A 23 years old female presented to the ER with a severe central abdominal pain associated with bilious vomiting. There was no significant past medical history, with no surgeries in the past. She is on contraceptive pills which she started recently She does not smoke, and drink alcohol occasionally. Her Father has a history of epilepsy, and her mother is diabetic.
On physical examination she was distressed secondary to severe pain. Afebrile, BP 178/105, HR 122. And RR 16. The abdomen was diffusely very tenderl, and the bowel sounds were present. The rest of the physical exam was normal.
The patient was admitted to the general medical floor and started on Intravenous normal saline, metoclopramide for the vomiting, and morphine for her abdominal pain. Next morning the nurse noticed while she was trying to insert a urinary catheter that the patient has weakness of both lower extremities. and called the intern. The intern noticed that she has left shoulder weakness exam and during the neurological exam she had a grand mal epeliptic seizure and transferred to the intensive care unit.
Laboratory evaluation shows WBC 17X10⁹/l, Hemoglobin 14 g/dl, Platelets 390x 10⁹/l, Sodium 123 meq/L, Potassium 3 meq/L, Chloride 102 meq/L Bicarb 22 meq/L Urea 15 mg/dL, serum Creatinine 1.1 mg/dL, Serum glucose 78 mg/dL Calcium 10 mg/dL, Billirubin 0.9 mg/dL, and AST, ALT 42 U/l, 44 U/L consequently
Her chest x-ray showed no evidence of acute changes. CT of the Brain and Abdomen were both normal
What is the most likely diagnosis?
How would you confirm your diagnosis?
How would you manage this patient?
How would you manage her Seizures?
Posts
6 comments:
hypercagulable state(oral contraceptive use)--> mesentric ischemia--> sepsis + hyponatremia causing seizures and neurodeficits.
Though the imaging studies ct abd/brain were normal would like imaging with contrast and Anticoagulation based on work up and imaging studies.
also gradual sodium correction.
1)I think she might have had mesentric vein thrombosis due to her OCP which presented with pain and vomitings.
2) Ct abdomen and Angigraphy will be helpful for diagnosis
3)Treatment with antithrombolytics but she should be taken for laprotomy if any infarction present.
4)Her seizures can be secondary to hyponatremia, should be treated with symptomatic treatment for seizures if uncontrolled and correction of sodium should be with 3% saline as she has symtoms but not more than 4-6meq/l in 2-3 hours.
1)porphyria, precipitated by OCP.
2) urinary ALA, PBG, and porphyrin concentrations
3)symptomatic tratment, D/C OCP, Water restriction for hyponatremia, Iv heme prepration.
4) no need of Dilantin unless it recurs.
for acute attack of porphyria, we can also use glucose infusion and cimetadine. Starving usually precepitates porphyria, while glucose infusion will treat attacks.
4) for seizure, initially use lorazepam. if recurs than dilantin. There is very nice article on porphyria's in NEJM in june 2008. it explains everything. I will leave that edition of NEJM in resident lounge, if anyone wants to read it.
1-The most likely clinical diagnosis is acute intermittent porphyria
2- To confirm your clinical diagnosis you have to check:
1- urinary PBG.
11- Erythrocyte PBG deaminase
3- You should:
a-Stop oral contraceptive pills and metoclopramide
b- High carbohydrate diet ,
c- IV D50
d-IV Hematin
e- Beta-blockers for HTN and tachycardia
4- IV Chlormethazole and diazepam
The key here is the combination of abdominal pain ,neurological finding, hypertension in a young female patient.
It is an autosomal dominant ,a family history is usually present, in this case her father most likely had the disease and probably had seizure secondary to AIP, Symptoms begins usually in the twenties in young women, and it is rare after menopause.
Remember ,in AIP there is no skin photosensitivity.
In this case the attack precipitated by OCP ,and metoclopramide made it worse.
Profound hyponatrenia usually occurs and for this patient it caused seizures. The eplipesy in this case is managed by trying to abate the condition with high caloric intake and by fluid restriction to prevent hyponatremia secondary to SIADH.It is not easy to find anti-epeliptic not known to cause AIP. Chlormethazole is safe relatively.
Absenense of fever goes against sepsis. Leukocytosis in this case most likely secondary to stress and dehydration.
Post a Comment