Objective: To investigate the genetic and genomic profiling of juvenile myelomonocytic leukemia (JMML) and factors affecting its survival rate. Methods: Clinical characteristics, cytogenetics, molecular biology results and survival status of children with 27 JMML cases admitted to the Hematology Department of Children's Hospital, Capital Institute of Pediatrics from December 2012 to December 2021 were analyzed retrospectively, and the outcomes of the children were followed up. Kaplan-Meier method was used for survival analysis. Univariate analysis was used for analyzing factors affecting the overall survival (OS) rates of patients who received hematopoietic stem cell transplantation (HSCT). Log-Rank test was used for comparison of survival curves. Results: Among 27 JMML cases, there were 11 males and 16 females. The age of disease onset was 28 (11,52) months. There are 20 cases of normal karyotype, 4 cases of monosomy 7, 1 case of trisomy 8,1 case of 11q23 rearrangement and 1 case of complex karyotype. A total of 39 somatic mutations were detected.Those involved in RAS signal pathway were the highest (64%(25/39)), among which PTPN11 mutation was the most frequent (44% (11/25)). A total of 17 cases (63%) received HSCT, 8 cases (30%) did not receive HSCT, and 2 cases (7%) lost follow-up. For children receiving transplantation, the follow-up time after transplantation was 47 (11,57) months. The 1-year OS rate of high-risk transplantation group (17 cases) and high-risk non transplantation group (6 cases) was (88±8)% and (50±20)% respectively, with a statistically significant difference (χ²=5.01, P=0.025). The 5-year OS rate of the high-risk transplantation group was (75±11)%. The survival time of those who relapsed or progressed to acute myeloid leukemia after transplantation was significantly shorter than that of those who did not relapse (χ²=6.80, P=0.009). The OS rate of patients with or without PTPN11 mutation was (81±12) % and (67±19)% respectively (χ²=0.85, P=0.356). Conclusions: The main pathogenesis involved in JMML is gene mutation related to RAS signaling pathway, and the most common driver gene of mutation is PTPN11. Allogeneic HSCT can significantly improve the survival rate of high-risk JMML patients. The recurrence or progression after transplantation was related to poor prognosis.
Male ; Female ; Child ; Humans ; Child, Preschool ; Leukemia, Myelomonocytic, Juvenile/therapy* ; Retrospective Studies ; Survival Analysis ; Mutation ; Hematopoietic Stem Cell Transplantation

Animals ; Cellular Reprogramming ; Dentate Gyrus ; cytology ; Fibroblasts ; cytology ; Mice ; Neural Stem Cells ; cytology ; transplantation ; Swine

OBJECTIVETo investigate the effects of platelet-rich plasma (PRP) on the proliferation of dermal papilla cells (DPCs) and hair follicle regeneration.

METHODSPRP was prepared using the double-spin method and applied to DPCs. The proliferative effect of activated PRP on DPCs was measured using MTT assay. To understand the influence of activated PRP on the hair-inductive capacity of DPCs, freshly isolated epidermal cells and DPCs of passage 4 were resuspended, mixed with various concentrations of a PRP (0%, 5% or 10%) and were then transferred to a grafting chamber, which was implanted onto the dorsal skin of nude mice. The chambers were removed 1 week after grafting and HF formation was monitored for 4 weeks; the graft site was harvested and processed for histological examination.

RESULTSActivated PRP increased the proliferation benefited the aggregative growth of DPCs. There are significant difference in the yield of hair follicles compared with 10% PRP (344 +/- 27) with 0% PRP (288 +/- 35) in the area of reconstituted skin (P < 0.05). The areas treated with PRP demonstrated an increase in hair follicles density of 19.4%. Ten percent PRP (18 +/- 1) d also can significantly shorten the time of hair formation, compared with 0% PRP (20 +/- 1) d (P < 0.05).

CONCLUSIONSThere is a considerable effect of PRP on the time of hair formation and the yield of hair follicles reconstitution.

