Background It is widely recognized that the diagnosis of parathyroid carcinoma （PC） is often difficult because of the overlap of characteristics between malignant and benign parathyroid tumors, especially at an earl...Background It is widely recognized that the diagnosis of parathyroid carcinoma （PC） is often difficult because of the overlap of characteristics between malignant and benign parathyroid tumors, especially at an early stage. Our study aimed to investigate the differential expression of Ki-67, galectin-3, fragile histidine triad （FHIT） gene, and parafibromin in PC, parathyroid adenoma （PA）, parathyroid hyperplasia （PH）, and normal parathyroid （NP） tissues; then to assess these expression values for use in differential diagnosis of malignant and benign parathyroid tumors. Methods Data of 15 cases with PC, 19 PAs, and 8 PHs were retrospectively analyzed for their clinical characteristics. The expression of Ki-67, galectin-3, FHIT, and parafibromin were detected via immunohistochemistry in the above-mentioned specimens and 6 NPs as control. Results Complete loss of parafibromin expression was seen in 9 of 15 （60%） carcinomas, and all normal parathyroid tissues and parathyroid benign tumors stained positive for parafibromin except for one （4%） adenoma. Galectin-3 staining was positive in 11 of 15 （73%） carcinomas, 5 of 19 （26%） adenomas, 1 of 8 （12%） hyperplasias, and 0 of 6 normal tissues. The Ki-67 proliferative index was high in 4 of 15 （27%） carcinomas, 1 of 19 （5%） adenomas, and none of the hyperplasia or normal tissues. FHIT expression did not differ appreciably among the tumor types. The combination of overexpression of galectin-3 or loss of parafibromin increased sensitivity for PC to 87%, while the specificity of both positive galectin-3 and positive Ki-67 could reach 100%. Conclusions These data suggested that loss of parafibromin and overexpression of galectin-3 and Ki-67 might help to distinguish parathyroid carcinoma from other parathyroid tumors. And the combination of two or three of these markers might produce better sensitivity and/or specificity for the diagnosis of parathyroid carcinoma.展开更多
Background Osteogenesis imperfecta （OI） is a rare bone disease and its effective treatment is relatively deficient. We tried to assess the effects of new bisphosphonate, ibandronate on children with OI.Methods In th...Background Osteogenesis imperfecta （OI） is a rare bone disease and its effective treatment is relatively deficient. We tried to assess the effects of new bisphosphonate, ibandronate on children with OI.Methods In this open-label, prospective, controlled study, 30 children with OI were enrolled. They received either infusions of ibandronate （2 mg） in every three months or oral calcitriol 0.25 μg daily for 24 months. All patients took 500 mg calcium plus 200 U vitamin D daily together. The endpoints were the change of annual new fracture rate （observed by case history and X ray films of spine）, bone mineral density （BMD, measured by dual energy X-ray absortiometry）, serum concentration of carboxy-telopeptide cross-links of type Ⅰ collagen （CTX, bone resorption marker） and alkaline phosphatase （ALP, bone formation marker） during the follow-up.Results After the cyclic infusions of ibandronate, the annual new fracture rate was significantly decreased from 1.9 to 0.13 time, obviously lower than that of calcitriol group, which decreased from 1.8 to 1.0 time after the treatment （P 〈0.001）.The significant increase of BMD at the lumbar spine, femoral neck, trochanter, total hip was found in the group of ibandronate by 59.0%, 42.0%, 47.5% and 36.6% in time dependent manner （compared with the baseline, P 〈0.001）. The increase of BMD in ibandronate group was greater than that of calcitriol group （P 〈0.001）. The concentrations of ALP and CTX were obviously decreased in ibandronate group, and the reduction of CTX was more significant than that of ALP （P 〈0.001）. The tolerance of the children to ibandronate was quite well. Mild fever and muscle pain were found in 9 cases within 1-3 days after the first infusion of ibandronate, which could relieve after 1-2 days without special management.Conclusions The benefits of cyclic infusions of ibandronate to children with OI are significant because ibandronate could significantly reduce annual bone fracture rate, increase lumbar and hip BMD, preserve vertebral morphometry of patients through inhibition of bone resorption.展开更多
