Disorders/Conditions

Question 1

Night Eating Syndrome (NES)

Answer 1

Defined by morning anorexia, evening hyperphagia, and insomnia. Often with evening tension/stress. Nocturnal awakenings to eat are common. 25-50% of daily calories in the evening; Carb-rich; Highly responsive to sertraline

Question 2

Binge Eating Disorder (neuroimaging changes)

Answer 2

Neuroimaging studies showed hyperactivity in the medial orbitofrontal cortex and hypoactivity in the prefrontal network

Question 3

Beckwith-Wiedermann Syndrome

Answer 3

Fetal overgrowth syndrome, chromosome 11p15.5, macroglossia, HSM, hemihyperplasia, hypoglycemia in 30-50% of babies, tumor development (Wilms tumor)

Question 4

POMC Deficiency

Answer 4

Autosomal recessive; hyperphagia and early onset obesity, adrenal crisis due to ACTH deficiency, hypo-pigmentation, pale skin and red hair in Caucasians

Question 5

Leptin Deficiency

Answer 5

Autosomal recessive, mutation of leptin gene, early-onset obesity, intense hyperphagia and impaired satiety, absence of growth spurt, hypogonadotrophic hypogonadism, T-cell immune dysfunction; frequent bacterial infections; clinical response to recombinant human leptin admin

Question 6

MC4R Deficiency

Answer 6

Most common monogenic or singe gene form of obesity (melanocortin-4 receptor mutation); increased linear growth and final height, increased fat mass, severe hyperinsulinemia, inherited in autosomal dominant manner

Question 7

Alstrom Syndrome

Answer 7

Very rare - ALMS1 mutation, childhood obesity, blindness, and hearing loss, endocrinopathy (T2DM, high TAGs)

Question 8

Cohen Syndrome

Answer 8

Chromosome 8q22 mutation, central obesity, MR, head and face defects, seizures, deafness

Question 9

Albright Hereditary Osteodystrophy

Answer 9

Short stature, skeletal defects, impaired olfaction, developmental delay, resistance to several hormones (PTH), mutations in GNAS gene

Question 10

Bardet-Biedl Syndrome

Answer 10

Autosomal recessive cillopathic genetic disorder, obesity, visual problems (retinitis pigmentosa, loss of vision), polydactyly, MR, hypogonadism, kidney failure

Question 11

Prader-Willi Syndrome

Answer 11

Paternal chrom 15q deletion; hypotonia at birth; voracious and insatiable appetite which starts around 2; almond shaped eyes, thin upper lips, thin faces, small hands/feet, hypogonadism, delayed motor development, intellectual and speech delay,

Question 12

Loss of Control Eating Disorder (LOC-ED)

Answer 12

Similar to binge eating disorder (BED) and is prevalent in children and affects only children <12 years of age. Milder form of BED and is associated with psychological and social problems that may transform into partial or full BED later in life.

Question 13

Predictor of Obesity

Answer 13

Strongest predictor for children > 6 years of age is parental obesity; child’s own weigh becomes strongest predictor of adult obesity after age 6

Question 14

Maturity-onset diabetes of the young (MODY)

Answer 14

Suggested by absent pancreatic auto antibodies, family history of similar presentation before age 30, Does not present with ketoacidosis and the HbA1c is in the lower abnormal range (<8%)

Question 15

Knee arthritis and Obesity

Answer 15

Obesity leads to altered joint loading through the knee and varus deformity. This causes MEDIAL compartment arthritis.