Clinical manifestations,diagnosis and long-term prognosis of adult autoimmune enteropathy:Experience from Peking Union Medical College Hospital

BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.

Pathogenesis,clinical manifestations,diagnosis,and treatment progress of achalasia of cardia

Achalasia cardia,type of esophageal dynamic disorder,is a relatively rare primary motor esophageal disease characterized by the functional loss of plexus ganglion cells in the distal esophagus and lower esophageal sphincter.Loss of function of the distal and lower esophageal sphincter ganglion cells is the main cause of achalasia cardia,and is more likely to occur in the elderly.Histological changes in the esophageal mucosa are considered pathogenic;however,studies have found that inflammation and genetic changes at the molecular level may also cause achalasia cardia,resulting in dysphagia,reflux,aspiration,retrosternal pain,and weight loss.Currently,the treatment options for achalasia focus on reducing the resting pressure of the lower esophageal sphincter,helping to empty the esophagus and relieve symptoms.Treatment measures include botulinum toxin injection,inflatable dilation,stent insertion,and surgical myotomy(open or laparoscopic).Surgical procedures are often subject to controversy owing to concerns about safety and effectiveness,particularly in older patients.Herein,we review clinical epidemiological and experimental data to determine the prevalence,pathogenesis,clinical presentation,diagnostic criteria,and treatment options for achalasia to support its clinical management.

COVID-19 infection with keratitis as the first clinical manifestation

·AIM:To report a case which keratitis is the first clinical manifestation of COVID-19 that occurred 3 d earlier than the common COVID-19 symptoms.·METHODS:Regular slit lamp examination,corneal scraping test,and chest computed tomography(CT)were performed for patients with COVID-19 infection.The ophthalmologic treatment included ganciclovir eye drop(50 mglmL,6 times/d).The treatment for diarrhea included Guifu Lizhong pills(TID).The antiviral therapy consisted of oseltamivir(75 mg capsule Q12 H);therapy preventing bacterial infection consisted of azithromycin(250 mg tablet QD)and moxifloxacin(0.4 g tablet Q12 H);and therapy for cough relief and fever prevention consisted of Chinese herbal decoction.·RESULTS:A 35-year-old male suddenly suffered pain,photophobia,and tears in his right eye for one day without systemic COVID-19 symptoms.Patient was diagnosed with keratitis,which was seemingly different from common keratitis.Ganciclovir eye drop was initiated.The corneal scraping test for COVID-19 was positive.The chest CT images were abnormal confirming the diagnosis of COVID-19 infection.The antiviral and antibacterial therapies were initiated.Chinese herbal therapy was used for cough relief and fever prevention.After roughly two weeks,patient recovered from COVID-19.·CONCLUSION:A new type of keratitis,atypical keratitis,is a clinical manifestation of COVID-19,and this clinical manifestation could appear 3 d earlier than fever and cough.The earlier a COVID-19 clinical manifestation is identified,the earlier can a patient be directed to stay at home,and significantly fewer people would be infected.

Clinical manifestations of patients with chronic pancreatitis

BACKGROUND: Recent advances in molecular and genomic technologies and pancreatic imaging techniques provided some insights into genetic, environmental, immunologic, and pathobiological factors for chronic pancreatitis (CP). This study was undertaken to investigate the clinical manifestations of patients with chronic pancreatitis at our hospital. METHODS: The data of the patients with CP who had been treated at our hospital between 1997 and 2004 were analyzed. RESULTS: The major symptoms of the patients with CP were abdominal pain, dyspepsia, loss of weight, diabetes mellitus, pancreatic pseudocyst, steatorrhea, and calcification. Biliary diseases were found to be the first cause of CP in this study; but alcohol abuse was the major cause of CP in men and biliary diseases were the first etiological factors for CP in women. The etiological difference of constituent ratio between men and women was related to alcohol comsumption (P<0.01). CONCLUSIONS: During the past 8 years, biliary diseases have been the major etiological factors for CP, but their constituent ratio is decreasing, and the constituent ratio of alcohol abuse is increasing gradually. Alcohol tends to replace biliary diseases as the primary etiological factor for CP.

