Abdominal lymphadenopathy in an adolescent with Kawasaki disease: a major sign?

Author(s):  
Danielle Bulkool ◽  
Ana Vidal de Carvalho ◽  
Aurea Grippa ◽  
Marcia Fernandes ◽  
Israel Figueiredo

Abstract In adolescence, Kawasaki disease (KD) may present with varied clinical pictures, and the classic clinical criteria are often insufficient to establish the diagnosis. This report presents a clinical case of KD in an adolescent and a brief review of the relevant literature, based on a PubMed search using the terms “adolescence” and “KD”. A 13-year-old girl presented with fever, rash, hepatomegaly, and ultrasonographic evidence of abdominal lymphadenopathy. On week 2 after onset of symptoms, the patient developed desquamation of the extremities and thrombocytosis. Echocardiography was normal during the acute illness and remained unchanged at 2-year follow-up. Clinicians should be aware that, in adolescents, KD poses a diagnostic challenge and rarely corresponds to the classic presentation. On retrospective analysis, we believe that abdominal lymphadenopathy should have been recognized as a substitute manifestation of the classic symptom of cervical lymphadenopathy to establish the diagnosis of this fearsome systemic vasculitis syndrome.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
yeo hyang kim ◽  
Chae Ok Shin ◽  
Myung Chul Hyun ◽  
Dong Seok Lee

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.


2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Nathan Jamieson ◽  
Davinder Singh-Grewal

Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices.Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles.Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual’s exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.


2020 ◽  
Vol 60 (5) ◽  
pp. 283-6
Author(s):  
Yudha Fadhol Arafah ◽  
Sasmito Nugroho ◽  
Noormanto Noormanto ◽  
Nadya Arafuri ◽  
Indah Kartika Murni

Kawasaki disease (KD), or mucocutaneous syndrome, is an acute, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients younger than five years.1  KD is the leading cause of childhood acquired heart disease in the developed world.2 The incidence in those aged under 5 years varies widely throughout the world, accounting for 8.4 per 100,000 in the UK, 17.5 to 20.8 per 100,000 in the USA, and 239.6 per 100,000 in Japan.2 The diagnosis of classic KD is based on the simultaneous presence of high fever for 5 or more days with at least four of five other symptoms (bilateral conjunctival hyperemia, ulcerations of the lips and inflammation of the oral cavity, polymorphous rash, edema and desquamation of the extremities, and cervical lymphadenopathy), or fever associated with less than 4 of the diagnostic criteria and echocardiographic abnormalities of the coronary arteries.3


2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tracey Dyer ◽  
Paul Dancey ◽  
John Martin ◽  
Suryakant Shah

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.


2015 ◽  
Vol 19 (4) ◽  
pp. 358-360 ◽  
Author(s):  
Dominik Alex Nowak ◽  
Jensen Yeung

Background: Steroid-induced osteonecrosis is unpredictable and highly morbid. Methods: MEDLINE (Ovid) and PubMed search for relevant literature. Results: Oral steroid doses typically considered to be associated with the condition are >20 mg of prednisone (or equivalent) per day or >2 g within a period of 2 to 3 months. Symptoms may be vague. Conclusions: Dermatologists must exercise a high degree of clinical suspicion and warn patients about steroid-related risks and conditions for follow-up.


2020 ◽  
Vol 8 (B) ◽  
pp. 99-103
Author(s):  
Nguyen Huu Son ◽  
Tran Kiem Hao ◽  
Nguyen Thi Hoang Anh

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.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
ITZEL E RIOS-OLIVARES ◽  
LUIS M GARRIDO-GARCIA

Background: Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Cardiac sequelae, such as coronary artery aneurysms (CAA), are one of the most important aspects of this disease. Actually KD is most frequently presented in children younger than 5-years old. Objective: To describe the clinical and laboratory features, cardiac sequelae and outcome in children older than 10-years old with KD who were attended at the Instituto Nacional de Pediatría in Mexico City. Methods: An observational, descriptive, retrospective and transversal case study. We reviewed the medical records of patients older than 10-years diagnosed with KD from August 1995 to May 2014, and analyzed gender, age, clinical manifestations, hemoglobin, leucocyte count, platelet count, ESR, CRP, albumin, sodium, potassium, AST, ASL, time from the onset of the symptoms to diagnosis, treatment used, the development of CAA and outcome in the acute phase of the disease. Results: We studied 18 cases of KD in patients older than 10-years old, 72.2% (13 of 18) were male with a mean age of 154 months (range 120 to 200). The time from the onset of the fever to diagnosis was 10.6 ± 5.8 days, (range 3 to 21 days). Skin lesions were the most common manifestation of KD and cervical lymphadenopathy was the least common clinical feature. 2 patients presented with KD shock syndrome. Complete KD was diagnosed in only 50% (9 of 18) of our cases. 16 patients received IVGG, 2 patients required a second GGIV dose and 10 patients also received steroids. 6 of 18 patients (30%) developed CAA. There were no deaths in our group. Conclusions: KD in patients older than 10-years old represent a clinical challenge because in the majority of the cases they presented with an atypical clinical picture which contribute to a delayed diagnosis. Also there is an increased risk of developing cardiac complications and CAA in this group of patients.


