The efficacy of anti–TNF-? agents in the treatment of juvenile idiopathic arthritis-associated uveitis in a pediatric cohort

Purpose: Biologic therapy has shown promising control in children with often intractable juvenile idiopathic arthritis (JIA)?associated uveitis (JIA?U). Methods: This is a retrospective cohort study of 35 eyes of 35 children who received biologics for JIA?U. Pretreatment and posttreatment data (at 3, 6, 9, 12, 18, 24, and >24 months) were analyzed to determine functional success (stable/improved visual acuity), quiescence success (?0.5 cells in the anterior chamber), complete steroid success (termination of systemic, periocular therapy and decreased topical drops to ?2/day) or systemic steroid success (termination of systemic steroids only), and complete success (all of the above). Results: This study included 35 eyes up to 12 months and 21 eyes beyond 24 months. Steroid?sparing, functional, and quiescence success showed a rate of success of 52.43%, 77%, and 91%, respectively, at 12 months and 66.67%, 85.7%, and 76.2%, respectively, beyond 24 months. Complete success was 34.29% at 12 months, peaking at 18 months (65.62%) and reached 57.14% beyond 24 months. In their final follow?up, the best corrected visual acuity (BCVA) remained the same in 45.71%, improved in 37.14%, and worsened in 17.14% children. Conclusion: Biologic therapy is effective in JIA?U, especially in termination of systemic steroids, stabilization of vision, and maintaining quiescence

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40?mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1?g weekly was started for 4 weeks followed by 500?mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

Efficacy and safety of eltrombopag combined with immunosuppressive therapy in the treatment of severe aplastic anemia：a meta-analysis / 中国药房

OBJECTIVE To systematically evaluate the efficacy and safety of eltrombopag combined with immunosuppressive therapy （IST） for severe aplastic anemia （SAA）， and to provide evidence-based basis for clinical treatment of SAA. METHODS Retrieved from PubMed， Embase， Cochrane Library， ClinicalTrials.gov， VIP， CNKI and Wanfang data， randomized controlled trials （RCTs） and cohort studies about eltrombopag combined with IST （trial group） versus IST alone （control group） were collected from the inception to May 2023. After data extraction and quality evaluation （Cochrane manual 5.1.0） of included studies， meta-analysis， subgroup analysis， sensitivity analysis and publication bias analysis were performed by using RevMan 5.4 software. RESULTS A total of 12 studies were screened， including 1 344 patients. Compared with control group， objective remission rate （ORR） （RR＝1.34， 95%CI was 1.06-1.69， P＝0.01） and complete response rate （CRR） （RR＝1.88， 95%CI was 1.31-2.71， P＝ 0.000 6） at 3 months， ORR （RR＝1.33，95%CI was 1.23-1.43， P＜0.000 01） and CRR （RR＝1.88，95%CI was 1.57-2.25，P＜0.000 01） at 6 months were significantly increased in trial group. There was no statistically significant difference between the two groups in ORR （RR＝0.99， 95%CI was 0.82-1.18， P＝0.88） and CRR （RR＝1.02， 95%CI was 0.78-1.34， P＝0.87） at 12 months， two-year overall survival （OS） rate （HR＝0.61， 95%CI was 0.31-1.22， P＝0.17）， two-year event-free survival （EFS） rate （HR＝0.81， 95%CI was 0.61-1.07， P＝0.14）， clone evolution rate（RR＝1.01， 95%CI was 0.51-2.00， P＝ 0.98） or the incidence of adverse drug reactions such as liver/renal insufficiency， rash （P＞0.05）. Results of subgroup analysis showed that ORR and CRR of trial group at 6 months were higher than those of the control group in RCT and the cohort study subgroups （P＜0.05）. There was no statistically significant difference in the two-year OS rate， two-year EFS rate or clone evolution rate between trial group and control group in the two subgroups （P＞0.05）. The results of sensitivity analysis and publication bias analysis showed that the results of this study were robust and the possibility of publication bias was small. CONCLUSIONS The addition of eltrombopag in the IST regimen of SAA can improve the early hematological remission rate of patients， has no significant impact on short-term survival， and will not increase the occurrence of adverse drug reactions and clonal evolution.

