Case Of Kawasaki Disease Research Articles

Syndrome d’activation macrophagique et maladie de Kawasaki : à propos de quatre observations

Macrophage activation syndrome (MAS) is a rarely reported complication of Kawasaki disease (KD). It must be sought during KD with unusual clinical signs, such as enlargement of the liver or spleen, cytopenia (including thrombocytopenia), and elevated serum triglycerides. Here, we report four cases from a single center. The first is the description of a case of KD occurring in an 11-month-old child 15 days after the occurrence of infectious spondylitis. For the second, acute myocarditis heralded KD in a 5-year-old child. A 15-year-old boy had multiorgan failure with shock. The last case describes an infant with atypical KD. All had hepatosplenomegaly, thrombocytopenia, and increased serum ferritin. For all of them, hemophagocytosis was present on the bone marrow smears. The condition rapidly evolved favorably with intravenous immunoglobulins or steroids. The extensive microbiological work-up was unrevealing. None had any coronary sequelae after the episode. KD should be added to the list of inflammatory conditions that may be complicated by MAS that modifies the presentation mimicking toxic shock or infection-associated activation syndrome and makes the diagnosis difficult. This association does not appear to have a prognostic impact on the course of the disease in the literature, confirmed by the analysis of this small series.

An atypical presentation of Kawasaki disease with thrombocytosis at the onset of the illness

Kawasaki disease (KD) is a systemic acute vasculitis, characterized by fever, bilateral non-exudative conjunctivitis, redness of the tongue, lips and oral mucosa, changes in the extremities, cervical lymph node, and polymorphic exanthema. Administration of IVIG within the first 10days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. There is currently no laboratory test for diagnosing KD. Rather, diagnosis is performed with reference to established clinical criteria. Unfortunately, atypical manifestations of KD appear to be on the rise decreasing the likelihood of timely diagnosis and appropriate treatment. Herein, we report an unusual case of KD: a 6-year-old boy presented with fever with desquamation of the fingers and thrombocytosis.

Kawasaki disease in an infant: Diagnostic and therapeutic challenges at the University Teaching Hospital of Yaoundé, Cameroon

Introduction: Kawasaki disease (KD) is an acute multi-systemic vasculitis which represents the leading etiology of acquired heart disease in children in high-income countries. Its rarity in black Africans may lead to misdiagnosis, delayed management with resultant fatal coronary artery lesions. We discuss a case of KD diagnosed in an infant in Yaoundé, Cameroon. Case presentation: A 10-month-old male Cameroonian presented with irritability, a generalised cutaneous eruption, and a prolonged high-grade fever. Although initial diagnosis of meningitis was made, the emerging laboratory and typical clinical features suggestive of KD prompted a quick diagnostic review. His clinical condition improved on Aspirin and corticosteroids. Conclusion: Due to the risk of potential complications from KD and the management challenges akin to resource-limited settings, we highlight the need for a high index of suspicion by healthcare providers when faced with febrile children with mucocutaneous lesions.

Utility of post mortem MRI in definition of thrombus in aneurismatic coronary arteries due to incomplete Kawasaki Disease in infants

Abstract Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that primarily affects the coronary artery (CA) and presents in childhood. The characteristic coronary arterial lesion is an aneurysm, which may lead to thrombosis, dilatation, stenosis, and occlusion. Such an aneurysm is typically calcified and generally develops five or more years after the onset of acute KD. It becomes more noticeable after ten years. KD is sometimes difficult to diagnose because of the limited clinical features, especially in infants younger than 6 months old, where the clinical presentations often do not fulfill the diagnostic criteria for KD. We report a case of Incomplete Kawasaki Disease (IKD) causing unexpected death in infants. A seven-month-old male baby, apparently well nourished and without fever or exanthema that was unexpectedly found agonal in his bed by his parents. He died in an emergency room a few hours later in spite of aggressive resuscitation efforts. Postmortem Magnetic Resonance Images were obtained during the autopsy, with evidence of an occlusive thrombus in left and right coronary artery aneurysms. Laboratory findings were consistent with IKD. The crucial role of postmortem imaging is discussed here in order to improve diagnosis tools for preventable events.

