Abstract 144: Lung Parenchymal Consolidation during typical Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Seiichi Sato
Keyword(s):  
Kawasaki Disease ◽  
Lymph Nodes ◽  
Past History ◽  
Systemic Vasculitis ◽  
Case Reports ◽  
Parvovirus B19 ◽  
Severe Pneumonia ◽  
Pulmonary Involvement ◽  
The Body ◽  
Barr Virus

Introduction: Kawasaki disease (KD) is a systemic vasculitis of childhood throughout the body. We report a patient who complicated by severe pneumonia of lung parenchymal consolidation (LPC) during typical KD clinical course. Case reports: A 7-yr-old male who had a past history of KD at 3-yr-old, visited a general practitioner with fever, sore throat and neck pain. Three days later, after being treated with CTRX/TBPMPI, he was transferred to our hospital because of bilateral lymph nodes swelling and a generalized erythema and rash. On admission he was still febrile and had bilateral conjunctival injections, cervical and axillary lymphadenopathy and markedly reddened pharynx, without edema. We diagnosed him as KD and treated him with aspirin and IVIG at a dose of 2.0g/kg, but the condition worsened; he developed dyspnea and dry cough. Crackles were noted on rt-lung auscultation, along with rt-basal dullness to percussion of the thorax. Chest radiography and a subsequent computed tomography scan revealed disseminated patchy infiltrates and ground-glass alterations with massive pleural effusions on rt-lung, and enlarged axillary and mediastinal lymph nodes. Some kinds of microbiology (including Mycoplasma) and virology (Epstein-Barr virus, cytomegalovirus, parvovirus B19), from all sampled sites (pleural effusion, blood, pharyngeal swab and cerebrospinal fluid), were negative for pathogenic specimens. So we treated him as intractable KD with second-IVIG and m-PSL, which led to the quick resolution of fever and progressive amelioration of pneumonia. Echocardiography showed normal ventricular size and function without coronary artery lesion (CAL). Thereafter, he was finally afebrile. At the first follow-up visit, 1 month after discharge, he had fully recovered. Clinically, echocardiographically and radiographically, no residues were noted. Conclusion: Several manifestations of broncho-pulmonary involvement in KD have been described. But there are very few case of LPC. We should consider the possibility that LPC were secondary to pulmonary arteritis.

scholarly journals Metastatic Lymphoepithelioma-like Carcinoma Treated with PD1-Antibody:Is Epstein-Barr Virus Eradication the Main Mechanism in 2 Refractory Cases in Children?

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5615-5615
Author(s):  
Haixia Guo ◽  
Xuedong Wu ◽  
Na LI ◽  
Jian LI
Keyword(s):  
Pleural Effusion ◽  
Lymph Nodes ◽  
Epstein Barr Virus ◽  
Conflicts Of Interest ◽  
Previous History ◽  
The Body ◽  
Whole Body ◽  
Barr Virus ◽  
Eb Virus ◽  
Epstein Barr

