Cysteamine - Revision history

Prab at 12:09, 21 March 2025

2025-03-21T12:09:16Z

← Older revision		Revision as of 12:09, 21 March 2025
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	~~{{intro}}~~		[[File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal\|thumb]]
			[[File:Cysteamine 3D ball.png\|Cysteamine 3D Ball\|thumb]]
	Cysteamine is a simple aminothiol molecule that is used to treat nephropathic cystinosis, due to its ability to decrease the markedly elevated and toxic levels of intracellular cystine that occur in this disease and cause its major complications.		Cysteamine is a simple aminothiol molecule that is used to treat nephropathic cystinosis, due to its ability to decrease the markedly elevated and toxic levels of intracellular cystine that occur in this disease and cause its major complications.

	{{livtox}}		{{livtox}}
	Cysteamine has been associated with serum enzyme elevations when given intravenously in high doses, but it has not been shown to cause clinically apparent acute liver injury.		Cysteamine has been associated with serum enzyme elevations when given intravenously in high doses, but it has not been shown to cause clinically apparent acute liver injury.

	{{moa}}		{{moa}}
	Cysteamine (sis tee' a meen) is a simple aminothiol molecule which is used to treat nephropathic cystinosis, a rare autosomal recessive disorder characterized by progressive renal tubular dysfunction and growth retardation, and which eventually leads to renal failure and need for dialysis or renal transplantation often within the first decade of life. The underlying defect in cystinosis is a deficiency of an amino acid carrier that transports cystine out of lysosomes. The absence of this transporter causes accumulation of toxic levels of cystine in tissues. Those organs most susceptible to the toxic effects of cystine include the kidney, endocrine tissue, eyes and muscle. Cysteamine readily penetrates cells and is concentrated in lysosomes where it acts by converting intralysosomal cystine to cysteine which is readily transported out of lysosomes. This action leads to a decrease in cellular cystine levels and prevents the renal failure, growth retardation and many of the other chronic complications of nephropathic cystinosis. Cysteamine was approved for the therapy of cystinosis in the United States in 1994. It is now available in several forms including cysteamine bitartrate salt under the commercial name Cystagon (given every 6 hours), as well as a delayed release preparation under the name ProCysBi (given every 12 hours).		Cysteamine (sis tee' a meen) is a simple aminothiol molecule which is used to treat nephropathic cystinosis, a rare autosomal recessive disorder characterized by progressive renal tubular dysfunction and growth retardation, and which eventually leads to renal failure and need for dialysis or renal transplantation often within the first decade of life. The underlying defect in cystinosis is a deficiency of an amino acid carrier that transports cystine out of lysosomes. The absence of this transporter causes accumulation of toxic levels of cystine in tissues. Those organs most susceptible to the toxic effects of cystine include the kidney, endocrine tissue, eyes and muscle. Cysteamine readily penetrates cells and is concentrated in lysosomes where it acts by converting intralysosomal cystine to cysteine which is readily transported out of lysosomes. This action leads to a decrease in cellular cystine levels and prevents the renal failure, growth retardation and many of the other chronic complications of nephropathic cystinosis. Cysteamine was approved for the therapy of cystinosis in the United States in 1994. It is now available in several forms including cysteamine bitartrate salt under the commercial name Cystagon (given every 6 hours), as well as a delayed release preparation under the name ProCysBi (given every 12 hours).

	{{dose}}		{{dose}}
	The typical maintenance dose is 2.0 grams daily in adults and children above the age of 12, and 1.3 to 1.95 grams/m2 daily in younger children. Cysteamine is usually initiated using a fraction of the maintenance dose, with gradual dose escalation. Both preparations of cysteamine have an unpleasant taste and odor which makes compliance with taking the high doses required to control intracellular cystine levels in cystinosis a frequent problem.		The typical maintenance dose is 2.0 grams daily in adults and children above the age of 12, and 1.3 to 1.95 grams/m2 daily in younger children. Cysteamine is usually initiated using a fraction of the maintenance dose, with gradual dose escalation. Both preparations of cysteamine have an unpleasant taste and odor which makes compliance with taking the high doses required to control intracellular cystine levels in cystinosis a frequent problem.