A case of Kawasaki disease with autoimmune hemolytic anemia

A case of Kawasaki disease with autoimmune hemolytic anemia

The incomplete kawasaki disease in child three moths old with formation of aneurysms of coronary and peripheral arteries

The Kawasaki disease is a systemic vasculitis of unclear etiology extremely rare occurring in children of the first three months of life. The disease often takes course in incomplete and atypical form and brings high risk of development of such cardio-vascular diseases as aneurysms of coronary and peripheral arteries. The disease progresses in acute form with fever, catarrhal manifestations and results in convalescence and hence it is very often considered as infection pathology. Nowadays, there is no specific diagnostic tests permitting verifying the disease. The article presents clinical case of Kawasaki disease in child of three months age who was not diagnosed timely. As result, up to nine months in boy aneurysms of coronary and axillary arteries were developed. This observation emphasizes complexity of diagnostic of Kawasaki disease in children of early age, absence of diagnostic criteria and high risk of development of aneurysms of arteries under incomplete and/or atypical forms, especially in children prior to age of three months.

Deep Neck Inflammation as a Presentation of Kawasaki Disease

Kawasaki disease is typically a self-limiting condition that is a common cause of pediatric vasculitis [1] and the leading cause of pediatric acquired heart disease in developed countries [2]. The disease most frequently occurs in children aged between 6 months and 5 years [3] and is associated with coronary artery aneurysms in up to 25% of untreated cases [4]. There is currently no laboratory test for diagnosing Kawasaki disease. Rather, diagnosis depends on recognizing established clinical criteria [5]. Unfortunately, atypical manifestations of KD appear to be on the rise, decreasing the likelihood of timely diagnosis and appropriate treatment. Herein, we report an unusual case of Kawasaki disease: a 5-year-old boy who presented with fever and right side cervical lymphadenitis.

A case of incomplete Kawasaki disease with only fever, complicated by coronary artery lesions

A case of incomplete Kawasaki disease with only fever, complicated by coronary artery lesions

Weighing Evidence and Art: A Challenging Case of Early-Onset Atypical Kawasaki Disease

A 3-month-old previously healthy boy first presented to the emergency department (ED) with a 2-day history of diarrhea, severe dehydration, and fussiness with apneic spells. He also had developed a maculopapular rash on his extremities and trunk that had almost completely resolved on presentation to the ED and had conjunctival erythema with discharge. Several children at home had “pink eye” the same week the infant became ill. From the ED, he was admitted to the PICU, where he was intubated. Although his initial laboratories were unremarkable (white blood cell [WBC] count 11.0 k/uL, 39% neutrophils, platelets 342 k/uL), he also underwent an extensive septic workup (including blood cultures, urine culture, cerebrospinal fluid analysis and culture, herpes simplex virus and enterovirus cultures, respiratory viral panel by polymerase chain reaction, respiratory culture, and influenza A/B direct antigen detection tests) along with empirical treatment with antibiotics for a total of 5 days. These laboratory and imaging studies (serial chest x-rays, head computed tomography scan) were not clinically significant. He defervesced for a 48 hours, but still experienced low-grade fever (Tmax 38.3°C) and intermittent episodes of irritability. After 8 days of hospitalization, he was discharged from the hospital after apparent resolution of most of his symptoms. At his follow-up outpatient pediatric visit 4 days after discharge, 12 days since the onset of his illness, he was afebrile and clinically quiet. ### Question Is there objective evidence that this patient had Kawasaki disease (KD) on initial visit? ### Discussion Tomisaku Kawasaki described the criteria for diagnosing KD in 1967.1 The criteria require presence of fever for duration of ≥5 days along with 4 of the 5 following physical findings without an alternative explanation:

Abstract 195: Kawasaki Disease in a four-year-old girl with History of Acute Myeloid Leukemia; Report of a Case and Literature Review

