Natural history and treatment outcome of radiotherapy for Kimura抯 disease

Background: Kimura抯 disease is a rare inflammatory disease that usually appears in head and neck region. We reported natural history of the disease and treatment outcome of radiotherapy (RT) in Chiang Mai University Hospital. Methods: A retrospective review was performed for all Kimura抯 disease patients treated with radiotherapy at our center between 2002 and 2017. Results: A total of 20 patients with Kimura抯 disease were reviewed. There were 14 men and 6 women. All patients presented with the mass in head and neck region. Eleven patients were treated with a definitive intent with RT, 9 patients were treated with RT after recurrence from other modalities. All patients were treated with local external beam RT with 2 Gy per fraction to a median total dose of 30 Gy (range 30�). The median follow-up time was 4 years (range 1�.5 years). One patient died from HIV opportunistic infection after 1.5 year of radiation with complete response of Kimura抯 disease. Most of the patients responded to radiotherapy and controlled the disease at the time of analysis. Two patients had multiple recurrences at new areas outside radiotherapy field. Skin toxicity grade 1 was the most common late side effect which was found in 8 (44%) patients. We did not find severe late toxicity or second malignancy in this patient cohort. Conclusion: Good local control of Kimura抯 disease can be achieved, with a radiation dose of 30� Gy, with insignificant late toxicities. We suggest that radi

Research progress on long-term developmental outcomes of offspring of pregnant women with systemic lupus erythematosus / 中国当代儿科杂志

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease that affects multiple organs and systems. It is more common in women of childbearing age. Compared with the general population, pregnant women with SLE are at a significantly increased risk of adverse perinatal outcomes such as preterm birth and intrauterine growth restriction. In addition, the offspring of SLE patients may also be adversely affected by in utero exposure to maternal autoantibodies, cytokines, and drugs. This article summarizes the long-term developmental outcomes of offspring of pregnant women with SLE in terms of the blood system, circulatory system, nervous system, and immune system.

Long-term Outcome of Total Femur Replacement

@#Introduction: Total femur replacement is an option instead of amputation for extensive bone tumour or after revision surgery with a massive bone loss. Over a long period of time the patients may need revision surgery, and this might affect the functional outcome. We reviewed all consecutive total femur replacements done for primary and revision surgery of primary bone tumours in our centre to evaluate the long-term functional outcome and survival. Materials and methods: All patients who had total femur resection and reconstruction with modular endoprosthesis replacement in our centre from June 1997 to May 2022 were reviewed. The respondents were surveyed through WhatsApp using google form which was translated into Bahasa Malaysia based on the Musculoskeletal Tumour Society Scoring System (MSTS). The data were presented as descriptive data on the final survival of the limb and prosthesis. Results: Ten patients underwent total femur replacement. There were eight osteosarcoma, one giant cell tumour and one chondromyxoid fibroma. Three patients with osteosarcoma succumbed to pulmonary metastases; all had good early post-operative functional outcomes without local recurrence. Seven patients were available for long term evaluation of function with a mean follow-up of 17.6 years (ranged 10-25 years). Four patients with total femur replacement had good functional outcomes (60-80%) without revision with 10-25 years follow-up. Three patients experienced acetabulum erosion and chronic pain that required early hip replacements. Two of them were complicated with superior erosions and bone loss and subsequently were managed with massive reconstruction using cemented acetabulum cage reconstruction. The other has diabetes mellitus with chronic infection following revision of distal femur endoprosthesis to total femur replacement and subsequently underwent limited hemipelvectomy after 14 years. Conclusion: Total femur replacement offers a good long term functional outcome and prosthesis survival and is a favourable option for limb salvage surgery.

