KAWASAKI DISEASE
History of Kawasaki disease
Epidemiology and etiology
Presentation and diagnosis
Treatment
Chronic cardiovascular manifestations
Follow up of patients
Questions in the chronic management
is an upper respiratory tract bacterial infection associated with a characteristic rash, which is caused by an infection with pyrogenic exotoxin (erythrogenic toxin) -producing GAS in individuals who do not have antitoxin antibodies In the past.
scarlet fever was thought to reflect infection of an individual lacking toxin-specific immunity with a toxin-producing strain of GAS.
Subsequent studies have suggested that development of the scarlet fever rash may reflect a hypersensitivity reaction requiring prior exposure to the toxin.
The hypertensive encephalopathy is a syndrome consisting of a sudden elevation of arterial pressure usually preceded by severe headache and followed by convulsions, coma or a variety of transitory cerebral phenomena.
Pediatric dengue management - Dr. Arunkumar, MD(Paed)Arun Kumar
A presentation on clinical management of dengue fever and severe dengue in children.
By
Dr. Arunkumar. A, MD(Pediatrics)
consultant pediatrician,
KMCH Erode.
is an upper respiratory tract bacterial infection associated with a characteristic rash, which is caused by an infection with pyrogenic exotoxin (erythrogenic toxin) -producing GAS in individuals who do not have antitoxin antibodies In the past.
scarlet fever was thought to reflect infection of an individual lacking toxin-specific immunity with a toxin-producing strain of GAS.
Subsequent studies have suggested that development of the scarlet fever rash may reflect a hypersensitivity reaction requiring prior exposure to the toxin.
The hypertensive encephalopathy is a syndrome consisting of a sudden elevation of arterial pressure usually preceded by severe headache and followed by convulsions, coma or a variety of transitory cerebral phenomena.
Pediatric dengue management - Dr. Arunkumar, MD(Paed)Arun Kumar
A presentation on clinical management of dengue fever and severe dengue in children.
By
Dr. Arunkumar. A, MD(Pediatrics)
consultant pediatrician,
KMCH Erode.
kawasaki disease is disease of pediatric age group leading to involvement of coronaries in 25% of case. some of presented as fetal complication. early diagnosis and treatment useful measure to prevent complications.
This includes a brief account on epidemiology, pathophysiology, clinical presentation, investigation, treatment, complications and disposition of a patient presenting with acute pancreatitis.
Webinar Series on COVID-19 vaccine: Jointly organized by Malaysian Society of Infection Control and Infectious Diseases (MyICID) & Institute for Clinical Research (ICR), NIH
Speaker: Dr. Richard Lim Boon Leong is a Consultant Palliative Medicine Physician and Head of Palliative Care Unit, Selayang Hospital, Ministry of Health Malaysia.
VITAMIN A DEFICIENCYAND HYPERVITAMINOSIS A
Vitamin A deficiency (VAD) is a major nutritional concern in poor societies, especially in lower income countries like INDIA.
Vitamin A is an essential nutrient needed in small amounts for the normal functioning of the visual system, and maintenance of cell function for growth, epithelial integrity, red blood cell production, immunity and reproduction.
Spirometry and peak flow metry in bronchial asthmaDr Pankaj Yadav
SPIROMETRY AND PEAK FLOMETRY in BRONCHIAL ASTHMA
SPIROMETRY IS A PULMONARY FUNCTION TEST THAT MEASURES THE VOLUME OF AIR AN INDIVIDUAL INHALES OR EXHALES AS A FUNCTION OF TIME.
SPIROMETRY MEASURES HOW MUCH AND HOW QUICKLY AIR CAN BE EXPELLED FOLLOWING A DEEP BREATH.
FLOW , OR THE RATE AT WHICH VOLUME IS CHANGING AS A FUNCTION OF TIME, CAN ALSO BE MEASURED WITH SPIROMETRY.
