ABSTRACT
Objective:To summarize the clinical features and gene mutation characteristics of a child with mitochondrial enoyl-CoA hydratase short chain 1 deficiency (ECHS1D) caused by enoyl-CoA hydratase short chain 1 ( ECHS1) gene mutation. Methods:The clinical characteristics and genetic test results of a child with ECHS1D who visited the Department of Neurology of Xuzhou Children′s Hospital in January 2021 were retrospectively analyzed, and the clinical features of the disease were also reviewed by searching relevant domestic and foreign literature.Results:The child was a 6 months and 4 days old male, with acute onset, the main clinical manifestation being limb movement disorder after admission. The child had slow motor development, his head was still upright and cannot turn over, the child also cannot sit alone, follow up and make a laugh, and the muscle tension of limbs was increased. The child′s blood lactate was increased to 6.2 mmol/L, which suggested metabolic acidosis, and magnetic resonance imaging (MRI) of the head showed abnormal signals in the basal ganglia on both sides, abnormal enhancement of the meninges of the left cerebral hemisphere. Whole exome sequencing revealed that the child had compound heterozygous mutations in ECHS1 gene, c.563C>T (p.A188V) and c.5C>T (p.A2V), respectively. The child′s father carried c.563C>T mutation, the mother carried c.5C>T mutation, all of which were missense mutations. Conclusions:ECHS1 gene mainly has missense mutations, most of which are compound heterozygous mutations, and a few are homozygous mutations. The ECHS1D caused by ECHS1 gene mutation often affects infants and young children. MRI suggests abnormal signals in the basal ganglia; for cases with the above clinical manifestations and abnormal signals in the basal ganglia on MRI, genetic testing should be considered to confirm the diagnosis.
ABSTRACT
Objective:To investigate COVID-19 vaccination status and relevant adverse reactions in patients with psoriasis treated with biological agents, and to explore the effect of COVID-19 vaccination on psoriatic lesions.Methods:Clinical data were collected from 572 psoriasis patients aged 18 - 60 years, who were registered in the management system of psoriasis patients treated with biological agents in the University of Hong Kong-Shenzhen Hospital from May 2019 to June 2021. The COVID-19 vaccination status was investigated by telephone interviews, and the vaccination-related information was obtained by fixed healthcare workers during a fixed time period according to a predesigned questionnaire. Measurement data were compared between two groups by using t test, and enumeration data were compared by using chi-square test or Fisher′s exact test. Results:The COVID-19 vaccination coverage rate was 43.13% (226 cases) among the 524 patients who completed the telephone interview, and was significantly lower in the biological agent treatment group (30.79%, 105/341) than in the traditional drug treatment group (66.12%, 121/183; χ2 = 60.60, P < 0.001) . The main reason for not being vaccinated was patients′ fear of vaccine safety (49.66%, 148/298) , followed by doctors′ not recommending (26.51%, 79/298) . In the biological agent treatment group after vaccination, the exacerbation of psoriatic lesions was more common in patients receiving prolonged-interval treatment (42.86%, 6/14) compared with those receiving regular treatment (4.40%, 4/91; Fisher′s exact test, P < 0.001) . Skin lesions were severely aggravated in two patients after COVID-19 vaccination, who ever experienced allergic reactions and whose skin lesions did not completely subside after the treatment with biological agents. Conclusions:The COVID-19 vaccination coverage rate was relatively low in the psoriasis patients treated with biological agents, and no serious adverse reaction was observed after vaccination. Prolonged-interval treatment due to COVID-19 vaccination ran the risk of exacerbation of skin lesions.
