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Idiopathic Pulmonary Hemosiderosis

Germain AG - MedPix (2008)

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Idiopathic Pulmonary Hemosiderosis

History: Patient with diagnosis of idiopathic pulmonary hemosiderosis per open lung biopsy 10/07 who has been relatively asymptomatic but with persistent presence of hemosiderin-laden macrophages in bronchoalveolar lavage and desaturation noted on exercise test in May 2008. HISTORY Patient was product of pregnancy complicated by pre-eclampsia. Patient was born at 27weeks after 2 days of antenatal steroids. Significant respiratory distress requiring intubation was noted immediately after birth. Neonatal course included multiple doses of surfactant. 68 days mechanical ventilation, and 3 months of NICU care. Complications included multiple episodes of pneumonia, presumed sepsis, NEC, anemia, and electrolyte abnormalities. The patient was discharged home on O2 which she used until 9 months of age, as well as Fe, diuretics, steroids. After 1 year of age, patient began to have multiple episodes of pneumonia, bronchitis, and asthma requiring hospitalization. Anemia, requiring 2 transfusions, was noted in context of one episode of pneumonia. In addition, the patient experienced failure to thrive, reaching less than 3% height/weight for her age. At the present, the patient is at 25% height, 3% weight. Parents otherwise deny recent URI, cough, wheeze. Hospitalizations: after birth x 3 mos 2004-pna 2005-pna and transfusion for anemia 2006-pulmonary hemorrhage, respiratory failure 2007-multifocal pna Surgeries: bronchoscopy 12/06, 12/07, 5/08 open lung bx 10/07 port cath placement 12/07 Meds: Prevacid 15 mg tablet QD Miralax, 17 gm QD Iron oral solution75mg/0.6mL- 25 mg/mL Hydroxychloroquine 25mg/mL- 6ml QD Tums – 1 tablet QD for calcium Flinstones MVT Immunizations: Up to date Allergies: Cow’s Milk Developmental History: delayed fine motor skills per MOP; otherwise met all milestones. Family History: non-contributory Social History: lives with mother, father, older brother. No pets, no smoking

Findings: During acute bleeding episode, areas of increased opacity and decreased lung volume are seen throughout the lungs, especially in the bases.

Ddx: DDX for diffuse alveolar hemorrhage: immune related- anti-basement membrane antibody disease (Goodpasture's syndrome), vasculitis and collagen vascular disease (systemic lupus erythematosus, Wegener's granulomatosis, systemic necrotizing vasculitis, and others), and pulmonary capillaritis associated with idiopathic rapidly progressive glomerulonephritis. Nonimmune-coagulopathy, necrotizing pulmonary infection, drugs (penicillamine), toxins (trimellitic anhydride), Heiner’s Syndrome, and idiopathic pulmonary hemosiderosis

Dxhow: Open Lung Biopsy, 10/07 - reviewed by TAMC pathology, AFIP, Texas Children’s Hospital Common findings on diagnostic tests: CXR: “butterfly or batwing” pattern – symmetrical alveolar infiltrates slanting upwards towards lateral chest wall CT: “ground glass appearance” Dx: BAL showing hemosiderin-laden macrophages Lung biopsy intact or minimally fragmented erythrocytes in the distal airways and alveoli haemosiderin-laden macrophages (siderophages) “bland” – no capillaritis, vasculitis, any focal or diffuse smooth muscle cell proliferations, vascular malformations, malignancy, pulmonary infarcts, granulomatous inflammation, or infectious agents.

Exam: VS: T 98.4 HR BP RR O2 Sat 98% on RA Wt 15.2 kg Ht 44cm Gen: Small for age; NORMAL ACTIVITY FOR AGE, ALERT AND RESPONSIVE, HEENT: left TM well visualized with good light reflex; right TM occluded by cerumen (unable to extract at this time); EOMI, PERRLA, no nasal discharge, oropharynx clear and w/o exudates or erythema, No LAD appreciated CV: reg rate and rhythm, no murmurs, rubs, or gallops appreciated Resp: CTAB GI: normoactive bowel sounds, NTND, no HSM, no masses Ext: FROM, no edema Skin: warm, dry, no lesions, no bruises Neuro: grossly intact LABS Normal: C3, IgG, IgA, IgM, IgE, Rheumatoid Factor, ESR, CH50, GBM ab, ACE, ANCA, Low Fe, elevated ferritin and TIBC

No MeSH data available.


Jan 2007: small areas of basilar atelectasis or fibrosis noted with several areas of normal-appearing lung seenin the right middle lobe.
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MPX1033_synpic45452: Jan 2007: small areas of basilar atelectasis or fibrosis noted with several areas of normal-appearing lung seenin the right middle lobe.


