[Adolescent female reproductive system dysplasia: a clinical study of 356 cases].

Objective: To explore the age of onset and consultation, the main clinical manifestations, common types of combined malformations, the relationship of endometriosis, surgical prognosis and different types of proportion of adolescent female reproductive system dysplasia. Methods: The medical records of 356 patients (aged 10-19) with female reproductive system dysplasia in Women's Hospital, School of Medicine, Zhejiang University from January 2003 to August 2018 were collected and retrospectively analyzed. Results: (1) Among the 356 adolescent dysplasia patients, uterine dysplasia (23.6%, 84/356), oblique vaginal septum syndrome (OVSS; 22.5%, 80/356) and vaginal dysplasia (21.6%, 77/356) were the most frequent ones, followed by multi-sectional dysplasia (16.0%, 57/356), other types of developmental abnormalities like external genitaliaand urogenital fistula (13.5%, 48/356) and Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome; 2.8%, 10/356). (2) There were significant differences between the median age of onset and the age of consultation of patients with OVSS and other types of abnormalities except hymen atresia (both P<0.05). In contrast, there were no significant differences between the age of onset and the age of consultation of the patients of uterine dysplasia, vaginal dysplasia, hymen atresia, MRKH syndrome and multi-sectional dysplasia (all P>0.05). (3) The clinical manifestations were lack of specificity, and mainly abnormal finding was lower abdominal pain. (4) After admission, the majority of patients underwent comprehensive cardiopulmonary examination (71.3%, 254/356) and urinary system examination (63.5%, 226/356). Only 18.3% (65/356) of patients had completed abdominal organ examination, and 5.9% (21/356) skeletal system examination. About other systemic malformations, urological malformations were the most common (27.5%, 98/356), followed by anorectal malformation (0.6%, 2/356), heart malformations (0.3%, 1/356), and spinal malformations (0.3%, 1/356). 46.4% (84/181) of the surgical patients were diagnosed with combined endometriosis. Patients with obstructive genital tract malformations were more likely to combine with endometriosis than non-obstructive ones [50.3% (74/147) vs 29.4% (10/34); P<0.05]. However, there was no significant difference between the severity of endometriosis of those two kinds (P>0.05). (5) Totally 308 patients were followed up successfully with a median of 25.0 years old, and 20 cases were treated again; 12.0% (37/308) of them were suffering from menstrual disorder and 33.1% (102/308) of them with dysmenorrhea. Totally 130 patients had sexually active reported no sexual problems. Conclusions: Uterine dysplasia, OVSS and vaginal dysplasia are the most common syndromes in adolescent female reproductive system dysplasia along with frequent cases of coexisting urinary malformations and increasing risks of endometriosis. Meanwhile, the lack of specificity of clinical manifestations might delay the timely diagnosis and treatment after the onset of symptoms. Nonetheless, most patients could achieve good surgical outcomes.

[Research progress in functional regeneration methods and mechanisms of taste buds].

Gustation is one of the most important human senses. Taste dysfunctions, which may be due to aging, tongue cancer surgery, radiotherapy and chemotherapy, affect life quality. That is why the need for taste bud regeneration has received more attention. At present, research on development and renewal of taste cells provides a basis for taste bud regeneration; molecular mechanisms related to taste bud regeneration are being continuously uncoverd, aiding in the identification of more accurate targets for therapy. New methods such as nerve regeneration, tissue engineering, and cytokine therapy have emerged. The author reviews the mechanism and the latest methods of taste bud regeneration of lingual epithelium, aiming to open new horizions for the prevention and treatment of gustatory diseases, and provide theoretical references for its regeneration.

[Diagnostic value of high frequency oscillation in localization of type â ¡ focal cortical dysplasia epilepsy].