Short-, mid-, and long-term outcomes of suburethral slings with concomitant pelvic organ prolapse surgeries / Philippine Journal of Obstetrics and Gynecology

Background@#This retrospective cohort study assessed the outcomes of combined surgeries for pelvic organ prolapse (POP) and stress urinary incontinence (SUI) in 31 patients with a follow-up of 7 years.@*Objectives@#The study aimed to determine the success and recurrence rates of POP and SUI while comparing the outcomes, analyzing the predictors for recurrence, and reporting on complications.@*Materials and Methods@#Demographic and clinical profiles were analyzed descriptively using frequency and percentages. Objective SUI cure rate was assessed via office cystometry with a cough stress test, while POP was evaluated using the POP-Q system. Subjective cure rates for both conditions were determined using a symptom severity checklist based on the King's Health Questionnaire. Chi-square tests assessed associations between objective outcome parameters and time elapsed since surgery, and other outcome predictors, with significance set at p < 0.05.@*Results@#The results showed a high objective cure rate of 96% for SUI and subjective cure rates of 96% for both SUI and POP. Long-term symptoms included urine frequency and retention, while mesh erosion occurred in one patient. The objective cure rate for POP was 67.7%, with recurrence in the anterior compartment at the midterm and in various compartments at long term. Vault fixation suggests efficacy in reducing apical prolapse recurrence. The preoperative age was identified as a significant risk factor for POP recurrence. @*Conclusion@#This study supports the durability and efficacy of combined surgeries for SUI and POP, with high patient satisfaction.

Long-term outcome of centrally located hepatocellular carcinoma treated by radical resection combined with adjuvant radiotherapy / 中华普通外科杂志

Objective:To investigate the long-term outcome of centrally located hepatocellular carcinoma treated by radical resection and adjuvant radiotherapy(RT).Methods:A retrospective study was used to collect and analyze the clinical and pathological data of 193 patients with centrally located HCC who underwent surgery from Jun 2015 to Jun 2020. According to whether RT was used, these patients were allocated into liver resection (LR) combined RT (88 cases) and LR alone group (105 cases).Results:The 1-, 3-, and 5-year OS rates were 98%, 85%, and 74% for patients in the LR+RT group, and 79%, 66%, and 59% for patients in the LR group, respectively. The 1-, 3-, 5-year RFS rates were 76%, 55% and 44% for patients in the LR+RT group, and 51%, 40%, and 37% for patients in the LR group, respectively. OS and RFS was significantly different in LR+RT group compared with that in LR group (χ 2=5.825, P=0.016;χ 2=5.230, P=0.022, respectively). Cox analysis showed that RT was the independent prognostic factor for centrally located HCC in OS and RFS ( P=0.009, P=0.017, respectively). Subgroup analysis suggested that RT could reduce early recurrence ( HR=0.41,95% CI:0.21-0.80, P=0.002). Conclusion:Liver resection combined with adjuvant radiotherapy for centrally located HCC is safe and effective.

Epilepsy and Neurodevelopmental Outcomes in a Cohort of West Syndrome Beyond Two Years of Age

Objective To determine epilepsy and neurodevelopmental outcomes beyond 2 y of age and their putative prognostic factors in children with West syndrome (WS). Methods This cross-sectional study was initiated after approval from Institutional Ethics Committee. A follow-up cohort of 114 children (aged?2 y) diagnosed and treated for WS at the authors' center were assessed in-person for epilepsy and neurodevelopmental outcomes using Vineland Social Maturity Scale - Malin’s adaptation for Indian children. Subsequently, age at onset, lead-time-to-treatment, etiology, and response to any of the standard therapies were analyzed as possible predictors of these outcomes. Results Of 114 children (mean age: 55±32 mo, 91 boys), structural etiology was the predominant underlying etiology (79.8%) for WS. At 2 y of age, 64% had ongoing seizures. At the last follow-up, 76% had social quotient<55, and 39% had cerebral palsy (spastic quadriparesis in 21%). An underlying structural etiology was associated with ongoing seizures [OR (95% CI) 3.5 (1.4–9); p=0.008] at 2 y of age and poor developmental outcomes [OR (95% CI): 3.3 (1.3–8.9); p=0.016]. Complete cessation of spasms with the standard therapy was signifcantly associated with better seizure control [OR (95% CI): 5.4 (2.3–13); p<0.001] and neurodevelopmental outcome [OR (95% CI): 5.2 (1.8–14.9); p<0.001]. Conclusion The majority of children with WS have a poor neurodevelopmental outcome and epilepsy control on follow-up. The underlying etiology and response to initial standard therapy for epileptic spasms have a prognostic role in predicting the neurological outcome in these patients on follow-up.

