The male patient was referred to the hospital at 44 days old due to dyspnea after birth and inability to wean off oxygen. His brother died three days after birth due to respiratory failure. The main symptoms observed were respiratory failure, dyspnea, and hypoxemia. A chest CT scan revealed characteristic reduced opacity in both lungs with a "crazy-paving" appearance. The bronchoalveolar lavage fluid (BALF) showed periodic acid-Schiff positive proteinaceous deposits. Genetic testing indicated a compound heterozygous mutation in the ABCA3 gene. The diagnosis for the infant was congenital pulmonary alveolar proteinosis (PAP). Congenital PAP is a significant cause of challenging-to-treat respiratory failure in full-term infants. Therefore, congenital PAP should be considered in infants experiencing persistently difficult-to-treat dyspnea shortly after birth. Early utilization of chest CT scans, BALF pathological examination, and genetic testing may aid in early diagnosis.
Infant ; Infant, Newborn ; Humans ; Male ; Bronchoalveolar Lavage/adverse effects* ; Pulmonary Alveolar Proteinosis/pathology* ; Dyspnea/etiology* ; Respiratory Insufficiency

Objective: To evaluate the efficacy and prognosis of basiliximab in the treatment of steroid-refractory or steroid-dependent acute graft-versus-host disease (SR/SD-aGVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) . Methods: Clinical data of 87 patients with SR/SD-aGVHD in the skin, intestine, and liver after allo-HSCT at the Institute of Hematology & Blood Diseases Hospital Transplantation Center from January 2015 to December 2018 were retrospectively analyzed. The administration plan of basiliximab was as follows: 20 mg for adults and children weighing ≥35 kg and 10 mg for children weighing<35 kg. The drug was administered once on the 1st, 4th, and 8th days, respectively, and then once weekly. The efficacy was evaluated on the 7th, 14th, 21st, and 28th days after basiliximab treatment. Results: ①There were 51 males (58.6%) and 36 females (41.4%) , with a median (range) age of 34 (4-63) years. There were 54 cases of classic aGVHD, 33 of late aGVHD, 49 of steroid-refractory aGVHD, and 38 of steroid-dependent aGVHD. ②Thirty-five patients (40.2%) achieved complete remission (CR) , 23 (26.4%) achieved partial remission (PR) , and 29 had no remission (NR) . The total effective rate[overall response rate (ORR) ] was 66.7% (58/87) . ③The ORR of the classic and late aGVHD groups was 77.8% (42/54) and 48.5% (16/33) , respectively. ④The median (range) follow-up time was 154 (4-1813) days, the 6-month overall survival (OS) rate of the 87 patients was 44.8% (95% CI 39.5%-50.1%) and the 1-year OS was 39.4% (95%CI 34.2%-44.3%) . ⑤After treatment with basiliximab, the 6-month OS in the CR (35 cases) , PR (23 cases) , and NR (29 cases) groups was 80.0% (95%CI 73.2%-86.8%) , 39.1% (95%CI 28.9%-49.3%) , and 6.9% (95%CI 2.2%-11.6%) , respectively (χ(2)=34.679, P<0.001) , and the 1-year OS was 74.3% (95%CI 66.9%-81.7%) , 30.4% (95%CI 20.8%-40.0%) , and 3.4% (95%CI 0%-6.8%) , respectively (χ(2)=43.339, P<0.001) . The OS of the classic and late aGVHD groups was 57.4% (95%CI 50.7%-64.1%) and 24.2% (95%CI 16.7%-31.7%) , respectively (χ(2)=9.109, P=0.004) , and the 1-year OS was 51.9% (95%CI 45.1%-58.7%) and 18.2% (95%CI 11.5%-24.9%) , respectively (χ(2)=9.753, P=0.003) . ⑥Univariate and multivariate analyses showed that late aGVHD (OR=3.121, 95%CI 1.770-5.503, P<0.001) , Minnesota score high-risk group before medication (OR=3.591, 95%CI 1.931-6.679, P<0.001) , active infection before medication (OR=1.881, 95%CI 1.029-3.438, P=0.040) , and impairment of important organ function caused by non-GVHD (OR=3.100, 95%CI 1.570-6.121, P=0.001) were independent risk factors affecting the efficacy of basiliximab. Conclusion: Basiliximab has good efficacy and safety for SR/SD-aGVHD, but not in patients with late aGVHD, high-risk group of Minnesota score, and infection or impaired function of important organs.
Acute Disease ; Adult ; Basiliximab/therapeutic use* ; Child ; Female ; Graft vs Host Disease/drug therapy* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Steroids/therapeutic use*

