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Pathobiology of Obesity

Chapter 413 | Part 12: Endocrinology and Metabolism · Part 12 – Endocrinology & Metabolism · Chapter 413


Key Clinical Points

  1. Obesity is defined as excess adipose tissue mass that adversely affects health; BMI (weight/height²) is the standard clinical proxy.
  2. WHO criteria: Overweight = 25–30 kg/m²; Obesity ≥ 30 kg/m².
  3. Waist-to-hip ratio >0.9 in women or >1.0 in men indicates central adiposity associated with increased risk for type 2 diabetes and dyslipidemia.
  4. Leptin resistance explains why elevated leptin levels in obesity do not prevent weight gain, though leptin still functions to defend against fat loss.
  5. Genetic factors are significant; up to 20% of children with severe obesity have rare chromosomal abnormalities or highly penetrant genetic mutations.
  6. Monogenic obesity (e.g., MC4R mutations) is the most common cause of severe early-onset obesity (~5–6% of cases).
  7. Setmelanotide (an MC4R agonist) is used for specific defects in POMC, PCSK1, and the leptin receptor.
  8. Insulin resistance is a hallmark of obesity, driven by adipose tissue inflammation and ectopic fat storage in non-adipose tissues (liver/muscle).
  9. Genetic diagnosis informs management (difficulty of weight loss via diet/exercise) and surgical risk assessment (bariatric surgery feasibility).
  10. Obesity complications include mechanical issues (osteoarthritis, sleep apnea) and metabolic ones (dyslipidemia, T2D, NAFLD).

DEFINITION & OVERVIEW

Definition: Obesity is defined as a state of excess adipose tissue mass that adversely affects health.Measurement Proxy: Body Mass Index (BMI) ◦ Calculation: weight/height² (kg/m²) • WHO Classifications: ◦ Overweight: 25–30 kg/m² ◦ Obesity: ≥ 30 kg/m² ◦ Note: BMI may overestimate fat in very muscular individuals. • Fat Distribution Metric: Waist-to-hip ratio ◦ Women >0.9 → associated with type 2 diabetes and dyslipidemia ◦ Men >1.0 → associated with type 2 diabetes and dyslipidemia


EPIDEMIOLOGY

Prevalence Trends: ◦ Significant increase over time; e.g., 14.5% (1976–1980) to 42.4% of U.S. adults (2017–2018). ◦ Global trend: Obesity nearly tripled worldwide since 1975. • Demographic Variations: ◦ Non-Hispanic Black: 49.6% ◦ Hispanic: 44.8% ◦ Non-Hispanic White: 42.2% ◦ Non-Hispanic Asian: 17.4% ◦ Prevalence is generally higher in women than men. • Pediatric Impact: ◦ Overweight ≥ 91st percentile; Obesity ≥ 99th percentile (adjusted for age and sex). ◦ 2019: 38 million children <5 years old were overweight or obese. ◦ 2016: 340 million children/adolescents aged 5–19 were overweight or obese.


ETIOLOGY & PATHOPHYSIOLOGY

Energy Balance: ◦ Accumulation of triglycerides in adipose tissue occurs when intake exceeds expenditure. ◦ Small excess (as little as 7 kcal/d) can lead to obesity over years or decades. • Genetic Predisposition: ◦ Significant role confirmed by twin studies; identical twins show similar adiposity regardless of environment. ◦ ~20% of children with severe obesity have rare chromosomal abnormalities or highly penetrant genetic mutations. • Leptin Dynamics: ◦ Produced by fat cells; levels rise as fat stores expand and fall during caloric restriction. ◦ Leptin Resistance: High leptin in obesity does not prevent weight gain but still defends against fat loss. ◦ Congenital Deficiency: Leads to severe hyperphagia and early-onset obesity. • Insulin Resistance (IR) Mechanisms (Figure 413-5):Pathway 1: Adipose Tissue Inflammation Expansion of adipose tissue → Recruitment of macrophages/immune cells → Release of inflammatory cytokines → Disturbance of insulin action in muscle and liver. ◦ Pathway 2: Lipotoxicity Expansion of adipose tissue → Limited fat cell capacity for continuing storage → Storage of lipid in non-adipose tissue (liver, muscle) → Defective glucose handling in liver and muscle. ◦ Outcome: Insulin resistance and compensatory hyperinsulinemia.


