Disease staging: CliniCal anD CoDeD Criteria - HCUP

Clinical Criteria with Description, Diagnostic Findings and ICD-9-CM codes
DXCat Number: RES20
MultiStage: No
DXCat Label: Pulmonary Alveolar Proteinosis Specificity: A
Etiology: Genetic (or hereditary); Idiopathic
Stage Description Diagnostic findings ICD-9-CM Codes
1.01 Pulmonary alveolar proteinosis, sporadic or Alveolar proteinosis [lung biopsy report or segmental bronchoalveolar lavage report]
adult type
DX 5160;
1.02 Congenital pulmonary alveolar proteinosis Alveolar proteinosis [immunostaining of lung biopsy or PCR of infant's blood, lung tissue of lavage
of lung
NO;
AND genetic deficiency of the lung surfactant apoprotein B (SP-B) due to a defect in a two base
pair insertion (121 ins 2) in codon 121 or deletion of exon 4 preventing the translation of SB
protein and generation of a restriction fragment length polymorphism
2.01 with hypoxemia Stage 1.01-1.02
AND hypoxemia:
2.02 with restrictive lung disease pattern Stage 1.01-2.01
AND restrictive lung disease:
3.01 with cor pulmonale Stage 1.01-2.01
AND cor pulmonale:
3.02 with respiratory failure Stage 1.01-3.01
AND respiratory failure:
3.03 with shock Stage 1.01-3.02
AND shock:
4.00 with death Stage 1.01-3.03
AND death
STAGE 1.01 + GROUP HYPOXEMIA;
NO;
STAGE 1.01-2.01 + GROUP
COR_PULMONAL;
STAGE 1.01-3.01 + GROUP
RESP_FAIL;
STAGE 1.01-3.02 + (GROUP
SHOCKC OR GROUP SHOCKN) ;
NO;
References:
Colten HR and Mello DE. Pulmonary alveolar proteinosis. In: Behrman RE, Kliegman RM, Arvin AM, Nelson WE, eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, PA:
WB Saunders; 1996:1222-1223.
King TE Jr, Cherniak RM, Schwarz MI. Idiopathic pulmonary fibrosis and other interstitial lung diseases of unknown etiology. In: Murray JF, Nadel JA, eds. Textbook of
Respiratory Medicine. Vol. 1, 2nd ed. Philadelphia, PA: WB Saunders; 1988:1827-1849.
Lee KN, Levin DL, Webb WR, et al. Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations. Chest. 1997;111(4):989-95.
Reynolds HY. Interstitial lung diseases. 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; 1998:1460-1466.
Stauffer JL. Lung. In: Tierney Jr LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis & Treatment. Stamford, CT: Appleton & Lange; 1998:251-333.
Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis. a review and an update. Chest. 1997;111(2):460-6.
Thursday, January 07, 2010 9:23:13 AM
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