ObjectiveTo analyze the clinical efficacy and safety of rapamycin in the treatment of Tuberous sclerosis complex ( TSC ) complicated with refractory epilepsy, and to provide scientific basis for the clinical treatment of this disease.MethodsRetrospective analysis was performed on 22 children with TSC complicated with refractory epilepsy admitted to Henan People's Hospital from 2017 to 2019, including 11 males and 11 females who met the inclusion criteria, with an average age of (27.91±36.92) months. They were treated with antiepileptic drugs and rapamycin at the same time, and followed up for at least 1 year.To observe the change of seizure frequency before and after treatment with rapamycin.ResultsThe mean reduction rate of seizure frequency in children with tuberous sclerosis complicated with refractory epilepsy was 52.1% 6 months after the addition of rapamycin, and 51.2% 12 months after the addition of rapamycin. The number of seizure-free days could be maintained. The difference before and after the addition of rapamycin was statistically significant (P<0.05).ConclusionThe addition of rapamycin in the treatment of TSC complicated with refractory epilepsy can reduce the frequency of seizure and increase the number of days without seizure, and the adverse reactions are mild/moderate. Rapamycin has certain safety in children with regular follow-up.
ObjectiveTo systematically review the risk factors of refractory mycoplasma pneumoniae pneumonia (RMPP) in children. MethodsPubMed, The Cochrane Library, EMbase, CNKI, CBM, VIP, and WanFang Data databases were electronically searched for case-control studies and cohort studies on the risk factors of RMPP in children from inception to March 2021. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Meta-analysis was then performed using RevMan 5.3 software. ResultsA total of 27 case-control studies involving 3 967 children with RMPP and 11 613 children with common MPP were included. The results of meta-analysis showed that heat course (OR=2.07, 95%CI 1.98 to 2.16, P<0.000 01), length of hospital stay (OR=1.42, 95%CI 1.14 to 1.69, P<0.000 01), recurrent respiratory tract infection (OR=8.51, 95%CI 6.15 to 11.77, P<0.000 01), level of IL-6 (OR=21.95, 95%CI 20.85 to 23.06, P<0.000 01), level of CRP (OR=2.41, 95%CI 1.94 to 2.87, P<0.000 01), level of LDH (OR=0.79, 95%CI 0.53 to 1.06, P<0.000 01), level of ESR (OR=2.65, 95%CI 1.13 to 4.18, P=0.000 6), combined pleural effusion (OR=9.42, 95%CI 3.65 to 24.31, P<0.000 01), combined with extrapulmonary complications (OR=3.33, 95%CI 2.42 to 4.58, P<0.000 01), large lung consolidation (OR=12.31, 95%CI 5.42 to 27.99, P<0.000 01) were the risk factors for RMPP. ConclusionsCurrent evidence indicates that heat course, length of hospital stay, repeated respiratory tract infection, high level of IL-6, high level of CRP, high level of LDH, high level of ESR, combined pleural effusion, combined extrapulmonary complications, and large lung consolidation are risk factors for children with RMPP. Due to limited quality and quantity of the included studies, more high-quality studies are required to verify the above conclusions.
In the treatment of drug-refractory epilepsy in children, surgical treatment has a good clinical effect. However, for children whose surgical site is difficult to determine and who cannot undergo resectional surgery, neuromodulation techniques are one of the treatments that can be considered. At present, new neuromodulation technologies in children mainly include transcutaneous vagus nerve stimulation (transcutaneous auricular vagus nerve stimulation, ta-VNS), deep brain stimulation (deep brain stimulation, DBS), reactive nerve stimulation (responsive neurostimulation, RNS), transcranial magnetic stimulation (transcranial magnetic stimulation, TMS), transcranial direct current stimulation (transcranial direct current stimulation, TDCS) and transcranial alternating current stimulation (transcranial alternating current stimulation, TACS). This article briefly discussed the clinical efficacy and safety of various currently available neuromodulation technologies, so as to provide a reference for the rational selection and application of neuromodulation technologies, and improve the clinical efficacy and quality of life of children with drug-refractory epilepsy.
