25/10/2025
Academic Discussion
ā§Šā§¨ āĻŦāĻāϰ āĻŦā§āϏ⧠āĻāĻ āϤāϰā§āĻŖā§, āĻāĻžāĻāϰāĻŋāĻā§āĻŦā§āĨ¤ āĻšāĻ āĻžā§ āĻāĻāĻĻāĻŋāύ āĻĒā§āϰāĻāĻŖā§āĻĄ āĻĒā§āĻ āĻŦā§āϝāĻĨāĻž āύāĻŋā§ā§ āĻā§āĻŽā§āĻŦāĻžāϰ⧠āĻāϞā§āύāĨ¤
āĻŦā§āϝāĻĨāĻž āϤā§āĻŦā§āϰ, āĻāĻŋāύā§āϤ⧠abdominal tenderness āύā§āĻ, ultrasound normalāĨ¤ āϰā§āĻā§āϰ āĻāĻžāώā§āϝāĻŽāϤ⧠āĻŦā§āϝāĻĨāĻž āĻāĻŽāύ āϝā§āύ āĻļāϰā§āϰā§āϰ āĻā§āϤāϰ āĻāĻā§āύ āϧāϰā§āĻā§āĨ¤ āϏāĻžāĻĨā§ āĻāĻŋāϞ:
āĻŦāĻŽāĻŋ, āĻā§āώā§āĻ āĻāĻžāĻ āĻŋāύā§āϝ
āĻšāĻžāϤ-āĻĒāĻž āĻāĻŋāύāĻāĻŋāύ āĻāϰāĻž
Stress, Anxiety
āĻĒā§āϰāĻĨāĻŽā§ āĻāĻžāĻŦāĻž āĻšāϞ⧠â gastritis, gallstone, āĻŦāĻž IBS!
āĻāĻŋāύā§āϤ⧠āĻĒāϰā§āĻā§āώāĻžā§ āĻāĻŋāĻā§āĻ āϧāϰāĻž āĻĒā§āϞ āύāĻžāĨ¤
đ âUrine test āĻĢāϞāĻžāĻĢāϞ? Urine āĻĒā§āϰāĻĨāĻŽā§ āĻšāĻžāϞāĻāĻž āĻšāϞā§āĻĻ, āĻāĻŋāύā§āϤ⧠āĻā§ā§āĻ āĻāĻŖā§āĻāĻž āĻĒāϰā§āĻ āĻāĻžā§ āϞāĻžāϞāĻā§-āĻŦāĻžāĻĻāĻžāĻŽā§ āĻšā§ā§ āĻā§āϞ! đ¯(Porphobilinogen â Porphobilin â Oxidized pigments)
āĻāĻāĻĻāĻŽ āĻā§āϞāĻžāϏāĻŋāĻ clueāĨ¤
āϤāĻžāϰāĻĒāϰ Urinary porphobilinogen (PBG) āĻ Î´-ALA āĻĒāϰā§āĻā§āώāĻž āĻāϰ⧠āĻĻā§āĻāĻž āĻā§āϞ Level High!!
Diagnosis? â Acute Intermittent Porphyria (AIP)
AIP āĻšāϞ⧠Heme synthesis pathway-āĻāϰ āĻāĻāĻāĻŋ enzyme deficiency, āϝā§āĻāĻžāύ⧠Porphobilinogen deaminase āĻ āĻŋāĻāĻāĻžāĻŦā§ āĻāĻžāĻ āĻāϰ⧠āύāĻžāĨ¤
āĻĢāϞāĻžāĻĢāϞ:
āĻļāϰā§āϰ⧠ALA āĻ PBG āĻāϰ Level āĻŦā§ā§ā§ āϝāĻžā§āĨ¤ āϝā§āĻā§āϞ⧠Autonomic nervous system āĻāĻŦāĻ CNS āĻā§ stimulate āĻāϰā§āĨ¤ āĻĢāϞ⧠abdominal pain, neurological āĻāĻŦāĻ psychiatric symptoms āĻĻā§āĻāĻž āĻĻā§ā§āĨ¤
â ī¸ Triggering Factors
Certain drugs (barbiturates, sulfadrugs, antiepileptics)
Alcohol
Fasting āĻŦāĻž crash dieting
Hormonal changes (menstrual cycle)
Stress, infection
đ Treatment
â
High carbohydrate (10% glucose infusion) â inhibits ALA synthase
â
Hemin therapy â sends negative feedback to the liver & controls heme pathway!
â
Trigger āĻā§āĻŋā§ā§ āĻāϞāĻž āĻā§āĻŦāĻ āĻā§āϰā§āϤā§āĻŦāĻĒā§āϰā§āĻŖāĨ¤
â
Control pain & nausea with supportive care
āϰā§āĻā§āĻā§ glucose infusion āĻ supportive care āĻĻā§āĻā§āĻžāϰ āĻĒāϰ āϤāĻŋāύ āĻĻāĻŋāύā§āϰ āĻŽāϧā§āϝā§āĻ āϤāĻžāϰ āĻŦā§āϝāĻĨāĻž āĻ
āύā§āĻāĻāĻžāĻ āĻāĻŽā§ āϝāĻžā§āĨ¤ āĻĒāϰāĻŦāϰā§āϤā§āϤ⧠triggering factors āϏāĻŽā§āĻĒāϰā§āĻā§ āϏāĻā§āϤāύ āĻāϰāĻž āĻšā§āĨ¤
At a glance:
Disease: Intermittent Porphyria (AIP)
Enzyme deficiency: Porphobilinogen deaminase
Inheritance: Autosomal Dominant
Accumulated substances: δ-ALA, Porphobilinogen
Symptoms: Abdominal pain, vomiting, neuropathy, psychiatric changes, dark urine (after exposure to light)
Treatment: Glucose + Hemin
Avoid: Certain drugs, alcohol, fasting, stress
đ§Š
AIP āĻāĻāĻāĻŋ âgreat masqueraderâ
āϤāĻžāĻ āϝāĻāύ āĻĒā§āĻāĻŦā§āϝāĻĨāĻž āϤā§āĻŦā§āϰ, āĻāĻŋāύā§āϤ⧠āĻĒāϰā§āĻā§āώāĻž-āύāĻŋāϰā§āĻā§āώāĻž āϏāĻŦ āϏā§āĻŦāĻžāĻāĻžāĻŦāĻŋāĻâ Think about AIP!!
đŠē Dr. Monirul Haque
MBBS, FCPS (FP)
MRCP(UK)- PACES
Khulna Medical College & Hospital.