A boy, aged 16 months, attended the hospital due to head and facial erythema for 15 months and vulva erythema for 10 months with aggravation for 5 days. The boy developed perioral and periocular erythema in the neonatal period and had erythema and papules with desquamation and erosion in the neck, armpit, and trigone of vulva in infancy. Blood gas analysis showed metabolic acidosis; the analysis of amino acid and acylcarnitine profiles for inherited metabolic diseases and the analysis of organic acid in urine suggested multiple carboxylase deficiency; genetic testing showed a homozygous mutation of c.1522C>T(p.R508W) in the HLCS gene. Finally the boy was diagnosed with holocarboxylase synthetase deficiency and achieved a good clinical outcome after oral biotin treatment. This article analyzes the clinical data of a child with holocarboxylase synthetase deficiency and summarizes the etiology, diagnosis, and treatment of this child, so as to provide ideas for clinicians to diagnose this rare disease.
Humans ; Male ; Biotin/therapeutic use* ; Holocarboxylase Synthetase Deficiency/drug therapy* ; Homozygote ; Mutation ; Rare Diseases/drug therapy* ; Infant

Objective: To analyze and compare therapy responses, outcomes, and incidence of severe hematologic adverse events of flumatinib and imatinib in patients newly diagnosed with chronic phase chronic myeloid leukemia (CML) . Methods: Data of patients with chronic phase CML diagnosed between January 2006 and November 2022 from 76 centers, aged ≥18 years, and received initial flumatinib or imatinib therapy within 6 months after diagnosis in China were retrospectively interrogated. Propensity score matching (PSM) analysis was performed to reduce the bias of the initial TKI selection, and the therapy responses and outcomes of patients receiving initial flumatinib or imatinib therapy were compared. Results: A total of 4 833 adult patients with CML receiving initial imatinib (n=4 380) or flumatinib (n=453) therapy were included in the study. In the imatinib cohort, the median follow-up time was 54 [interquartile range (IQR), 31-85] months, and the 7-year cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) were 95.2%, 88.4%, 78.3%, and 63.0%, respectively. The 7-year FFS, PFS, and OS rates were 71.8%, 93.0%, and 96.9%, respectively. With the median follow-up of 18 (IQR, 13-25) months in the flumatinib cohort, the 2-year cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) were 95.4%, 86.5%, 58.4%, and 46.6%, respectively. The 2-year FFS, PFS, and OS rates were 80.1%, 95.0%, and 99.5%, respectively. The PSM analysis indicated that patients receiving initial flumatinib therapy had significantly higher cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) and higher probabilities of FFS than those receiving the initial imatinib therapy (all P<0.001), whereas the PFS (P=0.230) and OS (P=0.268) were comparable between the two cohorts. The incidence of severe hematologic adverse events (grade≥Ⅲ) was comparable in the two cohorts. Conclusion: Patients receiving initial flumatinib therapy had higher cumulative incidences of therapy responses and higher probability of FFS than those receiving initial imatinib therapy, whereas the incidence of severe hematologic adverse events was comparable between the two cohorts.
Adult ; Humans ; Adolescent ; Imatinib Mesylate/adverse effects* ; Incidence ; Antineoplastic Agents/adverse effects* ; Retrospective Studies ; Pyrimidines/adverse effects* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy* ; Treatment Outcome ; Benzamides/adverse effects* ; Leukemia, Myeloid, Chronic-Phase/drug therapy* ; Aminopyridines/therapeutic use* ; Protein Kinase Inhibitors/therapeutic use*

Objective To evaluate the molluscicidal effect of 25% wettable powder of pyriclobenzuron sulphate (WPPS) against Oncomelania snails in hilly schistosomiasis-endemic regions and test its toxicity to fish. Methods In October 2020, a snail-infested setting which had been cleared was selected in Nanjian County, Yunnan Province and divided into several blocks, and the natural snail mortality was estimated. 25% WPPS was prepared into solutions at concentrations of 1 and 2 g/L, and 25% wettable powder of niclosamide ethanolamine salt (WPNES) was prepared into solutions at a concentration of 2 g/L. The different concentrations of drugs were sprayed evenly, and the same amount of water was used as blank control. Snails were surveyed using the systematic sampling method 1, 3 and 7 days post-treatment, and snail survival was observed. A fish pond was selected in Nanjian County, and 2 kg 25% WPPS was evenly sprayed on the water surface to allow the effective concentration of 20 g/L. Fish mortality was estimated 8, 24, 48 and 72 h post-treatment. Results One-day treatment with 1 and 2 g/L WPPS and 2 g/L WPNES resulted in 97.99%, 97.99% and 94.11% adjusted snail mortality rates (χ² = 3.509 and 3.509, both P values > 0.05), and the adjusted snail mortality was all 100% 3 d post-treatment with 1 and 2 g/L WPPS and 2 g/L WPNES, while 7-day treatment with 1 and 2 g/L WPPS and 2 g/L WPNES resulted in 91.75%, 86.57% and 57.76% adjusted snail mortality rates (χ² = 14.893 and 42.284, both P values < 0.05). Treatment with 2 g/L WPPS for 72 h resulted in a 0.67% cumulative mortality rate of fish. Conclusion 25% WPPS is effective for snail control and highly safe for fish, which is feasible for use in hilly schistosomiasis-endemic regions.