2020 ◽  
Author(s):  
Yusuf Ziya Varlı ◽  
Kazim Oztarhan

Abstract Background: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. This study aimed to determine the clinical characteristics, demographic features, frequency of coronary involvement, and resistance to intravenous immunoglobulin (IVIG) treatment in Turkey based on our data.Methods: Patients with KD were evaluated with demographic data, clinical, laboratory, and echocardiographic findings. Results: Between 2010–2019, a total of 259 patients (male/female: 1.67) were treated in our hospital, with 48 (%19) cases < 1 year of age. According to diagnostic criteria, 31% were diagnosed with typical KD and 69% with atypical (incomplete) KD. The frequency of clinical findings were as follows: changes in the lips and oral mucosa (79%); polymorphic rash (69%); conjunctivitis (65%); changes in the extremities (54%); and cervical lymphadenopathy (48%). There was no significant difference between typical and atypical KD in the frequency order. CAA development and IVIG resistance occurred in 11.6% and 12.3% of cases, respectively. IVIG resistance was more common in infants and hospitalization times were longer in this group. Coronary artery lesions existed in 45 patients; right coronary artery (RCA) alone (20%), left coronary artery (LCA) alone (44.5%), and RCA and LCA together were involved (35.5%). The left main coronary artery affected 20 patients, the left anterior descending artery (LAD) affected nine patients (45%), the left circumflex artery (LCx) affected two patients (10%), and the LAD and LCx together affected two patients (10%). None of the patients had myocardial infarctions or died during follow-up. Conclusion: KD is a systemic vasculitis common in pediatric infants in which coronary artery involvement affects prognosis. Due to IVIG resistance and increased coronary involvement accompanying this vasculitis, it is an important problem in countries where the disease is common. It is important to know the factors that increase the risk of coronary involvement and IVIG resistance development.


1991 ◽  
Vol 1 (3) ◽  
pp. 234-239 ◽  
Author(s):  
Elfriede Pahl ◽  
William H. Neches ◽  
José A. Ettedgui

SummarySummary Mucocutaneous lymph node syndrome, now called Kawasaki disease, affects children of all ages and races. The cause of this systemic vasculitis is unknown, thus the diagnosis is based solely on clinical criteria. Coronary arteritis and the formation of coronary arterial aneurysms are the most serious early cardiac complications, while long term sequels, such as coronary stenosis and myocardial infarction, may occur. Current medical therapy is aimed solely at preventing or decreasing the occurrence of these cardiovascular complications in patients with this disease. Past treatment included antibiotics, steroids, and nonsteroidal anti-inflammatory agents. Aspirin remains the most widely used drug, although the use of intravenous gammaglobulin has also become common. When used in combination, these two agents may reduce the incidence of coronary arterial aneurysms. The optimal dosage and duration of treatment has not yet been determined.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yifan Ren ◽  
Chuanxin Zhang ◽  
Xiaoqin Xu ◽  
Yu Yin

Abstract Background Kawasaki disease (KD) is the most common cause of acquired heart disease among children in developed countries, in which the resulting coronary artery (CA) abnormalities cause myocardial ischemia, infarction, and death. Prompt diagnosis was essential, and supplemental information should be used to assist the diagnosis when classical clinical criteria are incomplete. The elevated levels of serum transaminases in most KD patients are mild. Herein, a case of atypical KD child with severely elevated transaminase was reported. Case presentation A child with clinical manifestations of fever, high C-reactive protein (CRP) and severely elevated transaminases was reported. The treatment effect of antibiotic and liver-protecting drugs was not satisfactory. A bilateral diffuse dilation of the CA was detected on echocardiography on day 5 of the illness; thus, atypical KD was diagnosed. Elevated transaminases declined rapidly to normal after the treatment of intravenous immunoglobulin (IVIG). A 1-month follow-up revealed that CA returned to normal, and 2-month, 6-months, and 1-year follow-up revealed the child was in good general health. Conclusions This case highlighted that atypical KD clinical symptoms were diverse, and severely elevated transaminases might provide a clue to healthcare providers for the diagnosis and management of atypical KD.


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