Child & Adolescent Health Flashcards

Question

Height Calculation (2 formulas)

Answer 1

#1 FORMULA Father + Mum / 2 *Boys + 6.5 *Girls - 6.5 ----------------------------------------- #2 FORMULA  Boys: [father's height in cm + (mother's height in cm + 13 cm)]/2  Girls: [(father's height in cm – 13 cm) + mother's height in cm]/2

Answer 2

 Gross motor  Vision and fine motor  Hearing, speech, and language  Social, emotional, and behavioral

Answer 3

2 or more development areas delayed

Answer 4

Gross motor: Lifts chin up

Answer 5

Social, emotional, and behavioral: - Social smile

Answer 6

Social, emotional, and behavioral: - Recognize the mother

Answer 7

Gross motor: - Roll over (prone to supine) - Lifts head up 90 degrees when lying prone Vision and fine motor: - Grasps and plays/shakes with an object Hearing, speech, and language: - Turns to voice

Answer 8

Gross motor: - Rolls (supine to prone). So... Roll both directions

Answer 9

Gross motor: - Begins to sit with support Vision and fine motor: - Palmar grasp Hearing, speech, and language: - Babbling well established - Respond to own name

Answer 10

Vision and fine motor: - Transfers objects from hand to hand

Answer 11

Gross motor: - Sits without support - Crawling Vision and fine motor: - Inferior / Crude pincer grip Social, emotional, and behavioral: - Anxious with strangers Other: First tooth (review info)

Answer 12

Gross motor: - Standing with support (holding on) Hearing, speech, and language: - Understands "NO"

Answer 13

Gross motor: - Walk with support - Cruises around furniture Vision and fine motor: - Finger feeds Hearing, speech, and language: - First word Social, emotional, and behavioral: - Waves "goodbye" - Plays 'peek-a-boo' - Claps hands - Possible separation anxiety (since 6 months)

Answer 14

Gross motor: - Walks up and down stairs holding on Vision and fine motor: - Turns pages - Uses a spoon - Build a six-block tower - Scribbling - Pencil skills: LINE Hearing, speech, and language: - 2 to 4 words sentences

Answer 15

Gross motor: - Climbs stairs alternating foot - Rides a tricycle Vision and fine motor: - Build a nine-block tower - Pencil skills: Circle

Answer 16

Gross motor: Hops and stand on one foot > 2 sec Vision and fine motor: -Pencil skills (in order) *Cross *Square *Triangle Hearing, speech, and language: - Give first and last name

Answer 17

Gross motor: - Climb - Somersault Vision and fine motor: - Self toilet - Use a fork and spoon - Can get dressed and undressed Hearing, speech, and language: - Speaks very clearly - Name colors

Answer 18

Gross motor: - Riding a bicycle - Skipping

Answer 19

Gross motor: Walk alone or with one hand held Vision and fine motor: Neat/mature pincer grip

Answer 20

1. Pinching (pulp-to-pulp) 2. Pincing (tip-to-tip with curled fingers) 3. Pointing

Answer 21

Gross motor: - Pointing - Walks well - Probably holding hand upstairs Vision and fine motor: - Drinks from a cup - Builds a 2-3 block tower Hearing, speech, and language: - Says mama/dada appropriate - Points to body parts

Answer 22

Corrected age = Chronological age (-) # weeks or months premature weeks or months premature = 40 weeks (-) weeks at birth NOTE: The corrected age is taken into consideration when looking at milestones until the age of 2 years old.

Answer 23

Done at 2-4 days old Include 9 conditions: 1. Congenital hypothyroidism (CHT) 2. Sickle cell disorders 3. Cystic fibrosis (CF) 4. Phenylketonuria (PKU) 5. Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) 6. Maple syrup urine disease (MSUD) 7. Isovaleric acidaemia (IVA) 8. Glutaric aciduria type 1 (GA1) 9. Homocystinuria (pyridoxine unresponsive) (HCU)

Answer 24

1. Call for help 2. Stimulate (rubbing of soles of feet or chest) and assess response 3. Airway - opening manoeuvres - Head and neck positioned in a neutral position - Gently suction mouth and nostrils if necessary 4. Positive pressure ventilation: BAG AND MASK.

Answer 25

 RR > 60/ min  HR > 160/min  Grunting  Intercostal space drawing  Nasal flaring  Central cyanosis

Answer 26

1. Transient Tachypnoea of the Newborn (TTN) 2. Meconium Aspiration Syndrome (MAS) 3. Hyaline Membrane Disease (HMD)

Answer 27

1. Pulse oximetry (Continuous monitor) 2. Arterial blood gas (ABG): not indicated initially, unless an underlying condition is suspected or ongoing respiratory difficulty exists.

Answer 28

Chest X-Ray

Answer 29

- Medical care is supportive: Supplemental oxygen to maintain adequate arterial oxygen saturation.

Answer 30

CHEST X-RAY (diagnostic standard) - Prominent perihilar streaking, which correlates with the engorgement of the lymphatic system with retained lung fluid, and fluid in the fissures. - Small pleural effusions. - Patchy infiltrates.

Answer 31

Septicaemia **Necrotizing enterocolitis (NEC)** Retinopathy of prematurity (ROP) Hypertension Failure to thrive Intraventricular hemorrhage (IVH) Periventricular leukomalacia (PVL) - With associated neurodevelopmental and audio-visual handicaps

Answer 32

1. **CXR **IF: - Meconium aspiration syndrome suspicion - Neonatal pneumonia suspicion - Respiratory status worsens 2. **Echocardiogram** IF: - Persistent tachypnea > 5-6 days. Rule out congenital cardiac anomalies and function.

Answer 33

- Baby at term w/ sibling that died of the same, and now have severe resp distress Physiopathology: Over-production of surfactant proteins within the alveoli.

Answer 34

*Autoimmune (90%) *Secondary (4%) *Congenital (1%), depending on the gene involved: - Autosomal dominant - Autosomal recessive - X-linked recessive

Answer 35

1. Bronchoalveolar lavage. 2. Lung transplant.

Answer 36

* Smoking parents (passive smoking) * Parental narcotic/cocaine abuse * Prone position during sleep * Artificial feeding * Hyperthermia * Extreme prematurity * URTI

Answer 37

1. High-pitched cry 2. Hyperreflexia. 3. Tremors. 4. Seizures. 5. Hypertonia.

Answer 38

Heroine (2-3 days) Methadone (up to 1-2w)

Answer 39

Morphine to assist with gradual withdrawal, then decrease slowly.

