There have been several articles about aHUS Patient Registries on this website over the years. Patient Registries are an important part of understanding and finding a solution to rare diseases over time. Few rare diseases have Patient Registries.
aHUS benefits from having several Patient Registries.
Recently some research about aHUS Registries themselves was published. It was featured in Article No 438. The research was led by Prof. Nakysa Hooman from the Iran University of Medical Sciences. Her group looked at the scope and characteristics of 10 separate aHUS/TMA registries.
Prof. Nakysa Hooman
Nakysa has informed the aHUS alliance that a Registry has now been created for Iranian aHUS patients and recruitment has begun. It has a website which can be accessed using this link HERE.
The aim and scope of the Registry is given below.
The Iranian Haemolytic Uremic Syndrome Registry (IRI.HUS.REG) collects information on cases of this condition reported across the country. Data are recorded systematically, continuously, and longitudinally. IRI.HUS.REG addresses the major open scientific and clinical issues and aims to contribute to understand the etiology, clinical presentation, and the recurrence rate, early detect the preventive form or curable form of the disease, to evaluate genetically, to assess the medical management in order to reduce the morbidity mortality. We aim to improve the outcome of the children with HUS to have better quality of life and reduce the family burden. The registry is operated by the Aliasghar Childen Hospital(AACH) affiliated by Iran University of Medical Sciences(IUMS). Cases of the disease are mainly reported by the pediatric and adult nephrology centers participating in the registry.
With a population of over 85 million there could be upwards of 170 aHUS patients in Iran and perhaps around 40 new patients could onset each year. In time the differences and similarities with aHUS patients both within Iran and elsewhere in the world will be known.
Another aHUS Registry that aHUS alliance has been watching is that run by the European kidney organisation ERKNet. aHUS patients are recruited into its Thrombotic Microangiopathy thematic group.
Currently , of the approaching 12, 000 kidney patients in the Registry, the TMA Group has enrolled 386 children and 74 adults, of which 312 and 37 respectively are now actively being followed up. So it continues to grow albeit it remains more of a pediatric registry than adult. There are three patient representatives on the TMA thematic group governance but as far as is known none has ever communicated anything about what they do to patients.
Article No. 461