Kawasaki disease

Kawasaki disease US Medical PG Question 1: A 6-year-old boy presents to his pediatrician accompanied by his mother for evaluation of a rash. The rash appeared a little over a week ago, and since that time the boy has felt tired. He is less interested in playing outside, preferring to remain indoors because his knees and stomach hurt. His past medical history is significant for an upper respiratory infection that resolved uneventfully without treatment 2 weeks ago. Temperature is 99.5°F (37.5°C), blood pressure is 115/70 mmHg, pulse is 90/min, and respirations are 18/min. Physical exam shows scattered maroon macules and papules on the lower extremities. The abdomen is diffusely tender to palpation. There is no cervical lymphadenopathy or conjunctival injection. Which of the following will most likely be found in this patient?

• A. Coronary artery aneurysms
• B. Leukocytoclastic vasculitis (Correct Answer)
• C. Thrombocytopenia
• D. Mitral regurgitation
• E. Occult malignancy

Kawasaki disease Explanation: ***Leukocytoclastic vasculitis*** - This patient presents with symptoms highly suggestive of **Henoch-Schönlein Purpura (HSP)**, including a preceding URI, fatigue, low-grade fever, **abdominal pain**, and a **palpable purpura** primarily on the lower extremities. - HSP is characterized by IgA-mediated **small-vessel vasculitis**, which histologically presents as leukocytoclastic vasculitis with IgA deposition on immunofluorescence. *Coronary artery aneurysms* - **Coronary artery aneurysms** are a classic complication of **Kawasaki disease**, not Henoch-Schönlein Purpura. - Kawasaki disease presents with different clinical features, including prolonged fever, conjunctival injection, oral changes, and cervical lymphadenopathy. *Thrombocytopenia* - **Thrombocytopenia** is characterized by a low platelet count and often presents with petechiae, purpura, and bleeding, but the rash in HSP is due to inflammation and extravasation of red blood cells, not low platelets. - Platelet counts in HSP are typically **normal** or can be slightly elevated as an acute phase reactant. *Mitral regurgitation* - **Mitral regurgitation** is a common manifestation of **rheumatic fever**, particularly after recurrent episodes, caused by valvular damage. - Rheumatic fever is also preceded by infection (Group A Strep) but involves different symptoms like migratory polyarthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules, which are not described here. *Occult malignancy* - While an **occult malignancy** can cause paraneoplastic syndromes or constitutional symptoms, the specific constellation of symptoms, including the migratory rash, abdominal pain, and preceding URI, points much more strongly to **HSP** in a 6-year-old. - The presentation is more consistent with an acute, inflammatory process rather than a chronic, insidious malignant one.

Kawasaki disease US Medical PG Question 2: A 9-year-old boy is brought to the emergency department because of progressively worsening shortness of breath for 3 days. He has had fever and malaise for the past 5 days. He had a sore throat 3 weeks ago that resolved without treatment. He appears ill. His temperature is 38.6°C (101.5°F), pulse is 98/min and blood pressure is 84/62 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 93%. Examination shows jugular venous distension and bilateral ankle edema. There are erythematous, ring-shaped macules and patches over his trunk that are well-demarcated. Auscultation of the chest shows crackles at the lung bases bilaterally. An S3 is heard on cardiac auscultation. His hemoglobin concentration is 12.2 g/dL, leukocyte count is 13,600/mm3, and platelet count is 280,000/mm3. A urinalysis is normal. An x-ray of the chest shows cardiac silhouette enlargement with prominent vascular markings in both the lung fields. Which of the following is the most likely etiology of this patient's symptoms?