ABSTRACT
Objective:To investigate the clinical efficacy of anal dimple anorectoplasty on female infants with congenital anal atresia combined rectal vestibular fistula.Methods:Clinical data of 69 female infants with congenital anal atresia combined rectal vestibular fistula admitted to Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from July 2012 to July 2022 were retrospectively analyzed.They were divided into 2 groups according to the surgical methods: 34 cases of anal dimple anorectoplasty(group A) and 35 cases of anterior sagittal anorectoplasty(group B). The operation time, length of stay, short-term complications, long-term complications and bowel function (determined by the Rintala score at 6 months postoperatively) of the two groups were compared.The difference in the incidence of postoperative complications between groups was compared by Chi- square test, and the remaining differences between groups were compared by the paired t-test. Results:The operative time [(80.18±9.29) min vs.(103.85±8.26) min] and postoperative hospital stay[(6.10±1.52) d vs.(7.63±2.40) d] in group A were significantly shorter than those of group B ( t=11.40, 2.62; all P<0.05). The Rintala total score at 6 months postoperatively in group A was significantly higher than that of group B[(19.36±0.93) points vs.(18.76±0.44) points]( t=3.20, P<0.05). There were no significant differences in the incidences of short-term [(4/34, 11.8%) vs.(7/35, 20.0%)] and long-term complications [(2/34, 5.9%) vs.(4/35, 11.4%)]between group A and group B ( χ2=0.75, 0.75; all P>0.05). Conclusions:Anal dimple anorectoplasty for female infants with congenital anal atresia combined rectal vestibular fistula is safe and effective.
ABSTRACT
The clinical characteristics and gene mutation profiles of a child who was treated in Xuzhou Children′s Hospital, Xuzhou Medical University in June 2022 due to developmental and epileptic encephalopathy (DEE) combined with dyskinesia caused by the FRRS1L gene mutation was analyzed retrospectively.A male case 1 year and 9 months old presented developmental delay since childhood, hypotonia at the age of 6 months, treatment-resistant seizures (focal clonic seizures) at the age of 1 year and 7 months that were unable to be controlled by antiepileptic drugs, and developmental regression and abnormal movements of the hands and feet during the attack.Whole exome sequencing showed 2 heterozygous variants (missense mutation and deletion mutation) in the FRRS1L gene of the child.The missense mutation c. 754C>T (p.R252C) located in the 4 th exon was inherited from his mother, and the deletion mutation c. 438_c.459del (p.I146fs*4) located in the 2 th exon was inherited from his father, thus constituting a compound heterozygous mutation.Through literature review, all 6 relevant literatures involving 31 children with DEE were published in foreign countries.They presented similar clinical manifestations to this case, but the genotypes were different, all of which were homozygous mutations.The FRRS1L gene mutation can lead to DEE, which is characterized by the autosomal recessive inheritance pattern, refractory epilepsy onset in infancy, developmental regression and prominent dyskinetic movements with hyperkinesia, and poor long-term prognosis.
ABSTRACT
Objective:To summarize the practice and experience of medical service support for Shenzhou manned spaceflight mission, and to explore the accurate medical service support strategy with new regions and new types.Methods:A systematic summary was made from the force command, action framework, technical support, and the practice and experience in the application of medical support force for Shenzhou manned flight mission were sorted out.. Furthermore, the key and difficult problems of the new domains with new types of military medical service support were analyzed, and the scheme and significance of establishing the accurate support mode of the new medical service support force were proposed.Results:The Shenzhou emergency rescue mission was an elite combat supported by a large platform. It was an important basis to realize accurate medical service support by the experience of utilizing the medical force of Shenzhou emergency rescue and the strengthening of war-building mode optimization.Conclusions:This paper systematically summarizes the experience and characteristics of medical force application in Shenzhou manned spaceflight missions, and provides new ideas and methods for future diversified and accurate s medical service support with new regions and new types.
ABSTRACT
Objective:To review the characteristics and coping strategies of the rescue and landing medical support mission of Shenzhou-14 manned spacecraft.Methods:The characteristics of rescue and landing medical support mission of Shenzhou-14 manned spacecraft was analyzed, and the coping strategies and experience were discussed.Results:(1) The characteristics of rescue and landing medical support mission of Shenzhou-14 manned spacecraft included: long time in space station and high-intensity space missions; high-density space medical support mission in short term; special environmental factors in severe cold night; complex terrain of landing site; and the young medical support team. (2) The main coping strategies of rescue and landing medical support mission of Shenzhou-14 manned spacecraft included: strengthened the organization and leadership and improved the training model; reinforcement learning the medical treatment plan and strengthened the medical rescue skills training; optimized the carrying equipment and added the heat preservation and lighting measures; improved the medical rescue process and perfected the emergency plan; emphasized on the scientific research as important as mission; and strengthened the physical exercise and cold resistance exercise.Conclusions:The characteristics and coping strategies of rescue and landing medical support mission of Shenzhou-14 manned spacecraft are summarized to provide the experience for space medical rescue and offer the support for China's manned space industry.
