western states pediatric pulmonary case conference cough, hypoxia, and down syndrome emily deboer,...
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Western States Pediatric Pulmonary Case Conference
Cough, Hypoxia, and Down Syndrome
Emily DeBoer, MD
The Children’s Hospital
University of Colorado
September 22, 2010
Outline
Case presentation: Cough, Hypoxia, and Down Syndrome
Differential Diagnosis Multi-system complications of patients with
Trisomy 21 Treatment and Monitoring
Chief Complaint
9 month old female with Down Syndrome referred to pulmonary clinic for initial evaluation because of cough and persistent oxygen requirement
History of Present Illness
Daily cough for months - day and night No increased work of breathing Snoring Gags with jejunal feeds
Treated with RanitidineSevere oral aversion, tastes by mouth
Past medical/surgical history Trisomy 21 Born at 36 weeks in Colorado Esophageal atresia without TEF
GT placment DOL 1Gastric pull-through at 4 months
On and off oxygen Ventilated x 1 week after surgery Discharged at 5 months on ¼ lpm O2 via nasal
cannula
PMH/PSH
PDA ligation at 5 months of age Recent echo revealed – “normal function,
small left to right ASD, mild TR” Monthly esophageal dilations – tolerated
well
Meds at visit
Ranitidine Spironolactone/hydrochlorothiazide ¼ lpm oxygen
No inhaled medicines No steroids
Review of Systems
No fevers Adequate growth No hemoptysis No vomiting No steatorrhea Normal thyroid No hematuria Sitting with support
Family and Social History
No asthma, allergies, or lung disease in the family
Lives with mom, “adopted grandparents” in Denver Parents are from Senegal No known TB exposures No pets No smokers
Physical Exam Vitals HR 136 | RR 28 | Ht 65 cm (43%) | Wt 7.5 kg
(39%) SaO2 88% RA | SaO2 95% ¼ lpm
General: happy baby, + drooling HEENT: Down’s facies, small nares, +rhinorrhea Chest: Easy work of breathing, clear to auscultation,
prolonged expiratory phase CVS: RRR, normal S1 and S2, no murmur Abd: Soft, non-tender, no hepatosplenomegaly Ext: No clubbing Neuro: Decreased truncal tone
What should we do?
Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection
What should we do?
Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection
Because of her Down Syndrome, she is at risk for aspiration, heart disease, tracheal anomalies, obstructive sleep apnea, pulmonary hypertension
Bronchoscopy Erythematous mucosa Copious clear secretions Mild-mod malacia of trachea and both mainstem bronchi
LIPID INDEX = 0. IRON INDEX = 270.
Bacterial and viral cultures: negativeCOLOR COLORLESSCHARACTER HAZYNUCLEATED CELLS 910RBC 1705RBC MORPH NORMALSEGS 55LYMPHS 18MONOCYTES 7MACROPHAGES 17
Disorders with pulmonary capillaritis Disorders without pulmonary capillaritis
NoncardiovascularCardiovascular
Chronic heart failure Pulmary Hypertension Pulmonary veno-occlusive disease
Follow-up via phone
New symptomsRhinorrhea Increased cough, respiratory rateSleeping moreRequiring ½ - 1 lpm O2
Intervention5 days of oral steroids
Follow-up
Outpatient echocardiogram scheduled Improved for 2 weeks, then symptoms
returned Treated with 5 more days oral steroids by her
PCP Symptoms do not resolve – present to ED Further history – taking liquids by mouth for 6
weeks as instructed by therapy
Physical Exam in ED
Vitals HR 180 | RR 62 | SaO2 94% 3 lpm General: Infant in moderate respiratory distress HEENT: Down’s facies, +rhinorrhea Chest: Subcostal retractions, tachypneic,
coarse symmetric breath sounds CVS: Tachycardic, prominent S2, 2/6 systolic
flow murmur at LLSB Abd: Soft, non-tender, Liver down 3 cm
What should we do?
Differential Diagnosis – cough, hypoxemia, prominent S2, hepatomegaly
Pulmonary hypertension Aspiration Heart failure Infection
Laboratory
CBC 11.6 WBC 81% Segs, 8% Lymphs, 10% Monos Hb 16.3 g/dL / Hct 51.4 % Platelets 221
CBG pH 7.43CO2 34 mm Hg
Echocardiogram Small secundum ASD with bidirectional flow. Moderate right heart enlargement and moderate
septal flattening. Normal left ventricular size and systolic function. Low
normal RV systolic function. Systemic pulmonary hypertension (on 3 lpm NC O2).
TV jet 4.48 m/secondRV-RA grad 80 mm Hg (SBP 90/69)
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Cardiac catheterization30% FiO2Qp/Qs = 1.25:1; Rp/Rs = 0.46
21% FiO2Qp/Qs = 1.25:1; Rp/Rs = 0.64
100% FiO2 with 40 ppm iNOQp/Qs = 1.14:1; Rp/Rs = 0.47
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Esophagram
•Thread-like appearance of distal esophagus•Fundus of stomach superior to the diaphragm•No normal peristalsis – movement of feeds only with gravity
Swallow study•Deep laryngeal penetration with thin liquids. •No aspiration with pureeds.
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Polysomnogram
Mild sleep-disordered
breathing Apnea-hypopnea
index of 3 events/hour SpO2 in low 80s in RA SpO2 in mid 90s on
¼ lpm NC (≥92% for 99% of TST)
Obstructive Sleep Apnea does not explain PAH
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Further laboratories Protein C and S Prothrombin Antithrombin III Lupus Anticoagulant Factor V Leiden Homocysteine Beta 2 GP1 (antiphospholipid) antibodies Cardiolipin IgG and IgM
Pulmonary Hypertension Causes of secondary PAH
Cardiac/Vascular anomaly Chronic lung disease
Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)
Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV
Treatment of PAH – Vasodilation and Diuretics Continuous oxygen Oral Sildenafil – started at 0.5 mg/kg/dose
and titrated to 2 mg/kg/dose q6h Furosemide 1 mg/kg/dose TID
Thromboembolic disease can contribute to PAH Elevated Beta 2 GP1 antibodies and low
antithrombin III Discussed aspirin or coumadin therapy
Patient’s Echo Changes with Therapy
Date of Echo
Oxygen use via nasal cannula
RV-RA gradient (calculated from TR jet)
Degree of septal flattening
Right heart enlargement
Week 0 3 lpm 80 mm Hg Moderate Moderate
Week 1 1 lpm 60 mm Hg Moderate Moderate
Week 2 ½ lpm / off 50 mm Hg / 70 mm Hg
Mild / Moderate
Mild
Week 6 ½ lpm Unable to estimate; no TR jet
Normal geometry
None
Conclusions
Cause of Pulmonary HypertensionChronic lung disease
Primary Aspiration Trisomy 21 Overcirculation prior to PDA closure
?Thromboembolic disease Cause of increased iron index
Pulmonary Hypertension
Conclusions
Cause of her hypoxiaPulmonary hypertensionChronic lung disease
Cause of her coughChronic lung diseaseAirway protection (aspiration/reflux)Airway Malacia
Outline
Case presentation: Cough, Hypoxia, and Down Syndrome
Differential Diagnosis of Cough and Hypoxia Multi-system complications of patients with
Trisomy 21 Evaluation for Elevated Iron Index and
Pulmonary Hypertension Treatment and Monitoring of PAH
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