Objective To investigate the compliance of ketogenic diet in children with refractory epilepsy and its correlation with the curative effect, and to provide support and basis for the implementation and improvement of the long-term management of ketogenic diet in this patient population. MethodsA total of 106 children and their families who were followed up on ketogenic diet in the Department of Pediatrics of Fudan University from March 2019 to January 2022 in the Department of Ketogenic Multidisciplinary Treatment (MDT) were selected by convenience sampling method. General data questionnaire and ketogenic compliance questionnaire were used for investigation and follow-up. ResultsThe mean compliance of ketogenic diet in refractory epilepsy children was (13.27±3.68). The compliance scores of ketogenic children with different therapeutic effects and cognitive functions were significantly different. The compliance score was significantly correlated with the therapeutic effect and cognitive level, that is, the higher the therapeutic effect of ketogenic diet in children with higher compliance score, the better the cognitive improvement. ConclusionThe compliance of ketogenic diet in children with refractory epilepsy needs to be further improved. Improving the compliance of ketogenic diet is of great significance for the efficacy of ketogenic diet. Medical staff should actively develop the corresponding intervention program and follow-up management mode to further improve the treatment compliance of children's families, improve the treatment effect and improve the quality of life of children's families.
Objective To observe the therapeutic effect of autologous neurosensory retinal transplantation in repairing unhealed giant macular hole after pars plana vitrectomy (PPV). MethodsA prospective clinical study. From July 2022 to December 2023, 12 patients (12 eyes) with refractory large macular hole who received autologous neurosensory retinal transplantation treatment in Department of Ophthalmology of the First Affiliated Hospital of Zhengzhou University were selected for the study. The macular hole in affected eyes still did not close after PPV combined with inner limiting membrane removal or tamponade, and the diameter of macular hole were greater than 600 μm. All affected eyes received best corrected visual acuity (BCVA) and optical coherence tomography (OCT) examinations. The BCVA examination employed the international standard visual acuity chart, with results converted to logarithm of the minimum angle of resolution (logMAR) visual acuity for statistical analysis. During the surgery, a piece of healthy retinal neuroepithelial tissue, approximately 0.3 optic disc diameters larger than the macular hole, was removed from the upper retinal periphery and used as a graft. The graft was inserted into the macular hole with the aid of intraoperative OCT. Post-surgery, the vitreous cavity was filled with silicone oil or sterile air. The follow-up period after surgery was 6 months. The thickness of the retinal grafts was measured using the same equipment as before surgery at 3 days, 1, 3, and 6 months post-surgery. The primary focus was on observing the macular hole closure rate and changes in BCVA at 6 months post-operation. A paired t-test was used to compare BCVA before and after surgery. Results In the sample of 12 cases (12 eyes), there were 5 males with 5 eyes and 7 females with 7 eyes. The mean age was (50.4±12.6) years. The mean macular hole diameter was (1 085.6±344.0) μm; The mean eye axis length was (27.64±4.19) mm. At 6 months after surgery, all affected eyes showed macular hole were completely closed (100.0%, 12/12). The thickness of the retinal graft was measured as (206.8±21.0), (170.8±23.3), (165.6±31.6), and (157.9±31.1) μm at 3 days, 1, 3, and 6 months post-surgery, respectively. At before and 6 months after surgery, the logMAR BCVA of the affected eyes was 1.28±0.39 and 0.95±0.22, respectively. The difference in logMAR BCVA before and after surgery was statistically significant (t=3.40, P＜0.05). Conclusion Autologous neurosensory retinal transplantation could effectively improve the closure rate of refractory large macular hole and improve or stabilize vision in the short run.
ObjectiveTo observe the clinical efficacy of Xiao’er kang xian capsule added to anti-seizure medications (ASMs) in the treatment of children with refractory epilepsy and its influence on serum neuron-specific enolase (NSE) and cludter of differentiation 19+ (CD19+) levels. Methods A total of 60 children with refractory epilepsy were selected from the pediatric outpatient department and ward of Guangdong Provincial People's Hospital from February 2021 to June 2023. The study subjects were divided into two groups by numerical random method,with 30 cases in each group. The children with Xiao’er kang xian capsule added to the original treatment were the treatment group and the children without Xiao’er kang xian capsule added to the original treatment were the control group. The frequency, duration, EEG characteristics, adverse reactions and changes in serum NSE and CD19+ levels of the two groups were compared after treatment. Results Self-control before and after treatment in the treatment group: the frequency and duration of seizures were significantly reduced, with statistical difference (P<0.05). EEG discharge index in awake period and sleep period were significantly decreased, with statistical difference (P<0.05). After 6 months of treatment, comparison between the two groups of children: the seizure frequency of children in the treatment group was significantly decreased compared with the control group (P=0.03). There was a statistical difference (P<0.05), and the seizure duration in the treatment group was less than that in the control group (P=0.863), the clinical effective rate of treatment group 83.33% was higher than that of control group 63.33% (P=0.08), the effective rate of EEG in treatment group 80% was higher than that of control group 60% (P=0.091), serum NSE and CD19+ in treatment group were lower than that of control group, with no statistical difference (P>0.05). After 12 months of treatment, the frequency and duration of seizures in the treatment group were significantly decreased (P<0.05). The clinical efficacy and effectiveness of treatment group were significantly higher than that of control group (P=0.038). The incidence of adverse reactions in both groups was 16.67% (P>0.05). The effective rate of EEG in treatment group was significantly higher than that in control group (P=0.053). Serum NSE and CD19+ in treatment group were significantly lower than those in control group (P<0.05). ConclusionFor children with refractory epilepsy, the addition of Xiao’er kang xian capsule on the basis of the original treatment has obvious effect low adverse reaction and high safety. NSE and CD19+ can be used as monitoring indicators for the influence of the disease and prognosis evaluation during the treatment of children with epilepsy.