ABSTRACT
Objective:To study the application of scenario simulation teaching combined with modular training in nursing education of medical rescue in manned space flight.Methods:Twenty nurses from the medical rescue team of Strategic Support Force Characteristic Medical Center were selected as the research objects. The research objects were randomly divided into the scenario simulation combined with practical training group (practical training group, n=10) and traditional teaching group (control group, n=10). Scenario simulation teaching combined with modular training and traditional teaching were used to carry out nursing training. After the training, theoretical assessment, operation assessment and satisfaction survey were organized. Results:The scenario simulation teaching combined with modular training group was significantly better than the traditional training group in theory assessment, operation assessment and satisfaction survey of nursing staff (all P<0.05). Conclusions:Scenario simulation teaching combined with modular training has obvious teaching effect, which can improve the ability and quality of nursing staff, and help to complete the manned space medical rescue mission efficiently.
ABSTRACT
Objective:To explore the module of the construction and application of medical first-aid at the door of the space capsule and the air evacuation.Methods:According to the purpose, principle, and method set by the module, it was divided into two sub-modules: medical first-aid at the door of the space capsule and the air evacuation. During the comprehensive first-aid training, independent training and combined training were carried out respectively according to different mission stages of launch and recovery and different recovery terrain. Meanwhile, research was performed to ensure that medical carrying equipment was lightweight, modular, and portable, and the efficiency of modularization construction was tested in the comprehensive training.Results:The module of medical first-aid at the door of the space capsule and the air evacuation obviously shortened the rescuing time during the comprehensive training, the saving effect was remarkable, and the overall saving efficiency was significantly improved.Conclusions:The module of medical first-aid at the door of the space capsule and the air evacuation meet the requirements that the emergency support system of aerospace medicine should function on an immediate basis, ensuring accurate treatment and air evacuation without any delay, so that to achieve the aim of aerospace medicare.
ABSTRACT
In general, kidney transplantation is the final treatment for patients with end-stage kidney disease because it is more cost-effective and can improve the quality of patient′s life. In addition, survival rate after transplantation is far superior to hemodialysis. Due to the lack of living donor kindey, centers have begun using the expanded criteria donor, one of which is to use kidneys containing calculi. The purpose of this article is to describe the selection and treatment strategies of donor kidney containing calculi in kidney transplantation and the related treatment methods for transplanted kidney calculi.
ABSTRACT
@#A large number of people would be exposed to irradiation in large-scale nuclear and radiation accidents or nuclear terrorist attacks. Therefore, it is urgent to establish rapid and high-throughput biodosimetry for in triage, providing a basis for emergency management. Imaging flow cytometry (IFC) possesses the high through put advantages of traditional flow cytometry and the sensitivity and specificity of microscope, and has a good application prospect in the research and development of rapid, automated, and high-throughput biological dose estimation technology. This article reviews the application progress of IFC in biodosimetry, and provides a reference for the development of biological dose estimation and detection equipment for large-scale nuclear and radiation accidents.