HEMOLYTIC ANEMIA
Hemo: Referring to blood cells
Poiesis: “The development or production of”
The word Hemopoiesis refers to the production & development of all the blood cells:
Erythrocytes: Erythropoiesis
Leucocytes: Leucopoiesis
Thrombocytes: Thrombopoiesis.
Begins in the 20th week of life in the fetal liver & spleen, continues in the bone marrow till young adulthood & beyond!
The adrenal cortex produces three major classes of steroids:
glucocorticoids,
(2) mineralocorticoids, and
(3) adrenal androgens.
Consequently, normal adrenal function is important for
-modulating intermediary metabolism and immune responses through glucocorticoids;
blood pressure, vascular volume, and electrolytes through mineralocorticoids;
secondary sexual characteristics (in females) through androgens.
The adrenal axis plays an important role in the stress response by rapidly increasing cortisol levels.
Adrenal disorders include hyperfunction (Cushing's syndrome) and hypofunction (adrenal insufficiency) as well as a variety of genetic abnormalities of steroidogenesis.
Represents 30% of all deaths worldwide (15 million deaths/year)
Leading cause of death and disability
CVD burden in developing countries
Risk factors worldwide
Auditory brainstem response (ABR)
Approximately 1 of every 1000 children is born deaf. Many more are born with less severe degrees of hearing impairment, while others may acquire hearing loss during early childhood.
combination of technological advances in ABR and otoacoustic emissions (OAE) testing methods are used for evaluation of hearing in newborns.
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
Basavarajeeyam is an important text for ayurvedic physician belonging to andhra pradehs. It is a popular compendium in various parts of our country as well as in andhra pradesh. The content of the text was presented in sanskrit and telugu language (Bilingual). One of the most famous book in ayurvedic pharmaceutics and therapeutics. This book contains 25 chapters called as prakaranas. Many rasaoushadis were explained, pioneer of dhatu druti, nadi pareeksha, mutra pareeksha etc. Belongs to the period of 15-16 century. New diseases like upadamsha, phiranga rogas are explained.
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
2. Kawasaki Disease
• History of Kawasaki disease
• Epidemiology and etiology
• Presentation and diagnosis
• Treatment
• Chronic cardiovascular manifestations
• Follow up of patients
• Questions in the chronic management
drpankajyadav05@gmail.com
3. Kawasaki Disease(Mucocutaneous
Lymph Node Syndrome)
“A self-limited vasculitis of unknown
etiology that predominantly affects children
younger than 5 years. It is now the most
common cause of acquired heart disease in
children in the United States and Japan.”
Jane Burns, MD*
*Burns, J. Adv. Pediatr. 48:157. 2001.*Burns, J. Adv. Pediatr. 48:157. 2001.
drpankajyadav05@gmail.com
4. History of Kawasaki Disease
• Original case observed by Kawasaki January 1961
– 4 y.o. boy, “diagnosis unknown”
• CA thrombosis 1st
recognized 1965 on autopsy of
child prev. dx’d w/MCOS
• First Japanese report of 50 cases, 1967
• First English language report from Dr. Kawasaki
1974, simultaneously recognized in Hawaii
drpankajyadav05@gmail.com
5. What is Kawasaki Disease?
• Idiopathic multisystem disease
characterized by vasculitis of small &
medium blood vessels, including
coronary arteries
drpankajyadav05@gmail.com
6. Epidemiology
• Median age of affected children = 2.3 years
• 80% of cases in children < 4 yrs, 5% of
cases in children > 10 yrs
• Males:females = 1.5-1.7:1
• Recurs in 3%
• Positive family history in 1% but 13% risk
of occurrence in twins.
drpankajyadav05@gmail.com
7. Epidemiology
• Annual incidence of 4-15/100,000 children
under 5 years of age
• More in Asian-Americans, African-
Americans next most prevalent
• Seasonal variation
– More cases in winter and spring but occurs
throughout the year
drpankajyadav05@gmail.com
8. What is the Etiology of Kawasaki
Disease?