Retrospective analysis was conducted on 9 patients with type Ⅱ focal cortical dysplasia (FCD) who underwent stereo-electroencephalography (SEEG) implantation in the Department of Neurosurgery of the First Affiliated Hospital of Fujian Medical University from November 2020 to February 2023. The onset area, onset time, and frequency of high-frequency oscillations (HFO) were analyzed and the correlation of HFOs with interictal, preictal, and ictal periods. SEEG recordings of 80-500 Hz HFOs were observed in both interictal and ictal periods in 9 patients, with 6 patients exhibiting fast ripples (FR) in the range of 250-500 Hz. Surgical resection of the seizure onset area and FR-generating electrodes was performed, and postoperative follow-up for over 2 years indicated Engel I in 5 cases. 6 patients showed continuous discharge during the preictal period, and the distribution index of continuous discharge was positively correlated with seizure frequency. HFOs in the range of 80-500 Hz were present in all four seizure onset patterns during the ictal period. The onset area and FR-emitting electrode were surgically removed in 6 patients with continuous discharge and overlapping HFOs during the preictal period, with 5 cases of Engel I. Type Ⅱ FCD discharges exhibited complexity, high discharge indices, and a close association with HFOs. Compared with the spike wave, the electrode range of HF is more limited, and the incidence of HF before attack is significantly increased, which is closely correlated with the onset area. The simultaneous occurrence of HFO and the spike waves has higher diagnostic value than the individual occurrence, effectively enhancing surgical efficacy.

[Clinical pathologic analysis and review of literature on 11 cases of calcifying epithelial odontogenic tumor].

Objective: To improve the understanding of histological variants of calcifying epithelial odontogenic tumor (CEOT). Methods: In this retrospective study, 11 cases of CEOT diagnosed from January 2008 to March 2022 were enrolled in the Department of Oral Pathology of Nanjing Stomatological Hospital, Medical School of Nanjing University. Among them, 10 were male and 1 was female. The patients were 19 to 58 years old [(43.0±11.9) years] and the course of disease was 2 weeks to 5 years. The clinicopathological characteristics were analyzed and the follow-up of patients ranged from 1 to 8 years, including 8 cases with follow-up data and 3 cases lost to follow-up. Furthermore, the related domestic and international literature was reviewed. Results: Eleven cases of CEOT included 6 cases of classic CEOT, 2 cases of clear cell CEOT, 2 cases of Langerhans cell-rich variant of CEOT and 1 case of non-calcified CEOT. In 6 cases of classic CEOT, the ratio of occurrence in mandible to maxilla was 2∶1, the ratio in central parts to peripheral parts was 5∶1, 2 cases were associated with unerupted teeth and 3 cases showed local aggressiveness. Histopathologically, classic CEOT showed eosinophilic epithelial cells, amyloid and calcification with Ki-67 value<5%. Among 4 cases with follow-up information, 1 case recurred after 1 year and 3 cases did not recur for 3 to 8 years. In 2 cases of clear cell CEOT, they both occurred in the periphery of mandible, pathologically showing a mix of lamellar balloon-like clear cells and typical CEOT, positive for CK5/6 and p63 in the area where the epithelial cells and clear cells were located, scattered positive for periodic acid-Schiff (PAS) in clear cells, which indicated the presence of glycogen. The maximum Ki-67 value was 5% in this type. One case lost to follow-up and the other case did not recur for 1 year follow-up after surgery. In 2 cases of Langerhans cell-rich variant of CEOT, they were cystic solid lesions and both occurred in the anterior maxilla. Langerhans cells were scattered in the epithelium and non-calcified amyloid glomeruli were present. Two cases were followed up for 1 year and 2 years without recurrence after surgery. One case of non-calcified CEOT that occurs within the jan showed invasion of surrounding soft tissues and the highest of Ki-67 value at 8% in all 11 cases without recurrence at 1 year follow-up. Conclusions: The histological pattern of classic CEOT is unique, and it is necessary to prompt the understanding of several histological variants derived from it.

[Clinical analysis of 9 cases of a novel stent assisted intestinal bypass for preventing anastomotic leakage in laparoscopic assisted radical resection of rectal cancer].

