Case Reports in Immunology最新文献

{"title":"Mendelian Susceptibility to Mycobacterial Disease: The First Case of a Diagnosed Adult Patient in the Czech Republic.","authors":"Miroslav Prucha,&nbsp;Hana Grombirikova,&nbsp;Pavel Zdrahal,&nbsp;Marketa Bloomfield,&nbsp;Zuzana Parackova,&nbsp;Tomas Freiberger","doi":"10.1155/2020/8836685","DOIUrl":"https://doi.org/10.1155/2020/8836685","url":null,"abstract":"<p><p>We present a case of a 42-year-old woman with Mendelian susceptibility to mycobacterial disease. The disease was diagnosed at an adult age with relatively typical clinical manifestations; the skeleton, joints, and soft tissues were affected by nontuberculous mycobacteria: <i>Mycobacterium lentiflavum</i>, <i>M. kansasii</i>, and <i>M. avium</i>. A previously published loss-of-function and functionally validated variant NM_000416.2:c.819_822delTAAT in <i>IFNGR1</i> in a heterozygous state was detected using whole-exome sequencing. After interferon-<i>γ</i> therapy was started at a dose of 200 <i>µ</i>g/m² three times a week, there was significant clinical improvement, with the need to continue the macrolide-based combination regimen. In the last 4 months, she has been in this therapy without the need for antibiotic treatment.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7769627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39130536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"On Two Cases with Autosomal Dominant Hyper IgE Syndrome: Importance of Immunological Parameters for Clinical Course and Follow-Up.","authors":"Snezhina Mihailova Kandilarova,&nbsp;Spaska Stoyneva Lesichkova,&nbsp;Nevena Todorova Gesheva,&nbsp;Petya Stefanova Yankova,&nbsp;Nedelcho Hristov Ivanov,&nbsp;Guergana Petrova Stoyanova,&nbsp;Penka Ilieva Perenovska,&nbsp;Marta Petrova Baleva,&nbsp;Elissaveta Jordanova Naumova","doi":"10.1155/2020/6694957","DOIUrl":"https://doi.org/10.1155/2020/6694957","url":null,"abstract":"<p><p>Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare disease described in 1966. It is characterized by severe dermatitis, a peculiar face, frequent infections, extremely high levels of serum IgE and eosinophilia, all resulting from a defect in the <i>STAT3</i> gene. A variety of mutations in the SH2 and DNA-binding domain have been described, and several studies have searched for associations between the severity of the clinical symptoms, laboratory findings, and the type of genetic alteration. We present two children with AD-HIES-a girl with the most common <i>STAT3</i> mutation (R382W) and a boy with a rare variant (G617E) in the same gene, previously reported in only one other patient. Herein, we discuss the clinical and immunological findings in our patients, focusing on their importance on disease course and management.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7728468/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38733504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adverse Events of the BCG (Bacillus Calmette-Guérin) and Rotavirus Vaccines in a Young Infant with Inborn Error of Immunity.","authors":"Suleiman Al-Hammadi,&nbsp;Najla S Alkuwaiti,&nbsp;Ghassan A Ghatasheh,&nbsp;Huda Al Dhanhani,&nbsp;Hiba M Shendi,&nbsp;Abdulghani S Elomami,&nbsp;Farida Almarzooqi,&nbsp;Abdul-Kader Souid","doi":"10.1155/2020/8857152","DOIUrl":"https://doi.org/10.1155/2020/8857152","url":null,"abstract":"<p><strong>Background: </strong>The Bacillus Calmette-Guérin (BCG) and rotavirus vaccines are live-attenuated preparations. In the United Arab Emirates, these products are universally administered to the young infants. This unguided practice does not account for the children with immunodeficiency, which frequently manifests after the administration of these vaccines. We present here a young infant with immunodeficiency that developed disseminated tuberculosis infection and severe diarrhea due to these improper immunizations. <i>Case Presentation</i>. This young infant was diagnosed at six months of age with \"immunodeficiency type 19\" (MIM#615617) due to homozygous nonsense variant, NM_000732.4 (<i>CD3D</i>):c.128G > A, p.Trp43∗ (variation ClinVar#VCV000643120.1; pathogenic). This variant creates premature stop-gain in <i>CD3D</i> (CD3 antigen, delta subunit, autosomal recessive; MIM#186790), resulting in loss-of-function. He also had \"<i>X</i>-linked