Abstract 193: Hemophagocytic lymphohistiocytosis following kawasaki disease:Differential Diagnosis in IVIG Refractory Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hyun Ok Jun ◽  
Eun Kyung Cho ◽  
Jeong Jin Yu ◽  
So Yeon Kang ◽  
Chang Deok Seo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Skin Rash ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Symptoms ◽  
Early Recognition ◽  
Cervical Lymphadenopathy ◽  
Inflammatory Disorder ◽  
Laboratory Findings

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb<9.0), hypertriglyceridemia, high level of ferritin was shown and had splenomegaly on physical examination. In the suspicion of HLH, bone marrow biopsy was done and revealed hemophagocytosis, consistent with HLH. A second case of 11-month-old boy admitted for 8-day fever with Kawasaki feature. Although, he showed incomplete feature(fever, skin rash, conjunctival injection, cervical lymphadenopathy), echocardiography showed dilated left main coronary artery(3.5mm) and treated with IVIG. However, 2days after IVIG administration, he was still pyrexial. The laboratory findings fulfilled 5 diagnostic criteria of HLH; bicytopenia(anemia, thrombocytopenia), hypofibrinogenemia, hyperferritinemia, hemophagocytosis in bone marrow, raised level of soluble IL-2 receptor. In both cases, the patients treated according to the HLH protocol 2004, and after that clinical symptoms and laboratory findings were improved. Several causes of febrile illness, EBV, CMV, rubella, parvo-viral infection, for example, were excluded. Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.

scholarly journals Case Report: Infantile Urticaria as a Herald of Neonatal Onset Multisystem Inflammatory Disease With a Novel Mutation in NLRP3

2021 ◽  
Vol 12 ◽  
Author(s):  
Anna E. Patrick ◽  
Eden M. Lyons ◽  
Lisa Ishii ◽  
Alan S. Boyd ◽  
Joseph M. Choi ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Early Recognition ◽  
Novel Mutation ◽  
Genetic Diagnosis ◽  
Inflammatory Disorder ◽  
Laboratory Findings ◽  
Genetic Sequencing ◽  
Variants Of Unknown Significance ◽  
Unknown Significance ◽  
The Impact

Neonatal multisystem onset inflammatory disorder (NOMID) is a severe autoinflammatory syndrome that can have an initial presentation as infantile urticaria. Thus, an immediate recognition of the clinical symptoms is essential for obtaining a genetic diagnosis and initiation of early therapies to prevent morbidity and mortality. Herein, we describe a neonate presenting with urticaria and systemic inflammation within hours after birth who developed arthropathy and neurologic findings. Pathologic evaluation of the skin revealed an infiltration of lymphocytes, eosinophils, and scattered neutrophils. Genetic analysis identified a novel heterozygous germline variant of unknown significance in the NLRP3 gene, causing the missense mutation M408T. Variants of unknown significance are common in genetic sequencing studies and are diagnostically challenging. Functional studies of the M408T variant demonstrated enhanced formation and activity of the NLRP3 inflammasome, with increased cleavage of the inflammatory cytokine IL-1β. Upon initiation of IL-1 pathway blockade, the infant had a robust response and improvement in clinical and laboratory findings. Our experimental data support that this novel variant in NLRP3 is causal for this infant’s diagnosis of NOMID. Rapid assessment of infantile urticaria with biopsy and genetic diagnosis led to early recognition and targeted anti-cytokine therapy. This observation expands the NOMID-causing variants in NLRP3 and underscores the role of genetic sequencing in rapidly identifying and treating autoinflammatory disease in infants. In addition, these findings highlight the importance of establishing the functional impact of variants of unknown significance, and the impact this knowledge may have on therapeutic decision making.

scholarly journals A Retrospective Cohort Study of Kawasaki Disease in Hue Central Hospital for 10 Years (2010–2019)

2020 ◽  
Vol 8 (B) ◽  
pp. 99-103
Author(s):  
Nguyen Huu Son ◽  
Tran Kiem Hao ◽  
Nguyen Thi Hoang Anh
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Gamma Globulin ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Response To Therapy ◽  
High Rate ◽  
Unknown Etiology ◽  
Central Hospital ◽  
Laboratory Findings