• A. Systemic lupus erythematosus
• B. Acute rheumatic fever (Correct Answer)
• C. Kawasaki disease
• D. Viral myocarditis
• E. Infection with Borrelia burgdorferi

Kawasaki disease Explanation: ***Acute rheumatic fever*** - This patient presents with signs of cardiac involvement (**shortness of breath**, **S3 gallop**, **cardiomegaly**, **pulmonary edema**), a history of a recent **strep throat infection**, and **erythema marginatum** (the ring-shaped rash), all consistent with **acute rheumatic fever**. - **Acute rheumatic fever** is a delayed, non-suppurative complication of Group A Streptococcus pharyngitis, leading to systemic inflammation, particularly affecting the heart, joints, brain, and skin. *Systemic lupus erythematosus* - SLE can cause cardiac complications like pericarditis or myocarditis, but the characteristic rash of SLE (malar or discoid) differs from the described **erythema marginatum**. - While SLE can cause systemic symptoms, the clear history of a preceding **sore throat** and the specific rash point away from SLE. *Kawasaki disease* - Kawasaki disease primarily affects **children under 5 years old** and is characterized by fever, conjunctivitis, oral changes, rash, lymphadenopathy, and extreme irritability. - The patient's age (9 years) and the specific rash described (erythema marginatum) are not typical for Kawasaki disease. *Viral myocarditis* - Viral myocarditis can cause **heart failure** symptoms (shortness of breath, S3, cardiomegaly), but it typically lacks the preceding **sore throat** and the specific skin findings of **erythema marginatum** seen in this patient. - While a viral infection could cause myocarditis, the constellation of symptoms points strongly to a specific post-streptococcal etiology. *Infection with Borrelia burgdorferi* - *Borrelia burgdorferi* (Lyme disease) can cause cardiac manifestations like **carditis** (often AV block) and a characteristic rash called **erythema migrans**. - However, **erythema migrans** is typically a single, expanding bull's-eye rash, which is different from the described **erythematous, ring-shaped macules and patches** of erythema marginatum.

Kawasaki disease US Medical PG Question 3: A 4-year-old boy is brought to the emergency department by his mother with a rash on his trunk, malaise, and fever with spikes up to 38.5°C (101.3°F) for the past 2 weeks. The patient's mother says she tried giving him Tylenol with little improvement. Past medical history includes a spontaneous vaginal delivery at full term. The patient's vaccines are up-to-date and he has met all developmental milestones. On physical examination, his lips are cracking, and he has painful cervical lymphadenopathy. The rash is morbilliform and involves his trunk, palms, and the soles of his feet. There is fine desquamation of the skin of the perianal region. Which of the following anatomical structures is most important to screen for possible complications in this patient?

• A. Mitral valve
• B. Kidneys
• C. Gallbladder
• D. Coronary artery (Correct Answer)
• E. Pylorus

Kawasaki disease Explanation: ***Coronary artery*** - The constellation of symptoms, including prolonged fever, rash on trunk, palms, and soles, cracked lips, cervical lymphadenopathy, and perianal desquamation, is highly indicative of **Kawasaki disease**. - **Coronary artery aneurysms** are the most serious complication of Kawasaki disease, occurring in 15-25% of untreated children, necessitating close monitoring and screening. *Mitral valve* - While other forms of vasculitis or rheumatic fever can affect heart valves, **mitral valve** involvement is not a primary or characteristic complication of Kawasaki disease. - The main cardiac concern in Kawasaki disease is direct arterial inflammation, not valvular dysfunction. *Kidneys* - **Renal involvement**, such as acute kidney injury, is not a typical or prominent feature of Kawasaki disease. - Kawasaki disease primarily targets medium-sized muscular arteries throughout the body, with a predilection for the coronary arteries. *Gallbladder* - **Hydrops of the gallbladder** can occur in Kawasaki disease, leading to acute cholecystitis-like symptoms, but it is generally a self-limiting complication. - While it's a potential finding, it is not as life-threatening or essential to screen for as coronary artery complications. *Pylorus* - There is no direct association between Kawasaki disease and primary involvement or complications of the **pylorus**. - Gastrointestinal symptoms can occur, but these are typically non-specific and do not involve anatomical changes to the pylorus.

Kawasaki disease US Medical PG Question 4: A 28-year-old male presents to his primary care physician with complaints of intermittent abdominal pain and alternating bouts of constipation and diarrhea. His medical chart is not significant for any past medical problems or prior surgeries. He is not prescribed any current medications. Which of the following questions would be the most useful next question in eliciting further history from this patient?

