Introduction to Growth & Development Part 1

Introduction to Growth & Development Part 1

1. Beckwith-widemann Syndrome: Features

HOMO

• Hypoglycemia
• Omphalocel
• Macroglossia/Macrosomia
• Organomegaly (Splenomegaly,Cardiomegaly,Hepatomegaly, e.t.c)

NB. It's an overgrowth syndrome characterized by exomphalos, macroglossia, and gigantism, often with neonatal hypoglycemia; there is an association with hemihypertrophy Wilms tumors and adrenocortical cancer. Autosomal dominant inheritance, with most cases sporadic; influenced by genomic imprinting and uniparental disomy; caused by change in the P57 (KIP2) gene locus on chromosome 11p.

2. Dentition: eruption times of permanent dentition

"Mama Is In Pain, Papa Can Make Medicine":

• 1st Molar: 6 years
• 1st Incisor: 7 years
• 2nd Incisor: 8 years
• 1st Premolar: 9 years
• 2nd Premolar: 10 years
• Canine: 11 years
• 2nd Molar: 12 years
• 3rd Molar: 18-25 years

3. Head circumference with age

Remember 3, 9, and multiples of 5:

Newborn 35 cm

• 3 mos 40 cm
• 9 mos 45 cm
• 3 yrs 50 cm
• 9 yrs 55 cm

4. Pediatric milestones in development

1 year:

• single words

2 years:

• 2 word sentences
• understands 2 step commands

3 years:

• 3 word combos
• repeats 3 digits
• ides tricycle

4 years:

• draws square
• counts 4 objects

5. Russell Silver syndrome: features

ABCDEF:

• Asymmetric limb (hemihypertrophy)
• Bossing (frontal)
• Clinodactyly/ Cafe au lait spots
• Dwarf (short stature)
• Excretion (GU malformation)
• Face (triangular face, micrognathia)

6. Short stature: differential

ABCDEFG:

• Alone (neglected infant)
• Bone dysplasias (rickets, scoliosis, mucopolysaccharidoses)
• Chromosomal (Turner's, Down's)
• Delayed growth
• Endocrine (low growth hormone, Cushing's, hypothyroid)
• Familial
• GI malabsorption (celiac, Crohn's

7. Weights of children with age

Newborn 3 kg

• 6 mos 6 kg (2x birth wt at 6 mos)
• 1 yr 10 kg (3x birth wt at 1 yr)
• 3 yrs 15 kg (odd yrs, add 5 kg until 11 yrs)
• 5 yrs 20 kg
• 7 yrs 25 kg
• 9 yrs 30 kg
• 11 yrs 35 kg (add 10 kg thereafter)
• 13 yrs 45 kg
• 15 yrs 55 kg
• 17 yrs 65 kg

2. GENETICS

1. Chromosome 15: diseases

Chromosome 15 has its own MAP:

• Marfan syndrome
• Angelman syndrome
• Prader-Willi syndrome

2. Cri-du-chat syndrome: chromosomal deletion causing it is 5p(-)

What's another name for a cat that's five letters long and starts with a P? (Answer: pussy).

Why is the cat crying? Missing its P.

3. DiGeorge Syndrome: Symptoms

CATCH-22

• Cardiac abnormalities (esp. Fallot tetralogy)
• Abnormal fascies
• Thymic aplasia
• Cleft Palate 
• Hypocalcemia
• 22 deletion of one copy of chromosome 22q11.2

4. Down syndrome features: complete

"My CHILD HAS PROBLEM  ! !! !!

