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 To study the clinical efficacy of massage nursing intervention on children patients with bronchial pneumonia .Methods Totals of 80 children patients with bronchial pneumonia during the period from May 2012 to May 2013 were randomly divided into the control group ( routine nursing ) and the observation group ( massage nursing intervention ) . The clinical efficacy , hospitalization time , cost of hospitalization , and nursing satisfaction in the two groups were observed and compared .Results Compared with the control group, the effective rate was significantly increased (95.0% vs 80.0%), the hospitalization time [(6.5 ±1.5) vs (8.5 ±2.0)d] and cost of hospitalization [(1 900 ±100) vs (2 650 ±150)yuan] were significantly reduced , and the nursing satisfaction degree was obviously increased in the observation group ( great satisfaction:32 vs 24;satisfaction:6 vs 8;dissatisfaction:2 vs 8), and the differences were statistically significant (χ2 =4.11,t=2.21,3.47,Z=4.11,respectively;P<0.05).Conclusions For the children patients with bronchial pneumonia , the massage nursing intervention can significantly increase the clinical efficacy , promote early recovery .

Objective:To investigate the application effect of Precede-Proceed model nursing in postoperative anti-osteoporosis treatment of patients with osteoporotic thoracolumbar fracture (OTLF).Methods:A retrospective cohort study was conducted to analyze the clinical data of 168 patients with OTLF admitted to the Second Affiliated Hospital of Soochow University from September 2021 to June 2022, including 32 males and 136 females, aged 56-81 years [(72.0±6.6)years]. The fractured segments were T 1-T 10 in 29 patients, T 11-L 2 in 114 and L 3-L 5 in 25, all of whom were treated with percutaneous kyphoplasty (PKP). The 86 patients admitted from September 2021 to January 2022 were treated with conventional care (conventional nursing group) and the 82 patients admitted from February to June 2022 with Precede-Proceed model care (Precede-Proceed model nursing group). The compliance rates of anti-osteoporosis treatment at 1 and 6 months postoperatively and at the last follow-up were compared between the two groups, mainly including standardized medication, balanced diet, exercise and regular return visit. Chinese Osteoporosis Quality of Life (COQOL) questionnaire, including pain degree, physical function, social interaction ability, psychological and mental function, and total score were assessed before, at 6 months after surgery and at the last follow-up. Bone mineral density was measured preoperatively and at the last follow-up. Recurrence rate was recorded at the last follow-up. Results:All the patients were followed up for 12-16 months [(13.2±1.0)months]. There were 3 patients in the conventional nursing group and 2 in the Precede-Proceed model nursing group who were lost to follow-up. The compliance rates of standardized medication and regular return visit at 1 month after surgery were 97.5% (78/80) and 98.8% (79/80) in the Precede-Proceed model nursing group, which had no significant difference compared with 90.3% (75/83) and 96.4% (80/83) in the conventional nursing group ( P>0.05). The compliance rates of balanced diet and exercise at 1 month after surgery were 92.5% (74/80) and 91.3% (73/80) in the Precede-Proceed model nursing group, which were higher than those in the conventional nursing group [78.3% (65/83) and 73.5% (61/83)] ( P<0.05 or 0.01). The compliance rates of standardized medication, balanced diet, exercise and regular return visit at 6 months after surgery were 86.3% (69/80), 83.8% (67/80), 82.5% (66/80) and 90% (72/80) in the Precede-Proceed model nursing group, which were higher than those in the conventional nursing group [57.8% (48/83), 60.2% (50/83), 38.6% (32/83) and 37.3% (31/83)] ( P<0.01). At the last follow-up, the compliance rates of all the aspects in the Precede-Proceed model nursing group were 80% (64/80), 75% (60/80), 70% (56/80) and 82.5% (66/80), which were all higher than those of the conventional nursing group [36.1% (30/83), 54.2% (45/83), 26.5% (22/83) and 27.7% (23/83)] ( P<0.01). There were no statistical differences in COQOL scores including pain degree, physical function, social interaction ability, psychological and mental function and total scores between the two groups before surgery ( P>0.05). The pain degree, social interaction ability, psychological and mental function and total scores of COQOL in the Precede-Proceed model nursing group were lower than those in conventional nursing group at 6 months after surgery and at the last follow-up ( P<0.05 or 0.01). There was no statistical difference in the physical function of COQOL scores at 6 months after surgery ( P>0.05). The physical function of COQOL scores in the Precede-Proceed model nursing group were significantly lower than that of the conventional nursing group at the last follow-up ( P<0.05). There was no statistical difference in preoperative bone mineral density between the two groups ( P>0.05). Bone mineral density in the Precede-Proceed model nursing group was (-2.7±0.9)SD at the last follow-up, which was higher than that in the conventional nursing group [(-3.1±0.9)SD] ( P<0.05). At the last follow-up, bone mineral density of the Precede-Proceed model nursing group was significantly improved compared with that before surgery ( P<0.01), while there was no significant difference in the conventional nursing group ( P>0.05). The incidence of refracture in the Precede-Proceed model nursing group was 3.8% (3/80), which was lower than that in conventional nursing group [13.3% (11/83)] ( P<0.05). Conclusion:Compared with the conventional nursing mode, the Precede-Proceed model nursing can significantly improve the compliance rate of OTLF patients with postoperative anti osteoporosis treatment, improve their quality of life and bone quality, and reduce the incidence of refracture.

