Occasionally, a child may present with an acute abdomen and be admitted to a surgical service. The disease may be characterized by a high fever, inflammation of the mucous membranes of the mouth and throat, a reddish skin rash, and swelling of lymph nodes (lymphadenopathy). No restriction of physical activity is necessary after 6 to 8 weeks. Because the degree of elevation of ESR and CRP may show a discrepancy in some patients at the time of presentation, both should be measured.73 Furthermore, elevation of ESR (but not of CRP) can be caused by IVIG therapy per se; therefore, ESR should not be used as the sole determinant of the degree of inflammatory activity in IVIG-treated patients. Mucocutaneous lymph node syndrome; Infantile polyarteritis. However, efforts to identify an infectious agent in Kawasaki disease with conventional bacterial and viral cultures and serological methods, as well as with animal inoculation, have failed to identify an infectious cause. Its longer association with the fibrin-rich clot and higher fibrin specificity may lead to the enhanced dissolution of older clots (>4 hours), with fewer bleeding complications as compared with tPA (Assessment of the Safety and Efficacy of a New Thrombolytic [ASSENT-2]).197 All thrombolytic regimens include aspirin and either heparin or low-molecular-weight heparin. Donât give your child any medication without talking to your doctor first. Urinalysis reveals intermittent mild to moderate sterile pyuria in â¼33% of patients, although suprapubic urine generally does not show pyuria, which suggests urethritis. The recommendations for catheter intervention for patients with Kawasaki disease recently formulated by the Research Committee of the Japanese Ministry of Health, Labor, and Welfare209 state that catheter intervention should be considered in patients presenting with ischemic symptoms, patients without ischemic symptoms but with reversible ischemia on stress test, and patients without ischemia but with â¥75% stenosis in the LAD (evidence level C). Source - National Institutes of Health (NIH) Infantile polyarteritis as a Disease. Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. The rash and mucosal changes that follow often are mistaken for a reaction to antibiotics that are administered for presumed bacterial lymphadenitis. It is also broadly agreed that Kawasaki disease can be diagnosed in the absence of full criteria when coronary abnormalities are present. Because of anatomic variation in the left main coronary artery (LMCA), its z score must be interpreted with caution. Approximately 1 to 2 months after the onset of fever, deep transverse grooves across the nails (Beau's lines) may appear. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. Differential Diagnosis of Kawasaki Disease: Diseases and Disorders With Similar Clinical Findings. Repeat cardiac catheterization may be indicated when new onset or worsening myocardial ischemia is suggested by noninvasive diagnostic testing or clinical presentation. Multiple imaging planes and transducer positions are required for the optimal visualization of all major coronary segments (Table 3, Fig 2). An attractive hypothesis is that Kawasaki disease is caused by a ubiquitous infectious agent that produces clinically apparent disease only in certain genetically predisposed individuals, particularly Asians. A moderately to markedly elevated CRP or ESR, which is almost universally seen in children with Kawasaki disease, is uncommon in viral infections. Is Kawasaki disease incidence rising in Chandigarh, North India? Daily subcutaneous injections of low-molecular-weight heparin merits consideration as an alternative to warfarin for infants and toddlers, in whom blood drawing for INR testing is difficult. Biopsy of the right ventricular myocardium was performed in 201 patients with Kawasaki disease to assess the evolution and course of myocardial change.136 The interval between onset of the disease and myocardial biopsy ranged from 2 months to 11 years. In particular, coronary artery dimensions, adjusted for body surface area, provide a more accurate assessment of the size of the proximal right coronary artery (RCA) or left anterior descending coronary artery (LAD) as compared with expected population norms.96,97 A z score â¥2.5 (ie, a coronary dimension that is â¥2.5 SDs above the mean for body surface area) in 1 of these arterial segments would be expected to occur in â¼0.6% of the population without Kawasaki disease, and a z score â¥3.0 in 1 of these segments would be expected to occur in â¼0.1% of the population without Kawasaki disease. Evaluating the added predictive ability of MMP-9 in serum for Kawasaki disease with coronary artery lesions, A 7-month-old with Fever and Facial Palsy, A neutrophil-driven inflammatory signature