Hypercalcemic crisis, generally accepted as serum calcium concentration greater than 3.5 mmol/L,constitues a life-threatening endocrinologic emergency,and is most frequently caused by either primary hyperparathyroidi...Hypercalcemic crisis, generally accepted as serum calcium concentration greater than 3.5 mmol/L,constitues a life-threatening endocrinologic emergency,and is most frequently caused by either primary hyperparathyroidism （PHPT） or malignant diseases.Parathyroid cysts are uncommon lesions, most of that are located in the low part of the neck. By routine neck ultrasound scan investigation in a large series of 6621 patients, only 5 parathyroid cysts were detected, yielding a prevalence of 0.075% in setting of unselected patients.The parathyroid cysts in the mediastinum are much less frequently encountered, with only 106 cases reported in English literature.2,3 Moreover, less than half of these cases presented as functional with elevated serum calcium and parathyroid hormone, and only 10 cases were associated with hypercalcemic crisis.3 Herein, we present a rare case of mediastinal parathyroid cyst associated with recurrent hypercalcemic crisis, which diagnosed by ultrasound-guided fine needle aspiration （FNA）.展开更多
Background Fibroblast growth factor 9 （FGF9）, expressed in brain, kidney and developing skeletal tissues, can physiologically inhibit endochondral ossification; but little is known about how FGF9 affects osteoblasts...Background Fibroblast growth factor 9 （FGF9）, expressed in brain, kidney and developing skeletal tissues, can physiologically inhibit endochondral ossification; but little is known about how FGF9 affects osteoblasts and its detailed regulatory mechanism. Here we examined the effect of FGF9 on the activity of the murine Runt-related transcription factor2 （Runx2） gene promoter in preosteoblast MC3T3-E1 and premyoblast C2C12 cells. Methods Plasmids containing the Runx2 promoter region were transfected into MC3T3-E1 and C2C12 cells and stably transfected cell lines were established. The method of luciferase reporter gene activation was used to examine the effects of FGF9 on the promoter activity. Results FGF9 （10 ng/ml） increased Runx2 promoter activity in MC3T3-E1 cells. When MC3T3-E1 cells were treated with FGF9 plus the various inhibitors or activator of the intracellular signaling transducation pathways, including 10 μmol/L U0126 （the inhibitor of mitogen-activated protein kinase kinase）, 10 pmol/L SB203580 （the inhibitor of p38/mitogen activated protein kinase）, or 1 pmol/L C6 ceramide （an activator of mitogen activated protein kinase）, the luciferase expression did not change significantly compared with that of the cells treated with FGF9 only. However, when C2C12 cells were treated with 10 ng/ml FGF9, Runx2. gene promoter activity first decreased and then increased over a period of 1 to 5 days. Among the above inhibitors, only U0126 （10 μmol/L） completely blocked the effects of FGF9 on Runx2 gene promoter activity. Conclusions Our data showed that FGF9 can affect Runx2 gene promoter activity in MC3T3-E1 and C2C12 cells. The action of FGF9 appears to depend partly on the mitogen-activated protein kinase kinase/mitogen-activated protein kinase pathways in C2C12 cells.展开更多
Primary Intestinal lymphangiectasia （PIL） is a common cause of protein losing enteropathy （PLE）. It will affect enter-hepatic circulation of lipid-soluble vitamin, and absorption of electrolytes, cause malnutritio...Primary Intestinal lymphangiectasia （PIL） is a common cause of protein losing enteropathy （PLE）. It will affect enter-hepatic circulation of lipid-soluble vitamin, and absorption of electrolytes, cause malnutrition related osteomalacia or osteoporosis. While seldom health care workers noted to assess and treat osteomalacia or osteoporosis in PIL. Here we report a related case. We found increased parathyroid hormone, decreased 25（OH）D3, low bone mineral density, which indicated that the PIL patient had osteomalacia and/or osteoporosis. Adequate calcium and vitamin D supply can relieve the condition efficaciously. We should pay attention to osteomalacia and osteoporosis in PIL patients.展开更多
基金WANG Ou and WANG Chun-yan contributed equally to this work. This work was partly supported by the grant from the Project of National Natural Science Foundation of China （No. 81100559）. Conflict of interests： The authors declare that they have no conflict of interests.