Objective: To examine the safety and effectiveness of a novel stent assisted intestinal bypass for preventing anastomotic leakage in laparoscopic assisted radical resection of rectal cancer. Methods: The clinical data of 9 patients with rectal cancer who underwent laparoscopic radical resection and stent assisted intestinal bypass from September 2019 to June 2020 at the Department of Anus & Intestine Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University were retrospectively analyzed. There were 6 males and 3 females, aged (62.1±6.8) years (range: 53 to 75 years), underwent laparoscopic assisted radical resection of rectal cancer and stent assisted intestinal bypass. A degradable diverting stent was placed at the end of the ileum, and a drainage tube was placed at the proximal end of the stent to bypass the intestinal contents. After operation, the patients were given a diet with less residue. From the 14th day after operation, abdomen X-ray films were taken every 5 to 7 days to observe the destination of the stent dynamically. When the stent was observed to be disintegrated into pieces, the drainage tube was clamped for 3 days to observe any side effects before the tube was removed. The operation time, the time of removing the bypass tube and the total hospital stay were recorded. Results: Laparoscopic assisted radical resection of rectal cancer and stent assisted intestinal bypass were successfully performed in all patients. The operation time was (230.4±48.0) minutes (range: 150 to 318 minutes), and the time of removing shunt tube was (28.8±4.6) days (range: 22 to 34 days). The duration of hospitalization was (21.0±8.6) days (range: 9 to 34 days). Postoperative pathological examination showed 7 cases of moderately differentiated adenocarcinoma, 1 case of moderately well differentiated adenocarcinoma and 1 case of mucinous adenocarcinoma. There were 2 cases of T1, 4 cases of T2 and 3 cases of T3. The number of lymph node dissection was 13.4±3.5 (range: 6 to 18), 3 cases were positive and 6 cases were negative. The post-operation follow-up time was 6 to 16 months, no anastomotic leakage or stenosis was found. Conclusion: Stent assisted intestinal bypass for the prevention of anastomotic leakage in laparoscopic assisted radical resection of rectal cancer is safe and feasible, and shows good short-term effect.

Enhanced anticancer activity and endocytic mechanisms by polymeric nanocarriers of n-butylidenephthalide in leukemia cells.

PURPOSE: The purpose of this study was to investigate the antitumor mechanisms of n-butylidenephthalide (BP) and to further examine the delivery efficacy of polycationic liposome containing PEI and polyethylene glycol complex (LPPC)-encapsulated BP in leukemia cells. METHODS: MTS, flow cytometric and TUNEL assays were performed to assess cell viability and apoptosis. BP and BP/LPPC complex delivery efficiency was analyzed by full-wavelength fluorescent scanner and fluorescence microscope. The expressions of cell cycle- and apoptosis-related proteins were conducted by Western blotting. RESULTS: The results showed that BP inhibited leukemia cell growth by inducing cell cycle arrest and cell apoptosis. LPPC-encapsulated BP rapidly induced endocytic pathway activation, resulting in the internalization of BP into leukemia cells, causing cell apoptosis within 1 h. CONCLUSIONS: LPPC encapsulation enhanced the cytotoxic activity of BP and did not influence the effects of BP induction that suggested LPPC-encapsulated BP might be developed as anti-leukemia drugs in future.

[Safety of laser treatment for peri-implantitis: a review].

The peri-implantitis, one of the complications of dental implant surgery, has been bothered clinicians and researchers experts and scholars. Recently, laser, as an adjuvant therapy, has been applied to the treatment of peri-implantitis and achieved satisfactory results. The present review summarized the safety application of laser treatment for peri-implantitis.

[Curative effects and influenced factors of EMA-CO as an initial regimen for the treatment of high-risk gestational trophoblastic neoplasia].

Objective: This study aims to evaluate the efficacy of EMA-CO as an initial regimen for high-risk gestational trophoblastic neoplasia and to analysis the influenced factors. Methods: A total of 81 patients with high-risk gestational trophoblastic neoplasia (HRGTN) treated in Women Hospital Affiliated Zhejiang University from Jan 2007 to Jan 2017 whose primary chemotherapies were EMA-CO were enrolled.The International Federation of Gynecology and Obstetrics (FIGO) prognosis score of all the patients was ≥7.The relationships of different factors were analyzed by univariate and multivariate analysis. Results: Fifty-five of 81 patients (67.90%) achieved complete primary remission with single EMA-CO regimen, 12 patients (14.81%) achieved complete primary remission with EMA-CO regimen and surgery, and 14 patients (17.28%) achieved drug resistance.The univariate and multivariate analysis showed that the FIGO score (P=0.004, OR 1.336, 95%CI 1.099-1.623) was an independent predictive factor for the outcome of EMA-CO regimen. Conclusions: EMA-CO is the first-line regimen used to treat patients with HRGTN. Salvage therapies such as EMA-EP regimen and adjuvant surgery are still effective treatments. The FIGO score is an independent risk factors for the outcome of EMA-CO regimen.

Postoperative radiotherapy is dispensable for OSCC patients with micrometastases in lymph nodes.

