Objective To investigate the efficacy and safety of enriched-calorie formula in post-operative infants with congenital heart disease and malnutrition.Methods All malnourished infants less than 6 months diagnosed congenital heart disease: ventricular septal defect and had undergone surgery in Guangzhou Women and Children`s Medical Center from December 1,2014 to May 30,2015 were included in this study.All cases were randomly divided into intervention group(energy-enriched formula,intervention group)and control group(standard formula,control group)for enteral nutrition intervention and observed for 3 months.Body mass,body length,upper arm circumference,blood prealbumin(PA),retinol binding protein(RBP),and B-terminal pro-brain natriuretic peptide(NT-proBNP)were measured before and after ICU,after discharge,and 1 month and 3 months after operation.Results Fifty-one cases were in intervention group and 50 cases in control group,respectively.There were no significant differences in body mass,body length,arm circumference,PA,RBP,mean enteral nutrition starting time,mechanical ventilation time,length of ICU stay,hospitalization time,and average fluid intake between the two groups(all P>0.05).The average caloric intake in intervention group was significantly higher than in control group [(437.24±6.68)kJ vs.(312.43±86.22)kJ,P=0.001].There was no significant difference in NT-proBNP,PA,and RBP at different time points between the two groups(all P>0.05).The improvement of nutrition in intervention group was significantly higher than that in control group at 1 month(25.0％vs.4.9％,P=0.011)and 3 months(64.1％vs.15.7％,P<0.001)after operation.Body mass increased in intervention group [(0.067±0.348)kg] compared with that in control group,and decreased [(0.125±0.425)kg] in control group(P=0.015).Body weight[(5.46±1.36)kg vs.(4.80±1.01)kg,P=0.008],weight for age Z score(WAZ)(-2.79±1.28 vs.-3.75±1.27,P<0.001),and height for weight Z score(WHZ)(-2.47±1.43 vs.-3.62±1.77,P=0.001)one month after surgery were significantly higher than those before operation.Body weight [(6.78±1.42)kg vs.(5.72±1.01)kg,P<0.001] arm circumference [(12.80±1.17)cm vs.(12.00±0.90)cm,P<0.001],WAZ(-1.60±1.17 vs.-3.10±1.40,P<0.001),height for age Z score(HAZ)(-1.41±1.63 vs.-2.10±1.41,P=0.034),and WHZ(-0.86±1.31 vs.-2.59±2.13,P<0.001)of the two groups at 3 months postoperative were significantly higher than those before operation,and the growth rate of intervention group was faster than control group.There were no significant adverse reactions in both groups.Conclusion Calorie-enriched formula powder can help malnourished infants to catch up after congenital heart disease surgery.

Objective     To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods     The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results     There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion     There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.

Morita therapy has been bom for more than 100 years.Inpatient Morita therapy is highly oper-able and easy to master.It can improve many refractory neuroses through four-stage treatment.But more neuroses are treated in outpatient clinics,and Morita therapy cannot be used in hospitalized patients.Therefore,the formula-tion of expert opinions on outpatient operations is particularly important.This paper is based on domestic and for-eign references,and after many discussions by domestic Morita therapy experts,and then drew up the first version of the expert opinions on operation of outpatient Morita therapy.Meanwhile the operation rule of Morita therapy in three stages of outpatient treatment was formulated:in the etiological analysis stage,under the theoretical guidance of Morita therapy,analyze the pathogenic factors,to improve treatment compliance and reduce resistance;during the operating stage,guide patients to engage in constructive and meaningful actions,realizing the achievement of letting nature take its course principle;in the cultivating character and enriching life stage,pay attention to positive infor-mation,expanding the scope and content of actions,improving the ability to adapt to complex life,and preventing recurrence caused by insufficient abilities.It will lay a foundation for the promotion of Morita therapy in domestic outpatient clinics,so that more patients with neurosis and other psychological diseases could receive characteristic Morita therapy treatment in outpatient clinics.

OBJECTIVETo detect memory B lymphocyte (Bm) in peripheral blood (PB) of immune-related pancytopenia (IRP).

METHODS86 patients with IRP and 11 health volunteers were enrolled in this study. Bm (CD5⁺ CD19⁺ CD27⁺) and bone marrow mononucleated cell antibodies (BMMNC-Ab) were determined via fluorescence-activated cell sorting, and clinical outcomes of these patients were analyzed.