Infected aortic and iliac aneurysms:Clinical manifestations in the emergency departments of two hospitals in southern Taiwan,China

BACKGROUND: Accurate diagnosis of infected aortic and iliac aneurysms is often delayed, hampering timely treatment and potentially resulting in a fatal consequence. The aim of this study was to discover useful clinical features that can help physicians to identify these patients.METHODS: We reviewed the discharge notes from two hospitals and identifi ed all patients who had a diagnosis of infected aneurysms of the thoracoabdominal aorta and iliac arteries between July 2009 and December 2013. Eighteen patients, aged from 41 to 93, were reviewed. Only 6 patients were diagnosed accurately in their fi rst visit to our ED.RESULTS: Most patients had at least one underlying illness, and it took 1 to 30(9.9±6.5) days for physicians to diagnose their infected aneurysm. Localized pain and fever were the two most commonly presented symptoms. The majority(92%) of isolated microorganisms were gram-negative bacilli, including Salmonella spp, Klebsiella pneumoniae, and Escherichia coli. Two of the 3 patients who underwent non-operative therapy died, and all of the patients who underwent a combination of medical and operative therapies survived.CONCLUSION: We suggest that physicians liberally use computed tomography scans on patients with unknown causes of pain and inflammatory processes. A combination of surgical and medical treatments is indicated for all patients with infected aortic and iliac aneurysms.

Clinical Characteristics,Diagnosis,and Therapeutics of COVID-19:A Review

The novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)that suddenly emerged at the end of December 2019 and caused coronavirus disease 2019(COVID-19)continues to afflict humanity,not only seriously affecting healthcare systems but also leading to global social and economic imbalances.As of August 2022,there were approximately 580 million confirmed cases of COVID-19 and approximately 6.4 million confirmed deaths due to this disease.The data are sufficient to highlight the seriousness of SARS-CoV-2 infection.Although most patients with COVID-19 present primarily with respiratory symptoms,an increasing number of extrapulmonary systemic symptoms and manifestations have been associated with COVID-19.Since the outbreak of COVID-19,much has been learned about the disease and its causative agent.Therefore,great effort has been aimed at developing treatments and drug interventions to treat and reduce the incidence of COVID-19.In this narrative review,we provide a brief overview of the epidemiology,mechanisms,clinical manifestations,diagnosis,and therapeutics of COVID-19.

Clinical manifestation and humoral immuno-function of myasthenia gravis patients with abnormal and normal thymus gland

BACKGROUND： Myasthenia gravis （MG） is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG with and without thymic abnormality are different. OBJECTIVE： To explore the clinical manifestation and humoral immuno-function of MG with abnormal and normal thymus gland. DESIGN ： Contrast observation SETTTNG ： Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University PARTZCZPANTS ： A total of 49 inpatients with MG were selected from the Third Affiliated Hospital of Sun Yat-sen University from March 2000 to August 2005. All the patients had typical clinical manifestation of MG and positive neostigmine test. All the patients knew and agreed the laboratory examinations. There were 22 males and 27 females of 2-69 years old. Chest MRI or CT scan were performed to reveal thymus gland abnormality. According to whether there was tumor in superior mediastinum, all patients were divided into 2 groups, abnormal and normal groups. Normal thymus gland group （n=30） contained 16 males and 14 females of 6-43 years old. Abnormal thymus gland group （n=19） contained 6 male and 13 female of 2-69 years old, METHODS： ① All patients were questioned about initial symptoms. Meanwhile, main clinical manifestations were recorded at hospital admission. ② 7180A automatic biochemical analyzer and automatic microplate reader were used in detecting seroimmunity index. The levels of C3, C4, IgG, IgA, IgM and CH50 in blood serum were analyzed by nephelometry. ③ Clinical classification is based on modified Osserman classification. The patients with MG were divided into six types： I （Ocular myasthenia）, Ⅱ a （Mild generalized myasthenia）, Ⅱb （Moderately severe generalized myasthenia）, Ⅲ （Acute fulminating myasthenia）, Ⅳ（Late se- vere myasthenia）. MAZN OUTCOME MEASURES： ① Differences of initial symptoms and clinical manifestation of two group patients. ② Differences of age of onset and modified Osserman classification of two groups. ③The humoral immuno-functions of two groups were compared. RESULTS： All the 49 patients were involved in the final analysis of results. ① Differences of initial symptoms： Ptosis was the most common initial symptoms in both groups. Patients with ptosis of normal thymus gland were 25 （83%, 25/30）. Patients with ptosis of abnormal thymus gland were 13 （68%, 13/19）. Patients with normal thymus gland： dysphagia 2 （7%, 2/30）, diplopia 4 （13%, 4/30）, fatigue 4 （13%, 4/30）, dysarthria 3, （10 %, 13/30）. Patients with abnormal thymus gland： dysphagia 3 （16%, 3/19）, diplopia 6 （32%, 6/19）, fatigue 3 （16%, 3/19）, dysarthria 2 （10%, 2/19）. ② Differences of clinical manifestation of two groups： Ptosis was the most common clinical manifestation in both groups. Patients with ptosis of normal thymus gland were 29 （97%, 29/30）. Patients with ptosis of abnormal thymus gland were 15 （79%, 15/19）. The rates of fatigue and breathing disorder in patients with abnormal thymus gland were higher than patients with normal thymus gland. Myasthenia crisis occurred in 3 patients （16 %, 3/19） in abnormal thymus gland group, with 1 （3%, 1/30） in abnormal thymus gland group. ③ Differences of age of onset and modified Osserman classification： The rate of type ｜ （63%, 19/30） in patients with normal thymus gland was higher than patients （42%, 8/19） with abnormal thymus gland. The rates of type Ⅱ a, Ⅱ b and Ⅲ （58 %） in patients with abnormal thymus gland were higher than patients （37%, 8/19） with normal thymus gland. But no differences were found between two groups （P 〉 0.05）. Patient number of onset from 20 to 29 year old in abnormal group （47%） was higher than that in normal group （20%）. Comparison of two groups was X2=4.10 and P 〈 0.05.④ Comparison of the humoral immunol indexes of two groups： The levels of IgG, IgA, C3 and CH50 in abnormal group were higher than those in normal group. But no differences were found between two groups （P 〉 0.05）. CONCLUSZON： ① Ptosis was the most common initial symptom and clinical feature in both groups. ② Clinical manifestation in abnormal group were more severe, and ages of onset in abnormal group were more young.③ The humoral immuno indexes of two groups were not significantly different.

