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hereditary pyropoikilocytosis

An autosomal recessive disorder (OMIM:26614) characterised by haemolytic anaemia, microspherocytosis, poikilocytosis and an increased thermal sensitivity of red cells.

Molecular pathology
Caused by defects of SPTA1, which encodes an alpha erythrocyte spectrin and major scaffold protein that links the plasma membrane to the actin cytoskeleton.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
References in periodicals archive ?
Hypophosphatasia (HPP) is a rare, inherited metabolic bone disorder characterized by low serum alkaline phosphatase (ALP) activity and impaired bone mineralization [1].
When HPP is brought into mind in the differential diagnosis of patients who present with bone, teeth, and other systemic symptoms and findings, it is very easy to diagnose the disease by establishing the low ALP levels (5).
Concurrently, Dr Dahir and colleagues have entered into a partnership with the Biomechanics & Assistive Technology Laboratory at Vanderbilt University to use state-of-the-art 3D motion analysis equipment to test for abnormalities in gait and muscle weakness in adolescents and adults with HPP.
2 and Table 1, the crystallization behaviors of HPP, PP-B, and their blends are of great difference under the same crystal conditions.
HPP resolves when the patient becomes euthyroid so that the definitive treatment is either radioactive iodine or thyroidectomy.
Initial work-up revealed that a spectrin protein abnormality was the likely cause of the unusual erythrocyte thermal sensitivity and HPP erythrocytes are often deficient in this protein.
"HPP helps companies ensure food safety for refrigerated foods while dramatically increasing shelf life and enabling clean labeling," states Hewson.
Unlike pasteurisation, HPP doesn't damage any of the fruits' nutrients, retains more flavour and extends a juice's shelf life from 4-20 days to 40-90 days, as well as slashing the amount of waste generated.
Given the substantial defense costs HPP will face to defend the RHA litigation, whether its E&O policy is a liability policy will be critical to its bottom line.
HPP is a method of processing food and beverage products through a high-pressure chamber that can significantly extend product shelf life.
Soandaryan HPP, 171MW Shamb HPP and 157.2MW Tatev HPP) for $180mln.