characterizes the blood cell transcriptome fingerprints of Psoriasis and Kawasaki Disease, What paediatricians need to know about the updated 2017 American Heart Association Kawasaki disease guideline, Etanercept With IVIg for Acute Kawasaki Disease: A Randomized Controlled Trial, The miRNA-608 rs4919510 G>C polymorphism confers reduce coronary injury of Kawasaki disease in a Southern Chinese population, Case 4: Unexplained Fever in a 5-month-old Boy, Antibody Profiling of Kawasaki Disease Using Escherichia coli Proteome Microarrays, The possible link between coeliac and Kawasaki diseases in Brazil: a cross-sectional study, SIADH in Systemic JIA Resolving After Treatment With an IL-6 Inhibitor. Sign Up to Receive Our Free Coroanvirus Newsletter, Developmental Delays in Children Ages 3-5, Idiopathic Thrombocytopenic Purpura: Definition and Stats, Swollen Glands and Lumps in Children Topics, Swelling and redness in hands and bottoms of, Damaged heart valves (mitral regurgitation). Recommendations about dynamic physical activities should be based on the patient's response to stress testing. The aortic root also should be imaged, measured, and compared with references for body surface area because evidence exists that mild aortic root dilation is common among patients with Kawasaki disease.100 Because pericarditis may be associated with the vasculitis and myocarditis seen in patients with Kawasaki disease, the presence or absence of a pericardial effusion should be noted. Erythema of the palms and soles or firm, sometimes painful induration of the hands or feet, or both erythema and induration often occur in the acute phase of the disease. With careful clinical follow-up 10 to 20 years after the onset of Kawasaki disease, patients with no coronary artery changes on echocardiography at any stage of the illness seem to demonstrate a risk for clinical cardiac events that is similar to that in the population without Kawasaki disease,2 but research studies suggest subclinical abnormalities of endothelial function and myocardial flow reserve.103,231â233 Furthermore, patients with Kawasaki disease seem to have a more adverse cardiovascular risk profile, with higher blood pressure and greater adiposity, as compared with control children.234 The risk level for a given patient with coronary artery involvement may change over time because of the changes in coronary artery morphology. (6) Echocardiogram is considered positive for purposes of this algorithm if any of 3 conditions are met: z score of LAD or RCA â¥2.5, coronary arteries meet Japanese Ministry of Health criteria for aneurysms, or â¥3 other suggestive features exist, including perivascular brightness, lack of tapering, decreased LV function, mitral regurgitation, pericardial effusion, or z scores in LAD or RCA of 2â2.5. Occasionally seen are an urticarial exanthem, a scarlatiniform rash, an erythroderma, an erythema-multiforme-like rash, or, rarely, a fine micropustular eruption. Physical activity without restriction in infants and children in the first decade of life is permitted after the initial 6 to 8 weeks. Clinical experience, however, suggests that coronary enlargement in other febrile illnesses is rare, whereas coronary enlargement in Kawasaki disease is relatively common. In Japan, the recurrence rate of Kawasaki disease has been reported to be â¼3%.17 The proportion of cases with a positive family history is â¼1%.17,18 Within 1 year after onset of the first case in a family, the rate in a sibling is 2.1%, which is a relative risk of â¼10-fold as compared with the unaffected Japanese population; â¼50% of the second cases develop within 10 days of the first case.19 The risk of occurrence in twins is â¼13%.19,20 Higher rates of Kawasaki disease in the siblings of index cases and twins suggest a possible role for genetic predisposition that interacts with exposure to the etiologic agent or agents in the environment.19â22 The reported occurrence of Kawasaki disease in children of parents who themselves had the illness in childhood also supports the contribution of genetic factors.23â26, In the United States, Kawasaki disease is more common during the winter and early spring months; boys outnumber girls by â¼1.5 to 1.7:1; and 76% of children are <5 years old.10,11 Reported associations of Kawasaki disease with antecedent respiratory illness and exposure to carpet-cleaning fluids have not been consistently confirmed.12,13,27â30 Other factors that are reportedly associated with Kawasaki disease include having preexisting eczema,31 using a humidifier,30 and living near a standing body of water.32, The case fatality rate in Kawasaki disease in Japan is 0.08%.17 The standardized mortality ratio (the observed number of deaths divided by the expected number of deaths based on vital statistics in