Treatment of severe aplastic anemia and hematopoietic stem cell transplantation / 器官移植

Severe aplastic anemia (SAA) is a severe bone marrow failure syndrome caused by multiple causes, which is clinically manifested with severe anemia, infection and bleeding. The complex pathogenesis of SAA has not been fully understood. SAA is characterized with acute onset, severe disease condition and rapid progression. At present, with the in-depth study of SAA and the improvement of diagnosis and treatment, the therapeutic strategy for SAA has been evolved from classical immunosuppressive therapy based on antithymocyte globulin and cyclosporine to the application of thrombopoietin receptor agonist and combined treatment based on allogeneic hematopoietic stem cell transplantation, which may promote the reconstruction of hematopoietic function of SAA patients to varying degree and significantly improve survival and clinical prognosis, becoming the research hotspot of SAA treatment. In this article, new advances in the treatment of SAA at home and abroad were reviewed.

The latest progress on novel coronavirus vaccination in kidney transplant recipients / 器官移植

Due to long-term use of immunosuppressant, poor immune function and a higher risk of critical diseases after novel coronavirus pneumonia in kidney transplant recipients, it is of significance to deliver prophylactic vaccination for this high-risk population. Studies have shown that the immune reaction of kidney transplant recipients to novel coronavirus vaccine is significantly lower than that of healthy counterparts. Standard vaccination program in the United States, such as 2 doses of messenger RNA (mRNA) vaccine, fails to provide sufficient protection for kidney transplant recipients. Many studies have proven that increasing the frequency of vaccination for kidney transplant recipients may enhance the vaccine efficacy. Nevertheless, the role of adjusting immunosuppressive therapy in increasing vaccine efficacy remains to be elucidated. In this article, the importance, effectiveness and particularity of novel coronavirus vaccine for kidney transplant recipients and the effect of immunosuppressive therapy on the efficacy of novel coronavirus vaccine were reviewed, aiming to provide reference on the vaccination for kidney transplant recipients.

Highlights of human uterus transplantation / 器官移植

As a novel solid organ transplantation, uterus transplantation has become an important approach for women with uterine infertility to obtain biological offspring. Multiple technological challenges exist in uterus transplantation, such as acquisition of vascular pedicle, organ perfusion and vascular suture, etc. However, with the development and application of uterus transplantation in animal model and transplantation in human cadavers, a lot of problems have become new hot topics of discussion, such as the selection of uterus transplantation donors and recipients, selection of uterine vessels, prevention and treatment of complications after uterus transplantation, evaluation of graft vitality, timing of pregnancy and delivery, timing of hysterectomy, mental health of donors and recipients and offspring health, etc. According to current data and outcomes of human uterus transplantation worldwide, these hot topics were reviewed in this article, aiming to provide reference for promoting the development and progress of human uterus transplantation research in China.

Diagnosis and Management of Acquired Aplastic Anemia: Consensus Statement of Indian Academy of Pediatrics

Justification: In India, there is a lack of uniformity of treatment strategies for aplastic anemia (AA), and many children are managed only with supportive care due to non-availability of hematopoietic stem cell transplantation (HSCT). Process: Eminent national faculty members were invited to participate in the process of forming a consensus statement in Hyderabad in July, 2016. Draft guidelines were circulated to all members, and comments received in a online meeting in October, 2020 were incorporated into the final draft. These were approved by all experts. Objective: To facilitate appropriate management of children with acquired aplastic anemia. Recommendations: Key recommendations are: i) A bone marrow biopsy is must to make a diagnosis of AA; ii) Rule out inherited bone marrow failure syndromes (IBMFS), connective tissue disorders, viral infections, paroxysmal nocturnal hemoglobinuria (PNH), drug or heavy metal induced marrow suppression in all cases of AA; iii) Conservative approach to transfusions should be followed, with a target to keep hemoglobin >6 g/dL in children with no co-morbidities; iv) HLA-matched sibling donor HSCT is the preferred choice of treatment for newly diagnosed very severe/ severe AA; v) In absence of HLA-matched family donor, a matched unrelated donor (MUD) transplant or immunosuppressive therapy (IST) should be considered as alternate choice based on physician expertise; vi) Fludarabine, cyclophosphamide and anti-thymocyte globulin (ATG) based conditioning with cyclosporine and methotrexate as graft versus host disease (GvHD) prophylaxis is the preferred regimen; vii) Horse ATG and cyclosporine are the recommended drugs for IST. One should wait for 3-6 months for the response assessment and consideration of next line therapy.

