Nu Nu Nutrition!
These 3 findings: dermatitis, dementia, and diarrhea are associated with this vitamin deficiency.
What is niacin (B3) deficiency?
Niacin deficiency is associated with pellagra. It is most commonly seen in areas where people get most of their calories from maize/corn.
Infant has delayed umbilical cord separation. This is the disorder you should consider.
What is leukocyte adhesion defect Type 1 (LAD1)?
LAD1 presents with delayed umbilical cord separation, omphalitis, and severe gingivitis. Theses patients have a baseline leukocytosis because they lack closet of differentiation 18 (CD18) molecules, which are needed for the cells to leave the circulation and enter tissues to fight off infection. As a result, the leucocytes are stuck in the circulation and cannot get out of their job.
An infant is born with a severe seizure disorder that begins at birth. A CT of the head shows a brain without cerebral convolutions and with a poorly formed Sylvian fissure. This is the diagnosis.
What is lissencephaly or agyria?
It is characterized by smooth cerebral surface with a thickened cortical mantle of the brain. It lacks cerebral folds (gyro) and grooves (sulk) and has a poorly formed Sylvian fissure. It is caused by a defect in the neuronal migration during weeks 12-24 of gestation. It is associated with several disorders, especially isolated lissencephaly sequence, Miller-Dieker syndrome, and Walker-Warburg syndrome. These children have severe psychomotor impairment, seizures, poor feeding, recurrent pneumonia, and shorted lifespans.
This p value is considered statistically significant.
What is p ≤ 0.05?
The smaller the p value, the more confident we can be that the outcome of the study is not simply a change occurrence. For a study with a p value of 0.05, the likelihood that the results are due to change is only 1 in 20 (=5% or 0.05). A p value ≤ 0.05 is considered statistically significant but smaller p values (such as 0.01 or 0.001) imply even greater statistical significance.
This is the structure of origin of bleeding in most cases of epistaxis.
What is the Kisselbach plexus?
This is an area in the anterior portion of the nasal septum where 4 arteries converge. Most epistaxes come from this structure, usually caused by nose picking.
On a global health rotation, you learn a mother has been giving her infant only goat's milk. These are the vitamins that are deficient.
What are folate and B6?
Goat's milk doesn't contain adequate folate and B6. The most crucial clinically is folate. Folate deficiency is common in children who are a fed a diet of goat's milk. Folate deficiency is a primary cause of hematologic problems including leukocyte and cellular immune dysfunction. It is the #2 nutritional cause of anemia.
Previously sensitized T cells interact with an antigen, causing an inflammatory reaction that peaks in 1-3 days. Name this type of hypersensitivity reaction.
Type 4: cell-mediated hypersensitivity
Type 4 hypersensitivity reaction (aka delayed-type hypersensitivity) occurs due to exposure to a previously known antigen. TB skin testing is an example.
This is the name of Wendy's dog in Peter Pan.
What is Nana?
These are the 2 main observational study designs.
What are cohort and case-control studies?
Observation studies attempt to correlate exposures (e.g. smoking) with outcomes (e.g. lung cancer) but do not assign patients to one group or another.
Cohort studies entail following a group of individuals prospectively over time to see which exposures cause disease (e.g. to determine if there is a causal association b/n thimerosal exposure in infants and developmental disorders).
Case-control studies involve comparing people with the disease to those w/o the disease to identify relevant risk factors (e.g. to see if there is a causal relationship b/n acetaminophen and Reye syndrome).
This is the most common cause of visual loss in children (and adults <45YO).
What is amblyopia?
This can result from childhood refractive disorders, strabismus, cataracts, corneal opacities, or an unequal refractive error b/n the eyes (anisometropia). Amblyopia is generally not correctable with glasses or contacts, but patching the better-seeing eye is recommended. The earlier treatment is started, the better the outcome.
A 1YO presents with irritability, anorexia, follicular hyperkeratosis, corkscrew-coiled hairs, gingival bleeding, and normochromic/normocytic anemia. This is the diagnosis.
What is Vit C deficiency/scurvy?
Vit C, or ascorbic acid, deficiency is a rare in the US. Most cases occur b/n 6mo-2yo. It impairs the formation of collage (results in fragile capillaries and gingival hemorrhage) and chondroitin sulfate (results in brittle and easily fracturing bones).