• A. "Does the diarrhea typically precede the constipation, or vice-versa?"
• B. "Is the diarrhea foul-smelling?"
• C. "Please rate your abdominal pain on a scale of 1-10, with 10 being the worst pain of your life"
• D. "Are the symptoms worse in the morning or at night?"
• E. "Can you tell me more about the symptoms you have been experiencing?" (Correct Answer)

Kawasaki disease Explanation: ***Can you tell me more about the symptoms you have been experiencing?*** - This **open-ended question** encourages the patient to provide a **comprehensive narrative** of their symptoms, including details about onset, frequency, duration, alleviating/aggravating factors, and associated symptoms, which is crucial for diagnosis. - In a patient presenting with vague, intermittent symptoms like alternating constipation and diarrhea, allowing them to elaborate freely can reveal important clues that might not be captured by more targeted questions. *Does the diarrhea typically precede the constipation, or vice-versa?* - While knowing the sequence of symptoms can be helpful in understanding the **pattern of bowel dysfunction**, it is a very specific question that might overlook other important aspects of the patient's experience. - It prematurely narrows the focus without first obtaining a broad understanding of the patient's overall symptomatic picture. *Is the diarrhea foul-smelling?* - Foul-smelling diarrhea can indicate **malabsorption** or **bacterial overgrowth**, which are important to consider in some gastrointestinal conditions. - However, this is a **specific symptom inquiry** that should follow a more general exploration of the patient's symptoms, as it may not be relevant if other crucial details are missed. *Please rate your abdominal pain on a scale of 1-10, with 10 being the worst pain of your life* - Quantifying pain intensity is useful for assessing the **severity of discomfort** and monitoring changes over time. - However, for a patient with intermittent rather than acute, severe pain, understanding the **character, location, and triggers** of the pain is often more diagnostically valuable than just a numerical rating initially. *Are the symptoms worse in the morning or at night?* - Diurnal variation can be relevant in certain conditions, such as inflammatory bowel diseases where nocturnal symptoms might be more concerning, or functional disorders whose symptoms might be stress-related. - This is another **specific question** that should come after gathering a more complete initial picture of the patient's symptoms to ensure no key information is overlooked.

Kawasaki disease US Medical PG Question 5: A 7-year-old boy comes to the physician because of a generalized rash for 3 days. Over the past 5 days, he has also had a high fever and a sore throat. His 16-year-old sister was treated for infectious mononucleosis 2 weeks ago. He returned from a summer camp a week ago. His immunizations are up-to-date. Three years ago, he required intubation after an allergic reaction to dicloxacillin. The patient appears ill. His temperature is 38.2°C (100.8°F). Examination shows circumferential oral pallor. Cervical lymphadenopathy is present. There is tonsillar erythema and exudate. A confluent, blanching, punctate erythematous rash with a rough texture is spread over his trunk and extremities. His hemoglobin concentration is 13.3 g/dL, leukocyte count is 12,000/mm3, and erythrocyte sedimentation rate is 43 mm/h. Which of the following is the most appropriate next step in management?

• A. Amoxicillin therapy
• B. Doxycycline therapy
• C. Azithromycin therapy (Correct Answer)
• D. Cephalexin therapy
• E. Acyclovir therapy

Kawasaki disease Explanation: ***Azithromycin therapy*** - This patient presents with symptoms highly suggestive of **streptococcal pharyngitis** (sore throat, fever, tonsillar exudates, cervical lymphadenopathy) complicated by **scarlet fever** (confluent, blanching, punctate erythematous rash with a rough texture, circumferential oral pallor). - Given his **history of severe allergic reaction (intubation) to dicloxacillin**, a penicillin-class antibiotic, azithromycin (a macrolide) is the appropriate choice for treating **Group A Streptococcus** (GAS) infection in a penicillin-allergic patient. *Amoxicillin therapy* - **Amoxicillin** is a penicillin-class antibiotic and is **contraindicated** due to the patient's severe allergic reaction (intubation) to dicloxacillin, another penicillin. - Cross-reactivity between penicillins, especially in severe allergic reactions, is a significant concern, making this an unsafe choice. *Doxycycline therapy* - **Doxycycline**, a tetracycline, is not a first-line treatment for **streptococcal pharyngitis** and is primarily used for atypical bacterial infections or in specific cases of penicillin allergy where other agents are not suitable. - While it has some activity against GAS, macrolides like azithromycin are preferred alternatives for penicillin-allergic patients. *Cephalexin therapy* - **Cephalexin** is a first-generation cephalosporin, and while it can be used for GAS, roughly 5-10% of patients with a penicillin allergy may have a **cross-reaction** to cephalosporins, especially with a history of severe reactions. - Therefore, it is generally avoided in patients with a history of anaphylaxis or other severe reactions to penicillin. *Acyclovir therapy* - **Acyclovir** is an antiviral medication used to treat herpes simplex and varicella-zoster virus infections. - The patient's symptoms (pharyngitis, rash consistent with scarlet fever) are indicative of a **bacterial infection (GAS)**, not a viral infection that would respond to acyclovir.