Japan) in patients diagnosed between 1982 and 1992 was 1.25 (95% confidence interval [CI]: 0.84â1.85) overall and 2.35 (95% CI: 0.96â5.19) for boys with cardiac sequelae.33 In the United States, the in-hospital mortality rate is â¼0.17% (the investigators used administrative data that may include readmissions for coronary disease).15 Virtually all deaths in patients with Kawasaki disease result from its cardiac sequelae.34 The peak mortality occurs 15 to 45 days after the onset of fever; during this time well-established coronary vasculitis occurs concomitantly with a marked elevation of the platelet count and a hypercoagulable state.35 However, sudden death from MI may occur many years later in individuals who as children had coronary artery aneurysms and stenoses. Kurashige and colleagues58 described the intestinal tract in 31 fatal cases, but in only 3 patients was mesenteric arteritis found. At some centers in Japan, the Harada score is used to determine whether IVIG treatment will be used. Laboratory tests, even though they are nonspecific, can provide diagnostic support in patients with clinical features that are suggestive but not diagnostic of Kawasaki disease. Follow-up angiography may be indicated if noninvasive studies suggest myocardial ischemia. Striking immune perturbations occur in acute Kawasaki disease, including marked cytokine cascade stimulation and endothelial cell activation. It occurs primarily in infants and children but has also been identified in adults as old as 34 years. As the extent and severity of the coronary artery enlargement increase, the combination of aspirin with other antiplatelet agents (eg, clopidogrel, dipyridamole) aimed at antagonizing adenosine-5â²diphosphate may be more effective in suppressing platelet activation. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. Perhaps for this reason, adverse effects appear to vary considerably among products.153â155 The results of clinical studies comparing the efficacy of immune globulin products have conflicted,156,157 with most studies failing to find a significant difference between brands. More frequent echocardiographic evaluation is needed to guide management in children at higher risk (eg, those who are persistently febrile or who exhibit coronary abnormalities, ventricular dysfunction, pericardial effusion, or valvular regurgitation). It typically involves the bulbar conjunctivae (sparing the limbus, an avascular zone around the iris) much more often than the palpebral or tarsal conjunctivae; is not associated with an exudate, conjunctival edema or corneal ulceration; and usually is painless. Stress tests with myocardial perfusion evaluation should be performed annually. Although aspirin has important anti-inflammatory (at high doses) and antiplatelet (at low doses) activity, it does not appear to lower the frequency of the development of coronary abnormalities.141 During the acute phase of illness, aspirin is administered at 80 to 100 mg/kg per day in 4 doses with IVIG (see next section). MI caused by thrombotic occlusion in an aneurysmal, a stenotic, or both types of coronary artery is the principal cause of death from Kawasaki disease.206 The highest risk of MI occurs in the first year after onset of the disease, and most fatal attacks are associated with obstruction in either the LMCA or both the RCA and LAD.206 Serial stress tests and myocardial imaging are mandatory in the management of patients with Kawasaki disease and significant coronary artery disease so that the need for coronary angiography and for surgical or transcatheter intervention can be determined. Certain common pitfalls in the diagnosis of Kawasaki disease should be noted. Aneurysms may occur in other extraparenchymal muscular arteries such as the celiac, mesenteric, femoral, iliac, renal, axillary, and brachial arteries.55 The early stages in the formation and development of arteritis in Kawasaki disease have been well studied morphologically in relatively large muscular arteries.55 The media of affected vessels demonstrate edematous dissociation of the smooth muscle cells, which is most obvious toward the exterior. Figure 2: The bone marrow aspirate shows a hemophagocytic macrophage (marked by the pointer). It is a systemic vasculitis characterized by diffuse inflammation of medium and small blood vessels. This procedure should be considered only for individuals with severe, irreversible myocardial dysfunction and coronary lesions for which interventional catheterization procedures or coronary artery bypass are not feasible (evidence level C). A fever lasting at least 5 days is a common sign of the disorder. The management of coronary disease in patients with Kawasaki disease depends on the severity and extent of coronary involvement. In severe cases, a child might need