Leaders Best Practice No 165 Front line tests for the investigation of suspected porphyria

نویسندگان

  • A C Deacon
  • G H Elder
چکیده

The porphyrias are uncommon disorders of haem biosynthesis and their eVective management requires prompt and accurate diagnosis. This article describes methods for the determination of urinary porphobilinogen, urinary and faecal total porphyrins, and total porphyrins in erythrocytes and plasma that are suitable for use in non-specialist laboratories. The selection and interpretation of these methods, and the indications for further, more specialised, investigation are discussed. (J Clin Pathol 2001;54:500–507)

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Best practice guidelines on first-line laboratory testing for porphyria.

The porphyrias are disorders of haem biosynthesis which present with acute neurovisceral attacks or disorders of sun-exposed skin. Acute attacks occur mainly in adults and comprise severe abdominal pain, nausea, vomiting, autonomic disturbance, central nervous system involvement and peripheral motor neuropathy. Cutaneous porphyrias can be acute or chronic presenting at various ages. Timely diag...

متن کامل

Identification of the Best Infiltration Model in Order to Investigation of Spatial Variability of Infiltration Parameters (Case Study: Darabkola River Basin)

Infiltration is the process of water penetration from the ground surface into the soil and is an important process in the hydrological cycle by which surface runoff and groundwater recharge can be linked. Over the years, the importance of the infiltration process resulted in the development of several simplified analytical models for predicting infiltration. These infiltration models range from...

متن کامل

Hereditary coproporphyria: report of an Irish kindred and identification of a novel gene mutation.

Case Report The first step in the investigation of suspected acute porphyria is measurement of urinary s-aminolaevulic acid (ALA) and porphobilinogen (PBG). When raised, acute porphyria is suggested. These tests do not discriminate between the various types of porphyria, and they return to normal limits within 7-10 days of onset of symptoms. Genetic analysis subsequently confirmed a previously ...

متن کامل

P98: Effect of Attentional Focus of Self-Statements in Basketball Practice on State Anxiety of Novice Players before Free Throw in front of Audience

The purpose of present study was investigation of the effect of attentional focus of novice players’ self-statements in basketball free throw practice on their state anxiety in transfer test with spectators’ presence. Therefore, 38 novice females (mean of the age=22 yrs.) who had intermediate trait anxiety were selected randomly by sports competitive anxiety test (SCAT). The participants were a...

متن کامل

Best practice guidelines on clinical management of acute attacks of porphyria and their complications.

The British and Irish Porphyria Network guidelines describe best practice in the clinical assessment, investigation and management of acute porphyria attacks and their complications, including severe attacks with neuropathy. Acute attacks of porphyria may occur in acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP). Aminolaevulinic acid dehydratase d...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2001