Kawasaki disease US Medical PG Question 6: A 2-year-old boy is brought to the emergency department by his parents because of facial swelling that has now progressed to total body swelling. He also complains of nausea and abdominal pain. The child was in his usual state of health a week ago when they first notice swelling around his eyes. A few days later his legs started to swell. The boy was born at 39 weeks gestation via spontaneous vaginal delivery. He is up to date on all vaccines and is meeting all developmental milestones. Today, his blood pressure is 104/60 mm Hg, the heart rate is 90/min, the respiratory rate is 25/min, and the temperature is 37.1°C (98.8°F). On examination, he has facial edema, abdominal shifting dullness, and bilateral leg edema up to the knees. Urine dipstick shows 4+ protein and urinalysis shows fatty casts. Serum albumin is 2.2 g/dL. Which of the following is the most likely etiology of this patient condition?

• A. Acute glomerulonephritis
• B. Minimal change disease (Correct Answer)
• C. Congestive heart failure
• D. Kwashiorkor
• E. Protein-losing enteropathy

Kawasaki disease Explanation: ***Minimal change disease*** - This patient presents with **generalized edema**, **heavy proteinuria** (4+ protein with fatty casts), and **hypoalbuminemia** (< 2.5 g/dL), classic findings of **nephrotic syndrome**. - **Minimal change disease** is the most common cause of nephrotic syndrome in children, typically presenting between 2 and 6 years of age, matching the patient's age and clinical picture. *Acute glomerulonephritis* - This condition is typically characterized by **hematuria**, **hypertension**, and **mild proteinuria**, often following a streptococcal infection. - The patient's presentation with **massive proteinuria** and absence of hematuria or significant hypertension makes acute glomerulonephritis less likely. *Congestive heart failure* - While CHF can cause edema, it is usually accompanied by signs of **cardiac dysfunction** such as tachycardia, tachypnea, and an enlarged heart on imaging, which are not described. - The primary cause of edema in CHF is **fluid overload** due to impaired cardiac output, not massive proteinuria and hypoalbuminemia as seen here. *Kwashiorkor* - This is a form of **severe protein malnutrition** leading to edema and abdominal distension, often seen in regions with food scarcity. - The clinical context does not suggest malnutrition, and the presence of **heavy proteinuria** points to a renal pathology rather than a primary nutritional deficiency. *Protein-losing enteropathy* - This condition involves excessive protein loss through the **gastrointestinal tract**, leading to hypoalbuminemia and edema. - However, it typically presents with **diarrhea** and malabsorption symptoms, which are not reported in this patient.

Kawasaki disease US Medical PG Question 7: A 9-month-old boy is brought to the physician because of increased irritability, continual crying, and fever for 1 day. His mother has noticed that he refuses to lie down on his right side and keeps tugging at his right ear. One week ago, he had a runny nose that has since improved. He was born at term and has been otherwise healthy. He was exclusively breastfed until 2 months of age and is currently bottle-fed with some solid foods introduced. He has been attending a daycare center for the past 5 months. His temperature is 38.4°C (101.1°F) and pulse is 144/min. Otoscopic examination in this child is most likely to show which of the following?