Progressive neuropathy in patients with lepromatous leprosy after multidrug therapy

BACKGROUND The lepromatous pole is a stigmatising prototype for patients with leprosy. Generally, these patients have little or no symptoms of peripheral nerve involvement at the time of their diagnosis. However, signs of advanced peripheral neuropathy would be visible during the initial neurological evaluation and could worsen during and after multidrug therapy (MDT). Disabilities caused by peripheral nerve injuries greatly affect these patients' lives, and the pathophysiological mechanisms underlying nerve damage remain unclear. OBJECTIVES To evaluate the outcome of peripheral neuropathy in patients with lepromatous leprosy (LL) and persistent neuropathic symptoms years after completing MDT. METHODS We evaluated the medical records of 14 patients with LL who underwent nerve biopsies due to worsening neuropathy at least four years after MDT. FINDINGS Neuropathic pain developed in 64.3% of the patients, and a neurological examination showed that most patients had alterations in the medium- and large-caliber fibers at the beginning of treatment. Neurological symptoms and signs deteriorated despite complete MDT and prednisone or thalidomide use for years. Nerve conduction studies showed that sensory nerves were the most affected. MAIN CONCLUSIONS Patients with LL can develop progressive peripheral neuropathy, which continues to develop even when they are on long-term anti-inflammatory and immunosuppressive therapy.

Long term outcome of Wilms' tumor in tertiary care hospital

Background: Nephroblastoma, or Wilms’ tumor, is an embryonal tumor that develops from remnants of the immature kidney. It is the most common renal tumor of childhood. The aim is to analyze the long term outcome in Wilms’ tumor in perplex situations as double moiety and to correlate with multiple organ defects.Methods: It is a combined perspective and retrospective study that pediatric urology outpatient department (OPD) at the Institute of Child Health and Hospital for Children, Madras Medical College, Chennai. The study included patients with Wilms, who attended the pediatric surgery during the ten years, from March 2008 to February 2011. The patients were subjected to detailed clinical examination and relevant investigations were performed.Results: Among patients with stage I–II fumarate hydratase (FH) tumors, the relative risk (RR) of relapse and death were increased for loss of heterozygosity (LOH) 1p only (RR=2.2 for relapse; RR=4.0 for death), for LOH 16q only (RR=1.9 and RR=1.4), and LOH for both regions (RR=2.9 and RR=4.3) in comparison with patients lacking LOH at either locus.Conclusions: Stage I and II have a good prognosis. Stage III and IV need close surveillance since they have a high rate of recurrence. Stage V has a bad prognosis. Stage IV Wilms need lung irradiation. Neoadjuvant chemotherapy reduces tumor spillage in stage III and IV.

High-dose chemotherapy followed by autologous stem cell transplant for multiple myeloma: Predictors of long-term outcome

Background & objectives: Survival of patients with multiple myeloma (MM) has improved in the past two decades following use of novel agents and autologous stem cell transplantation. To determine predictors of long-term outcome, data of MM patients who underwent autologous stem cell transplantation (ASCT) at a tertiary care centre in north India were retrospectively analyzed. Methods: Between 1995 and 2016, 349 MM patients underwent ASCT. Patients' median age was 52 yr, ranging from 29 to 68 yr, 68.2 per cent were males. Thirty three per cent patients had international staging system (ISS) Stage III and 68.5 per cent had received novel agents-based induction. High-dose melphalan (200 mg/m2) was used for conditioning; patients with renal insufficiency (estimated glomerular filtration rate <40 ml/min) received melphalan 140-150 mg/m2. Results: Post-transplant, 317 of 349 (90.8%) patients responded; complete [complete response (CR)] ?213 (61%)], very good partial response (VGPR) ?62 (17.8%) and PR in 42 (12%)]. Induction with novel agents, pre-transplant chemosensitive disease, transplant in first remission and serum albumin (?3.5 g/dl) were predictors of significant response. At a median follow up of 73 months, median overall survival (OS) was 90 months [95% confidence interval (CI) 70.8-109.2], and progression-free survival (PFS) was 41 months (95% CI 33.0-49.0). On multivariate analysis, achievement of CR post-transplant, transplant in first remission, ISS Stages I and II (vs. III), absence of extramedullary disease and serum albumin ?3.5 g/dl were predictors of prolonged OS. For PFS, achievement of post-transplant CR and transplant in first remission were predictors of superior outcome. Interpretation & conclusions: Treatment with novel agents, achievement of complete remission post-transplant, ISS Stages I and II, absence of extramedullary disease and transplant in first remission were predictors of long-term survival for patients with MM.