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

The individualized precision management of hereditary pheochromocytoma (PHEO) and paraganglioma (PGL) syndromes (PPGLs) based on molecular diagnosis and molecular subtype is becoming more popular. The newly discovered MAX germline mutation-associated PPGLs are autosomally dominant and rare. To raise awareness and explore the effective management of individual diagnosis and treatment, the relevant literature published between January 2011 and February was systematically reviewed. There were a total of 101 patients in the 77 families, involving all 5 exons, containing 44 types of MAX germline mutations and mostly concentrated in exons 3 and 4 (64.4%), the main mutations were nonsense mutations and missense mutations (72.3%), and some were large fragment deletions or insertions, intron variant, gene fusion mutations were relatively infrequent. Furthermore, about 10% of the patients had a paternal parent-of-origin effect. Among the 101 patients, 96 (95.0%) developed PHEO including 15 metastatic PHEO, 61 bilateral PHEO and 35 unilateral PHEO. The age of diagnosis was (31.7±10.9) years (range: 13 to 80 years). The male to female ratio was 1.2∶1. Eleven were accompanied with chest and abdominal PGL. Eight (7.9%) were accompanied by functional pituitary adenoma. And 12 (11.9%) developed other neuroendocrine tumors (NET), of which 8 were accompanied by PHEO, including 4 hyperparathyroidism, 1 gangliocytoma and neuroblastoma, 1 pancreatic NET, 1 medullary thyroid carcinoma and 1 C cell hyperplasia. Six presented concomitant non-NET, including 1 tongue squamous cell carcinoma, 1 papillary thyroid carcinoma, 1 prostate cancer, 1 renal oncocytoma, 1 breast cancer with renal oncocytoma, and 1 thoracic chondrosarcoma with multifocal adenocarcinoma of lung. The remaining 5 cases (5.0%), including 4 other NET (2 ganglioblastoma, 1 abdominal neuroblastoma and 1 pancreatic NET) and 1 asymptomatic child, did not present PHEO. The MAX germline mutation may cause a novel multiple endocrine neoplasia, which can be described as type 5. A comprehensive baseline assessment of neural crest cell-derived diseases such as PPGL, pituitary adenoma, hyperparathyroidism, and/or gangliocytoma (neuroblastoma) was recommended for all people with MAX germline mutations, and the risk of bilateral and/or metastatic PHEO should also be considered. In contrast, patients with PPGLs combined with other NET, such as functional pituitary adenoma, should undergo genetic testing and pedigree screening that includes at least the MAX gene.

The individualized precision management of hereditary pheochromocytoma (PHEO) and paraganglioma (PGL) syndromes (PPGLs) based on molecular diagnosis and molecular subtype is becoming more popular. The newly discovered MAX germline mutation-associated PPGLs are autosomally dominant and rare. To raise awareness and explore the effective management of individual diagnosis and treatment, the relevant literature published between January 2011 and February was systematically reviewed. There were a total of 101 patients in the 77 families, involving all 5 exons, containing 44 types of MAX germline mutations and mostly concentrated in exons 3 and 4 (64.4%), the main mutations were nonsense mutations and missense mutations (72.3%), and some were large fragment deletions or insertions, intron variant, gene fusion mutations were relatively infrequent. Furthermore, about 10% of the patients had a paternal parent-of-origin effect. Among the 101 patients, 96 (95.0%) developed PHEO including 15 metastatic PHEO, 61 bilateral PHEO and 35 unilateral PHEO. The age of diagnosis was (31.7±10.9) years (range: 13 to 80 years). The male to female ratio was 1.2∶1. Eleven were accompanied with chest and abdominal PGL. Eight (7.9%) were accompanied by functional pituitary adenoma. And 12 (11.9%) developed other neuroendocrine tumors (NET), of which 8 were accompanied by PHEO, including 4 hyperparathyroidism, 1 gangliocytoma and neuroblastoma, 1 pancreatic NET, 1 medullary thyroid carcinoma and 1 C cell hyperplasia. Six presented concomitant non-NET, including 1 tongue squamous cell carcinoma, 1 papillary thyroid carcinoma, 1 prostate cancer, 1 renal oncocytoma, 1 breast cancer with renal oncocytoma, and 1 thoracic chondrosarcoma with multifocal adenocarcinoma of lung. The remaining 5 cases (5.0%), including 4 other NET (2 ganglioblastoma, 1 abdominal neuroblastoma and 1 pancreatic NET) and 1 asymptomatic child, did not present PHEO. The MAX germline mutation may cause a novel multiple endocrine neoplasia, which can be described as type 5. A comprehensive baseline assessment of neural crest cell-derived diseases such as PPGL, pituitary adenoma, hyperparathyroidism, and/or gangliocytoma (neuroblastoma) was recommended for all people with MAX germline mutations, and the risk of bilateral and/or metastatic PHEO should also be considered. In contrast, patients with PPGLs combined with other NET, such as functional pituitary adenoma, should undergo genetic testing and pedigree screening that includes at least the MAX gene.