Educational Objective:

Identify the treatment of choice for hypercholesterolemia.

Key Point:

Statins are the medication of choice in treating hypercholesterolemia. Their safety and efficacy are superior to other agents used to decrease cholesterol levels, such as nicotinic acid and bile-acid sequestrants (cholestyramine, colestipol, and colesevelam).

Explanation:

This patient has high levels of cholesterol, low density lipoprotein, and triglycerides, but she has a low level of high density lipoprotein. Her risk for cardiovascular events warrants drug therapy.

Several classes of drugs decrease atherogenic cholesterol levels and cardiovascular risk. In 2001, the Adult Treatment Panel III guidelines recommended a statin, bile-acid sequestrant, or nicotinic acid. Since then, however, efficacy and safety data have accumulated for statins, and generic, high-intensity statins are now available. Current guidelines from the American College of Cardiology and American Heart Association strongly recommend statins as first-line pharmacologic therapy. Furthermore, they advise clinicians to prioritize efforts to maximize statin dose and to try alternative statins or dosing regimens in patients with intolerance.

References:

Keaney JF Jr, Curfman GD, Jarcho JA. A pragmatic view of the new cholesterol treatment guidelines. N Engl J Med. 2014;370(3):275-278.

Martin SS, Blumenthal RS. Concepts and controversies: the 2013 American College of Cardiology/American Heart Association risk assessment and cholesterol treatment guidelines. Ann Intern Med. 2014;160(5):356-358.

Pencina MJ, Navar-Boggan AM, D'Agostino RB Sr, et al. Application of new cholesterol guidelines to a population-based sample. N Engl J Med. 2014;370(15):1422-1431.

Stone NJ, Robinson JG, Lichetenstein AH, et al; American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce atherosclerotic cardiovascular risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines [Erratum appears in J Am Coll Cardiol. 2014;63(25 pt B):3024-3025]. J Am Coll Cardiol. 2014;63(25 pt B):2889-2934.

Educational Objective:

Recognize the presentation of diabetic cardiomyopathy.

Key Point:

Diabetic cardiomyopathy manifests as left ventricular hypertrophy and diastolic HF.

Explanation:

The most likely cause of HF in this patient is diabetic cardiomyopathy. He shows signs of HF during diastole in which the ejection fraction remains normal, but there is decreased relaxation of the ventricles, thereby increasing filling pressure. Diabetic cardiomyopathy is an established cause of left ventricular hypertrophy and diastolic dysfunction. Although patients with diabetic cardiomyopathy can have systolic HF, it remains unclear if this is the later stages of diastolic HF or a different phenotype of diabetic cardiomyopathy.

Alcohol cardiomyopathy, idiopathic dilated cardiomyopathy, and viral cardiomyopathy are characterized by systolic dysfunction and reduced ejection fraction. This patient has normal vital signs and does not have any findings that suggest the development of systolic dysfunction.

Reference:

Battiprolu P, et al. Diabetic cardiomyopathy: mediators and mechanisms. In: McGuire D, Marx N. Diabetes in Cardiovascular Disease: A Companion to Braunwald's Heart Disease. 2015:290-301.

Educational Objective:

Define the principles of refusal of care.

Key Point:

Refusal of medical care is a process of informed decision-making and not just a signature on a form.

Explanation:

Refusal of medical care is a process of informed decision-making, not just a signature. Prior to honoring a patient's refusal of care, the physician should explore the patient’s rationale for refusal and attempt to resolve any barriers to care.

Competent adults have a legally protected right to refuse medical care. This right, however, is not absolute and may be overruled by compelling circumstances such as the prevention of suicide, preservation of life, or protection of third parties.

Physicians who honor a competent patient’s decision to refuse treatment are not liable for any subsequent bad outcomes related to treatment refusal. Indeed, even when the condition is life-threatening, physicians are more likely to be successfully sued for treating a competent patient over their objection rather than for any bad outcome resulting from honoring the informed refusal.

There are 4 essential components of the refusal process. These are:

1. Determining capacity for decision making
2. Ensuring an informed decision
3. Involving others to the extent possible
4. Documenting appropriately

 

Normal mental status and documentation of the patient’s rationale for refusal imply capacity. Informed decisions involve explaining the patient’s condition, potential complications of a procedure, and alternative treatments, if available. Patients have the legal right to forbid the physician from speaking with others about their care. However, involvement of family, friends, or a personal physician is encouraged whenever possible, because these people may be able to persuade the patient to accept the recommended care. The patient should sign a refusal form; but, if he or she declines to do so, this should be documented and the form signed by a hospital representative who witnessed the refusal.

Refusal of one intervention does not imply refusal of all medical care. The physician should continue to treat to the extent allowed by the patient. Patients should also be invited to change their mind or return for care at any time.

Reference:

Bitterman RA. Emergency medical treatment and active labor act and medicolegal issues. In: Marx JA, et al, eds. Rosen’s Emergency Medicine. 8th ed., 2014:e60-e78.

Neglect is the failure or refusal of caregivers to fulfill any of their duties or obligations to an elderly individual, which has resulted in or is likely to result in serious harm to the elderly individual. It can be intentional or unintentional and may consist of withholding of food, water, clothing, shelter, medications, medical equipment (e.g., walker, cane, glasses, hearing aid, dentures), or medical appointments. The others are also types of elder abuse, but neglect is the most common.

Reference:

Anglin, Dierdre et. al. Elder Abuse and Neglect. In: Marx JA, et al, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 8th ed., 2014

The time course of profound central nervous system and respiratory depression with rapid recovery is typical for gamma hydroxybutyrate (GHB) intoxication. GHB is an endogenous neurotransmitter with its own unique GHB receptor. Exogenous GHB also interacts with GABAb receptors and results in sedation that may progress to profound coma and respiratory depression. Vomiting, myoclonic motor activity, sialorrhea, and bradycardia may be seen with intoxication. Paradoxical agitation or abrupt agitation during intubation or other procedures may occur. It is uncertain whether GHB causes seizures in humans or if the seizure-like activity observed is myoclonic jerking.

Exogenous GHB is rapidly absorbed from the gastrointestinal tract, with an onset of symptoms within 15-20 minutes. Effects peak within 1 hour and recovery is rapid because the half-life is less than 1 hour. Precursors of GHB are also abused and include gamma-butyrolactone (GLB) and 1,4-butanediol (1,4 BD). The onset of action with GLB is also rapid, while the onset of symptoms after 1,4 BD is delayed because it requires metabolic conversion to GHB via alcohol and aldehyde dehydrogenase. Co-ingestion of ethanol will slow this conversion and may also result in prolonged toxicity with larger exposures of 1,4 BD.

Management of a GHB exposure is primarily supportive with airway/ventilatory support as needed. Aspiration should also be considered if there has been vomiting. Bradycardia rarely needs treatment.

If this patient remains alert with clearing of his mental state and improved behavior, then he may be medically cleared after a complete physical examination.

Illicitly produced GHB may result in caustic injury to the oral cavity, esophagus, and stomach from residual caustics used in the manufacture process.

References:
Barceloux DG. Gamma hydroxybutyrate and related drugs. In: Barceloux DG. Medical Toxicology of Drug Abuse: Synthesized Chemicals and Psychoactive Plants. 2012:89-109.
Farmer BM. Y-hydroxybutyric acid. In: Nelson LS, et al, eds. Goldfrank's Toxicologic Emergencies. 9th ed., 2010.
Gussow L, Carlson A. Sedative hypnotics. In: Marx JA, et al, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 7th ed., 2010:2071-2082.

Educational Objective:

Recognize the appropriate treatment of scalp lacerations.

Key Point:

Irrigation is required in all nonsurgical wounds. Deep scalp wounds can be closed by closing the epidermis, dermis, and galea with a single layer, usually with a 4-0 or thicker suture.

Explanation:

The heavy galea makes a great anchor for suture, so it should be used when it is available.

It is important to remember that the same color of suture as the patient's hair should NOT be used. Even if you trim the hair, it grows back quickly, making it difficult to find the sutures again.

Although digital exploration is still commonly taught, due to the risk of sharp foreign bodies and sharp skull fracture edges, using a closed straight hemostat as a probe is the preferred initial method. It is smaller and allows deeper penetration and is safer. If a gentle touch is used on the hemostat, then the "tick" of hard foreign bodies are easy to detect at a first pass. If nothing is found, then digital probing with a gloved finger can be considered as a follow-up step.

Reference:

Papa L, Goldberg SA. Head trauma (chapter 34). In: Walls R, et al. Rosen's Emergency Medicine Concepts and Clinical Practice. 9th ed., 2018:301-329.

Stress incontinence

The signs and symptoms of stress incontinence occur when the increased abdominal pressure caused by sneezing, laughing, and/or straining results in the leaking of a small amount of urine through a weakened sphincter. It is more common in younger women (45–49 years old). The urethra and bladder neck fail to close completely due to loss of connective tissue support.

Rheumatoid arthritis and degenerative joint disease

Enlargement of the proximal interphalangeal joint (middle joint) of a finger is called a Bouchard’s node and it is found in both rheumatoid arthritis and degenerative joint disease (or osteoarthritis). Heberden’s nodes (bony overgrowths) are classic signs of osteoarthritis. They are located at the distal interphalangeal joints. They are felt as hard, nontender nodules usually 2 to 3 cm in diameter, but sometimes encompass the entire joint.