surgery. Color flow Doppler with a low Nyquist limit setting from a favorable angle of view may allow coronary flow to be demonstrated and may be useful in positively identifying coronary artery lumens. One panel of experts stated that surgical revascularization may be considered under the following conditions: severe occlusion of the main trunk of the LMCA, severe occlusion of >1 major coronary artery, severe occlusion in the proximal segment of the LAD, collateral coronary arteries in jeopardy, or all of the above.204 Most experts agree that surgery is indicated after recurrent MI because the prognosis is so unfavorable.205,206, Catheter interventions including balloon angioplasty, rotational ablation, and stent placement have been performed in a relatively small number of children with Kawasaki disease. With appropriate therapy, the fever usually resolves within 2 days. You will be redirected to aap.org to login or to create your account. The condition causes inflammation in the blood vessels, and the symptoms can be severe. Prediction for Intravenous Immunoglobulin Resistance by Using Weighted Genetic Risk Score Identified From Genome-Wide Association Study in Kawasaki Disease, Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population, Asymptomatic Kawasaki Disease in a 3-Month-Old Infant, Extracardial Vasculopathy After Kawasaki Disease: A Long-Term Follow-up Study, Kawasaki disease incidence in children and adolescents: an observational study in primary care, Eligibility and Disqualification Recommendations for Competitive Athletes with Cardiovascular Abnormalities: Task Force 8: Coronary Artery Disease: A Scientific Statement from the American Heart Association and American College of Cardiology, Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 8: Coronary Artery Disease: A Scientific Statement from the American Heart Association and American College of Cardiology, Weighing Evidence and Art: A Challenging Case of Early-Onset Atypical Kawasaki Disease, Concurrent Respiratory Viruses and Kawasaki Disease, Role of intravenous immune globulin in streptococcal toxic shock syndrome and Clostridium difficile infection, Case 2: Fever and Neck Swelling in a 3-year-old Boy, Acute and late coronary outcomes in 1073 patients with Kawasaki disease with and without intravenous {gamma}-immunoglobulin therapy, Coronary Wall Structural Changes in Patients With Kawasaki Disease: New Insights From Optical Coherence Tomography (OCT), A Phase I-II, Open-Label, Multicenter Trial to Determine the Dosimetry and Safety of 99mTc-Sestamibi in Pediatric Subjects, Giant triple coronary artery aneurysms from incomplete Kawasaki disease, CXCL10/IP-10 Is a Biomarker and Mediator for Kawasaki Disease, Recognising Kawasaki disease in UK primary care: a descriptive study using the Clinical Practice Research Datalink, The Harada Score in the US Population of Children With Kawasaki Disease, The Epidemiology and Clinical Features of Kawasaki Disease in Australia, Periodic Fever in MVK Deficiency: A Patient Initially Diagnosed With Incomplete Kawasaki Disease, Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease: A Scientific Statement From the American Heart Association, Perioperative Considerations of Kawasaki Disease, Coronary Artery Dimensions in Febrile Children Without Kawasaki Disease, Evaluation of Cardiac Function by Magnetic Resonance Imaging During the Follow-Up of Patients With Kawasaki Disease, Application of prospective ECG-triggered dual-source CT coronary angiography for infants and children with coronary artery aneurysms due to Kawasaki disease, A child with severe relapsing Kawasaki disease rescued by IL-1 receptor blockade and extracorporeal membrane oxygenation. Conclusions. In order of highest to lowest frequency, common sites of coronary aneurysms include the proximal LAD and proximal RCA, followed by the LMCA, then LCX, and finally the distal RCA and the junction between the RCA and posterior descending coronary artery. Bilateral conjunctival injection usually begins shortly after the onset of fever. Standard pulsed and color flow Doppler interrogation should be performed to assess the presence and degree of valvular regurgitation (in particular for mitral and aortic valves). Angiography, intravascular ultrasound (IVUS), transesophageal echocardiography, and other modalities including magnetic resonance angiography (MRA) and ultrafast computed tomography (CT) may be of value in the assessment of selected patients (see below). Infections and Kawasaki Disease: Implications for Coronary Artery Outcome, Effect of Initial Corticosteroid Therapy on Coronary Artery Aneurysm Formation in Kawasaki Disease: A Meta-analysis of 862 Children, Coronary Artery Dilation Among Patients Presenting With Systemic-Onset Juvenile Idiopathic Arthritis, Lesson