Background: Kawasaki disease (KD) and acute myeloid leukemia (AML) both occur predominantly in young children. However, considering that the incidence rate of cancer during 0-14 years of age in Japan is 10.1 per a hundred thousand and that of KD during 0-4 years of age is 260 per a hundred thousand, there is little possibility of the same individual developing 2 diseases. Both inside and outside Japan we found at least 19 case reports of KD some relevant to cancer. Herein, we report a case of KD in a four-year-old Japanese girl with history of AML. Case report: A four-year-old Japanese girl was diagnosed with KD at 3 days of illness. She had the medical history of AML-M7 at her age of 1. She had no chimeric genes and was classed as intermediate risk. She had been treated following JPLSG AML05 protocol including etoposide, cytarabine (Ara-C), mitoxantrone, idarubicin, methotrexate (MTX), hydrocortisone (HDC) and triple intrathecal therapy (Ara-C, MTX and HDC). More than 2 years had passed without recurrence since her last chemotherapy. When we diagnosed her as KD, laboratory findings showed WBC 15.5 х103/μl with 85% neutrophils, hematocrit 33.7%, platelet count 19.1x10 6 /μl, CRP 10.5mg/dl, serum albumin 3.8g/dl and BNP 12.2pg/ml. She responded well to IVIG (2g/kg) plus prednisolone (2mg/kg/day) and ASA (30mg/kg/day). Although BNP transiently had increased to 151.8pg/ml at 5 days after onset, neither coronary arterial abnormalities nor myocardial complications were detected at 1 year of illness. Conclusions: We experienced the rare case of KD in a four-year-old Japanese girl with history of AML. She responded well to IVIG and any cardiovascular complications were not detected at 1 year of illness. Referring to only this case, it is unclear whether the history of AML could influence developing and progress of KD or not. The possibility of the same individual developing KD during 0-4 years of age and cancer during 0-14 years of age would be 2.6 per 10 million in Japan. However, among three hundred thousand cumulative patients of KD in Japan, 14 cases have been reported that they had developed cancer during 0-14 years of age. To discuss the relation to character of cancer, chemotherapy and severity or compilations of KD, more case should be evaluated.

Abstract 126: A Case Of Slowly Progressing Giant Coronary Aneurysm In A Patient With Intractable Kawasaki Disease

Background: Some patients with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) do not respond to intravenous methylprednisolone (IVMP). Infliximab (IFX; anti-TNF-α antibody) is an alternative medication for intractable KD. Its effectiveness in preventing coronary aneurysms from progressing is being reported. We herein report a case of intractable KD that slowly progressed to giant aneurysm despite a second IFX infusion. Methods: A 2-year-old boy visited our hospital with a 2-day history of fever. On day 3 after fever onset, a swollen cervical lymph node, redness of the BCG (bacilli Calmette Guérin) inoculation site, and indurated erythema of the palms with soles were observed. On day 5, conjunctival injection, strawberry tongue, red lips and truncal rash were present. The patient was diagnosed with KD and treated with IVIG and acetylsalicylic acid (ASA). Results: The fever persisted for 24 h after IVIG treatment. A second dose of IVIG was infused. However, the fever continued. Then IVMP was infused. On day 9, the fever resolved. On day 10, two-dimensional echocardiography (2DE) showed mild dilations (Φ 3.0 mm) of both coronary arteries (CAs). On day 18, the fever recurred. Follow-up 2DE showed dilated CAs (left main coronary artery [LM], Φ 4.0 mm; right coronary artery [RCA], Φ 4.0 mm). Therefore, IFX was intravenously infused. On day 19, the fever had resolved and the patient went home. On day 22, the fever recurred. Thus, a second infusion of IFX was done. On day 24, the fever resolved. Follow-up 2DE showed that the diameters of the CAs were 4.0 mm (LM), 7.0 mm (left anterior descending coronary artery [LAD]), 3.0 mm (RCA). On day 28, a mild fever was still present. On day 47, follow-up 2DE indicated that the diameters of the CAs were 3.0 mm (LM), 8.0 mm (LAD), 2.5 mm (RCA). On day 159, follow-up 2DE showed slightly increased regression of CAs. The patient has been undergoing regular 2DE and taking ASA since he left the hospital. Conclusions: This was a very rare case of KD that was resistant to standard treatment comprising high-dose steroids and TNF-α blockade. We have reported this case to inform clinicians who may encounter such patients with KD in the future. A high index of suspicion and keen observations are strongly recommended for cases of intractable KD.

