PMID- 11175623
OWN - NLM
STAT- MEDLINE
DCOM- 20010419
LR  - 20211203
IS  - 1516-3180 (Print)
IS  - 1516-3180 (Linking)
VI  - 119
IP  - 1
DP  - 2001 Jan 4
TI  - Rapidly progressive antineutrophil cytoplasm antibodies associated with 
      pulmonary-renal syndrome in a 10-year-old girl.
PG  - 29-32
AB  - CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe 
      the clinical manifestations of a great number of diseases in which pulmonary 
      hemorrhage and glomerulonephritis coexist. The classic example of this type of 
      vasculitis is Goodpastures syndrome, a term used to describe the association of 
      pulmonary hemorrhage, glomerulonephritis and the presence of circulating 
      antiglomerular basement membrane antibodies (anti-GBM). Among the several types 
      of systemic vasculitides that can present clinical manifestations of the 
      pulmonary-renal syndrome, we focus the discussion on two types more frequently 
      associated with antineutrophil cytoplasm antibodies (ANCA), microscopic 
      polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with 
      clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and 
      rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old 
      girl referred to our hospital for evaluation of profound anemia detected in a 
      primary health center. Five days before entry she had experienced malaise, pallor 
      and began to cough up blood-tinged sputum that was at first attributed to dental 
      bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit 
      = 14 %, platelets = 260,000, white blood cells = 8300, 74 % segmented, 4 % 
      eosinophils, 19 % lymphocytes and 3 % monocytes. Radiographs of the chest 
      revealed bilateral diffuse interstitial alveolar infiltrates. There was 
      progressive worsening of cough and respiratory distress during the admission day, 
      when she began to cough up large quantities of blood and hematuria was noted. 
      There was rapid and progressive loss of renal function and massive lung 
      hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen 
      specificity for myeloperoxidase (anti-MPO) was positive and the circulating 
      anti-GBM showed an indeterminate result.
FAU - Blanco Filho, F
AU  - Blanco Filho F
AD  - Pediatric Intensive Care Unit, Hospital Infantil Menino Jesus, Sao Paulo, Brazil. 
      blancoff@siteplanet.com.br
FAU - Ernesto, L C
AU  - Ernesto LC
FAU - Rosa, M A
AU  - Rosa MA
FAU - Stuginski, L A
AU  - Stuginski LA
FAU - Zlochevsky, E R
AU  - Zlochevsky ER
FAU - Blanco, F
AU  - Blanco F
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Brazil
TA  - Sao Paulo Med J
JT  - Sao Paulo medical journal = Revista paulista de medicina
JID - 100897261
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
SB  - IM
MH  - Anti-Glomerular Basement Membrane Disease/complications
MH  - Antibodies, Antineutrophil Cytoplasmic/*blood
MH  - Biopsy
MH  - Child
MH  - Churg-Strauss Syndrome/complications
MH  - Fatal Outcome
MH  - Female
MH  - Granulomatosis with Polyangiitis/complications
MH  - Hemorrhage/blood/*complications/therapy
MH  - Humans
MH  - IgA Vasculitis/complications
MH  - Kidney Diseases/blood/*complications/therapy
MH  - Lung Diseases/blood/*complications/therapy
MH  - Syndrome
EDAT- 2001/02/15 11:00
MHDA- 2001/04/21 10:01
CRDT- 2001/02/15 11:00
PHST- 2001/02/15 11:00 [pubmed]
PHST- 2001/04/21 10:01 [medline]
PHST- 2001/02/15 11:00 [entrez]
AID - S1516-31802001000100008 [pii]
AID - 10.1590/s1516-31802001000100008 [doi]
PST - ppublish
SO  - Sao Paulo Med J. 2001 Jan 4;119(1):29-32. doi: 10.1590/s1516-31802001000100008.