The efficacy of immunosuppression combined with Eltrombopag in the treatment of severe aplastic anemia in children / 中华实用儿科临床杂志

Objective:To explore the efficacy and clinical significance of Eltrombopag combined with immunosuppression therapy(IST) in the treatment of severe aplastic anemia (SAA) in children.Methods:Clinical data of 63 children with initially diagnosed SAA in the Department of Hematology of Xinxiang Central Hospital from May 2017 to June 2019 were retrospectively analyzed.All of them were all donors without siblings and they were classified into observation group (31 cases) and control group (32 cases). Patients in the observation group received IST combined with Eltrombopag treatment, and those in the control group received IST treatment.The Chi- square test was used to compare the overall remission (OR) rate and complete remission (CR) rate at 3 months, 6 months and 12 months, and incidence of infection and significant bleeding between groups.The t-test was used to compare the application of gra-nulocyte colony stimulating factor, the mean red blood cell transfusion, and the mean platelet infusion volume between groups.Kaplan-Meier method was adopted to analyze the 2-year overall survival (OS) rate and failure-free survival (FFS) rate, followed by the Log- rank test. Results:The 3-month and 6-month OR rate of the observation group were 61.29%(19/31 cases) and 80.64% (25/31 cases), respectively, which was significantly higher than that of the control group [37.50%(12/32 cases) and 59.38%(19/32 cases), χ2=45.27, 43.81, respectively, all P<0.05]. The 3-month and 6-month CR rate of the observation group were 32.26%(10/31 cases) and 45.16%(14 /31 cases), respectively, which was significantly higher than that of the control group [15.62%(5/32 cases) and 28.13%(9/32 cases), χ2=47.02, 48.35, respectively, all P<0.05]. The 12-month OR rate and CR rate in the observation group were 83.87%(26/31 cases), and 64.52%(20/31 cases), respectively, which were 81.25%(26/32 cases), and 59.38%(19/32 cases), respectively in the control group, and no significant differences in them were detected between the two groups (all P>0.05). The total amount of granulocyte colony stimulating factors [(13.58±4.28) doses vs.(23.24±6.68) doses, t=2.591], and the mean infusion volume of red blood cells [(5.48±1.67) U vs.(10.58±3.67) U, t=2.040] and platelets (4.15±2.47) bags vs.(9.15±3.87) bags, t=2.744) used in observation group within 6 months of treatment were significantly lower than those of the control group (all P<0.05). The rate of infection (16.13% vs.43.75%, χ2=47.12) and significant bleeding (16.13% vs.37.50%, χ2=44.52) in the observation group were significantly lower than those of the control group (all P<0.05). The 2-year OS rate of the observation group and control group were 93.55% (29/31 cases), and 87.50% (28/32 cases), respectively.No significant difference in the OS rate was found between groups ( P=0.407 3), nor as the 2-year FFS rate(87.10% vs.78.13%, P=0.326 6). Conclusions:IST combined with Eltrombopag can significantly improve the early treatment response rate of SAA children without a sibling identical donor, which can reduce red blood cell and platelet transfusion, and the incidence of infection and bleeding.