INTRODUCTION: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology which affects mainly children <5 years of age. If the disease is left untreated, it can lead to serious complications such as inflammation of the blood vessels. AIM: We aim to evaluate the clinical and laboratory findings and response to therapy of KD at Hue Central Hospital. METHODS: This is a retrospective study of patients with KD at Pediatric Center of Hue Central Hospital between January 2010 and December 2019. Clinical and laboratory examinations as well as the echocardiograms finding were analyzed. RESULTS: All patients were under 5 years old, in which boys were more than girls. Fever lasting over 5 days, changing in the mouth mucosa, and peripheral extremities were seen in all patients. About 73.2% had bilateral conjunctivitis and 78.0% had rash. About 42.3% of patients had cervical lymphadenopathy. Laboratory findings were noted with 84.5% of patients had hyperleukocytosis (>12,000/ mm3), 76.2% of patients had high serum C-reactive protein (CRP) levels (>100 mg/dl), 56% of patients had erythrocyte sediment rate >60 mm in the 1st h, and 34.5% of patients had thrombocytosis (platelet count >500,000/mm3) at the time of diagnosis. About 26.2% of patients had coronary artery lesions. Most patients (84.4%) had good outcome since the first dose of gamma-globulin and 13% of patients needed the second dose. There was a significant correlation between coronary artery abnormalities and no or late treatment of gamma-globulin. CONCLUSION: KD was very common in children under 5 years old with the high rate of coronary artery lesion. Treatment with gamma-globulin on or before 10 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.

Clinical aspects of splenomegaly as a possible predictive factor of coronary artery changes in Kawasaki disease

2018 ◽  
Vol 29 (3) ◽  
pp. 297-302 ◽  
Author(s):  
Dong Wan Kang ◽  
Sung Hoon Kim
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cervical Lymphadenopathy ◽  
Clinical Aspects ◽  
Acute Acalculous Cholecystitis ◽  
Coronary Artery Lesions ◽  
Incomplete Kawasaki Disease ◽  
Abdominal Ultrasonography ◽  
Laboratory Findings ◽  
Gallbladder Hydrops

AbstractBackgroundAlthough many clinical features that are not typically included in the diagnostic criteria for Kawasaki disease, such as gall bladder hydrops, are known to occur with Kawasaki disease, splenomegaly is not concerned. We investigated the relationship of splenomegaly with the development of coronary artery lesions in Kawasaki disease.Methods and resultsThis retrospective descriptive study was conducted through a review of medical records of children with Kawasaki disease from March 2011 to February 2017. We analysed information regarding clinical presentation, treatment, hospital stay, and outcome. A total of 396 patients during this 6-year period met the enrolment criteria. Of these, 77 (23.4%) underwent abdominal ultrasonography during the treatment period. The patients included 46 males and 31 females with an average age of 35.8 ± 26.1 months. Gallbladder hydrops were detected in 32 patients, and acute acalculous cholecystitis was not found. Splenomegaly was detected in 21 patients. Kawasaki disease patients with gallbladder hydrops had no statistical difference in clinical or laboratory findings or in development of coronary artery lesions compared to patients without gallbladder hydrops. However, patients with splenomegaly belonged more to incomplete Kawasaki disease, had longer fever duration, had more frequent cervical lymphadenopathy and polymorphous rash, had higher neutrophil percentage, N-terminal fragment of pro-brain natriuretic peptide, and alanine aminotransferase levels, and a higher incidence of coronary artery lesions than patients without splenomegaly.ConclusionSplenomegaly belongs to incomplete Kawasaki disease patients mainly with a higher incidence of coronary artery lesions than that of patients without it.

scholarly journals Desquamation in Kawasaki Disease

Children ◽  
2021 ◽  
Vol 8 (5) ◽  
pp. 317
Author(s):  
Ling-Sai Chang ◽  
Ken-Pen Weng ◽  
Jia-Huei Yan ◽  
Wan-Shan Lo ◽  
Mindy Ming-Huey Guo ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Laboratory Data ◽  
Ivig Treatment ◽  
Low Grade ◽  
High Grade ◽  
Laboratory Findings ◽  
Coronary Artery Abnormalities ◽  
Cell Counts ◽  
Hands And Feet

