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Shire Pharmaceuticals Group Plc

Shire Granted EU Conditional Marketing Authorisation for Natpar[®] (Parathyroid Hormone) for the Treatment of Patients with Chronic Hypoparathyroidism

Switzerland (ots/PRNewswire)

Natpar is the first and only licensed recombinant human parathyroid hormone therapy for chronic hypoparathyroidism

Shire plc (LSE: SHP, NASDAQ: SHPG) today announced that the European Commission (EC) has granted Conditional Marketing Authorisation for Natpar (rhPTH[1-84]), the first recombinant human protein with the full length 84-amino acid sequence of endogenous parathyroid hormone (PTH). Natpar will be the first and only approved hormone therapy indicated as adjunctive treatment for adult patients with chronic hypoparathyroidism who cannot be adequately controlled with standard therapy alone.[1] Hypoparathyroidism is a rare endocrine disease resulting from an inappropriately low circulating PTH concentration.[2]

''As the first and only licensed recombinant parathyroid hormone treatment in Europe for chronic hypoparathyroidism, Natpar represents a historical and timely innovation for patients who cannot be adequately controlled on calcium and vitamin D alone," said Philip J. Vickers, Ph.D., Global Head of Research and Development at Shire. "The approval of Natpar offers an important advance in the management of this rare endocrine disorder for patients in Europe."

The Conditional Marketing Authorisation is based on the outcomes from the Phase III efficacy and safety of rhPTH(1-84) clinical trial (REPLACE) in patients aged 19-74 years with chronic hypoparathyroidism.[1] The trial showed that Natpar maintained serum calcium while reducing oral calcium and active vitamin D supplemental doses.[1]

"Chronic hypoparathyroidism can carry a significant disease burden, and some patients are not well-controlled, showing fluctuations in their serum calcium levels,[3]" said Professor Maria Louisa Brandi, Department of Internal Medicine, University of Florence, Italy. "Clinical studies have shown Natpar maintains serum calcium while reducing the need for calcium and vitamin D for patients with chronic hypoparathyroidism.[1]"

With this approval, Shire is now authorized to market Natpar in the 28 Member States of the European Union (EU), as well as in Iceland, Liechtenstein and Norway. Natpar falls under the scope of European Commission Regulation as eligible for Conditional Marketing Authorisation because it is designated as an orphan medicinal product and fulfils an unmet medical need.

About hypoparathyroidism

Hypoparathyroidism is a rare disease that occurs when inadequate levels of PTH are secreted by the parathyroid glands, resulting in a mineral imbalance in the body expressed by a low concentration of calcium (hypocalcemia) and a high concentration of phosphate (hyperphosphatemia) in the blood.

Guidelines from the European Society of Endocrinology (ESE) state that a diagnosis of chronic hypoparathyroidism should be considered in a patient with hypocalcemia and an inappropriately low PTH concentration. Guidelines recommend that treatment should aim to restore mineral homeostasis and prevent symptoms of hypocalcemia while optimizing overall well-being. Typical symptoms of hypocalcemia include muscle spasms and cramping.

To date, management of chronic hypoparathyroidism has focused on maintaining serum calcium with oral calcium and active vitamin D supplements; however, some patients are not adequately controlled and still have variations in their serum calcium levels.[3]-[6]

About Natpar

Natpar is a recombinant human PTH and will be available as a 25, 50, 75 and 100 micrograms once-daily injection as adjunctive treatment for adult patients with chronic hypoparathyroidism who cannot be adequately controlled with standard therapy alone.

Natpar is approved in the United States under the trade name Natpara® (parathyroid hormone).