Graves' Disease: Can It Be Cured?

Whether or not Graves' hyperthyroidism can be really cured, depends on the definition of “cure.” If eradication of thyroid hormone excess suffices for the label “cure,” then all patients can be cured because total thyroidectomy or high doses of 1¹³¹I will abolish hyperthyroidism albeit at the expense of creating another disease (hypothyroidism) requiring lifelong medication with levothyroxine. I would not call this a “cure,” which I would like to define as a state with stable thyroid stimulating hormone (TSH), free thyroxine, and triiodothyronine serum concentrations in the normal range in the absence of any thyroid medication. Surgery and radioiodine are unlikely to result in so-defined cures, as their preferable aim as stated in guidelines is to cause permanent hypothyroidism. Discontinuation of antithyroid drugs is followed by 50% recurrences within 4 years; before starting therapy the risk of recurrences can be estimated with the Graves' Recurrent Events After Therapy (GREAT) score. At 20-year follow-up about 62% had developed recurrent hyperthyroidism, 8% had subclinical hypothyroidism, and 3% overt hypothyroidism related to TSH receptor blocking antibodies and thyroid peroxidase antibodies. Only 27% was in remission, and might be considered cured. If the definition of “cure” would also include the disappearance of thyroid antibodies in serum, the proportion of cured patients would become even lower.

Surgical repair for ventricular septal rupture in 105 patients complicating with acute myocardial infarction: Fuwai Hospital’s 16 years’ follow-up results / 中国胸心血管外科临床杂志

@#Objective    To explore our novel strategy of surgical treatment for ventricular septal rupture (VSR) and the long-term outcomes. Methods    All the patients referred to the Center of Adult Surgery, Fuwai Hospital were treated with integration treatment of vasoactive agents, intra-aortic balloon pump, or left ventricular assist device. The timing of surgical treatment was individually customized. One hundred and five consecutive patients with VSR (63 males, 42 females ) presented at the mean age of 63 (range, 41 to 80) years. We retrospectively analyzed the results and followed up patients who survived the surgical procedure. Results     They were divided into a hemodynamics stable group (25 patients, 2 received emergent operation and 23 received selective operation) and a hemodynamics unstable group (80 patients, 34 received vasoactive agents and selective operation, 4 received vasoactive agents and emergent operation, 20 received vasoactive agent, intra-aortic balloon counterpulsation (IABP), and selective operation, 16 received vasoactive agents, IABP, and emergent operation, 2 received vasoactive agents, IABP, ventilator support, and selective operation, 2 received vasoactive agents, IABP, and ventilator support and emergent operation, 2 received vasoactive agents, ventilator support, and selective operation). There were 3 in-hospital deaths. Ninety-nine patients were followed up, with a follow-up rate of 97.1%. The mean follow-up time was 76.56±47.78 months. There were 2 late deaths during follow-up. Conclusion    The timing of surgical treatment for ventricular septal rupture should be individually customized. The long-term outcomes of ventricular septal rupture patients who survived the surgery are satisfactory.

Long-term Tolvaptan Treatment of Autosomal Dominant Polycystic Kidney Disease in Korea

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Compared with height-adjusted total kidney volume, the rate of kidney growth reduced significantly from 7.33% to 0.66% annually, since commencement of the tolvaptan therapy. The liver enzyme profile and serum sodium level and osmolality were constantly within normal ranges. In Korea, this is the first reported case of a patient with ADPKD who received tolvaptan treatment for more than 1 year. This case demonstrates that long-term tolvaptan treatment appears to be safe, well tolerated, and effective for ADPKD.