Animals ; Cell Proliferation ; Cells, Cultured ; Female ; Hair Follicle ; cytology ; growth & development ; Mice ; Mice, Inbred BALB C ; Mice, Inbred C57BL ; Mice, Nude ; Platelet-Rich Plasma ; Regeneration ; Skin ; cytology ; Skin, Artificial

Objective To study the damage on organs from salt sensitivity hypertension or non-salt-sensitive hypertension and the selection of drug combination.Methods 120 hypertensive patiems including 60 cases salt-sensitive (SS) and 60 non-salt-sensitive (NSS) groups were selected in our hospital and their salt load tested.These two groups were randomly divided into two groups,each group with 30 patients,one was given felodipine and perindopril and the others were given indapamide sustained release tablets and peridopril to facilitate the 12-week treatment.Before and after the treatment,patients were tested for physiological indicators,such as sitting blood pressure,24-hour ambulatory blood pressure,insulin resistance index,comparing changes of various sub-index etc.Results Significantly different were seen in indices as fasting blood glucose and serum creatinine (P＜ 0.01),fasting insulin,left ventricular mass index,urinary albumin,body mass index,insulin resistance indices,while between the SS group and the NSS group(P＜0.05).In the SS group,when patients with various sub-indicators were using perindopril combined with indapamide treatment,the related detected indicators tended to be normal and with statistically significant differences (P＜0.05).In the NSS group,those related indexes also tended to be more normal when using felodipine combined with perindopril.However,there were statistically significant differences between the two groups (P＜0.05).Conclusion On SS hypertensive patients with target organ damages,perindopril and indapamide seemed to be more effective in NSS patients,indicating that the use of perindopril and felodipine combination,seemed to be more suitable.

OBJECTIVETo compare the short-term outcomes of surgical treatment for non-small cell lung cancer (NSCLC) by video-assisted thoracoscopic surgery (VATS) and open thoracotomy (OT).

METHODSData of 737 consecutive NSCLC patients who underwent surgical treatment for non-small cell lung cancer by video-assisted thoracoscopic surgery and 630 patients who underwent pulmonary resection via open thoracotomy (as controls) in Cancer Institute & Hospital, Chinese Academy of Medical Sciences between January 2009 and August 2011 were retrospectively reviewed. The risk factors after lobectomy were also analyzed.

RESULTSIn the 506 NSCLC patients who received VATS lobectomy, postoperative complications occurred in 13 patients (2.6%) and one patient died of acute respiratory distress syndrome (0.2%). In the 521 patients who received open thoracotomy (OT) lobectomy, postoperative complications occurred in 21 patients (4.0%) and one patient died of pulmonary infection (0.2%). There was no significant difference in the morbidity rate (P > 0.05) and mortality rate (P > 0.05) between the VATS group and OT group. In the 190 patients who received VATS wedge resections, postoperative complications occurred in 3 patients (1.6%). One hundred and nine patients received OT wedge resections. Postoperative complications occurred in 4 patients (3.7%). There were no significant differences for morbidity rate (P = 0.262) between these two groups, and there was no perioperative death in these two groups. Univariate and multivariate analyses demonstrated that age (OR = 1.047, 95%CI: 1.004 - 1.091), history of smoking (OR = 6.374, 95%CI: 2.588 - 15.695) and operation time (OR = 1.418, 95%CI: 1.075 - 1.871) were independent risk factors of postoperative complications.

CONCLUSIONSTo compare with the NSCLC patients who should undergo lobectomy or wedge resection via open thoracotomy, a similar short-term outcome can be achieved via VATS approach.