• Congenital heart disease/ Cataracts
• Hypotonia/ Hypothyroidism
• Incure 5th finger/ Increased gap between 1st and 2nd toe
• Leukemia risk x2/ Lung problem
• Duodenal atresia/ Delayed development
• Hirshsprung's disease/ Hearing loss
• Alzheimer's disease/ Alantoaxial instability
• Squint/ Short neck
• Protruding tongue/ Palm crease
• Round face/ Rolling eye (nystagmus)
• Occiput flat/ Oblique eye fissure
• Brushfield spot/ Brachycephaly
• Low nasal bridge/ Language problem
• Epicanthic fold/ Ear folded
• Mental retardation/ Myoclonus

5. Down syndrome: pathology

DOWN:

• Decreased alpha-fetoprotein and unconjugated estriol (maternal)
• One extra chromosome twenty-one
• Women of advanced age
• Nondisjunction during maternal meiosis

6. Edwards' syndrome: characteristics

EDWARDS:

• Eighteen (trisomy)
• Digit overlapping flexion
• Wide head
• Absent intellect (mentally retarded)
• Rocker-bottom feet
• Diseased heart
• Small lower jaw

7. Russell Silver syndrome: features

ABCDEF:

• Asymmetric limb (hemihypertrophy)
• Bossing (frontal)
• Clinodactyly/ Cafe au lait spots
• Dwarf (short stature)
• Excretion (GU malformation)
• Face (triangular face, micrognathia)

8. Turner syndrome: components

CLOWNS:

• Cardiac abnormalities (specifically Coartication)
• Lymphoedema
• Ovaries underdeveloped (causing sterility, amenorrhea)
• Webbed neck
• Nipples widely spaced
• Short

9. WAGR syndrome: components

WAGR:

• Wilm's tumor
• Aniridia
• Gential abnormalities
• Mental retardation

10. Williams syndrome: features

WILLIAMS:

• Weight (low at birth, slow to gain)
• Iris (stellate iris)
• Long philtrum
• Large mouth
• Increased Ca++
• Aortic stenosis (and other stenoses)
• Mental retardation
• Swelling around eyes (periorbital puffiness)

3. PEDIATRIC NUTRITION

1. Breast feeding: benefits

ABCDEFGH:

* Infant:

• Allergic condition reduced
• Best food for infant
• Close relationship with mother
• Development of IQ, jaws, mouth

 *Mother:

• Econmical
• Fitness: quick return to pre-pregnancy body shape
• Guards against cancer: breast, ovary, uterus
• Hemorrhage (postpartum) reduced

2. Drugs Contraindicated during Breast Feeding

BREAST

• Bromocriptine/Benzodiazepines
• Radioactive isotopes/Rizatriptan
• Ergotamine/Ethosuximide
• Stimulant laxatives/sex hormones
• Tetracycline

3. Kwashiorkor: distinguishing from Marasmus

FLAME:

• Fatty Liver
• Anemia
• Malabsorption
• Edema

4. Milk protein: women vs. cows

• Woman: Whey (mostly)
• Cow: Casein (mostly)

5. Vitamin toxicities: neonatal

• Excess vitamin A: Anomalies (teratogenic)
• Excess vitamin E: Enterocolitis (necrotizing enterocolitis)
• Excess vitamin K: Kernicterus (hemolysis)

4. NERVOUS SYSTEM

1. Cerebral palsy: general features

PALSY:

• Paresis
• Ataxia
• Lagging motor development
• Spasticity
• Young

2. Cerebral palsy (CP): most likely cause

• CP: Cerebral Palsy
• Child Premature

3. Cerebral Palsy: Types

SADAM

Spastic (Increase tone)

Athetoid/Diskinetic (Involuntary & uncontrolled movements)

Ataxic (cerebellar  damage with fine motor skills & balance difficulty)

Mixed

4. Clinical Presentation of brain tumors: Sign & symptoms

BAHENNS

• Blurring of vision
• Ataxia
• Headache
• Endocrine dysfunctions
• Nystagmus
• Nausea & vomitting
• Squint

5. Dandy-Walker syndrome: components

"Dandy-Walker Syndrome":

• Dilated 4th ventricle
• Water on the brain
• Small vermis

6. Fetal alcohol syndrome (FAS): features

FAS:

• Facial hypoplasia/ Forebrain malformation
• Attention defecit disorder/ Altered joints
• Short stature/ Septal defects/ Small I.Q

