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Naglazyme®


yapımcı: BioMarin'in

içeren

Naglazyme®, mukopolisakkaridoz VI'ya karşı bir ilaçtır.

Aktif maddeler

galsulfase


uygulama

Naglazyme® tedavi etmek için kullanılır:

  • Çok nadir kalıtsal hastalık,mukopolisakkaridoz VIenzim eksikliği nedeniyle.

Sadece hastanelerde kullanılır.


dozaj

İnfüzyon için bir çözelti olarak, seyreltmeden sonra, bir damar halinde kullanılabilir. Doz bireyseldir ve hastanın ağırlığından hesaplanır.

Olası yan etkileri

Çok yaygın (% 10'un üzerinde)

İnfüzyon sırasında reaksiyonlar ve rahatsızlık *

karın bölgesinde ağrı

nefes darlığı

Kulak ağrısı

Ortak (% 1-10)

Gastrointestinal sistemin iltihaplanması

Yüksek tansiyon, Boğaz iltihabı, Solunum durması, Durdurulmuş burun

göğüs ağrısı

Reflekslerin yokluğu

Yüzde sıvı birikmesi

Belirsiz görüş, göz iltihabı

* İnfüzyon sırasında reaksiyon ve rahatsızlık ateş, titreme, deri döküntüsü, kurdeşen, kas ağrıları içerir.



gebelik

Bilgi eksikliğinden dolayı kullanılmamalıdır. Bu metin, şirketin onaylanmış ürün bilgisinden (ürün özeti) farklıdır. Bilgi eksikliğinden dolayı kullanılmamalıdır.Bu metin şirketin onaylı ürün bilgisinden (ürün özeti) farklıdır.

emzirme

Bilgi eksikliğinden dolayı kullanılmamalıdır.

trafik

Uyarı yok.

kan veren kimse

Düşürme. Donör, bağışçı köyü tarafından ilan edilmelidir.

etki

  • Vücutta hastalığında eksik olan enzim aynı şekilde bazı maddeleri ayrıştırarak çalışır.
  • Kandaki (T½) yarı ömür yaklaşık olarak 25 dakika.

İlaç şekilleri

İnfüzyon için çözelti için konsantre olun. 1 ml 1 mg galsülfaz içerir.


Özel uyarılar

Naglazyme® aşağıdaki durumlarda dikkatli kullanılmalıdır:
  • Solunum hastalıkları.


Hibeler, teslimat, paketler ve fiyatlar

sübvansiyonlariadeDağıtım şekli ve gücüambalajKr cinsinden fiyat
Sadece hastanelere ulaştırılabilir. A grubundaki ilaçlara yönelik hükümlere göre teslim edilir.infüzyon çözeltisi için konsantre, çözünme 1 mg / ml1 x 5 ml15.055,85



Naglazyme®

FAQ - 💬

❓ What is Naglazyme used for?

👉 The first and only treatment for MPS VI. NAGLAZYME® (galsulfase) is an enzyme replacement therapy for the treatment of mucopolysaccharidosis VI (MPS VI). Naglazyme provides a recombinant version of the enzyme missing in individuals diagnosed with MPS VI.

❓ How is Naglazyme made?

👉 NAGLAZYME is a formulation of galsulfase, which is a purified human enzyme that is produced by recombinant DNA technology in a Chinese hamster ovary cell line. Galsulfase (glycosaminoglycan N–acetylgalactosamine 4-sulfatase, EC 3.1.

❓ How much does Naglazyme cost?

👉 The cost for Naglazyme intravenous solution (1 mg/mL) is around $2,212 for a supply of 5 milliliters, depending on the pharmacy you visit....Intravenous Solution.

QuantityPer unitPrice
5 milliliters$442.48$2,212.38

❓ Is there a cure for mucopolysaccharidosis?

👉 There is no cure for MPS 1, however, there are effective treatments that are proven to slow the progression of disease. Hematopoietic stem cell transplantation (HSCT) can help some patients who have the severe form of the disease, and is generally recommended within the first 1-2 years of life.

❓ What is MPS VI?

👉 Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome, is a progressive condition that causes many tissues and organs to enlarge, become inflamed or scarred, and eventually waste away (atrophy). Skeletal abnormalities are also common in this condition.

❓ How much does Cinryze cost?

👉 The cost for Cinryze intravenous powder for injection 500 units is around $3,091 for a supply of 1 powder for injection, depending on the pharmacy you visit....Intravenous Powder For Injection.

QuantityPer unitPrice
1$3,091.42$3,091.42

❓ How long do people with MPS live?

👉 The life expectancy of these individuals is 10 to 20 years. Individuals with mild MPS II also have a shortened lifespan, but they typically live into adulthood and their intelligence is not affected. Heart disease and airway obstruction are major causes of death in people with both types of MPS II.

❓ What causes mucopolysaccharidosis?

👉 The disorder is caused by deficiency of the enzyme beta-glucuronidase. In its rarest form, MPS VII causes children to be born with hydrops fetalis, in which extreme amounts of fluid are retained in the body. Survival in these cases is usually a few months or less. Most children with MPS VII are less severely affected.

❓ What is MPS disease symptoms?

👉 Individuals with severe MPS 1 may have a large head (macrocephaly), clouding of the cornea, hearing loss, recurrent ear and upper respiratory infections, enlarged organs including liver, spleen and vocal cords, heart disease, short stature, hernias, and carpal tunnel syndrome.

❓ How rare is VI MPS?

👉 Frequency. The incidence of MPS VI is unknown, although it is estimated to occur in 1 in 250,000 to 600,000 newborns.

❓ Is there a cure for hereditary angioedema?

👉 Hereditary angioedema cannot be cured, but medicines can help prevent and treat the swelling.

❓ What is Naglazyme used to treat?

👉 Naglazyme is used to treat some of the symptoms of a genetic condition called mucopolysaccharidosis VI (MYOO-koe-pol-ee-SAK-a-rye-DOE-sis type 6), or MPS VI, also called Maroteaux-Lamy syndrome. MPS VI is a metabolic disorder in which the body lacks the enzyme needed to break down certain sugars and proteins.

❓ What are the side effects of Naglazyme?

👉 NAGLAZYME has been shown to improve walking and stair-climbing capacity. Severe and life-threatening allergic reactions can occur during NAGLAZYME infusions and up to 24 hours after infusion. Typical signs of an allergic reaction include shock, difficulty breathing, wheezing, swelling of the throat, and low blood pressure.

❓ What is Naglazyme made of?

👉 The active substance in Naglazyme, galsulfase, is a copy of the human enzyme N-acetylgalactosamine 4-sulfatase. Naglazyme helps to break down the GAGs and stop them building up in the cells.

❓ What is the indication of Naglazyme for the treatment of MPS VI?

👉 Naglazyme is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Mucopolysaccharidosis VI (MPS VI; N-acetylgalactosamine 4-sulfatase deficiency; Maroteaux-Lamy syndrome) (see section 5.1).


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