1.Comparison ofhaploidentical donor versus HLA-matched sibling donor hematopoietic stem cell transplantation for severe aplastic anemia
Jiaying WU ; Wenfang LUO ; Yi XIAO ; Yang CAO ; Lifang HUANG ; Na WANG ; Jinhuan XU ; Jue WANG ; Fankai MENG ; Donghua ZHANG ; Yicheng ZHANG
Chinese Journal of Organ Transplantation 2021;42(12):738-743
Objective:To compare the clinical outcomes and safety of haploidentical donor (HID)and HLA-matched sibling donor(MSD)hematopoietic stem cell transplantation(HSCT)for severe aplastic anemia(SAA).Methods:From January 1, 2012 to December 31, 2019, retrospective review of clinical data was performed for 75 SAA patients undergoing HSCT at Department of Hematology, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology.Based upon donor sources, they were divided into two groups of MSD(49 cases)and HID (26 cases). And two groups were compared with regards to hematopoietic recovery, graft-versus-host disease(GVHD)infection and overall survival(OS).Results:Time of platelet and neutrophil engraftment of two groups was comparable(11 d vs.11 d, P=0.84; 11 d vs.12 d, P=0.08). Compared with HID group, MSD group had a lower incidence of acute GVHD(46.2% vs.18.4%, P=0.01)with a comparable incidence of grade Ⅱ-Ⅳ acute GVHD(26.9% vs.14.3%, P=0.24), grade Ⅲ-Ⅳ acute GVHD(15.4% vs.4.1%, P=0.09)and chronic GVHD(23.9% vs.23.1 %, P=0.71). A reactivation of CMV occurred in 27(55.1%)MSD and 22(84.6%)HID recipients( P=0.01). And the incidence of EB viremia was 69.4% and 61.5% respectively.After a median follow-up period of 54.0 and 18.5 months, the estimated 3-year OS rate of MSD and HID groups were 94.0% and 88.0% respectively ( P=0.35). Conclusions:HID HSCT is an effective and relatively safe option for SAA patients, especially for those in urgent need of treatment without MSD or refractory/relapse to immunosuppressive therapy.
2.Effects of oxygen saturation on all-cause mortality among the elderly over 65 years old in 9 longevity areas of China
Dan LIU ; Feng ZHAO ; Qingmei HUANG ; Yuebin LYU ; Wenfang ZHONG ; Jinhui ZHOU ; Zhihao LI ; Yingli QU ; Ling LIU ; Yingchun LIU ; Jiaonan WANG ; Zhaojin CAO ; Xianbo WU ; Chen MAO ; Xiaoming SHI
Chinese Journal of Preventive Medicine 2021;55(1):45-52
Objective:To investigate the association between oxygen saturation (SpO 2) and risk of 3-year all-cause mortality among Chinese older adults aged 65 or over. Methods:The participants were enrolled from Healthy Aging and Biomarkers Cohort Study in year of 2012 to 2014 in 9 longevity areas in China. In this prospective cohort study, 2 287 participants aged 65 or over were enrolled. Data on SpO 2 and body measurements were collected at baseline in 2012, and data on survival outcome and time of mortality were collected at the follow-up in 2014. Participants were divided into two groups according to whether SpO 2 was abnormal (SpO 2<94% was defined as abnormal). Results:The 2 287 participants were (86.5±12.2) years old, 1 006 were males (44.0%), and 315 (13.8%) were abnormal in SpO 2. During follow-up in 2014, 452 were died, 1 434 were survived, and 401 were lost to follow-up. The all-cause mortality rate was 19.8%, and the follow-up rate was 82.5%. The mortality rate of SpO 2 in normal group was 21.1%, and that of abnormal group was 41.6% ( P<0.001). After adjusting for confounding factors, compared to participants with normal SpO 2, participants with abnormal SpO 2 had increased risk of all-cause mortality with HR (95% CI) of 1.62 (1.31-2.02); HR (95 % CI) was 1.49 (0.98-2.26) for males and 1.71 (1.30-2.26) for females in abnormal SpO 2group, respectively; HR (95% CI) was 2.70 (0.98-7.44) for aged 65-79 years old, 1.22 (0.63-2.38) for aged 80-89 years old, and 1.72 (1.35-2.19) for aged over 90 years old in abnormal SpO 2 group, respectively. Conclusion:Abnormal SpO 2 was responsible for increased risk of 3-year all-cause mortality among Chinese elderly adults.