Long-term outcomes following mitral valvuloplasty versus replacement for native valve endocarditis: A case control study / 中国胸心血管外科临床杂志

@#Objective    To compare long-term outcomes following mitral valvuloplasty (MVP) and mitral valve replacement (MVR) for native valve endocarditis (NVE). Methods    Between November 1993 and August 2016, consecutive 101 patients with NVE underwent mitral surgery in our department, MVP for 52 patients and MVR for 49 patients. There were 69 males and 32 females at age of 38.1±14.9 years. The mean follow-up was 99.4±75.8 months. Results    There was no statistical difference in cardiopulmonary bypass time, aortic cross-clamp time, in-hospital mortality, duration of mechanical ventilation, ICU stay or hospital stay after surgery between the two groups. Survival rate at 1, 5, 10, 20 years after surgery was 100.0%, 97.6%, 97.6%, 97.6% for MVP, and 93.5%, 84.3%, 84.3%, 66.2% for MVR with a statistical difference between the two groups (P=0.018). There was no stroke in the patients with MVP during follow-up periods. However, stroke-free survival rate at 1, 5, 10, 20 years after surgery was 100.0%, 93.9%, 89.4%, 70.2% for MVR patients with a statistical difference between the two groups (P=0.023). There was no statistical difference in recurrence of infection, perivalvular leakage and reoperation between the two groups. Composite endpoint-free survival rate at 1, 5, 10, 20 years after surgery was 100.0%, 97.6%, 92.9%, 92.9% for MVP, and 91.3%, 79.6%, 75.8%, 51.0% for MVR with a statistical difference (P=0.006). Conclusion    MVP is associated with better outcomes than MVR in the patients  with NVE; generalizing MVP technique in the patients with NVE is needed.

Recent advances in the diagnosis and management of primary myelofibrosis

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) in which dysregulation of the Janus kinase/signal transducers and activators of transcription (JAK/STAT) signaling pathways is the major pathogenic mechanism. Most patients with PMF carry a driver mutation in the JAK2, MPL (myeloproliferative leukemia), or CALR (calreticulin) genes. Mutations in epigenetic regulators and RNA splicing genes may also occur, and play critical roles in PMF disease progression. Based on revised World Health Organization diagnostic criteria for MPNs, both screening for driver mutations and bone marrow biopsy are required for a specific diagnosis. Clinical trials of JAK2 inhibitors for PMF have revealed significant efficacy for improving splenomegaly and constitutional symptoms. However, the currently available drug therapies for PMF do not improve survival. Although allogeneic stem cell transplantation is potentially curative, it is associated with substantial treatment-related morbidity and mortality. PMF is a heterogeneous disorder and decisions regarding treatments are often complicated, necessitating the use of prognostic models to determine the management of treatments for individual patients. This review focuses on the clinical aspects and outcomes of a cohort of Japanese patients with PMF, including discussion of recent advances in the management of PMF.

Comparison of Apical and Asymmetric Septal Phenotype in Patients With Hypertrophic Cardiomyopathy: Clinical Features and Prognosis / 中国循环杂志

Objectives: To compare the clinical features and long-term outcomes of patients with apical hypertrophic cardiomyopathy (ApHCM) and patients with asymmetric septal hypertrophic cardiomyopathy (ASHCM). Methods: Data from 600 patients (300 with ApHCM and 300 with ASHCM) identified in a consecutive single-center cohort between 1996 and 2014 were retrospectively analyzed. The two groups were 1:1 matched by age of diagnosis, gender and the presence of outflow tract obstruction. Clinical features, cardiovascular mortalities, incidence of sudden cardiac death and cardiovascular morbidity (including unexplained syncope, atrial fibrillation, nonsustained ventricular tachycardia, progressive heart failure, embolic stroke or transient ischemic attack and myocardial infarction) were compared between the two groups. Results: Forty-two patients (14.0%) had a maximum LV wall thickness of ≥30 mm in the ASHCM group compared to only 11 patients (3.7%) in the ApHCM group (P<0.01). 156 patients in ApHCM group (52.0%)and 168 patients in ASHCM group(56.0%)underwent cardiovascular NMR examination, the incidence of late gadolinium enhancement was significantly lower in ApHCM group than in ASHCM group(26.9% vs 76.2%,P<0.01). The mean follow-up durations for ApHCM and ASHCM were (7.5 ± 4.0) years and (6.6 ± 5.4) years, respectively. The incidence of cardiovascular death (1.0% vs 5.7%), sudden cardiac death (0.33% vs 3.3%) and major adverse cardiovascular event (18.3% vs 40.3%) were significantly lower in the ApHCM group than in the ASHCM group (all P<0.01). Unexplained syncope, nonsustained ventricular tachycardia, and progressive heart failure were less common in ApHCM group than in ASHCM group (all P<0.05). Multivariate COX regression analysis showed that late gadolinium enhancement positivity (HR=4.62, 95% CI: 2.28- 68.0, P=0.02) and unexplained syncope (HR=8.56, 95% CI: 2.1-16.6, P<0.01) were independent predictors of cardiovascular mortality. Unexplained syncope was independent predictor for sudden cardiac death (HR=4.40, 95% CI: 1.5-15.2, P=0.02). Conclusions: After eliminating the interference of age at diagnosis, gender and outflow tract obstruction, patients with ApHCM represent a more benign prognosis with a lower incidence of cardiovascular mortality and morbidity than patients with ASHCM.