7. Sturge-Weber syndrome: hallmark features

Sturge-Weber:

• Seizures
• PortWine stain

8. WAGR syndrome: components

WAGR:

• Wilm's tumor
• Aniridia
• Gential abnormalities
• Mental retardation

9. Williams syndrome: features

WILLIAMS:

• Weight (low at birth, slow to gain)
• Iris (stellate iris)
• Long philtrum
• Large mouth
• Increased Ca++
• Aortic stenosis (and other stenoses)
• Mental retardation
• Swelling around eyes (periorbital puffiness)

5. CARDIOVASCULAR SYSTEM

1. Cyanotic congenital heart diseases: 5 types

5 T's:

• Truncus arteriosus
• Transposition of the great arteries
• Tricuspid atresia
• Tetrology of Fallot
• Total anomalous pulmonary venous return

2. Cyanotic heart diseases: 5 types

 *Use your five fingers:

• 1 finger up: Truncus Arteriosus (1 vessel)
• 2 fingers up: Dextroposition of the Great Arteries (2 vessels transposed)
• 3 fingers up: Tricuspid Atresia (3=Tri)
• 4 fingers up: Tetralogy of Fallot (4=Tetra)
• 5 fingers up: Total Anomalous Pulmonary Venous Return (5=5 words)

3. Tetrology of Fallot

"Don't DROP the baby":

• Defect (VSD)
• Right ventricular hypertrophy
• Overriding aorta
• Pulmonary stenosis

4. Vacterl syndrome: components

VACTERL:

• Vertebral anomalies
• Anorectal malformation
• Cardiac anomaly
• Tracheo-esophageal fistula
• Exomphalos (aka omphalocele)
• Renal anomalies
• Limb anomalies

6. RESPIRATORY SYSTEM

1. Cough (chronic): differential

When cough in nursery, rock the "CRADLE":

• Cystic fibrosis
• Rings, slings, and airway things (tracheal rings)/ Respiratory infections
• Aspiration (swallowing dysfunction, TE fistula, gastroesphageal reflux)
• Dyskinetic cilia
• Lung, airway, and vascular malformations (tracheomalacia, vocal cord dysfunction)
• Edema (heart failure)

2. Croup: symptoms

3 S's:

• Stridor
• Subglottic swelling
• Seal-bark cough

3. Cystic fibrosis: exacerbation of pulmonary infection

CF PANCREAS:

• Cough (increase in intensity and frequent spells)
• Fever (usually low grade, unless severe bronchopneumonia is present)
• Pulmonary function deterioration
• Appetite decrease
• Nutrition, weight loss
• CBC (leukocytosis with left shift)
• Radiograph (increase overaeration, peribronchial thickening, mucus plugging)
• Exam (rales or wheezing in previously clear areas, tachypnea, retractions)
• Activity (decreased, impaired exercise intolerance, increased absenteeism)
• Sputum (becomes darker, thicker, and more abundant, forming plugs)

4. Cystic fibrosis: presenting signs

CF PANCREAS:

• Chronic cough and wheezing
• Failure to thrive
• Pancreatic insufficiency (symptoms of malabsorption like steatorrhea)
• Alkalosis and hypotonic dehydration
• Neonatal intestinal obstruction (meconium ileus)/ Nasal polyps
• Clubbing of fingers/ Chest radiograph with characteristic changes
• Rectal prolapse
• Electrolyte elevation in sweat, salty skin
• Absence or congenital atresia of vas deferens
• Sputum with Staph or Pseudomonas (mucoid)

7. GASTROINTESTINAL SYSTEM

1. Duodenal atresia vs. Pyloric stenosis: site of obstruction

• Duodenal Atresia: Distal to Ampulla of vater.
• Pyloric stenosis: Proximal to it.

2. Gastroschisis: usual location

• GasTRoscHIsis usually occurs on the
• RIGHT side of the umbilicus.

(Unscramble the letters).

