Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjogren's syndrome

Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.

Autoimmune hepatitis-primary biliary cholangitis overlap syndrome complicated by various autoimmune diseases:A case report

BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.

Ziwan-Taoren herb pair can exert an therapeutical effect in primary Sjogren’s syndrome through inhibiting the TLR/NF-κB pathway

Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.

Study of the Effects of Glucocorticoid on Growth and Adult Final Height in Children with Primary Nephrotic Syndrome

Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.

Menstrual and Reproductive Characteristics of Patients with Primary Sjogren’s Syndrome:A 7-year Single-center Retrospective Study

Objective Primary Sjogren’s syndrome(pSS)is a systemic autoimmune disease that mainly affects the exocrine gland,especially in women.Currently,the results of studies on the menstruation or fertility of pSS patients remain controversial.This study aimed to examine the menstrual and reproductive characteristics of pSS patients.Methods Clinical data of 449 pSS patients who were admitted to Tongji Hospital in Hubei,China,from January 2015 to November 2021 were obtained and their menstrual and reproductive information analyzed.In addition,the clinical features of pSS patients with premenopausal or postmenopausal onset were compared.Results The spontaneous abortion rate of pSS patients was not higher than the reported rate of the general population and that the age of menarche,menstrual cycle,and menstrual period of pSS patients did not significantly differ from those reported in the general population;however,early menopause seemed to be more common in pSS patients.Skin involvement(27.96%vs.15.00%,P=0.005)and hyperglobulinemia(10.64%vs.4.16%,P=0.033)were more common in patients with premenopausal pSS onset,but patients with postmenopausal onset had a significantly greater incidence of interstitial lung disease(32.50%vs.17.02%,P=0.0004).Also,erythropenia(47.00%vs.31.25%,P=0.002),hypoalbuminemia(19.49%vs.8.22%,P=0.0009),and prevalence of high hypersensitive C-reactive protein levels(21.67%vs.10.94%,P=0.005)were more common in pSS patients with postmenopausal onset.Notably,the rate of abnormal pregnancy was significantly greater in patients with premenopausal onset(9.72%vs.2.50%,P=0.011).Conclusion Patients with pSS onset before or after menopause may have different risks in pulmonary involvement and laboratory manifestations.

Meta-analysis of the efficacy in treatment of primary sjogren's syndrome: Traditional Chinese Medicine vs Western Medicine

OBJCTIVE: To compare the clinical efficacy of Traditional Chinese Medicine(TCM) to Western Medicine in the treatment of primary Sj?gren's syndrome(p SS).METHODS: We collected randomized controlled trials of TCM vs Western Medicine for the treatment of p SS in Chinese and foreign databases. The study quality was evaluated as suggested in the Cochrane Handbook. The Meta-analysis was performed using Review Manager 5.0 statistical software.RESULTS: A total of 31 randomized controlled trials with 2137 cases were retrieved. The efficiencies of TCM and control treatments were 87.18% and65.63%, respectively. The results of heterogeneity tests showed that the data were homogeneous(P = 0.83), thus a fixed effects model was used for analysis. The results revealed an odds ratio of 3.74 with a 95% confidence interval of 2.99-4.69. The overall effectiveness value was 11.48(P < 0.000 01).These results suggest the efficacy of TCM therapy for p SS better than Western Medicine.CONCLUSION: Although our findings reveal that the TCM treatment of p SS had significant advantages over its counterpart, there were some flaws in the studies included. The findings warrant further investigation.

Effect of Chinese Herbal Medicine for Nourishing Yin,Supplementing Qi,and Activating Blood on the Th1/Th2 Immune Balance in Peripheral Blood in Patients with Primary Sjogren's Syndrome

