ObjectiveTo construct a family-centered care model for children with tuberculosis based on the double ABC-X model, and to evaluate its clinical effects. MethodsFrom December 2022 to October 2023, 64 newly admitted children with tuberculosis who met the criteria and their caregivers were recruited from the tuberculosis department of Shanghai Public Health Clinical Center were randomly divided into an experimental group (32 cases) and a control group (32 cases).The control group was given a conventional health care, while the experimental group was given a family-centered health care intervention based on the double ABC-X model, in which a multidisciplinary care team provided personalized information and emotional support for the caregivers and their children. Medication adherence of the children, caregiver’s teading burden, and disease management competence were compared between the 2 groups. ResultsA total of 29 cases in the experimental group and 27 cases in the control group completed the intervention. At 12 weeks of intervention, the medication adherence score (7.72±0.45 vs 7.41±0.50, P<0.05) and disease management competence score (36.97±7.85 vs 31.56±7.30, P<0.05) were higher in the experimental group than that in the control group while the caregiving burden score (31.79±13.40 vs 40.04±9.01, P<0.05) and difficulty of disease management score (30.41±12.41 vs 38.56±9.48, P<0.05) were lower than that in the control group. At 24 weeks of intervention, the medication adherence score (7.34±0.97 vs 6.70±1.14, P<0.05) and disease management competence score (42.07±6.93 vs 35.63±7.32, P<0.05) were higher in the experimental group than that in the control group as well, but the caregiving burden score (31.62±11.72 vs 39.63±10.17, P<0.05) and difficulty of disease management score (30.59±10.87 vs 37.81±9.32, P<0.05) were lower than that in the control group. ConclusionFamily-centered care based on the double ABC-X model can effectively promote medication adherence among children with tuberculosis, reduce caregivers’ care burden and disease management difficulties, and improve caregiver’s disease management competence.

Objective:To evaluate the efficacy and safety of chimeric antigen receptor T-cell (CAR-T) therapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with Ph-like acute lymphoblastic leukemia (Ph-ALL) .Methods:Patients with Ph-ALL who underwent CAR-T therapy followed by allo-HSCT from March 2018 to August 2023 at the First Affiliated Hospital of Soochow University were included, and their clinical data were retrospectively analyzed.Results:Of the 21 patients, 14 were male and 7 were female. The median age at the time of CAR-T therapy was 22 (6-50) years. Seven patients had ABL1-like rearrangements, and 14 had JAK-STAT rearrangements. Prior to CAR-T therapy, 12 patients experienced hematologic relapse; 7 were multiparameter flow cytometry minimal residual disease (MFC-MRD) -positive and 2 were MFC-MRD-negative. CAR-T cells were derived from patients’ autologous lymphocytes. Nine patients were treated with CD19 CAR-T cells, and 12 were treated with CD19/CD22 CAR-T cells. After assessment on day 28 after CAR-T therapy, 95.2% of the patients achieved complete remission, with an MRD-negative remission rate of 75%. Nineteen patients developed grade 0–2 cytokine release syndrome (CRS) and 2 patients suffered grade 3 CRS, all cases of which resolved after treatment. All patients underwent allo-HSCT after CAR-T therapy. The median time from CAR-T therapy to allo-HSCT was 63 (38-114) days. Five patients experienced relapse after CAR-T therapy, including four with hematologic relapse and one with molecular relapse. The 3-year overall survival (OS) rates in the ABL1 and JAK-STAT groups were (83.3±15.2) % and (66.6±17.2) %, respectively ( P=0.68) . The 3-year relapse-free survival (RFS) rates were (50.0±20.4) % and (55.6±15.4) % in the ABL1 and JAK-STAT groups, respectively. There was no significant difference in 3-year OS or RFS between the two groups. Conclusions:CAR-T therapy followed by allo-HSCT leads to rapid remission in most patients with Ph-ALL and prolongs leukemia-free survival.