agammaglobulinemia\" (MIM#300755) due to hemizygous missense variant, NM_001287344.1 (<i>BTK</i>):c.80G > A, p.Gly27Asp (novel). He had a sibling who passed away in infancy of unknown disease and family members with autoimmune disorders. Despite these clear clues, he was immunized with BCG at birth and rotavirus at 2 and 4 months. He was well in the first four months. He then developed high-fever, lymphadenopathy, and refractory diarrhea. Stool was positive for rotavirus, and lymph node biopsy showed acid-fast bacilli, consistent with tuberculosis lymphadenitis. These infections were serious and markedly complicated his clinical course, which included bone marrow transplantation from a matched sibling.</p><p><strong>Conclusions: </strong>These unfortunate events could have been avoided by compiling the available clinical information. This patient underscores the importance of implementing proper policies for BCG and rotavirus vaccinations. International registries of adverse events of universally administered vaccines are crucial.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8857152","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39079634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Prolonged Catatonia Caused by Sjögren's Syndrome.","authors":"Takahiko Inagaki,&nbsp;Kotaro Kudo,&nbsp;Naoki Kurimoto,&nbsp;Takashi Aoki,&nbsp;Kenichi Kuriyama","doi":"10.1155/2020/8881503","DOIUrl":"https://doi.org/10.1155/2020/8881503","url":null,"abstract":"<p><p>Sjögren's syndrome (SS) is a chronic autoimmune disorder, often associated with some neuropsychiatric symptoms as well as systemic lupus erythematosus. Although catatonia is frequently reported in patients with systemic lupus erythematosus, it has been rarely reported in patients with SS. Herein, we present a case of SS with catatonia effectively and safely treated with modified electroconvulsive therapy (ECT). A 58-year-old woman showed prolonged catatonia and depressive mood along with pathologically dried eye and mouth. Based on physical findings and blood tests, she was diagnosed with SS. Because of the presence of pressure sores, we were unable to perform lumbar puncture for the diagnosis of abacterial encephalitis. Alternatively, single-photon emission computed tomography of her brain revealed multifocal hypoperfused areas in the parietotemporal region. Consequently, we performed ECT for the treatment of catatonia comorbid with SS. Following 20 sessions of ECT, the catatonia was improved without obvious adverse effects. One week after the last ECT, elevated levels of interleukin-6 were identified in the cerebral fluid. After receiving steroid pulse therapy, she has not experienced catatonia for more than 5 years. SS can cause catatonia, and ECT is a safe and effective option for the treatment of catatonia with SS.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8881503","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38623569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Interleukin-1 Receptor-Associated Kinase 4 Deficiency in a Greek Teenager.","authors":"Panagiota Karananou,&nbsp;Anastasia Alataki,&nbsp;Efimia Papadopoulou-Alataki","doi":"10.1155/2020/8846827","DOIUrl":"https://doi.org/10.1155/2020/8846827","url":null,"abstract":"<p><strong>Background: </strong>Human interleukin- (IL-) 1 receptor-associated kinase 4 (IRAK-4) deficiency is a recently described primary immunodeficiency. It is a rare, autosomal recessive immunodeficiency that impairs toll/IL-1R immunity, except for the toll-like receptor (TLR) 3- and TLR4-interferon alpha (IFNA)/beta (IFNB) pathways. <i>Case Report</i>. We report the first patient in Greece with IRAK-4 deficiency. From the age of 8 months, she presented with recurrent infections of the upper and lower respiratory tract and skin abscesses. For this, she had been repeatedly hospitalized and treated empirically with intravenous antibiotics. No severe viral, mycobacterial, or fungal infections were noted. Her immunological laboratory evaluation revealed low serum IgA and restored in subsequent measurements; normal IgG, IgM, and IgE; and normal serum IgG subclasses. Peripheral blood immunophenotyping by flow cytometry and dihydrorhodamine (DHR) test revealed normal counts. She was able to make functional antibodies against vaccine antigens, including tetanus and diphtheria. She was administered with empirical IgG substitution for 5 years until the age of 12 years, and she has never experienced invasive bacterial infections so far. DNA analysis revealed a heterozygous variant in the patient: c.823delT (p.S275fs ^<i>∗</i> 13 at protein level) in the IRAK4 gene.