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Just to add to what some others have said, this is in First Aid as well. FA 2020 page 85:
Organic acidemias most commonly present in infancy with poor feeding, vomiting, hypotonia, a high anion gap metabolic acidosis, hepatomegaly, and seizures.
The organic acid accumulation does 2 things:
Organic acids inhibit gluconeogenesis, leading to fasting hypoglycemia which then causes ketoacidosis, causing a high AG metabolic acidosis
Organic acids inhibit the urea cycle, leading to hyperammonemia
C’nta phle mhcu on the tcxea einasngor yw,h ubt ereht ear a wfe oWrdlU enqossuit no itsh rehwe fi a eotenna hsa y,mpoeyahclgi ksoetis dan mrypemeaani,hmo a caorngi diac sdrdieor huosld be cdusestep rppo(inoci dcia ro neohmimlyclat d)ai.c ssLe uossiucspi fo na ATR usace ychygmialpeo si ont hsecaticarticr of t.tha
Ornithine transcarbamylase deficiency. Most common urea cycle disorder. X-linked recessive (vs other urea cycle enzyme deficiencies, which are autosomal recessive).
Interferes with the body’s ability to eliminate ammonia.
Often evident in the first few days of life, but may present later. Excess carbamoyl phosphate is converted to orotic acid (part of the pyrimidine synthesis pathway).
Findings: Increased orotic acid in blood and urine, Decreased BUN, symptoms of hyperammonemia. No megaloblastic anemia (vs orotic aciduria).
My reasoning on this questions was simpler - I think they wanted us to pick an autosomal recessive disease based on pattern of kids affected (both females are affected - can't be anything X-linked); boy was healthy - rule out mitochondrial. With rest - rule out because of increased ammonia and infantile presentation. Only organic acid metabolism disorder left