Review of risk factors,clinical manifestations,rapid diagnosis,and emergency treatment of neonatal perioperative pneumothorax

Perioperative neonatal pneumothorax(NP)is rare but very fatal.Most of the surgeries and treatments in the neonatal period are time-limited or emergent,and there are often some risk factors for pneumothorax before surgery.Physicians,surgeons and anesthesiologists need to identify possible risk factors for pneumothorax before surgery in preterm babies,patients receiving mechanical ventilation and those with underlying lung disease.The clinical presentation of NP is nonspecific,and patients may rapidly develop life-threatening complications if not promptly diagnosed and managed.This review highlights recent progress in the identification of risk factors,clinical manifestations,diagnosis and management of NP during the perioperative period.

Clinical manifestation, imageological and pathological characteristics of Wernicke encephalopathy

BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE.

Clinical manifestations and outcomes of ocular sarcoidosis in Saudi Arabia

Dear Sir,We write to report on the clinical manifestations and outcomes of ocular sarcoidosis in patients presenting to uveitis referral centers in the Kingdom of Saudi Arabia.This is the first report focused solely on ocular sarcoidosis from this country.

Hepatic manifestations of coronavirus disease 2019 infection:Clinical and laboratory perspective

The novel coronavirus disease 2019(COVID-19)pandemic,caused by severe acute respiratory syndrome coronavirus 2,has become a global challenge of unprecedented nature since December 2019.Although most patients with COVID-19 exhibit mild clinical manifestations and upper respiratory tract involvement,in approximately 5%-10%of patients,the disease is severe and involves multiple organs,leading to multi-organ dysfunction and failure.The liver and gastrointestinal tract are also frequently involved in COVID-19.In the context of liver involvement in patients with COVID-19,many key aspects need to be addressed in both native and transplanted organs.This review focuses on the clinical presentations and laboratory abnormalities of liver function tests in patients with COVID-19 with no prior liver disease,patients with pre-existing liver diseases and liver transplant recipients.A brief overview of the history of COVID-19 and etiopathogenesis of the liver injury will also be described as a prelude to better understanding the above aspects.