ABSTRACT
Background At present, radiation therapy is widely used in clinical treatment of tumors. However, while radiation therapy damages tumor cells, it also injures surrounding normal tissues. Studies have shown that hydrogen is a potential radiation-protective agent. Objective To investigate the neuroprotective mechanisms of hydrogen-rich water activating phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/cysteinyl aspartate specificproteinase-9 (Caspase-9) signaling pathway in acute radiation-induced brain injury. Methods Forty male SD rats were randomly divided into four groups: control group, irradiation only group (IR), high-dose hydrogen-rich water intervention group (IR+HHRW), and low-dose hydrogen-rich water intervention group (IR+LHRW), 10 rats in each group. Except for the control group, animals in each group received a single 20 Gy whole brain irradiation. Animals in all groups were gavaged once a day from 3 d before irradiation to 7 d after irradiation, pure water (20 mL·kg−1) was given to the control and the IR groups, and hydrogen-rich water (20 mL·kg−1, 10 mL·kg−1) was given to the IR+HHRW and the IR+LHRW groups. After 7 d of intervention, 5 rats in each group were selected for the Morris water maze experiment for behavioral evaluation. Autopsies were conducted after anesthesia for the remaining animals and blood samples were collected for hematological analysis. Rat brains were harvested for TUNEL staining to observe neuronal apoptosis. HE staining was performed to observe histopathological changes, enzyme-linked immunosorbent assay was adopted to detect oxidative stress-related indicators, and real-time PCR and Western blotting were used to measure the expressions of PI3K/AKT/Caspase-9 pathway-related genes and proteins. Results The body weight of rats receiving irradiation decreased after 7 d of irradiation compared with the control group (P<0.05), and the symptoms such as arched back and malaise occurred to varying degrees, and the symptoms of rats in the IR+HHRW group were significantly milder than those in the IR group. The behavioral test results showed that the escape latency of rats in the IR+HHRW group or the IR+LHRW group was shorter than that in the IR group from day 2 to day 5 (P<0.05), and it took less time for rats in the IR+HHRW group to reach the original position after removing the platform on day 6 (P<0.05). The hematological test results showed that red blood cell (RBC) count, hemoglobin (HGB) level, and white blood cell (WBC) count were significantly decreased in the IR group (P<0.05), and the changes in the IR+HHRW group were improved (P<0.05). The HE staining results showed that the number of abnormal nerve cells, broken and dissolved nuclei, and the degree of damage in the IR+HHRW group were significantly reduced than those in the IR group. The results of oxidative stress evaluation showed that the ability of the IR group to inhibit free radicals decreased, the level of malondialdehyde (MDA) increased (P<0.01); the MDA level decreased after LHRW intervention (P<0.05); the SOD activity was elevated after HHRW intervention (P<0.05). The TUNEL staining results showed that the apoptosis signals in the IR+HHRW group were sparser than those in the IR group (P<0.05). The real-time PCR results showed that compared with the IR group, the mRNA expression levels of PI3K and AKT in the IR+HHRW group and the IR+LHRW group increased (P<0.05), while the mRNA expression levels of Cytc and Caspase-9 decreased (P<0.05). The Western blotting results showed that compared with the IR group, the phospho-AKT (pAKT) protein expression level in the IR+HHRW group increased significantly (P<0.05), while the expression of Caspase-9 and Cytc proteins decreased significantly (P<0.05). Conclusion Hydrogen-rich water can significantly reduce inflammation and oxidative stress caused by acute irradiation-induced brain injury, and decrease neuronal apoptosis. The mechanism may be related to the PI3K/AKT/Caspase-9 signaling pathway.
ABSTRACT
This paper summarizes and discusses the research and achievements in the effect of irradiation on extending the shelf life and quality guarantee period of seafood, on the quality of seafood, and on seafood sterilization, and seafood irradiation biological dosimeter study, and defines the concepts related to seafood irradiation. Moreover, we propose that irradiation sterilization on severe acute respiratory syndrome coronavirus 2 of cold-chain seafood and seafood irradiation dose control are the main research content and directions.