Objective: To assess the diagnostic values of ¹¹C-methionine (MET) PET/CT semiquantitative parameters for detecting recurrence in patients who were diagnosed with suspicious recurrence by MRI after resection of supratentorial gliomas. Methods: A total of 164 patients (107 males, 57 females, age 6-74 years; high-grade 94, low-grade 63, unclear 7) with supratentorial gliomas who underwent ¹¹C-MET PET/CT between June 2015 and June 2017 in Beijing Tiantan Hospital were enrolled respectively. All patients were with suspicious recurrence after surgery showed by MRI and followed up for 6 months at least. The final diagnosis was determined with histopathological analysis or clinical follow-up. The maximum and mean standardized uptake value (SUV_max and SUV_mean), tumor-to-background ratios (TBR) of SUV_max and SUV_mean (TBR_max and TBR_mean) were recorded and compared between patients with recurrence or without recurrence using independent-sample t test. Receiver operating characteristic (ROC) curves were drawn to determine the threshold, and the diagnostic sensitivity and specificity of each parameter were calculated. Results: According to the clinical diagnosis, there were 139 patients with recurrence and 25 without recurrence. SUV_max, SUV_mean, TBR_max and TBR_mean were significantly higher for patients with recurrence than those without recurrence (4.19±1.95 vs 2.59±1.18, 2.34±1.08 vs 1.46±0.72, 2.95±1.17 vs 1.83±0.79, 2.64±1.11 vs 1.59±0.71; t values: 5.126-6.183, all P<0.01), but there was no difference in the areas under the ROC curve (AUC) for diagnosis of recurrence with the 4 parameters (z values: 0.265-1.674, all P>0.05), for which the optimal cut-off values were 3.05, 1.65, 1.96 and 1.79, respectively, and the corresponding sensitivities/specificities for the diagnosis of recurrence were 67.6%(94/139)/100%(25/25), 67.6%(94/139)/100%(25/25), 79.9%(111/139)/100%(25/25), 74.8%(104/139)/100%(25/25), respectively. Patients with (n=81) or without (n=13) recurrence had different semiquantitative parameters in high-grade glioma group (t values: 5.137-5.871, all P<0.01), and the AUC for TBR_mean was greater than that for SUV_mean (0.858 vs 0.802; z=1.982, P<0.05). Patients with (n=54) or without (n=9) recurrence in low-grade glioma group showed significant difference in the 4 parameters (t values: 2.730-7.009, all P<0.01), while the AUCs of the 4 parameters were not significantly different (z values: 0.444-1.407, all P>0.05). Among 37 patients with recurrence confirmed by pathology, there were no significant differences in the semiquantitative parameters between the high-grade and low-grade glioma groups and AUCs were not different either (t values: 1.387-1.937, z values: 0.106-1.752, all P>0.05). Conclusions Semiquantitative parameters of ¹¹C-MET PET/CT are equally accurate in the differentiation of recurrence from radiation injury in patients with gliomas, while TBR_mean was superior than SUV_mean in patients with the high-grade gliomas. Among the patients with recurrence confirmed by pathology, the value of the semiquantitative parameter is limited for the identification of high- and low- grade gliomas.