• A. Erythematous external auditory canal
• B. Vesicles in the ear canal
• C. Bulging erythematous tympanic membrane (Correct Answer)
• D. Brown mass within the ear canal
• E. Retracted opacified tympanic membrane

Kawasaki disease Explanation: ***Bulging erythematous tympanic membrane*** - The child's symptoms of **irritability**, **crying**, **fever**, **ear tugging**, and refusal to lie on one side, especially after a recent upper respiratory infection, are highly suggestive of **acute otitis media (AOM)**. - In AOM, the **tympanic membrane (eardrum)** becomes inflamed, red (**erythematous**), and often **bulges** outwards due to the accumulation of fluid and pus in the middle ear. *Erythematous external auditory canal* - An **erythematous external auditory canal** is characteristic of **otitis externa** (swimmer's ear), which typically presents with pain upon manipulation of the tragus or pinna, and discharge, rather than the systemic symptoms like fever and irritability seen here. - While some inflammation may extend to the external canal in AOM, the primary and most diagnostic finding for AOM is changes to the tympanic membrane itself. *Vesicles in the ear canal* - **Vesicles** in the ear canal or on the tympanic membrane, often accompanied by severe pain and possibly facial nerve palsy, are characteristic of **herpes zoster oticus** (Ramsay Hunt syndrome). - This condition is caused by viral reactivation and does not fit the common presentation of fever, irritability, and ear tugging in an infant following a runny nose. *Brown mass within the ear canal* - A **brown mass** within the ear canal could indicate a cerumen impaction (earwax plug), a foreign body, or potentially a **cholesteatoma** (a benign skin growth that can erode bone). - None of these conditions typically present with acute onset fever, irritability, and ear tugging in an infant, nor do they usually follow a recent upper respiratory infection. *Retracted opacified tympanic membrane* - A **retracted and opacified tympanic membrane** suggests changes in middle ear pressure, often seen in **eustachian tube dysfunction** or **otitis media with effusion (OME)**, where there is fluid but no acute infection. - While OME can precede AOM, the presence of fever, acute irritability, and significant pain (implied by ear tugging and refusal to lie down) points strongly to an active, acute infection rather than just effusion or retraction.

Kawasaki disease US Medical PG Question 8: A 3-year-old boy is brought to the physician for evaluation of developmental delay. He could sit alone at 12 months and started walking with support at the age of 2 years. He can name only very few familiar objects and uses simple two-word sentences. He cannot stack more than 2 blocks. His parents report that he does not like playing with other children. He is at the 80th percentile for head circumference, 85th percentile for height, and 50th percentile for weight. He has a long and narrow face as well as large protruding ears. His thumbs can be passively flexed to the ipsilateral forearm. This patient is at increased risk of developing which of the following conditions?

• A. Mitral regurgitation (Correct Answer)
• B. Type 2 diabetes mellitus
• C. Acute myeloid leukemia
• D. Aortic dissection
• E. Hyperuricemia

Kawasaki disease Explanation: ***Mitral regurgitation*** - The patient's presentation with **developmental delay**, **relatively large head circumference** (80th percentile), **long narrow face**, **large protruding ears**, and **hyperextensible joints** (thumbs to forearm) is highly suggestive of **fragile X syndrome**. - **Mitral valve prolapse** leading to **mitral regurgitation** is a common cardiac manifestation of fragile X syndrome, occurring in **50-80% of adult males** with the condition, due to **connective tissue dysplasia**. *Type 2 diabetes mellitus* - This condition is primarily associated with **obesity**, **insulin resistance**, and genetic predispositions unrelated to the features presented in this patient. - While fragile X patients may have general health concerns, there is **no specific increased risk** of developing type 2 diabetes mellitus directly linked to the syndrome's pathology. *Acute myeloid leukemia* - There is **no established association** between fragile X syndrome and an increased risk of developing **acute myeloid leukemia**. - AML is a **hematologic malignancy** with different risk factors, such as exposure to certain chemicals or prior chemotherapy. *Aortic dissection* - Aortic dissection is typically associated with conditions affecting **connective tissue** like **Marfan syndrome** or **Ehlers-Danlos syndrome**, or with **hypertension**. - While fragile X syndrome involves connective tissue abnormalities, **aortic dissection is not a typical or significantly increased risk** compared to other connective tissue disorders. *Hyperuricemia* - **Hyperuricemia** is most commonly associated with conditions like **gout**, **kidney disease**, or certain **genetic metabolic disorders** (e.g., Lesch-Nyhan syndrome). - There is **no direct link** between fragile X syndrome and an increased risk of hyperuricemia.