Reassessing the six months prognosis of patients with severe or very severe aplastic anemia without hematological responses at three months after immunosuppressive therapy / 中华血液学杂志

Objective: To reassess the predictors for response at 6 months in patients with severe or very severe aplastic anemia (SAA/VSAA) who failed to respond to immunosuppressive therapy (IST) at 3 months. Methods: We retrospectively analyzed the clinical data of 173 patients with SAA/VSAA from 2017 to 2018 who received IST and were classified as nonresponders at 3 months. Univariate and multivariate logistic regression analysis were used to evaluate factors that could predict the response at 6 months. Results: Univariate analysis showed that the 3-month hemoglobin (HGB) level (P=0.017) , platelet (PLT) level (P=0.005) , absolute reticulocyte count (ARC) (P<0.001) , trough cyclosporine concentration (CsA-C0) (P=0.042) , soluble transferrin receptor (sTfR) level (P=0.003) , improved value of reticulocyte count (ARC(△)) (P<0.001) , and improved value of soluble transferrin receptor (sTfR(△)) level (P<0.001) were related to the 6-month response. The results of the multivariate analysis showed that the PLT level (P=0.020) and ARC(△) (P<0.001) were independent prognostic factors for response at 6 months. If the ARC(△) was less than 6.9×10(9)/L, the 6-month hematological response rate was low, regardless of the patient's PLT count. Survival analysis showed that both the 3-year overall survival (OS) [ (80.1±3.9) % vs (97.6±2.6) %, P=0.002] and 3-year event-free survival (EFS) [ (31.4±4.5) % vs (86.5±5.3) %, P<0.001] of the nonresponders at 6 months were significantly lower than those of the response group. Conclusion: Residual hematopoietic indicators at 3 months after IST are prognostic parameters. The improved value of the reticulocyte count could reflect whether the bone marrow hematopoiesis is recovering and the degree of recovery. A second treatment could be performed sooner for patients with a very low ARC(△).

Significance of paroxysmal nocturnal hemoglobinuria clone in immunosuppressive therapy for children with severe aplastic anemia / 中国当代儿科杂志

OBJECTIVES@#To study the association between paroxysmal nocturnal hemoglobinuria (PNH) clone and immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA).@*METHODS@#A retrospective analysis was performed on the medical data of 151 children with SAA who were admitted and received IST from January 2012 to May 2020. According to the status of PNH clone, these children were divided into a negative PNH clone group (n=135) and a positive PNH clone group (n=16). Propensity score matching was used to balance the confounding factors, and the impact of PNH clone on the therapeutic effect of IST was analyzed.@*RESULTS@#The children with positive PNH clone accounted for 10.6% (16/151), and the median granulocyte clone size was 1.8%. The children with positive PNH clone had an older age and a higher reticulocyte count at diagnosis (P<0.05). After propensity score matching, there were no significant differences in baseline features between the negative PNH clone and positive PNH clone groups (P>0.05). The positive PNH clone group had a significantly lower overall response rate than the negative PNH clone group at 6, 12, and 24 months after IST (P<0.05). The evolution of PNH clone was heterogeneous after IST, and the children with PNH clone showed an increase in the 3-year cumulative incidence rate of aplastic anemia-PNH syndrome (P<0.05).@*CONCLUSIONS@#SAA children with positive PNH clone at diagnosis tend to have poor response to IST and are more likely to develop aplastic anemia-PNH syndrome.

Correlation between Serum G-CSF Level and Immune Function in Children with Aplastic Anemia / 中国实验血液学杂志

OBJECTIVE@#To observe the occurrence of immune dysfunction in children with aplastic anemia (AA) and the factors that may lead to immune dysfunction, analyze the relationship between the expression of granulocyte colony stimulating factor (G-CSF) and immune dysfunction.@*METHODS@#A total of 34 children with AA treated in our hospital from December 2016 to September 2018 were selected. All the children received immunosuppressive therapy (IST) for 6 months. According to whether the children had immune dysfunction after 6 months of treatment, they were divided into occurrence group and non occurrence group. General information and laboratory indices were compared between the two groups, and serum G-CSF level was tested, the relationship between serum G-CSF level and immune dysfunction in AA children after treatment with IST was observed and analyzed.@*RESULTS@#After treatment with IST for 6 months, 12 cases developed immune dysfunction (35.29%). Serum interferon (IFN)-γ level of the occurrence group was higher but G-CSF level was lower than those of the non occurrence group (P<0.05), while the difference of other baseline data was not statistically significant (P>0.05). Multiple regression analysis showed that overexpression of serum IFN-γ and low expression of G-CSF were both the influencing factors of immune dysfunction in AA children after IST treatment (OR>1, P<0.05). ROC curve was drawn, and the result showed that the area under the curve (AUC) of serum G-CSF level predicted the risk of immune dysfunction after IST was 0.843>0.80, when the index cut-off value was set at 6.614 pg/ml, the predictive value was ideal.@*CONCLUSION@#AA children have a higher risk of immune dysfunction after IST, which may be related to the low expression of serum G-CSF. The detection of serum G-CSF expression can be considered to predict the risk of immune dysfunction in AA children after IST, so as to guide early clinical intervention.

