ago, who has been referred with macrophage activation syndrome (MAS). She underwent biopsy due to dyspnea and a crazy paving pattern in HRCT of the lungs, leading to the diagnosis of lipoid pneumonia based on the interstitial lymphocytic inflammation and cholesterol granulomas. So far, there has been no report indicating MAS associated with cholesterol pneumonia. This is the second case reporting ELP in the adult onset still disease.

Telomere Shortening in Blood Leukocytes of Patients with Chronic Obstructive Pulmonary Disease.

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is characterized by airflow limitation that is not completely reversible by administration of inhaled bronchodilators. Many studies propose that telomere length shortening might have occurred in COPD patients. We aimed to determine the telomere length in COPD patients and compare the results of non-smoking and smoking control subjects. MATERIALS AND METHODS: In our case-control study, 84 clinically stable COPD patients were recruited on admission to Masih Daneshvari Hospital. Eighty-five healthy controls were also selected including 45 non-smokers and 40 smokers admitted for diseases other than COPD. Spirometry was done for all subjects. Telomere length was measured by quantitative real time PCR as described by Cawthon. The telomere repeat copy number (T) to single-gene copy number (S) ratio was calculated using the comparative Ct method. RESULTS: The mean ±SD of age was 64.33±10.04 years in patients and 65.06 ±10.02 years in controls (P=0.693). The mean ±SD of FEV1 was 1.62±0.75 L in patients, 2.84±0.54 L in smoker controls and 2.83±0.56 L in non-smoker controls; significant differences were detected in this regard between cases and controls (P<0.001). T/S ratio was significantly lower in COPD patients (0.61±0.08) than in the control subjects (0.69±0.09) (P<0.001). However, telomere length was shorter in the patients than in controls in each age group (P<0.001). Additionally, there were no statistically significant differences in telomere length between the smoker and non-smoker control subjects. Regarding the correlation between BMI and telomere length, there were no significant differences among the patients and control groups. CONCLUSION: In conclusion, we found that telomere length in COPD patients was shorter than that in smoker and non-smoker controls, irrespective of age, sex, spirometric variables, BMI and history of cigarette smoking.

Clonality of the immunoglobulin heavy chain genes in B cell non-hodgkin lymphoma using semi-nested PCR.

BACKGROUND: Identification of gene rearrangements and clonality analysis are important techniques for the diagnosis of malignant lymphoproliferative diseases. These methods have various sensitivities based on the type of primer used and method of determination of polymerase chain reaction (PCR) products. This study aimed at determining the clonality of B cell non-Hodgkin lymphoma in Iranian patients using PCR method and 2 primers of FR2 and FR3. MATERIALS AND METHODS: Paraffin embedded blocks of 67 patients with B cell lymphoma and 19 cases with lymphoid hyperplasia of the lymph nodes who presented to NRITLD, Masih Daneshvari Hospital were retrospectively reviewed. After extracting the genomic DNA using phenol and chloroform, clonal analysis was performed using semi-nested PCR by using two primers: FR2 and FR3. PCR products were determined using 2 techniques of heteroduplex analysis, polyacrylamide gel and silver staining and the conventional method of agarose gel and ethidium bromide staining. Appearance of 1 or 2 bands in the desired location were considered as a sign of clonality. RESULTS: Monoclonal gene rearrangement was observed in 62 out of 67 patients (92.5%) as one or two discrete bands appeared within 60-120 base pairs (bp) and 200-300 bp range. Of the mentioned patients, 53 cases (79.1%) had FR2 and 51 (76.1%) had FR3 rearrangement. Heteroduplex analysis along with silver nitrate staining detected 3 out of the remaining 5 cases of lymphoma to be monoclonal. These cases had been reported negative by the conventional technique. In total, 65 out of 67 patients (97%) showed monoclonal gene rearrangement using both the abovementioned techniques. All hyperplasia cases were polyclonal by this method. CONCLUSION: Our study showed that evaluation and detection of clonality using PCR, FR2 and FR3 primers along with heteroduplex analysis is a rapid sensitive technique for the diagnosis of malignant lymphomas.

Melioidosis: It is not Far from here.