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9. Etiology
• Infectious agent most likely
– Age-restricted susceptible population
– Seasonal variation
– Well-defined epidemics
– Acute self-limited illness similar to known
infections
• No causative agent identified
– Bacterial, retroviral, superantigenic bacterial toxin
– Immunologic response triggered by one of several
microbial agents
drpankajyadav05@gmail.com
10. New Haven Coronavirus
• Identified a novel human coronavirus in
respiratory secretions from a 6-month-old
with typical Kawasaki Disease
• Subsequently isolated from 8/11 (72.7%) of
Kawasaki patients & 1/22 (4.5%) matched
controls (p = 0.0015)
• Suggests association between viral infection
& Kawasaki disease
Esper F, et . J Inf Dis. 2005; 191:499-502
drpankajyadav05@gmail.com
11. Diagnostic Criteria
• Fever for at least 5 days
• At least 4 of the following 5 features:
1. Changes in the extremities
Edema, erythema, desquamation
2. Polymorphous exanthem, usually truncal
3. Conjunctival injection
4. Erythema&/or fissuring of lips and oral cavity
5. Cervical lymphadenopathy
• Illness not explained by other known disease
process
Modified from Centers for Disease Control. Kawasaki Disease. MMWR 29:61-63, 1980
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12. Atypical or Incomplete
Kawasaki Disease
• Present with < 4 of 5 diagnostic criteria
• Compatible laboratory findings
• Still develop coronary artery aneurysms
• No other explanation for the illness
• More common in children < 1 year of age
• 2004 AHA guidelines offer new evaluation
and treatment algorithm
drpankajyadav05@gmail.com
13. Differential Diagnosis
• Infectious
– Measles & Group A beta-hemolytic strep can
closely resemble KD
– Bacterial: severe staph infections w/toxin
release
– Viral: adenovirus, enterovirus, EBV, roseola
drpankajyadav05@gmail.com
16. Phases of Disease
• Acute (1-2 weeks from onset)
– Febrile, irritable, toxic appearing
– Oral changes, rash, edema/erythema of feet
• Subacute (2-8 weeks from onset)
– Desquamation, may have persistent arthritis or
arthralgias
– Gradual improvement even without treatment
• Convalescent (Months to years later)
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17. Trager, J. D. N Engl J Med 333(21): 1391. 1995.
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27. Cardiovascular Manifestations of
Acute Kawasaki Disease
• None
• Suggestive of myocarditis (50%)Suggestive of myocarditis (50%)
– Tachycardia, murmur, gallop rhythmsTachycardia, murmur, gallop rhythms
– Disproportionate to degree of fever & anemiaDisproportionate to degree of fever & anemia
• Suggestive of pericarditisSuggestive of pericarditis
– Present in 25% although symptoms are rarePresent in 25% although symptoms are rare
– Distant heart tones, pericardial friction rub,Distant heart tones, pericardial friction rub,
tamponadetamponade
drpankajyadav05@gmail.com
28. Role of Cardiology in the Acute
Setting
• Usually just to document baseline coronary
artery status–not an emergency
• If myocarditis suspected–an emergency
• Can help diagnose “atypical” disease
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29. Echocardiographic Findings
• Myocarditis with dysfunction
• Pericarditis with an effusion
• Valvar insufficiency
• Coronary arterial changes
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30. Coronary Arterial Changes
• 15% to 25 % of untreated patients
develop coronary artery changes
• 3-7% if treated in first 10 days of fever
with IVIG
• Most commonly proximal, can be distal
– Left main > LAD > Right
drpankajyadav05@gmail.com
31. Coronary Arterial Changes
• Vary in severity from echogenicity due
to thickening and edema or
asymptomatic coronary artery ectasia
to giant aneurysms
• May lead to myocardial infarction,
sudden death, or ischemic heart disease
drpankajyadav05@gmail.com
32. Coronary Aneurysms
• Size
– Small = <5 mm diameter
– Medium = 5-8 mm
– Giant = ≥ 8 mm
• Highest risk for sequelae
• Shape
– Saccular
– Fusiform
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33. Coronary Aneurysms
