Disease staging: CliniCal anD CoDeD Criteria - HCUP

Clinical Criteria with Description, Diagnostic Findings and ICD-9-CM codes
DXCat Number: PED01
MultiStage: No
DXCat Label: Anomaly: Adrenal Hyperplasia Specificity: A
Etiology: Congenital; Genetic (or hereditary)
Stage Description Diagnostic findings ICD-9-CM Codes
1.01 21-Hydroxylase deficiency Urinary 17-hydroxyprogesterone ≥ normal value/24 hrs. [chemistry report]
DX 2552;
OR positive urine pregnanediol [chemistry report]
AND clitoral hypertrophy in females
OR macrogenitosomia in males
1.02 11-Beta hydroxylase deficiency Serum elevations of 11-deoxycorticosterone [chemistry report]
NO;
AND serum elevations of 11-deoxycortisol [chemistry report]
OR urine elevations of tetrahydro 11-deoxycorticosol [chemistry report]
1.03 3-Beta hydroxysteroid dehydrogenase Serum elevations of dehydroepiandrosterone [chemistry report]
NO;
deficiency
OR serum elevation of DHEA-sulfate [chemistry report]
AND normal 17-hydroxyprogesterone level [chemistry report]
OR low 17-hydroxyprogesterone level [chemistry report]
2.01 Salt losing congenital adrenal hyperplasia Stage 1.01-1.03
AND hyponatremia:
3.01 with shock Stage 1.01-2.01
AND shock:
4.00 with death Stage 1.01-3.01
AND death
STAGE 1.01 + GROUP
HYPONATREMIA;
STAGE 1.01-2.01 + (GROUP
SHOCKC OR GROUP SHOCKN) ;
NO;
References:
New MI. Steroid 21-hydroxylase deficiency (congenital adrenal hyperplasia). American Journal of Medicine. 1995;98(1A):2S-8S.
Orth DN, Kovacs WJ, DeBold CR. The adrenal cortex. In: Wilson JD, Foster DW, eds. Williams Textbook of Endocrinology. 8th ed. Philadelphia, PA: WB Saunders; 1992:489-
620.
White PC. Inherited forms of mineralocorticoid hypertension. Hypertension. 1996;28(6):927-936.
Wilson JD, Griffin JE. Disorders of sexual differentiation. In: Fauci AS, Braunwald E, Isselbacher KJ, Wilson JD, Martin JB, Kasper DL, Hauser SL, Longo, DL, eds. Harrison’s
Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill; 1998:2119-2131.
Thursday, January 07, 2010 9:22:54 AM
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