文摘Background It is widely recognized that the diagnosis of parathyroid carcinoma （PC） is often difficult because of the overlap of characteristics between malignant and benign parathyroid tumors, especially at an early stage. Our study aimed to investigate the differential expression of Ki-67, galectin-3, fragile histidine triad （FHIT） gene, and parafibromin in PC, parathyroid adenoma （PA）, parathyroid hyperplasia （PH）, and normal parathyroid （NP） tissues; then to assess these expression values for use in differential diagnosis of malignant and benign parathyroid tumors. Methods Data of 15 cases with PC, 19 PAs, and 8 PHs were retrospectively analyzed for their clinical characteristics. The expression of Ki-67, galectin-3, FHIT, and parafibromin were detected via immunohistochemistry in the above-mentioned specimens and 6 NPs as control. Results Complete loss of parafibromin expression was seen in 9 of 15 （60%） carcinomas, and all normal parathyroid tissues and parathyroid benign tumors stained positive for parafibromin except for one （4%） adenoma. Galectin-3 staining was positive in 11 of 15 （73%） carcinomas, 5 of 19 （26%） adenomas, 1 of 8 （12%） hyperplasias, and 0 of 6 normal tissues. The Ki-67 proliferative index was high in 4 of 15 （27%） carcinomas, 1 of 19 （5%） adenomas, and none of the hyperplasia or normal tissues. FHIT expression did not differ appreciably among the tumor types. The combination of overexpression of galectin-3 or loss of parafibromin increased sensitivity for PC to 87%, while the specificity of both positive galectin-3 and positive Ki-67 could reach 100%. Conclusions These data suggested that loss of parafibromin and overexpression of galectin-3 and Ki-67 might help to distinguish parathyroid carcinoma from other parathyroid tumors. And the combination of two or three of these markers might produce better sensitivity and/or specificity for the diagnosis of parathyroid carcinoma.
文摘Background Osteogenesis imperfecta （OI） is a rare bone disease and its effective treatment is relatively deficient. We tried to assess the effects of new bisphosphonate, ibandronate on children with OI.Methods In this open-label, prospective, controlled study, 30 children with OI were enrolled. They received either infusions of ibandronate （2 mg） in every three months or oral calcitriol 0.25 μg daily for 24 months. All patients took 500 mg calcium plus 200 U vitamin D daily together. The endpoints were the change of annual new fracture rate （observed by case history and X ray films of spine）, bone mineral density （BMD, measured by dual energy X-ray absortiometry）, serum concentration of carboxy-telopeptide cross-links of type Ⅰ collagen （CTX, bone resorption marker） and alkaline phosphatase （ALP, bone formation marker） during the follow-up.Results After the cyclic infusions of ibandronate, the annual new fracture rate was significantly decreased from 1.9 to 0.13 time, obviously lower than that of calcitriol group, which decreased from 1.8 to 1.0 time after the treatment （P 〈0.001）.The significant increase of BMD at the lumbar spine, femoral neck, trochanter, total hip was found in the group of ibandronate by 59.0%, 42.0%, 47.5% and 36.6% in time dependent manner （compared with the baseline, P 〈0.001）. The increase of BMD in ibandronate group was greater than that of calcitriol group （P 〈0.001）. The concentrations of ALP and CTX were obviously decreased in ibandronate group, and the reduction of CTX was more significant than that of ALP （P 〈0.001）. The tolerance of the children to ibandronate was quite well. Mild fever and muscle pain were found in 9 cases within 1-3 days after the first infusion of ibandronate, which could relieve after 1-2 days without special management.Conclusions The benefits of cyclic infusions of ibandronate to children with OI are significant because ibandronate could significantly reduce annual bone fracture rate, increase lumbar and hip BMD, preserve vertebral morphometry of patients through inhibition of bone resorption.
