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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.


hyperkalemic periodic paralysis.
References in periodicals archive ?
Mexican scientists had set out to check the effects of HPP processing on a key group of antioxidants--the carotenoids--using the pulp of avocado, papaya and mango, which was subjected to HPP for three minutes.
As per K-P government documents, the PEDO was supplying the electricity to the consumers at Rs3 per kilowatt-hour from Reshun HPP when the flood destroyed the power house of the HPP and disconnected them.
It was noted that with the construction of new hydro power plants on the main tributaries of these rivers, a significant decrease in the amount of sediment entering the reservoir of the HPP of the Vakhsh cascade, including the Rogun HPP, should be expected.
HPP is caused by mutations in the gene encoding an enzyme known as tissue non-specific alkaline phosphatase (TNSALP) that plays a critical role in the proper mineralization of bones.
Overall, Spectracron AdvantEdge HPP primer helps our customers gain a competitive edge by providing exceptional performance in a heavy-metal-free formulation," said Scott Laney, PPG business development manager, industrial coatings.
A base engine model was used employing a calibration based on the series ECU calibration, due to the fact that the main focus of the investigations was set on the behavior of the injection system and the fundamental functionality of the HPP under high fuel temperatures.
7,10 The main treatment of HPP involves control of hyperthyroidism using antithyroid drugs, radioiodine ablation or thyroidectomy.
Originally HPP was thought to be a different disease than hereditary elliptocytosis (HE).
Joining Glenn is a team of industry founders and veterans with over 50 years of combined HPP experience.
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.
The Upper Naryn Cascade consists of four hydropower plants: Ak-Bulun HPP, Naryn HPP - 1, Naryn HPP - 2 and Naryn HPP - 3 with a total capacity of 237 MW.