AIM:To observe the concentration of RBP4 and IL-6 in vitreous of proliferative diabetic retinopathy (PDR). METHODS: A total of 65 patients (66 eyes) were enrolled in Department of Ophthalmology, Renmin Hospital of Wuhan University from February 2017 to July 2017 with the informed consent. The patients were divided into PDR group (23 cases) and NPDR group (16 cases). Twenty- six patients without diabetic mellitus (DM) served as control group. The demography was matched among the groups, but the course of DM, the blood glucose level and the HbA1c level were elevated in the PDR group and the NPDR group (all P<0.05). Vitreous samples were collected during the procedure of vitrectomy. RBP4,IL-6,TNF-α concentrations in vitreous specimens were detected by ELISA. The differences of vitreous RBP4, IL-6 and TNF-α in various groups were statistically analyzed by ANOVA, respectively. The correlations between RBP4 and IL - 6, TNF - α were calculated by Pearson correlation analysis. RESULTS:The concentration of RBP4 in PDR group,the NPDR group and control group were 13.68士2.66, 11 03士1 12,10.45士1.17μ g/Ml, and the concentration of IL-6 were 56.0士10.27, 20.92士5.77, 10.26士1.91pg/Ml. RBP4 and IL- 6 concentrations were elevated in PDR group compared with NPDR group and control group, with significant difference among three groups (F = 12. 135, 161.167; P < 0. 01). IL - 6 concentrations in vitreous increased in the NPDR group in comparison with control group(P<0.05). RBP4 concentrations had no significant difference between the NPDR group and the group(P>0 05). Pearson correlation coefficient was significant positive between RBP4 concentration and IL - 6 concentration(r=0.606,P=0.001). CONCLUSION: RBP4 is probability involved in the inflammation pathogenesis of PDR. These results indicate that RBP4 could be a new target for the diagnosis and treatment of PDR.

Objective: To evaluate the prognostic significance of early phase full donor chimerism (FDC) after myeloablative allogeneic peripheral blood stem cell transplantation (allo-PBSCT). Methods: The clinical data of 72 hematological patients received myeloablative allo-PBSCT from Feb. 2016 to Jul. 2017 were analyzed retrospectively. The median age was 36.5 years (range 4-59), 44 were males and 28 females. Of the donors, there were 35 HLA matched sibling donors, 27 haploidentical donors and 10 unrelated donors. Polymerase chain reaction amplification of short tandem repeat sequence (PCR-STR) was used to detect donor cell chimerism (DC) rate of recipient bone marrow at one, two and three months after transplantation. Results: The median follow-up was 462 d (range: 47-805 d), 55 cases were still alive, and 45 cases were disease-free survival (DFS) at the end of follow-up. The 2-year overall survival (OS) and DFS were (68.9±7.7)% and (59.5±6.3)%, respectively. A number of 16 cases underwent relapses, with 2-year cumulative incidence of (24.1±5.3)%. The median time of recurrence was 157(32-374) d. Forty cases (55.6%) developed acute graft-versus-host diseases (aGVHD), with median time of 35.5 (13-90) d. Chronic GVHD (cGVHD) occurred in 23 patients (31.9%), with median time of 169 (94-475) d. Univariate analysis found the following factors were not related to OS, DFS or relapse rate (RR), including age, sex, blood type and sex of donor-recipient, occurrence of aGVHD and cGVHD. The OS and DFS in cases reached FDC and no FDC at two months after transplantation were (85.2±6.9)% vs (66.1±7.7)% (P=0.051) and (76.7±7.7)% vs (48.9±8.1)% (P=0.021), respectively. The RR rate in FDC group was lower than that in no FDC group [(16.6±6.8)% vs (30.4±7.8)%, P=0.187, respectively]. Conclusion: The present study confirmed the important value for predicting the prognosis with whether or not the patients reached FDC at the early phase after allo-PBSCT. The OS and DFS in cases with FDC at two months after transplantation were significantly higher than those of no FDC patients.
Adolescent ; Adult ; Child ; Child, Preschool ; Chimerism ; Female ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Middle Aged ; Peripheral Blood Stem Cell Transplantation ; Prognosis ; Retrospective Studies ; Young Adult