Answer 40

- Pinpoint pupils - Lethargy - Mom's use of opioids: Meperidine (Demerol), pethidine & heroine

Answer 41

Treatment: - Next: Bag and mask ventilation - Best: Naloxone is tx. Should only be given if the mother has received narcotics < 2 hrs of delivery

Answer 42

Neonatal overdose syndrome. (History of Mom's use of opioids)

Answer 43

Clinical features: - **Jitteriness** **- Peripheral cyanosis** - Irritability - Poor feeding - Seizures - High pitched cry - Lethargy - Hypotonia (rare) Association with: - Hepatomegaly - Micropenis - Macrosomia - Cleft lip Investigations??? Treatment???

Answer 44

Jaundice may not be visible in the neonate's skin until the bilirubin concentration exceeds 70-100 micromol/L. Infants that are not jaundiced to the naked eye do not need routine bilirubin checking.

Answer 45

- >35 weeks gestation - >24 hours old for the first measurement. - TCB can be used for all subsequent measurements, providing the level remains <250 µmol/L and the child has not required treatment NOTE: If the patient is not a good candidate for TCB, SERUM BILIRUBIN should be performed

Answer 46

Still, send the child for serum bilirubin (SBR) ASAP - despite Kramer rule results

Answer 47

NICE treatment threshold graph

Answer 48

There are different types:  Single Light - Double Lights w/ Bili blanket or even Triple Lights.  They are all the same methods just different intensity of exposure. Give the baby eye protection Cease phototherapy when SBR is at least 50 micromol/L below the phototherapy range for the age. SUNLIGHT EXPOSURE IS NOT RECOMMENDED AS A TREATMENT FOR JAUNDICE

Answer 49

 overheating  water loss  diarrhoea  ileus (preterm infants)  rash (no specific treatment required)  retinal damage (theoretical)  parental anxiety/separation  'bronzing' of infants with conjugated hyperbilirubinemia.

Answer 50

 have cord blood haemoglobin < 100 g/L  have cord bilirubin > 80 micromol/L  are visibly jaundiced within 12 hours of birth.

Answer 51

During: * Continuing multiple phototherapy * Perform a double-volume exchange After: * Maintain continuous multiple phototherapy * Measure serum bilirubin level within 2 hours and manage according to threshold table

Answer 52

* apnoea * bradycardia * cyanosis * vasospasm * air embolism * infection * thrombosis * **necrotising enterocolitis**

Answer 53

Permanent clinical sequelae of bilirubin toxicity (UNCONJUGATED)

Answer 54

- Athetoid/Dyskinetic cerebral palsy (abnormal posturing, tone, and involuntary movements) - Developmental and intellectual delay - Hearing deficit - Dental dysplasia - Oculomotor disturbance

Answer 55

 Serum bilirubin >340 micromol/L in term neonates  Preterm infants may be at risk at lower SBR < 300 micromol/L  Rapidly rising bilirubin level of greater than 8.5 micromol/L per hour  Clinical features of acute bilirubin encephalopathy

Answer 56

**Initially:** - Decreased feeding (poor suck reflex) - Lethargy - Abnormal tone: hypotonia **Progress to:** - High-pitched cry - Abnormal tone: Hypertonia - Retrocollis and opisthotonus - Setting-sun sign (upward-gaze paresis) - Fever - Seizures

Answer 57

- Shorter lifespan of neonatal red blood cells - Immature liver function at birth - A relatively high concentration of β-glucuronidase in the small intestine

Answer 58

1. Preterm babies (higher bilirubin levels) 2. Exclusive breastfed babies 3. Babies with significant bruising or cephalohaematoma: The breakdown of RBCs within the cephalohaematoma causes higher bilirubin levels and predisposes to jaundice. 4. Previous sibling with neonatal jaundice requiring phototherapy

Answer 59

* Day 2-14 (> 21 in preterm) * Mild * Diagnosis of exclusion * Resolves in 2w * Rarely exceeds 220 micromol/L

Answer 60

* Too early < 24 hours of age * Too Long > 10 - 14 days of age (term: 2 weeks / preterm: 3 weeks) * Too high > 220 micromol/L * CONJUGATED Hyperbilirubinemia

Answer 61

- Physiological - Breast milk jaundice - Sepsis - Metabolic: * Gilbert’s syndrome * Congenital hypothyroidism * Crigler-Najjar syndrome - Hemolytic: * ABO/Rh incompatibility * Spherocytosis * G6PD deficiency * Sickle cell anemia

Answer 62

1. Biliary atresia 2. Neonatal hepatitis - TORCH infection - Idiopathic - Metabolic: Galactosemia, Wilson, alpha 1antitripsine. **NOTE: Always > 24 h** **Conjugated** bilirubin level **>25** micromol/L because this may indicate **serious liver disease**

Answer 63

Sepsis OR GIT obstruction

Answer 64

**Hemolysis** 1. ABO incompatibility (Most common) - Mother: O group; Child: A or B - Direct Coombs (+) - Peripheral smear: Spherocytes (some) 2. Spherocytosis - Peripheral smear: Predominant spherocytes - Direct Coombs (-) - FBE:↑ MCHC - FxHx: Anemia/spherocytosis/gallstones 3. Sickle Cell - Peripheral smear: Sickle and target cells - Direct Coombs (-) - African descendants 3. Rh incompatibility (Most severe) - Peripheral smear: NO spherocytes - Direct Coombs (+)

Answer 65

1. Physiological jaundice Most Common cause in the first week: - Term: 50% - Preterm: 80% finishes in 1-2 weeks (preterm 3 weeks) 2. Breast milk jaundice Lasts up to 6 weeks Diagnose: Suspending breastfeeding for 24-48 hrs = ↓ serum bilirubin 3. Neonatal sepsis End of the first week (4-7 days old) Lethargic + jaundice + hepatosplenomegaly ↑ BOTH, Direct and Indirect bilirubin.