Background: Lymphoepithelioma-like carcinoma (LELC) is a rare high-grade carcinoma that can occur throughout the body. It is usually associated with Epstein-Barr virus (EBV) infection in patients from Asian countries. Pulmonary LELC occurs mostly in Asian women,, in their fifth or sixth decade, with no previous history of smoking. Optimal treatment has not been clearly established. Treatment options are based on surgery for early stage and on multimodal therapy for advanced stages.There is no report on children treated with programmed cell death 1 (PD1) inhibitor. Case Description: Case 1, a 12-year-old female who was admitted for cough and serious neck swelling. PET-CT showed multiple lymph nodes enlargement in the whole body ;among them, the anterior mediastinal lesions were fused with each other and the boundary between adjacent large vessels was unclear; multiple lung and pleural metastasis . EB virus in blood was 7.48×104copies/mL.Bone marrow morphology: Naive lymphocytes account for 2.5%, some lymphocytes are irregular; Mature plasma cells easy to see.Bone marrow immunotypes: 0.56% CD19-CD5-CD10-abnormally mature B lymphocytes. Biopsy: (left neck lump) LELC, EBERs(+).Partial remission was achieved after 2 courses of paclitaxel / carboplatin/ apatinib protocal. Due to delay with varicella, the tumor came back. After the third course of treatment,she showed chest tightness and pleural effusion, EB virus in blood increased, PDL1 protein was TPS 80% positive. After one PD1 antibody combined with chemotherapy,lymph nodes and thoracic lesions significantly reduced (Image1-3), blood EB virus turned to negative. She is now continuing with PD1 inhibitor. Case 2 , 10-year-old boy with huge right mouth bottom and right neck mass was diagnosed as LELC with EBER(+), TP53 (3%, +), CMYC (80% +). After chemotherapy, surgery and radiotherapy,he achieved complete remission. However, multiple metastases in mediastinum lymph nodes, pleura and lung happened and surgery, chemotherapy followed by automatic stem cell transplantation and pulmonary radiotherapy were given. CT-led puncture biopsy of suspected pulmonary residual lesion showed chronic inflammation with the mild growth of the alveoli, no malignant tumor.The child took oral Tegafur as maintenance regimen. 2 months later, recurrence appeared in lymph nodes, lung ,pleural and acetabular with positive EB virus in pleural effusion. Gene detection with NGS, which includes 312 gene-wide exon region, 208 gene hotspots and 16 fusion genes, did not show mutations such as gene mutations, amplification, and fusion with a clear clinical correlation. Tumor mutation load (TMB) was low, however,the boy had attained good response after PD1 antibody followed by rescue regimen and negative blood EB virus .The 2 patients didn't develop side effects correlating with PD1 inhibitor. Conclusion: To our knowledge, this is the first report of the use of PD1 inhibitor in children with metastatic LELC. These 2 refractory cases were all Epstein-Barr virus (EBV)-driven LELC,are more likely to respond to PD-1 blockade regardless of PD1 expresssion or TMB. EB virus burden disappeared after PD1 blockade,which perhaps can explain the anti-tumor effect.More cases are needed to verify the potential benefits of PD1 inhibitors to treat refractory LELC in children. Figure Disclosures No relevant conflicts of interest to declare.

scholarly journals Incomplete Kawasaki disease in Egypt

2018 ◽  
Vol 2017 (3) ◽  
Author(s):  
Hala M Agha ◽  
Hala S Hamza
Keyword(s):  
Kawasaki Disease ◽  
Viral Infections ◽  
Systemic Vasculitis ◽  
Vascular Inflammation ◽  
Clinical Findings ◽  
Unknown Etiology ◽  
Cervical Lymphadenitis ◽  
Barr Virus ◽  
Childhood Illnesses ◽  
Epstein Barr

[first paragraph of article]Kawasaki disease (KD) is a hybrid condition at the junction of infectious diseases, immunology, rheumatology, and cardiology.1 KD is a systemic vasculitis of unknown etiology predominately affecting medium-sized vessels such as the coronary arteries, which mainly affects infants and children2. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in genetically susceptible hosts3. Untreated KD may lead to the formation of coronary artery aneurysms and sudden cardiac death in children. The diagnosis of KD is based on the clinical features of fever of at least 5 days together with at least 4 or 5 other features including rash, bilateral conjunctival injection, changes in peripheral extremities, lymphadenopathy and oropharyngeal changes4. The diseases that must be differentiated from KD because of similar clinical findings include viral infections (measles, adenovirus, enterovirus, and Epstein-Barr virus), scarlet fever, staphylococcal scaled skin syndrome, toxic shock syndrome, polyarteritis nodosa, bacterial cervical lymphadenitis, and juvenile rheumatoid arthritis5,6. Because each of the symptoms commonly occurs in other childhood illnesses, the disease can be difficult to diagnose, especially in children who present with an incomplete form of the disease. KD has not been previously reported from Egypt and there are special challenges in recognizing complete KD in a country where physicians have limited experience with the disease. The diagnosis of incomplete KD is thus even more challenging in this setting. 