Objective： To observe the effect of Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood on the expression of interferen- γ （IFN-γ,）/interleukin-4 （IL-4） in peripheral blood and disease activity in primary Sjogren＇s syndrome （pSS） patients, and to study the relationship between the immune balance of Th1/Th2 and the disease activity. Methods： A total of 66 pSS patients were randomized with tossing coins method into two groups： the integrative therapy group （34 cases） and the control group （32 cases）; and 28 healthy subjects were taken as the normal group. The integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets and the control group was treated with hydroxychloroquine sulfate tablets. The treatment course was 3 months for both groups. The levels of serum immunoglobulin G （IgG）, erythrocyte sedimentation rate （ESR）, IFN-γand IL-4 in peripheral blood were measured before and after treatment. Results： Compared with the normal group, the levels of IgG, ESR, IFN- γ and IL-4 were significantly increased in pSS patients （P〈0.05）. Remarkably, after 3 months of treatment, these levels were dramatically decreased in both the integrative therapy group and the control group, although still higher than the normal group. The levels of IgG, ESR, IFN- and IL-4 in the integrative therapy group were lower than the control group and the same group before treatment （P〈0.05）. The ratio of IFN-γ/IL-4 also significantly decreased after treatment. Moreover, the level of IFN- γ, and the ratio of IFN- γ/IL-4 in the integrative theraphy group were significantly lower than the control group （P〈0.05）. For all patients the ratio of IFN-γ/IL-4 before and after treatment was positive correlated with the levels of IgG and ESR. Conclusion： Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood can alleviate the disease activity of pSS by regulating the immune balance of Th1/Th2.

Therapeutic Effect of Chinese Herbal Medicine for Strengthening Qi,Nourishing Yin,and Removing Stasis on Serum Osteopontin and Quality of Life of Patients with Primary Sjogren's Syndrome

Objective：To observe the therapeutic effect of Chinese herbal medicine for strengthening qi, nourishing yin,and removing stasis on serum osteopontin（OPN） and quality of life（QOL） in patients with primary Sjogren＇s syndrome（pSS） and to study the correlation between OPN level and the disease.Methods：Sixtyeight pSS patients were randomly assigned to two groups：the treatment group（35 cases） treated by Chinese herbal medicine for strengthening qi,nourishing yin,and removing stasis combined with hydroxychloroquine sulfate tablet（HCQ） and the control group（33 cases） treated by HCQ only.Both were treated for 3 months. Before and after treatment,immunoglobulin G（IgG） level,erythrocyte sedimentation rate（ESR）,and OPN level were measured.The QOL in patients was evaluated using the Short Form-36 Health Survey（SF-36） before and after treatment.Thirty healthy females were taken as the normal control.Results：Before treatment,levels of IgG,ESR,and OPN in patients were higher than those in the normal control.After 3 months of treatment, those in both treatment groups decreased but were lower in the treatment group than those in the control group （P0.05）.The scores of 8 dimensions of SF-36 were lower in the pSS patients than those in the normal control （P0.05） and higher in the treatment group than in the control group after treatment（P0.05）.Furthermore, there was a positive correlation between the levels of OPN and the levels of IgG and ESR,and a negative correlation between OPN levels and the overall score of SF-36（P0.05）.Conclusions：Chinese herbal medicine for strengthening qi,nourishing yin,and removing stasis could alleviate pSS disease and improve the QOL.In addition,the OPN level might be used as an evaluating index for pSS disease.

A Case of Podocytic Infolding Glomerulopathy with Primary Sjogren's Syndrome and Hashimoto's Thyroiditis

To the Editor:Podocytic infolding glomerulopathy (PIG)was proposed as a new disease entity in 2008,[1] and great attention has been paid to PIG because of the rare pathological changes to glomeruli associated with it.PIG is characterized by specific changes to the thickened glomerular basement membrane (GBM), including microspheres,microtubular structures,and podocytic infolding.However,the clinical features and pathogenesis of PIG still remain unclear.To elucidate the characteristics of this glomerulopathy,accumulating more information from reported cases is necessary.Herein,we present a case of glomerulopathy showing podocytic infolding in association with primary Sjogren's syndrome (pSS)and Hashimoto's thyroiditis.

Effect of Chinese Herbal Medicines for Nourishing Yin,Supplementing Qi,and Activating Blood on Reproductive Endocrine Activity and Immune Functions in Patients with Primary Sjogren's Syndrome

