Early diagnosis of Kawasaki disease in patients with cervical lymphadenopathy

Kawasaki disease is an acute vasculitis of unknown etiology that occurs predominantly in infancy and early childhood. It is characterize by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy.1,2 Coronary arterial aneurysms, or ectasia, develop in approximately 15 to 25% of children with the disease, and may lead to myocardial infarction, sudden death, or chronic coronary arterial insufficiency.2–4

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Abstract 132: Methylprednisolone Pulse Therapy for Kawasaki Disease with Symptomatic Myocarditis

Circulation ◽

10.1161/circ.131.suppl_2.132 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

yeo hyang kim ◽

Chae Ok Shin ◽

Myung Chul Hyun ◽

Dong Seok Lee

Keyword(s):

Kawasaki Disease ◽

Systolic Function ◽

Systemic Vasculitis ◽

Cervical Lymphadenopathy ◽

Febrile Illness ◽

Pulse Therapy ◽

Left Ventricular ◽

Acute Stage ◽

Z Score ◽

Intravenous Methylprednisolone

Purpose: Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a systemic vasculitis, especially involving the coronary arteries. Although, sometimes, subclinical myocarditis is combined in KD, symptomatic myocarditis is extremely uncommon. We report a 7 year old boy who developed hypotension and decreased left ventricular systolic function (EF 40%) in the acute phase of KD. Case: A 7 year old boy (height 115 cm, body weight 20 kg) was admitted because of 2 days of persistent fever and left cervical lymphadenopathy (white blood cell count 17,870 /mm 3 , C reactive protein 23.6 mg/dL). Conjunctiva injection and lip redness developed on the 4th day of illness, and hypotension and tachycardia (SBP 59/DBP 29 mmHg, HR 153/bpm) were combined. The echocardiography revealed a decreased ejection fraction (EF) (40%) without chamber dilatation and normal coronary artery size (LM 1.9mm, z score=-1.3, RCA 2.3mm, z score=0.4). The level of N terminal pro BNP was 28,000 pg/mL. With a diagnosis of KD with myocarditis, he was initially treated with inotropics and intravenous immunoglobulin (2 g/kg). Without clinical improvement in spite of initial treatment, A change of coronary arterial size (LM 2.9mm, z score=1.2, RCA 3.1mm, z score=2.3) was developed and decreased LV systolic function (EF 45%) and fever were persisted. Then, he was given 3 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. He showed prompt clinical recovery after pulse therapy of intravenous methylprednisolone (SBP 95/DBP 49 mmHg, HR 98/bpm). Although EF was improved (59%), coronary arterial dilatation was progressed (LM 3.4mm, z score=2.4 RCA 5.5mm, z score=7.9). Conclusions: The present case serves to highlight the fact that methylprednisolone should be considered as the priority in children with KD who have symptomatic myocarditis during the acute stage.

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Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽

10.1542/peds.93.3.525 ◽

1994 ◽

Vol 93 (3) ◽

pp. 525-528

Author(s):

Julie Kim Stamos ◽

Kathleen Corydon ◽

James Donaldson ◽

Stanford T. Shulman

Keyword(s):

Kawasaki Disease ◽

Cervical Lymphadenopathy ◽

Febrile Illness ◽

The United States ◽

Acute Febrile Illness ◽

Conjunctival Injection ◽

Coronary Abnormalities ◽

Acquired Heart Disease ◽

Mucosal Changes ◽

Infants And Young Children

Kawasaki disease (KD) is an acute febrile illness primarily affecting infants and young children. Its importance relates to the fact that 20% to 25% of untreated patients develop coronary abnormalities that can lead to myocardial infarction or even to death.1 KD is a leading cause of acquired heart disease in children in many regions, including the United States.2 Because there are no specific diagnostic tests for KD, the diagnosis is established by the presence of fever and four of five criteria without other explanation for the illness: (1) nonexudative conjunctival injection; (2) oral mucosal changes; (3) changes of the peripheral extremities; (4) rash, primarily truncal; and (5) cervical lymphadenopathy.

