Porphyria for Fun and Profit
In search of an interesting example, something with a bit of pizzazz, it
occurred to me to look at a (rather rare) disease that has come to public
attention recently via the film The Madness of King George. We
can do the usual things: searching ANNIE, PAO, HOLLIS, the specialized
databases (in this case MEDLINE is the obvious one), and there's no
shortage of articles and books. In fact ANNIE yields only one:
You searched for the KEYWORD: porphyria W&L CATALOG
AUTHOR Macalpine, Ida.
TITLE George III and the mad-business, by Ida Macalpine & Richard
Hunter.
EDITION [1st American ed.]
PUBLISHER New York, Pantheon Books [1970, c1969]
DESCRIPT xv, 407 p. illus., facsims., geneal. tables, port. 25 cm.
SUBJECT George III, King of Great Britain, 1738-1820.
Porphyria.
LOCATION CALL NO.
1 > Leyburn Library DA506.A2 M28 1970
This book (written considerably before the biochemical niceties were
worked out) concentrates on contemporary diagnoses, reports of the King's
condition, and treatment regimes, but also includes genealogical tables
and descriptions of early psychiatry.
PAO yields 13 articles, all from medical sources.
And MEDLINE offers 1777 articles.
Obviously we could learn a lot by looking at these sources, but what I
really want to do is explore some other possibilities, some
sources of information and data that are not essentially bibliographic,
or in any case not only bibliographic.
Since we know that porphyria is somehow entangled with genetics, it's not
a bad guess to figure that Online Mendelian Inheritance in Man
will have useful things to say, and that does prove to be the case:
19 entries found, searching for "porphyria"
- *176100
PORPHYRIA CUTANEA TARDA; PCT
- *176000
PORPHYRIA, ACUTE INTERMITTENT; AIP
- *176200
PORPHYRIA VARIEGATA; VP
- *263700
PORPHYRIA, CONGENITAL
ERYTHROPOIETIC; CEP
- *125270
DELTA-AMINOLEVULINATE
DEHYDRATASE; ALADH; ALAD
- 176090
PORPHYRIA CUTANEA TARDA, TYPE I *176010 PORPHYRIA, CHESTER TYPE; PORC
- *121300
COPROPORPHYRIA *177000 PROTOPORPHYRIA, ERYTHROPOIETIC
- *143100
HUNTINGTON DISEASE; HD
- *600923
PROTOPORPHYRINOGEN OXIDASE; PPOX
- *276700
TYROSINEMIA, TYPE I
- *143890
HYPERCHOLESTEROLEMIA,
FAMILIAL; HC; FHC; FH
- *193500
WAARDENBURG SYNDROME; WS1
- *247100
LIPOID PROTEINOSIS OF
URBACH AND WIETHE
- #131800
EPIDERMOLYSIS
BULLOSA OF HANDS AND FEET
- #118200
CHARCOT-MARIE-TOOTH DISEASE; CMT1
- *232400
GLYCOGEN
STORAGE DISEASE III
- 219095
CUTANEOUS PHOTOSENSITIVITY AND COLITIS, LETHAL
OMIM leads us to the research literature, but what other web
possibilities are there? If we want to find documents on the web
which mention porphyria, we probably want to use one (and
probably more than one) of the indexes for the web, accessible via
the Home Page menu item Help with Campus Computing Resources, Internet
and WWW Tools, which offers "Searching the Web by Subject".
This array of searching tools changes as more powerful engines are added,
and currently Alta Vista seems to be the tool of choice. Here's
what I got when I searched for 'porphyria'.
And these are some of the choicest sites: