Symptoms

 

 

Signs

 

 

 

 

 

DAH can be inflammatory or noninflammatory.

Inflammatory DAH is due to small-vessel vasculitis/capillaritis from a variety of diseases, including granulomatosis with polyangiitis and microscopic polyangiitis. Similarly, systemic autoimmune diseases such as systemic lupus erythematosus can manifest as pulmonary capillaritis. Antibodies to the alveolar basement membrane, as are seen in Goodpasture’s disease, can also result in alveolar hemorrhage. In the early period after bone marrow transplantation, patients can develop a form of inflammatory DAH that can be catastrophic and life-threatening. The exact pathophysiology of this process is not well understood, but DAH should be suspected in patients with sudden-onset dyspnea and hypoxemia in the first 100 days after bone marrow transplantation.

 

 

 

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two ends of a continuum of life-threatening, reactive diseases.

Generally, SJS involves epidermal detachment of less than 10% body surface area (BSA) and TEN greater than 30%; TEN and SJS overlap between 10% and 30%.

Two or more mucosal sites are usually affected. The overall mortality of SJS is 1% to 5%, while that of TEN approaches 30%.1

SJS/TEN begins with a nonspecific prodrome of upper respiratory tract symptoms, fever, fatigue, myalgia, headache, and mucous membrane and skin sensitivities. A rash appears 1 to 3 days later with mucosal lesions first (erythema, erosions, and hemorrhagic crusting) and a simultaneous or lagging, generalized, dusky, erythematous rash. Bullae form, large sheets of epidermis separate from the dermis, and the involved skin is exquisitely tender to palpation. The Nikolsky sign is present when lateral pressure on unblistered skin causes the epidermis to slide off. Progression of involved skin can occur over a single day or slowly evolve over 14 days. In addition to the generalized “skin failure,” life-threatening sepsis, respiratory failure, metabolic derangements, and gastrointestinal hemorrhage may occur. These serious complications may be compounded by underlying comorbidities.

With a few exceptions, SJS/TEN results from a drug exposure, generally within 7 to 21 days previous to the prodrome. Sulfonamide antibiotics, aromatic anticonvulsants (phenytoin, phenobarbital, and carbamazepine), penicillins, nonsteroidal anti-inflammatory drugs (NSAIDs), allopurinol, lamotrigine, and antiretrovirals are common causes, although over 200 medications, including over-the-counter (pseudoephedrine) and herbal remedies, have been implicated. Mycoplasma pneumoniae and vaccinations have also been associated with SJS/TEN.

Management and Disposition

Stop the offending medication (sometimes requiring withholding all). Assess airway status and secure admission to a burn intensive care unit. SJS/TEN patients require careful attention to fluid management; burn protocols may overestimate fluid requirements and contribute to pulmonary edema (resuscitation should maintain urine output at ~0.5-1 mL/kg/h). Dress denuded skin with gauze bandage rolls and moisten with normal saline (transition to antibacterial solution in the burn unit). Emergently consult dermatology and ophthalmology. Meticulous supportive care is the foundation of treatment. With dermatology and burn unit consultation, consider intravenous immune globulins (IVIg) within 72 hours.

Pearls

  1. The only intervention proven to decrease mortality is to stop the offending medication.

  2. If a patient notes continued skin pain, even in areas of normal skin, expect additional sloughing.

  3. High-risk groups to develop TEN include cancer/hematologic malignancies, HIV/AIDS, immunosuppression, slow acetylator genotypes, anticonvulsant use associated with radiotherapy, and specific human leukocyte antigen alleles (HLA-B*1502 associated with carbamazepine and HLA-B*5801 associated with allopurinol).

  4. Infections and pulmonary edema/acute respiratory distress syndrome (ARDS) are the leading causes of death in TEN patients.

 

 

 

 

 

 

 

 

 

 

Most people with pyelonephritis do not have complications if appropriately treated with bacteria-fighting medications called antibiotics.

In rare cases, pyelonephritis may cause permanent kidney scars, which can lead to chronic kidney disease, high blood pressure, and kidney failure. These problems usually occur in people with a structural problem in the urinary tract, kidney disease from other causes, or repeated episodes of pyelonephritis.

Infection in the kidneys may spread to the bloodstream—a serious condition called sepsis—though this is also uncommon.

 

 

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Content 11

 

A

A 75-year-old triathlete complains of gradually worsening vision over the past year. It seems to be involving near and far vision. The patient has never required corrective lenses and has no significant medical history other than diet-controlled hypertension. He takes no regular medications. Physical examination is normal except for bilateral visual acuity of 20/100. There are no focal visual field defects and no redness of the eyes or eyelids. Which of the following is the most likely diagnosis?

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The correct answer is A. You answered A.

Age-related macular degeneration is a major cause of painless, gradual bilateral central visual loss. It occurs as nonexudative (dry) or exudative (wet) forms. Recent genetic data have shown an association with the alternative complement pathway gene for complement factor H. The mechanism link for that association is unknown. The nonexudative form is associated with retinal drusen that leads to retinal atrophy. Treatment with vitamin C, vitamin E, beta-carotene, and zinc may retard the visual loss. Exudative macular degeneration, which is less common, is caused by neovascular proliferation and leakage of choroidal blood vessels. Acute visual loss may occur because of bleeding. Exudative macular degeneration may be treated with intraocular injection of a vascular endothelial growth factor antagonist (bevacizumab or ranibizumab). Blepharitis is inflammation of the eyelids usually related to acne rosacea, seborrheic dermatitis, or staphylococcal infection. Diabetic retinopathy, now a leading cause of blindness in the United States, causes gradual bilateral visual loss in patients with long-standing diabetes. Retinal detachment is usually unilateral and causes visual loss and an afferent pupillary defect.

 

Mr. Jenson is a 40-year-old man with a congenital bicuspid aortic valve who you have been seeing for more than a decade. You obtain an echocardiogram every other year to follow the progression of his disease knowing that bicuspid valves often develop stenosis or regurgitation requiring replacement in middle age. Given his specific congenital abnormality, what other anatomic structure is important to follow on his biannual echocardiograms?

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The correct answer is A. You answered A.

The answer is A. (Chap. 282) Bicuspid aortic valve is among the most common of congenital heart cardiac abnormalities. Valvular function is often normal in early life and thus may escape detection. Due to abnormal flow dynamics through the bicuspid aortic valve, the valve leaflets can become rigid and fibrosed, leading to either stenosis or regurgitation. However, pathology in patients with bicuspid aortic valve is not limited to the valve alone. The ascending aorta is often dilated, misnamed “poststenotic” dilatation; this is due to histologic abnormalities of the aortic media and may result in aortic dissection. It is important to screen specifically for aortopathy because dissection is a common cause of sudden death in these patients.

 


 

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