Answer 66

G6PD deficiency OR Spherocytosis (FxHx of gall stones)

Answer 67

Biliary obstruction (Biliary atresia)

Answer 68

Polycythaemia

Answer 69

Hepatitis (↑ liver enzymes) OR Metabolic problems (Galactosemia)

Answer 70

1st Hemocultures 2nd ATB: Wich??

Answer 71

Inheritance: Autosomal recessive Deficit of glucuronyl transferase => ↑ unconjugated bilirubin Triggers: fasting, intercurrent illness, menstruation, stress, and dehydration Other blood tests are normal No treatment required.

Answer 72

Amoxiclav Flucoxaciline Erythromycin Rifampicin Radiographic agent

Answer 73

Fasting bilirubin Bilirubin after nicotinic acid Liver biopsy (normal) NOTE: Diagnostic tests are NOT performed in the majority of patients.

Answer 74

 **listless**  Goitre  prominent tongue  hoarse cry  puffy face  constipation  umbilical hernia  hypothermia  bradycardia  dry skin  failure to thrive

Answer 75

Mother with: - Diet: Iodine deficiency - Medication history: Amiodarone, methimazole - Auto-immune disease: Hashimoto

Answer 76

1. T4 and TSH levels 2. Serum bilirubin (SBR) if clinically indicated. 3. Thyroid scan: showing absent, lingual or increased uptake of radioisotope

Answer 77

 Referral to Endocrine team  Thyroxine replacement therapy ASAP  Growth and development must be closely monitored.  Hearing tests should be done.

Answer 78

Levotiroxine

Answer 79

Normal intellectual and physical development if the treatment is commenced promptly (<2 weeks old) and monitored closely.

Answer 80

Inheritance: Autosomal recessive UDP-G Absent Unconjugated bilirubin > 340 micromol/L Complication: Kernicterus, unless rapid treatment Management: Phototherapy, exchange transfusion, etc. Phenobarbital doesn’t help

Answer 81

Inheritance: Autosomal recessive UDP-G Decreased Unconjugated bilirubin < 340 micromol/L Management: Phenobarbital help

Answer 82

Inheritance: Autosomal dominant - English – Irish – Scottish descendants. - Gallstone family history - Splenomegaly - FBE: ↓ Hb ↑ MCHC - Blood smear: Abnormally shaped RBC or Spherocytes

Answer 83

Osmotic fragility test

Answer 84

Eosin-5-maleimide test

Answer 85

- X-linked Recessive (only boys) - Sudanese, African descents

Answer 86

Hemolytic anemia Jaundice Lethargy Dark urine

Answer 87

1. FBE: ↓ RBC count, ↓ Hb level, ↑ reticulocytes 2. Peripheral blood smears:* **Heinz bodies** (bite cell): Inclusions within red blood cells composed of denatured hemoglobin 3. LFT: ↑Total Bilirubin ↑Unconjugated (Haemolysis)

Answer 88

1. G6PD Assay NOTE: Beutler fluorescent spot test is for screening 2. **Genetic Screening** is recommended to all **1st degree relatives** – also to **exclude** other haemolytic anaemias like **Sickle** that is also common in the same community

Answer 89

1. Avoid oxidative stress triggers: * Infections * Cold weather * Hypoxia * Dehydration * Acidosis * Surgery * Certain foods like **fava beans** * Antioxidant drugs: Sulphonamides, antimalarial, nitrofurantoin, naphthalene, aspirin, high dose of Vit. C and K and 2. Vit E 3. SEVERE CASES: RCB TRANSFUSIONS

Answer 90

Obliteration of the extra-hepatic bile ducts - Fetal (20%) - Perinatal: after birth (80%) Type III it's the most common (90%), and involves all biliary ducts.

Answer 91

- Presentation: since the 1st week - Prolonged Jaundice (> 14 days in term and > 21 in preterm) - Dark urine - Clay-colored stools (even meconium is pale) - Abdominal pain (crying on changing diapers) - Hepatosplenomegaly ( >2cm below costal margin)

Answer 92

- LFT (all kids with JAUNDICE) - Total & Direct Bilirubin (↑CONJUGATED) - Full Blood exam: Haemoglobin level - Urgent: Abdominal US and GE review.

Answer 93

Percutaneous **biopsy** (gold-standard): **Bile ductular proliferation** (>specific), bile plugging, multinucleated giant cells, focal necrosis of liver parenchyma, extramedullary hemopoiesis, and inflammatory cell infiltrate.

Answer 94

- Surgery: * Portoenterostomy (Kasai procedure) *Within 70 days to prevent biliary cirrhosis OR - Liver Transplant

Answer 95

1. Cataracts 2. Chronic liver failure 3. Failure to thrive (FTT): fails to gain weight or height. 4. Delay: Slower than normal development of motor, cognitive, social, and emotional skills.

Answer 96

1. Lactose-free formula 2. Screen family members

Answer 97

Very common Develops within 2-4 days of birth, may peak at 7-15 days of age, and may persist for many weeks. No need to stop breastfeeding

Answer 98

- Watery or foamy stools - Farty baby (LOL) - Excoriation of buttocks - Normal growth - Abdominal bloating - Gurgling stomach - Presentation after an episode of viral gastroenteritis

Answer 99

TRIAL: Cut lactose from the diet for 2 weeks. RESULTS: * Successful: Lactose should be cut out from the diet for 8 weeks and then gradually re-introduced over a week. * Unsuccessful: Other causes should be suspected.

Answer 100

Stool acidity test: Unabsorbed lactose is fermented by colonic bacteria into lactic acid, which lowers the stool pH.

Answer 101

Endoscopy with Small bowel biopsy

Answer 102

- Continue breastfeeding unless severe excoriation or inadequate weight gain - Formula-fed infants should be placed in soy formula.

Answer 103

Clinical features: - Diarrhea - Malabsorption - Failure to thrive (FTT)

Answer 104

Complete elimination of cow's milk protein (CMP) challenge test.