Pharmacogenomics of mycophenolic acid in kidney transplantation: Contribution of immune response-related genes

Abstract Mycophenolic acid (MPA) inhibits IMPDH, involved in the guanosine nucleotides synthesis, and prevents DNA replication in immune cells. The repression of cell and humoral immunity by MPA induces allograft tolerance preventing acute rejection in solid organ transplantation. MPA is an effective and safe drug, but genetic and non-genetic factors have been implicated in the interindividual variability of drug response. Several studies have shown the impact of variants of pharmacokinetics or pharmacodynamics-related genes on MPA response in kidney transplantation. This review explored further the influence of genes involved in the immune response on clinical outcomes of kidney recipients on short- or long-term MPA treatment. Variants in genes related to T cell activation (CD28, CTL4, ICOS, PDPC1), pro-inflammatory cytokines (IL2, IL6, IL12A, IL12B, TNF, IFNG), immunomodulatory cytokines (IL4, IL10, TGFB1), and innate immune response (CD14, TLR2, TLR4) were shown to be associated with increased risk of acute rejection, graft function or survival, chronic graft nephropathy, viral infections or MPA-induced myelotoxicity. Some of the significant pharmacogenetic associations were confirmed by meta-analyses of kidney transplantation. These findings are suggestive that variants in immune response-related genes contribute to the variability of MPA response, and have potential application as biomarkers of acute rejection in kidney transplantation.

Diagnosis and treatment analysis of living kidney transplantation between identical twins / 中华泌尿外科杂志

The effect of living kidney transplantation between identical twins is satisfied, but it is rarely reported. From October 2019 to February 2021, two recipients received kidney transplantation from their twin sisters in the Second Xiangya Hospital of Central South University. The primary disease of the two recipients was acute glomerulonephritis in 1 case and diabetic nephropathy in 1 case. Two recipients received tacrolimus/cyclosporine+ mortemycophenol ester+ methylprednisolone after surgery. The patients were followed up for 3.0 and 1.5 years, respectively, with renal function recovering well.

Safety and efficacy of a simplified once-daily immunosuppressive regimen in kidney transplant recipients / 中华器官移植杂志

Objective:To observe the safety and efficacy of early conversion into a simplified once-daily immunosuppressive regimen of sirolimus plus low-dose extended-release tacrolimus in kidney transplant recipients.Methods:From April 2019 to August 2020, clinical data were collected from 44 recipients (22 pairs) of kidney transplantation. Two kidneys from the same donor were randomly divided into observed and control groups. The immunosuppressive regimen of two groups was the same within 1 month post-transplantation, i. e. tacrolimus plus mycophenolatemofetil (or mycophenolate sodium) and prednisone. Then the immunosuppressive regimen of observed group was switched into a simplified once-daily regimen of sirolimus plus low-dose extended-release tacrolimus and prednisone while control group remained unchanged. The changes of graft function, proteinuria and the incidence of related adverse events were recorded.Results:No inter-group difference existed in serum level of creatinine at Month 1 post-transplantation (163.40±51.57 vs 166.10±49.48 μmol/L). After regimen conversion, serum level of creatinine was slightly lower in observed group than that in control group at Months 3 and 6 post-transplantation (130.10±30.10 vs 134.90±28.97, 121.50±24.96 vs 136.30±27.06). However, there was no statistic difference. The 24-hour urinary total trace protein was slightly higher in observed group than that in control group (331.20±84.21 vs 279.50±80.91 and 209.60±66.02 vs 179.50±37.60 mg/24 h) at Months 3 and 6 post-transplantation. However, there was no statistic difference. No inter-group difference existed in the incidence of drug side effects or other adverse events.Conclusions:In kidney transplant recipients at Month 1 post-transplantation, a conversion of immunosuppressive regimen into a simplified once-daily of sirolimus plus low-dose extended-release tacrolimus can significantly boost patient compliance without an elevated risk of drug side effects or adverse events and offer an advantage of reducing nephrotoxicity.