	{{se}}		{{se}}
	Common side effects include nausea, anorexia, fatigue and diarrhea. Rare, but potentially severe adverse reactions include rash, skin fragility and benign intracranial hypertension. Joint hyperextension, leg pains, osteopenia, fractures and scoliosis also occur in cysteamine treated patients with cystinosis, but are more likely complications of the underlying disease than adverse effects due to cysteamine.		Common side effects include nausea, anorexia, fatigue and diarrhea. Rare, but potentially severe adverse reactions include rash, skin fragility and benign intracranial hypertension. Joint hyperextension, leg pains, osteopenia, fractures and scoliosis also occur in cysteamine treated patients with cystinosis, but are more likely complications of the underlying disease than adverse effects due to cysteamine.
	{{genetic agents}}		{{genetic agents}}
	{{coststubd}}		{{coststubd}}
	~~<gallery>~~
	~~File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal~~
	~~File:Cysteamine 3D ball.png\|Cysteamine 3D Ball~~
	~~</gallery>~~
	~~<gallery>~~
	~~File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal~~
	~~File:Cysteamine 3D ball.png\|Cysteamine 3D Ball~~
	~~</gallery>~~

Prab: CSV import

2025-02-20T00:59:40Z

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← Older revision		Revision as of 00:59, 20 February 2025
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			<gallery>
			File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal
			File:Cysteamine 3D ball.png\|Cysteamine 3D Ball
			</gallery>
	<gallery>		<gallery>
	File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal		File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal
	File:Cysteamine 3D ball.png\|Cysteamine 3D Ball		File:Cysteamine 3D ball.png\|Cysteamine 3D Ball
	</gallery>		</gallery>

Prab: CSV import

2025-02-20T00:56:34Z

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← Older revision		Revision as of 00:56, 20 February 2025
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			<gallery>
			File:Cysteamine-2D-skeletal.png\|Cysteamine 2D Skeletal
			File:Cysteamine 3D ball.png\|Cysteamine 3D Ball
			</gallery>

Prab: Created page with "{{intro}} Cysteamine is a simple aminothiol molecule that is used to treat nephropathic cystinosis, due to its ability to decrease the markedly elevated and toxic levels of in..."

2019-05-07T23:54:05Z

Created page with "{{intro}} Cysteamine is a simple aminothiol molecule that is used to treat nephropathic cystinosis, due to its ability to decrease the markedly elevated and toxic levels of in..."

New page

{{intro}}
Cysteamine is a simple aminothiol molecule that is used to treat nephropathic cystinosis, due to its ability to decrease the markedly elevated and toxic levels of intracellular cystine that occur in this disease and cause its major complications.

{{livtox}}
Cysteamine has been associated with serum enzyme elevations when given intravenously in high doses, but it has not been shown to cause clinically apparent acute liver injury.

{{moa}}
Cysteamine (sis tee' a meen) is a simple aminothiol molecule which is used to treat nephropathic cystinosis, a rare autosomal recessive disorder characterized by progressive renal tubular dysfunction and growth retardation, and which eventually leads to renal failure and need for dialysis or renal transplantation often within the first decade of life. The underlying defect in cystinosis is a deficiency of an amino acid carrier that transports cystine out of lysosomes. The absence of this transporter causes accumulation of toxic levels of cystine in tissues. Those organs most susceptible to the toxic effects of cystine include the kidney, endocrine tissue, eyes and muscle. Cysteamine readily penetrates cells and is concentrated in lysosomes where it acts by converting intralysosomal cystine to cysteine which is readily transported out of lysosomes. This action leads to a decrease in cellular cystine levels and prevents the renal failure, growth retardation and many of the other chronic complications of nephropathic cystinosis. Cysteamine was approved for the therapy of cystinosis in the United States in 1994. It is now available in several forms including cysteamine bitartrate salt under the commercial name Cystagon (given every 6 hours), as well as a delayed release preparation under the name ProCysBi (given every 12 hours).

{{dose}}
The typical maintenance dose is 2.0 grams daily in adults and children above the age of 12, and 1.3 to 1.95 grams/m2 daily in younger children. Cysteamine is usually initiated using a fraction of the maintenance dose, with gradual dose escalation. Both preparations of cysteamine have an unpleasant taste and odor which makes compliance with taking the high doses required to control intracellular cystine levels in cystinosis a frequent problem.

{{se}}
Common side effects include nausea, anorexia, fatigue and diarrhea. Rare, but potentially severe adverse reactions include rash, skin fragility and benign intracranial hypertension. Joint hyperextension, leg pains, osteopenia, fractures and scoliosis also occur in cysteamine treated patients with cystinosis, but are more likely complications of the underlying disease than adverse effects due to cysteamine.
{{genetic agents}}
{{coststubd}}