Answer 105

- Breastfeeding: The mother should eliminate all foods containing cow's milk protein (cheese, yogurt, and butter) - Extensively hydrolyzed hypoallergenic formulas. - Supplements of vitamin D and riboflavin. - It doesn’t improve with soy, sheep’s or goat’s milk

Answer 106

- Iron - Vit C

Answer 107

Age: 1 - 3 yo Diarrhea with undigested food (a piece of carrot) Reassure and explain

Answer 108

Clinical features: - Crying for 3 hours in late afternoon and early evening - Flexing legs - Clenching fists - Appears in pain - Everything else is normal

Answer 109

1. Exclude organic causes 2. Reassure 3. Infacol colic relief drops Note: Severe cases can trial CMP elimination

Answer 110

* Malrotation and volvulus * Duodenal atresia * Meconium ileus * Meconium plug * Hirschsprung’s disease

Answer 111

* Pyloric stenosis * Transoesophageal atresia * Gastro-Oesophageal Reflux Disease (GORD) * Infections: Gastroenteritis, UTI, Otitis media.

Answer 112

 Abnormal rotation of the gut during embryogenesis.  1:6000 live births  Associated with duodenal atresia-stenosis, abdominal wall defects, choanal atresia  Most patients will develop volvulus within the first week of life.

Answer 113

 Bilious vomiting  Abdominal Distension is minimal (very late symptom)  Abdominal x-ray may be normal.

Answer 114

* Abdominal xray is grally(-) * USG: Malposition of the superior mesenteric vessels

Answer 115

Requires urgent surgery

Answer 116

 In 80% of cases, the obstruction is distal to the papilla of Vater => bilious vomiting  Incidence: 1:5000 - 10000  M > F  Associated with Down syndrome in 25% of cases  Antenatal: Polyhydramnios  X-ray: 'double-bubble’  Mx: referral for surgery

Answer 117

Thick tenacious meconium in the bowel causes obstruction.  15% of newborns with cystic fibrosis  90% of patients with meconium ileus have cystic fibrosis. Presentation:  Acute early marked bowel distension within first 24hs w/ bilious vomiting. DRE: mucus plugs is still present.

Answer 118

 Volvulus  Bowel perforation and/or meconium peritonitis

Answer 119

Xray  distended loops of the intestine with thickened bowel walls  meconium mixed with swallowed air produces a 'ground-glass' sign typical of meconium ileus  Free air, very large air-fluid levels => bowel perforation.

Answer 120

Plan:  uncomplicated meconium ileus: hypertonic enemas & IV fluids  Immediate surgery for infants with complicated meconium ileus – perforations.

Answer 121

Most common form of functional distal intestinal obstruction in the neonate (1:500-1:1000). Caused by meconium in the distal colon or rectum. Clinical Features: * Acute marked abdominal distension and failure to pass meconium in the first 24hs of life.

Answer 122

 X-Ray: Generalised intestinal dilatation (LBO)

Answer 123

 Enema or digital exam is usually curative.

Answer 124

 Causes 15-20 % of newborn intestinal obstructions - 1:4000  Abnormal innervation of the colon => absence of ganglion cells in the Auerbach and Meissner Plexus  Distal spastic zone (contracted) w/ Proximal dilated zone (normal)

Answer 125

* 80% in the first 6 weeks of life - male > female * Failure to pass meconium in the first 24 hours but w/ gradual onset of abdominal distension of days to weeks. * Persistent and progressive constipation. * Vomiting late  On digital rectal examination we have explosive poo.  The most serious complication is enterocolitis  Enemas should be avoided during episodes of enterocolitis because of the possibility of perforating the colon

Answer 126

 Rectal suction biopsy – Gold standard

Answer 127

 Laxatives (mild cases)  Surgery

Answer 128

Rapidly progressive disease in (premature) newborns or w/low APGAR score. * Causes extensive bowel necrosis, infection and perforation * 10 -12 days after birth * Associated with RDS TIP: represents mesenteric ischemia in adult

Answer 129

* Gastric retention * Bilious vomiting * Ileus * Abdominal distension * Bloody stools – red currant

Answer 130

* Abdominal X-Ray => Dilated bowel loops * Pneumatosis intestinalis

Answer 131

 Gastric decompression & IV fluids  Surgery for perforation, Clinical deterioration, metabolic acidosis (ischemia)

Answer 132

Laboratory investigations:  Baseline blood tests (FBC, CRP): CRP may be raised and there may be** thrombocytopenia and neutropenia.**  Blood cultures: non-specific in NEC and are commonly reported as negative. However, if a bacterial, viral or fungal agent is isolated this can be useful for guiding treatment.  Blood gas: may show a **raised lactate or acidosis.** Imaging  **Abdominal ultrasound**: ultrasound is a safe **first choice** of imaging due to the lack of exposure to ionising radiation. Signs that are indicative of NEC include **air in the portal system, ascites and perforation.** ** Abdominal X-ray**: may show thickening of the bowel wall, dilated bowel loops filled with gas and distended bowel.  If the **bowel has perforated**, **Rigler’s sign** may be visible. This occurs when both sides of the bowel wall are visible due to the presence of gas inside the lumen and within the peritoneal cavity.

Answer 133

 Symptoms presents at 2 - 6 weeks of age Clinical features: * Projectile vomiting * Baby appears HUNGRY * FTT * Visible gastric peristalsis * Pyloric mass (olive-shaped)

Answer 134

 First: Palpation during test feed  USG  FBE, electrolytes, ABGs.

Answer 135

 Loss of H2O and HCl  Dehydration causes conservation of H2O and Na+ in exchange for H+ and K+ Result: **Hypovolemia, metabolic alkalosis**

Answer 136

 Shock: NS 20 ml/kg  IV Fluid replacement: 0.45% NS + 5% Dextrose  Potassium replacement once baby passes urine (KCl)  Surgery: Refer for Pyloromyotomy  Good prognosis

Answer 137

Vomiting from first feeding As the narrowing is above the level of the biliary duct, the vomit would also appear the same color as the milk ingested.