Age Factors ; Carcinoma, Non-Small-Cell Lung ; mortality ; pathology ; surgery ; Female ; Humans ; Length of Stay ; Lung Neoplasms ; mortality ; pathology ; surgery ; Lymphatic Metastasis ; Male ; Middle Aged ; Operative Time ; Pneumonectomy ; adverse effects ; classification ; methods ; Postoperative Complications ; etiology ; Respiratory Distress Syndrome, Adult ; etiology ; Retrospective Studies ; Smoking ; Thoracic Surgery, Video-Assisted ; adverse effects ; Thoracotomy ; adverse effects ; methods

BACKGROUNDDiabetic patients undergoing percutaneous coronary intervention (PCI) have a higher incidence of contrast-induced nephropathy (CIN) than nondiabetic patients, and no pharmacological approach has been demonstrated to offer consistent protection. Therefore, identifying individuals who are at increased risk becomes essential. This study was designed to assess the predictive role of the ratio of contrast medium volume to estimated glomerular filtration rate (CMV/eGFR) in diabetic patients undergoing elective PCI who developed CIN.

METHODSWe retrospectively investigated clinical factors associated with the development of CIN in 114 diabetic patients who had undergone elective PCI. The risk factors for CIN included age, gender, body mass index (BMI), left ventricular ejection fraction (LVEF), hemoglobin (Hb), fasting plasma glucose (FPG), hemoglobin A1c (HbA1c), volume of contrast medium, basic levels of serum creatinine (Scr), the number of treated vessels and the number of stents used. We conducted a stepwise regression analysis to evaluate the predictive role of these risk factors in the incidence of CIN.

RESULTSThe incidence of CIN was 18.4% (21/114). There were no significant differences in age, gender, BMI, LVEF, Hb, FPG, HbA1c, and incidence of hypertension and number of acute myocardial infarction (AMI) in patients between the CIN (n = 21) and the non-CIN (n = 93) groups. However, the eGFR was significantly lower ((72.0 ± 12.5) ml·min(-1)·1.73 m(-2) vs. (82.0 ± 16.5) ml·min(-1)·1.7 m(-2), P = 0.010), and the basic serum creatinine level ((1.07 ± 0.12) mg/dl vs. (0.97 ± 0.19) mg/dl P = 0.014) was significantly higher in the CIN group. In addition, the volume of contrast medium was significantly larger ((253 ± 75) ml vs. (211 ± 71) ml, P = 0.017) and the CMV/eGFR ratio was significantly greater (3.64 ± 1.26 vs. 2.70 ± 1.11, P = 0.001) in the CIN group. Stepwise regression analysis showed that the CMV/eGFR ratio was a significant independent predictor for the development of CIN (P = 0.001). At a cut-off point of > 3.1, the CMV/eGFR ratio exhibited 71% sensitivity and 70% specificity for detecting CIN.

CONCLUSIONThe CMV/eGFR ratio could be a valuable predictor of CIN for diabetic patients after elective PCI. At a cut-off point of > 3.1, the CMV/eGFR ratio was an optimal predictor for the incidence of CIN.

Aged ; Angioplasty, Balloon, Coronary ; Contrast Media ; adverse effects ; Diabetes Mellitus ; therapy ; Diabetic Nephropathies ; chemically induced ; Female ; Glomerular Filtration Rate ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors

BACKGROUNDThe incidence of no reflow phenomenon limits the clinical outcomes of percutaneous coronary intervention (PCI). This randomized controlled study was designed to evaluate the immediate protective effects of intensive statin pretreatment on myocardial perfusion and myocardial ischemic injury during PCI.

METHODSAltogether 228 patients with acute coronary syndrome (ACS) were randomly assigned to standard statin group (SS group, n = 115) and intensive statin group (IS group, n = 113). Patients in the SS group received 20 mg simvastatin and patients in the IS group received 80 mg simvastatin for 7 days before PCI. Thrombolysis in myocardial infarction (TIMI) flow grade (TFG), corrected TIMI frame count (CTFC) and TIMI myocardial perfusion grade (TMPG) of the intervened vessel were recorded before and after stent deployment. Creatine kinase (CK) isoenzyme MB, troponin I and plasma level of high sensitive-C reactive protein (hs-CRP), P-selectin and intercellular adhesion molecule (ICAM) were measured before and 24 hours after the procedure.