3. Pyloric stenosis (congential): presentation

Pyloric stenosis is 3 P's:

• Palpable mass
• Paristalsis visible
• Projectile vomiting (2-4 weeks after birth)

8. HEMATOLOGY

1. Hemolytic-Uremic Syndrome (HUS): components

"Remember to decrease the RATE of IV fluids in these patients":

• Renal failure
• Anemia (microangiopathic, hemolytic)
• Thrombocytopenia
• Encephalopathy (TTP)

2. Henoch schonlein purpura: signs and symptoms

NAPA:

• Nephritis
• Arthritis, arthralgias
• Purpura, palpable (especially on buttocks and lower extremities)
• Abdominal pain (need to rule out intussusception)

3. Sickle Cell disease: Signs

SICKLE

• Splenomegaly
• Infections
• Cholelithiasis
• Kidney (Hematuria)
• Live congestion & Leg ulcers
• Eye changes

9. INFECTIONS

1. Measles: complications

"MEASLES COMP" (complications):

• Myocarditis
• Encephalitis
• Appendicitis
• Subacute sclerosing panencephalitis
• Laryngitis
• Early death
• Shits (diarrhea)
• Corneal ulcer
• Otis media
• Mesenteric lymphadenitis
• Pneumonia and related (bronchiolitis-bronchitis-croup)

3. Rashes: time of appearance after fever onset

"Really Sick Children Must Take No Exercise":

 *Number of days after fever onset that a rash will appear:

• 1 Day: Rubella
• 2 Days: Scarlet fever/ Smallpox
• 3 Days: Chickenpox
• 4 Days: Measles (and see the Koplik spots one day prior to rash)
• 5 Days: Typhus & rickettsia (this is variable)
• 6 Days: Nothing
• 7 Days: Enteric fever (salmonella)

4. Rubella: congenital signs

"Rubber Ducky, I'm so blue!" (like the "Rubber Ducky" song):

• Rubber: Rubella
• Ducky: Patent Ductus Arteriosus, VSD and pulmonary artery stenosis.
• I'm: Eyes (cataracts, retinopathy, micropthalmia, glaucoma).
• Blue: "Blueberry Muffin" rash (extramedullary hematopoesis in skin +purpura)

5. Septic Arthritis: most common cause

• Staphylococcus Aureus is the most common cause of
• Septic Arthritis in the pediatric population.

10. NEONATOLOGY

1. Apgar Score: Components

APGAR

• Appearance: Cyanosed or not
• Pulse: Rate
• Grimace: Response to stimulus
• Activity: Movements of the baby (muscle tone)
• Respiration: Rate

2. Bilirubin: phototherapy

• BiLirUbin absorbs light maximally in the
• BLUe range.

3. Neonatal Resuscitation: Steps

Do What Pediatricians Said Totally  Or Be Inviting Costly Malpractice case

• Drying
• Warming
• Positioning 
• Suctioning
• Tactile Stimulation
• Oxygen
• Bagging
• Intubatation (endotracheal)
• Chest compressions
• Medications

11. MISCELLANEOUS

1. Ataxia-Telangiectasia (AT): common sign

AT:

• Absent
• Thymus

3. Guthrie card: diseases identified with it

"Guthrie Cards Can Help Predict BadMetabolism":

• Galactosaemia
• Cystic fibrosis
• Congenital adrenal hyperplasia
• Hypothyroidism
• Phenylketonuria
• Biotidinase deficiency
• Maple syrup urine disease

4. Hematuria: differential in children

ABCDEFGHIJK:

• Anatomy (cysts, etc)
• Bladder (cystitis)
• Cancer (Wilm's tumour)
• Drug related (cyclophosphamide)
• Exercise induced
• Factitious (Munchausen by proxy)
• Glomerulonephritis
• Haematology (bleeding disorder, sickle cell)
• Infection (UTI)
• In Jury (trauma)
• Kidney stones (hypercalciuria)

5. Perez reflex

Eliciting the PErEz reflex will make the baby PEE.