Answer 138

* Peaks at 4 months * Self-limiting – usually resolves by 18 months  Clinical features: * Irritability * Crying * Milky vomitus * Spontaneous effortless regurgitation of gastric contents

Answer 139

 Invagination of the proximal segment of bowel into the distal bowel lumen.  The commonest is segment of ileum  colon through the ileocecal valve. Most common age: 3m - 1yo. HSP kid or teen w/ skin Preutz-Jeger px – at any age Clinical Presentation: * Intermittent abdominal pain which is colicky, and severe. The child may draw up the legs. * Episodic 2-3 times/hour, increasing over next 12-24 hours. * Pallor and lethargy - Vomiting * Diarrhoea- blood or mucus - classic red currant jelly stool is a late sign

Answer 140

MX  On PE: Abdominal mass - sausage shaped mass RUQ or crossing midline in epigastrium or behind umbilicus, palpable in about 60% of children and signs of an acute bowel obstruction will be present. * Plain abdominal Xray is the 1st step in Dx: * Target sign - 2 concentric circular radiolucent lines usually in the right upper quadrant or Crescent sign - a crescent shaped lucency usually in the left upper quadrant with a soft tissue mass * Ultrasound scan next step in Dx – also preferred in HSP Abd pain. * Barium or Air enema: best step - is both diagnostic and therapeutic, as long as there is a paeds surgical team available on site as there is a risk of perforation. Mx plan:  Gas or barium enema only in a setting where there is a pediatric surgeon available in case of perforation.  Hydrostatic reduction under USG.  Surgery: If enema failed, peritonitis or septicemia

Answer 141

- BradIcardia (AV block) in newborn. - Mums with primary Sjogren or undifferentiated SLE

Answer 142

AntiRo antibodies

Answer 143

1. Gonorrhoea - Most dangerous. - Complication: Corneal perforation. - Presentation: 1-5d after birth. - Tx: IV Cefotaxime for 7d. 2. Chlamydia: - Most common - Presentation: 10- 14d after birth. - Tx: Erythromycin for 21d + eye toilet

Answer 144

- Mucopurulent discharge - Watery eye - Worse on waking - Conjunctiva not inflamed

Answer 145

- Acute: Birth-neonates: Immediate referral

Answer 146

1. Massage the nasolacrimal sac 2. Wash with salt water 3. Warm compress 4. Chloramphenicol drops until tears clear

Answer 147

- Umbilical mass in neonates - Swelling - Non-mucopurulent discharge - The cord is swollen and pink

Answer 148

1st: Salt 2nd: Silver Nitrate for 5 days

Answer 149

# CA Urine discharge Differential diagnoses: Fistula Urachal cyst Umbilical calculus Patent urachus

Answer 150

* Creatinine and urea levels from discharge -> confirm it’s a urinary discharge * U/S: Rule out patent urachus

Answer 151

Clean debris Apply dressing (wound) Swab for culture and sensitivity A/B Refer for surgical correction

Answer 152

Urinary Tract Infection (UTI) (especially in the case of full patency and bladder diverticulum) Cyst infection Abdominal pain Haematuria

Answer 153

Infection of the umbilicus and/or surrounding tissues. Purulent (±bloody) discharge from the umbilical cord Surrounding: induration, erythema, and tenderness. Systemic signs such as fever, lethargy or poor feeding are suggestive of a more severe infection.

Answer 154

Unplanned home birth or septic delivery Low birth weight Prolonged rupture of membranes (PROM) Umbilical catheterization Chorioamnionitis

Answer 155

Staphylococcus aureus (MCC) Streptococci group A and group B Gram-negative bacilli

Answer 156

* Admission *Septic workup * Cultures (including dry swab of the discharge) * IV antibiotics: <1 month: Refer to neonatal > 1 month: Flucloxacillin OR Gentamicin

Answer 157

Soft swelling, varying in size, of the umbilical area – usually easily reduced. More prominent during coughing, crying, or straining and could became larger in the first 6 months of life. Presentation: - Most cases are asymptomatic. - Incarcerated or strangulated umbilical hernias are extremely rare.

Answer 158

- Prematurity - Low birth weight - African descent - Congenital Hypotiroidism - Certain syndromes: Patau (13), Edwards' (18), and Down syndrome (21).

Answer 159

Asymptomatic: Observation until 4-5 years of age is preferred since the closure of the umbilical ring is complete in most children by the age of 5-6 years old. Symptomatic: SURGERY Incarceration (Firm, irreducible, tender, red, associated with vomiting) or Very large/persistently growing hernias NOTE: Surgical correction in children < 4 years old has a higher complication Spontaneous closure is less likely in cases of a hernia >1.5 cm, older age, underlying predisposing condition (e.g. Ehlers Danlos syndrome).

Answer 160

- Swelling - White exudate - Redness

Answer 161

1. Local penile toilet 2. Mupirocin 3. Penis shaft skin swollen to the pubis = IV atbs

Answer 162

Scarring of preputial opening. Tight ring or “rubber band” of foreskin around the tip of the penis, preventing full retraction.

Answer 163

- Mild: Steroid topical cream - Severe: Circumcision

Answer 164

Phimosis & balanitis Absolute CI: Hypospadias

Answer 165

Dorsal penile nerve block

Answer 166

- Dysarthria (MC symptom) - Descending paralysis - Low reflexes - Dry mouth - Blurry vision - Urinary retention

Answer 167

- Blood test for toxin

Answer 168

1. Antitoxin 2. Notifiable disease

Answer 169

- 2 to 5 years old - Does not cross the midline - Asymptomatic abdominal mass (maybe abdominal pain, HTA, and gross hematuria)

Answer 170

Ultrasound

Answer 171

Best Investigation: CT/MRI to stage

Answer 172

Nephrectomy + Chemo

Answer 173

- < 2 years old - Crosses the midline - Painful abdominal mass (adrenal medulla) - Racoon eyes (retroorbital metastasis) - Associated with Horner syndrome (cervical ganglia)

Answer 174

Qx + chemo and radio

Answer 175

- < 5years old - Abdominal mass with intermittent abdominal pain - Hx of Familial adenomatous polyposis (FAP)

Answer 176

Autosomal dominant Clinical features: 1. Edema in: - Respiratory tract (cough) - Eyelids (swelling) - Intestines (abd pain) 2. The attacks last 2 to 5 days, usually slowly increasing. Are preceded by prodromal symptoms, including a rash (erythema marginatum) 3. Triggers: Trauma, infections, stress or procedures, ACE inhibitors, and estrogens.

