1000-2000 5 ьшò

Oral glucose tolerance test (OGTT) annually after age 10 years

Head CT: Abnormal sinus CT findings are nearly universal in CF and may include mucosal thickening, intraluminal sinus polyps, and sinus effusions.

Chest CT (not routine): useful when unusual findings noted on CXR

Diagnostic Procedures/Other

Flexible bronchoscopy Bronchoalveolar lavage

TREATMENT

GENERALMEASURES

CF Foundation Guidelines call for yearly evaluation:

–Four office visits, four respiratory cultures, PFTs q6mo, and at least 1 evaluation by a multidisciplinary team including dietitian, GI, and social worker

–PFT goals: >75% predicted for adults, >100% predicted for children <18 years old

–Annual screening for ABPAfor patients >6 years with total serum IgE concentration

–Annual influenza vaccination for all CF patients age >6 months

–Screen all adults for osteoporosis with a DXAscan.

–Annual measurement of fat soluble vitamins to r/o vitamin deficiencies

–Annual LFTs

–Decrease exposure to tobacco smoke.

All patients should be followed in a CF center (accredited sites are listed at www.cff.org).

Infant care:

–Monthly visits for first 6 months of life and then every 2 months until 1 year of life

–Fecal elastase testing and salt supplementation after diagnosis

–Consider palivizumab for RSV prophylaxis in infants with CF <2 years (4) [A].

MEDICATION

Pathogens for pulmonary infections: methicillin-resistant Staphylococcus aureus (MRSA) and methicillin-sensitive S. aureus (MSSA),

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Stenotrophomonas maltophilia, Pseudomonas aeruginosa, Burkholderia cepacia, nontuberculous mycobacteria (NTM)

– Antibiotics for acute pulmonary infections should be prescribed according to most likely pathogen; most antibiotic courses are for 2 weeks.

Pulmonary infections:

–Antibiotics, oral

S. aureus: Bactrim (MRSA), doxycycline (MRSA) or cephalexin

P. aeruginosa: fluoroquinolones

–Antibiotics, inhaled

Tobi (tobramycin): for P. aeruginosa, nebulizer twice daily for 28 days; stop for 28 days, and then resume use (5)[A].

Colistin (more commonly used in Europe)

Cayston (aerosolized aztreonam) (5)[A]

–Antibiotics, IV

S. aureus: cefazolin or nafcillin

MRSA: vancomycin or linezolid

P. aeruginosa: Zosyn or ceftazidime plus aminoglycoside (tobramycin)

Dual therapy synergistic (5)[A]

Medications recommended for chronic use in pulmonary disease:

–Recombinant human DNAse (Dornase alfa) (5)[A]

–Hypertonic saline

–High-dose ibuprofen in patients 6 to 17 years old with FEV1 ≥60 PPV

–Inhaled tobramycin or aztreonam in P. aeruginosa–positive patients

–Azithromycin in P. aeruginosa–positive patients

–Ivacaftor (VX770) and lumacaftor: CFTR potentiators approved in 2015 for

patients with two copies of the F508del mutation. This vastly increases the number of people with CF eligible for this therapy (>50%) (1)[A].

Inhaled steroids are not recommended for chronic use in the absence of asthma or ABPA.

Insufficient evidence to recommend for or against chronic use: inhaled β- agonist, inhaled anticholinergics, leukotriene modifiers, inhaled colistin

–Pancreatic enzymes (87.3%) (1)[A]

Often combined with H2 blocker or PPI to increase effectiveness

–Fat-soluble vitamin supplementation (A, D, E, and K)

–Liver disease (cholestasis)

Ursodeoxycholic acid has not been proven effective.

ADDITIONALTHERAPIES

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High-frequency chest wall oscillation vest is the most widely used airway clearance technique.

Aerobic exercise is used as an adjunct therapy for airway clearance.

– CF-related bone disease: Consider bisphosphonate therapy.

SURGERY/OTHER PROCEDURES

Timing for lung transplantation (bilateral) is polyfactorial (6)[A].

5-year posttransplant survival is up to 62%.

Liver transplantation is reserved for progressive liver failure ± portal hypertension with GI bleeding.

Nasal polypectomy in 4.5% of CF patients (1)[A]

ADMISSION, INPATIENT, AND NURSING CONSIDERATIONS

Pulmonary exacerbation (most common reason for admission)

Bowel obstruction (due to DIOS, previously known as meconium ileus equivalent [MIE])

Pancreatitis (in pancreatic-sufficient patients)

CF exacerbations should always be admitted on contact precautions and private rooms.

Nasal cannula oxygen when the patient is hypoxemic (SaO2 <90%)

Increased salt loss increases risk of hyponatremic hypochloremic dehydration.