Objective To explore the influence of muscle and fat distribution on sit-up ability of female college students. Methods A total of 1560 female students majoring in non-physical education in a Guangxi University were randomly selected. Body composition was measured by the bioresistance antibody component meter. According to the National Physical Health Test Standard, the height, weight and sit-ups of female college students were measured. The students were divided into four groups according to the score of sit-ups:<60 points, 60-69 points, 70-79 points, ≥80 points. SPSS 22.0 statistical software was used to analyze the data. Results There were statistically significant differences in body fat rate, total fat mass, trunk fat mass, trunk fat rate, visceral fat area, visceral fat mass and subcutaneous fat mass in different sit-ups (F value were 3.414, 2.914, 2.881, 3.347, 3.064, 3.235, 2.766, P<0.05). That was, the fat parameters in the 60-69 group were the highest and ≥80 the lowest. However, there was no significant difference between total muscle mass and trunk muscle mass in each score segment. After correcting for age, height, and weight factors, except for visceral fat area and visceral fat mass, the correlation between other fat parameters and sit-up score were negatively correlated, which were statistically significant (r<0, P<0.05). There was a positive correlation between each muscle parameter and sit-up performance (r>0, P<0.05). Compared with other parameters, the correlation coefficient between trunk muscle mass and sit-ups was the largest. Conclusion The distribution of fat and muscle has different effects on sit-up ability. Female college students' sit-up ability is greatly affected by subcutaneous fat and trunk muscle, and has nothing to do with visceral fat.

In 2013,China pharmaceutical regulatory department issued guiduance on postmarketing drug safety monitoring for industry. It aimed to encourage industry to carry out postmarketing drug safety monitoring including hospital-based intensive monitoring of postmarketing Chinese patent medicine. Subsequently,more and more such kind of studies have been performed all over China. However,in view of the current situation in this field,the development of hospital-based intensive monitoring of postmarketing Chinese patent medicine lacks standardization,such as unreasonable design,omission of reports about adverse drug reactions,inadequate process of quality control,non-standardized interpretation of adverse reactions,etc. Therefore,it is necessary to formulate relevant technical specifications to guide this area. The developing of this technical specification refered to the international post-marketing safety monitoring model and advanced design concepts and methods. We developed it under the guidance of relevant laws,regulations and technical documents in China. Meanwhile the characteristics of Chinese patent medicines and the real situation in this area were considered. The aim of this technical specification is to obtain the incidence,type,degree and clinical manifestation of adverse drug reactions of Chinese patent medicines,to find new risk signals of adverse reactions,to identify risk factors,and to provide a basis for the formulation of risk management and control plans. This specification has been approved by China association of Chinese medicine which is numbered T/CACM011-2016.
Adverse Drug Reaction Reporting Systems ; China ; Hospitals ; Medicine, Chinese Traditional ; Nonprescription Drugs ; Product Surveillance, Postmarketing

Objective To understand the intention to quit smoking and its influencing factors among current smokers in Gansu Province, so as to provide scientific basis for tobacco control. Methods A multi-stage sampling method was used to extract current smokers aged 15-69 years, and a face-to-face survey was conducted using the questionnaire on smoking among residents in China. Intention to quit smoking between current smokers with different characteristics was analyzed. Logistic regression was used to explore influencing factors of intention to quit smoking. Results The intention of current smokers to quit smoking in Gansu Province was 16.4% (95% CI:15.5%-17.3%). Multivariate logistic regression analysis showed that current smokers who were in rural areas (OR=1.199, 95% CI:1.022-1.408, P=0.026); family smoking prohibited (OR=1.767, 95% CI: 1.273-2.454, P=0.001), medical staff discouraged smoking within 12 months (OR=1.599, 95% CI:1.359-1.842, P<0.001), visited smoking clinics (OR=3.089, 95% CI:2.031-4.698, P＜001), higher educational level of junior high school, senior high school and college or above (OR=1.383, 95% CI:1.101-1.736; OR=1.627, 95% CI:1.252-2.116; OR=1.374, 95% CI:1.009-1.873, all P<0.05), tobacco hazards knowledge with higher scores of 1-, 3- and 5-6 (OR=1.248, 95% CI:1.030-1.514; OR=1.574, 95% CI:1.289-1.922; OR=2.288, 95% CI:1.879-2.786, all P<0.05) were more likely to quit smoking; furthermore, smokers aged 20-, 30- years or smoking 20-, 30- years had a lower chance of quit smoking (all P<0.05). Conclusions The intention of current smokers to quit smoking in Gansu province is generally not high. In the future, knowledge of tobacco hazards should be further promoted, medical staff should provide more smoking cessation services during the treatment process, and more smoking cessation clinics should be established.