(1) Background: Desquamation is a common characteristic of Kawasaki disease (KD). In this study, we analyzed patients’ varying desquamation levels in their hands or feet, in correlation with clinical presentation, to assess the relationship. (2) Methods: We retrospectively reviewed children with KD. We analyzed their age, laboratory data before intravenous immunoglobulin (IVIG) treatment and coronary artery abnormalities (CAA) based on the desquamation level of their hands and feet. We classified the desquamation level from 0 to 3 and defined high-grade desquamation as grade 2 and 3. (3) Results: We enrolled a total 112 patients in the study. We found the hands’ high-grade desquamation was positively associated with age and segmented neutrophil percentage (p = 0.047 and 0.029, respectively) but negatively associated with lymphocyte and monocyte percentage (p = 0.03 and 0.006, respectively). Meanwhile, the feet’s high-grade desquamation was positively associated with total white blood cell counts (p = 0.033). Furthermore, we found that high-grade hand desquamation had less probability of CAA formation compared with that of a low grade (7.1% vs. 40.8%, p = 0.016). (4) Conclusions: This report is the first to demonstrate that the desquamation level of hands or feet in KD is associated with different coronary artery abnormalities and laboratory findings.

scholarly journals Relationship between ocular involvement and clinical manifestations, laboratory findings, and coronary artery dilatation in Kawasaki disease

Eye ◽  
2020 ◽  
Vol 34 (10) ◽  
pp. 1883-1887 ◽  
Author(s):  
Reza Shiari ◽  
Mohsen Jari ◽  
Saeed Karimi ◽  
Omid Salehpour ◽  
Khosro Rahmani ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Clinical Manifestations ◽  
Ocular Involvement ◽  
Laboratory Findings ◽  
Coronary Artery Dilatation

scholarly journals Torticollis as Presentation for Atypical Kawasaki Disease Complicated by Giant Coronary Artery Aneurysms

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tracey Dyer ◽  
Paul Dancey ◽  
John Martin ◽  
Suryakant Shah
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
High Dose ◽  
Coronary Artery Aneurysms ◽  
Low Dose Aspirin ◽  
Atypical Kawasaki Disease ◽  
Mucosal Changes

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis can be made in a patient who presents with a prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes (including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervical lymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present. We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations for elevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed with atypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treated with high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol were added due to the presence of giant aneurysms.

Lack of Association of Cervical Lymphadenopathy and Coronary Artery Complications in Kawasaki Disease

2006 ◽  
Vol 25 (6) ◽  
pp. 521-525 ◽  
Author(s):  
Rita Y. T. Sung ◽  
Yin-Ming Ng ◽  
Kai-Chow Choi ◽  
Geoffrey C. F. Mok ◽  
Y W. Cheng ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cervical Lymphadenopathy

scholarly journals Epidemiological Features and Clinical Study of Kawasaki Disease in Iran

2021 ◽  
Author(s):  
Parvin Akbariasbagh ◽  
Saharnaz Talebiyan ◽  
Yahya Aghighi ◽  
Reza Raeeskarai ◽  
Amirhosein Seyedhoseinpour ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Clinical Features ◽  
Systemic Vasculitis ◽  
Febrile Illness ◽  
Developed Countries ◽  
Response To Treatment ◽  
Unknown Etiology ◽  
Laboratory Findings ◽  
Female Ratio

Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown etiology and the major cause of pediatric acquired cardiac disease worldwide, particularly in developed countries. This study characterizes the epidemiologic and clinical features of KD in the Pediatric Rheumatology Department service in a general hospital. 120 patients with the diagnosis of KD between 1990 and 2009 were enrolled. We investigated the epidemiologic and clinical features of coronary artery involvement of the patients. Frequency of many parameters including age, sex, season, clinical and laboratory findings, response to treatment, and complications of the patients determined. During the 20-year study period, 120 patients <15 years of age were admitted for KD. Among them, 39.2% were at the extremes of the age spectrum, with 2.5% <6 months and 36.7% >5 years of age, male to female ratio of 1.3:1 and the classic KD to incomplete KD ratio of 3.1:1. KD recurred in 5% of all cases. It occurred most frequently in the winter and least frequently in the summer. The occurrence of coronary artery abnormalities (CAA) was 4.2%. Kawasaki disease should be considered in any pediatric patients with a prolonged refractory febrile illness in order to prevent CAA formation.

scholarly journals Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Chloe Kupelian ◽  
Bindu Sathi ◽  
Deepika Singh
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Cervical Lymphadenopathy ◽  
Clinical Findings ◽  
Normocytic Anemia ◽  
Coronary Aneurysms

Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans’ syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings.

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