Ward Flashcards
What does FeNO stand for?
Fraction exhaled nitric oxide
What is FeNO and what deos it tell us
- Gaseous molecule produced in response to an inflammatory process and may aid in differentiating asthma from other lung conditions
- A non-invasive marker for eosinophilic airway inflammation and a good predictor of response to corticosteroids
- Nitric oxide (NO) plays an important role in the immune system by controlling the vascular and bronchial tone
- In addition to asthma, FeNO has value in differentiating types of asthma from COPD and other conditions presenting with similar symptoms such as gastroesophageal reflux disease (GERD), vocal cord dysfunction, and eosinophilic bronchitis
How is FeNO measured?
A device that a patient breathes into analyzes the air and detects NO
FeNO levels
FeNO levels of < 25 of parts per billion (ppb) is considered normal, 25 ppb - 50 ppb as intermediate, and > 50 ppb high
Which condition can we find elevated FeNO?
- High FeNO levels indicate your airways are inflamed or irritated
- Although definitive data linking asthma and FeNO is lacking, some strong conclusions can be made, thus improving the correlation of FeNO to the condition in asthmatics. Research suggests that the patients with asthma generally have (1) higher concentration of NO detected in their breath test, (2) inflammatory insult leads to elevation of FeNO, (3) FeNO levels fluctuate due to hyper-inflammation leading to high FeNO levels
What is stevens Johnson syndrome?
- A rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss accompanied by systemic symptoms
- Toxic epidermal necrolysis (TEN) is a variant of the same condition
What is the cause of Johnson syndrome?
- Medications are causative in over 80% of cases (unpredictable reaction
How is Stevens Johnson syndrome classified as?
Stevens-Johnson syndrome/toxic epidermal necrolysis is classified by the extent of the detached skin surface area:
- Stevens-Johnson syndrome: less than 10% body surface area
- Overlap Stevens-Johnson syndrome/toxic epidermal necrolysis: 10% to 30% body surface area
- Toxic epidermal necrolysis more than 30% body surface area
How rare is Stevens Johnson syndrome and who does it affect?
- Estimated to affect two to seven per million people each year
- more common in older people and women
Which drugs most commonly cause Steven Johnsons syndrome?
The drugs that most commonly cause Stevens-Johnson syndrome/toxic epidermal necrolysis are:
- Anticonvulsants: lamotrigine, carbamazepine, phenytoin, phenobarbitone
- Allopurinol, especially in doses of more than 100 mg per day
- Sulfonamides: cotrimoxazole, sulfasalazine
- Antibiotics: penicillins, cephalosporins, quinolones, minocycline
- Paracetamol/acetaminophen
- Nevirapine (non-nucleoside reverse-transcriptase inhibitor)
- Nonsteroidal anti-inflammatory drugs (NSAIDs) (oxicam type mainly)
- Contrast media
What does HLA stand for (immunology)?
human leukocyte antigen
Pathophysiology
The initial step for Stevens-Johnson syndrome/toxic epidermal necrolysis may be interaction/binding of a drug-associated antigen or metabolite with the major histocompatibility complex (MHC) type 1 or cellular peptide to form an immunogenic compound
- T cell mediated
- Involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNF–alpha)/death receptor pathway
- Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis.
Symptoms of stevens Johnson syndrome
- Early stage Steven-Johnson syndrome can include flu-like symptoms, such as fever, sore throat, cough or joint pain. A few days later, a skin rash may appear on any part of the body and may feel like sunburn or lesions. Other symptoms, which appear shortly after the initial issues, may include:
Blisters on the skin, mouth, eyes, nose and genitals
Shedding skin after the blisters form
Unexplained pain on the skin
Swelling of the mouth, lips, throat, tongue or face
The illness begins with nonspecific symptoms such as fever and malaise, upper respiratory tract symptoms such as a cough, rhinitis, sore eyes, and myalgia. Over the next three to four days, a blistering rash and erosions appear on the face, trunk, limbs, and mucosal surfaces.
Erythematous, targetoid, annular (shaped like a ring), or purpuric macules
Flaccid bullae
Large painful erosions
Nikolsky-positive (lateral pressure on the skin results in shedding of the epidermis)
Early on, toxic epidermal necrolysis displays widespread tender erythroderma and erosions (with or without targetoid rash), whereas Stevens-Johnson syndrome is characterized more by targetoid rash, with fewer areas of denudation.
Mucosal ulceration and erosions can involve lips, mouth, pharynx, esophagus and gastrointestinal tract, eyes, genitals, upper respiratory tract. About half of patients have involvement of three mucosal sites.
The patient is very ill, anxious, and in pain. Liver, kidneys, lungs, bone marrow, and joints may be affected by Stevens-Johnson syndrome/toxic epidermal necrolysis. Typical symptoms include:
Fever, malaise, headache, anorexia, pharyngitis
Symptoms due to acute dysfunction of ocular, pulmonary, cardiovascular, gastrointestinal, renal, and hematological systems.
Features may overlap with other severe cutaneous adverse reactions (SCAR), such as acute generalized exanthematous pustulosis (causing subcorneal pustules) and drug hypersensitivity syndrome (causing a morbilliform eruption and involving other organs)
SJS treatment
Care of a patient with Stevens-Johnson syndrome/toxic epidermal necrolysis requires supportive care, including:
- Cessation of the suspected causative drug(s)
- Hospital admission: preferably to an intensive care and/or burn unit
- Fluid replacement (crystalloid)
- Nutritional assessment: may require nasogastric tube feeding
- Temperature control: warm environment, emergency blanket
- Pain relief
- Infection control
- Supplemental oxygen and, in some cases, intubation with mechanical ventilation
Sterile/aseptic handling
It is unknown whether systemic corticosteroids are beneficial, but they are often prescribed in high doses for the first three to five days of admission
Complications of steven Johnson’s syndrome
In the acute phase, sepsis is the most common serious risk of Stevens-Johnson syndrome/toxic epidermal necrolysis. Organ failure may occur, including pulmonary, hepatic, and renal systems.
The most common long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis are ocular (including blindness), cutaneous (pigmentary changes and scarring), and renal. Mucosal involvement with blisters and erosions can lead to strictures and scarring