OBJECTIVE: To investigate the effectiveness of sequential plate internal fixation in the correction of Madelung deformity after ulnar osteotomy and shortening. METHODS: The clinical data of 13 patients with Madelung deformity admitted between September 2015 and July 2021 were retrospectively analyzed. There were 5 males and 8 females with an average age of 18.3 years ranging from 17 to 23 years. The disease duration ranged from 12 to 24 months, with an average of 17 months. Three cases had a clear history of trauma. All patients had external radial deviation deformity and limited movement of the ulnar deviation, and the ulnar impact pain was significant during ulnar deviation movement; 9 patients had limited wrist joint supination movement, and the supination movement was normal. In the first stage, ulnar osteotomy and shortening combined with external fixator were used to correct wrist deformity in 13 patients. After operation, bone transfer was performed 6 times per day, with adjustments made every 4 hours, which was 1 mm per day. After the osteotomy was in place, the ulnar plate internal fixation was performed to reconstruct the ulnar stability in the second stage. The Cooney wrist joint score was used to assess the pain, function, range of motion, flexion and extension range of motion, and grip strength of the wrist joint before operation and before the removal of internal fixator. The subjective feeling and appearance satisfaction of patients were recorded. RESULTS: After the second-stage operation, all the 13 patients were followed up 10-22 months, with an average of 15 months. The deformity of wrist joint disappeared after operation, and the flexion, extension, and ulnar deviation were basically normal. There was no complication such as ulnar impingement sign, nonunion or infection. Wrist function, pain, and range of motion were significantly improved after operation, except for 1 patient who had no significant improvement in rotation and pain. The ulnar internal fixator was removed at 10-18 months after the second-stage operation. The scores of pain, function, range of motion, flexion and extension range of motion, and grip strength in the Cooney wrist score before removal of internal fixator significantly improved when compared with those before operation ( P<0.05). Subjective and appearance satisfaction of patients were excellent in 9 cases, good in 3 cases, and fair in 1 case. CONCLUSION Ulnar osteotomy and shortening with sequential plate internal fixation for correction of Madelung deformity, with mild postoperative pain, can effectively avoid bone nonunion, improve wrist joint function, and have significant effectiveness.
Male ; Female ; Humans ; Adolescent ; Retrospective Studies ; Ulna/surgery* ; Osteochondrodysplasias ; Radius Fractures/surgery* ; Wrist Joint/surgery* ; Osteotomy ; Range of Motion, Articular ; Treatment Outcome

CRISPR-Associated Protein 9 ; Gene Editing ; CRISPR-Cas Systems/genetics*

Venous thromboembolism (VTE) is a complication in children with acute lymphoblastic leukemia (ALL). The Chinese Children's Cancer Group-ALL-2015 protocol was carried out in China, and epidemiology, clinical characteristics, and risk factors associated with VTE were analyzed. We collected data on VTE in a multi-institutional clinical study of 7640 patients with ALL diagnosed in 20 hospitals from January 2015 to December 2019. First, VTE occurred in 159 (2.08%) patients, including 90 (56.6%) during induction therapy and 108 (67.92%) in the upper extremities. T-ALL had a 1.74-fold increased risk of VTE (95% CI 1.08-2.8, P = 0.022). Septicemia, as an adverse event of ALL treatment, can significantly promote the occurrence of VTE (P < 0.001). Catheter-related thrombosis (CRT) accounted for 75.47% (n = 120); and, symptomatic VTE, 58.49% (n = 93), which was more common in patients aged 12-18 years (P = 0.023), non-CRT patients (P < 0.001), or patients with cerebral thrombosis (P < 0.001). Of the patients with VTE treated with anticoagulation therapy (n = 147), 4.08% (n = 6) had bleeding. The VTE recurrence rate was 5.03% (n = 8). Patients with VTE treated by non-ultrasound-guided venous cannulation (P = 0.02), with residual thrombus (P = 0.006), or with short anticoagulation period (P = 0.026) had high recurrence rates. Thus, preventing repeated venous puncture and appropriately prolonged anticoagulation time can reduce the risk of VTE recurrence.
Humans ; Child ; Venous Thromboembolism/etiology* ; East Asian People ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/epidemiology* ; Risk Factors ; Thrombosis/chemically induced* ; China/epidemiology* ; Anticoagulants/adverse effects* ; Recurrence