ABSTRACT
Objective:To investigate clinical characteristics of bullous pemphigoid (BP) developing after the treatment with dipeptidyl peptidase-Ⅳ inhibitors (DPP4i) in patients with diabetes mellitus.Methods:A total of 116 inpatients with BP complicated by diabetes mellitus were collected from the Seventh People′s Hospital of Shenyang between January 2014 and December 2020, and divided into 2 groups: DPP4i-BP group treated with DPP4i before the onset of BP, and general BP group receiving no treatment with DPP4i. General clinical data, skin lesion area, laboratory indicators, treatment regimens, and prognosis were analyzed and compared between the above 2 groups, the time interval from the administration of DPP4i to the diagnosis of BP was recorded in the DPP4i-BP group. One-way analysis of variance was used to compare measurement data among multiple groups, two-independent-sample t test was used for comparisons between two groups, and paired t-test for intra-group comparisons before and after treatment; chi-square test was used to compare enumeration data between groups. Results:There were 32 patients aged 77.17 ± 15.32 years in the DPP4i-BP group, with a male-to-female ratio being 15∶17; there were 84 patients aged 76.65 ± 19.32 years in the general BP group, with a male-to-female ratio being 43∶41. The time interval from the administration of DPP4i to the diagnosis of BP was 14.61 ± 3.93 months in the DPP4i-BP group. The time interval for vildagliptin was the shortest (5.42 ± 2.84 months) , and there was a significant difference in the time interval among vildagliptin, sitagliptin, linagliptin and saxagliptin ( F= 8.93, P < 0.001) . The proportion of patients with severe BP was significantly higher in the DPP4i-BP group (16 cases, 50%) than in the general BP group (25 cases, 29.76%; Z= 2.63, P= 0.008) . There was no significant difference in the positivity rate of anti-BP180 antibody between the two groups ( χ2= 0.03, P= 0.870) . However, the level of anti-BP180 antibody was significantly higher in the DPP4i-BP group than in the general BP group before and after treatment ( P= 0.015, < 0.001, respectively) , and the decrease in the level of anti-BP180 antibody was significantly less in the DPP4i-BP group than in the general BP group after treatment ( t= 5.11, P < 0.001) . There was no significant difference in the average effective dose of glucocorticoids required to control the disease between the two groups ( t= 1.00, P= 0.322) . However, the DPP4i-BP group showed a significant increase in the average time required to control the disease and in the proportion of patients requiring combined treatment with immunosuppressants or other drugs compared with the general BP group ( t= 6.72, 10.05, P < 0.001,= 0.002, respectively) . Within 6 months after the start of systemic treatment, the recurrence rate was significantly higher in the general BP group (17 cases, 27.86%) than in the DPP4i-BP group (2 cases, 7.69%; χ2= 4.35, P= 0.037) ; at 6 months, the average dose of glucocorticoids was also significantly higher in the general BP group than in the DPP4i-BP group ( t= 7.04, P < 0.001) . Conclusions:Among the DPP4i hypoglycemic drugs, vildagliptin was the most common drug administrated by patients before the onset of BP, with the shortest interval from the administration to the onset of BP. DPP4i-BP may be difficult to control at the early stage, but the prognosis is good.
ABSTRACT
Objective:To summarize the clinical and gene mutation characteristics of a child with developmental epileptic encephalopathy (DEE) caused by ubiquitin-like modifier-activating enzyme 5( UBA5 )gene mutation, and to perform literature review. Methods:Clinical characteristics and genetic test results of a case of DEE treated in Department of Neurology, Xuzhou Children′s Hospital, Xuzhou Medical University, in March 2020 were retrospectively analyzed.Relevant literatures reporting DEE cases caused by UBA5 gene mutations published before June 2020 were searched in the PubMed, CNKI, Wanfang and other online databases with the following key words: epilepsy encephalopathy, developmental encephalopathy, epileptic encephalopathy, Developmental encephalopathy and UBA5. Results:A female case with 7 months and 23 days old presented epileptic spasms at 4 months of age, and the condition was uncontrolled by medication of adrenocorticotropic hormone (ACTH) and several antiepileptic drugs.The patient later progressed to recurrent, treatment-resistant seizures with arrested development, short stature, microcephaly, expressionless face, irritability, unsteady head, lack of follow-up vision, lack of laughing, and limb hypotonia.Whole exome sequencing revealed a missense mutation and a microdeletion in the UBA5 gene, and the missense mutation was paternal c. 722A>C (p.E241A), located in the 8 th exon region.The microdeletion deletion was maternally derived from the 5 th to 11 th exon, which constituted a compound heterozygous mutation.A total of 5 foreign literatures involving 18 children with DEE and 0 domestic literatures were retrieved.Combined with the present case, all 19 cases presented refractory seizures in the early infancy, and most of them were epileptic spasms (63.2%, 12/19 cases), followed by myoclonus (31.6%, 6/19 cases). The birth history of all children was unremarkable, and they later presented developmental disabilities at varying degrees, mainly including microcephaly (94.7%, 18/19 cases), lack of follow-up vision (89.5%, 17/19 cases), and short stature (94.7%, 18/19 cases), intellectual disabilities (89.5%, 17/19 cases), movement disorders (84.2%, 16/19 cases) and hypotonia (100.0%), 13/19 cases (68.4%) died.EEG results mainly revealed normal or hypsarrhythmia, but 1 case presented suppression- burst.Brain magnetic resonance imaging (MRI) findings mainly included delayed myelination (47.4%, 9/19 cases), brain atrophy (52.6%, 10/19 cases), and thin corpus callosum (26.3%, 5/19 cases). Conclusions:Children with UBA5 gene mutations often have refractory seizures in the early infancy, which are mainly epileptic spasms.They also show severe psychomotor developmental delay, microcephaly and dystonia, with an extremely poor prognosis.Brain MRI suggested varying degrees of myelin dysplasia, brain trophy, and thin corpus callosum.For cases with the above clinical manifestations, genetic testing should be considered to confirm the diagnosis.
