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Looking for ideas on the following case:

5 year old otherwise healthy male with a one and a half year history of
intermittent severe, colicky, incapacitating abdominal pain.
Occasionally associated with non-bilious vomiting. Occurs about once a
month and does not appear food related. Child was found to have
bilateral hydronephrosis prenatally. PE is normal. Weight is about 10th
percentile. Patient was admitted with an episode of gross hematuria one
month ago, without pain, which quickly resolved. Negative workup has
included the following: Upper GI, Barium Enema; IVP, non-contrast abd
CT, and renal ultrasound have shown progression of hydronephrosis on the
right, but no evidence of nephrolithiasis. CBC, electrolytes, lipase,
hepatic enzymes are normal. Stool studies negative. Urinalysis presently
notable for a ph of 8, otherwise normal. Peds GI and Peds Urology are on
the case.

I witnessed one of the episodes and they are quite impressive. The child
doubles up, grunts in excruciating pain with teeth clenched, hands in
fists, and extremities trembling, lasting about a minute followed by
persistent peri-umbilical pain. The child appears pale during the
episodes. He can communicate with you in between the spasms. The family
is appropriate and at the end of their rope. Abdominal migraine has been
suggested by the family and I find this dx lacking.

? Intermittent UPJ obstruction, SMA syndrome, porphyria ?

Thanks

Jay Fisher MD

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