Objective: To observe the clinical efficacy of umbilical therapy with herbal cakes made of Ge Gen Qin Lian Tang prescription of different dosages in treating damp-heat diarrhea in young children. Methods: Seventy-two kids with diarrhea of damp-heat pattern were recruited and divided into a high-dosage group and a low-dosage group using the random number table method, with 36 cases in each group. They all received conventional antidiarrheal treatment and umbilical application with herbal cakes. However, the herbal cakes for the high-dosage group were made of the mixture of Ge Gen Qin Lian Tang powder and water, and those for the low-dosage group consisted of 10％ Ge Gen Qin Lian Tang powder and 90％ auxiliary materials (corn starch) plus water. The treatment duration was 3 d. The clinical efficacy, antidiarrheal rate, effective rate for symptoms and signs of traditional Chinese medicine (TCM), TCM symptoms score, and safety indicators were compared between the two groups. Results: After the treatment, the TCM symptoms scores dropped significantly in both groups (P<0.01) and were lower in the high-dosage group than in the low-dosage group (P<0.05). The clinical efficacy was more significant in the high-dosage group than in the low-dosage group after the treatment, and the between-group difference was statistically significant (P<0.05); the antidiarrheal rate was markedly higher in the high-dosage group than in the low-dosage group (P<0.05). Regarding the TCM symptoms and signs, the high-dosage group showed better results in improving the greasy and yellowish tongue coating, bowel movement frequency, watery excrement, short and dark urine, red tongue body, red anus, vomiting, bowel sounds, and abdominal bloating compared with the low-dosage group, and the between-group differences were statistically significant (P<0.01). Conclusion: Umbilical therapy with herbal cakes made of Ge Gen Qin Lian Tang is safe, reliable, and effective in treating damp-heat diarrhea in young children; the high-dosage herbal cakes produce more significant efficacy than the low-dosage ones and are worth further investigation.

Standardized specialist training is key to postgraduate medical education. China is introducing the specialist training programs, yet without covering specialist training in obstetrics and gynecology(O&G). Shanghai took the lead in 2003 to pilot O&G specialists training programs within the region. This study introduced the specialist training programs in O&G in the US, and compared them with those in Shanghai, and recommending on launching O&G specialist training nationwide based on China′s specifics.

Objective:To explore the effect of dialectical diet on traditional Chinese medicine (TCM) syndrome score of cirrhotic ascites patients based on "Gu Ben Kai Qu" theory.Methods:From March 2019 to January 2020, 84 patients with liver cirrhosis and ascites admitted to Shuguang Hospital Affiliated to Shanghai University of TCM were randomly divided into two groups according to the different dialectical types of the subjects, 14 cases in each group. Three non-syndrome differentiation diet groups were given routine nursing care of liver cirrhosis ascites. On the basis of routine nursing, the corresponding medicinal diet was selected according to syndrome differentiation based on "Gu Ben Kai Qu" theory. Patients with spleen and kidney yang deficiency syndrome selected Shenqi lean meat decoction. Patients with Yin deficiency of liver and kidney selected Wolfberry and ophiopogon spareribs decoction. Patients with qi stagnation and blood stasis syndrome selected Danggui Sanqi spareribs decoction. The TCM syndrome score scale for liver disease and the curative effect evaluation of cirrhosis ascites were used to evaluate the effect.Results:Eighty effective cases were included. On the first day of admission, the 14th day and the second week after discharge, the TCM syndrome scores of liver disease were as follows: the group (a1b1) with the spleen and kidney yang deficiency syndrome was 46.38±8.56, 34.20±8.42, 31.40±4.22, respectively. The group (a1b2) with the liver kidney yin deficiency syndrome was 41.50±8.71, 31.35±8.63, 31.12±4.94. The group(a1b3) with the qi stagnation and blood stasis syndrome was 45.92±7.86, 35.17±7.57, 30.83±7.32, respectively. The non-syndrome differentiation diet group (a2b1) with the spleen and kidney yang deficiency syndrome was 46.29±8.38, 39.79±7.65, 36.64±6.83, respectively. The non-syndrome differentiation diet group (a2b2) with the liver and kidney yin deficiency syndrome was 40.50±8.12, 38.10±8.93, 35.38±8.24, respectively. The non-syndrome differentiation diet group (a2b3) with the qi stagnation and blood stasis syndrome was 45.62±7.99, 41.83±7.31, 38.83±7.96, respectively. The comparison of TCM syndrome scores of liver disease at three time points was statistically significant ( χ2 value was 63.998, P<0.05), and the comparison between groups was statistically significant ( χ2 value was 20.993, P<0.05). On the 14th day and the second week after discharge, there were significant differences between the groups with the syndrome differentiation diet and another three groups with non-syndrome differentiation diet ( F values were 3.244, 3.489, all P<0.05). Conclusions:Based on the theory of "strengthening the foundation and opening channels", the syndrome differentiation group can effectively reduce the TCM syndrome score of patients with cirrhosis ascites, improve the symptoms and enhance the curative effect. With the development of time, the score of TCM syndrome in patients with liver disease become lower. On the 14th day of admission, patients with Yin deficiency of liver and kidney given medicated diets had significant effect; patients with spleen kidney yang deficiency syndrome or qi stagnation and blood stasis had significant effect in 2 weeks after discharge; which can effectively improve the clinical symptoms of patients with cirrhosis ascites to worthy of clinical application.