RESULTS(1)43 initial patients achieved obvious remission in all 52 initial cases after conventional immunosuppression therapy. 16 relapsed patients with IRP received Rituximab (RTX) and 14 cases achieved obvious remission, among which 7 cases were refractory to conventional immunosuppression therapy, 5 cases exhibited obvious remission, and 2 cases did not respond. Other 18 relapsed cases received conventional immunosuppression therapy and 13 cases achieved obvious remission. (1)The level of Bm in PB in 52 initial patients with IRP was(1.81 ± 0.97)%, and no significant difference was observed between the initial patients and health volunteers (1.75 ± 0.55)% (P>0.05). The level of Bm in PB in 34 relapsed patients with IRP was obviously higher than that in the initial IRP patients and health volunteers (P<0.05). Significant difference was observed in the level of Bm in PB in 16 relapsed IRP patients between pre-therapy and post-therapy with RTX (P<0.05). No statistical difference was found between the remission and no-response groups in relapsed patients treated with RTX. RTX regimen produced more effective outcome than conventional immunosuppression therapy, which better eliminated Bm than the latter (P<0.05). Initial patients with IRP who relapsed within a two-year follow-up period had a lower level of Bm in PB compared with un-relapsed patients (P<0.05). Majority of BMMNC- Ab antibodies in relapsed patients were IgG (82.4%) and IgM (69.2%) autoantibodies in patients with initial IRP.

CONCLUSIONThe level of Bm in PB was associated with relapsed patients with IRP. Bm did not respond to conventional immunosuppression therapy,but responded to RTX.

Adolescent ; Adult ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; B-Lymphocyte Subsets ; immunology ; Female ; Humans ; Immunologic Memory ; Immunosuppression ; Male ; Middle Aged ; Pancytopenia ; immunology ; therapy ; Recurrence ; Rituximab ; Treatment Outcome ; Young Adult

OBJECTIVETo investigate the changes of relative telomere length (RTL) of peripheral blood (PB) CD3⁺, CD3⁺CD4⁺, CD3⁺CD8⁺T lymphocytes, CD19⁺B lymphocytes and bone marrow (BM) CD34⁺ cells and its association with disease severity in untreated patients with immuno-related pancytopenia (IRP).

METHODSThe PB CD3⁺ , CD3⁺ CD4⁺ , CD3⁺ CD8⁺ T lymphocytes, CD19⁺ B lymphocytes, and BM CD34⁺ cells were purified by magnetic activated cell sorting (MACS), and RTL were measured with flow-fluorescence in situ hybridization (FLOW-FISH).

RESULTSThe RTL of CD3⁺, CD3⁺CD4⁺ , and CD3⁺CD8⁺T lymphocytes in untreated IRP patients were (27.754 ± 16.323)%, (7.526 ± 3.745)% and (25.854 ± 14.789)%, respectivly, which were significantly shorter than those in healthy-controls (54.555 ± 19.782)%, (12.096 ± 2.805)%, and (38.367 ± 4.626)% (P<0.05). The RTL of CD19⁺ lymphocytes in untreated IRP patients was (22.136 ± 16.142)%, which was significantly shorter than that in healthy controls (42.846 ± 16.353)% (P<0.01). There was no significant difference of BM CD34⁺ cells RTL between the untreated IRP patients (22.528 ± 21.601)% and the healthy controls (23.936 ± 19.822)% (P>0.05). There were significantly positive correlations between the RTL of B lymphocytes and the count of white blood cell (r=0.706, P=0.015). There were negative correlations between RTL of B lymphocytes and the clinical symptoms (r=-0.613, P=0.045) and positive correlations with therapeutic effect (r=0.775, P=0.005).

CONCLUSIONThe shorter RTL of CD3⁺, CD3⁺CD4⁺, CD3⁺CD8⁺, CD19⁺ lymphocytes, and the normal RTL of BM CD34⁺ cells in untreated IRP patients were identified, which might imply that IRP is a type of acquired autoimmune diseases.

Adolescent ; Adult ; B-Lymphocyte Subsets ; immunology ; Child ; Female ; Humans ; Lymphocytes ; ultrastructure ; Male ; Middle Aged ; Pancytopenia ; immunology ; pathology ; T-Lymphocyte Subsets ; immunology ; Telomere ; ultrastructure ; Young Adult