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Galactosemia

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Galactosemia

MLT 215 Unit 3

MLT 215 Unit 3

Alkaptonuria
Disorders of carbohydrate metabolism: Pathology review
Disorders of amino acid metabolism: Pathology review
Cystinuria (NORD)
Homocystinuria
Hartnup disease
Maple syrup urine disease
Ornithine transcarbamylase deficiency
Phenylketonuria (NORD)
Essential fructosuria
Galactosemia
Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)
Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)
Purine and pyrimidine synthesis and metabolism disorders: Pathology review
Lesch-Nyhan syndrome
Lower urinary tract infection
Kidney stones
Alport syndrome
Goodpasture syndrome
IgA nephropathy (NORD)
Poststreptococcal glomerulonephritis
Diabetic nephropathy
Membranoproliferative glomerulonephritis
Minimal change disease
Acute pyelonephritis
Chronic pyelonephritis
Nephritic syndromes: Pathology review
Nephrotic syndromes: Pathology review
Urinary tract infections: Pathology review
Cystinosis

Key Takeaways

Galactosemia is an autosomal recessive disorder of galactose metabolism. People with galactosemia lack one of the enzymes that break down galactose, which results in the accumulation of certain toxins in the liver, brain, and eyes. Symptoms of galactosemia include diarrhea, vomiting, jaundice, and damage to the liver and brain..

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Flashcards

Galactosemia

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Questions

USMLE® Step 1 style questions USMLE

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A 1-month-old infant is brought to the emergency department because of failure to thrive and recurrent vomiting. The patient was born full-term at home via an uncomplicated vaginal delivery. Shortly after birth, the patient began experiencing nausea and vomiting after feedings. The patient was at the 50th percentile for weight at birth. At today’s visit, the patient is at the 25th percentile. Temperature is 36.9°C (98.4°F), pulse is 108/min, blood pressure is 77/40 mmHg, and respiratory rate is 56/min. Physical examination reveals jaundice and hepatomegaly. Fundoscopic examination reveals bilateral clouding of the lens. Urine dipstick is negative for glucose. Additional testing reveals the presence of reducing substances in the urine. Which of the following is the most likely diagnosis?