Cautious use of IV fluids with worsening lung disease

Nursing assignments should involve only 1 CF patient per nurse for isolation purposes.

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Upon discharge for a pulmonary exacerbation, follow-up with CF provider within 2 to 4 weeks.

Routine clinic visits every 3 months, with airway cultures and pulmonary function testing

Annual comprehensive nutritional evaluation (4)[A]

DIET

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High-calorie, high-fat diet titrated to specific BMI goals established by the CF

Foundation nutrition guidelines. If not meeting nutritional goals, dietitian, pancreatic enzyme, oral or tube supplemental feeding should be considered if indicated.

PATIENT EDUCATION

Cystic Fibrosis Foundation: www.cff.org

PROGNOSIS

Median survival is 39.3 years.

Progression of lung disease usually determines length of survival.

REFERENCES

1.Cystic Fibrosis Foundation. Patient Registry: Annual Data Report to the Center Directors 2013. Bethesda, MD: Cystic Fibrosis Foundation; 2014.

2.Grigoriadis C, Tympa A, Theodoraki K. Cystic fibrosis and pregnancy: counseling, obstetrical management and perinatal outcome. Invest Clin. 2015;56(1):66–73.

3.Gilljam M, Ellis L, Corey M, et al. Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Chest. 2004;126(4):1215–1224.

4.Borowitz D, Robinson KA, Rosenfeld M, et al; for Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(Suppl 6):S73–S93.

5.Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; for Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187(7):680–689.

6.Weill D, Benden C, Corris PA, et al. Aconsensus document for the selection of lung transplant candidates: 2014—an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34(1):1–15.

ADDITIONALREADING

Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2014;(3):CD002010.

Mall MA, Boucher RC. Pathophysiology of cystic fibrosis lung disease. In:

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Mall MA, Elborn JS , eds. Cystic Fibrosis. Vol 64. Sheffield, United

Kingdom: European Respiratory Society; 2014:1–13.

CODES

ICD10

E84.9 Cystic fibrosis, unspecified

E84.11 Meconium ileus in cystic fibrosis

E84.0 Cystic fibrosis with pulmonary manifestations

CLINICALPEARLS

Meconium ileus is virtually pathognomonic for CF.

When sweat test is equivocal, CFTR genetic testing is diagnostic.

CF must be considered in any child with chronic diarrhea, especially if associated with poor growth or failure to thrive.

All children with nasal polyps, digital clubbing, or bronchiectasis should be evaluated.

Arapid decline in pulmonary function suggests the acquisition of resistant organisms (e.g., B. cepacia), CFRD, ABPA, or GERD.

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DE QUERVAIN TENOSYNOVITIS

Caitlin G. Waters, MD J. Herbert Stevenson, MD

BASICS

DESCRIPTION

First identified in 1895 by Fritz De Quervain, de Quervain tenosynovitis is a painful condition due to stenosis of the tendon sheath in the 1st dorsal compartment of the radial aspect of the wrist.

Caused by repetitive motion of the extensor pollicis brevis (EPB) and abductor pollicis longus (APL) over the radial styloid with resultant irritation of the surrounding tendon sheath

EPIDEMIOLOGY

The predominant age range is 30 to 50 years.

Women are affected more commonly than men (1).

With new occupational and professional demands, the prevalence of this condition is increasing gradually.

Incidence

The overall incidence of de Quervain tenosynovitis is 0.9/1,000 person-years.

For patients age >40 years, the incidence is 1.4/1,000 person-years compared with 0.6/1,000 person-years for those <20 years.

Women have an incidence rate ratio of 2.8/1,000 person-years compared with 0.6/1,000 person-years in men.

The incidence ratio rate of de Quervain tenosynovitis is 1.3/1,000 personyears in blacks and 0.8/1,000 person-years in whites (1).

ETIOLOGYAND PATHOPHYSIOLOGY

Repetitive motions of the wrist and/or thumb result in microtrauma and thickening of the tendons (EPB, APL) and surrounding tendon sheath.

EPB and APL movement is resisted as they glide over the radial styloid causing pain with movements of the thumb and wrist.

RISK FACTORS

Women age 30 to 50 years

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Pregnancy (primarily 3rd trimester and postpartum)

African American

Systemic diseases (e.g., rheumatoid arthritis)

Participation in activities that include repetitive motion or forceful grasping with thumb and wrist deviation such as golf, fly fishing, racquet sports, rowing, or bicycling, video gaming, and more recently text messaging

Repetitive movements with the hand/thumb requiring forceful grasping with wrist involving ulnar/radial deviation; dental hygienists, musicians, carpenters, assembly workers, and machine operators

GENERALPREVENTION

Avoid overuse or repetitive movements of the wrist and/or thumb associated with forceful grasping and ulnar/radial deviation.