3.Effects of oxygen saturation on all-cause mortality among the elderly over 65 years old in 9 longevity areas of China
Dan LIU ; Feng ZHAO ; Qingmei HUANG ; Yuebin LYU ; Wenfang ZHONG ; Jinhui ZHOU ; Zhihao LI ; Yingli QU ; Ling LIU ; Yingchun LIU ; Jiaonan WANG ; Zhaojin CAO ; Xianbo WU ; Chen MAO ; Xiaoming SHI
Chinese Journal of Preventive Medicine 2021;55(1):45-52
Objective:To investigate the association between oxygen saturation (SpO 2) and risk of 3-year all-cause mortality among Chinese older adults aged 65 or over. Methods:The participants were enrolled from Healthy Aging and Biomarkers Cohort Study in year of 2012 to 2014 in 9 longevity areas in China. In this prospective cohort study, 2 287 participants aged 65 or over were enrolled. Data on SpO 2 and body measurements were collected at baseline in 2012, and data on survival outcome and time of mortality were collected at the follow-up in 2014. Participants were divided into two groups according to whether SpO 2 was abnormal (SpO 2<94% was defined as abnormal). Results:The 2 287 participants were (86.5±12.2) years old, 1 006 were males (44.0%), and 315 (13.8%) were abnormal in SpO 2. During follow-up in 2014, 452 were died, 1 434 were survived, and 401 were lost to follow-up. The all-cause mortality rate was 19.8%, and the follow-up rate was 82.5%. The mortality rate of SpO 2 in normal group was 21.1%, and that of abnormal group was 41.6% ( P<0.001). After adjusting for confounding factors, compared to participants with normal SpO 2, participants with abnormal SpO 2 had increased risk of all-cause mortality with HR (95% CI) of 1.62 (1.31-2.02); HR (95 % CI) was 1.49 (0.98-2.26) for males and 1.71 (1.30-2.26) for females in abnormal SpO 2group, respectively; HR (95% CI) was 2.70 (0.98-7.44) for aged 65-79 years old, 1.22 (0.63-2.38) for aged 80-89 years old, and 1.72 (1.35-2.19) for aged over 90 years old in abnormal SpO 2 group, respectively. Conclusion:Abnormal SpO 2 was responsible for increased risk of 3-year all-cause mortality among Chinese elderly adults.
4. Effects of estimated glomerular filtration rate on all-cause mortality in the elderly aged 65 years and older in 8 longevity areas in China
Qing CHEN ; Feng ZHAO ; Qingmei HUANG ; Yuebin LYU ; Wenfang ZHONG ; Jinhui ZHOU ; Zhihao LI ; Yingli QU ; Ling LIU ; Yingchun LIU ; Jiaonan WANG ; Zhaojin CAO ; Xianbo WU ; Xiaoming SHI ; Chen MAO
Chinese Journal of Epidemiology 2020;41(1):36-41
Objective:
To investigate the association between estimated glomerular filtration rate (eGFR) and all-cause mortality in the elderly aged 65 years and older in longevity areas in China.
Methods:
Data used in this study were obtained from Healthy Aging and Biomarkers Cohort Study, a sub-cohort of the Chinese Longitudinal Healthy Longevity Survey, 1 802 elderly adults were collected in the study during 2012-2017/2018. In this study, the elderly were classified into 4 groups, moderate-to-severe group [<45 ml·min-1·(1.73 m2)-1], mild-to-moderate group [45- ml·min-1·(1.73 m2)-1], mild group [60- ml·min-1·(1.73 m2)-1] and normal group [≥90 ml·min-1·(1.73 m2)-1] according to their eGFR levels.