ABSTRACT
Background The mechanisms of silicon dioxide (SiO2)-induced inflammation and cell injury in pulmonary macrophages are not fully characterized. Objective To investigate the potential roles of inhibition of toll-like receptor 4 (TLR4)/nuclear factor-kappa B (NF-κB) signaling in inflammation and macrophage polarization in mouse Raw264.7 cells in response to SiO2 stimulation. Methods Sixteen 6- to 8-week-old C57BL/6 mice, half male and half female, were intratracheally instilled with 50 µL of SiO2 (50 mg·mL−1 in saline) or normal saline via oropharyngeal route, and the lungs of mice were harvested at 14 d and 28 d post the first challenge of SiO2. HE staining of mouse lung was used for histopathological analysis. The expressions of TLR4 signaling-related proteins were detected by Western blotting (WB) and immunofluorescent (IF) assay, including TLR4, myeloid differentiation factor 88 (Myd88), and TNF receptor associated factor 6 (TRAF6). Raw264.7 cells were stimulated with SiO2 (100 μg·cm2) for 12 h in absence or presence of TLR4 inhibitor M62812 for 13 h before the culture supernatants and cell lysates were harvested for analysis. The expressions of key components of TLR4 signaling cascade including TLR4, Myd88, and phosphorylated nuclear factor-kappa B P65 (P-NF-κB P65), P-1NF-kappa-B inhibitor α (P-1κbα), tumor necrosis factor-α (TNF-α), and interleukin 6 (IL-6), M1 phenotype markers inducible nitric oxide synthase (iNOS) and cluster of differentiation 86 (CD86), as well as M2 phenotype arginase-1 (Arg-1) were accessed by WB and IF. The expressions of inflammation factors IL-6 and TNF-α in supernatants were determined by enzyme-linked immunosorbent assay (ELISA). Results After SiO2 intratracheal instillation for 14 d, the HE staining results showed obvious fibrotic nodules in the lung tissues of mice. The results of WB analysis revealed more abundant TLR4, Myd88, and TRAF6 in the silicosis mouse lung samples than in the controls. The results of IF assay showed an increased abundance of TLR4 and Myd88 proteins in the lung samples of silicosis mice at 14 d post the silica challenge, compared to the controls, indicating TLR4 signaling activation. As seen in the in vitro experiment, significant upregulations after the exposure to 100 μg·cm2 SiO2 were observed in TLR4 and P-1κbα at 6, 12, and 24 h (P<0.05); Myd88 at 12 and 24 h (P <0.05); and P-NF-κB P65 at 12 h (P<0.05). The inhibitor significantly suppressed the expressions of TLR4, Myd88, TRAF6, P-NF-κB P65, TNF-α, and IL-6 in Raw264.7 cells. In addition, the SiO2-induced M1 phenotype marker iNOS was significantly suppressed, but the M2 phenotype marker Arg-1 was increased in the Raw264.7 cells. Conclusion The inhibition of TLR4/NF-κB signaling could result in a reduction of the inflammation response and the transition of M1 toward M2 phenotypes of macrophages in response to SiO2 challenge.