Clinical features,radiological imaging,and treatment strategies of nonmetallic intraorbital foreign bodies:a retrospective analysis

AIM:To provide comprehensive data on nonmetallic intraorbital foreign bodies(IOFBs)by summarizing and analyzing material types,clinical manifestations,imaging features,and treatment strategies.METHODS:Totally 28 nonmetallic IOFB cases treated at Shengjing Hospital of China Medical University from 2012 to 2020 were retrospectively reviewed.The types of foreign bodies,clinical features,imaging manifestations,and treatment outcomes were analyzed.RESULTS:Among all cases,67.8%(19/28)of the foreign bodies were organic.The top three entrances were the upper eyelid skin(7/28),lower fornix conjunctiva(6/28),and lower eyelid skin(4/28).In most cases(11/28,39.3%),foreign bodies remained in the medial orbits.The major clinical manifestations included eyelid redness and swelling(20/28,71.4%),conjunctival congestion and edema(17/28,60.7%),and ophthalmoptosis(15/28,53.6%).Infection was the main complication,which occurred in 57.1%(16/28)of all cases.Computerized tomography(CT)values differed for different foreign bodies and varied in the different periods after injury.The plant-and grease-derived foreign bodies and the surrounding pus cysts showed different signals on magnetic resonance imaging(MRI).The prognosis varied with different foreign body types,surgery timing,and intraoperative management.CONCLUSION:The majority of nonmetallic IOFBs are organic and often remain in the superior,medial,and inferior areas of the orbit.Clinical manifestations vary owing to their different textures.CT and MRI facilitate the identification of foreign body materials.Plant-derived foreign bodies should be completely removed,and surgical treatment is a complicated process.

The value of toxicological analysis in acute poisoning patients with uncertain exposure histories:a retrospective and descriptive study from an institute of poisoning

BACKGROUND:In clinical practice,some patients might not be able or unwilling to provide a thorough history of medication and poison exposure.The aim of this study was to use toxicological analysis to examine the clinical characteristics of patients with acute poisoning whose exposure history was uncertain from a toxicological analysis perspective.METHODS:This was a retrospective and descriptive study from an institute of poisoning.Patient registration information and test reports spanning the period from April 1,2020 to March 31,2022,were obtained.Patients with uncertain exposure histories and who underwent toxicological analysis were included.Clinical manifestations and categories of toxics were analyzed.RESULTS:Among the 195 patients with positive toxicological analysis results,the main causes of uncertain exposure history was disturbance of consciousness(62.6%),unawareness(23.6%)and unwillingness or lack of cooperation(13.8%).The predominant clinical manifestations were disturbed consciousness(62.6%),followed by vomiting and nausea(14.4%)and liver function abnormalities(8.7%).A comparison of clinical manifestations between patients with positive and negative(n=99)toxicological analyses results revealed significantly different proportions of disturbances in consciousness(63%vs.21%),dizziness(1.5%vs.5.1%),multi-organ failure(1.5%vs.7.1%),and local pain(0 vs 4%).The main categories of substances involved were psychiatric medications(23.1%),sedatives(20.5%),insecticides(13.8%),and herbicides(12.8%).CONCLUSION:The clinical manifestations of acute poisoning in patients with an uncertain exposure history are diverse and nonspecific,and toxicological analysis plays a pivotal role in the diagnosis and differential diagnosis of such patients.

Guidelines for the standardized diagnosis and treatment of non-specific orbital inflammation(2024)

Non-specific orbital inflammation(NSOI)is a noninfectious orbital inflammation.Although it is often considered the most common diagnosis in orbital biopsies,it is an exclusionary diagnosis that requires ruling out systemic disease or other possible causes.Its characteristics include acute orbital signs and symptoms,including pain,proptosis,periorbital edema,chemosis,diplopia,and visual impairment.The clinical manifestations and histological findings of NSOI are heterogeneous,without specific diagnostic criteria or treatment guidelines,which poses significant challenges for diagnosis and treatment.This guideline provides a detailed description of the definition,classification,diagnosis,and treatment of NSOI.

Rectus sheath hematoma combined with COVID-19:A case report

BACKGROUND Rectus sheath hematoma(RSH)is uncommon,and because people have limited knowledge about it,it is difficult to recognize the symptoms in time,often delaying optimal treatment.CASE SUMMARY Herein,we report a case of a 77-year-old female with RSH.The patient was treated at our hospital for coronavirus disease 2019.Anticoagulant treatment was administered during this period because of thrombosis.On the 8th d of treatment,the patient complained of abdominal pain.Ultrasonography revealed a solid cystic mass in the pelvic cavity.An emergency laparotomy was performed,and a huge hematoma was found in the deep layer of the rectus abdominis muscle.We used anticoagulants with caution based on the patient’s condition.CONCLUSION Optimal management of patients with RSH s depends on timely diagnosis and when to reintroduce anticoagulants.