Cauda equina syndrome

Acute cauda equina syndrome is a surgical emergency. It requires decompression surgery of the spine to prevent permanent nerve damage of the affected nerve roots. The cauda equina contains 18 nerve roots. MRI is the best imaging test for evaluating this condition. The most common cause of compression is a herniated nucleus pulposus. Most herniations occur either at L4– L5 or L5–S1. The spinal nerves can also become infected (e.g., in tuberculosis, herpes, cytomegalovirus infections). Symptoms include low-back pain, sciatica (unilateral or bilateral), saddle pattern paresthesias, bladder and/or bowel incontinence, and lower extremity motor and sensory abnormalities. Sciatic pain is typically described as sharp or searing, rather than dull. Some patients experience a “pins-and-needles” sensation, numbness or weakness, or a prickling sensation down the leg.

Neurofibromatosis type 1, also known as von Recklinghausen disease, is the condition most commonly associated with NF1 mutations. Mutations have been identified throughout the gene, but gross deletions of NF1 of a maternal origin are associated with developmental delay, learning disability, and dysmorphic features.

The diagnosis of neurofibromatosis type 1 requires the presence of 2 or more of the following:

• ≥ 6 cafe-au-lait macules > 5 mm in greatest diameter in prepubertal individuals and > 15 mm in greatest diameter after puberty
• ≥ 2 neurofibromas of any type or 1 plexiform neurofibroma
• Freckling in the axillary or inguinal regions
• Optic pathway tumor
• ≥ 2 Lisch nodules

Hemolytic anemia and diabetes are rarely associated with neurofibromatosis type 1. Bilateral schwannomas are associated with neurofibromatosis type 2 that results from NF2 mutations.

Gross deletions are also a major contributor to several notable cancers and inherited diseases. Cancer genomes are highly unstable and often exhibit gross losses of chromosomal material. Gross deletions have been linked with leukemia, ovarian, breast, and prostate cancers.

References:

Chen, J-M, Cooper DN, Ferec C, Kehrer-Sawatzki, H, Patrinos GP. Genomic rearrangements in inherited disease and cancer. Semin Cancer Biol. 2010;20:222-233.

Sadikovic B, Al-Romaih K, Squire JA, Zielenska M. Cause and consequences of genetic and epigenetic alterations in human cancer. Curr Genomics. 2008;9:394-408.

Upadhyaya M, Ruggieri M, Maynard J, et al. Gross deletions of the neurofibromatosis type 1 (NF1) gene are predominantly of maternal origin and commonly associated with a learning disability, dysmorphic features and developmental delay. Hum Genet. 1998;102:591-597.

In children with failure to thrive, malnutrition initially results in wasting (deficiency in weight gain) and then in stunting (deficiency in linear growth). In general, stunting occurs after months of malnutrition. Head circumference is spared, except with very chronic, ongoing malnutrition.

Symmetric failure to thrive (ie, proportional weight, height, and head circumference abnormalities), as is the case with this infant, suggests very longstanding malnutrition. It is true that chromosomal abnormalities, congenital infection, or teratogenic exposure can also act as the etiology for symmetric failure to thrive, but these are not the only possibilities.

This is not a child with asymmetric failure to thrive, and it is unlikely that this child has an uncommon, underlying genetic defect, because the etiology of most cases of failure to thrive is malnutrition. Culturally appropriate feeding practices do not generate failure to thrive, nor does a child with genetic short stature present with this degree of failure to thrive.

Reference:

Christian CW, Blum NJ. Failure to thrive. In: Marcdante KJ, Klinegman RM, eds. Nelson Essentials of Pediatrics. 7th ed., 2015:67-74.

Educational Objective:

Identify psychomotor deficits associated with iron deficiency anemia.

Key Point:

Iron deficiency anemia is associated with impaired psychomotor development.

Explanation:

In the United States, iron deficiency anemia is common, especially in toddlers. Iron deficiency anemia can be associated with impaired psychomotor and mental development. Other common factors associated with iron deficiency anemia are insufficient iron intake, decreased absorption due to poor dietary sources of iron, introduction of unmodified cow milk before 12 months of age, and occult blood loss secondary to protein-induced colitis due to cow milk.

For infants younger than 12 months of age who are not breastfed or are partially breastfed, the American Academy of Pediatrics recommends the use of only iron-fortified formulas (12 mg/L iron).

References:

Clark KM, Li M, Zhu B, et al. Breastfeeding, mixed, or formula feeding at 9 months of age and the prevalence of iron deficiency and iron deficiency anemia in two cohorts of infants in China. J Pediatr. 2017;181:56-61.

Hirata, M, et al. “Risk Factors of Infant Anemia in the Perinatal Period.” Pediatrics International : Official Journal of the Japan Pediatric Society., U.S. National Library of Medicine, Apr. 2017