Long-term outcomes of prophylactic autologous pericardium tricuspid annuloplasty:propensity score matching of 832 patients with rheumatic heart disease / 实用医学杂志

Objective To investigate the safety and longterm outcomes of prophylactic autologous pericardium tricuspid valve annuloplasty(TVA)in patients with rheumatic heart disease(RHD). Methods A total of 832 patients with RHD were enrolled in this study ,including 146 patients with mild FTR but without TVA(observation group);434 patients with mild FTR underwent TVA(control group A)and 434 patients with moderate or severe FTR underwent TVA(control group B). Propensity score and survival analysis were used to evaluate perioperative safety ,FTR progression ,CHF and MACCE incidence after prophylactic TVA. Results A total of 192 patients were successfully matched. There was no significant difference in the perioperative complications between the observation group and the control groups(P>0.05). The progression rate of FTR in the observation group was significantly lower than that in the control groups (P = 0.005 & 0.032 ). There was no significant difference in the incidence of CHF and MACCE events between the observation group and the control groups (P > 0.05). Conclusions The treatment strategy of prophylactic autologous pericardium TVA at the time of left heart valve surgery for patients with RHD doesn′t increase operation costs ,perioperative complications and mortality ,but effectively prevent postoperative FTR recurrence or progression.

Clinical efficacy of laparoscopic liver resection versus open liver resection for hepato-cellular carcinoma / 中国肿瘤临床

Objective:To compare the short-and long-term outcomes of laparoscopic liver resection (LLR) with those of open liver re-section (OLR) for hepatocellular carcinoma (HCC). Methods:Clinical data from patients who suffered from HCC and received LLR or OLR from January 2013 to May 2016 in The First Affiliated Hospital of Fujian Medical University were analyzed restrospectively. To over-come selection bias, a 1:1 match was performed via a case-control study. After case-control matching was completed, 105 patients were included in each group. Short-term outcomes of operation and postoperation as well as long-term outcomes, including disease-free survival and overall survival rates, were evaluated. Relevant statistical methods were used for statistical analysis. Results: The postoperative hospital stay of the laparoscopic group was shorter (8.68 ± 2.82 vs. 10.61 ± 2.95 days, P<0.01) and its use of portal triad clamping was less (20.0%vs. 41.0%, P<0.01) than those of the open group. The abdominal drainage tube of the laparoscopic group was also removed at an earlier time than that of the open group (4.45±2.53 vs. 5.40±2.43 days, P<0.01). The 1-, 2-, and 3-year overall survival rates of the laparoscopic group were 96.88%, 87.54%, and 79.50%, respectively. By comparison, the 1-, 2-, and 3-year overall survival rates of the open group were 94.91%, 86.29%, and 76.37%, respectively (P=0.670). The 1-, 2-, and 3-year disease-free survival rates of the laparoscopic group were 72.09%, 60.16%, and 52.08%, respectively, while the 1-, 2-, and 3-year disease-free survival rates of the open group were 69.48%, 56.50%, 48.13%, respectively (P=0.388). Conclusion:LLR is a safe and feasible procedure. LLR in the selected patients with HCC showed similar long-term outcomes to those of OLR. The postoperative hospital stay of these patients who underwent LLR was shorter and their use of portal triad clamping was less than those of the patients who received OLR. The abdomi-nal drainage tube of the former was also removed at an earlier time than that of the latter. Therefore, the short-term outcomes of LLR were better than those of OLR.