Spectrum of COVID-19 induced liver injury:A review report

The coronavirus disease 2019(COVID-19)pandemic has caused changes in the global health system,causing significant setbacks in healthcare systems worldwide.This pandemic has also shown resilience,flexibility,and creativity in reacting to the tragedy.The severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection targets most of the respiratory tract,resulting in a severe sickness called acute respiratory distress syndrome that may be fatal in some individuals.Although the lung is the primary organ targeted by COVID-19 viruses,the clinical aspect of the disease is varied and ranges from asymptomatic to respiratory failure.However,due to an unorganized immune response and several affected mechanisms,the liver may also experience liver cell injury,ischemic liver dysfunction,and drug-induced liver injury,which can result in respiratory failure because of the immune system’s disordered response and other compromised processes that can end in multisystem organ failure.Patients with liver cirrhosis or those who have impaired immune systems may be more likely than other groups to experience worse results from the SARS-CoV-2 infection.We thus intend to examine the pathogenesis,current therapy,and consequences of liver damage concerning COVID-19.

Ambispective epidemiological observational study of varicella-zoster virus infection: An 18 year-single-center Bulgarian experience

BACKGROUND Varicella(chickenpox)and herpes zoster(shingles)are outcomes of varicella-zoster virus(VZV)infection,and understanding their incidence trends is vital for public health planning.AIM To conduct an ambispective epidemiological study by analyzing the main epidemiological characteristics of VZV infection during an 18 year-period(2000-2018).METHODS We used descriptive and epidemiological methods to characterize chickenpox in Bulgaria,the city of Plovdiv and the region for a period of 18 years(2000-2018).RESULTS The average incidence of varicella-zoster infection for the period 2000–2018 in the Plovdiv region was estimated at 449.58‰.The highest relative share of the infection was assessed in the month of January at 13.6%,and the lowest in the months of August and September at 2.9%(both months).The age group most affected by the infection was 1-4 years,followed by 5-9 years.This corresponds to the so-called"pro-epidemic population"-a phenomenon typical for airborne infections,confirming their mass impact on the perpetuation of VZV infection.CONCLUSION Our findings reveal significant insights into VZV epidemiology,including age-specific incidence rates,clinical manifestations,and vaccination impact.This comprehensive analysis contributes to the broader understanding of VZV infec-tion dynamics and may inform evidence-based preventive measures.

Clinical and molecular research of neuroacanthocytosis

Neuroacanthocytosis is an autosomal recessive or dominant inherited disease characterized by widespread, non-specific nervous system symptoms, or spiculated ＂acanthocytic＂ red blood cells. The clinical manifestations typically involve chorea and dystonia, or a range of other movement disorders. Psychiatric and cognitive symptoms may also be present. The two core neuroacanthocytosis syndromes, in which acanthocytosis is atypical, are autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome. Acanthocytes are found in a smaller proportion of patients with Huntington＇s disease-like 2 and pantothenate kinase-associated neurodegeneration. Because the clinical manifestations are diverse and complicated, in this review we present features of inheritance, age of onset, neuroimaging and laboratory findings, as well as the spectrum of central and peripheral neurological abnormalities and extraneuronal involvement to help distinguish the four specific syndromes.

Clinical Characteristics, Imaging Findings and Surgical Outcomes of Chiari Malformation Type I in Pediatric and Adult Patients

A growing number of children and adolescents are being diagnosed as Chiari malformation type I （CM- I ） for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM- I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM- I underwent surgical treatment in our department. We divided the patients into two groups： pediatric group （n=l 1, age 〈18 years） and adult group （n=73, age 〉18 years）. Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis （36.4%） and developmental delay （36.4%） were more common in pediatric patients, whereas, sensory disturbance （58.9%） and motor weakness （41. 1%） were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group （P〈0.05）. Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments （P〈0.05）. The total Chicago Chiari Outcome Scale （CCOS） score in pediatric patients at the last follow- up was significantly higher than that in adult patients （P=0.002）. In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM- I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.