Lymph node metastasis is a decisive factor for performing postoperative radiotherapy for oral squamous cell carcinoma (OSCC). However, whether OSCC patients with only micrometastasis need postoperative radiotherapy is unclear. In this study, OSCC patients (n = 311) with negative (n = 247), only micrometastasis (n = 44) and macrometastasis (n = 20) were detected and selected by HE staining. Micrometastasis was re-assessed using immunohistochemical staining of cytokeratin (CK) in HE-negative patients to find out the false negative cases. The results indicated that, among the negative lymph node cases (n = 247), the positive rate of CK was 4.94% (n = 12). Besides, the clinical features of the primary tumor in relation to the only micrometastatic status and the value of the postoperative radiotherapy on the only micrometastasis patients were evaluated. Patients with only micrometastasis had higher T stage and inferior worst pattern of invasion (WPOI) than patients without micrometastasis, but they had longer overall survival (OS), metastasis-free survival (MFS), and disease-free survival (DFS) than macrometastasis patients. However, the survival time of only micrometastasis patients with or without postoperative radiotherapy was comparable, even in patients with inferior WPOI. Radiotherapy, however, may only benefit patients with IV/V levels of micrometastasis. These data indicated that postoperative radiotherapy is dispensable for only micrometastasis OSCC patients.

[Role and clinical significance of coagulation and inflammatory factors in moderate and severe ovarian endometriosis].

Objective: To determine the levels of coagulation and inflammatory factors in women with moderate and severe ovarian endometriosis so as to investigate the possible role of coagulation and inflammatory factors in the pathogenesis, diagnosis and treatment of this disease. Methods: From June 2015 and June 2017, clinical data of 366 patients with pathologically diagnosed moderate and severe ovarian endometriosis (case group) and 244 patients with pathologically diagnosed benign ovarian cysts (control group) in Women's Hospital of Zhejiang University School of Medicine were retrospectively analyzed. The levels of coagulation indicators, inflammatory factors and serum tumor markers were measured. Then, the values of these indicators in diagnosis of endometriosis were analyzed. Results: (1) The levels of plasma prothrombin time (PT) and thrombin time (TT) in patients with ovarian endometriosis [median: 12.8 s (range: 12.4-13.2 s) and 15.5 s (range: 15.1-15.9 s), respectively] were significantly shorter than those with benign ovarian cysts [median: 13.0 s (range: 12.5-13.4 s) and 15.7 s (range: 15.3-16.1 s), respectively; allP<0.01]. The levels of plasma fibrinogen (FIB) and D-dimer [D-D; median: 3.1 g/L (range: 2.8-3.5 g/L) and 0.9 mg/L (range: 0.6-2.1 mg/L) , respectively] in patients with ovarian endometriosis were significantly higher than those with benign ovarian cysts [median: 2.8 g/L (range: 2.6-3.2 g/L) and 0.6 mg/L (range: 0.4-1.2 mg/L), respectively; P=0.000]. Moreover, neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio [PLR; median: 2.3 (range: 1.8-3.1) and 144 (range: 113-179), respectively] in patients with ovarian endometriosis were significantly higher than those with benign ovarian cysts [median: 2.1 (range: 1.6-2.8) and 128 (range: 104-165), respectively; P<0.01]. Furthermore, in patients with ovarian endometriosis, the levels of PT were significantly shorter in stage Ⅳ endometriosis than that in stage Ⅲ endometriosis (P<0.05). The levels of FIB and PLR in patients with stage Ⅳ endometriosis were significantly higher than those in patients with stage Ⅲ endometriosis (P<0.01) . (2) The cut-off value of CA(125) was 27.2 kU/L with a sensitivity of 83.6%, the cut-off value of FIB was 3.1 g/L with a sensitivity of 53.2%, while the sensitivity of combination index (FIB×CA(125)) was 84.9%. Conclusion: The abnormality of coagulation and inflammatory factors may be involved in the pathogenesis of moderate and severe ovarian endometriosis, and the detection of coagulation and inflammatory factors may be have important clinical significance for the diagnosis and treatment of moderate and severe ovarian endometriosis.

The burden of inpatient care for diabetic and non-diabetic patients with osteoporotic hip fractures-does it differ? An analysis of patients recruited into a fracture liaison service in Southeast Asia.

