Paediatrics Marrow Pg 61-70 (Nutrition)
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What is the daily dose of Vitamin D recommended for a 1-year-old child with rickets?

  • 6000 IU/day
  • 3000 IU/day (correct)
  • 4500 IU/day
  • 1500 IU/day

    • Familial hypophosphatemic rickets is caused by a mutation in the PHEX gene.

    True

    What is the primary monitoring method used to assess the response to rickets treatment after 4 weeks?

    X-Ray

    In familial hypophosphatemic rickets, there is an excess of __________ in the urine due to the active phosphatonin.

    <p>PO4^3-</p> Signup and view all the answers

    Match the following types of rickets with their characteristics:

    <p>Familial Hypophosphatemic Rickets = X-linked dominant inheritance Vitamin D-dependent Rickets (VDDR) = Dependent on Vitamin D availability RTA associated rickets = Involves renal tubular acidosis CKD associated rickets = Chronic kidney disease related bone abnormalities</p> Signup and view all the answers

    What is the key indicator of abdominal obesity for girls?

    <blockquote> <p>80 cm waist circumference</p> </blockquote> Signup and view all the answers

    BMI interpretation for children is based on the Indian Association of Paediatrics chart.

    <p>True</p> Signup and view all the answers

    What are the two main causes of constitutional obesity?

    <p>Excess food intake and inadequate exercise</p> Signup and view all the answers

    The _____ ratio is used as an indicator of general obesity.

    <p>waist-hip</p> Signup and view all the answers

    Match the following parameters related to obesity with their respective interpretations:

    <p>BMI &gt; 27 = Obesity in adults Waist circumference &gt; 90 cm in boys = Abdominal obesity Waist-hip ratio &gt; 1 in males = Obesity indicator 85th percentile weight for height = Obesity in children</p> Signup and view all the answers

    Which of the following is a monogenic cause of obesity?

    <p>Leptin deficiency</p> Signup and view all the answers

    Hypothyroidism can contribute to pathological obesity.

    <p>True</p> Signup and view all the answers

    What is the primary clinical feature of Prader-Willi Syndrome?

    <p>Hypogonadism</p> Signup and view all the answers

    The condition known as ________ syndrome is associated with macrogossia and organomegaly.

    <p>Beckwith-Wiedemann</p> Signup and view all the answers

    Match the following obesity syndromes with their clinical features:

    <p>Prader-Willi Syndrome = Hypogonadism, small hands and feet, almond-shaped eyes Beckwith-Wiedemann Syndrome = Obesity, macroglossia, organomegaly Lawrence-Moon Syndrome = Obesity, hypogonadism, intellectual disability Biedl-Bardet Syndrome = Obesity, retinitis pigmentosa</p> Signup and view all the answers

    What is a common dietary source that helps prevent rickets?

    <p>Fish</p> Signup and view all the answers

    Craniotabes refers to the hardening of the skull bone.

    <p>False</p> Signup and view all the answers

    What condition is associated with the appearance of a 'rachitic rosary'?

    <p>Rickets</p> Signup and view all the answers

    Rickets is primarily caused by a deficiency of _______.

    <p>vitamin D</p> Signup and view all the answers

    Match the following deformities with their descriptions related to rickets:

    <p>Craniotabes = Softening of skull bone Rachitic rosary = Widening of costochondral junction Frontal bone bossing = Protrusion of the forehead Osteoid expansion = Accumulation of unmineralized bone matrix</p> Signup and view all the answers

    What is the primary radiological tool used for diagnosing rickets?

    <p>X-Ray</p> Signup and view all the answers

    Genu varum is characterized by knock knees.

    <p>False</p> Signup and view all the answers

    What does a loss of zone of provisional calcification indicate in an X-ray of a child suspected of having rickets?

    <p>It indicates rickets.</p> Signup and view all the answers

    A child with rickets may present with delayed __________.

    <p>dentition</p> Signup and view all the answers

    Match the following clinical findings with their descriptions:

    <p>Widening of wrist = A sign associated with rickets Lateral deviation of ends = Splaying Irregularity at ends = Cupping Cartilage hypertrophy above lateral malleolus = Double malleolus sign</p> Signup and view all the answers

    What is the primary goal of the F-75 diet used during the beginning of feeds?