ABSTRACT
Objective:To analyze clinical and imaging features of Sturge-Weber syndrome in children.Methods:Clinical data were collected from 27 children with Sturge-Weber syndrome in Xuzhou Children′s Hospital, Xuzhou Medical University from July 2013 to December 2019, and analyzed retrospectively.Results:Among the 27 children, 17 were males and 10 were females. Their age at the clinic visit ranged from 2 days to 10 years and 7 months, and averaged 2.54 years. All the 27 patients presented with facial port-wine stains of varied color from light red to purple red, which were all distributed across the facial midline, including 21 with predominantly unilateral port-wine stains and 6 with bilateral symmetrical port-wine stains. There were 17 patients with ocular choroidal vascular malformations, including 14 with congenital glaucoma, 5 with high intraocular pressure, and 1 with optic nerve atrophy accompanied by transient blindness. Neurological impairment occurred in 12 patients, and all manifested as epilepsy. All the 27 children underwent imaging examination, and abnormalities were found in 20. Among the 10 patients with abnormal computed tomography images, local calcification was observed in 8, and local thickening of the skull on the side affected by skin lesions in 8; 13 of 14 patients with abnormal magnetic resonance imaging scan results had signs of brain atrophy, 9 showed enhanced gyrus-like blood vessel formation by enhanced magnetic resonance imaging, and 5 showed decreased branches of the anterior and middle cerebral artery on the affected facial side by magnetic resonance angiography.Conclusions:Children with Sturge-Weber syndrome are clinically characterized by predominantly unilateral port wine stains on the face, some of whom are accompanied by epilepsy, glaucoma or mental retardation, and imaging examinations mainly show local calcification, brain atrophy, local thickening of the skull plate, enhanced gyrus-like blood vessel formation, etc. Early definite diagnosis and comprehensive systemic treatment are needed to reduce disability and mortality rates in patients with Sturge-Weber syndrome, and long-term follow-up should be considered.
ABSTRACT
Objective:To investigate the value of the size-specific dose estimate (SSDE) on dose estimations of children's head CT scans.Methods:A retrospective study was conducted on plain head CT scans of 252 patients with the 64-row detector CT device of Discovery 750HD in the Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from January to September in 2019. The volume CT dose index (CTDI vol)values were recorded. The head circumference (HC), area, and CT value were measured using a self-developed computer program, and the water equivalent diameter (WED), size-specific conversion factors ( f293 and f220), and absorbed dose (SSDE 293 and SSDE 220) were calculated according to the AAPM reports 293 and 220.The patients were divided into three groups by the quartering meth odaccording to their HC(<47.8 cm, 47.8-52.7 cm, >52.7 cm)and four groups based on their ages(0-2, 3-6, 7-10 and 11-14 years old). The difference between parameters ( f220 and f293, SSDE 220 and SSDE 293、SSDE 293 and CTDI vol) were compared for different groups, and the correlation of HC with f293 and SSDE 293 was analyzed. Results:There was an overestimation of f220 by 11.11% ( t=252.61, P<0.05) compared with f293. SSDE 220 was overestimated by 10.31% ( t=228.21, P<0.05) compared with SSDE 293, and SSDE 293 was underestimated by 9.60% ( t=-31.34, P<0.05)compared with CTDI vol. For the three HC groups, SSDE 220 was overestimated by 8.54%, 10.37%, and 11.57% ( t=73.73, 438.58, 275.52, P<0.05)compared with SSDE 293, and SSDE 293 was underestimated by 1.30%, 9.79%, and 14.61% ( t=-1.91, -60.95, -47.64, P<0.05)compared with CTDI vol. For the four age groups SSDE 220 was overestimated by 8.45%, 10.00%, 10.57%, and 11.36% ( t=63.58, 232.29, 247.84, 302.95, P< 0.05)compared with SSDE 293, and SSDE 293 was underestimated by 1.49%, 8.27%, 10.63%, and 13.78% ( t=-1.83, -28.27, -37.30, -49.80, P< 0.05)compared with CTDI vol. Furthermore, HC was highly correlated with f293 and SSDE 293 ( r2=0.88 and 0.76, respectively, P< 0.05). Conclusions:The radiation dose in children′s head CT scanning can be more accurately estimated according to the AAPM Report 293, while it can be overestimated by CTDI vol. Meanwhile, the CT radiation dose can be patently overestimated with the AAPM Report 220 compared with Report 293.HC is closely correlated with f293 and SSDE 293 and it can be used to estimatee more accurately for SSDE and the radiation dose received by children during head CT scanning.