RESULTSThe TFG after stent deployment was significantly improved with less TIMI 0-1 and more TIMI 3 blood flow in the IS group than in the SS group (all P < 0.05). Patients with no reflow phenomenon were less in the IS group (P < 0.001). The CTFC was lower in the IS group than in the SS group (P < 0.001). TMPG was also improved in the IS group than in the SS group (P = 0.001). Although PCI caused a significant increase in CK-MB 24 hours after the procedure, the elevated CK-MB value was lower in the IS group than in the SS group (18.74 +/- 8.41 vs 21.78 +/- 10.64, P = 0.018). Similar changes were also found in troponin I (0.99 +/- 1.07 in the IS group vs 1.47 +/- 1.54 in the SS group, P = 0.006). CK-MB elevation occurred in 27.8% (32/115) of the patients in the SS group vs 15.9% (18/113) in the IS group (P = 0.030). Myocardial necrosis was detected in 4.4% (5/115) of the patients in the SS group, whereas 0.9% (1/113) in the IS group (P = 0.341). But no myocardial infarction was found. Similarly, the patients with increased level of troponin I were much more in the SS group (36.5%, 42/115) than in the IS group (19.5%, 22/113) (P = 0.04). Among them, myocardial necrosis was detected in 13.0% (15/115) of the patients in the SS group, while 4.4% (5/113) in the IS group (P = 0.021). Myocardial infarction was found in 4.4% (5/115) of the patients in the SS group and 0.9% (1/113) in the IS group (P = 0.213).

CONCLUSIONSIntensive statin pretreatment for 7 days before PCI can further improve myocardial blood perfusion, protect the myocardium from ischemic injury. These effects are associated with the lowered levels of hs-CRP, P-selectin and ICAM.

Acute Coronary Syndrome ; drug therapy ; pathology ; therapy ; Aged ; Angioplasty, Balloon, Coronary ; methods ; Anticholesteremic Agents ; therapeutic use ; Female ; Heart ; drug effects ; Humans ; Male ; Middle Aged ; Myocardium ; pathology ; Simvastatin ; therapeutic use ; Treatment Outcome

Objective To explore the establishment of the mimetic aging effect in guinea pigs induced by D-galactose, and to detect the biological indicatrix associated with hearing loss and provide a new tool for molecular pathogenesis of hearing loss.Methods Total of 51 guinea pigs were randomly divided into three groups: group A ( model aging group , n = 25) , which were injected with D-galactose (200 mg ·kg-1·d-1 ) by intra peritoneum for 6 weeks, group B ( model control group , n = 18), which were given the same amount of saline only, and group C(vacant group, n = 15) were not treated.Then, The guinea pigs in group A and B were exposed in noise for 8 days, 8 hours once a day.Auditory brainstem response (ABR) was used to test the hearing threshold of guinea pigs thrice, first before the drug administered, then after 6 weeks the drug used, third after noise exposure.And colorimetry was used to analyze the activity of superoxide dismutase (SOD) and malon dialdehyde (MDA) in brain and liver tissue.The DNA of inner ear tissue was harvested and amplified fragment length polymorphism (AFLP) was used to detect the differential polymorphic markers.Results After injection, there was no significant difference in elevation of ABR threshold between the group A and group B ( t = 1.14, P>0.05 ).However, exposure of noise later, elevation in ABR threshold of ( 22.97±10.56) dB PeSPL was observed in group A, and ( 14.16±7.36) dB peSPL in group B.The was significant difference in variation of hearing threshold between group A and group B ( t = 2.78 ,P<0.05 ).The activity of SOD in brain and liver tissue in group A was lower than that in group B.the level of MDA was opposite between group A and group B.The difference between group A and group B was significant ( P<0.01 ) .A differential polymorphic marker was observed by AFLP. Conclusions The mimetic aging effect of the guinea pigs can be induced by D-galactose, and this model can not directly induce the hearing loss.The differential polymorphic marker possibly act as a predisposing factor which can greatly enhance the sensitivity of the ear to the noise.