•• Patients most likely to develop aneurysms
– Younger than 6 months, older than 8 years
– Males
– Fevers persist for greater than 14 days
– Persistently elevated ESR
– Thrombocytosis
– Pts who manifest s/s of cardiac involvement
drpankajyadav05@gmail.com
35. Coronary Aneurysm
• Approximately 50% of aneurysms resolve
– Smaller size
– Fusiform morphology
– Female gender
– Age less than 1 year
• Giant aneurysms (>8mm) worst prognosis
drpankajyadav05@gmail.com
36. Cardiovascular Sequelae
• 0.3-2% mortality rate due to cardiac disease
– 10% from early myocarditis
• Aneurysms may thrombose, cause MI/death
• MI is principal cause of death in KD
– 32% mortality
– Most often in the first year
– Majority while at rest/sleeping
– About 1/3 asymptomatic
drpankajyadav05@gmail.com
37. Acute Kawasaki Disease:
Treatment
• IVIG: 2g/kg as one-time dose
– Mechanism of action is unclear
– Significant reduction in CAA in pts
treated with IVIG plus aspirin vs. aspirin
alone (15-25%3-5%)
– Efficacy unclear after day 10 of illness
drpankajyadav05@gmail.com
38. Acute Kawasaki Disease:
Treatment
• IVIG
– 70-90% defervesce & show symptom
resolution within 2-3 days of treatment
– Retreat those with failure of response to
1st
dose or recurrent symptoms Up to
2/3 respond to a second course
drpankajyadav05@gmail.com
39. Acute Kawasaki Disease:
Treatment
• Aspirin
– High dose (80-100 mg/kg/day) until afebrile
x 48 hrs &/or decrease in acute phase
reactants
– Need high doses in acute phase due to
malabsorption of ASA
– Dosage of ASA in acute phase does not
seem to affect subsequent incidence of CAA
drpankajyadav05@gmail.com
40. Acute Kawasaki Disease:
Treatment
• Aspirin
– Decrease to low dose (3-5 mg/kg/day) for 6-8
weeks or until platelet levels normalize
– No evidence f/effect on CAA when used
alone
– Due to potential risk of Reye syndrome
instruct parents about symptoms of influenza
or varicella
drpankajyadav05@gmail.com
41. Acute Kawasaki Disease:
Treatment
• Aggressive support with diuretics &
inotropes for some patients with
myocarditis
• Antibiotics while excluding bacterial
infection
drpankajyadav05@gmail.com
42. Acute Kawasaki Disease:
Treatment
• Conflicting data about steroids
– Reports of higher incidence of aneurysms &
more ischemic heart dz in pts w/aneurysms
– Case report of KD refractory to IVIG but
responsive to high-dose steroids &
cyclosporine.
drpankajyadav05@gmail.com
43. Patient Follow-Up Categories
• Five categories based on coronary arteries
findings
– No coronary changes at any stage of illness
– Transient CA ectasia, resolved within 6-8 wks
– Small/medium solitary coronary aneurysm
– One or more large or giant aneurysms or
multiple smaller/complex aneurysms in same
CA, without obstruction
– Coronary artery obstruction
drpankajyadav05@gmail.com
First, I want to give an overview of the disease. I’ll discuss: Its history as a disease entity. The epidemiology and etiology of the disease. Its general presentation and diagnostic criteria. And then the pharmacologic treatment. Then, I want to discuss the chronic cardiovascular manifestations and the epidemiology of these sequelae. I’ll discuss the guidelines for follow up care by cardiologists. Finally, I want to discuss some of the unknown or controversial issues. For example, Can we predict who will develop cardiac sequelae? Are there long term cardiac issues in the face of “normal” coronary arteries? And how do we follow these patients?
A good place to start is to define the disease. This quote is a as good a definition as I’ve found. Kawasaki disease is a self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children in the United States and Japan. Another important point to make is that, in part due to the fact that we have no etiology, there is no specific diagnostic test for Kawasaki disease. For this reason, some people call it Kawasaki syndrome and not Kawasaki disease. Therefore, guideline have been developed to make the diagnosis. But, the diagnosis of Kawasaki disease is still one of exclusion.