Nonnasopharyngeal Lymphoepitheliomas (Undifferentiated Carcinomas) of the Upper Aerodigestive Tract

1995 ◽  
Vol 104 (4) ◽  
pp. 305-310 ◽  
Author(s):  
Douglas K. Frank ◽  
Fred Cheron ◽  
Damian DiCostanzo ◽  
Hyun Cho ◽  
Anthony P. Sclafani
Keyword(s):  
Malignant Tumors ◽  
Case Reports ◽  
Strong Association ◽  
Dna Amplification ◽  
Aerodigestive Tract ◽  
The Body ◽  
World Health ◽  
Upper Aerodigestive Tract ◽  
Barr Virus ◽  
Ebv Infection

Lymphoepitheliomas are malignant tumors of epithelial origin with various amounts of reactive lymphocytic infiltrate. Although initially described in the nasopharynx (World Health Organization type 3 nasopharyngeal carcinoma), these tumors have been identified in various locations throughout the body. A strong association with Epstein-Barr virus (EBV) infection has been established for the nasopharyngeal type. Outside the nasopharynx, lymphoepitheliomas are exceedingly rare in the upper aerodigestive tract, with only isolated case reports of tumors in the larynx, trachea, and hypopharynx. This article features a rare case of lymphoepithelioma of the pyriform sinus. Furthermore, serologic testing, as well as in situ tumor DNA amplification (using the polymerase chain reaction) and hybridization techniques, demonstrated an association of this lesion with EBV infection. The characteristic histopathologic features common to this disease entity are presented, and the literature is reviewed with regard to lymphoepitheliomas of the upper aerodigestive tract outside the nasopharynx. Association of lymphoepitheliomas with EBV infection will be discussed.

scholarly journals Epstein-Barr Virus Induces Expression of the LPAM-1 Integrin in B CellsIn VitroandIn Vivo

2018 ◽  
Vol 93 (5) ◽  
Author(s):  
Susanne Delecluse ◽  
Ming-Han Tsai ◽  
Anatoliy Shumilov ◽  
Maja Bencun ◽  
Sebastian Arrow ◽  
...  
Keyword(s):  
B Cells ◽  
Lymph Nodes ◽  
B Cell ◽  
Epstein Barr Virus ◽  
Digestive System ◽  
The Body ◽  
Barr Virus ◽  
Ebv Infection ◽  
Infected Cells ◽  
Epstein Barr

ABSTRACTEpstein-Barr virus (EBV) infects the oropharynx but, surprisingly, frequently induces B cell proliferation in the gut of immunosuppressed individuals. We found that EBV infectionin vitroinduces the expression of the LPAM-1 integrin on tonsillar B cells and increases it on peripheral blood cells. Similarly, LPAM-1 was induced in the tonsils of patients undergoing primary infectious mononucleosis. EBV-induced LPAM-1 bound to the MAdCAM-1 addressin, which allows B cell homing to the gastrointestinal mucosa-associated lymphoid tissue (GALT). Thus, we hypothesized that EBV-induced LPAM-1 could induce relocation of infected B cells from the tonsil to the GALT.In situhybridization with an EBER-specific probe revealed the frequent presence of EBV-infected cells in the pericolic lymph nodes of healthy individuals. Relocation of infected B cells into the GALT would expand the EBV reservoir, possibly protecting it from T cells primed in the oropharynx, and explain why EBV induces lymphoid tumors in the gut.IMPORTANCEEBV causes tumors in multiple organs, particularly in the oro- and nasopharyngeal area but also in the digestive system. This virus enters the body in the oropharynx and establishes a chronic infection in this area. The observation that the virus causes tumors in the digestive system implies that the infected cells can move to this organ. We found that EBV infection induces the expression of integrin beta 7 (ITGB7), an integrin that associates with integrin alpha 4 to form the LPAM-1 dimer. LPAM-1 is key for homing of B cells to the gastrointestinal tract, suggesting that induction of this molecule is the mechanism through which EBV-infected cells enter this organ. In favor of this hypothesis, we could also detect EBV-infected cells in the lymph nodes adjacent to the colon and in the appendix.

Abstract 184: An Atypical Presentation of Incomplete Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Mario Briceno-Medina ◽  
Michael Perez ◽  
Benjamin R Waller ◽  
Shyam Sathanandam
Keyword(s):  
Kawasaki Disease ◽  
Clinical Symptoms ◽  
Systemic Vasculitis ◽  
Pulmonary Arteries ◽  
Cervical Lymphadenopathy ◽  
The Body ◽  
High Dose ◽  
Systemic Involvement ◽  
Systemic Artery ◽  
Echocardiographic Assessment