Results:
After 6 years of follow-up, 852 participants died, with a mortality rate of 47.3
5.Effects of sensory integration therapy on cognitive function and self-care ability in patients with mild cognitive impairment after stroke
Ying ZHAO ; Li TIAN ; Xiaoping WANG ; Wenfang ZHANG ; Hongmei LIU ; Jie ZHANG ; Xindi CAO ; Hongfei XU
Chinese Journal of Practical Nursing 2020;36(34):2655-2659
Objective:To investigate the changes of cognitive function and self-care ability in patients with mild cognitive impairment after stroke after sensory integration therapy.Methods:One hundred patients with mild cognitive impairment after stroke were divided into control group and intervention group according to the random number table method. The control group was given the routine health education path and rehabilitation training, and the intervention group was given the cognitive rehabilitation through sensory integration therapy on the basis of the control group. The cognitive function and self-care ability of the patients were evaluated by the Montreal cognitive assessment(MoCA) and daily self-care ability scale(ADL) before and after intervention.Results:After intervention, the total MoCA score, visual space, nominalization, language, abstraction and memory scores of the intervention group were 26.02±1.15, 3.50±0.76, 2.98±0.14, 2.90±0.30, 2.84±0.37 and 3.18±0.69, respectively, while those of the control group were 23.32±1.90, 2.86±1.20, 2.78±0.42, 2.82±0.39, 1.58±0.54 and 2.82±0.94, respectively. The difference between the two groups was statistically significant ( t value was -18.296--2.064, all P<0.05). The scores of attention and directional force in the intervention group were 5.44±0.67 and 5.18±0.85, respectively, while those in the control group were 5.32±0.84 and 5.14±0.78, respectively. There was no statistically significant difference between the two groups ( t value was -1.288, -0.704, P>0.05).After intervention, the total ADL score, stool, urine, toilet, eating, dressing and bathing scores of the intervention group were 65.00±5.98, 5.90±2.19, 6.40±2.27, 7.40±2.72, 7.80±2.70, 7.50±2.53 and 5.50±1.52, respectively, The control group was 53.60±10.20, 3.50±3.23, 5.00±3.50, 5.50±3.23, 5.40±3.76, 5.90±3.45, and 4.40±2.79, respectively. The difference between the two groups was statistically significant ( t value was -7.573~-2.615, all P<0.05). Conclusions:Sensory integration therapy can improve the cognitive function and self-care ability of patients with mild cognitive impairment after stroke.
6.Application value of Clavien-Dindo classification in evaluation of postoperative short-term complications of Da Vinci robotic-assisted or laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy
Weikai CHEN ; An ZHANG ; Jinling WU ; Aimin ZHU ; Xuan ZHANG ; Nan LI ; Wenfang ZHAO ; Xinping WANG ; Wen′an WANG ; Jing WANG ; Jianping YU ; Ruiyu TAO ; Zhengkai LI ; Kun LI ; Le LI ; Long YAN ; Tingbao CAO ; Dengwen WEI ; Hongbin LIU
Chinese Journal of Digestive Surgery 2020;19(9):976-982
Objective:To investigate the application value of Clavien-Dindo classification in evaluation of postoperative short-term complications of Da Vinci robotic-assisted or laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy. Methods:The retrospective cohort study was conducted. The clinicopathological data of 262 patients with gastric cancer who were admitted to the 940th Hospital of Joint Logistic Support Force of Chinese People′s Liberation Army from January 2016 to January 2019 were collected. There were 214 males and 48 females, aged (58±11) years, with a range from 17 to 81 years. Of 262 patients, 120 cases undergoing Da Vinci robotic-assisted total gastrectomy + D 2 lymphadenectomy + Roux-en-Y anastomosis were divided into robotic group, and 142 cases undergoing laparoscopic-assisted total gastrectomy + D 2 lymphadenectomy + Roux-en-Y anastomosis were divided into laparoscopic group. Observation indicators: (1) intraoperative and postoperative situations; (2) postoperative pathological examination; (3)complications; (4) stratified analysis; (5) follow-up. Follow-up using outpatient examination and telephone interview was performed to detect complications, tumor recurrence and survival of patients within postoperative 2 months. The follow-up was up to May 2019. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was analyzed using the t test. Measurement data with skewed distribution were represented as M (range), and comparison between groups was analyzed using the Mann-Whitney U test. Count data were described as absolute numbers or percentages, and comparison between groups was analyzed using the chi-square test or Fisher exact probability. Comparison of ranked data between groups was analyzed using the rank sum test. Results:(1) Intraoperative and postoperative situations: cases undergoing conversion to open surgery, the operation time, volume of intraoperative blood loss, the number of lymph node dissected, time to first flatus, time to initial fluid diet intake, duration of postoperative hospital stay of the robotic group were 1, (243±42)minutes, 100 mL(range, 100-150 mL), 