Answer 177

Complement test (Low C3 & C4)

Answer 178

C1-inhibitor protein (low) and function

Answer 179

Bluish grey lesion in buttocks, lower back, extensor surfaces of extremities. Common in black, Asian, and Latin kids. Disappears by 1- 2 years old

Answer 180

1. Kawasaki Disease 2. Scarlet fever 3. Allergies 4. Toxic shock syndrome (associated with tampons, diaphragms, skin inf, pneumonia, osteomyelitis)

Answer 181

Toxic shock syndrome: S. Aureus TSS1-toxin

Answer 182

Autosomal Recessive Defects in the ion protein channel CFTR. Viscoid secretions in the lungs, pancreas, and gut. Clinical features: - Meconium Ileus - Prolonged neonatal jaundice - Failure to thrive and poor weight gain - Chronic lung diseases - Bronchiectasis - Fat globules on feces examination (Diff from fatty crystals in celiac and CMPA) - Vas deferens atrophy - Increase sweat CI: Hyponatremic, hypochloremic, metabolic alkalosis

Answer 183

1. Newborns: Heel Prick test. 2. If (-) but parental concern: Do test for gene mutations: 1 mutation: Sweat chloride test. If (+) CF clinic, if (-) healthy carrier ≥2 mutations: CF clinic

Answer 184

1. Fix nutritional deficiencies: High-calorie and high-fat diets with supplemental fat-soluble vitamins to compensate for malabsorption and maintain a healthy weight. 2. Minimize chest infections.

Answer 185

Bronchiolitis peaks between the age of 3-6 months

Answer 186

ALSO: Otherwise, unexplained low FEV1 or PEF (historical or serial readings) Otherwise, unexplained peripheral blood eosinophilia

Answer 187

Adult teeth replace baby teeth between 6-12yo

Answer 188

- Babies and toddlers: brush teeth 2x/day - 18m-6y: brush with LOW fluoride toothpaste

Answer 189

- Daytime control: 4yo - Nighttime control: ≥7yo - Primary: Never was dried - Sec: Dried for 6m and wet again MCC: Bladder infection

Answer 190

1. Urotherapy: Increasing daytime fluid intake (50 ml/kg/day). Regular voiding every 2–3 hours. 2. Bedwetting Alarm: Start from 7yo. Do for 6-8w, overlearn for 2w more. 2. Desmopressin: If the kid is going to school camp, prefer oral or sublingual. Is not recommended because of the high risk of HypoNa.

Answer 191

Pubertal Changes Secuence: 1. Enlargement of testes & scrotum (>2.5cm or >4ml) 2. Pubic hair (6 m) 3. Lengthening of penis (12 - 18 m) 4. Axillary hair > medial thigh hair and Growth spurt. Deepening of the voice (2 y after initial signs of puberty) TANNER STAGES Stage 1: kid (< 9 yo) Stage 2: scrotal and testicular growth (9 -11 yo) Stage 3: gynaecomastia and voice break (11 - 13 yo) Stage 4: Axillary hair and acne (13 - 15 yo) Stage 5: Adult (> 15 yo) Delayed: >14 yo (testis and axillary hair) Ix: Bone age Preconscious: 9.5 yo 1st Ix: FSH and LH 2nd Ix: MRI of brain Management: Refer to the endocrinologist

Answer 192

Pubertal Changes Secuence: 1. Boobs 2. Grow 3. Pubes 4. Flow TANNER STAGES Stage 1 Telarche: 2 y till menarche Stage 2 Adrenarche: Axillary and pubic hair, body odor (9 -11 yo) Stage 3: Menarche (11 - 13 yo) Stage 4: Pubic hair (13 - 15 yo) Stage 5: Adult (> 15 yo) Delayed: > 13 -14 yo (Bubs, axillary and pubic hair) 1st Ix: Bone age Preconscious: < 8 yo 1st Ix: FSH and LH 2nd MRI of brain Management: 1. If only isolated growth public hair < 8yo: Follow up in 6 months 2. Refer to the endocrinologist

Answer 193

- Males <1.62 Females <1.52 - If constitutional delay BA

Answer 194

1. Bone age x-ray (left hand wrist) 2. TFT, LFT, IGF-1

Answer 195

GH Tx only if height<1st centile for age, or Growth velocity <25th centile

Answer 196

- Clinical features: guilt, anger, sexual behavior with other kids, unexplained physical symptoms, sleep disturbance, poor school performance. - Org dx: Chlamydis, syphilis, gonorrhea, and HIV.

Answer 197

- Prepubertal: Visual examination - Postpubertal: Speculum

Answer 198

Refer to CPS

Answer 199

- Blinking - Eye twitch - Facial grimacing - Shoulder shrugging with sniffing - Coprolalia - Echolalia

Answer 200

1. Behavioural therapy 2. Risperidone

Answer 201

Difficulty concentrating, social withdrawal, decline in school performance, becoming superstitious

Answer 202

1-3yo Frustration->breath hold-> cyanosis ->LOC DD: In seizures it's LOC->Cyanosis

Answer 203

Behavioural therapy

Answer 204

- Child 4 - 7yo (NO <2yo) - Most common in boys - Social, academic, and occupational impairment - Slurred speech

Answer 205

- School reports - Psychoeducational Assessment - Audiology - vision Assessment

Answer 206

1. Behavioral modification 2. Methylphenidate (MOA: inhibition of Dopamine and NE reuptake). If bad compliance use the slow release

Answer 207

- Low inteligence - Bad social interaction and communication - Repetitive stereotype behaviors - - - Enjoys being alone

Answer 208

Risperidone (if aggressive)

Answer 209

- Normal to superior intelligence - Impaired social and communication skills - Seek friendships

Answer 210

Behavioral therapy

Answer 211

- Since > 6 m -Defiant, negative, and disobedient. - Hostile behavior toward authority figures that are present - Interfere with academic, social, and occupational functioning - No violent or aggressive behaviour

Answer 212

Best: Family therapy

Answer 213

<18yo Violation of rules, theft, destruction of property, aggression cruelty toward people and animals

Answer 214

Child 5-7yo. Wakes up, cannot be consoled by parents, cannot remember anything the day after

Answer 215

Family reassurance

Answer 216

- Daytime sleepiness - Disturbed sleep - HTN - Narcolepsy

Answer 217

Polysomnography

Answer 218

1. Adenotonsillectomy 2. Weight loss 3. IN steroids for rhinitis 4. CPAP

Answer 219

- Obese pre or adolescent - Atraumatic hip, thigh, or knee (medial obturator nerve) pain - Limping - Inability to bear weight - Limited internal rotation (Drehmann sign)

Answer 220

- Obesity - Male sex - Periods of rapid growth - Prior radiation therapy

Answer 221

Hip XR AP and frog-leg lateral views

Answer 222

Surgical: Open Reduction and Internal Fixation

Answer 223

Femoral head osteonecrosis

Answer 224

- Barlow maneuver: Gentle ABDUCT and "Hip clicks" - Ortolani maneuver: ADDUCT the hip *DD: In dislocated hip: jerk or clunk* - Trendelenburg gait - Toe walking - Leg length discrepancies

Answer 225

- Breech position (most significant) - Female sex - Family history - Oligohydramnios - Multiple pregnancy (twins) - Delivery by C-Section - Large for gestational age (LGA)

Answer 226

US 3–4 months

Answer 227

X-ray: AP of the pelvis

Answer 228

< 18 m: Hip spica cast (close reduction) Complication: Avascular necrosis of the femoral head. > 18 m: Surgery, Open reduction.

