scholarly journals Incomplete Kawasaki disease - a diagnostic and therapeutic challenge

2014 ◽  
Vol 9 (4) ◽  
pp. 30-35
Author(s):  
S Datta ◽  
S Maiti ◽  
G Das ◽  
A Chatterjee ◽  
P Ghosh

Background The diagnosis of classical Kawasaki Disease was based on clinical criteria. The conventional criteria is particularly useful in preventing over diagnosis, but at the same time it may result in failure to recognize the incomplete form of Kawasaki Disease. Objective To suspect incomplete Kawasaki Disease, because early diagnosis and proper treatment may reduce substantial risk of developing coronary artery abnormality which is one of the leading causes of acquired heart disease in children. Method Nine cases of incomplete Kawasaki Disease were diagnosed over a period of one year. The diagnosis of incomplete Kawasaki Disease was based on fever for five days with less than four classical clinical features and cardiac abnormality detected by 2D- echocardiography. A repeat echocardiography was done after 6 weeks of onset of illness. The patients were treated with Intravenous Immunoglobulin and/or aspirin. Result The mean age of the patients was 3.83 years and the mean duration of symptoms before diagnosis was 12.1 days. Apart from other criteria all of our patients had edema and extreme irritability. All the patients had abnormal echocardiographic finding. Five patients received only aspirin due to nonaffordability of Intravenous Immunoglobulin and four patients received both aspirin and Intravenous Immunoglobulin, but the outcome was excellent in all the cases. Conclusion Incomplete Kawasaki Disease can be diagnosed with more awareness and aspirin alone may be used as a second line therapy in case of non affordability of Intravenous Immunoglobulin. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 30-35 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10234

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.


2013 ◽  
Vol 35 ◽  
pp. 505-511 ◽  
Author(s):  
Hwa Jin Cho ◽  
Young Earl Choi ◽  
Eun Song Song ◽  
Young Kuk Cho ◽  
Jae Sook Ma

Incomplete Kawasaki disease (iKD) is considered to be a less complete form of Kawasaki disease (cKD), and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group). Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study. We obtained four measurements of serum procalcitonin levels and those of other inflammatory markers from each patient. Samples were taken for analysis on the day of diagnosis (thus before treatment commenced; D0) and 2 (D2), 14 (D14), and 56 days (D56) after intravenous immunoglobulin infusion. We obtained control group data at D0. The mean D0 serum procalcitonin levels of cKD patients (0.71±1.36 ng/mL) and controls (0.67±1.06 ng/mL) were significantly higher than those of iKD patients (0.26±0.26 ng/mL) (P=0.014andP=0.041, resp.). No significant difference in mean procalcitonin level was evident among groups at any subsequent time. In conclusion, the serum procalcitonin level of patients with acute-stage cKD was significantly higher than that of iKD patients.


CNS Spectrums ◽  
2006 ◽  
Vol 11 (7) ◽  
pp. 501-508 ◽  
Author(s):  
Joseph Jankovic ◽  
Kevin Dat Vuong ◽  
Madhavi Thomas