Abstract 141: A Case of Kawasaki Disease with Re-dilatation of a Coronary Artery Aneurysm after Regression

Background: In patients with Kawasaki disease (KD), re-dilatation of coronary artery lesions (CAL) after regression is very rare. Here we report a case of KD with CAL re-dilatation after regression. Case report: A 15-year-old boy was diagnosed with KD at 1 year of age and was treated with intravenous immunoglobulin (IVIG, 400 mg/kg х 5 days). On day 14, echocardiography revealed CAL on the right coronary artery (RCA) and left coronary artery (LCA). Coronary arteriographic findings were as follows: segment 1, 3 mm in diameter; segment 6, 5 mm in diameter. He was prescribed aspirin, ticlopidine, and warfarin for 3 years. When he was 3 years old, coronary angiography showed complete CAL regression. His medications were discontinued and he underwent routine follow-up by echocardiography on which the CAL were not seen. At 14 years of age, coronary CT revealed re-dilatation of the LCA. Coronary angiography showed the same findings: 7 mm in diameter at the bifurcation between the LAD and the left circumflex artery, while the other regions were intact. He restarted aspirin and warfarin. The mechanism of CAL re-dilatation remains unclear; however, it is very important to follow patients carefully and routinely using echocardiography and/or coronary computed tomography, especially in cases with a history of CAL.

Abstract 73: A Case Of Fatal Kawasaki Disease Associated With Fulminant Myocarditis

Introduction: Acute phase Kawasaki disease (KD) is often associated with myocarditis. In most cases, such myocarditis is self-limiting. Fulminant myocarditis is rare as a cause of death for acute KD. We report a case of fatal KD associated with fulminant myocarditis. Case: A 4-year-old boy presented high fever and consulted family doctors for the second successive day. Although he received antibiotics, high fever persisted. He consulted to our hospital on the 4th day of illness. At the initial visit, conjunctival congestion, redness of pharyngeal and lip, bilateral cervical lymphadenitis and erythema on neck, palms and abdomen were observed. Initial laboratory data were shown as follows: WBC count 20,800 / μL, CRP 14.18 mg / dL. He was hospitalized with the diagnosis of acute febrile disease including KD. At first he was prescribed acetylsalicyclic acid and intravenous infusion of antibiotics, however high fever still persisted. On the 6th day of illness, immunoglobulin (IG) and prednisolone were administered intravenously because he was anticipated to be refractory to IG. On echocardiography, left ventricular contraction was normal. There were no findings of coronary artery abnormalities or pericardial effusion. After the first administration of IVIG, high fever persisted. Inflammatory markers on blood examination exacerbated. Laboratory data on the 8th day of illness were as follows: WBC 23,500 / μL, CRP 27.35 mg / dL, CPK 1,451 IU / L (MB 2%). Therefore, we added the second IVIG, steroid pulse therapy and urinastatine. On the next day (9th day), however, he suddenly suffered from cardiopulmonary arrest and deceased. Pathological findings revealed scattered myocardial necrosis on both ventricles. The CD 3, CD 8 positive T lymphocytes were infiltrated around myocytes. There were no findings of coronary artery aneurysms or thromboembolisms. These findings were compatible with fulminant myocarditis. Discussion: Recent advances in therapy for acute KD significantly reduce coronary artery lesions and subsequent mortality. However, clinical feature of KD has a lot of variety. Although fulminant myocarditis associated with KD in early acute phase is extremely rare, we should prepare for refractory KD and its abrupt change.