Objective To investigate the clinicalmanifestations, electroencephalogram (EEG) characteristics, surgical treatment and prognosis of epilepsy secondary to Sturge-Weber syndrome (SWS) in children.Methods The data of 7 children with epilepsysecondaryto Sturge-Weber syndrome who were treated by surgery from May 2015 to May 2020 in our Children's Epilepsy Center were retrospectively reviewed. Their demographic characteristics, seizure forms, results of EEG and cranial imaging investigations, surgical methods, postoperative pathological reports and prognosis during follow-upwere summarized and analyzed. The prognosis were evaluated byEngel classificationat the last time point during follow-up. Results Totally 7 pediatric patients were enrolled, including 1 male (16/25, 64.0%) and 6 females.All the 7 cases presented with focal seizures at the onset among whom 2 cases developed status epilepticus during the course of the disease(epilepsiapartialiscontinuain 1 case),one case had epileptic spasmsand 1 case was characterized by cluster seizures. The interictal EEG manifestations of the cases gradually deteriorated as the course of the disease prolonged, including the slow wave on the affected side gradually increases (7/7), the amplitude gradually decreases (7/7), and the physiological wave disappears (4/7). Besides, no epileptiform discharges/incidental or a few epileptiform dischargeswere found in their interictal EEGs. Four cases underwent hemispherotomy, 1 case underwent temporo-parieto-occipital disconnection and 2 cases underwent lesion resection. The cases were followed up for 6 months to 5 years, and the average follow-up duration was 79.29 months. Six cases were rated as Engel Ⅰa during the regularfollow-up. Only 1 case was rated asEngel Ⅱ and Engel Ⅲ at 3 month and 1 year after the operation. ConclusionChildren with epilepsy secondary to SWS usually present with focal seizures and have diverse seizure forms. The EEG show characteristic changes. For the caseswith drug refractory epilepsy, detailed preoperative evaluation and reasonable surgical methods can result in a better therapeutic effect.
ObjectiveTo observe the effectiveness and safety of pars plana vitrectomy (PPV) combined with inner limiting membrane (ILM) removal and 41G microneedle subretinal injection of balanced salt solution (BSS) in the treatment of refractory macular hole. MethodsA prospective clinical study. From January to June 2023, 20 cases (20 eyes) of refractory macular hole patients diagnosed through examination at The Affiliated Eye Hospital of Nanchang University were included in the study. The basal diameter of the affected eye's basal diameter (BD) was ＞1 000 μm. Macular hole index (MHI) was ＜0.5. The affected eye received treatment with 23G PPV combined with ILM removal and 41G microneedle subretinal injection of BSS. Best corrected visual acuity (BCVA), microperimetry, and optical coherence tomography angiography (OCTA) were performed before and 1, 2, 3, and 6 months after surgery for the affected eye. BCVA examination was performed using standard logarithmic visual acuity chart, and convert it to logarithmic minimum resolution angle (logMAR) visual acuity for statistical purposes. MP-3 microperimetry was used for micro view examination, record the mean sensitivity (MS) of the retinal within a 12° range of the fovea. OCTA was used to measure the area of the avascular zone of the macula (FAZ), perimeter of the FAZ (PERIM), retinal vascular length density (VLD), and vascular perfusion density (VPD). The changes in BCVA, MS, FAZ area, PERIM, VLD, VPD before and after surgery were compared and analyzed. After the same time, the closure of macular hole and the occurrence of complications after surgery were observed. Single factor analysis of variance was used to compare the observation indicators at different times before and after surgery. The correlation between various observation indicators and preoperative minimum diameter (MD), BD, and hiatus height at 6 months after surgery were analyzed using Pearson correlation analysis. ResultsAmong the 20 cases with 20 eyes, there were 2 males with 2 eyes and 18 females with 18 eyes. Age was (61.45±8.56) years old. The logMAR BCVA, MS, FAZ area, PERIM, VLD, and VPD of the affected eye were 1.46±0.21, (16.20±5.81) dB、(0.40±0.17) mm2, (2.89±0.99) mm, (6.23±3.59) mm−1, (0.17±0.10)%, respectively. Six months after surgery, out of 20 eyes, macular