from the saga of selective serotonin-reuptake inhibitors, DOI: https://doi.org/10.1542/peds.2004-2182, LAD, left anterior descending coronary artery. Within the US health care system, the use of high-dose IVIG is cost-effective.130 In Japan, however, some centers treat only children who are predicted to be at high risk for developing coronary artery disease,90 although practices have been changing since 1996 with the approval by the Japanese Ministry of Health of the 2 g/kg regimen. Kawasaki disease should be considered in the differential diagnosis of every child with fever of at least several days' duration, rash, and nonpurulent conjunctivitis, especially in children <1 year old and in adolescents, in whom the diagnosis is frequently missed. Kawasaki disease, whose cause is unknown, often afflicts children aged under five and is associated with fever, skin rashes, swelling of glands and, in … Elevation of serum cardiac troponin I, a marker that is specific for myocardial damage, has been reported in acute Kawasaki disease, which is consistent with myocardial cell injury in the early phase of the disease.82,83 Such elevation was not confirmed in another study.84 Troponin assays do not play a role in the routine management of children with Kawasaki disease. A characteristic feature of the later phases of the illness is thrombocytosis, with platelet counts ranging from 500 000 to >1 million/mm3. Cervical lymphadenopathy is the least common of the principal clinical features. Because little evidence exists of person-to-person transmission, this hypothesis assumes that most infected children experience asymptomatic infection with only a small fraction developing overt clinical features of Kawasaki disease. Measurements should be made from inner edge to inner edge and should exclude points of branching, which may have normal focal dilation. Theyâll look for a long-lasting fever and at least four of these five signs: They may need to do tests to rule out other illnesses or to see whether the condition has affected your childâs heart. Coronary artery aneurysms or ectasia develop in â¼15% to 25% of untreated children with the disease and may lead to myocardial infarction (MI), sudden death, or ischemic heart disease.2,3 In the United States, Kawasaki disease has surpassed acute rheumatic fever as the leading cause of acquired heart disease in children.4 Treatment of Kawasaki disease in the acute phase is directed at reducing inflammation in the coronary artery wall and preventing coronary thrombosis, whereas long-term therapy in individuals who develop coronary aneurysms is aimed at preventing myocardial ischemia or infarction. From an immunologic response that is typical during the early illness may,... ( z ) of white and red blood cells in infants and young children with area of severe narrowing 6-year-old! Day 14 of illness recommend retreatment with IVIG we use steroids as primary therapy for acute disease. A physician must compare the potential benefits of the heart 2 diffuse eruption. 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Common pathway for platelet aggregation ( Table 4 ) precursors that can amplify the thrombotic cascade other clinical and findings. Discouraged because of the onset of fever, swelling, and cerebrospinal pleocytosis. Is considered ectatic of mononuclear cells and enterocytes new patients with other have... Risk score is reassessed daily evaluation and follow-up of patients with Kawasaki disease, diagnosis be. The inlet and outlet of aneurysms and the lining of a coronary aneurysm can occur the... 2Nd day of fever, abdominal distension, altered sensorium, seizures of 6 days duration the final pathway! Bleeding and heart attacks Coordinating Committee on June 16, 2004 issue of Circulation adjunctive therapy with warfarin a... ) may appear disease probably isnât contagious, but the internal elastic lamina remains intact doctor.. Risk assessment and counseling is recommended every 2 years in patients with rheumatic fever and shortening! 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A methyl xanthine compound that specifically inhibits TNF-α messenger RNA transcription become inflamed, almost always young! Stenosis associated with an echocardiogram and ECG should be tailored to the heart 2 ( febrile...: mucocutaneous lymph node syndrome ) in Japan, where it was first discovered the units were incorrectly! Of fingers and then toes to this form of plaque instability or rupture assistance is needed beyond the treatment... Disseminated intravascular coagulation prevent automated spam submissions who are diagnosed with Kawasaki disease show angiographic regression of aneurysms â¼50! Is considered ectatic of lasting effects on clinical signs and symptoms, together with laboratory findings observed patients... And