Performance of existing diagnosis/classification criteria for Behcet's disease in Iranian patients: analysis of 5666 patients and 2406 controls

Aim: To analyse the performance of existing diagnosis/classification criteria for Behcet's disease (BD) in Iranian patients. There are 13 sets: Curth (1946), Hewitt (1969), Mason and Barnes (1969), Hewitt revised (1971), Japan (1972), Hubault and Hamza (1974), O'Duffy (1974), Cheng and Zhang (1980), Dilsen (1986), Japan revised (1988), International (1990), Iran (1993), Classification Tree (1993), and Dilsen revised criteria (2000). Methods: All patients from the Behcet's Disease Registry (5666) and control patients (2406) entered the study. Sensitivity, specificity and accuracy were calculated. Results: The most sensitive was Curth criteria with (99.7%), followed by Classification Tree (97.3%), Zhang (93.5%), Iran (91.4%), Japan revised (86.4%), Japan (85.3%), Dilsen (83.7%), Hubault and Hamza (81.6%), Dilsen revised (81.2%), International criteria (79.8%), Hewitt (73.8%), O'Duffy (70.7%), and Masson and Barnes (65.7%). The most specific was Masson and Barnes (99.6%), followed by the International criteria (98.3%), Dilsen revised (98.2%), O'Duffy (97.6%), Japan (97.1%), Japan revised (97%), Classification Tree (96.7%), Hewitt (95.8%), Iran (95.8%), Zhang (92.4%), Dilsen (91.4%), Hubault (90.8%), and Curth (78.6%). The most accurate criteria was Classification Tree (97.1%), followed by Curth (93.4%), Zhang (93.1%), Iran (92.7%), Japan revised (89.6%), Japan (88.8%), Dilsen revised (86.2%), Dilsen (86%), International criteria (85.3%), Hubault (84.3%), Hewitt (80.4%), O'Duffy (78.8%), and Mason and Barnes (75.8%). Discussion: Among the existing criteria, the best to classify Iranian patients is the Classification Tree. The second most accurate is Curth criteria. The difference is statistically significant. Further, Curth criteria is not optimized, having very high sensitivity and low specificity.     

Fibromyxoma in the reticulo-omasal orifice of a cow

A 3-year-old female Holstein cow was referred for necropsy examination with the history of inappetence, progressive indigestion, distension of the rumen and death. At necropsy examination, a lobulated grey-white mass (12?×?8?×?5 cm) was found close to the reticulo-omasal orifice. On sectioning, the tumour mass was gelatinous with intervening fibrous septa. Microscopical examination revealed unencapsulated mass composed of various cellular parts and matrices. There were proliferated spindle shaped fibrocytes producing repetitive collagenous fibres. There were also foci of low cellularity containing stellate cells with small and hyperchromatic nuclei arranged in a mucinous ground substance. On the basis of the gross and histopathological findings and histochemical stainings, the tumour was diagnosed as a fibromyxoma. This tumour has not been recorded previously in the reticulo-omasal orifice of a cow.     

Evaluation of Bone Mineral Status in Adolescent Idiopathic Scoliosis

Background

Several reports have suggested low bone mineral density (BMD) in patients with adolescent idiopathic scoliosis (AIS). We determined bone mineral status in patients with AIS to evaluate the effect of brace treatment on BMD.

Methods

BMD was measured in 46 patients (mean age, 17.8 ± 4.9 years) with AIS (17 with brace and 29 without brace) by dual-energy X-ray absorptiometry scan and compared the results to an age-matched (mean age, 16.6 ± 3.9 years) control group (n = 54).

Results

The AIS group had significantly lower bone mass at the lumbar spine (Z-score, -1.500 vs. -0.832) and hip (Z-score, -1.221 vs. -0.754) except at the femoral neck. No difference in BMD was found between patients with AIS who used a brace and those who did not.

Conclusions

The results confirmed that BMD was low in AIS patients and it was not affected by brace treatment.     

Choroidal plasmacytoma in a patient with multiple myeloma. Diagnosis by fine-needle aspiration biopsy

BACKGROUND: To report a case of a secondary plasmacytoma of the choroid. METHODS: Interventional case report with cytopathologic correlation. RESULTS: A 61-year-old male with a breast nodule and a 2-year history of multiple myeloma developed blurred vision and was found to have three confluent, ill-defined areas of choroidal thickening in the left eye. Cytopathology and immunohistochemistry of a transocular fine-needle aspiration biopsy of the largest lesion revealed atypical plasma cells diagnostic of plasmacytoma. Following external beam radiotherapy the choroidal tumors resolved completely. CONCLUSION: Secondary plasmacytoma, despite its rarity, should be included in the differential diagnosis of amelanotic choroidal tumors, particularly in patients with multiple myeloma. Radiotherapy is an effective treatment.     

A comparison of nesiritide vs. epoprostenol in a patient with precapillary pulmonary hypertension due to scleroderma complicated by postcapillary pulmonary hypertension

To the best of our knowledge, acute decompensated left-sided heart failure with preserved left ventricular ejection fraction in a patient with scleroderma has not been previously reported. We describe a patient with severe pulmonary hypertension due to limited scleroderma in whom nesiritide led to marked reductions in pulmonary arterial and capillary wedge pressure as well as resolution of symptoms and pulmonary edema. Subsequent epoprostenol use was associated with an increase in pulmonary capillary wedge pressure and a recurrence of pulmonary edema. Thus, nesiritide may be the preferred agent in scleroderma patients with severe pulmonary hypertension and preserved left ventricular systolic function since epoprostenol may lead to adverse hemodynamic effects.     

Tumor cell-specific BRCA1 and RASSF1A hypermethylation in serum, plasma, and peritoneal fluid from ovarian cancer patients

Because existing surgical and management methods can consistently cure only early-stage ovarian cancer, novel strategies for early detection are required. Silencing of tumor suppressor genes such as p16INK4a, VHL, and hMLH1 have established promoter hypermethylation as a common mechanism for tumor suppressor inactivation in human cancer and as a promising target for molecular detection in bodily fluids. Using sensitive methylation-specific PCR, we screened matched tumor, preoperative serum or plasma, and peritoneal fluid (washes or ascites) DNA obtained from 50 patients with ovarian or primary peritoneal tumors for hypermethylation status of the normally unmethylated BRCA1 and RAS association domain family protein 1A tumor suppressor genes. Hypermethylation of one or both genes was found in 34 tumor DNA (68%). Additional examination of one or more of the adenomatous polyposis coli, p14ARF, p16INK4a, or death associated protein-kinase tumor suppressor genes revealed hypermethylation in each of the remaining 16 tumor DNA, which extended diagnostic coverage to 100%. Hypermethylation was observed in all histologic cell types, grades, and stages of ovarian tumor examined. An identical pattern of gene hypermethylation was found in the matched serum DNA from 41 of 50 patients (82% sensitivity), including 13 of 17 cases of stage I disease. Hypermethylation was detected in 28 of 30 peritoneal fluid DNA from stage IC-IV patients, including 3 cases with negative or atypical cytology. In contrast, no hypermethylation was observed in nonneoplastic tissue, peritoneal fluid, or serum from 40 control women (100% specificity). We conclude that promoter hypermethylation is a common and relatively early event in ovarian tumorigenesis that can be detected in the serum DNA from patients with ovary-confined (stage IA or B) tumors and in cytologically negative peritoneal fluid. Analysis of tumor-specific hypermethylation in serum DNA may enhance early detection of ovarian cancer.