Discharge against medical advice (DAMA) conflicts with the purpose of disease treatment in children. Some research has shown that there are high proportions of extremely preterm infants and infants with asphyxia or congenital malformation in neonates with DAMA. This suggests that the sustainable development of neonatology needs cooperation and co-development with obstetrics, neonatal surgery, and radiology to reduce the rate of DAMA. With reference to the current status of research in both China and other countries, this article reviews the causes for DAMA and the strategies for reducing the rate of DAMA, in order to provide a theoretical basis for effectively reducing the rate of DAMA from the neonatal intensive care unit, improving treatment outcomes of the neonates, and increasing hospitals' comprehensive benefits.
Ethics, Medical ; Health Services Needs and Demand ; Humans ; Infant, Newborn ; Insurance, Health ; Intensive Care Units, Neonatal ; Patient Discharge ; Prenatal Care ; Treatment Refusal

Objective To investigate the influence of high intensity interval exercise (HIIT) on peroxidation and vascular endothelial function for experimental hyperhomocysteinemia (HHcy) rats. Methods Thirty five male rats were randomly divided into 4 groups. Control group (n=8) was given ordinary feed. High methionine group (n=27) was given 3% methionine on this basis, and divided into model group, folic acid group and HIIT+ folic acid group, with 9 rats per group for 16 weeks. Serum homocysteine (Hcy) , content of plasma malondialdehyde (MDA) , hydroxyl radical (OH-), total antioxidant capacity (T-AOC), activity of glutathione peroxidase (GSH-PX) and superoxide dismutase (SOD) were measured, as well as the level of Nitric Oxide (NO), Nitric Oxide Synthase (NOS) and Endothelin 1 (ET-1) . The pathology of abdominal aortas was analyzed.Results Sixteen weeks after intervention, there was no significant difference between HIIT + folic acid group and the control group (P>0.05) . The levels of serum Hcy in the model group, folic acid group and the HIIT+folic acid group were (23.95±3.35) μmol/L,(8.73±0.60) μmol/L, and (6.19±0.34) μmol/L respectively (P<0.05) . Sixteen weeks after intervention, the content of MDA in HIIT+ folic acid group reduced, and there was no significant difference compared with the control group (P>0.05). The level of SOD and GSH-PX increased in HIIT+ folic acid group and folic acid group, and there was a significant difference compared with the model group. There were significant differences in activities of SOD and GSH-PX in HIIT+ folic acid group when compared with folic acid group (P<0.05). Compared with the control group, there were significant differences in levels of ET-1, NOS and NO in folic acid group (P<0.05), while there was no significant difference in the level of ET-1 and NOS between HIIT+folic acid group and control group (P>0.05) . Mild atherosclerotic lesions were observed in the HIIT+folic group. Conclusion High methionine diet can reduce the level of serum Hcy in HHcy rats, and high intensity interval exercise combined with folic acid intervention could reduce the level of serum Hcy, improve oxidative stress state, reduce the injury of endothelial function, and thus to alleviate atherosclerotic lesion.