Background: Kawasaki disease (KD) is a systemic panvasculitis that can cause coronary artery aneurysms (CAA) in up to 25% if left untreated. Rarely, aneurysms of other medium-sized arteries have also been reported to occur. The incidence of systemic artery aneurysms (SAA) with typical KD can be as high a 2.2%. Incomplete KD with SAA is not well described. We report a case of diffuse SAA in a 12-year-old boy with incomplete KD and giant CAA. Case Presentation: The patient presented with fever, malaise, abdominal pain, rash and cervical lymphadenopathy. Suspicion for the presence of a pericardial effusion on an abdominal CT scan prompted echocardiographic assessment. By echo, multiple giant CAA in all three coronary arteries was noted. Incidentally, on an aortogram after performing pericardiocentesis, it was noted that he had diffuse ectasia and aneurysms of every arterial branch off the aorta. Selective angiograms confirmed the presence of aneurysms in all medium-sized arteries throughout the body including pulmonary arteries. The patient was treated with IVIG, methylprednisolone and high dose aspirin. Incomplete KD was suspected. However, because of the systemic vasculitis, cyclophosphamide therapy was administered. Patient responded well to therapy with improvement in clinical symptoms. Anticoagulation with heparin was transitioned over to maintenance warfarin therapy. Patient remains asymptomatic 2 years later with large, but stable CAA. Conclusions: Incomplete KD can manifest with giant CAA and SAA. Immunosuppressant therapy may be necessary for systemic involvement. Anticoagulation is needed to prevent thromboembolic manifestations.

scholarly journals Case Report of a Rare Cause of Reactive Arthritis: Leptospirosis

2021 ◽  
Vol 11 (3) ◽  
Author(s):  
Sudipan Dey ◽  
Arun Kumar Sipani ◽  
Rajdeep Das
Keyword(s):  
Case Report ◽  
Hip Joint ◽  
Reactive Arthritis ◽  
Past History ◽  
Case Reports ◽  
Female Child ◽  
The Body ◽  
Pathogenic Leptospira ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Introduction: Leptospirosis is a zoonosis caused by infection with pathogenic Leptospira species. Leptospirosis has protean manifestations and rare, unusual presentations should be kept in mind in relevant epidemiological scenario. Reactive arthritis refers to acute non-purulent arthritis complicating an infection elsewhere in the body. It is attributed to an immune activation following certain infections; it is, therefore considered as aseptic arthritis. Very few case reports are available attributing leptospirosis as an established cause of reactive arthritis. We present a case of reactive arthritis of the hip joint due to leptospirosis. Case Report: Here, we present a case of a 12-years- old female child who was admitted to our hospital with complaints of fever, headache, and pain in the right hip joint since past 5 days from admission. Subsequent elaboration revealed a past history of fever, headache, and myalgia for around 5-7 days around a week before the present complaints. There was rat infestation near her house and her father was working as sewage cleaner. Routine investigations, Ultrasonography (USG), Magnetic Resonance Imaging (MRI) of both hips and subsequently, diagnostic hip aspiration was performed. USG revealed synovitis, MRI revealed hip joint arthritis of infective or inflammatory origin. Diagnostic hip aspiration was negative for any microorganism. On 10th day of admission, patient started developing icterus with yellowish discolouration of urine. Patient was evaluated for the cause of jaundice. Screening for Leptospira was positive. Synovial biopsy of hip was performed, which showed inflammation with no specific pathology and no growth of any microorganism. In addition, Leptospira IgM MAC ELISA was done which was positive. Patient was thus confirmed to be having leptospirosis and reactive arthritis as a consequence of it. Conclusion: The presentation of reactive arthritis secondary to leptospirosis is rare. Leptospirosis can be an etiological fa

Scanning electron microscopy of freeze-fractured prescapular lymph node of caprine

1984 ◽  
Vol 42 ◽  
pp. 244-245
Author(s):  
O. Faroon ◽  
F. Al-Bagdadi ◽  
T. G. Snider ◽  
C. Titkemeyer
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Lymphatic System ◽  
Three Dimensional ◽  
Meat Products ◽  
The Body ◽  
Morphological Data ◽  
The World ◽  
Cellular Constituent ◽  
Scanning Electron

The lymphatic system is very important in the immunological activities of the body. Clinicians confirm the diagnosis of infectious diseases by palpating the involved cutaneous lymph node for changes in size, heat, and consistency. Clinical pathologists diagnose systemic diseases through biopsies of superficial lymph nodes. In many parts of the world the goat is considered as an important source of milk and meat products.The lymphatic system has been studied extensively. These studies lack precise information on the natural morphology of the lymph nodes and their vascular and cellular constituent. This is due to using improper technique for such studies. A few studies used the SEM, conducted by cutting the lymph node with a blade. The morphological data collected by this method are artificial and do not reflect the normal three dimensional surface of the examined area of the lymph node. SEM has been used to study the lymph vessels and lymph nodes of different animals. No information on the cutaneous lymph nodes of the goat has ever been collected using the scanning electron microscope.

