CCS Practice Case 4 Flashcards
Location: Office
Presenting complaint: 2 y/o M, failure to gain weight and loose stools
Vitals: HR 100, BP 80/56, Temp 101.2 F, RR 18, 27.2” tall, 16 lb
HPI: 2 y/o Caucasian child brought to the office by his parents for evaluation of loose greasy stools and failure to gain weight despite adequate nutrition. Intermittent productive cough and rhinorrhea. Wheezing and coughing up purulent expectoration for 4 days. Deny fever or chills. Pneumonia 4x since birth. Delivery and neonatal course were uncomplicated. Breast-fed until age 4 months. History of CF in the family of both of his parents.
ROS:
No rashes or lesions, +nasal discharge, no joint swelling, no GU complaints, delayed development vaccinations updated
How to approach this case?
Failure to thrive - DDx includes organic and non-organic causes. Etiology is most likely cystic fibrosis in the case - clues include +family history, repeated chest infections, malabsorptive diarrhea
Do a full physical exam. Results:
Emaciated. Height and weight are lower than expected for age. Chest exam shows generalized hyper-resonance and scattered crepitations bilaterally. Also has wheezing especially during expiration.
Initial orders?
Stat unless otherwise noted Admit to ward Pulse ox, stat and every 4 hours IV access Sputum gram stain Sputum culture and sensitivity Blood cultures CBC w/diff BMP CXR - PA/Lateral Sinus X-ray Sweat chloride, routine 72 hour fecal fat estimation, routine
Initial treatment?
Oxygen inhalation, continuous (if saturations are <92% on RA)
Amoxicillin and clavulonic acid, oral, continuous (Augmentin)
Nebulized albuterol, inhalation, continuous QID
MV tablets, oral, continuous
Chest physiotherapy
Vitals Q6hrs
D5NS, IV, continuous
Regular diet
Ambulation at will
Results:
CBC - neutrophilic leukocytosis Sweat chloride: 85 meQ/L (elevated) BMP: low Na, low K CXR: hyperinflation in both lung fields X-ray PNS shows opacification of paranasal sinuses GS of sputum does not show any predominant organism Sputum culture pending 72-hour fecal fat pending
AR Disorder, commonly affecting Caucasians
Manifestations include acute or persistent respiratory symptoms, FTT, meconium ileus, diarrhea, rectal prolapse, nasal polyps, electrolyte or acid-base disorders, and hepatobiliary disease
Dx: evidence of CFTR dysfunction (elevated sweat chloride on 2 separate occasions) + typical clinical features or positive family history
Other important tests include gram stain, culture, and sensitivity of sputum, CXR, and X-ray of paranasal sinuses. 24 hour fecal fat testing should be used to diagnose malabsorption. Genetic testing is not required to make the diagnosis.
Initial evaluation of CF + next steps for follow-up?
Admit to the hospital if suspected and not previously diagnosed for baseline testing, accurate diagnosis, initiation of treatment, and education of patient and parents
F/u every 2-3 months - H&P, staining and culture of sputum or pharyngeal swab at each f/u
Immunize against influenza, measles, and pertussis
Which ABX should be given in patients with CF? When are they indicated?
Acute or subacute increase in sputum production, cough, dyspnea, and/or fever
Based on results of sputum culture (performed at least yearly)
PO when mild, IV when severe or resistance to oral ABX
S. aureus - cephalexin, dicloxacillin, amox-clav
P. aeruginosa - ciprofloxacin (PO), tobramycin + piperacillin (IV)
What other treatments are used in CF?
Bronchodilators like albuterol or salmeterol if airflow obstruction
DNase - daily productive cough + airflow obstruction
Physiotherapy + exercise if retained purulent secretions
Inhaled glucocorticoids if evidence of airway hyperactivity
O2 if hypoxemia at night or rest or pulmonary HTN
Lung transplantation - definitive if severe infections and grossly damaged lungs with FEV1 of 30% or less of predictive value
Nutrition: high protein, high calorie; fat-soluble vitamins and pancreatic enzyme replacement
72-hour fecal fat elevated
Sputum culture grew S. aureus, sensitive to cephalexin
Next steps?
D/C Augmentin, D/C IV fluids Stat cephalexin, PO, continuous Influenza vaccine Pneumococcal vaccine Consult dietician Pancreatic enzymes, oral, continuous Genetic counseling F/u at 2-3 months
Primary diagnosis?
Cystic fibrosis