OBJECTIVETo observe the intervention of liuwei dihuang pill (LDP) on therapeutic effectiveness and adverse reaction of hormonotherapy in treating nephrotic syndrome.

METHODSPatients allocated in two groups were medicated with initial dose of prednisone 1 mg/kg once a day at 8 am in the morning. After being medicated for 8 to 12 weeks, the dose of prednisone was decreased by 5.0 mg every 2 weeks till 0.5 mg/kg per day. Then the medication was changed to that two days' dosage orally take once a day with the daily dose reduced by 5.0 mg/kg every 2 to 3 weeks, and maintained at 0.4 mg/kg once every two days. At same time, necessary symptomatic treatment was given. To the treated group oral administration of LDP 8 capsules was given additionally, 3 times per day until prednisone decreased to maintenance dose.

RESULTSTherapeutic effect in the treated group was significant better than that in the control group (P < 0.05). Urinary protein, plasma albumin, triglyceride (TG) and total cholesterol (TC) in both groups were obviously improved (P < 0.05 or P < 0.01). However, as compared with the control group, the improvement was better, and the recurrent rate was lower (P < 0.05) in the treated group. Scores of Yin-deficiency caused excessive Fire syndrome and incidence rate of adverse reaction in the treated group were lower than those in the control group (P < 0.05 or P < 0.01).

CONCLUSIONLDP can markedly improve the therapeutic effectiveness and counteract the adverse reaction of hormonotherapy in treating nephrotic syndrome, and reduce the recurrence of the disease.

Adolescent ; Adult ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Nephrotic Syndrome ; drug therapy ; Obesity ; chemically induced ; prevention & control ; Phytotherapy ; Prednisone ; adverse effects ; therapeutic use ; Yin Deficiency ; chemically induced ; prevention & control

OBJECTIVEAn accurate clinical TNM staging of lung cancer is essential for the precise determination of the extent of the disease in order that an optimal therapeutic strategy can be planned. This is especially true in patients with marginally resectable tumors. Clinical over-staging of the disease may deny a patient the benefit of surgery, whereas under-staging may oblige a patient to accept a fruitless or even harmful surgery. We aimed to analyze preoperative clinical (c-TNM) and postoperative surgico-pathologic staging (p-TNM) of lung cancer patients in order to evaluate the accuracy of our clinical staging and its implications on the surgical strategy for lung cancer.

METHODSWe did a retrospective comparison of c-TNM and p-TNM staging of 2007 patients with lung cancer surgically treated from January 1999 to May 2003. Preoperative evaluation and c-TNM staging of all patients were based on physical examination, laboratory studies, routine chest X-ray and CT scan of the chest and upper abdomen. Other examinations included sputum cytology, bronchoscopy, abdominal ultrasonography, bone scintiscan, brain CT/MRI, and mediastinoscopy whenever indicated.

RESULTSIn the present study the comparison of c-TNM and p-TNM staging of 2007 patients with lung cancer revealed an overall concurrence rate of only 39.0%. In the entire series the extent of disease was clinically underestimated in 45.2% and overestimated in 15.8% of the patients. Among all c-TNM stages the c-IA/B stage of 1105 patients gave the highest rate (55.2%) of underestimating the extent of disease. Clinical staging of T subsets was relatively easy with an overall accuracy rate of 72.9%, while that of N subsets was relatively more difficult with an overall accuracy rate of 53.5%. Analysis also showed that c-IV stage may not be an absolute contraindication to surgery, because in half of the patients, c-M1 turned out to be p-M0, providing the possibility of resectional surgery depending on the status of T and N.

CONCLUSIONFor reasons to be further determined, the present preoperative clinical TNM staging of lung cancer remains a crude evaluation. Further efforts to improve its accuracy are needed.

Adult ; Aged ; Aged, 80 and over ; Carcinoma, Non-Small-Cell Lung ; pathology ; surgery ; Female ; Humans ; Lung Neoplasms ; pathology ; surgery ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplasm Staging ; Pneumonectomy ; Retrospective Studies