ABSTRACT
OBJECTIVE: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency.MATERIALS AND METHODS: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained.RESULTS: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy.CONCLUSION: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.
ABSTRACT
Attention deficit/hyperactivity disorder(ADHD)is a common neuropsychiatric disorder in childhood, and its etiology and pathogenesis have not been fully elucidated.The main clinical manifestations are attention deficit, hyperactivity and impulsive behavior.Studies have shown that cognitive dysfunction is an important pathogenic factor, and executive dysfunction is a core deficiency of ADHD, which has poor performance in terms of attention, inhibition control and planning ability.ADHD patients often suffer from learning difficulties, conduct disorders, and oppositional defiantness.The clinical symptoms and executive dysfunction of some children with ADHD can persist into adulthood, causing serious effects on their academic, quality of life, and social functions.Therefore, the study of ADHD executive function has significant clinical and social significance.This article summarizes the research on the neural basis of executive dysfunction in ADHD children in recent years, improves the understanding of the disease, and provides guidance for clinical objective diagnosis, evaluation and treatment.
ABSTRACT
Objective:To preliminarily evaluate clinical efficacy and safety of tazarotene 0.05%/betamethasone dipropionate 0.05% cream in the treatment of psoriasis vulgaris.Methods:A multicenter, randomized, double-blinded, single-dummy, parallel-controlled clinical trial was conducted. Subjects with mild to moderate psoriasis vulgaris were randomized into 4 groups at a ratio of 2∶1∶1∶1, including tazarotene 0.05%/betamethasone dipropionate 0.05% cream (Taz/Bp) group, betamethasone dipropionate 0.05% cream (Bp) group, tazarotene 0.05% gel (Taz) group and cream vehicle control (Plb) group. The treatment lasted 4 weeks. After 1, 2 and 4 weeks of treatment, efficacy and safety of drugs were evaluated in the above groups. Two-way analysis of variance model with main effects was used to compare continuous indices, least significant difference t-test was used for multiple comparisons, and chi-square test or Fisher′s exact test for comparisons of categorical data. Results:A total of 300 subjects were enrolled from 7 research centers, including 120 in the Taz/Bp group, 60 in the Bp group, 60 in the Taz group and 60 in the Plb group. After 4 weeks of treatment, proportions of patients achieving a 75% reduction in PASI (PASI75) were 35.83%, 20.00%, 18.33% and 6.67% in the Taz/Bp, Bp, Taz and Plb groups respectively, and there was a significant difference among the 4 groups ( P < 0.05) ; the proportion of patients achieving PASI75 was significantly higher in the Taz/Bp group than in the Plb group (α = 0.05, P < 0.05) and Taz group (α = 0.025, P < 0.025) , but there was no significant difference between the Taz/Bp group and Bp group (α = 0.016 7, P > 0.016 7) ; the proportions of patients achieving PASI90 were 25.00%, 8.33%, 5.00% and 1.67% in the Taz/Bp, Bp, Taz and Plb groups respectively, which significantly differed among the 4 groups ( P < 0.05) , and the Taz/Bp group showed a significantly increased proportion of patients achieving PASI90 compared with the Plb group ( P < 0.05) , Taz group ( P < 0.025) and Bp group ( P < 0.016 7) . All the tested drugs were well tolerated in the 4 groups. Adverse drug reactions occurred in 15 (12.50%) , 5 (8.33%) , 19 (31.67%) and 9 (15.00%) patients in the Taz/Bp, Bp, Taz and Plb groups respectively. The incidence rate of adverse drug reactions significantly differed among the 4 groups ( P = 0.004) , and was significantly lower in the Taz/Bp group than in the Taz group ( P < 0.05) , but insignificantly different between the Taz/Bp group and Bp or Plb group (both P > 0.05) . Conclusion:Tazarotene 0.05%/betamethasone dipropionate 0.05% cream is effective and safe for the treatment of psoriasis vulgaris.