ABSTRACT Objective： To investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with primary Sjogren＇s syndrome （pSS）. Methods： Seventy pSS patients were randomly assigned to two groups using a randomized digital table： the integrative therapy group （36 cases） and the control group （34 cases）. Thirty healthy subjects were taken as a normal group. The control group was treated with hydroxychloroquine sulfate tablets alone, and the integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets. The treatment course was 6 months for both groups. Before and after treatment, serum estradiol （E2）, testosterone （T）, luteinizing hormone （LH）, prolactin （PRL） by radioimmunoassay and immunoglobulin （IgG） by immunodiffusion, erythrocyte sedimentation rate （ESR） by Westergren, interferon-γ （IFN-γ） and interleukin-4 （IL-4） by enzyme linked immunosorbent assay were determined. Results： E2 and T levels in all patients were lower than those of normal subjects before treatment （P〈0.05） and were increased significantly after 6-month treatment （P〈0.05）. ESR, FSH, LH, IgG, IFN - γ, IL - 4 and ratios of E2/T, and IFN --γ/IL in the patients were higher than those of normal subjects before the treatments （P〈0.05）, and were reduced significantly after the treatments （P〈0.05）. The T and IFN -γ levels and E2/T ratio in the patients treated with integrative therapy were reduced significantly compared with the control group （P〈0.05）. However, the PRL levels before and after treatment were not significantly changed in the two groups （P〉0.05）. The ratios of E2/T and IFN -γ/IL-4, and levels of IgG and ESR were positively correlated before and after treatment （P〈0.05）. Conclusions： The ratios of E2/T and IFN - γ/ IL-4 might be used as indicators of pSS activity. Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with Western medicine could improve the therapeutic effect by regulating the reproductive endocrine-immune network in pSS patients.

Primary membranous nephrotic syndrome with chylothorax as first presentation:A case report and literature review

BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.

The Relationship between Golgi Protein 73, Alpha-Fetoprotein, Liver Function Indicators, and Traditional Chinese Medicine Syndrome Types of Primary Liver Cancer

Objective Our objective was to analyze the correlation between Golgi protein 73(GP73),alpha-fetoprotein(AFP),liver function indicators,and traditional Chinese medicine(TCM)syndrome types of primary liver cancer(hereinafter referred to as"liver cancer").Methods In total,156 liver cancer patients(liver cancer group)and 52 healthy individuals(health group)were selected as the research subjects to detect their GP73,AFP expression,and liver function-related indicators.The obtained data were statistically analyzed using SPSS 21.0 software.Results(1)The positive expression rate of GP73 in the liver cancer group was 50%;the positive expression rates of qi stagnation and blood stasis syndrome,heat tgxin in liver and galader yndroeand yndefceny flieran kidney yndg1i,%54.3,and 59.6%,respectively.There was no statistically significant difference betwgep the groups(p>0.05),The positive expreion rate of AFP was 50%.he positiy 32.8 expression rates of qi stagnation and blood stasis syndrome,heat-toxin in liyer ane gallbladder syndrome,and yin deficiency of liver and kidney syndrome were 41.7,54.3 an59.6%,respectively.There was a statistically significant difference between the groups(p<0.05).(2)The GP73 levels of patients with different syndrome types in the liver cancer group were ranked from high to low as yin deficiency of liver and ki41.3 syndrome,heat toxin in liver and gallbladder syndrome,and qi stagnation and brooa stasis syndrome.The differences between the groups were statistically significant(p<0.05).The AFP levels of patients with different syndrome types in the liver cancer group were ranked from high to low as heat toxin in liver and gallbladder syndrome,yin deficiency of liver and kidney syndrome,and qi stagnation and blood stasis syndrome.There was no statistically significant difference between the groups(p>0.05).(3)Analysis of liver function indicators in the liver cancer group:the alanine transaminase s(ALT)levels of patients with different syndrome types were in descending order from high to low,including yin deficiency of liver and kidney syndrome,heat toxin in liver and gallbladder syndrome,and qi stagnation and blood stasis syndrome.The differences between groups were statistically significant(p<0.05).The aspartate aminotransferase(AST)levels of patients with different syndrome types were ranked from high to low as follows:heat toxin in liver and gallbladder syndrome,yin deficiency of liver and kidney syndrome,and qi stagnation and blood stasis syndrome.The difference between groups was statistically significant(p<0.05).The levels of albumin(ALB)in patients with different syndrome types were ranked from high to low,including heat toxin in liver and gallbladder syndrome,qi stagnation and blood stasis syndrome,and yin deficiency of liver and kidney syndrome.The differences between groups were statistically significant(p<0.05).Conclusion GP73 and indicators such as ALT,AST,and ALB are of great significance in the diagnosis of TCM syndrome differentiation and classification of liver cancer patients.