Abstract 49: Kawasaki Disease With Tsutsugamushi Disease: A Case Report

Background : Clinical and epidemiologic features suggest infectious agents as a possible cause of Kawasaki disease; however, the etiology of Kawasaki disease still remains unknown. A number of microorganisms were hypothesized as an etiology of the illness. This is the first reported case of Kawasaki disease with tsutsugamushi disease. Case presentation: We report the case of a 4-year-old boy who presented with fever of 7 days duration and skin rash and bilateral conjunctival injection. He had a history of visiting a rural area with his grandmother. On admission, he had fever of 39.4 °C. His heart rate was 90/minute and his blood pressure was 90/60 mmHg. His pharynx was slightly injected and there was red lip. His neck was swollen with cervical lymphadenitis. He had erythematous macular rash on her trunk. Examination of his skin revealed an eschar on penile base of right scrotum. His laboratory results showed WBC 4,720/mm 3 , 42% polymorphonuclear leucocytes, 39% lymphocytes, hemoglobin 10.3 gm/dL, platelet count 148,000/mm 3 , CRP 3.23mg/dl, pro-BNP 316.5 pg/ml. The respiratory viruses using a multiplex real-time-PCR kit (Adenovirus, Influenza A, Influenza B, Metapneumovirus, Rhino A virus, Respiratory syncytiai virus, Parainfluenza ) were all negative. Mycoplasma pneumonia IgM was negative. R.tsutsugamushi Ab was positive. Echocardiographic findings 1 day after admission was mild dilatation of LCA (RCA=1.8mm, LCA=3mm). He was treated on oral roxithromycin for presumptive diagnosis of tsutsugamushi disease along with clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin and aspirin. Over the next 48 hours, he became afebrile and his rash improved. He was placed on low-dose aspirin for 8 weeks. His echocardiogram were within normal limit (RCA= 1.9mm, LCA= 2.7mm) at 2 months after the onset of his illness. Conclusion: This case report suggests that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.

INCOMPLETE KAWASAKI DISEASE IN A THREE MONTH OLD INFANT

Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a threemonth- old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a high index of suspicion for KD and even, higher for IKD. Keywords: Coronary ectasia, Incomplete Kawasaki Disease (IKD), Thrombocytosis, Vasculitis

Kawasaki disease refractory to standard treatments that responds to a combination of pulsed methylprednisolone and plasma exchange: Cytokine profiling and literature review

We present a case of Kawasaki Disease (KD) that was refractory to plasma exchange (PE), but which finally responded to concurrent intravenous methylprednisolone pulse (IVMP) and PE treatment. To determine direct and indirect evidence for the efficacy of this combination therapy, we analyzed data of patients with refractory KD by review of the literature using medical databases and cytokine profiling. For literature searches, we used the Pubmed™ and Ichushi™ databases. Search terms used included “Kawasaki disease” and “plasma exchange” to extract articles that described KD cases treated with PE. For cytokine profiling, we measured interleukin (IL)-6, soluble tumor necrosis factor-α receptor (sTNF-αR) type 1 and type 2 before and after PE and PE with IVMP. Our search revealed 201 KD patients treated with PE, of which PE treatment was effective in 188 patients (93.5%), but not in 13 cases (6.5%). All 13 cases were treated successfully with additional treatment. Of the 13 cases, only six (2.5%) had recurrence during the PE treatment period. In our case, cytokine profiling showed PE treatment decreased IL6, while sTNF-αR type1 and type2 remained at high levels. PE and IVMP decreased IL-6 and sTNFα-R type 1 and type 2 levels. Conclusion: PE concurrent with additional anti-inflammatory treatment such as IVMP might be a very promising treatment option for PE refractory patients.

A Case of Atypical Kawasaki Disease With Myositis.