In the modern world, with developed traveling facilities, tourism is an important factor in emerging new infectious diseases in non-endemic areas. Therefore, the epidemiology of infections is a considerable issue for physicians and should be taken into account. We report a case of melioidosis in a 69-year-old Iranian man during his trip to Southeast Asia. On admission, he was febrile with tachycardia and tachypnea and had diabetes mellitus and hypertension since eleven years ago. Bronchoscopy and bronchoalveolar lavage (BAL) were performed. Blood and BAL cultures revealed heavy growth of Burkholderia pseudomallei. According to the aforementioned culture results, the patient was treated with meropenem and TMP-SMX, while other antibiotics were discontinued. After 3 weeks, the patient was discharged with stable status and normal pulmonary function; and eradication therapy with TMP-SMX continued for about 3 months. The control lung CT scan after one month demonstrated significant improvement.

The diagnostic value of Ki-67 and repp86 in distinguishing between benign and malignant mesothelial proliferations.

CONTEXT: The differentiation of benign mesothelial proliferations from malignant mesotheliomas may be difficult, especially when evaluating small specimens from pleural biopsies. OBJECTIVE: To explore the potential value of 2 proliferative cell markers, Ki-67 and restrictedly expressed proliferation-associated protein 86 kDa (repp86), in distinguishing between malignant mesothelioma (MM) and benign reactive mesothelial hyperplasia (MH). DESIGN: Thirty-six cases of MM from 26 men and 10 women with a mean age of 62.9 years (range, 36-80 years) and 22 cases of benign reactive MH from 14 male and 8 female patients with a mean age of 51.5 years (range, 15- 88 years) were included in this study. The proliferative status of the lesions was assessed by immunohistochemistry using monoclonal antibodies to Ki-S2 (repp86) and Ki-S5 (Ki-67). The labeling indices were quantified. RESULTS: The mean labeling indexes for Ki-67 in MM and benign reactive MH were 24.6% (range, 1%-66%) and 6.23% (range, 0%-25%), respectively. The mean labeling indexes for repp86 in MM and benign reactive MH were 26.3% (range, 0%-50%) and 3.26% (range, 0%- 21%), respectively. The average proliferative cell count was significantly higher in MM compared with benign reactive MH (P < .001). Furthermore, both markers showed a significant correlation in their expression in MM and benign reactive MH (r = 77.5, P < .001). Sensitivities of 88% and 92% and specificities of 92% and 94% were obtained at a cutoff point of 9% for Ki-67 and repp86, respectively. CONCLUSIONS: Used in combination, Ki-67 and repp86 appear to be useful markers in differentiating MM from benign reactive MH.

Arylamine N-acetyltransferase 2 slow acetylator polymorphisms in unrelated Iranian individuals.

OBJECTIVE: To determine the frequency of mutations at the polymorphic gene coding for arylamine N-acetyltransferase 2 (NAT2, EC 2.3.1.5) and NAT2 genotypes associated with slow acetylation in healthy Iranian individuals. METHODS: The polymorphisms in the NAT2 gene from 88 unrelated healthy subjects (48 men/40 women) from the general Tehran population were discriminated using polymerase chain reaction (PCR) with allele-specific primers (341 C > T) and PCR-restriction fragment length polymorphism analysis (481 C > T, 590 G > A, and 857 G > A). RESULTS: Frequencies of the studied polymorphisms showed the most common alleles to be NAT2*4 (0.43) and NAT2*5, 481 C > T (0.32), followed by NAT2*6 (0.19) and NAT2*7 (0.06), previously referred to as WT, M1, M2, and M3, respectively. The most prevalent genotypes were NAT2*4/*5 [(31.8%; 95% confidence interval (CI): 29-34%] and *4/*4 (18.2%; 95% CI: 16-21%). When grouped according to the expected phenotypical effects, the resulting genotypes revealed the significant prevalence of the subjects with slow (32.9%) and intermediate (48.9%) acetylation status compared with wild-type rapid (18.2%) acetylators (P < 0.01). CONCLUSIONS: The overall allele pattern and acetylator status distribution in Iranians displayed the considerable prevalence of "slow acetylators" over "rapid acetylators," similar to those of Caucasians except for a minor difference observed in the frequency of the NAT2*7 allele. Nucleic acid testing for common NAT2 mutations might be a potentially useful tool for an accurate phenotype interpretation and identification of Iranian individuals at risk.