Answer 229

Idiopathic avascular necrosis of the capital femoral epiphysis of the femoral head - Male (MC) between 4 - 9 yo - Limping - Pain localized to the hip or referred to the knee or thigh - Limited abduction and internal rotation - Asoc w/ coagulopathies (Haemophilia)

Answer 230

US: Widening of joint space (epiphyseal cartilage hypertrophy) X-ray: AP of the pelvis ????

Answer 231

Immobilize and refer immediately

Answer 232

- Age: 3 - 8 yo (MC 6 yo) - Male sex - Fever (30%) - Unilateral hip pain and limping. - Refusal to bear weight. - Presentation with the hip flexed, abducted, and externally rotated. - PE: Limited abduction and internal rotation, Patrick test (Pain on the ipsilateral anterior side) - Preceding: URTI, trauma, or bacterial infection (post-streptococcal toxic synovitis)

Answer 233

*Rule out Septic Arthritis* 1. WBC, CRP, ESR 2. Hip X-ray

Answer 234

US w/ join aspiration: Diagnostic and therapeutic. Intracapsular effusion. SA: - Positive gram stain - PMN > 90% - glucose < 40 mg/dL

Answer 235

supportive care and rest from activity. NSAIDs can be used for pain control. Complete resolution of symptoms often takes up to 1 to 2 weeks (sometimes within 48 hours) <3

Answer 236

NORMAL: - 0-1y: Metatarsus varus - 1-3y: Internal tibial torsion - 5-6yr: Medial femoral torsion

Answer 237

Acyanotic {Left to the right} 1. VSD - Ventricular Septal Defect* 2. ASD - Atrial Septal Defect 3. PDA - Patent Ductus Arteriosus Cyanotic {Right to the left} 1. TOF - Tetralogy of Fallot 2. HLHD - Hypoplastic Left Heart Dx 3. TGV - Transposition of the great vessels

Answer 238

Most common congenital heart disease. Asoc w/ Turner Sx Presentation age: Infant 5 to 6 weeks Clinical Features: 2-3/6 harsh holosystolic murmur heard along the LSB more prominent with small VSD and absent with very Large VSD SMALL - Moderate: 3-6mm - Asymptomatic - 50% will close spontaneously at 2y Mod - LARGE - Symptomatic & require Sx repair - SOB with feeding & crying - Recurrent chest infections - Heart failure from 3 months of age - FTT

Answer 239

- Small VSD: No physical restrictions, reassurance, periodic follow-up & endocarditis prophylaxis. - Symptomatic VSD: Medical treatment initially with afterload reducers & diuretics. - Indications for Surgical Closure:  Large VSD w/ medically uncontrolled symptomatology & continued FTT.  Ages 6-12 mo w/ large VSD & Pulmonary HT

Answer 240

Secundum ASD – Fossa Ovalis, most common Primum ASD – lower in position, more serious Clinical Features: - Initial: NO MURMUR - PS: Systolic ejection murmur - LSB - RV heave. - Fixed widely split S2 (Ao then Pulm Valve closes) - Asymptomatic or easy fatigability or mild growth failure. High risk of developing right heart failure with pulmonary HT

Answer 241

* Surgical or device closure for Secundum ASD * Closure performed between ages 2 & 5 years * Sx correction is done earlier in children w/ CHF or significant pulmonary HTN.  NOT Endocarditis prophylaxis

Answer 242

- Persistence of the Ductus Arteriosus (PA to the Aorta) - Normally closes in the 1st wk of life. - Associated with Turner Sx - Associated with Ao coarctation, VSD, and TORCH inf Rubella!!) Clinical Features: - Continuous machinery systolic murmur: Gibson Murmur - Small PDA: Asymptomatic - Large PDA: CHF, growth restriction, and FTT.

Answer 243

1. Indomethacin 2. Surgical: Ligation or catheter closure by insertion of a device or embolization coils.

Answer 244

Most common cyanotic CHD 1. Pulmonary stenosis (1st worse) 2. VSD (2nd worse) 3. Overriding Ao 4. Right ventricular hypertrophy Clinical Features: - Cyanosis after the neonatal period (4m approx) and progressive - Clubbing fingers(Scharmoth's sing) - Hypoxemic spells (“Tet spells”) - Systolic ejection murmur - LSB (PS)

Answer 245

Corrective Sx at about 6 months

Answer 246

- LV and aorta are abnormally small (hypoplastic). - Early days (2-7d) of life & need urgent Sx to survive. - HLH is dependent on PDA for survival

Answer 247

The aorta arises from the RV & receives "blue" blood, whilst the Pulmonary Artery arises from the LV. Immediately after birth, and needs urgent treatment. Survival depends on the PDA or the FO remaining open in the early days of life until treatment.

Answer 248

1. The FO can be enlarged with a catheter procedure, called Balloon 2. Septostomy

Answer 249

Hyperoxia Test: ABG after 100% oxygen for 10 minutes. PaO2 will remain low & not rise

Answer 250

TGV: ABG after 100% oxygen for 10 minutes. PaO2 will remain low & not rise ◦ Pulmonary disease (not cyanotic CHD) is suspected if the PaO2 increases to more than 150 mm Hg with oxygen.