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P061 Kawasaki disease: a case series from a tertiary Hospital in Kenya

Rheumatology ◽

10.1093/rheumatology/keab722.053 ◽

2021 ◽

Vol 60 (Supplement_5) ◽

Author(s):

C Njeru ◽

A Migowa

Keyword(s):

Kawasaki Disease ◽

Incidence Rate ◽

Low Dose ◽

Clinical Signs ◽

Case Series ◽

Cervical Lymphadenopathy ◽

Patient Characteristics ◽

Tertiary Hospital ◽

High Dose ◽

True Incidence

Abstract Background Kawasaki disease (kDa) is a childhood vasculitides that affects small and medium-sized arteries. It is self-limiting but when left untreated can cause coronary artery aneurysms in about 25% of children1. The diagnosis is clinical and is made with the criteria of fever for at least five days, and at least four out of five other clinical signs: bilateral non-exudative conjunctivitis, oral mucous membrane changes, peripheral extremity changes, polymorphous rash, and cervical lymphadenopathy. Incomplete kDa is diagnosed with fever for at least five days and at least 2–3 of the principal signs, with suggestive lab investigations and lack of an alternative diagnosis1. kDa is named after Dr Tomisaku Kawasaki who first described it in 1967 in Japan2. The highest incidence of kDa continues to be reported among Asian children, with an incidence rate of 264.8 per 100 000 population aged 0–4 years as per Japan’s latest nationwide survey3. In Africa, the true incidence is unknown but several case reports have been published. A 2016 paper by Gorrab et al.4 found that the annualized incidence rate of kDa in the Maghreb children living in Quebec (18.49/year/100 000 children under 5 years of age) was 4–12 times higher than reported in their countries of origin- Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively) suggesting a likelihood of under-diagnosis. This case series sought to highlight the characteristics, presentation, and management of patients diagnosed with kDa in a tertiary hospital in Kenya. Methods This was a retrospective cross-sectional study carried out by reviewing the charts of all the patients with a discharge diagnosis of kDa from January 2013 to December 2017 at the Aga Khan University Hospital, Nairobi. Their demographics, presentation, diagnostic work-up, and management are reported. Analysis was done by descriptive statistics using the Microsoft Excel 2016 Application. Results A total of 15 cases were identified and the patient characteristics and presentation are as tabulated below: In addition to elevated inflammatory markers (C reactive protein and/or Erythrocyte Sedimentation Rate), a significant number of the patients also had sterile pyuria (9/9), hypoalbuminemia (8/10), thrombocytosis (8/15), and anaemia (11/15). Nine out of eleven had negative blood cultures. Fourteen out of the fifteen patients had echocardiograms done during admission. Only one patient was found to have abnormal findings of bilaterally dilated coronaries arteries. Five patients had at least one documented repeat echocardiogram. Fourteen patients received Intravenous Immunoglobulin (IVIG), with 13 of them responding to treatment. No adverse effects were reported after treatment. One patient did not improve and needed a second dose of IVIG and intravenous methylprednisone (30 mg/kg). Fourteen patients received aspirin but dosing varied from high (80–90 mg/kg/day) to moderate (30–50 mg/kg/day) to low dose (3 mg/kg/day). One patient on high-dose aspirin was noted to have developed symptoms consistent with aspirin-induced bronchospasm and was changed to low dose. Conclusion This case series highlights the presence of kDa in the Kenyan pediatric population with patient characteristics similar to what is reported globally. Diagnosis was made after a mean of about 7 days, possibly due to low awareness of the disease among healthcare professionals. Management with IVIG in most cases was successful but more guidance is needed around the use of steroids and the dosing of aspirin.

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P062 Vasculitis in children: epidemiological, clinical and Para clinical profile in the oriental region of Morocco—about 33 cases

Rheumatology ◽

10.1093/rheumatology/keab722.054 ◽

2021 ◽

Vol 60 (Supplement_5) ◽

Author(s):

A Hamami ◽

A Babakhouya ◽

A El Ouali ◽

A Ghannam ◽

M Rkain ◽

...

Keyword(s):