EFFECT OF DIFFERENT DOSES OF PREDNISOLONE AND ADRENOCORTICOTROPHIC HORMONE (ACTH) ON LYMPHATIC LUNG CLEARANCE AND ON EXPERIMENTAL SILICOSIS

1970 ◽  
Vol 63 (2) ◽  
pp. 325-337
Author(s):  
Carl-Johan Göthe
Keyword(s):  
Lymph Nodes ◽  
Lymphatic System ◽  
The Body ◽  
Lung Weight ◽  
Lung Clearance ◽  
Dust Transport ◽  
Fine Particulate ◽  
Quartz Dust ◽  
Different Doses ◽  
Stimulation Of

ABSTRACT The effect of three doses of prednisolone and ACTH respectively on the weight of the body, the lungs and the hilar lymph nodes was studied on rats killed one month after the intratracheal (i.t.) injection of 50 mg of fine-particulate quartz. The prednisolone was administered via the drinking water, and the ACTH was injected intraperitoneally during the period between the i.t. injection of quartz dust and the killing of the animals. Prednisolone causes the rats to become cachectic and reduces the weight of the hilar lymph nodes. It also retards the transport of quartz dust from the lungs via the lymphatics. All these effects increase with increasing doses of prednisolone. However, its effect on the lung weight is insignificant. ACTH does not affect the body weight, but retards the weight increase of the lungs and the hilar lymph nodes. These effects increase with increasing doses of ACTH, and seem to be connected with an ability of ACTH to promote the clearance of quartz dust from the lungs and hilar lymph nodes. The method used, however, does not make it possible to differentiate quantitatively between any ACTH effects on the bronchogenie and lymphatic lung-clearance mechanisms. Available data, however, indicate that the stimulation of the dust transport from the lungs and hilar lymph nodes is, at least to some extent, related to the lymphatic system.

Linezolid Induced Skin Reactions in a Multi Drug Resistant Infective Endocarditis Patient: A Rare Case

2020 ◽  
Vol 15 (3) ◽  
pp. 222-226 ◽  
Author(s):  
Asha K. Rajan ◽  
Ananth Kashyap ◽  
Manik Chhabra ◽  
Muhammed Rashid
Keyword(s):  
Case Report ◽  
Infective Endocarditis ◽  
Rare Case ◽  
Case Reports ◽  
Multidrug Resistant ◽  
The Body ◽  
Prior History ◽  
Skin Reactions ◽  
Rare Case Report ◽  
Multiple Drugs

Rationale: Linezolid (LNZ) induced Cutaneous Adverse Drug Reactions (CADRs) have rare atypical presentation. Till date, there are very few published case reports on LNZ induced CADRs among the multidrug-resistant patients suffering from Infective Endocarditis (MDR IE). Here, we present a rare case report of LNZ induced CARs in a MDR IE patient. Case report: A 24-year-old female patient was admitted to the hospital with chief complaints of fever (101°C) associated with rigors, chills, and shortness of breath (grade IV) for the past 4 days. She was diagnosed with MDR IE, having a prior history of rheumatic heart disease. She was prescribed LNZ 600mg IV BD for MDR IE, against Staphylococcus coagulase-negative. The patient experienced flares of cutaneous reactions with multiple hyper-pigmented maculopapular lesions all over the body after one week of LNZ therapy. Upon causality assessment, she was found to be suffering from LNZ induced CADRs. LNZ dose was tapered gradually and discontinued. The patient was prescribed corticosteroids along with other supportive care. Her reactions completely subsided and infection got controlled following 1 month of therapy. Conclusion: Healthcare professionals should be vigilant for rare CADRs, while monitoring the patients on LNZ therapy especially in MDR patients as they are exposed to multiple drugs. Moreover, strengthened spontaneous reporting is required for better quantification.

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