A 5YO presents with difficulty walking, h/o chronic sinus infections with hospitalizations for pneumonia, and elevated a1-fetoprotein. The is the likely diagnosis.
What is ataxia-telangiectasia?
AT is an AR disorder with ataxia occurring early in life; telangiectasia may not become evident until 5YO. They appear on bulbar conjunctivae and skin. Note that these children have elevated a1-fetoprotein. Immunodeficiency is variable and can affect both B and T cells. Patients are prone to sinopulmonary infections. They have an increased risk of cancers.
16YO boy presents with unilateral retroorbital pain that feels like an "ice pick", ipsilateral lacrimation, eye redness, and nasal congestion. The headache has occurred at the same time of day for the last 3 days. This is the best acute treatment.
What is oxygen?
This boy is suffering from cluster headaches. The 1st line treatment options for cluster headaches include oxygen therapy and triptans. Cluster headaches are rare in children <10YO but become increasingly common b/n 10-20yo. Boys are affected much more than girls (3:1 to 4:1). They present with daily attacks that commonly occur at the same hour each day. The pain is strictly unilateral, severe, and is supraorbital/retroorbital/temporal. Ipsilateral autonomic symptoms are common.
These are the number of examinations required to determine brain death.
What are 2 separate examinations?
In the US, children must undergo 2 separate physical examinations (including brainstem testing) before brain death can be declared. The examinations must be separated by an observation period, the length of which depends on the age of the child.
LBW newborns are/have farsighted/nearsighted/normal vision.
What is nearsighted?
The normal eye at birth, due to its size and shape, is farsighted (hyperopic). As the visual system matures, the eye elongates and becomes less farsighted. Although most full-term infants are mildly hyperopic at birth, premature and LBW infants tend to be less hyperopic or even myopic (nearsighted), often with some degrees of astigmatism. ~45% of premature infants are myopic compared to 20% of full-term infants.
This book is about a fish that doesn't want to share his scales.
What is the rainbow fish?
A 2WK old boy with eczema presents for circumcision. Circ is performed but the boy has profuse bleeding that is difficult to stop. CBC reveal thrombocytopenia. On peripheral smear, small platelets are found. This is the likely diagnosis.
What is Wiskott-Aldrick syndrome?
WAS is an X-linked disease with a classic trade: thrombocytopenia, eczema, and susceptibility to encapsulated bacterial and opportunistic infections.
Patient presents with hypoactive or absent deep-tendon reflexes, ataxia, corticospinal tract dysfunction, diabetes mellitus, hypertrophic cardiomyopathy, and impaired vibratory and proprioceptive function. Name this disorder.
What is Friedreich ataxia?
It is an AR disorder and maps to chromosome 9. It occurs in about 1/30K - 1/50K in the Caucasian population.
The genie was stuck in the lamp for these many years before Aladdin got him out.
What is 10,000 years?
A healthy 16YO male competitive swimmer presents to your office with drainage from his right ear, muffled hearing, and pain with movement of his right ear. These are the 2 most common organisms causing this teen's discomfort. (name both!)
What are S. aureus and P. aeruginosa?
Using the Holliday-Segar equation, this is the estimated daily caloric requirement (kcal per day) for a healthy 20kg child.
What is 1500kcal/day.
Equation: (100kcal/kg for the first 10kg)+(50kcal/kg for the next 10kg)+(20kcal/kg for the rest of the weight)
Name this PBS show in which kids are sent on quests by a dog.
What is the Ruf Ruffman show?
This is the most common inheritable cause of a stroke.
Activated protein C (APC) resistance (Factor 5 Leiden)
This the most common inheritable cause of venous thrombosis. It is more common than deficiencies of protein C, protein S, or antithrombin. APC resistance can also cause hemiplegic cerebral palsy in neonates.
Autonomy is the framework for ethical reasoning in adults. This is the framework used in most pediatric cases.
What is beneficence?
Beneficence is the obligation of the practitioner to act in the best interest of the patient. Many factors must be taken into account, including the patient's developmental capacity, the benefits/risks associated with the treatment, and input from parents.
This is the name of Walt Disney's first cartoon character.
What is Oswald the Lucky Rabbit?