To explore therapeutic effect of acupuncture combined electrical stimulation on aged patients with post‐stroke depression .Methods : A total of 78 patients with post‐stroke depression were randomly and equally divided into routine treatment group (received health education based on routine treatment ) and combined treatment group (received acupuncture combined low‐frequency electrical stimulation based on routine treatment group ).After four‐week treatment , score of Hamilton rating scale for depression (HAMD) and therapeutic effect were compared between two groups .Results : Compared with before treatment , there was significant reduction in HAMD score in two groups after treatment ;compared with routine treatment group after treatment , there was significant reduction in HAMD score [(19.72 ± 2. 04) scores vs.(14. 94 ± 1. 86) scores] in combined treatment group , P＝0. 001 all.To‐tal effective rate of combined treatment group was significantly higher than that of routine treatment group (87.18% vs.64.1%, P＝0.018).Conclusion : Acupuncture combined low‐frequency electrical stimulation possesses significant therapeutic effect on post‐stroke depression , which is worthy of clinical application .

Objective To compare the curative effect and short-term benefits of laparoscopic liver resection with open liver resection in elderly patients with malignant liver tumors and medical comorbidities.Methods Patients aged 70 and over who received liver resections for malignant liver tumors between January and October 2015 were enrolled.The perioperative outcomes of 17 patients with laparoscopic approach were matched and compared with those of 34 patients with conventional open approach in a 1:2 ratio.Results There was no significant difference found between the two groups with regard to age,gender,incidence of comorbid illness,hepatitis B positivity,and Child-Pugh grading of liver function.The median tumor size was 3 cm for both groups.The types of liver resection were similar between the two groups with no significant difference in the duration of operation (laparoscopic: 195 min vs.open: 210 min,P=0.436).The perioperative blood loss was 150 mL in the laparoscopic group and 330 mL in the open group (P=0.046) with no significant difference in the number of patients with blood transfusion.The duration of hospital stay was 6 days (3-15 days) for the laparoscopic group and 8 days (5-105 days) for the open group (P=0.005).Conclusion Laparoscopic liver resection is safe and feasible for elderly patients.The short-term benefits of laparoscopic approach proves to be evident for geriatric oncological liver surgery.

Background:The Food and Drug Administration recently announced that the use of morcellation may cause fibroids or pelvic dissemination and metastasis of uterine sarcoma;therefore,the use of morcellation is limited in the USA.A large sample study is necessary to assess the proportion of uterine malignant tumors found in patients with laparoscopic myomectomy.Methods:A national multicenter study was performed in China.From 2002 to 2014,33,723 cases were retrospectively selected.We calculated the prevalence and recorded the clinical characteristics of the patients with malignancy after morcellation application.A total of 62 cases were finally pathologically confirmed as malignant postoperatively.Additionally,the medical records of the 62 patients were analyzed in details.Results:The proportion of postoperative malignancy after morcellation application was 0.18％ (62/33,723) for patients who underwent laparoscopic myomectomy.Nearly 62.9％ (39/62) of patients had demonstrated blood flow signals in the uterine fibroids before surgery.And,23 (37.1％) patients showed rapid growth at the final preoperative ultrasound.With respect to the pathological types,38 (61.3％) patients had detectable endometrial stromal sarcoma,13 (21.0％) had detectable uterine leiomyosarcoma,only 3 (3.2％) had detectable carcinosarcoma,and 5 (8.1％) patients with leiomyoma had an undetermined malignant potential.Conclusions:The proportion of malignancy is low after using morcellation in patients who undergo laparoscopic myomectomy.Patients with fast-growing uterine fibroids and abnormal ultrasonic tumor blood flow should be considered for malignant potential,and morcellation should be avoided.

OBJECTIVETo evaluate the therapeutic effect of three-dimensional digital orthopedic techniques in treatment of acetabular fractures.

METHODSWe retrospectively analyzed 50 cases of acetabular fracture treated between March, 2007 and December, 2013. The lamellar CT scanning data were imported into Mimics software, and 3D anatomical models of the pelvic and proximal femur were reconstructed. Computer-assisted analysis was carried out to understand the condition of fractures and simulate fracture reduction. The pelvic models were manufactured by rapid prototyping technique for definite diagnosis and typing of acetabular fractures and subsequent surgical treatment.

RESULTSThree-dimensional reconstruction images and rapid prototyping pelvic models faithfully represented the findings in operations. Preoperative simulation of the operation shortened the time of operation and reduced the volume of bleeding in the operation. All the patients were followed up for 6 to 24 months. According to Matta imaging score, anatomical reduction was achieved in 41 cases and satisfactory reduction in 9 cases. According to the Harris functional criteria, 32 patients had excellent, 12 had good and 6 had acceptable outcomes with a rate of excellent and good outcomes of 88%.

CONCLUSIONThree-dimensional digital orthopedic techniques allow accurate display of the acetabulum and the spatial relation of the anatomic structures to assist in fracture diagnosis, typing and treatment.

Acetabulum ; pathology ; Femur ; Fracture Fixation, Internal ; Fractures, Bone ; Humans ; Imaging, Three-Dimensional ; Models, Anatomic ; Orthopedics ; methods ; Retrospective Studies ; Software ; Tomography, X-Ray Computed