Late Diagnosis of Turner Syndrome in Adulthood;a Case Study from the Endocrinology-Diabetology Nutrition Department of the National Hospital of Pikine Senegal

Introduction: Turner syndrome is a rare genetic disorder characterised by the presence of one X chromosome and the absence of part or all of an X or Y chromosome and patients may experience delayed puberty and infertility. Our study aimed to evaluate the diagnostic delay in our practice and analyze the impact of this diagnostic delay on the effectiveness of patient management. Patients and Methods: Turner syndrome patients were identified from the endocrinology-diabetology nutrition department Database We examined the records of patients in whom the karyotype analysis favoured Turner syndrome. Results: We have selected 5 patients’ records of female patients with Turner syndrome. The mean age was 25, ranging from 19 to 29 years. Primary amenorrhea and characteristic dysmorphic features were observed in all patients. One married patient, who sought consultation for infertility, expressed a desire for pregnancy. Short stature was identified in 3 patients. Primary hypothyroidism and hypertension were respectively found in 1 and 2 patients. Gonadal dysgenesis was noted in 100% of cases. Karyotype analysis revealed monosomy X in 2 patients and mosaic patterns in others. All patients received estrogen-progestin treatment. Antihypertensive therapy was initiated for 2 patients. One patient is on L-thyroxine. In the short term, treatment led to the onset of menstruation after the initial months. Evaluation of treatment efficacy on internal genital organs is yet to be performed. Due to uncertain benefits at this age, growth hormone therapy was not considered for our patients. We provided counseling on assisted reproductive options for couples desiring to conceive. In our study, all patients were placed on estrogen-progestin therapy, and the response appeared favorable. Conclusion: In our practice, the diagnosis of Turner syndrome occurs very late in adulthood, at an age when growth hormone treatment is nearly ineffective. Treatment typically revolves around estrogen-progestin therapy, along with managing other comorbidities such as hypertension and primary hypothyroidism.

Performance of transient elastography in assessing liver fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome

AIM To investigate the performance of transient elastography(TE) for diagnosis of fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis(AIHPBC) overlap syndrome.METHODS A total of 70 patients with biopsy-proven AIH-PBC overlap syndrome were included. Spearman correlation test was used to analyze the correlation of liver stiffness measurement(LSM) and fibrosis stage. Independent samples Student's t-test or one-way analysis of variance was used to compare quantitative variables. Receiver operating characteristics(ROC) curve was used to calculate the optimal cut-off values of LSM for predicting individual fibrosis stages. A comparison on the diagnostic accuracy for severe fibrosis was made between LSM and other serological scores.RESULTS Patients with AIH-PBC overlap syndrome had higher median LSM than healthy controls(11.3 ± 6.4 k Pa vs 4.3 ± 1.4 k Pa, P < 0.01). LSM was significantly correlated with fibrosis stage(r = 0.756, P < 0.01). LSM values increased gradually with an increased fibrosis stage. The areas under the ROC curves of LSM for stages F ≥ 2, F ≥ 3, and F4 were 0.837(95%CI: 0.729-0.914), 0.910(0.817-0.965), and 0.966(0.893-0.995), respectively. The optimal cut-off values of LSM for fibrosis stages F ≥ 2, F ≥ 3, and F4 were 6.55, 10.50, and 14.45 k Pa, respectively. LSM was significantly superior to fibrosis-4, glutaglumyl-transferase/platelet ratio, and aspartate aminotransferase-to-platelet ratio index scores in detecting severe fibrosis(F ≥ 3)(0.910 vs 0.715, P < 0.01; 0.910 vs 0.649, P < 0.01; 0.910 vs 0.616, P < 0.01, respectively).CONCLUSION TE can accurately detect hepatic fibrosis as a noninvasive method in patients with AIH-PBC overlap syndrome.

Management of primary ciliary dyskinesia/Kartagener＇s syndrome in infertile male patients and current progress in defining the underlying genetic mechanism

Kartagener＇s syndrome （KS） is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia （PCD）. As it is accompanied by many complications, PCD/KS severely affects the patient＇s quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility.

Comparison of total/active ghrelin levels in primary open angle glaucoma,pseudoexfoliation glaucoma and pseudoexfoliation syndrome