Hospital care and mortality of diabetic and non-diabetic osteoporotic Asian patients undergoing hip fracture surgery were explored with no difference in length of hospitalization, incidence of post-operative complications, or mortality between diabetics and non-diabetics seen. Time to operation correlated with post-operative complications occurrence and therefore surgery should be expeditiously done. INTRODUCTION: Whether burden of inpatient care, problems after admission, and mortality rates differ between diabetics and non-diabetics undergoing surgery for osteoporotic hip fractures has not been explored in Asian populations. METHOD: Three hundred eighty-nine multi-ethnic diabetic and non-diabetic patients recruited into a FLS at a large Asian hospital with new osteoporotic hip fractures requiring operative repair were analyzed. RESULTS: 87.9% were Chinese, 6.4% Malay, and 3.6% Indians. BMI and age did not significantly differ between diabetics and non-diabetics. Median (IQR) length of hospitalization (LOHS) in days was 12 (9, 17) in diabetics and 11 (8, 14) in non-diabetics (p = 0.011). Median time from admission to operation (TTO) was 3 (2, 5) in diabetics versus 2 (1, 4.5) in the non-diabetics (p = 0.003). Occurrence of aggregate post-operative complications did not differ between diabetics and non-diabetics. No in-hospital mortalities occurred in either group. Thirty-day and 1-year mortality rates did not differ between the two groups. One-year mortality was 2.8% in the entire cohort. On multivariate regression analysis adjusted for age and race, only TTO (ß; 1.8, 95% CI 1.5-2.0, p < 0.001) and occurrence of post-operative complications (ß; 6.3, 95% CI 3.7-7.9, p < 0.001) correlated with LOHS. TTO and age-adjusted Charlson's Comorbidity Index (CCI) correlated significantly with the development of post-operative complications. CONCLUSIONS: Diabetes was not independently associated with LOHS in patients undergoing hip fracture surgery. Aggregate post-operative complications did not differ between diabetics and non-diabetics. TTO and occurrence of post-operative complications significantly affected LOHS. TTO correlated with post-complications development. Surgery should be expeditiously done in both diabetics and non-diabetics to avoid the development of post-operative complications and to prevent prolonged hospital stay.

[Clinical analysis on hysteroscopic surgery for the treatment of type â ¡ cesarean scar pregnancy in the first trimester].

Objective: To investigate the safety and efficacy of hysterosopic management of type Ⅱ cesarean scar pregnancy (CSP) and the value of prophylactic uterine artery embolization (UAE). Methods: Totally 104 patients with type Ⅱ CSP treated with hysteroscopic surgery at the Women's Hospital, School of Medicine, Zhejiang University, during Jan. 2009 to Jun. 2016 were analyzed retrospectively, 67 patients combined with UAE (UAE group) and 37 patients without combined with UAE (non-UAE group). Laparoscopy or sonography guidance was conducted simultaneously. The following clinical parameters were compared, including: primary cure rate, uterine packing rate, uterine perforation rate, hemoglobin level change, the time for the mass absorption and the return of ß-hCG to normal, complications, hospital days and hospital stay cost. Results: Median gestational age, size of mass, thickness of the anterior myometrium and ß-hCG level in UAE group versus non-UAE group were 47 versus 47 days, 30 versus 30 mm,2 versus 2 mm, 36 524 versus 32 226 U/L (all P>0.05). Out of 104, 100 patients were managed successfully with hysteroscopic surgery, and 4 patients transformed to laparoscopic or laparotomy surgery. Hysteroscopic surgery was effective in 63 out of 67 patients (94%) in UAE group and 34 out of 37 patients (92%) in non-UAE group (P>0.05). There was no significant differences regarding uterine perforation rate, uterine packing rate, hemoglobin change and recovery time between UAE group and non-UAE group (all P>0.05). The median hospital day was 7 days in UAE group versus 5 days in non-UAE group (P<0.01). The median hospital stay cost was 13 654 yuan in UAE group versus 9 108 yuan in non-UAE group (P<0.01). Serious complication occurred in 4 patients (6%, 4/67) in UAE group and 2 patients (5%, 2/67) in non-UAE group (P=0.906). Conclusions: Hysteroscopic surgery is effective and safe for patients with type Ⅱ CSP in the first trimester with size ≤30 mm in diameter and gestation age<7 weeks. The value of prophylactic UAE is uncertain.

[Study on the expression of epidermal growth factor receptor protein during benzo(a)pyrene induced carcinogenesis].