Pericardial Effusion ◽

Kawasaki Disease ◽

Early Diagnosis ◽

Renal Insufficiency ◽

Clinical Profile ◽

Lower Limbs ◽

University Hospital ◽

Age Group ◽

Abdominal Symptoms ◽

Appropriate Treatment

Abstract Background Vasculitis in children constitute a heterogeneous group of pathologies considered as a rarity in our context and therefore deserves to be better known. Despite their individualization for more than forty years throughout the world, they continue to be a challenging condition both in terms of diagnosis and treatment. The objective of our study is to report the epidemiological profiles of vasculitis and to assess the clinical and Para clinical profile of our patients, in order to contribute to a better knowledge of these conditions in our context, whose early diagnosis allows the starting of an appropriate treatment and the improvement of the prognosis. Material and methods This is a retrospective study, in which we collected data of all children in whom the diagnosis of vasculitis was confirmed, and who were hospitalized in the pediatric department of the University Hospital Center (CHU) Mohamed VI of Oujda over a period of 3 years from July 2014 to July 2017. Results Thirty-three patients were selected; 25 boys (76%) and 8 girls (24%), including 16 patients with Kawasaki disease; 14 patients with Henoch-Schonlein purpura (HSP); 2 patients with Polyarteritis nodosa (PAN); and 1 patient with Takayasu. A male predominance was noted with a sex ratio of 3.16. HSP represented 42.42% of the cases, the most affected age group was between 5 and 9 years in 45% of cases. Purpura was the most frequent symptom (100%) with petechial appearance in 77% of cases and localized in the lower limbs in all cases (100%). Joints were affected in 13 cases (93%), abdominal symptoms were found in 81.82% of cases, with one case of melena and 3 cases of rectal bleeding. The renal symptoms were mainly biological and were dominated by urinary proteinuria (45%), microscopic hematuria in one case, macroscopic hematuria in 3 cases, hypertension in one case and nephrotic syndrome in one case. Five patients developed renal insufficiency, two children had moderate renal insufficiency and three had severe renal insufficiency. Kawasaki disease represented the majority of cases: 16 cases (48.48%), affecting children between 4 and 41 months of age with an average age of 22 months, with most affected age group was between 12 and 24 months in 55%. The appeal signs were multiple dominated by fever which represents 100% of cases, skin rash in 82%, ocular redness in 45%, enanthema in 45%, oedema of the extremities in 45%, and adenopathy in 6% (one case). The inflammatory syndrome was present in all patients. Cardiac Doppler ultrasound was performed in 14 patients. It was abnormal in 22% of cases. 11 patients received a course of IGIV (54.54%), at a dose of 2 g/Kg in a single 12-h infusion. Aspirin was administered in all patients. The evolution was favorable in 15 patients, only one case was complicated by giant aneurysms of both coronaries, which evolved to a slight decrease in size and disappearance of the pericardial effusion. Valvular abnormality was noted in one case and minimal pericardial effusion in one case. Conclusion This study confirmed that incidence of vasculitis in children in our context is average, this can be seen on the non-negligible number of cases (33 cases) collected over a period of 3 years. Early diagnosis of this condition key to allow an initiation of an appropriate treatment and improvement of the prognosis.

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

Circulation ◽

10.1161/circ.131.suppl_2.193 ◽

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.

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Kawasaki Disease Initially Presenting as Cervical Lymphadenopathy

JCR Journal of Clinical Rheumatology ◽

10.1097/rhu.0000000000001263 ◽

2019 ◽

Vol Publish Ahead of Print ◽

Author(s):

Anna Otani ◽

Kazuki Iio ◽

Kazuhiro Uda ◽

Takahiro Matsushima ◽

Hiroshi Sakakibara ◽

...

Keyword(s):

Kawasaki Disease ◽

Cervical Lymphadenopathy

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BP Neural Network Model for early Diagnosis of Kawasaki Disease

Advanced Materials Research ◽

10.4028/www.scientific.net/amr.468-471.723 ◽

2012 ◽

Vol 468-471 ◽

pp. 723-726 ◽

Cited By ~ 1

Author(s):

Jiang Huang ◽

Jian Feng Chen

Keyword(s):

Neural Network ◽

Kawasaki Disease ◽

Early Diagnosis ◽

Infectious Diseases ◽

Network Model ◽

Neural Network Model ◽

Bp Neural Network ◽

Clinical Symptoms ◽

Laboratory Data ◽

Bp Neural Network Model

In order to diagnose Kawasaki Disease during early phase, clinical symptoms (temperature, rash, conjunctival injection, erythema of thelips, and oral mucosal changes) and laboratory data (white blood cell, neutrophil, platelet, high sensitive c-reactive protein, and erythrocyte sedimentation rate) of 138 children with Kawasaki disease or infectious diseases were used to develop a BP neural network model. 90 random cases were trained using MATLAB software for setting up the BP neural network model. The other 48 cases were analyzed to predict Kawasaki disease using this model. Results showed that the predict accuracy in patients with Kawasaki disease and children with infectious diseases are 95.6% and 88%, respectively. Our result indicates that the BP neural network model is likely to provide an accurate test for early diagnosis of Kawasaki disease.

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