Kawasaki disease US Medical PG Question 9: A 4-day-old male infant is brought to the physician because of respiratory distress and bluish discoloration of his lips and tongue. He was born at term and the antenatal period was uncomplicated. His temperature is 37.3°C (99.1°F), pulse is 170/min, respirations are 65/min, and blood pressure is 70/46 mm Hg. Pulse oximetry on room air shows an oxygen saturation of 82%. A grade 3/6 holosystolic murmur is heard over the left lower sternal border. A single S2 that does not split with respiration is present. Echocardiography shows defects in the interatrial and interventricular septae, as well as an imperforate muscular septum between the right atrium and right ventricle. Further evaluation of this patient is most likely to show which of the following?

• A. Elfin facies
• B. Tracheal bowing on chest x-ray
• C. Increased pulmonary vascular markings on chest x-ray
• D. Delta wave on electrocardiogram
• E. Left-axis deviation on electrocardiogram (Correct Answer)

Kawasaki disease Explanation: ***Left-axis deviation on electrocardiogram*** - The combination of **cyanosis**, a **holosystolic murmur**, and defects in the **interatrial and interventricular septa** along with an **imperforate tricuspid valve** (muscular septum between the right atrium and right ventricle) is highly suggestive of **tricuspid atresia** with a **ventricular septal defect (VSD)** and an **atrial septal defect (ASD)**. - In tricuspid atresia, the **right ventricle is hypoplastic**, leading to LV dominance and a characteristic **left-axis deviation** on the ECG due to an enlarged left ventricle taking over the pumping action for the systemic and pulmonary circulation. *Elfin facies* - **Elfin facies** is a characteristic feature of **Williams syndrome**, which is associated with **supravalvular aortic stenosis** and not typically with cyanotic heart defects like tricuspid atresia. - There is no clinical information in the vignette that suggests features of Williams syndrome. *Tracheal bowing on chest x-ray* - **Tracheal bowing or compression** is commonly seen in conditions like a **vascular ring** or **large mediastinal masses**, which can cause respiratory distress but generally do not present with the specific cardiac findings described. - This finding is not characteristic of tricuspid atresia. *Increased pulmonary vascular markings on chest x-ray* - In tricuspid atresia, **pulmonary blood flow is often decreased** due to reduced flow to the right ventricle and pulmonary artery, especially if the VSD is restrictive or there's pulmonary stenosis. This would lead to **decreased, not increased, pulmonary vascular markings**. - Increased pulmonary vascular markings suggest conditions with **left-to-right shunting** or **pulmonary venous congestion**, which is contradictory to the physiology of complicated tricuspid atresia. *Delta wave on electrocardiogram* - A **delta wave** on an ECG is characteristic of **Wolff-Parkinson-White (WPW) syndrome**, indicating a pre-excitation pathway. - While WPW can occur with congenital heart disease, it is not a direct or expected consequence of tricuspid atresia and does not explain the cyanosis or specific cardiac defects mentioned.

Kawasaki disease US Medical PG Question 10: A 3500-g (7.7-lbs) girl is delivered at 39 weeks' gestation to a 27-year-old woman, gravida 2, para 1. Apgar scores are 8 and 9 at 1 and 5 minutes, respectively. The mother had regular prenatal visits throughout the pregnancy. She did not smoke or drink alcohol. She took multivitamins as prescribed by her physician. The newborn appears active. The girl's temperature is 37°C (98.6°F), pulse is 120/min, and blood pressure is 55/35 mm Hg. Examination in the delivery room shows clitoromegaly. One day later, laboratory studies show: Hemoglobin 12.8 g/dL Leukocyte count 6,000/mm3 Platelet count 240,000/mm3 Serum Na+ 133 mEq/L K+ 5.2 mEq/L Cl− 101 mEq/L HCO3− 21 mEq/L Urea nitrogen 15 mg/dL Creatinine 0.8 mg/dL Ultrasound of the abdomen and pelvis shows normal uterus and normal ovaries. Which of the following is the most appropriate next step in the management of this newborn patient?