38±15, (2.8±1.0)days, 3 days(range, 3-4 days), 11 days(range, 9-13 days), respectively. The above indicators of the laparoscopic group were 2, (244±38)minutes, 100 mL(range, 100-150 mL), 34±14, (3.2±1.0)days, 4 days(range, 3-5 days), 10 days(range, 9-13 days), respectively. There were significant differences in the number of lymph node dissected, time to first flatus, time to initial fluid diet intake between the two groups ( t=2.068, -3.030, Z=-3.370, P<0.05), and there was no significant difference in cases undergoing conversion to open surgery, the operation time, volume of intraoperative blood loss, duration of postoperative hospital stay between the two groups ( χ2=0.000, t=-0.158, Z=-1.824, -0.088, P>0.05). (2) Postoperative pathological examination: cases with well differentiated tumor, moderately differentiated tumor, poorly differentiated tumor, signet ring cell carcinoma or other types of tumor, cases in stage T1b, T2, T3 or T4a (pT staging), cases in stage N0, N1, N2, N3a or N3b (pN staging), cases in stage ⅠB, ⅡA, ⅡB, ⅢA, ⅢB or ⅢC (pTNM staging) of the robotic group were 6, 50, 55, 9, 10, 22, 63, 25, 42, 19, 19, 24, 16, 17, 22, 23, 20, 23, 15, respectively. The above indicators of the laparoscopic group were 4, 42, 84, 12, 6, 18, 81, 37, 39, 27, 32, 19, 25, 13, 19, 28, 39, 16, 27, respectively. There was no significant difference in the above indicators between the two groups ( Z=-1.880, -1.827, -0.140, -1.460, P>0.05). (3) Complications: cases with complication classified as grade Ⅰ, grade Ⅱ, grade Ⅲa, grade Ⅲb, grade Ⅳa, grade Ⅳb of Clavien-Dindo classification, cases with death, cases with overall complications, cases with severe complications of the robotic group were 9, 6, 3, 2, 2, 0, 0, 22, 7, respectively. The above indicators of the laparoscopic group were 12, 15, 9, 6, 3, 1, 1, 47, 20, respectively. There were significant differences in cases with overall complications, cases with severe complications between the two groups ( χ2=7.309, 4.790, P<0.05), and there was no significant difference in cases with complication classified as grade Ⅰ, grade Ⅱ, grade Ⅲa, grade Ⅲb, grade Ⅳa, grade Ⅳb of Clavien-Dindo classification, cases with death between the two groups ( χ2=0.080, 2.730, 1.042, 0.704, 0.000, 0.000, 0.000, P>0.05). (4) Stratified analysis: of the patients with overall complications in robotic group, cases of male or female, cases aged ≥65 years or <65 years, cases with body mass index (BMI) ≥24 kg/m 2 or <24 kg/m 2, cases with tumor diameter ≥5 cm or <5 cm, cases with or without abdominal surgery, cases with tumor located at upper stomach or middle stomach, cases in Ⅰ-Ⅱ grade or Ⅲ grade of American Society of Anesthesiologists (ASA) classification, cases with well differentiated tumor or undifferentiated tumor, cases in stage Ⅰ-Ⅱ or stage Ⅲ (pTNM staging), cases with operation time ≥250 minutes or <250 minutes, cases with volume of intraoperative blood loss ≥150 mL or <150 mL, cases with the number of lymph node dissected ≥25 or <25 were 15, 7, 14, 8, 11, 11, 16, 6, 4, 18, 19, 3, 15, 7, 7, 15, 8, 14, 12, 10, 12, 10, 14, 8, respectively. The above indicators of patients with overall complications in the laparoscopic group were 33, 14, 17, 30, 16, 31, 36, 11, 11, 36, 27, 20, 31, 16, 13, 34, 14, 33, 24, 23, respectively. Of the patients with overall complication, there were significant differences in cases of male, cases aged ≥65 years or <65 years, cases with BMI<24 kg/m 2, cases with tumor diameter≥5 cm, cases without abdominal surgery, cases with tumor located at middle stomach, cases in Ⅰ-Ⅱ grade or Ⅲ grade of ASA classification, cases with well differentiated tumor, cases in stage Ⅲ (pTNM staging), cases with operation time ≥250 minutes, cases with volume of intraoperative blood loss <150 mL, cases with the number of lymph node dissected ≥25 between the two groups ( χ2=6.683, 4.207, 6.761, 7.438, 4.297, 6.325, 9.433, 3.970, 4.850, 4.911, 3.952, 3.915, 6.865, 4.128, P<0.05) and there was no significant difference in cases of female, cases with BMI≥24 kg/m 2, cases with tumor diameter <5 cm, cases with abdominal surgery, cases with tumor located at upper stomach, cases with undifferentiated tumor, cases in stage Ⅰ-Ⅱ (pTNM staging), cases with operation time < 250 minutes, cases with volume of intraoperative blood loss ≥150 mL, cases with the number of lymph node dissected <25 between the two groups ( χ2=0.277, 1.052, 1.996, 1.552, 2.172, 2.594, 2.244, 3.771, 1.627, 3.223, P>0.05). (5) Follow-up: 262 patients were followed up postoperatively for 2 months. During the follow-up, no patient was diagnosed with tumor recurrence, and one patient in the laparoscopic group died of severe infection. Conclusions:The Clavien-Dindo classification can be used in evaluating postoperative short-term complications of Da Vinci robotic-assisted or laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy. Compared with laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy, Da Vinci robotic-assisted total gastrectomy with D 2 lymphadenectomy has the advantages of minimally invasiveness, low incidence of overall and severe complication.