</p><p><strong>Conclusions: </strong>The importance of clinical suspicion is emphasized in order to confirm the diagnosis by IRAK4 gene sequencing and provide the appropriate treatment for this rare primary immunodeficiency, as soon as possible.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8846827","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38528410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IPEX Syndrome with Normal FOXP3 Protein Expression in Treg Cells in an Infant Presenting with Intractable Diarrhea as a Single Symptom.","authors":"Ali Al Maawali,&nbsp;Beata Derfalvi,&nbsp;Johan Van Limbergen,&nbsp;Andrew Issekutz,&nbsp;Thomas Issekutz,&nbsp;Hasan Ghandourah,&nbsp;Mohsin Rashid","doi":"10.1155/2020/9860863","DOIUrl":"https://doi.org/10.1155/2020/9860863","url":null,"abstract":"<p><p>IPEX (immune dysregulation-polyendocrinopathy-enteropathy-X-linked) syndrome is a rare, potentially fatal multisystem disorder caused by mutations in the <i>FOXP3</i> gene. This can lead to quantitative or functional deficiency of regulatory T cells (Treg), thereby affecting their immune-suppressive actions which can in turn cause autoimmune and inflammatory disorders. We describe an infant with IPEX syndrome with unremarkable maternal family history whose only presentations were severe diarrhea and malnutrition. The patient had a normal percentage of Treg cells and FOXP3 protein expression, but further testing revealed a hemizygous missense mutation in the <i>FOXP3</i> gene. IPEX syndrome should be considered in young children even if severe intractable diarrhea is the only symptom with no other autoimmune manifestations. Sequencing of the <i>FOXP3</i> gene should always be considered for accurate diagnosis to look for mutations even in the face of normal FOXP3 protein expression in the Treg cell.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9860863","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38506002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature.","authors":"Aysun Ata,&nbsp;Samim Özen,&nbsp;Damla Gökşen,&nbsp;Neslihan Edeer Karaca,&nbsp;Güzide Aksu,&nbsp;Necil Kütükçüler,&nbsp;Hüseyin Onay,&nbsp;Şükran Darcan","doi":"10.1155/2020/8891902","DOIUrl":"https://doi.org/10.1155/2020/8891902","url":null,"abstract":"<p><p>Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeability followed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy was admitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, and oral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchy edema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L, hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourth day, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immunoglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased on the fifth day, and the patient was deemed clinically well. There was no compartment syndrome, rhabdomyolysis, or pulmonary edema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) and enterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serum glucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive. However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit, 22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis, there was no ISCLS attack. <i>Conclusion</i>. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare in childhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of the disease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to the literature since there have been no pediatric diabetic patients with ISCLS reported.