文摘Hypercalcemic crisis, generally accepted as serum calcium concentration greater than 3.5 mmol/L,constitues a life-threatening endocrinologic emergency,and is most frequently caused by either primary hyperparathyroidism （PHPT） or malignant diseases.Parathyroid cysts are uncommon lesions, most of that are located in the low part of the neck. By routine neck ultrasound scan investigation in a large series of 6621 patients, only 5 parathyroid cysts were detected, yielding a prevalence of 0.075% in setting of unselected patients.The parathyroid cysts in the mediastinum are much less frequently encountered, with only 106 cases reported in English literature.2,3 Moreover, less than half of these cases presented as functional with elevated serum calcium and parathyroid hormone, and only 10 cases were associated with hypercalcemic crisis.3 Herein, we present a rare case of mediastinal parathyroid cyst associated with recurrent hypercalcemic crisis, which diagnosed by ultrasound-guided fine needle aspiration （FNA）.
文摘Background Fibroblast growth factor 9 （FGF9）, expressed in brain, kidney and developing skeletal tissues, can physiologically inhibit endochondral ossification; but little is known about how FGF9 affects osteoblasts and its detailed regulatory mechanism. Here we examined the effect of FGF9 on the activity of the murine Runt-related transcription factor2 （Runx2） gene promoter in preosteoblast MC3T3-E1 and premyoblast C2C12 cells. Methods Plasmids containing the Runx2 promoter region were transfected into MC3T3-E1 and C2C12 cells and stably transfected cell lines were established. The method of luciferase reporter gene activation was used to examine the effects of FGF9 on the promoter activity. Results FGF9 （10 ng/ml） increased Runx2 promoter activity in MC3T3-E1 cells. When MC3T3-E1 cells were treated with FGF9 plus the various inhibitors or activator of the intracellular signaling transducation pathways, including 10 μmol/L U0126 （the inhibitor of mitogen-activated protein kinase kinase）, 10 pmol/L SB203580 （the inhibitor of p38/mitogen activated protein kinase）, or 1 pmol/L C6 ceramide （an activator of mitogen activated protein kinase）, the luciferase expression did not change significantly compared with that of the cells treated with FGF9 only. However, when C2C12 cells were treated with 10 ng/ml FGF9, Runx2. gene promoter activity first decreased and then increased over a period of 1 to 5 days. Among the above inhibitors, only U0126 （10 μmol/L） completely blocked the effects of FGF9 on Runx2 gene promoter activity. Conclusions Our data showed that FGF9 can affect Runx2 gene promoter activity in MC3T3-E1 and C2C12 cells. The action of FGF9 appears to depend partly on the mitogen-activated protein kinase kinase/mitogen-activated protein kinase pathways in C2C12 cells.
文摘Primary Intestinal lymphangiectasia （PIL） is a common cause of protein losing enteropathy （PLE）. It will affect enter-hepatic circulation of lipid-soluble vitamin, and absorption of electrolytes, cause malnutrition related osteomalacia or osteoporosis. While seldom health care workers noted to assess and treat osteomalacia or osteoporosis in PIL. Here we report a related case. We found increased parathyroid hormone, decreased 25（OH）D3, low bone mineral density, which indicated that the PIL patient had osteomalacia and/or osteoporosis. Adequate calcium and vitamin D supply can relieve the condition efficaciously. We should pay attention to osteomalacia and osteoporosis in PIL patients.