7.Clinical characteristic and prognosis of primary renal sarcoma in adults
Yiming TANG ; Yun CAO ; Yan GUO ; Wei CHEN ; Wenfang CHEN ; Bing LIAO ; Xu CHEN ; Jinhuan WEI ; Zhiling ZHANG ; Junhang LUO
Chinese Journal of Urology 2019;40(5):370-373
Objective To investigate the clinical manifestations,imaging and pathological features and treatment prognosis of primary renal sarcoma in adults.Methods A retrospective analysis was performed on the clinical data of 48 patients with primary renal sarcoma from January 2009 to December 2018 in the first affiliated hospital and cancer center of Sun Yat-Sen university.There were 30 males and 18 females.Their aged ranged from 27 to 76 yrs with an average age of 50 yrs.A total of 24 patients presented with lumbar and abdominal pain.Abdominal mass was found in 2 cases.Gross hematuria was noticed in 4 cases.Febrile was recorded in 2 cases and 21 cases were diagnosed by physical examination.31 tumors located in the left kidney and 17 tumors located in the right kidney.The tumor diameter ranged from 3 to 16 cm with an average diameter of 8 cm.All patients underwent ultrasound or CT/MRI examination of the urinary system before surgery.The ultrasound showed the undistributed echo inside the tumor with the undistinguished border.The image of necrosis and liquefaction could be seen in some cases.The CT/MRI examination showed the lesion site with the necrosis,liquefaction or cystic changing.The mass exhibited the unregular enhancement with undistinguished border line.The mean diameter of liposcarcoma was 10.2 cm.CT scan demonstrated the relative low density of tumor,which was hard to be identified with AML.The average diameter of leiomyosarcoma was 6.5 cm.The enhanced CT scan showed the low density of tumor,compared with renal parenchyma.46 patients underwent radical nephrectomy,2 patients underwent renal tumor biopsy.And postoperative follow-up was performed.Results Pathological diagnosis revealed that 19 cases with liposarcoma,9 cases with leiomyosarcoma and 4 cases with synovial sarcoma,especially 4 cases with Ewing' s sarcoma and 12 cases with other sarcoma.36 cases were followed up and survived for 4 to 64 months.The average survival time was 28 months.The longest mean survival time was seen in patients with liposarcoma,which was 32 months (ranging 11 to 64 months).The mean survival time of synovial sarcoma group was 25 months (ranging 5-58 months).The mean survival time of Ewing's sarcoma group was 22 months(ranging 12-46 months).and the survival time of leiomyosarcoma group was the shortest 20 months (ranging 4-36 months).Conclusion Renal sarcoma is rare and highly malignant.It needs to be diagnosed with clinical manifestations,imaging and pathological data together.
8. The application of Precede-Proceed Model on medication management training in schizophrenic inpatients
Xiaojie ZHU ; Lin NI ; Haitao XIA ; Wenfang LU ; Xinmei CAO
Chinese Journal of Practical Nursing 2019;35(17):1319-1323
Objective:
To evaluate the application effect of the medication management training based on Precede-Proceed Model in schizophrenic inpatients.
