Infantile Rosai-Dorfman Disease With Isolated Brain Lesions Disseminated to the Parenchyma and Intraventricular Ependyma, Alteration of Leukocytes as a Promotion Factor in Immune Defense, and New Proposals: A Case Report and Literature Review.

The patient is a one-year-old girl referred to the hospital for an enlarged head after a 1.5-month history of two falls, followed by polydipsia, polyuria, and slow movement and growth. Three subsequent magnetic resonance imaging (MRI) examinations of the brain revealed nodular lesions disseminated in the brain parenchyma and intraventricular ependyma, resulting in obstructive hydrocephalus. Thoracic and abdominopelvic sonography showed no additional lesions. The preliminary diagnosis was a primary or metastatic neoplasm or infection. A biopsy of a lesion in the right frontal lobe was taken. The histological examination revealed features of Rosai-Dorfman disease (RDD), consisting of limited perivascular lymphoplasma cell infiltration with intervening sheets of proliferated histiocytes, with some of the histiocytes showing endocytosis of a single intact lymphocyte (emperipolesis).

Clavicular Chondrosarcoma: A Case Report and Brief Review of the Literature.

Clavicular bone tumors occur in less than 0.5 percent of bone tumors. Primary chondrosarcoma is very rare even among clavicle tumors. The main symptom is a touchable mass in 69 % of patients. Dedicated centers using FNA and cytology can reach a correct diagnosis in 94% of cases. Treatment planning is done using simple X-ray, CT-scan, shoulder MRI, chest CT-scan and whole body technetium scan. Treatment of choice for primary chondrosarcoma of clavicle is surgical resection.

The therapeutic effects of MSc1 nanocomplex, synthesized by nanochelating technology, on experimental autoimmune encephalomyelitic C57/BL6 mice.

PURPOSE: Currently approved therapies for multiple sclerosis (MS) at best only slow down its progression. Therefore, it is necessary to utilize novel technologies in order to synthesize smart multifunctional structures. In the present study, for the first time we evaluated the therapeutic potential of MSc1 nanocomplex, which was designed based on novel nanochelating technology. MATERIALS AND METHODS: MSc1 cell-protection capacity, with and without iron bond, was evaluated against hydrogen peroxide (H2O2)-induced oxidative stress in cultured rat pheochromocytoma-12 cells. The ability of MSc1 to maintain iron bond at pH ranges of 1-7 was evaluated. Nanocomplex toxicity was examined by estimating the intraperitoneal median lethal dose (LD50). Experimental autoimmune encephalomyelitic mice were injected with MSc1 14 days after disease induction, when the clinical symptoms appeared. The clinical score, body weight, and disease-induced mortality were monitored until day 54. In the end, after collecting blood samples for assessing hemoglobin and red blood cell count, the brains and livers of the mice were isolated for hematoxylin and eosin staining and analysis of iron content, respectively. RESULTS: The results showed that MSc1 prevented H2O2-induced cell death even after binding with iron, and it preserved its bond with iron constant at pH ranges 1-7. The nanocomplex intraperitoneal LD50 was 1,776.59 mg/kg. MSc1 prompted therapeutic behavior and improved the disabling features of experimental autoimmune encephalomyelitis, which was confirmed by decreased clinical scores versus increased body mass and 100% survival probability. It did not cause any adverse effects on hemoglobin or red blood cell count. Histopathological studies showed no neural loss or lymphocyte infiltration in MSc1-treated mice, while the hepatic iron content was also normal. CONCLUSION: These results demonstrate that MSc1 could be a promising beneficial novel agent and has the capacity to be evaluated in further studies.

Accurate cut-off point for free to total prostate-specific antigen ratio used to improve differentiation of prostate cancer from benign prostate hyperplasia in Iranian population.

PURPOSE: Our aim was to determine a more predictive cut-off value for free to total prostate-specific antigen ratio (f/tPSA) to better differentiate prostate cancer (PCa) from benign prostate hyperplasia (BPH) in Iranian patients with serum PSA levels between 4 and 20 ng/mL. MATERIALS AND METHODS: This study was performed on 332 men with serum tPSA level of 4 to 20 ng/mL. All patients underwent transrectal ultrasound guided biopsies. Serum levels of tPSA and fPSA were measured by Roche immunoassay Elecsys 2010. Relationship between f/tPSA and cases of PCa was determined. RESULTS: Prostate cancer detected in 49 (15%) patients. Incidence of PCa for serum tPSA level < 10 ng/mL and serum tPSA level of 10.1 to 20 ng/ mL was 17 (6.7%) and 32 (39.5%), respectively. Mean f/tPSA value was significantly lower in PCa patients (0.12 +/- 0.01) than in benign histology group (0.16 +/- 0.03). Among patients with serum PSA level of 4 to 10 ng/mL (n = 251), mean f/tPSA in benign histology group (n = 234) was 0.16 +/- 0.08 and in PCa group (n = 17) was 0.13 +/- 0.06 (P < .05). For serum PSA level of 10.1 to 20 ng/mL (n = 81), mean f/tPSA in benign histology group (n = 49) was 0.16 +/- 0.08 and in PCa group (n = 32) was 0.12 +/- 0.05 (P < .05). The cut-off value of 0.12 produced 76% sensitivity and 71% specificity, whereas the cut-off value of 0.14 yielded 83.5% sensitivity and 61% specificity. CONCLUSION: Determination of f/tPSA ratio improves differentiation of Pca from BPH. We recommend a cut-off value of 0.14 to be applied to Iranian patients.

