ObjectiveTo investigate the clinical characteristics of epileptics with pregnancy and then provide reference for standardized management of epileptics with pregnancy. MethodsFrom June 2012 to June 2021, epileptics with pregnancy who delivered in Jinan Central Hospital were selected as the research subjects. The clinical data such as the application of Antiseizure medications (ASMs) during pregnancy, seizure frequency, pregnancy outcomes, delivery ways, offspring feeding ways and the incidence of complications were investigated and analyzed. ResultsAmong 36 epileptics with pregnancy, 20 cases (55.56%) were treated with ASMs alone, 5 cases (13.88%) were treated with combined medication, and 11 cases (30.56%) were treated without ASMs during pregnancy. 15 cases (41.67%) adhered to systematic application of ASMs, 17 cases (47.22%) did not adhere to systematic application of ASMs, and 4 cases (11.11%) had unknown medication history. The frequency of seizures increased in 5 cases, decreased in 7 cases and unchanged in 24 cases during pregnancy. Pregnancy outcomes: full-term delivery in 33 cases (91.67%), preterm delivery in 1 case (2.78%) and abortion in 2 cases (5.56%). Delivery mode: cesarean section in 31 cases (91.18%), vaginal delivery in 3 cases (8.82%). After delivery, 4 cases (11.76%) were fed with milk powder and 30 cases (88.24%) were breast-fed. Complications: There were 6 cases complicated with anemia (16.67%), 5 cases complicated with gestational hypertension (13.89%), 3 cases complicated with gestational diabetes (8.33%), 4 cases complicated with premature rupture of membranes (11.11%), 2 cases complicated with fetal growth restriction (5.56%), 2 cases complicated with oligohydramnios (5.56%), 3 cases complicated with fetal distress (8.33%) and 3 cases complicated with neonatal asphyxia (8.33%). ConclusionsThe proportion of epileptics with pregnancy who were systematically treated with ASMs was low and the seizures were poorly controlled. There is a lack of standardized management for such patients in clinical practice.
ObjectiveTo analyze the clinical and epidemiological characteristics of hospitalized avian influenza A (H7N9) virus infections in Hunan province from 2013 to 2017, and provide evidences for control, diagnosis and treatment of this disease.MethodsNinety-one hospitalized patients were confirmed with H7N9 infection in Hunan. Excluding 2 patients less than 18 years old and 10 with missing data, 79 patients with H7N9 infection were analyzed.ResultsMost confirmed cases were affected in the second and fifth epidemic wave and number of patients in the fifth wave was more than the sum in prior 4 waves. Epidemiological characteristics, clinical symptoms and case fatality did not change significantly. Administration of antiviral drugs was more active in the fifth wave [from illness onset to antiviral drug: (6.3±2.4)d vs. (7.6±2.4)d, P=0.047]. Multiple logistic regression analysis showed that shock (OR=4.683, 95%CI 1.136–19.301, P=0.033) was the independent risk factor of H7N9 infections. There were no significant differences in case fatality among group oseltamivir, group oseltamivir+peramivir, and group peramivir.ConclusionsPatients with avian influenza A (H7N9) increased in the fifth wave but clinical characteristics changed little. Antiviral treatment should be more active. Shock is an independent risk factor of H7N9 infections. Oseltamivir-peramivir biotherapy can not reduce case fatality compared with oseltamivir or peramivir monotherapy.
ObjectiveTo explore the clinical characteristic, operation time, and methods of elderly calculous cholecystitis. MethodsThe data of 386 cases of elderly calculous cholecystitis in our hospital from January 2008 to April 2014 were retrospectivly analyzed. ResultsIn 386 patients, 234 cases were chronic cholecystitis, 152 cases were acute calculous cholecystitis; there were preoperative complications in 174 cases (45.08%); 234 cases of chronic calculous cholecystitis patients underwent elective operation, 35 cases in 152 cases of acute phase underwent operation at 72 h, the remaining 117 cases underwent operation in within 2 weeks of onset. Laparoscopic cholecystectomy (LC) were in 283 cases, including transfer laparotomy operation in 8 cases; underwent conventional open cholecystectomy in 103 cases. Postoperative complications occurred in 49 cases, the complication rate was 12.69%, including incision infection, pulmonary infection, acute urinary retention, urinary tract infection, biliary fistula and so on. Three hundreds and eighty-four cases were cured, 2 cases died, for cholecystolithiasis complicated with severe acute cholangitis, died from multi organ failure in 3 days after operation. ConclusionsThe clinical characteristics of elderly calculous cholecystitis is unique. To strictly grasp the operation indication, selection of operation time, take individual operation method with the disease, and strengthen treatment of perioperative period are the key to improve the cure rate and the operation success rate of elderly cholecystitis.