OBJECTIVE：To study the effects of augmented renal clearance （ARC）on blood trough concentration of patients receiving high-dose regimen of teicoplanin. METHODS ：Patients who received high-dose regimen of teicoplanin in the ICU were prospectively collected from the Affiliated Suzhou Hospital of Nanjing Medical University/Suzhou Municipal Hospital during Jul. 2018-Jun. 2020. They were divided into ARC group and normal renal function group according to corrected creatinine clearance. The dosage regimen of teicoplanin in the two groups were loading dose of 600 mg，q12 h×3 doses，maintenance dose of 6-10 mg/kg，qd，and the dosage was adjusted in combination with creatinine clearance rate and blood trough concentration. The trough concentration of blood samples which were collected 30 min before the 4th and 8th-10th dosage of teicoplanin were determined by HPLC. Trough concentration ，clinical efficacy ，Gram-positive bacterial clearance rate and the occurrence of ADR were compared between 2 groups. RESULTS ：A total of 56 patients were included and divided into ARC group （18 cases）and normal renal function group （38 cases）. ARC group had younger age （P＜0.001）and lower serum albumin level （P＝0.025）than normal renal function group. The trough concentrations before administration of the 4th and 8th-10th dosage in ARC group were lower than normal renal function group （P＝0.034；P＝0.035）. The trough concentrations in the ARC group and normal renal function group before 8th-10th dosage were all higher than 30 min before the 4th dosage （P＝0.003；P＜0.001）. The clinical efficacy rate and the clearance rate of Gram-positive bacteria in ARC group were 77.8％ and 76.2％，which were lower than those of the normal renal function group ，but there was no statistical difference （P＝0.195；P＝0.223）. There was no liver function damage ，hemocytopenia and allergic reaction in both groups ，but in the normal renal function group ，the causal relationship between acute renal damage and teicoplanin was assessed as “very likely ”in one patient. CONCLUSIONS ：ARC patients are younger ，most of them have hypoproteinemia，and the blood trough concentrations of teicoplanin in high-dose regimen are significantly lower than those of normal renal function patients. For critical ill ARC patients ，it is advisable to increase the loading dose of teicoplanin to make the trough concentration reach the target concentration range quickly.

Objective:To analyze the correlation between clinical phenotypes and genotypes in 6 children with primary distal renal tubular acidosis (dRTA).Methods:The clinical data of 6 children confirmed as dRTA in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from November 2017 to August 2019 were collected, and related auxiliary examination was performed to assess their growth and development.The venous whole blood was reserved for Trio whole exome sequencing, and full spectrum genetic disease accurate diagnosis cloud platform was applied to systematic data screening and analysis.The suspected mutations were checked by Sanger sequencing, and then the role of protein was predicted by software.Results:Clinical manifestations, signs and auxiliary examination results of the 6 children accorded with the diagnostic criteria of dRTA, and the prominent characteristics was growth retardation.One case had knee valgus, one had osteoporosis, and the auxiliary examination results showed that both of them had alkaline urine, metabolic acidosis, and hypokalemia.Three children had nephrocalcinosis, and 2 children had nephrolithiasis.The parents of the 6 patients were all normal without phenotypes.Mutations in the SLC4A1 gene were identified in 4 patients, including 1 child with a reported homozygous autosomal recessive missense mutation(c.2102G>A, p.G701D), who had dRTA and hemolytic anemia, and 3 children with the reported de novo heterozygous autosomal dominant missense mutation(c.1766G>A, p.R589H, c.1765C>T, p.R589C), whose age at diagnosis was related to abnormal renal imaging.Compound heterozygous autosomal recessive mutations in the ATPV1B1 gene were identified in 1 patient, and they were novel heterozygous missense mutations (1153C>A, p.P385T and c. 806C>T, p.P269L). A novel homozygous autosomal recessive missense mutation was identified in 1 patient in the ATPV0A4 gene(c.1899C>A, p.Y633X, 208). Conclusions:Mutations in SLC4A1, ATP6V1B1, ATP6V0A4 genes are identified as the main causes of the primary dRTA, and the phenotypes was related to the mutation features and genotypes.Genetic test should be conducted on patients suspected as dRTA for early molecular diagnosis, thereby improving clinical phenotypic screening and individualized treatment.