Distribution of solitary parathyroid adenoma over the parathyroid glands and its surgical management.

OBJECTIVE: To determine the distribution of solitary parathyroid adenoma over the parathyroid glands in a group of patients with primary hyperparathyroidism Design: A case-series. PLACE AND DURATION OF STUDY: Departments of Surgery and Pathology at Shariati Hospital, Tehran, Iran, between the years 1981 and 2003. PATIENTS AND METHODS: A retrospective review of surgery reports of 118 patients with primary hyperparathyroidism was performed. All of the patients had solitary parathyroid adenoma and the anatomical location of each adenoma was clearly defined during operation. Serum calcium, phosphorus and parathormone levels along with clinical characteristics were also included. RESULTS: The distribution of adenomas over the superior and inferior parathyroid glands showed a significant higher incidence of adenoma in the lower parathyroids (p < 0.001). The right to left distribution of adenomas was not significant (p=0.4). CONCLUSION: Surgical exploration for primary hyperparathyroidism should be initiated from the lower parathyroid glands provided that pre-operative localization scans are not helpful.

Are biochemical values predictive of adenoma's weight in primary hyperparathyroidism?

BACKGROUND: Preoperative laboratory findings may carry some predictive value about the size of the abnormal parathyroid tissue that needs to be removed in primary hyperparathyroidism. METHODS: In a retrospective study from 1988 to 2003, records of 71 patients with parathyroid adenoma were reviewed. The correlation between preoperative serum calcium, phosphate and parathyroid hormone (PTH) with adenoma's weight was analysed separately. RESULTS: There was a significant correlation between preoperative serum PTH and calcium with adenoma's weight (P < 0.001 and P = 0.03, respectively). The correlation between preoperative serum phosphate and adenoma's weight was not significant (P = 0.1). CONCLUSION: Preoperative PTH level cannot be used as a definite guide to the parathyroid adenoma's weight. Large parathyroid adenomas seem to secrete less PTH per unit weight than small adenomas. Calcium and phosphate do not seem to be of much value in predicting adenoma's weight in primary hyperparathyroidism.

Primary hyperparathyroidism: a review of 177 cases.

BACKGROUND: We designed a comprehensive study to explore different aspects of primary hyperparathyroidism in a series of Iranian patients. Our study aimed to elucidate the demographic, pathologic, biochemical, and surgical findings of these patients. MATERIAL/METHODS: In a retrospective study from 1981 to 2003, we reviewed records of 177 patients operated on with the diagnosis of primary hyperparathyroidism in Shariati Hospital. Patients with secondary or tertiary hyperparathyroidism and those being re-operated were excluded. RESULTS: 88.1% of the lesions were adenoma, 9.6% hyperplasia, and 1 case proved to be carcinoma. In 1.7% of patients, no pathologic gland(s) were found. The majority of patients were female (female-to-male ratio: 5.5) and their mean age was 41.9+/-13.6 years. Most of the patients (77.6%) had mild hypercalcemia, and the mean calcium level was 11.2+/-0.8 mg/dl prior to surgery. Patients with hyperplasia had lower calcium levels than patients with adenoma 24 hours post-operation, with mean calcium levels of 8.1+/-1.2 mg/dl versus 9.03+/-1.2 mg/dl, respectively (p=0.02). Mean weight of adenomas was 4.1 gram (range 0.8-25 g). Adenomas were mostly (74%) located in lower parathyroid glands and 6.3% of them were ectopic. CONCLUSIONS: Primary hyperparathyroidism should be expected at younger ages in Iran. Parathyroid hyperplasia in a patient should alert the surgeon of the higher risk of postoperative hypocalcemia.

Concomitant keratoconus and macular corneal dystrophy.

OBJECTIVE: To report the concomitant presentation of keratoconus and macular corneal dystrophy in two cases. METHODS: Two siblings with concomitant keratoconus and macular corneal dystrophy are presented. Complete ophthalmologic examination and computerized videokeratography were performed in both patients. Case 1 underwent penetrating keratoplasty in both eyes sequentially; histopathologic examination of excised corneal buttons with special stains was performed. Case 2 was observed. RESULTS: Both patients manifested typical signs and topographic evidence of keratoconus associated with macular dystrophy. Histopathologic studies in case 1 confirmed the same clinical diagnosis. CONCLUSION: To our knowledge, this is the first report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic link between these 2 entities.