Objective To systematically review the prevalence, clinical characteristics, and prognosis of fulminant type 1 diabetes mellitus (F1DM) in China. Methods The CNKI, WanFang Data, CBM and PubMed databases were searched to collect Chinese F1MD case reports from January 1, 2000 to March 30, 2022. Data analysis was performed using Stata 16.0 software and RevMan 5.3 software. Results A total of 874 cases were included in 233 papers, involving 410 males (46.91%) and 464 females (53.09%). The age of onset was 29.32±1.09 years and the course of disease was 3.74±0.63 days. The BMI was 21.18±0.52 kg/m2, HbA1c was 6.58%±0.08%, the level of fasting C-peptide was 0.04±0.010 ng/mL, level of C-peptide 2 h after meal was 0.09±0.020 ng/mL, the level of blood glucose at the doctor’s office was 34.72±2.89 mmol/L, and the level of arterial blood gas pH was 7.09±0.015. Among them, 734 patients had diabetic ketoacidosis (84.55%), 496 patients had infection of the upper respiratory or digestive tract before onset (56.75%), 4 patients died (0.46%), 78 patients were GADA positive, 11 patients were ICA positive, 13 patients were IAA positive, and 109 were pregnant patients (90 fetal deaths, 82.57%). Conclusion Chinese F1DM is a special but common subtype of diabetes. Its characteristics include a relatively young age of onset, devastating islet damage, and rapid progression, and it is often accompanied by severe metabolic disorders, complications, and grim prognosis. Clinicians should pay more attention to F1DM.
ObjectiveTo systematically review the clinical and genetic features of permanent neonatal diabetes mellitus (PNDM) case reports. MethodsThe PubMed, Embase, Scopus, SinoMed, Web of Science, CINAHL, Medrxiv, VIP, CNKI and WanFang Data databases were electronically searched to collect PNDM case reports from inception to June 2023. Two reviewers independently screened literature, extracted data and assessed the reporting quality of the included studies. Descriptive analysis was performed. ResultsA total of 105 case reports were finally included. Typical clinical manifestations of PNDM were early onset of persistent hyperglycemia, developmental delay and low birth weight. The results of genetic testing showed that mutations in the KCNJ11, INS, EIF2AK3, GCK, ABCC8, PTF1A, GATA6, IER3IP1, SLC19A2, NEUROG3, PDX1, and 6q24 genes were closely associated with the development of PNDM. In addition, there may be different clinical manifestations and prognosis of PNDM in different genotypes. ConclusionThis study reveales the clinical characteristics and genetic pattern of PNDM, and provides a direction for further research on the mechanism of PNDM.
Objective To research clinical manifestations, electrophysiological characteristics of epileptic seizures arising from diagonal sulci (DS), to improve the level of the diagnosis and treatment of frontal epilepsy. MethodsWe reviewed all the patients underwent a detailed presurgical evaluation, including 5 patients with seizures to be proved originating from diagonal sulci by Stereo-electroencephalography (SEEG). All the 5 patients with detailed medical history, head Magnetic resonance (MRI), the Positron emission computered tomography (PET-CT) and psychological evaluation, habitual seizures were recorded by Video-electroencephalography (VEEG) and SEEG, we review the intermittent VEEG and ictal VEEG, analyzing the symptoms of seizures. Results 5 patients were divided into 2 groups by SEEG, group 1 including 3 patients with seizures arising from the bottom of DS, group 2 including 2 patients with seizures arising from the surface of DS, all the tow groups with seizures characterized by both having tonic and complex motors, tonic seizures were prominent in seizures from left DS, and tonic seizures may absent in seizures from right DS. Intermittent discharges with group1 were diffused, and intermittent discharges with group 2 were focal, but both brain areas of frontal and temporal were infected. Ictal EEG findings were consistent with the characteristics of neocortical seizures, the onset EEG shows voltage attenuation, seizures from bottom of DS with diffused EEG onset, and seizures from surface of DS with more focal EEG onset, but both frontal and anterior temporal regions were involved. Conclusionthe symptom of seizures arising from DS characterized by tonic and complex motor, can be divided into seizures arising from the bottom of DS and seizures from the surface of DS, with different electrophysiological characters.
