Classification of BH4 Response Sample Clauses

Classification of BH4 Response. ‌ Fasting plasma phenylalanine concentrations were measured immediately before and after one month of 20 mg/kg/day BH4 therapy (sapropterin dihydrochloride; Kuvan®; BioMarin Pharmaceutical Inc., Novato, California, USA). BH4 response was classified using sequentially applied criteria, detailed elsewhere [185]. Briefly, patients were initially classified based on the percent change in plasma phenylalanine concentrations after one month of BH4 therapy. Non-responders experienced less than a 15% decrease in plasma phenylalanine concentrations, discontinued BH4 therapy, and continued their diet therapy regimen. Patients with at least a 15% decrease in plasma phenylalanine concentrations at month one were further evaluated and differentiated. Definitive responders were able to improve their dietary phenylalanine tolerance by at least 300 mg/day (or consume an unrestricted diet) and could decrease their medical food needs by at least 25% compared to baseline (or no longer needed medical food) while maintaining blood phenylalanine concentrations below 360 µmol/L. Provisional responders, despite an initial decrease in plasma phenylalanine concentrations, could not meet both of the diet liberalization criteria while maintaining blood phenylalanine concentrations below 360 µmol/L.
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Related to Classification of BH4 Response

  • Form and Timing of Response (a) Intermediary agrees to provide, promptly upon request of the Fund or its designee, the requested information specified in paragraph 1 above. If requested by the Fund or its designee, Intermediary agrees to use best efforts to determine promptly whether any specific person about whom it has received the identification and transaction information specified in paragraph 1 is itself a financial intermediary (“indirect intermediary”) and, upon further request of the Fund or its designee, promptly either (i) provide (or arrange to have provided) the information set forth in paragraph 1 for those shareholders who hold an account with an indirect intermediary or (ii) restrict or prohibit the indirect intermediary from purchasing, in nominee name on behalf of other persons, securities issued by the Fund. (b) Responses required by this paragraph must be communicated in writing and in a format mutually agreed upon by the parties. (c) To the extent practicable, the format for any transaction information provided to the Fund should be consistent with the NSCC Standardized Data Reporting Format

  • JOC Pricing of Itemized List of RS Means Non-Prepriced Items No response The Vendor may download the optional Pricing of Itemized List of RS Means Non-Prepriced Items form from the attachment tab, fill in the requested information, and upload the completed spreadsheet. DO NOT UPLOAD encrypted or password protected files. Valid Reference Email addresses are REQUIRED on the spreadsheet. The vendor must download the References spreadsheet from the attachment tab, fill in the requested information and upload the completed spreadsheet. DO NOT UPLOAD encrypted or password protected files.

  • Timing of Company Response The Company shall respond to such claimant within 90 days after receiving the claim. If the Company determines that special circumstances require additional time for processing the claim, the Company can extend the response period by an additional 90 days by notifying the claimant in writing, prior to the end of the initial 90-day period, that an additional period is required. The notice of extension must set forth the special circumstances and the date by which the Company expects to render its decision.

  • Rectification of Safety Hazard Where, because of the existence of a safety hazard, a site has been stopped for a defined period of time and Employees sent off site by agreement between Site Managers and any combination of Union Official/s, Health and Safety Committee, those people who remain on site to do rectification work will be paid at the rate of double time for all such work.

  • Lobbying Activities - Standard Form - LLL No response Do not upload this form unless Vendor has reportable lobbying activities. There are Attributes entitled, “2 CFR Part 200 or Federal Provision - Xxxx Anti-Lobbying Amendment – Continued.” Properly respond to those Attributes and only upload this form if applicable/instructed. If upload is required based on your response to those Attributes, the Disclosure of Lobbying Activities – Standard Form - LLL must be downloaded from the “Attachments” section of the IonWave eBid System, reviewed, properly completed, and uploaded to this location.

  • Emergency Response Partners must develop, maintain, and carry out a response plan for public water system emergencies, including disease outbreaks, spills, operational failures, and water system contamination. Partners must notify DWS in a timely manner of emergencies that may affect drinking water supplies.

  • Optional Xactimate Response Attachment (Part 2)

  • Company’s Response Upon receipt by the Company of a copy of a Conversion Notice, the Company shall as soon as practicable, but in no event later than one (1) Business Day after receipt of such Conversion Notice, send, via email, facsimile or overnight courier, a confirmation of receipt of such Conversion Notice to such Holder indicating that the Company will process such Conversion Notice in accordance with the terms herein. Within two (2) Business Days after the date of the Conversion Confirmation, the Company shall have issued and electronically transferred the shares to the Broker indicated in the Conversion Notice; should the Company be unable to transfer the shares electronically, it shall, within two (2) Business Days after the date of the Conversion Confirmation, have surrendered to FedEx for delivery the next day to the address as specified in the Conversion Notice, a certificate, registered in the name of the Holder, for the number of shares of Common Stock to which the Holder shall be entitled.

  • Corporate Social Responsibility The Parties affirm the importance of each Party encouraging enterprises operating within its Area or subject to its jurisdiction to voluntarily incorporate into their internal policies those internationally recognised standards, guidelines and principles of corporate social responsibility that have been endorsed or are supported by that Party.

  • Financial Responsibility You understand that you remain, solely and exclusively responsible for any and all financial risks, including, without limitation, insufficient funds associated with accessing the Service. The Credit Union shall not be liable in any manner for such risk unless Credit Union fails to follow the procedures described in materials for use of the service. You assume exclusive responsibility for the consequences of any instructions you give to the Credit Union, for your failures to access the Service properly in a manner prescribed by the Credit Union, and for your failure to supply accurate input information, including, without limitation, any information contained in an application.

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