    <p>80 kcal/kg/d + 0.8 - 1 g protein/kg/d</p> Signup and view all the answers

    Dehydration is treated only after stabilization in pediatric rehabilitation.

    <p>False</p> Signup and view all the answers

    What should be ensured regarding edema before discharge?

    <p>There should be no edema for at least 2 weeks.</p> Signup and view all the answers

    The osmolarity of WHO ORS solution is __________ mOsm/L.

    <p>245</p> Signup and view all the answers

    Match the rehabilitation phase with its specific characteristic:

    <p>Stabilization (Day 1-2) = Cautious feeding begins Rehabilitation (Days 3-7) = Energy dense feeds for catch-up growth Rehabilitation (Weeks 2-6) = Micronutrients including iron</p> Signup and view all the answers

    Which of the following is a potential complication of obesity?

    <p>Insulin resistance</p> Signup and view all the answers

    Orlistat works by increasing fat breakdown and absorption in the body.

    <p>False</p> Signup and view all the answers

    Name one anti-epileptic drug that is associated with obesity.

    <p>Valproate</p> Signup and view all the answers

    Acanthosis nigricans is characterized by __________ patches on the neck and axilla.

    <p>hyperpigmented velvety</p> Signup and view all the answers

    Match the management approaches for obesity with their descriptions:

    <p>Lifestyle changes = Includes dietary adjustments and increased physical activity Medical management = Utilizes medications like Orlistat and Metformin Surgical management = Considered as a last resort for severe cases Nutritional changes = Focuses on reducing sugar and unhealthy fats intake</p> Signup and view all the answers

    What is the definition of hypoglycemia?

    <p>Blood glucose &lt; 54 mg/dL</p> Signup and view all the answers

    Hypothermia is defined as an axillary temperature of less than 35.5°C.

    <p>True</p> Signup and view all the answers

    What is the initial treatment for symptomatic hypoglycemia?

    <p>IV 10% Dextrose: 5 mL/kg STAT</p> Signup and view all the answers

    The treatment for asymptomatic hypoglycemia includes administering __________.

    <p>oral glucose</p> Signup and view all the answers

    Match the following complications with their definitions:

    <p>Hypoglycemia = Blood glucose level &lt; 54 mg/dL Hypothermia = Axillary temperature &lt; 35.5°C</p> Signup and view all the answers

    What is the required daily calorie intake for a child with Severe Acute Malnutrition (SAM)?

    <p>175 kcal/kg/day</p> Signup and view all the answers

    Complicated SAM presents with poor appetite and generalized edema.

    <p>True</p> Signup and view all the answers

    What are the two primary types of Severe Acute Malnutrition (SAM)?

    <p>Uncomplicated SAM and Complicated SAM</p> Signup and view all the answers

    _____ is an antibiotic commonly used in the management of SAM.

    <p>Amoxicillin</p> Signup and view all the answers

    Match the following RUTF ingredients with their purposes:

    <p>Milk Solids = Provides protein and energy Sugars = Enhances taste and energy content Vegetable oil = Supplies essential fatty acids Peanut butter &amp; paste = Contributes to caloric density</p> Signup and view all the answers

    What is the primary condition indicated by low weight for height?

    <p>Wasting</p> Signup and view all the answers

    Stunting is classified under moderate malnutrition if it is between -2 to -3 S.D.

    <p>True</p> Signup and view all the answers

    What is the predominant deficiency in Kwashiorkor?

    <p>Protein</p> Signup and view all the answers

    The condition known as ________ refers to chronic undernutrition characterized by low height for age.

    <p>stunting</p> Signup and view all the answers

    Match the following conditions of malnutrition with their implications:

    <p>Wasting = Acute severe weight loss Stunting = Chronic undernutrition Underweight = Refers to both wasting and stunting</p> Signup and view all the answers

    Study Notes

    Treatment for Rickets

    • Vitamin D dosage for children under one year old is 3000 IU/day for 12 weeks.
    • Calcium supplement: 50-75 mg/kg/day.
    • X-ray shows changes within 4 weeks, while biochemical investigations show changes after 12 weeks.
    • Refractory Rickets occurs when there is no response to Vitamin D supplements.
    • Common disorders causing Refractory Rickets are Familial Hypophosphatemic Rickets, Vitamin D-dependent Rickets (VDDR), RTA associated rickets, and CKD associated rickets.
    • Most common inherited form of rickets is Familial Hypophosphatemic Rickets.