Diagnosis and treatment specification for immunosuppressive therapy and rejection of liver transplantation in China (2019 edition) / 器官移植

The liver is an "immune-privileged organ". The incidence and severity of acute rejection after liver transplantation are significantly lower than those after the transplantation of other organs. However, postoperative rejection is still relatively common, and standardized immunosuppressive therapy is the key to guarantee the efficacy of transplantation. To further standardize the immunosuppressive therapy and diagnosis and treatment of rejection after liver transplantation, Branch of Organ Transplantation of Chinese Medical Association organized liver transplant experts to summarize the latest research progress at home and abroad, integrate international guidelines and clinical practice and formulate the "Diagnosis and treatment specification for immunosuppressive therapy and rejection of liver transplantation in China (2019 edition)" based upon the application principle and common regime of immunosuppressant, as well as diagnosis and treatment of various types of rejection after liver transplantation.

Anemia aplásica / Aplastic anemia

Resumen La pandemia de enfermedad por coronavirus 2019 (COVID-19) ha cambiado la perspectiva médica para el tratamiento de no solo de enfermedades hematológicas, sino en general de la medicina. Respecto a la anemia aplásica (AA), principalmente la muy severa, en la que el paciente se presenta con menos de 200 neutrófilos absolutos, el riesgo de infección potencialmente mortal es alta y el inicio de terapia inmunosupresora también representa un riesgo, al menos temporal, para COVID-19. Se ha recomendado incluso aplazar el trasplante de células progenitoras hematopoyéticas en muchos pacientes para evitar un contagio. Una inmunosupresión moderada preferentemente ambulatoria que incluya agentes trombomiméticos es la opción terapéutica en tiempos de la pandemia actual. En esta revisión se enlistan las recomendaciones internacionales y nacionales respecto al tratamiento y seguimiento de pacientes con AA con base en experiencias de países que ya han pasado por esta emergencia sanitaria.

Abstract Medical practice in general has changed due to coronavirus disease 2019 (COVID-19) pandemic. Some hematologic diseases require immunosuppresive therapy placing patients at high risk of infection, including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Aplastic anemia (AA) especially the very severe type in which the count of absolute neutrophils is less than 200/ml is a life-threatening condition. Although bone marrow transplant is a potential curative treatment, it should be delayed temporally in order to prevent a contagion. Hospitalization may expose patients to infection, thus an ambulatory immunosuppression with oral cyclosporine and thrombopoietin agonist should be an adequate option. This work reviews international and national treatment recommendations and follow-up of patients with AA based on experiences from countries that have already faced this health emergency.

Eritema multiforme limitado à cavidade oral em um cão: relato de caso / Multiforme erythema limited to the oral cavity in a dog: case report

O eritema multiforme é uma doença incomum em cães, que afeta pele e mucosas, cuja etiologia ainda não foi completamente elucidada. Contudo, o envolvimento exclusivo da cavidade oral é considerado raro, tendo sido descrito poucas vezes até o presente momento. O objetivo deste trabalho é descrever um caso de eritema multiforme limitado à cavidade oral em um canino. Um cão, fêmea, Akita, com sete anos de idade, apresentou histórico de ulcerações na cavidade oral e nas laterais da língua, sem alterações cutâneas ou sistêmicas. O diagnóstico definitivo foi realizado por meio do exame histopatológico da mucosa oral, e a terapia imunossupressora empregada mostrou-se eficaz. Embora o eritema multiforme seja considerado incomum na espécie canina, este relato de caso apresenta a forma mais rara da doença, com poucos casos descritos em medicina veterinária.(AU)

