Pathophysiology
The mechanism of Hermansky–Pudlak syndrome indicates that platelets in affected individuals accumulate abnormally with thrombin, epinephrine, and adenosine diphosphate, furthermore platelets in these individuals have a lower amount of dense bodies

Diagnosis
The diagnosis of HPS is established by clinical findings of hypopigmentation
of the skin and hair, characteristic eye findings, and demonstration of absent
dense bodies on whole mount electron microscopy of platelets. Molecular
genetic testing of the HPS1 gene is available on a clinical basis for
individuals from northwestern Puerto Rico. Molecular testing of the HPS3 gene
is available on a clinical basis for individuals of central Puerto Rican or
Ashkenazi Jewish heritage. Sequence analysis is available on a clinical basis
for mutations in HPS1 and HPS4. Diagnosis of individuals with other types of
HPS is available on a research basis only. Treatment
While there is no cure for HPS, treatment for chronic hemorrhages associated with the disorder includes therapy with vitamin E and the antidiuretic dDAVP. Considerations for patients
A preoperative pulmonology consultation is needed. The anesthesia team should
be aware that patients may have postoperative pulmonary complications as part
of the syndrome.Preoperative hematology consultation is advisable prior to elective ocular
surgeries. Since patients with the syndrome have bleeding tendencies,
intraoperative, perioperative, and postoperative hemorrhages should be
prevented and treated. If platelet aggregation improves with desmopressin, it
may be administered in the preoperative period. However, sometimes
plasmapheresis is needed in the perioperative period.Ophthalmologists should try to avoid retrobulbar blocks in patients with the
syndrome. Whenever possible, patients with HPS may benefit from general
endotracheal anesthesia. Phacoemulsification may help prevent intraoperative
and postoperative bleeding in patients with the syndrome.
12 is one of the two sublime numbers, with the other being 76 digits long. List of basic calculations
In science
The number of polynucleotide strands in a DNA double helix. The first magic number. The atomic number of helium. The ASCII code of "Start of Text". 2 Pallas, a large asteroid in the main belt and the second asteroid ever to be discovered. The Roman numeral II (usually) stands for the second-discovered satellite of a planet or minor planet (e.g. Pluto II or (87) Sylvia II Remus). A binary star is a stellar system consisting of two stars orbiting around their center of mass. The number of brain and cerebellar hemispheres. In sports
The number of points scored on a safety in American football
A field goal inside the three-point line is worth two points in basketball. The two in basketball is called the Shooting Guard
2 represents the catcher position in baseball. Other
In pre-1972 Indonesian and Malay orthography, 2 was shorthand for the reduplication that forms plurals: orang (person), orang-orang or orang2 (people). In Astrology, Taurus is the second sign of the Zodiac. For Pythagorean numerology (a pseudoscience) the number 2 represents duality, the positive and negative poles that come into balance and seek harmony. See also
List of highways numbered 2
Binary number

References
External links

Prime curiosities: 2