DIAGNOSIS

HISTORY

Repetitive motion activity; overuse of wrist or thumb

Gradually worsening pain along the radial aspect of the thumb and wrist with certain movements, particularly ulnar deviation of the wrist

Pregnancy

Sports, leisure, and occupational history

Trauma (rare)

PHYSICALEXAM

Pain over the radial styloid exacerbated when patients move the thumb or make a fist

Crepitus with movement of the thumb

Swelling over the radial styloid and base of the thumb

Decreased range of motion of the thumb

Pain over the 1st dorsal compartment on resisted thumb abduction or extension

Tenderness may extend proximally or distally along the tendons with palpation or stress.

Finkelstein test: The examiner grasps the affected thumb and deviates the hand sharply in the ulnar direction. Apositive test occurs when there is pain along the distal radius.

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Eickhoff test: Patient grasps a flexed thumb, and the examiner deviates the wrist in an ulnar direction.

Finkelstein test is more sensitive for determining tenosynovitis of the APL and EPB tendons (2)[A].

DIFFERENTIALDIAGNOSIS

Scaphoid fracture

Scapholunate ligament tear

Dorsal wrist ganglion

Osteoarthritis of the 1st carpometacarpal (CMC) joint

Flexor carpi radialis tendonitis

Infectious tenosynovitis

Tendonitis of the wrist extensors

Intersection syndrome

Trigger thumb

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests (lab, imaging)

Primarily a clinical diagnosis

Radiographs of the wrist to rule out other pathology, such as CMC arthritis, if the diagnosis is in question

MRI is the imaging test of choice to rule out coexisting soft tissue injury or wrist joint pathology.

Follow-Up Tests & Special Considerations

Ultrasound can help to detect anatomic variations in the 1st dorsal extensor compartment of the wrist and target corticosteroid injections (3,4).

Test Interpretation

Inflamed and thickened retinacular sheath of the tendon

TREATMENT

Most cases of de Quervain syndrome are self-limited.

Rest and NSAIDs (2)[A]

Ice (15 to 20 minutes 5 to 6 times a day)

Immobilization with a thumb spica splint (2)[A]

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Occupational therapy

Corticosteroid injection (ultrasound guided)

Consider surgery if conservative measures fail >6 months.

GENERALMEASURES

If full relief is not achieved, a corticosteroid injection of the tendon sheath can improve symptoms.

Anatomic variation, including two tendon sheaths in the 1st compartment or the EPB tendon traveling in a separate compartment may complicate treatment. Ultrasound can distinguish these variants and improve anatomic accuracy of injections (3).

Surgical release may be indicated after 3 to 6 months of conservative treatment if symptoms persist. Surgery is highly effective and has a relatively low rate of complications.

MEDICATION

First Line

Splinting, rest, and NSAIDs

Second Line

Corticosteroid injection of the tendon sheath has shown significant cure rates. Additional injections are sometimes required.

Corticosteroid injection plus immobilization is more effective than immobilization alone (5)[B].

Percutaneous tenotomy and/or injection of platelet-rich plasma are newer techniques that show promise for treatment of de Quervain tenosynovitis.

ISSUES FOR REFERRAL

Referral to a hand surgeon is indicated if there is no improvement with conservative therapy.

ADDITIONALTHERAPIES

Hand therapy, along with iontophoresis/phonophoresis, may help improve outcomes in persistent cases.

Patients may use thumb-stretching exercises as part of their rehabilitation.

SURGERY/OTHER PROCEDURES

Indicated for patients who have failed conservative treatment

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Endoscopic release may provide earlier relief, fewer superficial radial nerve complications, and greater patient satisfaction with resultant scar compared to open release (5)[B].

ADMISSION, INPATIENT, AND NURSING CONSIDERATIONS

Hospitalization for care associated with surgical treatment

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Additional corticosteroid injection may be performed at 4 to 6 weeks if symptoms persist. Caution with repeat steroid injections.

Avoid repetitive motions and activities that cause pain.

DIET

As tolerated

PATIENT EDUCATION

Activity modification: Avoid repetitive movement of the wrist/thumb and forceful grasping.

PROGNOSIS

Extremely good with conservative treatment

Complete resolution can take up to 1 year.

95% success rates have been shown with conservative therapy >1 year.

Up to 1/3 of patients will have persistent symptoms.

COMPLICATIONS

Most complications are secondary to treatment. These include GI, renal, and hepatic injury secondary to NSAID use.

Nerve damage may occur during surgery.

Hypopigmentation, fat atrophy, bleeding, infection, and tendon rupture have been reported as potential adverse events from corticosteroid injection. Ultrasound guidance reduces the rate of complications.

If not appropriately treated, thumb flexibility may be lost due to fibrosis.

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