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8891902","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38497165","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor.","authors":"Yike Jiang,&nbsp;Mihail Firan,&nbsp;Sarada L Nandiwada,&nbsp;Anaid Reyes,&nbsp;Rebecca A Marsh,&nbsp;Tiphanie P Vogel,&nbsp;Joud Hajjar","doi":"10.1155/2020/8841571","DOIUrl":"https://doi.org/10.1155/2020/8841571","url":null,"abstract":"<p><p>X-linked lymphoproliferative disease (XLP1) is a rare primary immunodeficiency characterized by EBV-triggered immune dysregulation, lymphoproliferation, dysgammaglobulinemia, and lymphoma. Early childhood mortality from overwhelming inflammation is expected in most patients. The only curative therapy is hematopoietic stem cell transplant (HSCT); however, whether to perform HSCT on an asymptomatic patient remains debatable. This uncertainty arises because the natural history of XLP1 patients without transplantation is not clear. In this case report, we present the natural history of XLP1 in a 43-year-old male patient who did not receive HSCT. We also review the literature on untransplanted XLP1 patients who lived into mid-adulthood. Despite surviving childhood presentations that are typically fatal, we found that these rare patients remain susceptible to manifestations of XLP1 decades later.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8841571","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38362961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Terrible Triad of Checkpoint Inhibition: A Case Report of Myasthenia Gravis, Myocarditis, and Myositis Induced by Cemiplimab in a Patient with Metastatic Cutaneous Squamous Cell Carcinoma.","authors":"Nikeshan Jeyakumar,&nbsp;Mikel Etchegaray,&nbsp;Jason Henry,&nbsp;Laura Lelenwa,&nbsp;Bihong Zhao,&nbsp;Ana Segura,&nbsp;L Maximilian Buja","doi":"10.1155/2020/5126717","DOIUrl":"https://doi.org/10.1155/2020/5126717","url":null,"abstract":"<p><strong>Background: </strong>We report a case of a patient with squamous cell carcinoma (SCC) who developed myasthenia gravis (MG), myositis, and myocarditis after receiving cemiplimab, an anti-PD-1 immune checkpoint inhibitor (ICI). <i>Case Presentation.</i> An 86-year-old man with metastatic periocular SCC presented with decreased vision in the left eye, severe fatigue, and lower back and bilateral hip pain 3 weeks after receiving cemiplimab. Within hours, he developed dysphonia, pharyngeal secretions, and dysphagia, necessitating intubation. Endomyocardial biopsy revealed active lymphocyte-mediated necrosis consistent with ICI-induced myocarditis. Anti-striated muscle and anti-acetylcholine receptor antibodies were elevated, consistent with myositis and myasthenia gravis. Despite plasma exchange therapy, steroids, and intravenous immunoglobulin, he died from cardiac arrest.</p><p><strong>Conclusions: </strong>The presence of myasthenia gravis, myocarditis, or myositis should prompt evaluation for all three toxicities as they may represent an overlap syndrome. The severity of these immunotoxicities highlights the need for clinicians to suspect multiple simultaneous adverse effects of ICIs.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/5126717","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38179180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Injection Site Erythema in a Patient on Therapeutic Anticoagulation with Low Molecular Weight Heparin after Mechanical Aortic Valve Replacement: A Rare Presentation of Heparin- and Protamine-Induced Thrombocytopenia.","authors":"Caroline Holaubek,&nbsp;Paul Simon,&nbsp;Sabine Eichinger-Hasenauer,&nbsp;Franz Gremmel,&nbsp;Barbara Steinlechner","doi":"10.1155/2020/4503598","DOIUrl":"https://doi.org/10.1155/2020/4503598","url":null,"abstract":"<p><p>Previous exposition to heparin and protamine in patients undergoing cardiopulmonary bypass and postoperative therapeutic anticoagulation with LMWH may lead to the development of heparin-induced thrombocytopenia (HIT) and/or protamine-induced thrombocytopenia (PIT). This case deals with a rare clinical presentation of circulating IgG antibodies against heparin/platelet factor 4 complexes and heparin/protamine complexes after cardiac surgery. Ensuing purpura and skin necrosis (blisters) at the injection sites of LMWH and clinical symptoms improved rapidly after replacement of LMWH by an alternative anticoagulant. The aim of this report is to draw attention to the several different clinical manifestations of heparin- and/or protamine-induced thrombocytopenia and shows a possible course of treatment and recovery.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2020-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/4503598","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37867067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}