CLINICAL FEATURES

Syndromic Disorders (Table 413-1):Prader-Willi: Autosomal dominant; features include hypotonia, failure to thrive in infancy, developmental delay, short stature, hypogonadotropic hypogonadism, sleep disturbance, and obsessive behavior. ◦ Bardet-Biedl: Autosomal recessive; features include syndactyly/brachydactyly/polydactyly, developmental delay, retinal dystrophy or pigmentary retinopathy, hypogonadism, and renal abnormalities. ◦ Carpenter’s: Autosomal recessive; features include acrocephaly, brachydactyly, developmental delay, congenital heart defects, growth retardation, and hypogonadism. ◦ Tubby: Autosomal recessive; features include progressive cone-rod dystrophy and hearing loss. • Monogenic & Genetic Mutations (Table 413-2):Leptin: Autosomal recessive; severe hyperphagia, frequent infections, hypogonadotropic hypogonadism, mild hypothyroidism. ◦ Proopiomelanocortin (POMC): Autosomal recessive; hyperphagia, cholestatic jaundice or adrenal crisis due to ACTH deficiency, pale skin and red hair. ◦ Carboxypeptidase E: Autosomal recessive / Autosomal dominant; severe insulin resistance. ◦ Single-minded 1 (SIM1): Autosomal dominant; hyperphagia, accelerated linear growth, speech and language delay, autistic traits. ◦ TrkB: Autosomal dominant; hyperphagia, speech and language delay, variable developmental delay, hyperactivity, behavioral problems including aggression. ◦ MC4R Mutations: Common in severe early-onset obesity (~5–6% of cases); associated with hyperphagia.


DIFFERENTIAL DIAGNOSIS

Primary vs. Secondary Obesity:Primary: Genetic or endocrine conditions (e.g., Leptin deficiency, MC4R mutations). ◦ Secondary: Resulting from other systemic disorders. ◦ Note: Clinical assessment should screen for treatable endocrine and neurologic conditions to inform management and surgical risk.


INVESTIGATIONS & DIAGNOSIS

  1. Initial Assessment: ◦ Calculate BMI (weight/height²). ◦ Measure waist-to-hip ratio to assess risk for T2D and dyslipidemia.
  2. Genetic Screening: ◦ Recommended for severe cases to identify treatable endocrine/neurologic conditions. ◦ Identifies specific mutations (e.g., POMC, PCSK1, leptin receptor) to guide pharmacotherapy.

MANAGEMENT & TREATMENT

  1. Pharmacologic Therapy:Setmelanotide: MC4R agonist; indicated for children with genetic defects in POMC, PCSK1, and the leptin receptor. ◦ Targeted Therapy: Selection of treatment depends on specific genetic diagnosis (e.g., identifying mutations in the melanocortin pathway).
  2. Surgical Intervention:Bariatric Surgery: Decision-making guided by genetic profile; feasible in some patients but high risk in others.

PROGNOSIS & COMPLICATIONS

Mechanical Complications: ◦ Osteoarthritis of knees. ◦ Reflux esophagitis. ◦ Obstructive sleep apnea. • Metabolic/Endocrine Complications: ◦ Dyslipidemia. ◦ Type 2 diabetes (T2D). ◦ Non-alcoholic fatty liver disease (NAFLD). ◦ Polycystic ovarian syndrome (PCOS).


KEY PEARLS & CLINICAL TRAPS

Leptin Paradox: High leptin in obesity does not prevent weight gain due to resistance, but it still acts to defend against fat loss. ◦ Genetic Screening Importance: Essential for severe cases to identify treatable conditions and determine if the patient can manage weight via diet/exercise or requires specific drugs like setmelanotide. ◦ Early Intervention: Potential for early-life interventions (in utero or postnatal) to address 'epigenetic' programming of obesity.


Reference Tables

TABLE 413-1 Classical Genetic Obesity Syndromes SYNDROME Prader-Willi

Harrison's 22e, p.3184

SYNDROME INHERITANCE ADDITIONAL CLINICAL
FEATURES
Prader-Willi Autosomal
dominant
Hypotonia, failure to thrive in
infancy, developmental delay,
short stature, hypogonadotropic
hypogonadism, sleep
disturbance, obsessive
behavior
Autosomal
dominant
Bardet-Biedl Autosomal
recessive
Syndactyly/brachydactyly/
polydactyly, developmental
delay, retinal dystrophy or
pigmentary retinopathy,
hypogonadism, renal
abnormalities
Autosomal
recessive
Carpenter’s Autosomal
recessive
Acrocephaly, brachydactyly,
developmental delay,
congenital heart defects;
growth retardation,
hypogonadism
Autosomal
recessive
Tubby Autosomal
recessive
Progressive cone-rod
dystrophy, hearing loss

TABLE 413-2 Obesity Syndromes due to Mutations in Genes Controlling Energy Homeostasis Pathways

Harrison's 22e, p.3185

GENE AFFECTED INHERITANCE ADDITIONAL CLINICAL FEATURES
Leptin Autosomal
recessive
Severe hyperphagia, frequent
infections, hypogonadotropic
hypogonadism, mild hypothyroidism
Autosomal
recessive
Proopiomelanocortin Autosomal
recessive
Hyperphagia, cholestatic jaundice
or adrenal crisis due to ACTH
deficiency, pale skin and red hair
Autosomal
recessive
Carboxypeptidase E Autosomal
recessive
Severe insulin resistance
Autosomal
dominant
Single-minded 1 Autosomal
dominant
Hyperphagia, accelerated linear
growth, speech and language delay,
autistic traits
Autosomal
dominant
TrkB Autosomal
dominant
Hyperphagia, speech and language
delay, variable developmental delay,
hyperactivity, behavioral problems
including aggression
Autosomal
dominant