scholarly journals Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Krishan Pratap ◽  
Logan S. Gardner ◽  
David Gillis ◽  
Martin Newman ◽  
Dana Wainwright ◽  
...  

Abstract Background Kawasaki Disease (KD) is the most common paediatric vasculitis affecting small to medium arteries. Although the average age of diagnosis is 3.4 years with a well-defined clinical presentation, older patients with KD including adolescent and adult patients demonstrate a less classical presentation with prominent findings including hepatitis, cervical lymphadenopathy, and arthralgia. We describe a case of an adolescent presentation of Kawasaki Disease presenting with a predominantly cholestatic hepatic picture. Case presentation We describe a case of KD in a 16-year-old Caucasian female with predominately hepatic disease that showed resistance to intravenous immunoglobulin (IVIG). The formal diagnosis of KD was made on her 8th day of symptoms. She displayed classical symptoms commencing with fever, followed by peripheral desquamation, strawberry tongue, cervical lymphadenopathy. She became clinically jaundiced with evidence of hepatic artery narrowing on ultrasound that resolved with treatment. Her disease was biphasic and required further IVIG for non-hepatic symptoms. She did not develop coronary aneurysms. Conclusion Significant hepatic dysfunction with clinical jaundice is rare in KD without associated gall bladder hydrops and tends to occur in older patients. We describe such a case and review the five described cases in the literature. Diagnostic delay is more common in adolescent patients and given that the prognosis of KD is closely correlated to diagnostic timing and provision of care, it is important to consider Kawasaki Disease in older demographics especially with undiagnosed hepatic disease.

Author(s):  
Itai Ghersin ◽  
Lior H. Katz

Abstract Background Gastric cancer occurs mainly in older patients, with a peak incidence over 60 years of age. It is relatively rare among younger individuals. However, the frequency of gastric cancer in young patients appears to be on the rise worldwide. Case presentation We report the case of a 19-year-old female soldier who, after a considerable diagnostic delay, was diagnosed with gastric adenocarcinoma. She is one of the youngest gastric adenocarcinoma patients ever reported in Israel. Conclusion This case should serve as a reminder that gastric carcinoma is a possible diagnosis even in young patients. It also highlights the critical importance of obtaining a thorough medical history in the process of clinical decision making.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Chloe Kupelian ◽  
Bindu Sathi ◽  
Deepika Singh

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.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Houda Boudiaf ◽  
Moussa Achir

Background: Kawasaki disease (KD) is an acute multisystem vasculitis of unknown etiology that occurs predominantly in infants and young children. The incidence of KD is increasing worldwide ; however the epidemiological data available for Algerian patients remains insufficient Objective: To describe the demographic, clinical, and laboratory features of Algerian children with KD and to highlight the practical difficulties. Methods: This retrospective study included children admitted with Kawasaki disease at the only pediatric tertiary referral hospital in Algiers over a period of 8 years from January 2006 to December 2013. Results : 108 patients with KD, with a mean age of 31 months (range: 8-84 months)were identified. There were 63 boys and 45 girls (sex ratio: 1.4). The clinical data were similar to previously described studies, with some difference: the cervical lymphadenopathy was less frequent :25%(27/108). 22%( 24/108) of children had evidence of cardiac complications: 20%( 22/108) had coronary artery abnormalities ; one child had mitral regurgitation, one had pericardial effusion. Of the 22 children with coronary abnormalities , 9 had coronary dilatation, 13 had coronary aneurysms (10 small and medium, 3 giant). These abnormalities regressed in 13 cases on follow up. During this period, catheter and surgical coronary intervention were performed to treat coronary ischemia in one patient, two years after onset. The only independent variable for prediction of coronary involvement was fever duration at the time of initial presentation(p=0.016). The therapeutic used in this study included an immunoglobulin treatment for only 63%(69/108) of the children, half of which received it within the first ten days of the onset of the disease. Conclusion: This work demonstrates the necessity of a registry that will allow better appreciation of the incidence of this disease and improve the diagnosis and treatment of KD in Algeria