This study was purposed to investigate the engraftment, graft-versus-host disease (GVHD), transplantation related mortality (TRM), relapse and survival in hematologic patients received unrelated umbilical cord blood transplantation (UCBT). A total of 25 patients with hematological disease underwent UCBT, including 8 pediatric and 17 young adult patients. Among them 3 cases received single unit of UCBT and 22 cases received double units of UCBT. For donor/recipients human leukocyte antigen (HLA) matching: HLA 6/6 loci matched in 9 cases, HLA 4-5/6 loci matched in 16 cases. There were 19 patients with hematologic malignancies, including 3 cases in the period of disease progression and 6 cases of non-hematologic malignancies. Conditioning regimens were TBI/Cy ± Flu ± ATG or BuCy ± Flu ± ATG for 21 patients and Cy+Flu+ATG for 4 patients. For prophylaxis of acute graft-versus-host disease (aGVHD) the regimen of cyclosporine (CsA) as dominant drug was used. The results showed that among 16 patients (80.0%) achieved engraftment, 20 patients survived for more than 42 d after transplantation. The cumulative neutrophil recovery rate on day 42 after transplant was 64.0%, with a median time of 17.0 d;the cumulative platelet recovery rate on day 100 after transplant was 60.0 %, with a median time of 35.0 d. The cumulative rate of grade II-IV and III-IV aGVHD after transplantation 100 d was 44.0% and 30.7%, respectively. Until the end of the follow-up, the cumulative rate of TRM was 54.3%. For all the patients, overall survival rate was 42.7%. Out of 17 evaluable patients with hematologic malignancies 7 cases (41.2%) survived to date, and only 1 case relapsed, so event-free survival rate was 35.3%. Out of 5 evaluable patients with non-hematologic malignancies, 4 patients survived and 2 patients were in stable engraftment state, 2 cases with autologous hematopoietic recovery. Among 3 cases of hematologic malignancies at advanced stage, only 1 case survived to date. It is concluded that HLA-4-6/6 loci matched UCBT is an effective option to treat hematological diseases. Double cord blood transplantation (dUCBT) can overcome the disadvantage of insufficient cells of single cord blood UCBT to treat overweight children and adult.
Adolescent ; Adult ; Child ; Child, Preschool ; Cord Blood Stem Cell Transplantation ; methods ; Female ; Fetal Blood ; Graft vs Host Disease ; Hematologic Diseases ; therapy ; Histocompatibility Testing ; Humans ; Male ; Survival Rate ; Young Adult

Objective To study the optimal waist circumference (WC) cut-off values for identifying metabolic risk factors in middle-aged and elderly subjects in Shandong Province of China.
Methods A total of 2 873 men and 5 559 women were included in this cross-sectional study. Metabolic syndrome (MetS) was diagnosed according to the definition of Chinese Diabetes Society in 2004. The relation between WC and MetS was analyzed by multivariate logistic regression analysis. The optimal WC cut-off values were identified using the area under the ROC curve and the different diagnostic criteria for central obesity were compared.
Results The WC was the risk factor for MetS independent of BMI, blood glucose, blood lipid, and blood pressure. The optimal WC cut-off value was 83.8 cm and 91.1 cm for identifying MetS in women and men, respectively. Compared with 80 cm and 85 cm for women and men, 85 cm and 90 cm had a higher Youden index for identifying all metabolic risk factors and MetS in women and men.
Conclusion The appropriate WC cut-off value is 85 cm and 90 cm for identifying central obesity and MetS in women and men in Shandong Province of China.

The study of hemodynamics, which refers to dynamics inside the blood circulation, mainly includes the flow rate, flow resistance, pressure, shear stress, disturbed flow, as well as their associations in between. Therefore, with its important significance in the clinical treatments of vessel curvature, arterial stenosis or occlusion, pathological artery branches and aneurism, study about hemodynamics is essential to human health. Currently, extensive researches on hemodynamics have been conducted with respect to artery bypass, coronary arterial stenosis, abdominal aortic aneurysm, atherosclerosis, cerebral aneurysm and swirling flow. With the development of such research on hemodynamics, surgical planning and interventional therapy have improved rapidly. The influence mechanism of hemodynamic parameters, including pressure, flow resistance, flow rate, wall shear stress, blood viscosity, flow separation, turbulent flow, vortex on the post-operation complications could be deeply explored with the help of more and more clinical apparatus and have gained some achievements.