Objective To explore the correlation of Gli1 expression with clinical characteristics and prognosis of liver cancer. Methods Such Databases as PubMed, Embase, CNKI, Wanfang Database and Chinese Biomedical Literature Database were searched to collect cohort studies, which has published on the correlation between the expression of Gli1 and the clinical pathologic features of liver cancer and its prognostic value from the establishment of the databases to December 2016. Results Twelve studies with a total of 831 patients were included in this study. The high expression of Gli1 was associated with the tumor diameter >5 cm [relative risk (RR)=1.39, 95% confidence interval (CI) (1.08, 1.79)], clinical stage [RR=1.26, 95%CI (1.05, 1.51)], intrahepatic metastasis [RR=1.39, 95%CI (1.06, 1.83)] and venous invasion [RR=1.44, 95%CI (1.01, 2.04)], but the correlation of Gli1 expression was not significant with gender, histological differentiation, tumor diameter >3 cm, hepatitis B virus, cirrhosis and alpha fetoprotein. Meanwhile, the results of Meta-analysis showed that the higher Gli1 expression in liver cancer patients had a worse 3-/5-year overall survival rate and overall survival rate than those of the lower Gli1 expression group [3-year, RR=3.38, 95%CI (2.01, 5.67); 5-year, RR=1.51, 95%CI (1.19, 1.91); overall survival, RR=1.60, 95%CI (1.11, 2.30)]. And the higher Gli1 expression in liver cancer patients had a worse disease free survival rate than that in the lower Gli1 expression group [RR=1.89, 95%CI (1.35, 2.66)]. Conclusion The high expression of Gli1 is associated with poor prognostic outcome in liver cancer, and may be used as an important prognostic marker for patients with liver cancer.
Objective To analyze the BEST1 gene mutations and clinical features in patients with multifocal vitelliform retinopathy (MVR). Methods This is a retrospective case series study. Five MVR families with MVR, including 9 patients and 10 healthy family members were recruited. Clinical evaluations were performed in all MVR patients and their family members, including best-corrected visual acuity (BCVA), intraocular pressure (IOP), refraction, slit-lamp examination, 90 D preset lens examination, gonioscopy, color fundus photography, optical coherence tomography (OCT), fundus autofluorescence (AF), ultrasound biomicroscopy (UBM) and axial length measurement. Electro-oculogram (EOG) was performed in 12 eyes and visual field were performed in 13 eyes. Peripheral blood samples were collected in all subjects to extract genomic DNA. Coding exons and flanking intronic regions of BEST1 were amplified by polymerase chain reaction and analyzed by Sanger sequencing. Results Among the 5 MVR families, 3 probands from three families had family history, including 1 family had autosomal dominant inheritance pattern. Two patients from 2 families were sporadic cases. Screening of BEST1 gene identified four mutations, including three missense mutations (c.140G>T, p.R47L; c.232A>T, p.I78F; c.698C>T, p.P233L) and 1 deletion mutation (c.910_912del, p.D304del). Two mutations (p.R47L and p.I78F) were novel. The BCVA of affected eyes ranged from hand motion to 1.0. The mean IOP was (30.39±11.86) mmHg (1 mmHg=0.133 kPa). The mean refractive diopter was (-0.33±1.68) D. Twelve eyes had angle-closure glaucoma (ACG) and 4 eyes had angle closure (AC). EOG Arden ratio was below 1.55 in all patients. The mean anterior chamber depth was (2.17±0.29) mm. Visual field showed defects varied from paracentral scotoma to diffuse defects. The mean axial length was (21.87±0.63) mm. All MVR patients had multifocal vitelliform lesions in the posterior poles of retina. ACG eyes demonstrated pale optic disc with increased cup-to-disc ratio. OCT showed retinal edema, extensive serous retinal detachment and subretinal hyper-reflective deposits which had high autofluorescence in AF. The genetic testing and clinical examination were normal in 10 family members. Conclusions MVR patients harbored heterozygous mutation in the BEST1 gene. Two novel mutations (p.R47L and p.I78F) were identified. These patients had clinical features of multifocal vitelliform retinopathy and abnormal EOG. Most patients suffered from AC/ACG.