ABSTRACTIntroduction: Psychogenic disorders, also referred to as somatoform, conversion, somatization, hysteria, and medically unexplained symptoms, are among the most challenging disorders to diagnose and treat. Psychogenic movement disorders are increasingly encountered in specialized clinics, and represent ~15% of all patients evaluated in the Baylor College of Medicine Movement Disorders Clinic.Objective: To characterize psychogenic tremor and provide data on prognosis and long-term outcome in a large group of patients with psychogenic tremor followed in a movement disorders clinic.Methods: Patients evaluated at the Baylor College of Medicine Movement Disorders Clinic in Houston, Texas, between 1990 and 2003 with the diagnosis of psychogenic movement disorder (PMD), who consented to be interviewed, were administered a structured questionnaire designed to assess current motor and psychological function.Results: psychogenic tremor is the most common PMD, accounting for 4.1 % of all patients evaluated in our clinic. We were able to obtain clinical information on a total of 228 of 517 (44.1%) patients with PMD, followed for a mean of 3.4±2.8 years. Among the 127 patients diagnosed with psychogenic tremor, 92 (72.4%) were female, the mean age at initial evaluation was 43.7±14.1 years, and the mean duration of symptoms was 4.6±7.6 years. The following clinical features were considered to be characteristic of psychogenic tremor: abrupt onset (78.7%), distractibility (72.4%), variable amplitude and frequency (62.2%), intermittent occurrence (35.4%), inconsistent movement (29.9%), and variable direction (17.3%). Assessment of long-term outcome showed that 56.6% of patients reported improvement in their tremor. Factors predictable of a favorable outcome were elimination of stressors and patient's perception of effective treatment by the physician.Conclusion: This largest longitudinal study of patients with psychogenic tremor provides data on the clinical characteristics and natural history of this most common PMD. The accurate diagnosis of psychogenic tremor is based not only on exclusion of other causes but is also dependent on positive clinical criteria, the presence of which should avoid unnecessary investigation. The prognosis of psychogenic tremor may be improved with appropriate behavioral and pharmacologic management.


Author(s):  
Maria Hörl ◽  
Holger Michel ◽  
Stephan Döring ◽  
Markus-Johann Dechant ◽  
Florian Zeman ◽  
...  

Abstract Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. Early diagnosis and treatment is mandatory in incomplete KD to reduce the risk of coronary involvement. Between 2002 and 2018, 124 patients have been diagnosed suffering from KD at the University Children’s Hospital Regensburg (KUNO). We assessed luminal diameters of both CAs normalized as Z-scores by 2D-echocardiography. A total of 94 patients were analyzed. Of them, 31 (33%) were affected by an incomplete form of KD. In 24 children (26%), serial echocardiography was necessary in order to confirm diagnosis. Mean Z-scores for the left main coronary artery (LMCA), right main coronary artery (RMCA), and left anterior descending artery increased significantly between the initial (LMCA 0.79z, RMCA 0.15z, LAD 0.49z) and second (LMCA 1.69z, RMCA 0.99z, LAD 1.69z) examination (p < 0.05). Conclusion:To confirm diagnosis of KD, it might not be necessary to detect dilation or aneurysms. Our observation suggests that patients suspected having KD should be monitored with serial echocardiography in order to detect a possible enlargement of the CA diameters, even if Z-scores are within the normal range. What is Known:• Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications.• Due to less classic clinical criteria in patients with incomplete KD, the risk for CA pathology is even higher. What is New:• A significant progression of patients’ CA Z-scores in serial echocardiographic measurements may be helpful to ensure diagnosis of KD early even if Z-scores are within the normal range.• Twenty-seven patients (90%) with incomplete KD could be diagnosed within 10 days of fever, early enough to prevent significantly higher rates of CA aneurysm.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Carles Bautista ◽  
Joan Sanchez-de-Toledo ◽  
Clark C Bradley ◽  
Jetrho Herberg ◽  
Fanny Bajolle ◽  
...  

Introduction: Following the SARS-CoV-2 pandemic peak, children suffering from a multiorgan inflammatory disease that often leads to shock have been reported. This condition shares features with Kawasaki disease, but its etiopathogenesis is unknown. Hypothesis: We aimed to describe presentation and hospital course for this pediatric inflammatory multisystemic syndrome associated with COVID-19 (PIMS-TS). Methods: Data were collected from a retrospective review of children from 33 participating European, Asian and American sites. Results: We included 183 patients (109 males, 59·6%) with PIMS-TS, at a mean age of 7·0 (±4·7) years. Fifty-six (30·6%) had black ethnicity and obesity was present in 48 (26·2%) cases. Overall, 114/183 (62·3%) had biological evidence of current or recent SARS-CoV-2 infection. At admission, all presented with fever, 117/183 (63·9%) with gastrointestinal symptoms and 79/183 (43·2%) with shock, that was associated with more frequent black ethnicity, higher inflammatory markers and more cardiac involvement. Twenty-seven patients (14·7%) fulfilled criteria for Kawasaki disease. They were younger with no shock, fewer gastrointestinal, cardio-respiratory and neurological symptoms. Among the remaining PIMS-TS patients, 77 (49·3%) had mainly fever and inflammation with less cardiac involvement. For the entire cohort of 183 patients, the mean duration of admission was 8·6 (±5·6) days. Inotropic support, mechanical ventilation and ECMO were indicated in 72 (39·3%), 43 (23·5%) and 4 (2·2%) patients, respectively. Three patients (1·6%) died. A shorter duration of symptoms before admission was a risk factor for worse outcome and for ECMO/death, with 63% increased risk per day reduction (95%CI 0·39-0·93, p=0·03) and with 51·4% increased risk per day reduction (95%CI 0·36-0·9, p=0·03), respectively. Conclusions: We describe the first largest international series of children with PIMS-TS. Life-threatening shock is a common presentation. A shorter duration of symptoms prior to admission characterizes the fulminant form of the disease with potentially worse outcome.