Idiopathic Pulmonary Hemosiderosis

Germain AG - MedPix (2008)

Jan 2007: small areas of basilar atelectasis or fibrosis noted with several areas of normal-appearing lung seenin the right middle lobe.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX1033&req=5

MPX1033_synpic45452: Jan 2007: small areas of basilar atelectasis or fibrosis noted with several areas of normal-appearing lung seenin the right middle lobe.

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Idiopathic Pulmonary Hemosiderosis

History: Patient with diagnosis of idiopathic pulmonary hemosiderosis per open lung biopsy 10/07 who has been relatively asymptomatic but with persistent presence of hemosiderin-laden macrophages in bronchoalveolar lavage and desaturation noted on exercise test in May 2008. HISTORY Patient was product of pregnancy complicated by pre-eclampsia. Patient was born at 27weeks after 2 days of antenatal steroids. Significant respiratory distress requiring intubation was noted immediately after birth. Neonatal course included multiple doses of surfactant. 68 days mechanical ventilation, and 3 months of NICU care. Complications included multiple episodes of pneumonia, presumed sepsis, NEC, anemia, and electrolyte abnormalities. The patient was discharged home on O2 which she used until 9 months of age, as well as Fe, diuretics, steroids. After 1 year of age, patient began to have multiple episodes of pneumonia, bronchitis, and asthma requiring hospitalization. Anemia, requiring 2 transfusions, was noted in context of one episode of pneumonia. In addition, the patient experienced failure to thrive, reaching less than 3% height/weight for her age. At the present, the patient is at 25% height, 3% weight. Parents otherwise deny recent URI, cough, wheeze. Hospitalizations: after birth x 3 mos 2004-pna 2005-pna and transfusion for anemia 2006-pulmonary hemorrhage, respiratory failure 2007-multifocal pna Surgeries: bronchoscopy 12/06, 12/07, 5/08 open lung bx 10/07 port cath placement 12/07 Meds: Prevacid 15 mg tablet QD Miralax, 17 gm QD Iron oral solution75mg/0.6mL- 25 mg/mL Hydroxychloroquine 25mg/mL- 6ml QD Tums – 1 tablet QD for calcium Flinstones MVT Immunizations: Up to date Allergies: Cow’s Milk Developmental History: delayed fine motor skills per MOP; otherwise met all milestones. Family History: non-contributory Social History: lives with mother, father, older brother. No pets, no smoking

Findings: During acute bleeding episode, areas of increased opacity and decreased lung volume are seen throughout the lungs, especially in the bases.

Ddx: DDX for diffuse alveolar hemorrhage: immune related- anti-basement membrane antibody disease (Goodpasture's syndrome), vasculitis and collagen vascular disease (systemic lupus erythematosus, Wegener's granulomatosis, systemic necrotizing vasculitis, and others), and pulmonary capillaritis associated with idiopathic rapidly progressive glomerulonephritis. Nonimmune-coagulopathy, necrotizing pulmonary infection, drugs (penicillamine), toxins (trimellitic anhydride), Heiner’s Syndrome, and idiopathic pulmonary hemosiderosis

Dxhow: Open Lung Biopsy, 10/07 - reviewed by TAMC pathology, AFIP, Texas Children’s Hospital Common findings on diagnostic tests: CXR: “butterfly or batwing” pattern – symmetrical alveolar infiltrates slanting upwards towards lateral chest wall CT: “ground glass appearance” Dx: BAL showing hemosiderin-laden macrophages Lung biopsy intact or minimally fragmented erythrocytes in the distal airways and alveoli haemosiderin-laden macrophages (siderophages) “bland” – no capillaritis, vasculitis, any focal or diffuse smooth muscle cell proliferations, vascular malformations, malignancy, pulmonary infarcts, granulomatous inflammation, or infectious agents.

Exam: VS: T 98.4 HR BP RR O2 Sat 98% on RA Wt 15.2 kg Ht 44cm Gen: Small for age; NORMAL ACTIVITY FOR AGE, ALERT AND RESPONSIVE, HEENT: left TM well visualized with good light reflex; right TM occluded by cerumen (unable to extract at this time); EOMI, PERRLA, no nasal discharge, oropharynx clear and w/o exudates or erythema, No LAD appreciated CV: reg rate and rhythm, no murmurs, rubs, or gallops appreciated Resp: CTAB GI: normoactive bowel sounds, NTND, no HSM, no masses Ext: FROM, no edema Skin: warm, dry, no lesions, no bruises Neuro: grossly intact LABS Normal: C3, IgG, IgA, IgM, IgE, Rheumatoid Factor, ESR, CH50, GBM ab, ACE, ANCA, Low Fe, elevated ferritin and TIBC

No MeSH data available.