- Abderhalden-Kaufmann-Lignac syndrome.

"Abderhalden-Kaufmann-Lignac syndrome"

"cystinosis"

-Def:- it is an autosomal recessive disorder involving deposits of cystine crystals in lysosomes of liver,spleen,bone marrow,cornea, and renal tubules due to abnormal metabolism of cystine .

-Incidence :- 1 : 100,000- 1 :200,000.

-It is the most common cause of Fanconi syndrome in the pediatric age group.

-Clinical picture:-

- Fanconi's syndrome.

"Fanconi's syndromes"

* There are many types of Fanconi's syndrome according to
etiology :-

-Primary type

-Hereditary:-

• Abderhalden-Kaufmann-Lignac syndrome "cystinosis" (click here).
• Oculo-cerebro-renal Syndrome (Lowe's Syndrome) (click here).
• Galactosemia.
• Wilson disease.

-Drug induced:-

• Out dated tetracycline.
• Cyclosporin.
• Heavy metals (e.g lead poisoning).

*Fanconi's syndrome ( primary type)*

-Def:- it is an autosomal recessive disorder due to multiple defects in proximal renal tubules which decrease the urinary re-absorption of phosphate,bicarbonate,glucose, amino acids and may be Potassium ( they all are lost in urine).

-It is named after Guido Fanconi, a Swiss pediatrician.

-Clinical picture:-

- What is mesothelioma?

What is mesothelioma?

Mary Hesdorffer, MS, APRN, nurse practitioner at the Mesothelioma Applied Research Foundation explains mesothelioma.

- Complete blood count exams.

Some examples of Complete blood count exams

from Mansoura University Children Hospital

If you know the answer ...post it in a comment

CBC 1

CBC 2

CBC 3

CBC4

CBC 5

CBC 6

Dr Ibrahim

- Grading of Vesicoureteric Reflux.

International Classification of Vesicoureteric  Reflux.

•  Grade I: – reflux into lower part of a  non-dilated ureter.
•  Grade II: – reflux into the renal pelvis and calyces without dilatation
•  Grade III: – reflux into a mildly dilated ureter, renal pelvis and calyces with minimal blunting of the fornices
•  Grade IV :– marked dilaed ureter.
•  Grade V:– massive reflux with gross dilatation of the ureter, pelvis and calyces; ureteral tortuosity; loss of papillary impressions.

- N.B:-

*In grades I,II ureter isn't dilated.

Return to list of medical grades (click here)