Author(s):  
Michael J. Ellis ◽  
Lesley J. Ritchie ◽  
Patrick J. McDonald ◽  
Dean Cordingley ◽  
Karen Reimer ◽  
...  

AbstractObjectives: To summarize the clinical characteristics and outcomes of pediatric sports-related concussion (SRC) patients who were evaluated and managed at a multidisciplinary pediatric concussion program and examine the healthcare resources and personnel required to meet the needs of this patient population. Methods: We conducted a retrospective review of all pediatric SRC patients referred to the Pan Am Concussion Program from September 1st, 2013 to May 25th, 2015. Initial assessments and diagnoses were carried out by a single neurosurgeon. Return-to-Play decision-making was carried out by the multidisciplinary team. Results: 604 patients, including 423 pediatric SRC patients were evaluated at the Pan Am Concussion Program during the study period. The mean age of study patients was 14.30 years (SD: 2.32, range 7-19 years); 252 (59.57%) were males. Hockey (182; 43.03%) and soccer (60; 14.18%) were the most commonly played sports at the time of injury. Overall, 294 (69.50%) of SRC patients met the clinical criteria for concussion recovery, while 75 (17.73%) were lost to follow-up, and 53 (12.53%) remained in active treatment at the end of the study period. The median duration of symptoms among the 261 acute SRC patients with complete follow-up was 23 days (IQR: 15, 36). Overall, 25.30% of pediatric SRC patients underwent at least one diagnostic imaging test and 32.62% received referral to another member of our multidisciplinary clinical team. Conclusion: Comprehensive care of pediatric SRC patients requires access to appropriate diagnostic resources and the multidisciplinary collaboration of experts with national and provincially-recognized training in TBI.


2019 ◽  
Vol 36 (2) ◽  
Author(s):  
Seema Sakina ◽  
Syeda Sobya Owais ◽  
Ejaz Ahmed Khan ◽  
Abdul Malik Sheikh

Objective: To describe the demographics; clinical, laboratory, echocardiographic findings; treatment and outcome in a cohort of children with Kawasaki disease in a tertiary care hospital. Methods: This is a descriptive, observational, retrospective cohort study conducted at Shifa International Hospital, Islamabad, from January 2013-June 2019. Children who met the criteria for Kawasaki disease according to the American Heart Association and American Academy of Pediatrics guidelines were included. Results: A total of 25 children who met the criteria of Kawasaki disease were included. Their mean age was 43 months (4-150 months). Majority (76%) were males. Eighteen (72%) had complete Kawasaki disease and 7 (28%) had incomplete Kawasaki disease. Fever (> five days) was present in 20 (80%) patients. Eight patients (32%) had echocardiographic changes, out of which two patients (25%) had complete Kawasaki disease and six patients (75%) had incomplete Kawasaki disease. Intravenous immunoglobulin was given to all patients. Fifteen children (60%) received intravenous immunoglobulin within 10 days of fever. None required a second dose. All patients received high dose aspirin at diagnosis which was reduced to antiplatelet dose after resolution of fever for ≥48hrs. Eighteen patients (72%) came for regular follow up. Follow up at 6 months showed complete resolution of echocardiographic changes in six patients (75%), 1 (12.5%) was lost to follow up and one (12.5%) child had persistent coronary artery dilatation. Conclusion: Complete KD was present in 72% of our cohort of children. Coronary artery abnormalities were present in one third of these children, at younger age and more common in those with incomplete KD but recovered in most. doi: https://doi.org/10.12669/pjms.36.2.910 How to cite this:Sakina S, Owais SS, Khan EA, Sheikh AM. Kawasaki disease: Clinico-laboratory spectrum & outcome in a cohort of children treated at a tertiary care hospital in Islamabad, Pakistan. Pak J Med Sci. 2020;36(2):---------. doi: https://doi.org/10.12669/pjms.36.2.910 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