Answer 251

Clinical features: Persistent and non-progressive. 1. Delayed motor milestones 2. Unexplained irritability 3. Muscle tone abnormalities: - Stiffness (most common) or Floppy - Preference for one side of the body Differentiate from spinal muscular atrophy (SMA): Intelligence is unaffected

Answer 252

1. Paracetamol 2. NSAIDs 3. Oxycodone 4. IN Fentanyl CI: Codeine<18yo because can cause respiratory depression

Answer 253

o Kawasaki o Rheumatic fever o Juvenile rheumatic arthritis (otherwise, always Paracetamol)

Answer 254

typical facies + hypotonia + single palmar crease Down syndrome (trisomy 21) is based on typical facial features (flat facies, slanting eyes, prominent epicanthic folds, small ears), hypotonia, intellectual disability, and a single palmar crease.

Answer 255

* Seizures (usually later onset) * Impaired hearing * Leukaemia * Hypothyroidism * Congenital anomalies (e.g. heart, duodenal atresia, Hirschsprung, TOF) Alzheimer-like dementia (fourth–fifth decade) * Atlantoaxial instability * Coeliac disease * Diabetes

Answer 256

chromosome 8 Coloboma Heart abnormalities Choanal atresia Development retardation GU anomalies Ear abnormalities

Answer 257

Trisomy 18 * Microcephaly * Facial abnormalities, e.g. cleft lip/palate * Malformations of major organs, e.g. heart * Malformations of hands and feet—clenched hand posture neural tube defect Prognosis is poor—about one-third die in first month, <10% live beyond 12 months. Prenatal diagnosis is available.

Answer 258

Trisomy 13 * Microcephaly * Brain and heart malformation * Cleft lip/palate * Polydactyly * Neural tube defect Prognosis is poor—50% die within first month.

Answer 259

characteristic facies + intellectual disability + large testes Large prominent ears Long narrow face Macro-orchidism Intellectual disability. Most common inherited cause known of developmental disability M:F ratio 2:1 Females may appear normal but may be affected Variable spectrum of characteristic features, making detection difficult

Answer 260

X chromosome Intellectual disability (IQ <70) Autism or autistic-like behaviour Attention deficit in 10% (with or without hyperactivity) Seizures (20%) Connective tissue abnormalities Learning disability and speech delay Coordination difficulty Primary ovarian insufficiency Late-onset tremor/ataxia syndrome Big testis Long Face Strabismus Flat feet Hypotonia

Answer 261

neonatal hypotonia + failure to thrive + obesity (later) chromosome 15 Hypotonic infants with weak suction and failure to thrive, then voracious appetite causing morbid obesity Usually manifests at 3 years Intellectual disability Narrow forehead and turned-down mouth Small hands and feet Hypogonadism

Answer 262

‘elfin’ face + intellectual disability + aortic stenosis chromosome 7 Elfin facial appearance Mild pre- and postnatal growth retardation Mild microcephaly Mild-to-moderate developmental delay. In the first 2 years of life, feeding problems, vomiting, irritability, hyperacusis, constipation and failure to thrive may lead to presentation, but children are rarely diagnosed at this stage.

Answer 263

tall stature + dislocated lens and myopia + aortic root dilatation * Disproportionally tall and thin * Long digits—arachnodactyly * Kyphoscoliosis * Joint laxity (e.g. genu recurvatum) * Myopia and ectopic ocular lens * High arched palate * Aortic dilatation and dissection * Mitral valve prolapse If untreated, death in the 30s and 40s is common Chromosome 15 Autosomal dominant

Answer 264

facies + short stature + pulmonary stenosis chromosome 11 * Characteristic facies—down-slanting palpebral fissures, widespread eyes, low-set ears ± ptosis * Short stature * Pulmonary valve stenosis * Webbed neck * Failure to thrive, usually mild * Abnormalities of cardiac conduction and rhythm ± Intellectual disability

Answer 265

Abnormal chromosome 15 * Hand flapping * ‘Puppet’-like ataxia * Frequent laughter/smiling * Microcephaly by age 2 years * Developmental delay * Speech impairment * Seizures * Cannot live independently Treatment with minocycline is promising.

Answer 266

47, XXY genotype lanky men + small testes + infertility Approximately 2 out of 3 are never recognised * Marked variation but usually: * tall men with long limbs * small firm testes ≤2 cm (10 mL) * infertility (azoospermia) * sparse facial hair * reduced libido * learning difficulties, especially reading * intellectual ability may range from normal to disability * gynaecomastia * increased risk of DVT, breast cancer and diabetes (screening indicated)

Answer 267

Increased gonadotrophin Low to normal testosterone

Answer 268

Transdermal testosterone

Answer 269

short stature + webbed neck + facies * Short stature—average adult height 143 cm * Primary amenorrhoea in XO patient; infertility * Webbing of neck * Typical facies: micrognathia, low hairline * Lymphoedema of extremities * Cardiac defects (e.g. coarctation of aorta) Mental deficiency is rare. 99% of conceptions are miscarried

Answer 270

Hormone-based (e.g. growth hormone, hormone replacement therapy)

Answer 271

45 chromosomes of XO karyotype (**typical Turner** karyotype in 50% of cases) Many are **mosaics** (45X/46XX)

Answer 272

Deficiency of the activity of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT) X-linked recessive Most often affects males * Uric acid levels are abnormally high * Impaired kidney function * Acute gouty arthritis * Self-mutilating behaviors such as lip and finger biting and/or head banging. * Involuntary muscle movements * Neurological impairment

Answer 273

Autosomal dominant Chromosome 19 Intestinal polyposis syndrome

Answer 274

Hamartomatous polyps in the gastrointestinal tract Hyperpigmented macules on the lips and oral mucosa (melanosis) - appear before 5 years of age

Answer 275

Intrussusception Substantial risk of cancer, especially of the breast and gastrointestinal tracts. Colorectal is the most common malignancy. DD: Addison's disease

Answer 276

Barium enema radiograph showing multiple polyps (mostly pedunculated) and at least one large mass at the hepatic flexure coated with contrast in a patient with Peutz–Jeghers syndrome Some suggestions for surveillance for cancer include the following...

Answer 277

Colonoscopy + Biopsy

Answer 278

Small intestine with small bowel radiography every two years Esophagogastroduodenoscopy and colonoscopy every two years CT scan or MRI of the pancreas yearly Ultrasound of the pelvis and testes yearly Mammography from age 25 annually Papanicolaou smear (Pap smear) annually beginning at age 18–20