About the REPLACE study

In the double-blind, placebo-controlled, randomized Phase III study 124 patients with hypoparathyroidism were randomized in a ratio of 2:1 to either 50 micrograms once daily of rhPTH(1-84) or placebo for 24 weeks. The primary endpoint was a 50 percent or greater reduction from baseline in their daily dose of oral calcium and active vitamin D while maintaining a stable albumin corrected serum calcium concentration greater than or equal to baseline concentration (baseline was 2.1 mmol/L for the rhPTH[1-84] group and 2.2 mmol/L for the placebo group) and less or equal to the upper limit of normal (normal range 2.1-2.6 mmol/L). At the end of the treatment period, 54.8 percent of the patients on rhPTH(1-84) achieved the primary endpoint compared with 2.5 percent of patients in the placebo group (p<0.0001).[1] There were no differences in the proportion of patients maintaining a stable albumin corrected serum calcium concentration at the end of treatment between subjects randomized to rhPTH (1-84) and placebo.[7]

Natpar Safety Information for Europe

Please consult the Natpar Summary Product Characteristics (SmPC) before prescribing.

Natpar treatment should be supervised by a physician or other qualified healthcare professional experienced in the management of patients with hypoparathyroidism.

Contraindications

Natpar is contraindicated in patients with hypersensitivity to the active substance or to any of the excipients, who are receiving or who have previously received radiation therapy to the skeleton, with skeletal malignancies or bone metastases, who are at increased baseline risk for osteosarcoma, with unexplained elevations of bone-specific alkaline phosphatase, with pseudohypoparathyroidism.

Warnings and Precautions

Monitoring of patients during treatment pre-dose and in some cases post-dose serum calcium levels must be monitored during treatment with Natpar.

Hypercalcemia this was reported in clinical trials with Natpar. Hypercalcemia commonly occurred during the titration period, during which doses of oral calcium, active vitamin D, and Natpar were being adjusted. Hypercalcemia may be minimized by following the recommended dosing, the monitoring information and asking patients about any symptoms of hypercalcemia. If severe hypercalcemia develops, hydration and temporarily stopping Natpar, calcium and active vitamin D should be considered until serum calcium returns to the normal range. Then consider resuming Natpar, calcium and active vitamin D at lower doses.

Hypocalcemia a common clinical manifestation of hypoparathyroidism, was reported in clinical trials with Natpar. Most of the hypocalcemic events occurring in the clinical trials were mild to moderate severity. The risk for serious hypocalcemia was greatest after the withdrawal of Natpar. Temporary or permanent discontinuation of Natpar must be accompanied by monitoring of serum calcium levels and increase of exogenous calcium and/or vitamin D sources as necessary. Hypocalcemia may be minimized by following the recommended dosing, the monitoring information, and asking patients about any symptoms of hypocalcemia.

Concomitant use with cardia glycosides Hypercalcaemia of any cause may predispose to digitalis toxicity, monitor serum calcium and cardiac glycoside levels and patients for signs and symptoms of digitalis toxicity.

Severe renal or hepatic disease Natpar should be used with caution in patients with severe renal or hepatic disease because they have not been evaluated in clinical trials.

Use in young adults Natpar should be used with caution in young adult patients with open epiphyses.

Tachyphylaxis the calcium-raising effect of Natpar may diminish over time in some patients. The response of serum calcium concentration to administration of Natpar should be monitored at intervals to detect this and the diagnosis of tachyphylaxis considered.

Adverse Reactions

The most commonly observed adverse events with Natpar treatment were hypercalcaemia, hypocalcaemia, headache, diarrhea, vomiting, paraesthesia, hypoesthesia and hypercalciuria.



Very common

(frequency        Hypercalcemia, hypocalcaemia, headache, 
hypoesthesia,
greater than or   paraesthesia, diarrhoea, nausea, vomiting, 
arthralgia, and
equal to1/10):    muscle spasms.
Common            Hypomagnesaemia, tetany, anxiety, insomnia, 
somnolence,
palpitations, hypertension, cough, abdominal 
pain upper,
muscle twitching, musculoskeletal pain, 
myalgia, neck pain,
pain in extremity, hypercalciuria, pollakiuria,
asthenia,
(greater than or  chest pain, fatigue, injection site reactions, 
thirst,
equal to1/100 to  anti-PTH antibody positive, blood 
25-hydroxycholecalciferol
<1/10):           decreased, vitamin D decreased.

NOTES TO EDITORS

About Shire

Shire is the leading global biotechnology company focused on serving people with rare diseases and other highly specialized conditions. We strive to develop best-in-class products, many of which are available in more than 100 countries, across core therapeutic areas including Hematology, Immunology, Neuroscience, Ophthalmics, Lysosomal Storage Disorders, Gastrointestinal / Internal Medicine / Endocrine and Hereditary Angioedema; and a growing franchise in Oncology.