1988 ◽  
Vol 9 (7) ◽  
pp. 209-217
Author(s):  
Hirohisa Kato ◽  
Osamu Inoue ◽  
Teiji Akagi

Kawasaki disease or mucocutaneous lymph node syndrome is an acute febrile illness recognized most often in children younger than 4 years of age. It is characterized by mucosal inflammation, indurative edema of the hands and feet, skin rash, and cervical lymphadenopathy. The diagnostic criteria prepared by the Kawasaki disease research committee supported by the Ministry of Health and Welfare of the Japanese government are shown in Table 1. These criteria make up the characteristic symptoms and laboratory findings of the disease. It was first described as a clinical entity in Japan in 1967, by Dr Tomisaku Kawasaki (Fig 1). Kawasaki disease has been most prevalent in Japan; however, it has now been recognized in the United States and other countries. Kawasaki disease has aroused intense interest because of the catastrophic sequelae: coronary arteritis accompanied by coronary aneurysms and thrombotic occlusion which may lead to sudden death or ischemic heart disease. In the early days, the prognosis for individuals with Kawasaki disease was considered to be good. However, the first nationwide survey in Japan in 1971 clarified that 1.7% of the patients had died from acute heart failure or myocardial infarction, and all autopsies had shown coronary arteritis accompanied by aneurysms and thrombotic occlusion.


2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Tsukasa Tanaka ◽  
Masaki Shimizu ◽  
Oshi Tokuda ◽  
Hiroko Yamamoto ◽  
Natsuki Matsunoshita ◽  
...  

Background. Kawasaki disease (KD) is typically characterized by fever, oral cavity erythematous changes, bilateral bulbar conjunctival injection, skin rash, erythema and edema of the hands and feet, and cervical lymphadenopathy. Some atypical patients with KD initially develop cervical and pharyngeal cellulitis; however, an initial presentation with inguinal cellulitis is extremely rare. In addition, to our knowledge, no report has documented the cytokine profile in a KD patient with cellulitis. Case presentation. A previously healthy 8-year-old Japanese girl was hospitalized following a 2-day history of fever and a 5-day history of pain and erythema in the left inguinal region. She was diagnosed with bacterial inguinal cellulitis and was administered antibiotics. The next day, a polymorphous rash emerged on her trunk. After 3 days of antibiotics, however, her fever continued and the cellulitis had spread over the entire lower abdomen. Simultaneously, the bilateral bulbar conjunctival injection without exudate became more prominent and her lips became erythematous. In addition, erythematous changes on her palms appeared a few hours later, which led to the diagnosis of KD. Since she had a high risk score that predicted no response to initial intravenous immunoglobulin (IVIG) at the initiation of treatment, she was treated with IVIG, intravenous prednisolone (PSL), and oral aspirin. The KD symptoms improved the next day, but the cellulitis did not completely resolve until 2 months after discharge. The patient’s serum cytokine profile at admission had an IL-6 dominant pattern which was consistent with that of patients with KD despite her initial lack of KD symptoms, and the pattern observed at admission was sustained until IVIG and PSL administration. Conclusion. KD should be included in the differential diagnosis for patients presenting with inguinal cellulitis who are unresponsive to initial empiric antibiotics.


