-Duhin-Johnson syndrome.

Duhin-Johnson syndrome.

It is Autosomal recessive disease characterized by Partial defect in excretion of conjugated bilirubin into the bile.

*Clinical picture:-It is usually asymptomatic.

1. Jaundice.
2. Good general condition.

*Investigations:-

1. Bilirubin: mild elevation of direct bilirubin
2. Biopsy (dark pigment inside hepatocytes i.e the liver is black in colour), it had been proved to be a metabolite of catecholamines.
3. +ve Bromosulphthalein (BSP) Test: Normally 90% of this dye is removed from blood within 45 minutes after I.V injection in normal liver.
In Dubin-Johnson syndrome there is a late rise in plasma SSP after an initial fall due to reflux of the dye from the liver reflecting hepatic excretion defect.
4. No evidence of obstruction.
5. No evidence of LCF.

*Treatment:-

No treatment (almost normal life span).

Dr Ibrahim

Return to list of medical syndromes here

- Classification of Graves' eye disease.

Classification of Graves' eye disease. 

"NO SPECS"

• Class 0: No signs or symptoms.
• Class 1: Only signs (limited to upper lid retraction and stare, with or without lid lag).
• Class 2: Soft tissue involvement (oedema of conjunctivae and lids, conjunctival injection, etc.).
• Class 3: Proptosis.
• Class 4: Extraocular muscle involvement.
• Class 5: Corneal involvement.
• Class 6: Sight loss (due to optic nerve involvement).

Enjoy,,

Dr Ibrahim

- Source:-

• Werner SC. Modification of the classification of the eye changes of Graves' disease: recommendations of the Ad Hoc Committee of the American Thyroid Association. J Clin Endocrinol Metab 1977;44: 203-4.

- Local causes of complicated cataract.

Local causes of complicated cataract.

"Rigid"

• long standing Retinal detachment
• Inflammatory conditions:- chronic iridocyclitis,chorioretinitis
• Glaucoma.
• Intraocular tumors
• Degenerative conditions:- retnitis pigmentosa, degenerative myopia. 
local Drugs:- corticosteroids, pilocarpine, adrenaline eye drops.

Mechanism:-

• Interference with lens metabolism.
• The posterior cortex is affected early,as the posterior capsule is thin and devoid of sub capsular epithelium.

Enjoy,,,

Dr Ibrahim

- Structures of angle of Anterior chamber.

Structures of angle of Anterior chamber.

"I Can See Till Schwalbe's Line"

• Iris root.
• Cilliary Body.
• Scleral spur.
• Trabecular Meshwork.
• Schwalbe's Line.

These Structures are seen by gonioscopy.

Enjoy,,,

Dr Ibrahim

- Arterial line placement.

Arterial line placement.

An intra-arterial catheter (A-line) is a very small plastic tube (called catheter) placed in one of your blood vessels (an artery) by highly trained personnel. This is usually done during or before certain types of surgery or in the Intensive Care Unit.

To see how to undergo Arterial line see the following video from New Engeland Journal of Medicine.

you can see the video online on NEJM here or

Download it directly from here 

  or watch it now :D