Abraham JohnUnited States UniversityFNP592: Common Illnesses Across the Lifespan – Clinical PracticumDr. Irene BenliroDecember 1, 2022Diagnosis Presentation: Kawasaki disease
Kawasaki disease (KD): Overview
KD is a disorder that results in inflammation of the blood vessel walls in some of our body parts.
It most frequently affects babies and young children.
Fever and a rash are signs in the early stages. High temperature and skin peeling are symptoms.
In advanced stages, inflammation of medium-sized blood vessels is possible (vasculitis). Lymph nodes, skin, and mucous membranes, including those in the mouth, are also affected.
Usually, Kawasaki disease is curable. Aspirin and intravenous immunoglobulin therapy are two initial treatments that are administered in a medical setting.
Kawasaki Disease
The risk that a patient would experience mucocutaneous signs of KD is most strongly influenced by variations in age.
About 90% of patients with KD have oral mucous membrane abnormalities,
Followed by polymorphous rash in 70% to 90% of cases.
Expect to see limb changes in 50% to 85% of cases,
Lastly, eye changes in >75% of cases, and cervical lymphadenopathy in 25% to 70% of cases.
So watch out for these signs for KD!!
The most frequent sign of KD is an elevated body temperature.
Bilateral nonexudative conjunctivitis is seen in more than 90% of patients with conjunctivitis.
As KD worsens, mucositis frequently becomes visible. A "strawberry tongue" and cracked, red lips are traits.
Rash – The cutaneous symptoms of KD can take many different forms. The perineal erythema and desquamation that commonly precede the rash's onset during the first few days of illness are followed by macular, morbilliform, or targetoid skin lesions on the trunk and limbs.
Changes to the extremities — Changes to the extremities typically manifest last. Children experience generalized erythema on their palms and soles as well as indurated edema on the dorsum of their hands and feet.
Tachycardia that is out of proportion to fever and gallop noises are two cardiac symptoms that might appear within the first week to 10 days of an illness.
Anatomy & physiology
Most reported in Japan, the incidence has been reported to be 250/100,000 children under the age of 5, compared to the United States, where it is roughly 25/100,000 children under the age of 5.
85% of individuals affected are younger than 5 years old, with the disease typically manifesting in infancy and early childhood.
In the US, KD is the main contributor to childhood heart disease.
Coronary artery aneurysm incidence in untreated KD patients ranges from 15 to 25%.
The primary cause of death is myocardial infarction brought on by thrombotic blockage of the coronary arteries.
Patients of Asian heritage seem to have KD more frequently. The amount of the heart involvement and the promptness of medical care determine the prognosis.
A&P continued
KD's etiology is a mystery. Small to medium sized vessels, particularly the coronary arteries, are affected by the inflammation in KD.
When the immune system accidentally damages the blood vessels, Kawasaki disease results.
The crucial element that determines morbidity and mortality is the degree of coronary artery involvement.
According to researchers, vascular damage is promoted by mediators including tumor necrosis factor (TNF), interleukin (IL)-1B, interferon (INF), and IL-6 generated by activated T-cells and macrophages. The subendothelial buildup of T cells, mononuclear cells, macrophages, and monocytes is the earliest pathogenic change in the vessel wall that has been documented.
What diagnostic do we do!?
There are three phases in KD.
The acute phase starts with a high temperature without a clear cause that may linger for one to two weeks. Vasculitis and perivasculitis may cause acute myocardial infarction during the acute phase.
The fever decreases during the subacute period, which could last 2-4 weeks. In this stage, the skin may desquamate and coronary artery aneurysms may develop. The number of platelets increases and may exceed 106 per millimeter three. ESR and CRP are typically increased as well as other acute phase reactants.
Within 6 to 8 weeks of the illness's beginning, the symptoms of the convalescent phase end and the platelet count and ESR return to normal.
Diagnostic!?
Since KD is considered as a systemic inflammation both C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) must be checked.
Leukocytosis and a rise in immature neutrophils in the white blood cell count should be assessed, as thrombocytosis typically manifests after the seventh day of sickness.
Another acute-phase reactant that is increased in inflammatory circumstances in KD is ferritin.
Lymphocyte generally decrease during the acute stage of KD, then drastically increase during recovery.
By the second week of the illness, platelet counts typically increase and may approach 1,000,000/mm3.
WBCs are frequently detected by urinary microscopy.
