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.
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.
ObjectivesTo identify the clinical characteristics and prognosis for CRKP (Carbapenem-resistant Klebsiella pneumonia, CRKP) infection among ICU patients in the Second Affiliated Hospital of Anhui Medical University. MethodsWe conducted a retrospectively analysis in which 19 patients infected by CRKP with another 21 CSKP (Carbapenem-sensitive Klebsiella pneumoniae, CSKP) infected patients from January 2017 to April 2018. Risk factors for CRKP infection were assessed. ResultsThe lower respiratory tract is the most common site of CRKP infection in our department. CRKP infection was associated with several clinical symptoms, particularly a higher incidence of sepsis shock (χ2=8.338, P=0.004), more application of the combined medicine (χ2=26.3, P<0.001), prolonged hospital stays (χ2=–2.217, P=0.027) and more expenses on antibiotics (χ2=12.855, P=0.005), and the declined survival rates in 14 days (χ2=4.269, P=0.039) and 21 days (χ2 =5.647, P=0.017). The resistance rate of CRKP strains was high, however no resistance to tegafycline was found. The risk factors of CRKP infection included three generations of cephalosporin and/or hydrocarbonase antibiotics exposure (χ2 =6.388, P=0.041), exposure time of three generations of cephalosporin (U=–2.187, P=0.029), exposure time of hydrocarbonase antibiotics (U=–2.103, P=0.035), tracheal intubation (χ2=6.352, P=0.012), tracheotomy (χ2 =4.821, P=0.028), SOFA score (t=4.505, P<0.001) and Charlson comorbidity index (t=3.041, P=0.004). The SOFA score was the only factor independently associated with CRKP bacteremia (P=0.02). ConclusionsCRKP infections in ICU directly affect the course of disease, survival time and treatment expenses of patients. Therefore, monitoring bacterial resistance, rational use of antibiotics, and protection of the immune function are of great significance for prevention and treatment of CRKP infection.
ObjectiveTo discuss clinical characteristics and nursing countermeasure of pregnant/delivery women with tuberculosis. MethodsFrom January 2012 to December 2013, 52 cases of pregnant women complicated with tuberculosis were included. We offered specific nursing according to the psychological counseling, reasonable administration, diet nursing, fever and prevention of hospital infection on the base of their clinical features, and then analyzed the effect. ResultsIn 52 patients who had undergone the tuberculosis and obsterrics and gynecology treatment, 4 puerperal patients died of severe pneumonia and multiple organ failure; in 48 gestational patients with tuberculosis, 23 early-middle term patients accept termination of pregnancy, and in 25 middle-late term patients, 3 had full-term natural labor, 4 underwent full-term cesarean section, 5 had premature labor, and 13 continued the pregnacy. Sixteen neonatus (including 4 given at the other hospitals) had normal results of physical examination without any deformity, in whom 5 had low body weight (body weight less than 2500 g), with negative results of tuberculin test and the result of 3-moth follow-up was normal. Three months after the anti-tuberculosis treatment, the lesion was obviously absorbed in 25 patients and in 23 patients within half of a year. No patients had hospital infection. ConclusionNursing care of pregnant patients with tuberculosis should strengthen the guidance on the patients health education, improve their compliance and self-protection knowledge. Also should strengthen the basic level medical personnel training and improve the ability of early diagnosis of tuberculosis and early treatment to reduce maternal mortality.
ObjectiveTo discuss the clinical characteristics of liver cirrhosis in elderly patients. MethodsWe retrospectively analyzed the clinical data of 67 patients (elderly group) with liver cirrhosis aged ≥60 treated between January 1998 and December 2010. Then, we compared these cases with another 72 liver cirrhosis patients (non-elderly group) aged<60. ResultsThe incidence of jaundice, ascites and albumin deficiency in the elderly patients was significantly higher than that in the non-elderly patients (P<0.05). Complications in the elderly group were relatively more, including electrolyte imbalance, infections, gastrointestinal bleeding, hepatic encephalopathy, liver cancer, liver and kidney syndrome and liver and lung syndrome, and the incidence of these complications was all significantly higher than the non-elderly group (P<0.05) except the liver and kidney syndrome (P>0.05). The causes of liver cirrhosis in both groups were similar. The most common cause was hepatitis B virus infection, followed by chronic alcoholism, but in the elderly group, chronic alcoholism, cholestasis, poisoning from medicines and poisons and liver blood circulation disorders were more common than the non-elderly group (P<0.05); hepatitis B and non-alcoholic fatty hepatitis were more common in the non-elderly group than in the elderly group (P<0.05). The elderly group had more Child-Pugh class C cases (P<0.05), while there were more class A cases in the non-elderly group (P<0.05). Twenty-six patients died in the elderly group with a mortality rate of 38.8%; while only 13 died in the non-elderly group with a mortality rate of 18.1%. The difference of mortality rate was significant between the two groups (P<0.05). Common causes of death in the elderly group were infection, hepatic encephalopathy, and electrolyte disorders and gastrointestinal bleeding, while the common causes of death in the non-elderly group were gastrointestinal bleeding and electrolyte disorders. ConclusionThe etiology, clinical manifestations and prognosis of liver cirrhosis in elderly patients differ from those in younger patients. We must pay more attention on treating complications of liver cirrhosis in elderly patients.