Correlation Analysis between Electrophysiological Features and Mechanical Ventilation and Long-term Outcome in Patients with Guillain-Barré Syndrome / 中国医科大学学报

Objective To study the correlation between the electrophysiological features and mechanical ventilation and long?term outcome in Guil?lain?Barré syndrome(GBS)patients. Methods Electrophysiological and clinical data were retrospectively collected,and compared between venti?lated and not ventilated patients,as well as among each subtype of GBS. Results Totally 38 GBS patients were included in the study,among which 12(32%)were ventilated. The p/d CMAP ratio of the common peroneal nerve was significantly lower in ventilated group compared to not ventilated group(48.7±15.3 vs 80.8±24.0,P=0.005). AIDP was the most common subtype in ventilated patients compared with AMAN and undetermined (46%vs 0%and 9%,P=0.027). The long?term outcome score of AMAN was significantly lower than AIDP(4.3±1.3 vs 2.5±0.9,P=0.028). Conclusion Electrophysiological testing was predictive for mechanical ventilation and long?term outcome:low p/d CMAP ratio of the common pero?neal nerve was helpful for predicting mechanical ventilation,AIDP was prone to develop respiratory failure and had a worse outcome compared to AMAN.

A Meta analysis of predictive value of Wilms tumor gene 1 level on long-term outcome of acute myeloid leukemia / 中国综合临床

Objective To explore the predictivevalue of Wilms tumor gene 1 (WT1) level on long-term outcome of acute myeloid leukemia(AML).Methods The study in accordance with the inclusion criteria were collected by computer retrieving PubMed,ScienceDirect,EBSCO,Web of Science,CNKI,VIP and Wan Fang database.The retrieval time was from inception to March 2015,the two researchers according to the inclusion and exclusion independently selected studies and extracted the data and assessed the quality,then Revman 5.2 was used for Meta analysis.Results There were 11 relevant published articles were included with 1497 case patients.Meta analysis showed that High expression of WT1 gene was a negative factor for overall survival rate (OS) and disease free survival (DFS) in patients with AML (HR =1.34,95％CI 1.14-1.58,P ＜ 0.01;HR =1.35,95％CI 1.11-1.64,P =0.003).For cytogenetically normal AML patients,the high expression of WT1 gene was still the unfavorable factor of OS (HR =1.41,95％ CI 1.01-1.99,P =0.05).Conclusion WT1 expression level could be an useful prognosis long-term outcome marker for AML patients.

Clinical Characteristics of Patients with Bronchopulmonary Dysplasia Admitted to Pediatric Intensive Care Unit

PURPOSE: As the incidence of bronchopulmonary dysplasia (BPD) has increased, it is important to understand the clinical outcomes of BPD patients discharged from neonatal intensive care units (NICU). The purpose of our study was to describe the characteristics of BPD patients who are re-hospitalized in a pediatric intensive care unit (PICU) and to evaluate the prognostic outcome factors. METHODS: We retrospectively reviewed the medical records of BPD patients who were admitted to our PICU between May 2006 and November 2014. In total, we identified 101 cases which were divided into two groups, group 1, those who required intensive care for an acute illness or disease aggravation (n=62), and group 2, those who were admitted for post-operative care unrelated to having BPD as a control group (n=39). We subsequently compared the characteristics. RESULTS: Most patients in group 1 were aged less than 1 year, with weight below the 3rd percentile for age at the time of their PICU admission. The main cause for their admission was respiratory failure, requiring mechanical ventilation. When comparing the two groups, group 1 showed higher gestational age at birth, and a longer duration of mechanical ventilation, oxygen support, and NICU hospitalization than group 2. However, we failed to identify any factor significantly associated with the duration of the PICU stay, hospital stay, and mortality. Further large-scale, long-term follow-up studies will be necessary. CONCLUSION: As the majority of patients are admitted to PICU because of respiratory symptoms during their infantile period, careful follow-up with supportive care and prevention of respiratory infection are required.