Summary of Clinical Presentations and Relevant Investigation Findings in Previously Reported Cases of Kawasaki Disease With Myositis.TitleAuthor, Journal, Year Clinical PresentationRelevant Investigation Findings ConclusionMyositis in Kawasaki disease 8 Gama et al, Pediatr Neurol , 19908-Year-old boy, previously well, presented with clinical features of KD, diffused peripheral weakness, and respiratory failure•• CK: 1509 to 2657 IUMyositis is one of several neurological complications encountered in KD. The degree of CK elevation may be useful in predicting the severity of myopathy.•• EMG: myopathic pattern •• Muscle biopsy: atrophy and degenerationA case of polymyositis associated with Kawasaki disease 9 Sugie et al, Brain Dev , 19853-Year-old boy, previously well, was diagnosed with KD, and developed painful proximal muscle weakness in all extremities•• CK: 152 IU Polymyositis might be a complication of KD. Proximal muscle weakness suggests inflammatory myopathy, even if serum CK was not significantly elevated. •• EMG: myopathic change•• Muscle biopsy: mild architecture distortion, fiber atrophy, inflammatory cell infiltratesMyositis with Kawasaki’s disease

Neonatal Kawasaki Disease: A Case Report

Objective: To report a case of Kawasaki disease (KD) with unusual age at presentation. Case Report: A 26-day-old Saudi boy presented with fever, irritability and poor feeding for 3 days. There was right cervical lymph node enlargement. There was mild throat congestion and bilateral congested tympanic membrane. The liver was palpable. Otherwise, there were no more abnormal physical findings. Laboratory findings revealed high erythrocyte sedimentation rate and positive C-reactive protein. The patient was admitted into an isolation room and antibiotics were started. On the 5th day, the condition of the patient deteriorated. Ampicillin was discontinued and vancomycin was started till getting the results of the culture sensitivity. On the 7th day, he developed diaper rash and fever spikes continued. On the 8th day, the patient developed swelling, redness and hotness of the right hand. Possibility of sepsis was considered and the administered antibiotics were imipenem and vancomycin, while cefotaxime was discontinued. On the 12th day, the patient developed anemia and leukocytosis. Gamma glutamyl transpeptidase was high. On the 15th day, the patient started to develop maculopapular rash on the trunk and desquamation of both hands and feet with swelling, cracked lips, bilateral conjunctival injection and unilateral neck lymph node swelling. So, the infant’s condition was clinically diagnosed as KD. Aspirin was started and 2 doses of intravenous immunoglobulins were given. On the 18th day, the condition of patient improved and the fever subsided. Conclusions: The diagnosis of KD among neonates is a clinical challenge. Diagnosis is based on clinical criteria after the exclusion of other diseases presenting with high persistent fever. Early treatment by administering intravenous immunoglobulins and aspirin can help prevent cardiac complications. There is a pressing need to raise awareness among pediatricians about this disease.

A case of incomplete and refractory Kawasaki disease: Diagnostic and therapeutic challenges

The diagnosis of incomplete Kawasaki disease (KD) – i.e. cases that do not fulfil all the diagnostic criteria – requires a high index of suspicion. We report a case of incomplete KD that was refractory to treatment with intravenous immunoglobulin and subsequently responded to treatment with intravenous methylprednisolone.

Kawasaki Disease with Acute Respiratory Distress Syndrome after Intravenous Immunoglobulin Infusion

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. We report a case of KD with acute respiratory distress syndrome (ARDS) after intravenous immunoglobulin (IVIG) infusion. Lung manifestations associated with KD have previously been reported in the literature. Although IVIG infusion is an effective therapy for acute KD, there are some reported complications related to IVIG infusion: hypotension, aseptic meningitis, acute renal failure, hemolytic anemia, etc. The case of KD reported here was treated with IVIG and aspirin. A few days after recovery from KD, the patient developed fever and maculopapular rash. A diagnosis of relapse KD was made and retreated with IVIG infusion. However, the patient developed ARDS four days after the second IVIG infusion. The patient recovered from ARDS after nine days of ICU care, which included high frequency oscillation ventilation with inhaled nitric oxide, steroid treatment and other supportive care.