Author(s):  
Chiara Isidori ◽  
Lisa Sebastiani ◽  
Susanna Esposito

Background: Kawasaki disease (KD) is a childhood acute febrile vasculitis of unknown aetiology. The diagnosis is based on clinical criteria, including unilateral cervical lymphadenopathy, which is the only presenting symptom associated with fever in 12% of cases. A prompt differential diagnosis distinguishing KD from infective lymphadenitis is therefore necessary to avoid incorrect and delayed diagnosis and the risk of cardiovascular sequelae. Case presentation: We describe the case of a 4 years old boy presenting with febrile right cervical lymphadenopathy, in which the unresponsiveness to broad-spectrum antibiotics, the following onset of other characteristic clinical features and the evidence on the magnetic resonance imaging (MRI) of retropharyngeal inflammation led to the diagnosis of incomplete and atypical KD. On day 8 of hospitalisation (i.e., 13 days after the onset of symptoms), one dose of intravenous immunoglobulins (IVIG; 2 g/kg) was administered with rapid defervescence, and acetylsalicylic acid (4 mg/kg/day) was started and continued at home for a total of 8 weeks. Laboratory examinations revealed a reduction in the white blood cell count and the levels of inflammatory markers, thrombocytosis, and persistently negative echocardiography. Clinically, we observed a gradual reduction of the right-side neck swelling. Fifteen days after discharge, the MRI of the neck showed a regression of the laterocervical lymphadenopathy and a resolution of the infiltration of the parapharyngeal and retropharyngeal spaces. Conclusion: Head and neck manifestations can be early presentations of KD, which is frequently misdiagnosed as suppurative lymphadenitis or retropharyngeal infection. A growing awareness of the several possible presentations of KD is therefore necessary. Computed tomography (CT) or MRI can be utilised to facilitate the diagnosis.


2020 ◽  
Author(s):  
Mohammed Nashawi ◽  
Friedrich Reichert ◽  
Friederike Blankenburg ◽  
Anita Heinkele ◽  
Christian Stirnkorb ◽  
...  

Abstract Background: Kawasaki disease (KD) is a systemic vasculitis which affects medium-sized arteries. Although diagnostic and classification criteria exist, differentiation from other diseases can be difficult.Case Presentation: We present a 3-year old patient with a diagnosis of complete KD with positivity for urine-PCR on leptospirosis. In our patient conjunctivitis, rash, fever, cervical lymphadenopathy, palmar swelling, exanthema were seen, indicating all criteria for complete KD. After 2 weeks periungual desquamation developed. Besides this patient two further cases were retrieved form the literature with similar clinical courses indicating leptospirosis associated KD.Conclusion: These cases indicate that leptospirosis may not only mimic but also be associated with KD shown by specific findings like desquamation of fingers, toes and oropharyngeal mucous membrane changes. To distinguish between KD, leptospirosis and leptospirosis associated KD a proper history taking including travel history and contact to animals is fundamental as well as thorough clinical and cardiac examinations in the course of the disease. While diagnostic procedures and observation might take time, treatment of acute KD should not be delayed.


2019 ◽  
Vol 105 (10) ◽  
pp. 1004-1006 ◽  
Author(s):  
Audrey Dionne ◽  
David Burgner ◽  
Sarah De Ferranti ◽  
Davinder Singh-Grewal ◽  
Jane Newburger ◽  
...  

Intravenous immunoglobulin (IVIG) reduces coronary aneurysms in patients with Kawasaki disease (KD), but additional management options remain challenging, with no generalisable evidence-based recommendations. We performed a survey of 724 physicians from 73 countries to assess variation in practice. IVIG was the preferred initial treatment by 659 (91%) of respondents. Criteria for adjunctive primary treatment varied considerably and definitions of IVIG resistance varied markedly by geographical continent, Human Development Index tiers and medical specialty. A second dose of IVIG was used most often for patients with coronary aneurysm non-responsive to initial treatment (572, 79%), but corticosteroids (379, 52%) and tumour necrosis factor alpha inhibitors (208, 29%) were also frequently used. Our findings highlight the need for international collaborative efforts to optimise management of patients with KD worldwide.


2003 ◽  
Vol 13 (1) ◽  
pp. 103-105 ◽  
Author(s):  
Kenji Suda ◽  
Masahiko Matsumura ◽  
Shigeru Ohta

An 8-month-old boy presented with right hemiplegia of sudden onset after 20 days of Kawasaki disease, which was not initially treated by gamma globulin. Cranial X-ray computed tomography confirmed cerebral infarction as the cause of the right hemiplegia. In subsequent weeks, he developed multiple thromboses in coronary aneurysms. He successfully underwent intracoronary thrombolysis using tissue plasminogen activator without haemorrhagic complications. Cerebral infarction as a complication of Kawasaki disease is rare, and is a difficult clinical situation to manage.


1991 ◽  
Vol 1 (3) ◽  
pp. 254-255
Author(s):  
Jane W. Newburger

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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