Diagnotic continued
KD diagnosis in accordance with clinical recommendations:
The existence of a fever lasting 5 days along with at least four of the following five physical characteristics, without a compelling medical reason
Bilateral injections into the bulbar conjunctiva
Oral mucous membrane alterations, such as strawberry tongue, injected pharynx, or fissured or injected lips.
Peripheral extremity alterations, such as periungual desquamation, erythema of the palms or soles, or edema of the hands or feet.
Multifaceted rash
Cervical lymphadenopathy (at least one lymph node with a diameter of 1.5 cm or greater)
Treatment
The American Heart Association's (AHA's) and the American Academy of Pediatrics' (AAP's) recommendations for treating patients with KD are Intravenous immune globulin (IVIG; 2 g/kg), given as a single infusion over 8 to 12 hours, is the recommended initial therapy
Due to its anti-inflammatory actions (such as a shortening of the duration of a fever) and antiplatelet effects, aspirin was one of the first medications utilized for KD. But it's not obvious if using aspirin in addition to IVIG has more anti-inflammatory effects.
The recommended range for aspirin dosage to have an anti-inflammatory impact during the acute phase of disease is 30 to 100 mg/kg per day in four divided doses, this is considered a heavy dose for someone young.
Treatment
Glucocorticoids — Glucocorticoids have been shown to decrease the rate of CA abnormalities in Japanese patients with KD at high risk for IVIG resistance
Tumor necrosis factor inhibition — Some KD patients have been shown to have elevated levels of tumor necrosis factor (TNF) alpha. Anti-TNF-alpha medications like etanercept or infliximab have so been investigated as both a primary illness adjuvant therapy and a monotherapy for refractory KD.
Information relating to the needs of the patient
Although older children can sometimes be affected, children under the age of five are most frequently diagnosed with Kawasaki disease.
KD is more prevalent in the winter and spring, and it affects boys more frequently. Although the precise cause of KD is uncertain, it is thought that an infection may be the starting point. Children who are genetically predisposed to the condition may also experience it, moreover, there is no spread of Kawasaki disease.
Kawasaki disease cannot be diagnosed using a particular test.
Instead, a child's symptoms and a physical examination are used to make the diagnosis of Kawasaki disease.
A protracted fever (defined as one that lasts for more than five days) is frequently the first sign that prompts a clinician to think about Kawasaki disease.
Lab analyses could support a diagnosis. Blood and urine tests, an electrocardiogram (ECG) to examine the heart's electrical system, and an echocardiography (heart ultrasound) to assess the size of the blood arteries around the heart and the condition of the heart are possible.
Information cont…
Due to the concern over the association between aspirin use and Reye syndrome, parents are occasionally hesitant to provide aspirin to their children. Reye syndrome, an inflammatory condition that affects the brain and liver, has been seen in aspirin-taking individuals who have chicken pox or influenza.
Most medical professionals advise getting your child vaccinated against influenza if they are taking aspirin for an extended period of time for any reason. You should seek medical help right away if your child is taking aspirin and has chicken pox or has been exposed to it.
Information cont….
Because damage to the coronary arteries frequently does not manifest itself for several weeks, follow-up care is crucial. Fortunately, most kids with KD don't experience any long-term effects with the right care.
Children with coronary artery involvement require routine cardiology exams. The degree of coronary alterations may influence how frequently these visits are made. These individuals typically experience a positive outcome with the appropriate medical care.
Refereneces
Kawasaki Syndrome | CDC. (n.d.). https://www.cdc.gov/kawasaki/index.html
McCrindle, B. W., Rowley, A. H., Newburger, J. W., Burns, J. C., Bolger, A. F., Gewitz, M., Baker, A. L., Jackson, M. A., Takahashi, M., Shah, P. B., Kobayashi, T., Wu, M. H., Saji, T. T., & Pahl, E. (2017). Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation, 135(17). https://doi.org/10.1161/cir.0000000000000484
TePas, E. (2022). Kawasaki disease: Clinical features and diagnosis. UpToDate. https://www.uptodate.com/contents/kawasaki-disease-clinical-features-and-diagnosis?search=kawasaki%20disease%20children&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#
Sundel, R. (2022). Kawasaki disease: Initial treatment and prognosis. UpToDate. https://www.uptodate.com/contents/kawasaki-disease-initial-treatment-and-prognosis?search=kawasaki%20disease%20children%20treatment&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H4233359