AIM：To investigate the levels of ghrelin（Gh）,acylated ghrelin（AGh） and AGh/Gh ratio in the humor aqueous（HA） of cases with pseudoexfoliation syndrome（PXS）,pseudoexfoliation glaucoma（PXG）,primary open angle glaucoma（POAG） and to compare these with control subjects.METHODS：A prospective examination was made of the total Gh,and AGh levels in HA of 67 patients undergoing cataract surgery.Patients were divided into 4 groups.HA samples were aspirated at the beginning of the surgery,stored at -70℃.Gh and AGh quantification was performed with ELISA kits and the AGh/total-Gh ratios were calculated.ANOVA,Kruskal-Wallis,Chi-square and post-hoc tests were used for statistical analysis.RESULTS：Total Gh levels in HA were 189.2±45.6 pg/mL in the control group,199.2±32.9 pg/mL in PXS,180.6±20.9 pg/mL in PXG and 176.8±21.4 pg/mL in POAG groups（P〉0.05）.AGh levels in HA were 23.09±5.01 pg/mL in the control group,24.13±5.22 pg/mL in PXS,22.29±1.55 pg/m L in PXG and 19.69±2.93 pg/mL in POAG groups（P〉0.05）.The ratio of AGh/Gh was 10.3%±2.34% in the control group,13.03%±2.58% in PXS,12.3%±1.54% in PXG and 11.79%±1.41% in POAG groups（P=0.044）.The difference between the PXS and control groups was significant（P=0.03）.CONCLUSION：In spite of statistically insignificant results,the HA total Gh levels were lower than those of the control subjects but not parallel with the AGh levels in glaucoma patients.The relative increase in the AGh/Gh ratio in glaucoma cases supports the view that proportional increases of AGh might play a role in the pathogenesis of glaucoma.

Primary Sjgren's syndrome related optic neuritis

AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were analyzed retrospectively.RESULTS:Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS.They presented initially with the signs and symptoms of non-specific optic neuritis,and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland,and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients.There were serum positive titers for anti-Sjogren syndrome A(SSA)in 7 patients and anti-Sjogren syndrome B(SSB)in 8 patients.Anti-aquaporin-4(AQP4)antibody was negative in all the 8 patients.Both glucocorticoids and immunosuppressive agent were administered,and visual acuity elevated in 8 eyes(66.7%),3 patients(37.5%)recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed.The conventional therapies for optic neuritis could not control the recurrence.

Comparison of the meibomian gland dysfunction in patients with chronic ocular graft-versus-host disease and Sjogren's syndrome

AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a major form of aqueous deficient DED and meibomian gland dysfunction(MGD), a common cause of evaporative DED.METHODS: A total 135 eyes of 135 subjects included in this study: patients with DED associated with coGVHD(n=30), patients with SS(n=35), patients with MGD(n=35), and normal controls(n=35). All participants completed the Ocular Surface Disease Index(OSDI) questionnaire, ocular surface examination [Schirmer test, tear film breakup time(TFBUT), and ocular surface staining], and meibomian gland assessment [meiboscore(gland dropout detected on meibography using infrared camera of the Keratograph 5 M), meibum expressibility score(MES), meibum quality score(MQS), lid margin abnormality]. In addition, correlations of meibomian gland characteristics with ocular surface parameters as well as disease severity score were investigated in coGVHD group.RESULTS: The coGVHD group showed significantly higher meiboscore, MES, and MQS than the other 3 groups(all P<0.05). In the coGVHD group, parameters of meibomian gland showed a significant correlation each other and those of ocular surface. The correlation between meibomian gland parameters and severity score of co GVHD was also established(meiboscore, r=0.62; MES, r=0.47; MQS, r=0.47; lid margin abnormality score, r=0.55; all P<0.05).CONCLUSION: Patients with DED associated with co GVHD show poorer gland morphology and worse glandfunction than other types of DED. In addition, meibomian gland damage is not only associated with ocular surface damage but also disease severity of coGVHD.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome:A case report

BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.

Acute pancreatitis with hypercalcemia caused by primary hyperparathyroidism associated with paraneoplastic syndrome:A case report and review of literature

BACKGROUND Although acute pancreatitis associated with hyperparathyroidism has occasionally been reported,acute pancreatitis with metabolic encephalopathy caused by hyperparathyroidism combined with paraneoplastic syndrome is an extremely rare entity and poorly described in the literature.CASE SUMMARY We present a case of a 56-year-old female with upper abdominal discomfort and intermittent nausea and vomiting for 1 wk,without apparent abdominal pain or bloating,no jaundice and decreased blood pressure at the outset.The patient was ultimately diagnosed with moderately severe acute pancreatitis(according to the revised Atlanta classification of acute pancreatitis)combined with metabolic encephalopathy secondary to hypercalcemia caused by primary hyperparathyroidism associated with paraneoplastic syndrome.After active treatment of acute pancreatitis,massive fluid resuscitation,resection of parathyroid and uterine malignant tumors,neoadjuvant chemotherapy and other treatments,her serum calcium eventually returned to the normal level.The patient was successfully discharged from hospital.CONCLUSION This is the first case of acute pancreatitis caused by primary hyperparathyroidism associated with paraneoplastic syndrome.