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Long chain acyl coa dehydrogenase deficiency nbme 16
Long chain acyl coa dehydrogenase deficiency nbme 16










This is different from the induration we see in delayed type IV hypersensitivity reactions where CD4 T cells release cytokines interferon gamma that promote T cell- and macrophage-mediated tissue damage because type IV hypersensitivity reactions develop over days rather than hours due to the time needed for cellular amplification.Ĭhron's causes transmural inflammation that can potentially cause perforation peritonitis and sepsis bc of spilled abdo contents. Cationic proteins major basic protein, eosinophil peroxidase released from eosinophils cause tissue damage, which usually manifests as a palpable, indurated lesion hours following the early-phase reaction. Late phase: IgE stimulates type 2 helper T cells to release cytokines IL-5 that activate eosinophils. The results superficial dermal edema and erythema wheal and flare reaction that can progress to a more systemic response anaphylaxis.

long chain acyl coa dehydrogenase deficiency nbme 16

Discussed in table FA'18 page This NBME question is outdated Based on a similar question in UWRLD these findings of urticaria followed by induration in a few hours are consistent with the early and late phases of a type I hypersensitivity reaction.Įarly phase: repeat exposure bound IgE cross-link and stimulate release of preformed histamine and leukotrienes that cause vasodilation and increased capillary permeability. Symptoms on the right side means that the lesion is contralateral in the brain due to decussation rules. Vibration Posterior column and pain spinothalamic both ultimately synapse in the VPL thalamus. I had no idea this was central Stroke Syndrome. Glucose can easily be crossed out because is not an electrolyte and the question asks for electrolyte changes.

long chain acyl coa dehydrogenase deficiency nbme 16

The low Naemia causes cerebral edema, which leads to seizures. It is more likely that the pt gets hyponatremic than hypochloremic considering that, before giving H2O, chloremia was high and Na was low. However, the parents give the infant only H2O for 24 hh, which means that Na and Cl get diluted. Due to secretory diarrhea, the pt has eunatremic hypovolemia with hyperchloremia. This kiddo has most likely secretory diarrhea there are no clues in the question that this is osmotic diarrhea. Alzheimer can be ruled out because pt is "pleasant and cooperative", whereas non-cognitive symptoms of Alzheimer include "aggression, irritability, agitation". Sphingomyelinase is an acidic lysosomal hydrolase. That's what I found: Sphingomyelinase is deficient in Niemann-Pick.












Long chain acyl coa dehydrogenase deficiency nbme 16