Our employees come to work every day with a shared mission: to develop and deliver breakthrough therapies for the hundreds of millions of people in the world affected by rare diseases and other high-need conditions, and who lack effective therapies to live their lives to the fullest.

http://www.shire.com

Forward-Looking Statements

Statements included herein that are not historical facts, including without limitation statements concerning future strategy, plans, objectives, expectations and intentions, the anticipated timing of clinical trials and approvals for, and the commercial potential of, inline or pipeline products, are forward-looking statements. Such forward-looking statements involve a number of risks and uncertainties and are subject to change at any time. In the event such risks or uncertainties materialize, Shire's results could be materially adversely affected. The risks and uncertainties include, but are not limited to, the following:

- Shire's products may not be a commercial success;
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  result of governmental regulations and market developments may 
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  products and is reliant on third party contract manufacturers to 
  manufacture other products and to provide goods and services. Some 
  of Shire's products or ingredients are only available from a single
  approved source for manufacture. Any disruption to the supply chain
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  continue marketing or developing a product or may result in Shire 
  being unable to do so on a commercially viable basis for some 
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  oversight by various regulatory agencies. Regulatory approvals or 
  interventions associated with changes to manufacturing sites, 
  ingredients or manufacturing processes could lead to, among other 
  things, significant delays, an increase in operating costs, lost 
  product sales, an interruption of research activities or the delay 
  of new product launches;
- certain of Shire's therapies involve lengthy and complex processes,
  which may prevent Shire from timely responding to market forces and
  effectively managing its production capacity;
- Shire has a portfolio of products in various stages of research and
  development. The successful development of these products is highly
  uncertain and requires significant expenditures and time, and there
  is no guarantee that these products will receive regulatory 
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  including Shire's ability to enforce and defend patents and other 
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  from other companies and organizations;
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  respect to Shire's acquisitions, including NPS Pharmaceuticals 
  Inc., Dyax Corp. or Baxalta Incorporated may adversely affect 
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  its product portfolio through external collaborations, which, if 
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  could have a material adverse effect on Shire's revenues, financial
  condition or results of operations;
- Shire incurred substantial additional indebtedness to finance the 
  Baxalta acquisition, which may decrease its business flexibility 
  and increase borrowing costs; and

a further list and description of risks, uncertainties and other matters can be found in Shire's most recent Annual Report on Form 10-K and in Shire's subsequent Quarterly Reports on Form 10-Q, in each case including those risks outlined in "ITEM 1A: Risk Factors", and in Shire's subsequent reports on Form 8-K and other Securities and Exchange Commission filings, all of which are available on Shire's website.

All forward-looking statements attributable to us or any person acting on our behalf are expressly qualified in their entirety by this cautionary statement. Readers are cautioned not to place undue reliance on these forward-looking statements that speak only as of the date hereof. Except to the extent otherwise required by applicable law, we do not undertake any obligation to update or revise forward-looking statements, whether as a result of new information, future events or otherwise.

References

1. Natpar® Summary of Product Characteristics.
2. Bilezikian JP, et al. J Clin Endocrinol Metab. 2016;101:2313-2324.
3. Mitchell DM, et al. J Clin Endocrinol Metab. 2012;97:4507-4514.
4. Bollerslev J, et al. Eur J Endocrinol. 2015;173:G1-G20.
5. Brandi ML, et al. J Clin Endocrinol Metab. 2016;101:2273-2283.
6. Hadker N, et al. Endocr Pract. 2014;20:671-679.
7. Mannstadt M, et al. Lancet Diabetes Endocrinol. 2013;1:275-283.

Contact:

, please contact:

Investor Relations
Ian Karp
ikarp@shire.com
+1-781-482-9018

Robert Coates
rcoates@shire.com
+44(0)1256-894874

Media
Annabel Cowper
annabel.cowper@shire.com
+41-79-630-8619

Debbi Ford
debbi.ford@shire.com
+1-617-949-9083

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