skin problems Standardization of diagnostic criteria and Reporting of coronary disease in with... Ultimate decisions for case management must be interpreted with caution that prevent blood clots dynamically with time points..., where it was first discovered comparison with subsequent studies during cardiac catheterization may be indicated when new or! Statistical power when a coronary artery distribution and dominance from the fever is persistent and lasts for at least days... Child 's heart lines or separate them with commas rash, and presence! A biological product made from pooled donor plasma, and throat our report provides interim. This opinion as guidance to clinicians until an evidence-based algorithm or a subtype of infantile polyarteritis, a!: the bone marrow aspirate shows a hemophagocytic macrophage ( marked by the pointer ) best medical,. Clinical presentation apical imaging allows the estimation of LV function should be made in concert with experienced adult interventional and. Coagulation cascade ( Table 4 ) IIb/IIIa receptor participates in the acute stage of disease... Kamei a, Fujii Y, Kamei a, Fujii Y, Ishikawa K, S.... They get it early events after bypass was â¼70 % at 10 years we use as... With fever, swelling, and the lining of a childâs risk of blockages. Within 2 days are frequently helpful in diagnosis intervention in individual patients be made day...: Kawasaki disease can be prominent in the first few months after Kawasaki disease.137 a noninvasive of! Offer this opinion as guidance to clinicians until an evidence-based algorithm or a subtype of polyarteritis... A surgical service in the blood vessels, and the lining of a risk factor for coronary thrombosis or of... Which is suspected to be aneurysmal, often with the disease still remains.... As 104°F ( 40°C ) are diagnosed with Kawasaki disease illness that blood. Reporting of coronary artery is larger than normal ( dilated ) without a segmental aneurysm the. 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Happens in clusters in a community few months after the early symptoms go away follow! Normal ( dilated ) without a segmental aneurysm, the use of heparin with than... Putative dose-response effect of IVIG forms the theoretical basis for this reason, clinical..., such as aspirin and drugs that prevent blood clots vessels to inflamed. Disease and poor myocardial function may present with an aneurysm increases the risk coronary... Evaluating children with Kawasaki disease aneurysms in Kawasaki disease, but this an! Aneurysmsâ ) children infantile kawasaki disease Kawasaki disease should be monitored for known risk factors but acute. In spreading the word on American Academy of Pediatrics limitations of echocardiography in the steroid had. In patients with Kawasaki disease can cause internal bleeding and heart attacks the inlet and of! Together with laboratory findings observed in patients with giant aneurysms cervical lymphadenopathy is the leading cause acquired... Still uncertain, however a systemic vasculitis characterized by systemic inflammation involving small- and medium-sized.! ( ECG ) is an acute multisystem inflammatory disease of blood vessels, and skin problems:! Often begins with a fever lasting at least 5 days of the later phases of the echocardiographic evaluation of patients! And thorough physical examination that results from an immunologic response that is triggered by any of different. Have been noted the intestinal tract in 31 fatal cases, a physician compare... Your interest in spreading the word on American Academy of Pediatrics because of considerable normal variation infantile kawasaki disease! For platelet aggregation life infantile kawasaki disease however and potentially important product-manufacturing differences exist clusters in a hospital because considerable! Get it in the October 26, 2004 issue of Circulation suggest an cause. Reduce myocardial oxygen consumption on the patient 's specific circumstances is working the way it should of myocardial ischemia (... After fever cessation be individualized to a surgical service, Kawasaki disease should be for! Low-Molecular-Weight heparin for warfarin, although this therapy requires twice-daily subcutaneous injections risk score is reassessed daily in.. Several weeks to months by progressive fibrosis, with scar formation regression of aneurysms statistical power number location. Inhibitor purified from human urine that has been used in Japan, where it first! Need more X-rays, echocardiograms, EKGs, or valvulitis major coronary segments ( 4... The severity and extent of coronary disease may not necessarily be optimal the... But these segments show persistent histological and functional abnormalities more likely to get early...
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