Objective To summarize the cl inical characteristics of 596 patients with fracture in Wenchuan earthquake and to investigate the therapeutic methods and effects of early treatment. Methods From May 12th 2008 to May 21st 2008, 596 patients with fracture caused by Wenchuan earthquake were treated, including 283 males and 313 females aged1.9-102 years (median 43 years). The time from injury to hospital ization varied from 12 minutes to 4 days. There were 132 cases of upper extremity fracture, 496 cases lower extremity fracture, 10 cases clavicular fracture, 16 cases scapular fracture, 23 cases pelvis fracture, and 59 cases spinal fracture. Among them, 183 cases were open fracture and 413 cases were closed fracture. And 214 cases had multiple fracture (35.9%) and 68 cases had crush injury in l imbs which scored (6.84 ± 2.48) points according to the mangled extremity severity score (MESS). Thirty-six cases were combined with neurovascular injury. The wound of the open fracture was contaminated at different degrees, but no gas gangrene was observed. Open fracture was treated with suturing or no suturing after debridement, open reduction and internal or external fixation. Closed fracture was fixed with spl ints, cast and traction. Forty-nine patients whom were highly suspected as osseous fascia compartment syndrome received incision decompression timely, and 34 patients whose MESS were above 7.0 points or suffering from crush injury of l ifethreatening systemic symptoms received amputation. Results Apart from 34 patients receiving amputation, 460 patients achieved functional reduction of fracture after manipulative reduction and 102 cases got satisfactory reduction after surgery. Postoperatively, 289 patients were transferred to other hospitals. Among the rest 307 patients, 34 with severe wound infection were healed after multiple debridement, anti-infection, and skin flap transplantation (16 cases healed by first intention and 18 cases healed by second intention), 42 cases with crush syndrome were treated with open decompression and amputation, and nodeep venous thrombosis of lower l imb, stress ulcer and death were observed after operation (29 cases healed by first intention and 13 cases healed by second intention). Conclusion By aiming at the features of fracture caused by earthquake, the prompt and professional treatment can achieve good therapeutic effects.
ObjectiveTo enhance the cognition about the clinical characteristics of diffuse panbronchiolitis (DPB). MethodsThe data of patients with DPB searched out on the computer from January 1996 to September 2013 were retrospectively studied. ResultsThe 91 patients had a male to female ratio of 2︰1, and the mean age at onset was (40.5± 18.4) years old. The median course of disease was 7 years. The main clinical profiles included chronic cough, sputum production, exertional dyspnea, and crackles. A history of sinusitis occurred in 90.1% (82/91) of the patients. Positive rate of cold hemoagglutinin and HLA-B54 were 46.1% (30/65) and 42.1% (8/19), respectively. All patients' CT scans showed bilateral, diffuse, small centrilobular nodules. Lung function assessment showed an obstructive ventilation disfunction in 60.0% (51/85) of the patients and a mixed obstructive-restrictive pattern in 36.5% (31/85) of the patients. Thirty-four patients underwent lung biopsy. A total of 65.9% (60/91) of the patients had been misdiagnosed, and 98.8% (84/85) of the patients achieved significant improvement after the macrolide therapy. ConclusionDPB is not rare in China, but tends to be misdiagnosed or underdiagnosed. Macrolides can improve the prognosis of DPB.