• A. Spironolactone therapy
• B. Estrogen replacement therapy
• C. Dexamethasone therapy
• D. Genital reconstruction surgery
• E. Hydrocortisone and fludrocortisone therapy (Correct Answer)

Kawasaki disease Explanation: ***Hydrocortisone and fludrocortisone therapy*** - The newborn presents with **clitoromegaly** and electrolyte abnormalities including **hyponatremia** and **hyperkalemia**, which are characteristic findings of **salt-wasting congenital adrenal hyperplasia (CAH)**. - CAH is caused by a deficiency in 21-hydroxylase enzyme, leading to inadequate production of cortisol and aldosterone. **Hydrocortisone** (glucocorticoid) and **fludrocortisone** (mineralocorticoid) are essential for replacing these deficient hormones and preventing adrenal crisis. *Spironolactone therapy* - **Spironolactone** is an **aldosterone antagonist** and would worsen the existing salt-wasting state and hyperkalemia seen in CAH. - It works by blocking aldosterone, leading to increased sodium excretion and potassium retention, which is the opposite of what is needed in CAH. *Estrogen replacement therapy* - **Estrogen replacement therapy** is not indicated at this stage. It would not address the underlying hormonal deficiencies (cortisol and aldosterone) or correct the electrolyte imbalances in CAH. - Estrogen is involved in female sexual development but does not play a primary role in the acute management of adrenal insufficiency in newborns with CAH. *Dexamethasone therapy* - **Dexamethasone** is a potent **glucocorticoid**, but it is generally not the first-line treatment for chronic management in infants with CAH due to its prolonged half-life and higher risk of growth suppression compared to hydrocortisone. - While it could address the cortisol deficiency, it does not provide mineralocorticoid activity, which is crucial for managing the salt-wasting component. *Genital reconstruction surgery* - **Genital reconstruction surgery** may be considered later in life for cosmetic or functional reasons, but it is not the immediate or most appropriate next step in managing a newborn with CAH. - The immediate priority is to stabilize the child's hormonal and electrolyte balance to prevent potentially life-threatening adrenal crisis.

Kawasaki disease Flashcard 1 of 7

Question: Patients with Kawasaki disease may develop coronary artery _____, which can rupture; therefore, get a(n) _____

Answer: aneurysms

Extra Information:  https://onlinemeded.org?ref=anki 

Kawasaki disease Flashcard 2 of 7

Question: What is the mnemonic for the criteria for Kawasaki disease? _____

Answer: Conjunctival injection Rash Adenopathy (cervical) Strawberry tongue Hand-foot changes fever

Extra Information:  https://onlinemeded.org/spa/rheumatology/vasculitis/acquire?ref=anki 

Kawasaki disease Flashcard 3 of 7

Question: Treatment for Kawasaki disease includes _____ and _____

Answer: aspirin

Extra Information:   Watch Large & Medium Vessel Vasculitides [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-vascular/videos/medical-pathophysiology-vascular-vasculitides-large-and-medium-vessel-vasculitides?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/surgery-subspecialty/vascular/acquire?ref=anki 

Kawasaki disease Flashcard 4 of 7

Question: Patients with Kawasaki disease may develop coronary artery _____, which can rupture; therefore, get a(n) _____

Answer: aneurysms

Extra Information:   Watch Large & Medium Vessel Vasculitides [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-vascular/videos/medical-pathophysiology-vascular-vasculitides-large-and-medium-vessel-vasculitides?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/surgery-subspecialty/vascular/acquire?ref=anki 

Kawasaki disease Flashcard 5 of 7

Question: What demographic is classically affected by Kawasaki disease? _____

Answer: Asian children < 4 years old

Kawasaki disease Flashcard 6 of 7

Question: Patients with Kawasaki disease may develop coronary artery _____, which can lead to myocardial infarction

Answer: thrombosis

Kawasaki disease Flashcard 7 of 7

Question: What vasculitis is treated with aspirin? _____

Answer: Kawasaki disease