    Familial Hypophosphatemic Rickets

    • X-linked dominant inheritance.
    • Mutation in PHEX gene causes inactivation of phosphatonin, resulting in increased phosphate excretion in urine, leading to hypophosphatemia.

    Clinical Features of Familial Hypophosphatemic Rickets

    • Lower limb deformities are often seen.
    • No features of hypocalcemia like Tetany are present.
    • Dental abnormalities include pulp exposure and an increased risk of dental abscess.

    Obesity in Children

    • Obesity is defined as having an excess of adipose tissue deposition.
    • In urban India, over 20% of children are obese.
    • Energy intake is significantly greater than energy expenditure, leading to a build-up of fat, primarily in the abdominal region.
    • Central obesity is associated with a greater risk of complications.
    • The human body has an unlimited capacity for fat storage, but not for carbohydrates or proteins.

    Definitions

    • Body Mass Index (BMI) is used for children over 5 years old and is calculated as Weight (in kg) / Height (in m²) x100.
    • Overweight is indicated by a BMI greater than 23 adult equivalent.
    • Obesity is indicated by a BMI greater than 27 adult equivalent.
    • Weight for height is another indicator of obesity. Values above the 85th percentile indicate obesity.
    • Waist circumference is an indicator of abdominal obesity. Values above the 75th percentile, above 80 cm in girls, or above 90 cm in boys are concerning.
    • Waist-hip ratio is age-independent and calculated by dividing waist circumference by hip circumference. Values above 1 in males, above 0.85 in females, or between 0.5 and 1 are associated with obesity.
    • Waist-height ratio is also age-independent and is calculated by dividing waist circumference by height.
    • Interpretation of BMI should reference the Indian Association of Paediatrics BMI chart.

    Causes of Obesity

    • Constitutional obesity is the most common cause and is often related to excess food intake and inadequate exercise.
    • Pathological obesity is usually associated with short stature and is characterized by tall stature due to increased insulin and Insulin-like Growth Factor (IGF) levels.

    Causes of Pathological Obesity

    • Monogenic Causes:
      • Hereditary single gene defects like Mineralocorticoid-4-receptor defect (MCAR) and Leptin deficiency or resistance result in uncontrolled appetite.
    • Endocrine Causes:
      • Cushing's syndrome: Characterized by truncal obesity, buffalo hump, striae, and moon-face.
      • Hypothyroidism, Pseudohypoparathyroidism, and Growth hormone deficiency can also contribute to obesity.
    • Hypothalamic Obesity:
      • Lesions in the Ventro-medial nucleus (VMN), which is responsible for satiety, can cause uncontrolled appetite. These lesions can be caused by head injury, radiation, tumors, or infections.
    • Syndromic Obesity:
      • Prader-Willi Syndrome: A genetic imprinting disorder characterized by hypogonadism, small hands and feet, and almond-shaped eyes.
      • Beckwith-Wiedemann syndrome: Characterized by obesity, macroglossia, organomegaly, and hemi hypertrophy.
      • Lawrence-moon (Lm), Biedl-Bardet (BB) syndrome: Characterized by obesity, hypogonadism, intellectual disability, and retinitis pigmentosa.
    • ROHHAD:
      • Rapid Onset Obesity with Hypothalamic dysfunction, Hypoventilation, Autonomic Dysregulation.

    Rickets

    • Occurs due to Vitamin D deficiency.
    • Dietary deficiencies can be caused by insufficient intake of foods like fish, fish liver oil, and egg yolk.
    • Certain drugs like anti-epileptics (Phenytoin, phenobarbitone, carbamazepine), anti-tubercular drugs (Isoniazid and rifampicin), and decreased sunlight exposure can also contribute to vitamin D deficiency.
    • Malabsorption syndromes and chronic liver disease can hinder vitamin D absorption.