2015 ◽  
Vol 96 (4) ◽  
pp. 619-623
Author(s):  
E F Lukushkina ◽  
V V Meshcheryakova ◽  
M G Afraymovich ◽  
T V Konopleva ◽  
T Yu Kostareva ◽  
...  

Kawasaki disease causes diagnostic difficulties due to insufficient knowledge of doctors, and therefore often goes under the «disguise» of other diseases and therefore is under-recognized in Russia so far. Laboratory tests reveal leukocytosis, increased erythrocyte sedimentation rate, hypochromic anemia and thrombocytosis, accompanied by hypercoagulation. Late diagnosis and treatment of Kawasaki disease increase the risk for thrombosis and coronary arteries aneurysms, myocardial infarction at a young age, death. The results of the original research targeted to analyze the clinical course and outcomes of Kawasaki disease in 15 infants treated at the department of children under one year of age of the Nizhny Novgorod Regional Pediatric Clinical Hospital during the last 6 years, are presented. The cardiovascular conditions included pericarditis in 4 patients, coronary arteritis in 2 patients, coronary arteritis with dilation of the coronary arteries entrance and thickening of the walls - in 4, myocarditis - in 1 patient, 1 patient had no signs of cardiovascular involvement. All patients without coronary artery aneurysms were treated with intravenous immunoglobulin (1-2 g/kg per course), acetylsalicylic acid (80-100 mg/kg daily) up to fever reduction, followed by 3-5 mg/kg per day up to the normal values of acute inflammation markers and platelet counts, and aggregation inhibitors. In 2 patients, Kawasaki disease led to death, thus in both cases the diagnosis and, consequently, treatment initiation were late. Early diagnosis and rational treatment of Kawasaki disease (high doses of intravenous immunoglobulin in combination with acetylsalicylic acid) is the key to a favorable outcome. Fever of unknown origin, which lasts 7 days or more in a child of an early age, is an absolute indication for echocardiography with mandatory visualization of the coronary arteries. Tactics of following up patients, who underwent Kawasaki disease, is determined by the degree of coronary lesions and the risk of myocardial ischemia.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Elif Erdem ◽  
Emine Kocabas ◽  
Hande Taylan Sekeroglu ◽  
Özlem Özgür ◽  
Meltem Yagmur ◽  
...  

A 7-year-old girl had presented with high body temperature and joint pain which continued for 3 days. Because of the prolonged history of unexplained fever, rash, bilateral nonpurulent conjunctival injection, oropharyngeal erythema, strawberry tongue, and extreme of age, incomplete Kawasaki disease was considered and started on an intravenous immunoglobulin infusion. Six days after this treatment, patient was referred to eye clinic with decreased vision and photophobia. Visual acuity was reduced to 20/40 in both eyes. Slit-lamp examination revealed bilateral diffuse corneal punctate epitheliopathy and anterior stromal haze. Corneal epitheliopathy seemed like crystal deposits. One day after presentation, mild anterior uveitis was added to clinical picture. All ocular findings disappeared in one week with topical steroid and unpreserved artificial tear drops. We present a case who was diagnosed as incomplete Kawasaki disease along with bilateral diffuse crystalline-like keratopathy. We supposed that unusual ocular presentation may be associated with intravenous immunoglobulin treatment.


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