GpIIb/IIIa is essential to blood coagulation since the activated receptor has the ability to bind fibrinogen (as well as von Willebrand factor, fibronectin, and vitronectin), which is required for fibrinogen-dependent platelet-platelet interaction (aggregation). Understanding of the role of GpIIb/IIIa in Glanzmanns thrombasthenia led to the development of GpIIb/IIIa inhibitors, a class of powerful antiplatelet agents. Diagnosis
Light transmission aggregometry is widely accepted as the gold standard diagnostic tool for assessing platelet function, and a result of absent aggregation with any agonist except ristocetin is highly specific for Glanzmanns thrombasthenia. Following is a table comparing its result with other platelet aggregation disorders:

Treatment
Therapy involves both preventive measures and treatment of specific bleeding episodes. Dental hygiene lessens gingival bleeding
Avoidance of antiplatelet agents such as aspirin and other anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, and anticoagulants
Iron or folate supplementation may be necessary if excessive or prolonged bleeding has caused anemia
Hepatitis B vaccine
Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid (Amicar)
Desmopressin (DDAVP) does not normalize the bleeding time in Glanzmanns thrombasthenia but anecdotally improves hemostasis
Hormonal contraceptives to control excessive menstrual bleeding
Topical agents such as gelfoam, fibrin sealants, polyethylene glycol polymers, custom dental splints
Platelet transfusions (only if bleeding is severe; risk of platelet alloimmunization)
Recombinant factor VIIa, AryoSeven or NovoSeven FDA approved this drug for the treatment of the disease in July 2014. Hematopoietic stem cell transplantation (HSCT) for severe recurrent hemorrhages

Eponym
It is named after Eduard Glanzmann (1887-1959), the Swiss pediatrician who originally described it.
Angiotensin converting enzyme inhibitors are thought to be beneficial, as they reduce intraglomerular pressure and, presumably, reduce renal tubular congestion with RBCs.Possible treatment regimens

General
Angiotensin inhibition
Reduce the risk of nephrolithiasis

Pain control
Opioid therapy
Inpatient therapy
Intravenous opioid regimen
Antiemetic drugs
Pruritus management
Maintenance therapy between pain exacerbations

Invasive therapy
Implantable drug delivery system
Surgical renal denervation
Renal autotransplantation
Nephrectomy (NOT recommended)
Other invasive therapies (but not proven effective)
Pulse radio frequency
Celiac plexus block
Intraureteric capsaicin infusionSurgery (autotransplantation) is thought by some to be of benefit in selected individuals and advocated in some centres, but usually considered the last resort.Physicians discourage surgery, as LPHS symptoms often re-occur after autotransplantation. Epidemiology
LPHS is listed as a rare disease in the US National Institute of Health Rare Diseases database. While exact numbers worldwide are not available, the primary LPHS research clinic located in Ohio has over 200 patients. In addition, several hundred other patients have been reported in one study as of 2006. The prevalence of LPHS is estimated  at about 0.012 percent, which qualifies LPHS as a rare disease (prevalence less than 0.07 percent) according to the Rare Diseases Act of 2002. Those affected are usually young, with an average age of 31 years, and 70% to 80% are women. See also
Pelvic pain

References
External links

Loin Pain - Patient UK