MCOS was the original name of Kawasaki disease --- it stands for mucocutaneous ocular syndrome. This child had died suddenly & unexpectedly.
Kawasaki disease is virtually unheard of in children older than 15 years of age.
So, the biggest question in Kawasaki Disease research is: What is the etiology of the disease? Two corollaries to this are: Can we develop a test to accurately diagnose the disease? Can we develop better treatments? Days could be spent on these questions. Despite a large literature, there are no definitive answers. Overall, a lot of evidence points to an infectious etiology with some evidence of the involvement of a super antigen. But, after over 30 years of research, no one is sure.
A number of each of these has been implicated in individual cases or clinical series but typically data are conflicting. A variant strain of Propionibacterium acnes with dust mites playing a role as vectors has been proposed as the causative agent by several investigators but no causative link has been established despite multiple investigations.
The diagnosis of Kawasaki Disease requires fever persisting at least 5 days (although US and Japanese experts now agree that only 4 days of treatment are necessary before initiating treatment) and the presence of at least 4 of the following 5 principal features: changes in extremities, a polymorphous rash, conjunctival injection, changes in lips and oral cavity, and cervical lymphadenopathy. In addition, other diagnoses must also be ruled out. For example scarlet fever, toxic shock syndrome, and adenoviral infections have similar presentation. The fever is usually high, greater than 102°F, does not respond well to antipyretics, and can last 1-2 weeks. The important thing to remember though is that Kawasaki disease is a diagnosis of exclusion without confirmatory tests s and based on a constellation of signs and symptoms and/or consistent ultrasound findings
In fact, these 2 etiologies have been found to account for over 80% of patients initially thought to have Kawasaki disease but ultimately not diagnosed as such. These would include toxic shock syndrome & staph scalded skin syndrome.
As I said at the onset, Kawasaki disease is a vasculitis of small and medium-sized vessels so we must consider some immunologic/rheumatologic etiologies in the differential diagnosis. However, in Stevens-Johnson the conjunctivitis is more likely to be exudative while the rash is typically vesicular with crusting. If you remember back to the description of typical physical exam I gave earlier these characteristics don’t really fit with the conjunctivitis or the rash we expect to see in Kawasaki disease.
In this convalescent phase remaining symptoms resolve & laboratory values normalize.
Acute changes seen in the hands and feet are usually seen several days into the course. There is erythema and edema of hands and feet, usually on the dorsal surfaces. Children may also refuse to walk or have trouble putting on their shoes. Erythema of palms & soles &/or painful edema of hands/feet Usually start 3-5 days after onset of fevers Subungual desquamation in subacute phase Beau lines may be seen 2-3 months after onset
Polymorphous, non-vesicular rash Present in 99% of cases Usually maculopapular Characteristic confluence in perineum (60%) Often not pruritic
In the first week, patients can develop bilateral, painless bulbar conjunctival injection without exudate. There tends to be no redness adjacent to the pupil and these patients may also have anterior uveitis which is shown on acute phase slit lamp exam in 80% In the first week, they may also have erythema and cracking of lips, a strawberry tongue, and/or diffuse injection of oral and pharyngeal mucosae.
The lymphadenopathy occurs early in the disease, and can be very transient. It may or may not be bilateral but is not generalized throughout the body. Lymph nodes, by definition, must be 1.5 cm in diameter or larger. About 90% of patients will have fever, conjunctival erythema, and oral changes and 70% will have lymphadenopathy.