ObjectiveTo retrospectively study the acute epileptic seizures of patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR)and anti-leucine-rich glioma-inactivated 1(anti-LGI1)encephalitis. The characteristics and short-term prognosis provide reference for early clinical diagnosis and treatment.MethodsThe patients with anti-NMDAR and anti-LGI1 encephalitis who were admitted to the Department of Neurology of Sichuan Provincial People’s Hospital from January 2018 to June 2020 were continuously included. The general information, clinical manifestations, acute seizures and types of seizures were reviewed and analyzed.To evaluate the difference between the characteristics of two kinds of autoimmune encephalitis in the acute phase of seizures and the short-term prognosis.ResultsA total of 75 patients with anti-NMDAR encephalitis and anti-LGI1 encephalitis (41 males and 34 females) were included, of which average ages are(32.8±17.9)years, average courses are(1.8±1.1)months.59 and 16 are respectively positive for anti-NMDAR and anti-LGI1 antibodies, respectively. Of the 75 cases, 56 cases (74.7%) had seizures in the acute phase. Among the 56 cases of seizures, 38 cases (67.8%) were accompanied by disturbances of consciousness, 5 cases (8.9%) of autonomic dysfunction, and 24 cases of decreased oxygenation capacity. (42.9%) and 20 cases (35.7%) who were admitted to NICU, there was a significant statistical difference compared with the seizure-free group (P<0.05). The median age of anti-NMDAR encephalitis in the acute stage of seizures was 23 years, and that of anti-LGI1 encephalitis was 56.5 years (P<0.05). Anti-NMDAR encephalitis and anti-LGI1 encephalitis are common in the acute phase of epileptic seizures (55.9%vs.53.8%). Anti-NMDAR encephalitis has more frequent seizures and status epilepticus in the acute phase (P<0.05). After early and rational use of anti-epileptic drugs(AEDs) and immunotherapy and other symptomatic and supportive treatments, 70% of 56 patients were effectively controlled for seizure. Follow-up 3 months later, 18 patients (32.1%) stopped using anti-epileptic drugs (AEDs), While 30 patients (53.5%) continued to receive AEDs treatment, of which 25 patients (44.6%) had no seizures.ConclusionBoth anti-NMDAR encephalitis and anti-LGI1 encephalitis have a higher risk of seizures in the acute phase. Patients with seizures are more likely to have disturbances in consciousness, decreased oxygenation capacity, and higher rates of admission to NICU. Anti-NMDAR encephalitis is more common in young people around 30 years old, and anti-LGI1 encephalitis is more likely to develop around 60 years old. Patients with anti-NMDAR encephalitis are more likely to have abnormal electroencephalograms, have a longer average hospital stay, and are more likely to have recurrent seizures and status epilepticus in the acute phase. After timely diagnosis and intervention treatment, most patients' seizures can be well controlled. After the acute phase, AEDs can be withdrawed in one third of patients.
Objective To analyze the clinical features and outcomes of pregnant women with acute diarrhea. Methods We collected the clinical data of 133 pregnant women with acute diarrhea (group A) admitted into Tangshan Maternal and Child Health Hospital and Kailuan General Hospital between June 2009 and June 2015. Another 100 acute diarrhea patients without pregnancy (group B) and 100 pregnant women without acute diarrhea (group C) were regarded as controls. All the patients’ medical history, symptoms and signs, laboratory examination results, diagnosis and treatment, termination of pregnancy and pregnancy outcomes were retrospectively analyzed. Results The average age of group A patients was (29.72±5.19) years old, and 83.46% of them came to hospital within 24 hours. There were significant differences in vomiting, abdominal pain, white blood cells, neutrophils, C-reactive protein, white blood cells in the feces, fibrinogen, prothrombin time, activated partial prothrombin time, thrombin time and the recovery time of the laboratory indicators between group A and group B (P<0.05). The neonatal weight of group A was lower than that of group C, and the difference was statistically significant (P<0.05). Regression analysis showed that hemoglobin and albumin had significant influence on acute diarrhea during pregnancy (P<0.05). Conclusion Pregnancy with acute diarrhea is a kind of severe obstetric complication, which may lead to adverse pregnancy.
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.
ObjectiveTo investigate the clinical characteristics of tracheobronchial stenosis due to tracheobronchial tuberculosis (TBTB).MethodsWe recruited all patients diagnosed as TBTB within one year from four centers of Guangdong province, southern China. The prevalence, risk factors and bronchoscopy characteristics of tracheobronchial stenosis were analyzed. The location and pathological type of stenosis were also compared between different genders.ResultsA total of 345 patients were diagnosed with TBTB, 206 cases (59.7%) of which were concomitant with varying degrees of tracheobronchial stenosis. The stenosis was mainly located at upper lobe (50.0%) and gave priority to unilateral infiltration. The proportion of left and right-side stenosis were 53.9% and 44.2%, respectively. Forty-nine cases (23.8%) with tracheobronchia stenosis developed severe airway narrowing, the proportion of which was similar between left and right-side lung (49% vs. 51%). Severe stenosis in the left-side lung mainly involved in upper lobe (37.5%), main bronchus (37.5%) and the right-side mainly involved in upper lobe (44.0%), middle lobe or intermediate bronchus (40.0%). Among the TBTB patients, females, those with age ≥18 years, non-smokers, and those with symptom duration ≥4 weeks were more likely to be with tracheobronchial stenosis (P<0.05). The differences in gender between the patients with airway stenosis were as follows: the median age of female tracheobronchial stenosis patients was smaller than that of male patients (31 years vs. 43 years, P<0.05); stenosis were more likely involved in left side and main bronchus in female patients; ulceration necrosis of stenosis was more frequently seen in the female patients (P<0.05).ConclusionsTracheobronchial stenosis due to tracheobronchial tuberculosis is more frequently seen in young and middle-aged female patients. There are different stenosis location and lesion types between different genders.
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.