Fabry disease is an X-linked disorder due to mutations in alpha-galactosidase A gene. It affects the kidney in virtually all patients with classical and some late onset variants. Podocytes, endothelial cells, vascular smooth muscle, tubular and mesangial cells are involved in different ways. Proteinuria and chronic kidney disease are the result of the progressive accumulation of the enzyme substrates globotriaosylceramide (GB3) and lyso-GB3 in the cytoplasm of these cells (mainly in lysosomes), which leads to cellular and organ dysfunction and eventually renal failure and end-stage kidney disease. Specific enzyme replacement therapy and pharmacological chaperone are at present the main therapeutic approach. After enzyme infusion, the delivered enzyme is differentially uptaken by kidney cells in three different ways: By Mannose-6-phosphate receptor, megalin and sortilin. The delivered enzyme gradually clears cells from the accumulation of the glycosphingolipids and contributes to a cellular healthier status. The recent pandemic caused by SARS-CoV-2 has led to the collapse of health systems around the world and to thousands of deaths. Kidney involvement has been reported to range from proteinuria to acute kidney injury, 30% of which may require renal replacement therapy. In this review the potential causes for which Fabry patients should be at increased risk and the necessity not to discontinue therapy are discussed.

 

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Saturday, 24 October 2020 23:53

World Kidney Day on March 12, 2015 Kidney Health for All

World Kidney Day will be celebrated on March 12, 2015. This time the theme is on Kidney Health for All.

The theme of World Hypertension Day 2014: Know your Blood Pressure

Firstly World Hypertension Day inaugurated in May 2005 and has become an annual event ever since. The aim of the World Hypertension Day is to promote public alertness of high blood pressure and to promote citizens of all countries to prevent and control this silent killer, the modern epidemic.World Hypertension Day was initiated firstly by the World Hypertension League.High blood pressure (hypertension) is considered as the silent killer since it has no apparent symptoms. Investigations show that the disease involves more than 1.5 billion people global, and around seven million people die every year from hypertension. 

Chronic Kidney Disease-Mineral Bone Disorder (CKD-MBD)

The definition of CKD-MBD is different than the previously recognized as "renal osteodystrophy", and it refers to a systemic disorder of mineral and bone metabolism due to CKD manifested by either one or a combination of the following:
•Abnormalities of calcium, phosphorus, parathyroid hormone (PTH), or vitamin D metabolism.
•Abnormalities in bone turnover, mineralization, volume, linear growth, or strenght.
•Vascular or other soft-tissue calcification.
The term "renal osteodystrophy" is now limited to an alteration of bone morphology in patients with CKD, and it is one measure of the skeletal component of the systemic disorder of CKD-MBD that is quantifiable by histomorphometry of bone biopsy.

 

World Kidney Day 2014:chronic kidney disease and aging

World Kidney Day (WKD) is a joint initiative of the International Society of Nephrology (ISN) and the International Federation of Kidney Foundations (IFKF).Seven years on from the first campaign, World Kidney Day (WKD) has turned into a global phenomenon. On March 13, 2014, medical professionals, government officials, the general public, celebrities and patients will take action locally.
Implication for health policy/practice/research/medical education
In 2014 World Kidney Day (WKD) will focus on chronic kidney disease and aging.The mission of WKD is to raise awareness so that everyone cares for their kidneys and, if appropriate, check to assess if they are at risk for kidney disease. Prevention of kidney disease, early detection, and subsequent kidney protection are critical aims for World Kidney Day.
Our research group is working on the below projects
1-Oxford classification in Iranian IgA nephropathy patients [Renal pathology unite of Dr. Baradaranlaboratory, Isfahan, Iran].
2- Significance of C4d deposits in IgA nephropathy [Renal pathology unite of Dr. Baradaran laboratory, Isfahan, Iran].
3-Renal tubular cell protection by herbal anti-oxidants [Medical Plants Research Center; Shahrekord University of Medical Sciences, Shahrekord, Iran]
4-R229Q Polymorphism of NPHS2 Gene in Patients with Late-Onset Steroid-Resistance Nephrotic Syndrome [Chronic Kidney Disease Research Center; Tabriz University of Medical Sciences,Tabriz, Iran]

Recently Published Papers
Baradaran A. Antiphospholipid syndrome-associated nephropathy; a nephropathy needs classification. J Nephropharmacol. 2012; 1(1):7-9.
Implication for health policy/practice/research/medical education
A suggested classification for antiphospholipid syndrome-associated nephropathy, should be simple and practical. However, the suggestion of a new classification for antiphospholipid syndrome-associated nephropathy will involve a magnificent amount of work and will necessitate a working group, hence, more studies on this topic is suggested.

Nasri H. Antiphospholipid syndrome-associated nephropathy: Current concepts . J Ren Inj Prev 2013; 2(1): 1-2.
Implication for health policy/practice/research/medical education
Renal pathologists and nephrologists should be aware of the morphologic characteristics of APS-nephropathy when they reviewbiopsies of lupus nephropathy patients, especially those with positive antiphospholipid antibodies.

Mubarak M, Nasri H.What nephrolopathologists needto know about antiphospholipid syndrome-associated nephropathy: Is it time for formulating a classification for renal morphologic lesions? J Nephropathology. 2014; 3(1): 4-8.
Implication for health policy/practice/research/medical education
There is sufficient epidemiological, clinical and histopathological evidence to show that antiphospholipid syndrome-associated nephropathy is a distinctive lesion caused by antiphospholipid antibodies in patients with different forms of antiphospholipid syndrome. It is now time to devise a classification for an accurate diagnosis and prognostication of the disease.