Objective To analyze the death causes of of dermatomyositis(DM)and polymyositis(PM)patients with interstitial pneumonia.Methods A retrospectively analysis of the clinical and laboratory characteristics of 15 dead DM and PM patients with interstitial pneumonia was carried out,and 23 survivors served as controls.Results The age of the dead group was(50.1?12.0)years old,and 12(80%)patients died within the six months after onset.The mortality was higher in the acute or subacute interstitial pneumonia patients than that in the chronic patients.With longer course of the disease,lung infections became the major cause of death.Significantly higher serum lactate dehydrogenase,glutamate- pyruvate transaminase,glutamic-oxalacetic transaminase levels were found in the dead group.The dead group had higher incidence of dyspnea and“Velcro”-like crackles and louer arterial oxygen pressure.The elevation of lactate dehydrogenase indicated worse prognosis.The elevation of creatine kinase and anti Jo-1 antibody werent the death causes. Conclusion The early onset of interstitial lung disease and lung infection were the major death causes in dermatomyositis and polymyositis patients.The earlier treatment of pneumonia-may improve the prognosis and reduce mortality.

OBJECTIVE: The large sample retrospective cohort study were used to compare the diagnostic efficiency of PET/CT with conventional work-up (CWU) for evaluating nasopharyngeal carcinoma (NPC) distant metastasis. METHOD: Five hundred and fourteen patients with NPC were divided into PET/CT group and CWU group according the method of detecting distant metastasis. Chest film, abdominal ultrasonography, and bone scan were used in CWU group. Then the diagnostic efficiency of the two groups was compared. RESULT: Two hundred and sixteen patints were enrolled in PET/CT group and two hundred and nineteen-eight ones in CWU group. There were 28 out of 412 suspicious patients in CWU group were confirmed, another 3 patients confirmed without positive findings, compared with PET/CT group that all 32 suspicious patients were confirmed. The sensitivity and specificity of PET/CT were 100.0% (32/32) and 100.0% (184/184), as compared to 90.3% (28/31) and 94.8% (253/267) with CWU respectively, while there was no statistical significance. Further research found out that the percentage of patients with multiple distant metastatic sites and multiple organ metastases was higher in PET/CT group (P < 0.05), and similarly of patients with distant metastasis in N2-3 stages (P < 0.01). CONCLUSION Our results suggest that PET/CT appears to be slightly superior to conventional work-up in assessment of distant metastasis in NPC patients, but CWU is still a cheap and practical method.
Aged ; Carcinoma ; Cohort Studies ; Female ; Fluorodeoxyglucose F18 ; Humans ; Male ; Middle Aged ; Nasopharyngeal Carcinoma ; Nasopharyngeal Neoplasms ; diagnosis ; secondary ; Positron-Emission Tomography ; methods ; Radiopharmaceuticals ; Retrospective Studies ; Sensitivity and Specificity ; Tomography, X-Ray Computed ; Ultrasonography

OBJECTIVE: To explore the pathological characteristics and significance of RET proto-oncogene screening in multiple endocrine neoplasia type 2A (MEN2A) with cutaneous lichen amyloidosis (CLA). METHODS: Clinical data of 51 members from 7 unrelated pedigrees of MEN2A-CLA were collected. Systemic clinical investigations including biochemical testing, imaging examination, germline RET variant screening and histopathological examination were carried out. RESULTS: RET gene variants were detected in 28 patients with MEN2A (C634G/F/R/S/W and C611Y) including 12 males and 16 females, with the mean age of diagnosis being (41.1 ± 18.3) years old, which were consistent with their clinical manifestations. The incidence of medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), hyperparathyroidism (HPTH) and CLA among 28 MEN2A patients were 89.3%, 28.6%, 7.1% and 28.6%, respectively. Comparison of the incidence of MTC/PHEO/HPTH and CLA between C611Y and C634G/F/R/S/W, only PHEO and CLA in C611Y were lower than those in C634G/F/R/S/W (P < 0.05; P < 0.05). Among 8 patients with CLA, the male to female ratio was 2 : 6. The clinical features included pruritus in the interscapular region and presence of dry, thickened, scaly, brown pigment, clustered or desquamate-like plaques. The mean onset age of CLA [(18.4 ± 4.6) years] versus the mean age at diagnosis of CLA or MEN2A were significantly different (P < 0.001; P < 0.001). CONCLUSION MEN2A-CLA may be the early clinical manifestation of MEN2A and most frequently occurred along with RET-C634 variant. To facilitate the recognition of MEN2A-CLA, to combine family investigation and screening of RET variant are helpful for early diagnosis and standardized treatment, which can improve the long-term outcome of MEN2A-specific tumors.
Adolescent ; Adrenal Gland Neoplasms ; Adult ; Amyloidosis, Familial ; Carcinoma, Neuroendocrine ; China ; Female ; Humans ; Lichens ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a/genetics* ; Pheochromocytoma ; Proto-Oncogene Proteins c-ret/genetics* ; Skin Diseases, Genetic ; Thyroid Neoplasms/genetics* ; Young Adult