Multiforme erythema is an uncommon disease in dogs that affects the skin and mucous membranes, the etiology of which has not yet been fully elucidated. However, the exclusive involvement of the oral cavity is considered rare, having been described few times until the present moment. The aim of this work is to describe a case of multiforme erythema limited to the oral cavity in a canine. A seven-year-old female dog, akita, presented a history of ulcerations in the oral cavity and on the sides of the tongue, with no cutaneous or systemic changes. The definitive diagnosis was made through the histopathological examination of the oral mucosa and the immunosuppressive therapy used proved to be effective. Although multiforme erythema is considered uncommon in the canine species, this case report presents the rarest form of the disease, with few cases described in veterinary medicine.(AU)

Treatment progress of severe aplastic anemia / 器官移植

Severe aplastic anemia (SAA) is a rare type of bone marrow hematopoietic failure, which is associated with toxic T lymphocyte-based immune dysfunction, abnormal hematopoietic microenvironment and damage of hematopoietic stem cells in patients. SAA characterized by acute onset, rapid progression and high mortality rate, which requires rapid and stable recovery of the patients' hematopoietic function. In this article, the therapeutic progresses on immunosuppressive therapy (IST), sibling human leukocyte antigen (HLA)-matched allogenetic hematopoietic stem cell transplantation (allo-HSCT), replacement of donor hematopoietic stem cell transplantation and unrelated umbilical cord blood hematopoietic stem cell transplantation (UCBT) for SAA were reviewed.

Case report of a successful term pregnancy following renal transplantation

Pregnancy with renal disease is associated with high risk for both mother and fetus with adverse outcomes. Criteria for considering pregnancy in renal transplanted patients include good post-transplant health for 2 years, stable allograft function with a serum creatinine <1.5 mg/dl, absence of rejection, control of blood pressure, absence of proteinuria. Authors report a case of 26-year-old, primigravida had renal transplantation done in NRI Medical College and treated with immunosuppressive therapy with tablets Tacrolimus 1mg twice daily, Azathioprine 50mg twice daily, Prednisolone 10mg once daily and continued till delivery. Developed gestational hypertension treated with tablet Amlodipine 5mg once daily. Elective caesarean section done for contracted pelvis. Post-natal period was uneventful and discharged on immunosuppressive therapy and contraceptive advice. Post-renal transplantation pregnancy should have multidisciplinary approach for. With close medical and obstetric follow up, successful outcome for both mother and infant is possible.

Hidradenitis Suppurativa: A Systematic Review and Meta-analysis of Therapeutic Interventions

Hidradenitis suppurativa is a chronic inflammatory condition that affects skin regions bearing apocrine glands. Although hidradenitis suppurativa is difficult to treat and cure, the currently available treatments are directed toward managing the lesions and associated symptoms. This review presents an evidence-based outline of the available treatment options. We searched four electronic databases and extracted data from retrieved studies for qualitative or quantitative analysis. Meta-analysis was conducted using the comprehensive meta-analysis software to generate pooled standardized mean differences or risk ratios. Numerous medical treatments are available for hidradenitis suppurativa such as antibiotics, retinoids, antiandrogens, immunosuppressive and anti-inflammatory agents and radiotherapy for early lesions. Adalimumab, an anti-tumor necrosis factor antibody, was superior to placebo in reducing Sartorius score (standardized mean difference = −0.32, confidence interval [−0.46, −0.18], P < 0.0001) and pain (risk ratio = 1.42, confidence interval [1.07, 1.9], P = 0.02), when given weekly (not every other week). Combination therapies (such as antibiotics and hyperbaric oxygen therapy) have been tested, which have shown promising results that are yet to be confirmed. Based on the quality of evidence, the most recommended treatments for hidradenitis suppurativa include adalimumab and laser therapy. Surgery (either by simple excision or complete local excision followed by skin graft) is the first choice for intractable disease presenting in the late stages. However, the evidence on most of these treatments is deficient and further randomized trials are needed to establish the most efficient therapies for hidradenitis suppurativa management.