Objective: To explore the expression of epidermal growth factor receptor(EGFR) protein during benzo(a)pyrene (BaP) induced carcinogenesis. Methods: This study, we firstly utilized immunofluorescence assay and Western-blot to examine EGFR expression of the BaP which was constructed previously by project team induced malignant transformation human bronchial epithelial cell (BTC) and the control (16HBE cell). Then, we selected 36 healthy SD rats, divided into two groups according to simple random method, 18 rats each group. The constructed rat lung neoplasm model induced by pulmonary injection of BaP (10 mg/ml of BaP solution in 0.2 ml corn oil), contrast group use 0.2 ml corn oil, lung tissue was collected and the EGFR expression of lung tissue was detected by immunofluorescence assay and Western blot. T analysis was used to test the different of EGFR between two groups. Results: Immunofluorescence analysis showed that the EGFR expression in BTC was significantly higher than 16HBE cell. Meanwhile, Western blot also was used to confirmed this result, the relative expression of EGFR protein in the rats of the model group the control group were 1.04±0.13 and 2.32±0.12, respectively, and the difference was statistically significance (t=12.39, P<0.001). In vivo, well-defined tumor was found in the rat with pulmonary injection of BaP, and the lung showed diffuse alveolar septal thickening, alveolar wall destruction and pulmonary alveoli fusion, which suggested that the rat lung neoplasm model was constructive successfully. Furthermore, we found the EGFR expression of lung was increased dramatically in the rat lung neoplasm model by immunofluorescence detection and Western blot. The relative expression of EGFR protein in the rats of the model group the control group were 0.21±0.03 and 1.30±0.07, respectively, and the difference was statistically significance (t=12.84, P<0.001). Conclusion: Expression of EGFR protein was increased during BaP carcinogenesis, and EGFR may play an important role in the carcinogenesis of BaP.

[Analysis of clinical characteristics and outcome of patients with very high risk primary immunoglobulin light-chain amyloidosis].

Objective: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. Method: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk' group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. Results: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20-84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%) , and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 µg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001) . Conclusion: Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.

Impact of pretransplant induction therapy on autologous stem cell transplantation for patients with newly diagnosed POEMS syndrome.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a rare plasma cell dyscrasia sometimes treated with a haematopoietic cell autotransplant. We analyzed data from 138 subjects with newly diagnosed POEMS syndrome receiving a autotransplant at our center. Thirty-two subjects with severe end-organ dysfunction ineligible for immediate autotransplant received pretransplant therapy, which made a subsequent autotransplant feasible. Pretransplant therapy resulted in vascular endothelial growth factor (VEGF) remissions in 15 (47%). Thirty-three transplant recipients (24%) had early posttransplant complications. Risk factors for these complications identified through multivariate analysis included age >50 years (odds ratio (OR) 2.79, 95% confidence interval (CI) 1.09-7.14; P=0.033), time from symptom onset to transplant >5 years (OR 4.71, 95% CI 1.10-20.18; P=0.037) and pleural effusion (OR 3.39, 95% CI 1.26-9.12; P=0.016). Subjects receiving pretransplant therapy had fewer early complications than those who did not (OR 0.17, 95% CI 0.04-0.71; P=0.015), especially in subjects with a VEGF remission (OR 0.05, 95% CI 0.01-0.49; P=0.010). Autotransplants resulted in hematological remission in 60 (50%), VEGF remissions in 76 (72%) and improvements in other organ functions (65-90%). The 5-year progression-free survival (PFS) and overall survival were 76% (95% CI 64-84%) and 94% (95% CI 87-97%), respectively. Hematological (5-year PFS 83 vs 66%, P=0.008), VEGF (5-year PFS 79 vs 57%, P=0.021) remissions and especially both (5-year PFS 95 vs 61%, P=0.004) were associated with better PFS.

Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. In its dozens of clinical features, those with independent prognostic value are still not well characterized. We retrospectively included 362 patients with newly diagnosed POEMS syndrome at our institute from 2000 to 2015. On the basis of a randomized sample splitting, we first identified four baseline clinical variables, including age >50 years (hazards ratio (HR) 4.07, 95% confidence interval (CI) 1.41-11.76, P=0.009), pulmonary hypertension (HR 3.99, 95% CI 1.44-11.04, P=0.008), pleural effusion (HR 3.81, 95% CI 1.23-11.79, P=0.02) and estimated glomerular filtration rate <30 ml/min/1.73 m2 (HR 8.25, 95% CI 2.18-31.25, P=0.002), associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model. These factors were incorporated together to develop a prognostic nomogram. Concordance index calculation (0.727, 95% CI 0.601-0.853, P=0.018) and calibration curve plotting demonstrated its significant predictive and discriminatory capacity in the validation cohort. This nomogram could be a useful and convenient tool in clinical practice to evaluate individualized prognosis in patients with newly diagnosed POEMS syndrome.