    Pathophysiology of Rickets

    • Vitamin D deficiency leads to decreased absorption of calcium and phosphate.
    • Less mineralized bone results in weak bones and cartilage hypertrophy at the growth plate.
    • Osteoid expansion and swelling are also observed.

    Deformities in Rickets

    • Head:
      • Craniotabes: Softening of skull bone with a sponge ball-like consistency.
      • Enlarged fontanelles.
      • Frontal bone bossing.
      • Craniotabes can also be seen in congenital syphilis and hydrocephalus.
    • Chest:
      • Costochondral junction widening leads to a rachitic rosary (string of beads appearance) which is painless and round. This can also be seen in osteogenesis imperfecta.
    • Limbs:
      • Widening of the wrist.
      • Genu varum (bow leg).
      • Genu valgus (knock knees).
      • Double malleolus sign: Cartilage hypertrophy (swelling) above the lateral malleolus.

    Other Findings in Rickets

    • Delayed dentition.
    • Seizures or tetany can occur in infants due to acute hypocalcemia.

    Investigations for Rickets

    • Blood investigations:
      • Decreased Vitamin D: specifically measured by the level of 25 hydroxyvitamin D.
      • Decreased serum calcium due to poor absorption, leading to increased parathyroid hormone (PTH), ultimately resulting in normal serum calcium levels.
      • Decreased serum phosphate.
      • Increased alkaline phosphatase (ALP) due to increased bone resorption by PTH.

    Classification of Vitamin D Deficiency

    Category Serum Vitamin D level (ng/ml)
    Sufficiency > 20
    Insufficiency 12-20
    Deficiency < 12

    Radiological Investigation for Rickets

    • Primary diagnostic tool is an X-ray.
    • Loss of zone of provisional calcification (ZPC): White line at the end of the long bone.
    • Other observations:
      • Splaying: Lateral deviation of the ends of the bones.
      • Cupping: Irregularity at the ends of the bones.

    Obesity: Causes

    • Drugs:
      • Anti-epileptic drugs like Valproate and Lamotrigine.
      • Steroids can cause Cushingoid appearance.

    Obesity: Complications

    • Metabolic Complications:
      • Insulin resistance.
      • Metabolic syndrome.
      • Polycystic Ovary Syndrome (PCOS) in women.
    • Obesity (Increased Adipose):
      • Acanthosis nigricans (hyperpigmented velvety patches on the neck and axilla).
    • Non-Alcoholic Fatty Liver Disease (NAFLD):
      • Steatohepatitis (inflammatory changes in the liver) can lead to chronic fibrosis.

    Obesity: Management

    • Lifestyle Changes:
      • Avoid processed sugars, sweetened beverages, and packaged foods.
      • Avoid trans fatty acids and saturated fatty acids, while increasing intake of unsaturated fatty acids.
      • Increase intake of fruits, vegetables, nuts, and grains.
      • Aim for 60 minutes of moderate-intensity exercise daily (more than 30 minutes during school hours).
    • Medical Management:
      • Treatment of any underlying endocrine disorders.
      • Orlistat: Inhibits gastric and pancreatic lipase, reducing fat breakdown and absorption.
      • GLP-1 analogue (Liraglutide): Slows gastric emptying, reducing fat absorption.
      • Metformin: Indicated for known diabetes and women with PCOS.
    • Surgical Management (Last Resort):
      • Laparoscopically adjustable gastric banding (LAGB): This procedure restricts the size of the stomach, leading to reduced food intake.

    Severe Acute Malnutrition (SAM)

    • Criteria for SAM diagnosis:
      • Weight for height: < -3.5 SD
      • Bipedal edema (diagnosis of exclusion)
      • Mean Arm Circumference (MAC): < 11.5 cm
      • Presence of any of the above conditions confirms SAM

    Types of SAM

    • Uncomplicated SAM:
      • Good appetite.
      • Absence of generalized edema.
      • Absence of medical complications.
      • Supervised home management is adequate.
    • Complicated SAM:
      • Poor appetite.
      • Presence of generalized edema.
      • Medical Complications present.
      • Requires hospital management.

    Home Management of SAM

    • Nutrition Goal: 175 kcal/kg/day + 4-6 g/kg/day protein.
    • Diets:
      • Home-made foods are acceptable.
      • Ready-to-use therapeutic food (RUTF) is also recommended.

