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Archiver > Hypophosphatasia > 1998-08 > 0902846444


From: "Margery Callen" <>
Subject: [Hypophosphatasia-L] Wheeless' Textbook Main Menu Home Page on Hypophosphatasia
Date: Tue, 11 Aug 1998 10:40:44 -0400


Good Morning Everyone: Message from Marge

I found this on the Internet—thought it was interesting as this is from
Wheeless' Textbook of Orthopaedics giving their description of
Hypophosphatasia — notice Del that femoral pseudofractures are listed and I
believe that you and I have this difficulty — thank goodness we do not have
all the symptoms at once!

Hypophosphatasia:

------------------------------------------------------------------------

- Discussion:
- rare form of rickets which is often hereditary (autosomal
recessive);
- characterized by reduction in alkaline phosphatase;
- premature loss of teeth is common in children and adults;
- skeletal changes:
- may present in infants, children, or may be delayed until
adulthood;
- radiographs show an excessive amount of unmemeralized osteoid
tissue (similar to rickets);
- infants:
- hypercalcemia may occur;
- this often needs to be treated w/ calcium chealing
agents or renal dialysis;
- functional craniosynostosis may raise intracranial pressure;
- early mortality may be as high as 50%;
- children:
- short sature and frontal bossing is common;
- calcium and phosphate levels are usually normal;
- on radiographs, look for radiolucent tongues extending from
the growth
plates into the metaphysis;
- adults:
- femoral pseudofractures and chondrocalcinosis is common;
- calcium and phosphate levels are usually normal

- femoral pseudofractures and chondrocalcinosis is common;
- calcium and phosphate levels are usually normal;

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