Pseudohypoparathyroidism for MRCPCH clinical

BY

DR \ MohammedAyadMRCPCH

In which stations could it be ?

Always in OTHERS station as

Short stature or obesity or general

How could I suspect it ?

Short , obese , round face and may be slightly retarded

Clinical examination

1- GeneralObese , short , round face , decreased mentality ..Subcutaneous calcifications and delayed puberty .

2- handsKnucle knucle dimple dimple sign ( short 4th and 5th

metacarpals ) .Brachydactyly ( short fingers) may be unilateral or bilateral , UL or LL or both .Potters thumb sign ( short distal thumb phalanges )..

Elbow deformities ..

Cubitus valgus..

3- LL

Genu varum or valgus..

4- TEETHING PROBLEMS

I’d like to complete my examination by

1- elicit signs of hypocalcemia ( chovsk’s and traussau )..

2- testing hearing , vision and smell .

Diagnostic studies

Ca– PO4 – PTH –ALP – Vit d

Screening for associated pituitary disorders

X ray hands

Ct brain for basal ganglia calcification

ECG for prolonged QT

Geneting testing

Management

1- MDT

2- alpha calcidol and calcium

3- management of hypocalcemic crisis .