hole closure and incomplete closure were 18 (90.0%, 18 /20) and 2 (10.0%, 2 /20) eyes, respectively. The logMAR BCVA, MS, FAZ area, PERIM, VLD, and VPD were 0.80±0.20, (22.20±4.60) dB, (0.18±0.10) mm2, (1.83±0.80) mm, (9.54±2.88) mm−1, (0.31±0.14)%. Compared with before surgery, the differences were statistically significant (P＜0.05). The correlation analysis results showed a positive correlation (P＜0.05) between preoperative BD and postoperative 6-month PERIM and VPD. There was a negative correlation between preoperative MD and postoperative VLD at 6 months (P＜0.05). There was a negative correlation between preoperative MHI and logMAR BCVA and VPD at 6 months after surgery (P＜0.05). No complications such as elevated or decreased intraocular pressure, damage to retinal pigment epithelium, retinal hemorrhage, endophthalmitis, or retinal detachment occurred after surgery in all affected eyes. ConclusionMinimally invasive PPV combined with ILM removal and 41G microneedle subretinal injection of BSS can effectively improve the closure rate of refractory macular hole patients in the short term, improve vision, and have good safety.
ObjectiveTo investigate the effects of continuous positive airway pressure (CPAP) ventilation on blood pressure and related inflammatory factors in patients with obstructive sleep apnea hypopnea syndrome (OSAHS) and refractory hypertension, and explore the correlation between respiratory function and related inflammatory factors.MethodsPatients with OSAHS and refractory hypertension who visited Shenzhen Nanshan People’s Hospital between January 2014 and September 2018 were randomly divided into a CPAP group and a control group. The control group received routine treatment. The CPAP group received CPAP on the basis of conventional treatment. Each intervention lasted for 7 days. The changes of systolic and diastolic blood pressure were measured before and after 1, 3, 5, and 7 days, respectively. The respiratory function and related inflammatory factors were measured after 7 days of intervention.ResultsAfter 7 days of treatment in the CPAP group, the levels of apnea hyperpnoea index (AHI), lowest oxygen saturation and oxygen desaturation index were significantly better than those before intervention, with the improvement of respiratory function significantly higher than that of the control group after 7 days. Meanwhile the systolic and diastolic blood pressure levels were significantly lower in the CPAP group than those of the control group at 1, 3, 5, and 7 days, and lower than the pre-intervention after 7 days of intervention. After 7 days of intervention, the inflammatory factors in both groups were significantly improved (all P<0.05). However, the serum levels of high sensitivity C-reactive protein (hs-CRP), interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α) and endothelin-1 (ET-1) in the CPAP group were significantly lower than those of the control group after 7 days of treatment (P<0.05). There was a significant positive correlation between AHI level and hs-CRP, IL-6, TNF-α and ET-1 in the CPAP group after intervention (P<0.001).ConclusionsCPAP can significantly improve the blood pressure of patients with OSAHS and refractory hypertension. CPAP may improve the related inflammatory factors by improving the respiratory function of patients, thus reduce the patient’s blood pressure.
ObjectiveIn order to evaluate the efficacy, safety and tolerability of adjunctive perampanel in children with refractory epilepsy. MethodsThis study collected medical records of 34 children with refractory epilepsy, who were admitted to Children’s Hospital of Soochow University from January 2020 to January 2021. By comparing the baseline status with the status at 4, 8, 12, 24, 36, and 48 weeks of follow-up, the efficacy and adverse reactions of perampanel were evaluated. ResultsThe mean age of the patients treated with perampanel was 8.1±4.1 years. The male-to-female ratio was 1: 1. After the addition of perampanel, the average responder rate at the 4th, 8th, 12th, 24th, 36th, 48th weeks were 37.5%, 46.7%, 50.0%, 47.4%, 53.8%, 42.9%. The adverse events were reported by 32.4%, and the retention rate was 88.2%. ConclusionsPerampanel has good efficacy, safety and tolerability in the treatment of refractory epilepsy. Moreover, personalized treatment and better baseline seizure control may increase the effectiveness and retention rate of perampanel.