    RUTF Ingredients and Advantages

    • Ingredients:
      • Milk solids
      • Sugars
      • Vegetable oil
      • Peanut butter and paste
    • Advantages:
      • Good palatability
      • Semi-solid consistency
      • Long shelf-life

    Additional Interventions for SAM (3 As)

    • Antibiotic: Oral amoxicillin (dosage based on age: 400mg for children 2 years old).
    • Albendazole (Deworming): 12 months - 2 Lakh IU.
    • Vitamin A: 12 months - 2 Lakh IU.

    RUTF Nutrient Value

    • 100g of RUTF provides 543 kcal + 15g protein.

    Hospital Management of SAM

    • Stabilization Phase: Management of complications using the mnemonic "SHIELDED"

    Phase 1: Stabilization

    • Complications*
    • 1. Hypoglycemia (Sugars)
      • Definition: Blood glucose: < 54 mg/dL
      • Treatment: * Symptomatic hypoglycemia (e.g. seizures): IV 10% Dextrose 5 mL/kg STAT
    • Asymptomatic hypoglycemia: Oral glucose 50mL
    • Glucose infusion
    • 2. Hypothermia
      • Definition: Axillary temp < 35.5°C or Rectal temperature < 36°C
      • Treatment: Warm blankets, warm fluids, warm environment
    • 3. Dehydration
      • Definition: Electrolyte imbalance
      • Treatment: IV fluids
    • 4. Electrolytes
      • Definition: Electrolyte imbalance
      • Treatment: IV fluids
    • 5. Infection
      • Definition: Symptomatic infection
      • Treatment: Antibiotics (e.g. ceftriaxone 50-75mg/kg/ day)
    • 6. Micronutrients
      • Treatment: 1-2 mg/kg/day
    • 7. Cautious feeding
      • Definition: Feed small amounts every few hours
      • Treatment: Gradually increase the amount of food as tolerated
    • 8. Catch-up growth
      • Treatment: High calorie and high-protein diet
    • 9. Sensory stimulation
      • Treatment: Environment that is stimulating and encourages interaction
    • 10. Prepare for follow-up
      • Treatment: Regular appointments with their doctor

    Resomal v/s WHO ORS solution

    Resomal WHO ORS
    Osmolarity (mOsm/L) 300 245
    Sodium (Na) 75 45
    Glucose 75 125
    Potassium (K) 20 40
    Chloride (Cl) 65 70

    Phase II: Rehabilitation (Mnemonic: BEST)

    • B: Beginning of Feeds
      • Up to 1 week
      • Cautious feeding
      • F-75 diet: 75 kcal + 0.9 g protein/100 g
      • Goal: 80 kcal/kg/d + 0.8-1 g protein/kg/d
    • E: Rehabilitation
      • After 1 week
      • Energy-dense feeds for catch-up growth
      • F-100: 100 kcal + 2.9 g protein/100 g
      • Goal: 175 kcal/kg/d + 4-6 g protein/kg/d
    • S: Stabilization (Days 1-2):
      • Hypoglycemia
      • Hypothermia
      • Dehydration
      • Electrolytes
      • Infection
    • T: Rehabilitation (Days 3-7):
      • Hypoglycemia
      • Hypothermia
      • Dehydration
      • Electrolytes
      • Infection
      • Micronutrients
      • Cautious feeding
      • Catch-up growth
      • Sensory stimulation
    • T: Rehabilitation (Weeks 2-6):
      • Hypoglycemia
      • Hypothermia
      • Dehydration
      • Electrolytes
      • Infection
      • Micronutrients (with iron)
      • Cautious feeding
      • Catch-up growth
      • Sensory stimulation
      • Prepare for follow-up

    Treatment Completion Summary

    • No edema for at least 2 weeks.
    • Weight for height: - 2 SD/MAC reaches 12.5 cm
    • Deworming prior to discharge: Albendazole 400mg single dose.

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    Description

    This quiz covers essential information regarding the treatment and clinical features of Rickets, focusing on Familial Hypophosphatemic Rickets. Key topics include Vitamin D dosage, calcium supplementation, and the genetic aspects of the condition. Test your knowledge on these important aspects of pediatric health.

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