Methods:
In this self-control study, 60 schizophrenic inpatients were chosen for this investigation and were undergoing the medication management training on Precede-Proceed Model with conventional nursing care. By using Insight and Treatment Attitudes Questionnaires (ITAQ), The Brief Psychiatric Rating Scale (BPRS) and Nurses′ Observation Scale for Inpatient Evaluation (NOSIE) after the first 3 months and 6 months of the intervention, in order to evaluate their results with their initial readings.
Results:
The total scores of ITAQ, the total scores of BPRS, lacking in activity factor, reaction factor, hostile-suspiciousness factor, the total scores of NOSIE, total positive and negative factors before the intervention were (183.3±15.0) points, (71.7±10.9) points and (13.6±8.8) points; three months after intervention were (189.0±15.8) points, (75.3±11.1) points and (11.6±7.2) points; six months after intervention were (193.8 months after intervention were15.2) points, (71.8 ±9.6) points and (10.1±7.0) points. There were significant differences between the total score and the total negative factor score before and after treatment (
9.The effects of isometric resistance training conducted using handgrip exercise on blood pressure variability in hypertensive patients with minor ischemic stroke
Jie ZHANG ; Wenfang ZHANG ; Hongfei XU ; Xindi CAO ; Yangyang JIN
Chinese Journal of Practical Nursing 2018;34(24):1850-1854
Objective The present study aimed to evaluate the effects of isometric resistance training(IRT)conducted using handgrip exercise on blood pressure variability (BPV) and heart rate variability (HRV) in hypertensive patients with minor ischemic stroke (MIS).Methods One hundred and twenty-five hypertensive patients with MIS were included in the present study. Patients were randomized into two groups with random number table. Patients in the control group (n=60) were nursed in the routine care in department of neurology, while patients in the experimental group (n=65) received the IRT.The 24-hour ambulatory blood pressure-monitoring assessment was performed using validated oscillometric recorders (A&D TM- 2430, A&D Inc.,Tokyo,Japan).The 24- hour ambulatory electrocardiography measurement was performed using electrocardiocorder(PI200A-A, QunTian Inc.,Shanghai,China).The BPV ratio and the HRV ratio were assessed in the two groups. Results 24 hours after the intervention, intervention group 24 hours systolic blood pressure variation coefficient and diastolic blood pressure variation coefficient were(10.16 ± 1.95)%,(12.6 ± 7.15)%,the control group, respectively (12.92 ± 2.79)%, (17.38±4.49)%, two groups compare the difference was statistically significant (t=6.450, 4.435, P<0.05). Intervention group of normal sinus R- R period between the standard deviation, to the phase difference between adjacent R- R , root mean square value of the whole difference in more than 50 ms continuous period of percentage between normal R-R and triangle Index were (173.3±58.5) ms, (115.9±74.4), (54.8± 24.1)%, (53.3 ± 15.1).The control group was(128.7 ± 40.2)ms, (82.1 ± 35.2),(39.9 ± 17.1)%, (30.6 ± 14.9), and the difference between the two groups was statistically significant(t=-8.439--3.207, all P<0.05). Conclusions Isometric resistance training conducted using handgrip exercise could increase the HRV and decrease the BPV in hypertensive patients with MIS. These results indicated that the IRT may improve the life quality of hypertensive patients with MIS.
10.Two case reports about immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome and literature review
Wenfang DONG ; Qing CAO ; Jian WANG ; Biru LI
Chinese Pediatric Emergency Medicine 2018;25(10):753-756
Objective To explore the clinical manifestation,laboratory findings,treatment and prog-nosis of immune dysregulation,polyendocrinopathy,enteropathy,X-linked ( IPEX) syndrome,and to improve pediatricians'knowledge of this disease. Methods Clinical data of two cases of IPEX were retrospectively analyzed,and related literatures were reviewed. Results One of the two male children showed severe and early-onset enteropathy,another showed insulin-dependent diabetes onset. Both of them complicated with sep-sis. DNA sequencing of whole-genome exon group showed a mutation in FOXP3 gene. Finally,one of the two IPEX children accepted allogeneic hematopoietic stem cell transplantation( HSCT) . Another one was waiting for the treatment of HSCT. Conclusion IPEX should be considered also in infants with typical symptoms including early-onset refractory diarrhea, multiple endocrine disease and severe recurring infections. Gene sequencing mayl help diagnose the disease. Early HSCT can improve the patients'outcomes.

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