The complete list of associated symptom is too long to go into in detail here,but virtually every organ system can be involved. Patients can have cough, rhinorrhea, or a pulmonary infiltrate. They can have diarrhea, vomiting, abdominal pain, hydrops of gallbladder, mild jaundice, and mild increase of serum transaminase levels. They can have striking irritability and an aseptic meningitis with a mononuclear pleocytosis in cerebrospinal fluid as well as a facial palsy and hearing loss. Finally, they can develop myositis, arthralgias, and arthritis. Heme: hemophagocytosis Renal: ARF, renal artery aneurysms Skin: transverse furrows of fingernails (Beau’s lines) during convalescence Others: Peripheral gangrene, orbital myositis, Avascular necrosis of the femoral head
In acute phase, the most common blood laboratory findings are: a leukocytosis with left shift, mild anemia, an increased erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, elevation of liver transaminases Later in the illness, the platelet count tends to rise during the second week and can remain elevated for longer than 6-8 weeks. Finally, the urine can show sterile pyuria of urethral origin and occasional proteinuria.
From the cardiovascular standpoint, Patients can have no signs or symptoms. Sometimes, they have tachycardia out of proportion to the extent of fever. They can have a gallop rhythm or distant heart sounds suggestive of myocarditis or pericarditis with an effusion. They can have a flow murmur or a murmur due to valvar insufficiency. Or they can have frank congestive heart failure and/or an infarction. Their EKG can show a number of changes, including arrhythmias, abnormal Q waves, prolonged PR and/or QT intervals, low voltage, and ST-T–wave changes. Their chest x-ray can show cardiomegaly or pulmonary edema.
The murmur may be due to valvar insufficiency or to flow in a high output state.
Despite all of these potential findings, cardiologists are usually consulted in the acute phase to document the baseline status of the coronary arteries and to meet the family. If myocarditis or a pericardial effusion is suspected, we should be consulted immediately. However, the risk of acute mortality is low (0.08 % in the latest Japanese survey). In addition, our input can be helpful in diagnosing atypical Kawasaki disease.
Echocardiographic finding include myocarditis and depressed function, pericarditis with an effusion, valvular insufficiency, or coronary artery changes. The most common finding is mild coronary artery changes. Echo is 80-90% sensitive for the diagnosis of proximal coronary artery aneurysms
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
Aneurysms are classified as small, medium and giant. Giant aneurysms have the greatest risk for complications and have an incidence of 0.8 % in the latest Japanese survey. The aneurysms are also classified as saccular, if they have axial and lateral dimensions being of about equal size, or fusiform, if there is dilation with proximal and distal tapering.
These signs/symptoms would include mitral regurgitation or pericardial effusion.
Angiography is the gold standard and can define proximal and distal coronary anatomy better than echocardiography. However, performing angiography on all patients is unreasonable. In addition, angiography may miss abnormalities in the arterial wall, including thromboses or dysfunction.
And this is non-giant aneurysms. Resolution usually occurs within 1 to 2 years. Factors associated with an increased likelihood of resolution are… Some people opt to treat these patients with an anticoagulant such as coumadin to prevent thrombosis.
Rarely, aneurysms may rupture.
Typically given as this one time dose although different regimens have been used including 500/kg/day x 4 days. … and actually it was tried after the publication of successful IVIG therapy of ITP in 1981. It was endorsed as recommended therapy by the Committee on Infectious Diseases of the AAP in 1988. A multicenter prospective trial in the US has demonstrated … In addition to fewer giant coronary aneurysms children receiving IVIG also had more rapid resolution of abnormalities of myocardial function. Despite this, it’s typically recommended that children diagnosed after 10 days still be treated if there is evidence of continuing inflammation or evolving coronary artery disease.
Otherwise the platelet level can be repeated a month later . By 6-8 weeks if the coronaries continue to remain normal appearing it is extremely unlikely that they will develop any any any late changes.
However there have also been studies that have shown either no ill effects or even possible benefits such as lower prevalence of aneurysms, shorter duration of fever, and shorter duration of hospitalization
Further follow up is based on guidelines published in 1994. This follow up is based on a risk stratification dividing patients based on coronary artery findings. The categories are: 1. No coronary artery changes at any stage of the illness 2. Transient coronary artery ectasia that disappears during the acute illness 3. A single small to medium coronary artery aneurysm 4. One or more giant aneurysms or multiple small to medium sized aneurysms, without evidence of obstruction. 5. Coronary artery obstruction.
The management of patients is divided into four categories. . .