#Cardiology 🫀

Congenital Heart Diseases

💙Cyanotic Heart Diseases💙
(R -> L), 5 Ts
- Truncus Arteriosus
- Transposition of great vessels
- Tricuspid atresia
- Total anomalous pulmonary vascular return
- Teratology of Fallot TOF:


Tetralogy of Fallot TOF:
1. Rt ventricular ouflow obstruction 🚫
2. Rt ventricular hypertrophy
3. Ventricular septal defect VSD
4. Overriding aorta
• Dx: Xray🩻: Boot 🥾
• Mx of Tet Spells (hypercyanotic, hypoxic episodes):
- O2, Squatting, Sedation
- No improvement -> Fluid, Beta blockers

❤️Acyanotic Heart Diseases❤️
(L -> R)
- ASD
- VSD
- Patent ductus arteriosus PDA
- Coarctation of Aorta CoA

CoA
• Common in turner syndrome
• Dx: Different pulse btw upper and lower limbs
• DDx: Aortic dissection: different pulse btw both arms
• Mx:
<4 mo: Surgical repair
>=4 mo: Balloon angioplasty🎈

AVSD
• Mx:
Initial -> Medical
Definitive -> Surgery at 3-6 mo

• Infants w congenital heart diseases require more nutrition
• Xray:🩻
- Egg on string🥚-> Transposition of great vv
- Boot shape 🥾-> TOF
- Rib notching -> CoA
• Mx of cyanosed neonate👶:
1. ABC
2. Prostaglandin to keep the duct open

Murmurs🩺👶
• VSD: Harsh holosystolic murmur over left sternal border
• ASD: Wide fixed splitting of S2
• Endocardial cushion defect ECD/AVSD: Wider fixed split S2 + apical harsh holosystolic murmur radiating to left axilla
• PDA: Continuous machine-like murmur🚜
• TOF: VSD murmur, Harsh pulmonic stenosis murmur
• Still’s Murmur:
- Most common innocent murmur in children 😇
- Commonly at 3-5 yo
- Change w different positions
- Pt is asymptomatic

Hypertrophic Obstructive Cardiomyopathy HOCM
• Congenital heart disease
• Sx: recurrent exertional chest pain and syncope in youg pt, Ejection systolic murmur
• Mx:
First line > BB
Second > CCB
• Hx of sudden cardiac arrest in young family member while walking🚶‍♂️☠️-> HOCM

HTN
• Diagnosing a child w HTN requires BP readings above 95th percentile

#GI 🍜

Celiac Disease
• Autoimmune disease causing malabsorption
• Sx: Diarrhea, Steatorrhea, Abdominal bloating, N&V, Loss of subcutaneous fat, Dermatological (Dermtitis herpetiformis)
• Dx:
- Serology: IgA Tissue Transglutaminase Antibody (tTG IgA), or Anti-endomysial antibody (2nd line)
- Confirmatory > EGD w Small Intestine Biopsy

Eosinophilic Esophagitis
• Chronic immune-mediated esophageal inflammation
• Associated w Atopy (Asthma, rhinitis, atopic dermtitis)
• Sx: Dysphagia (compensated by excessive chewing), reflux, chest pain
• Dx: Endoscopy & biopsy
• Mx:
- First line: PPIs
- Second: Topical steroids
- Dietary modifications

Sandifer Syndrome
• Movement disorder characterized by torticollis & arching of the spine
• Associated w GERD & Hiatal hernia

Lactose intolerance
• Dx -> Hydrogen breath test

#Neurology 🧠

Febrile Siezure🤒
• Siezure + Fever (>=38) in 6 mo-5 yo
• Simple vs Complex
- Simple (one of the following):
<15 min
Generalized tonic-clonic
No recurrence in 24 hr
- Complex (one of the following):
>15 min
Focal onset
Recur in 24 hr
• Mx:
- If >=5 min: Abortive tx (IV lorazepam)
- Antipyretics
- Reassurance

Rolandic Epilepsy (Benign Epilepsy w Centrotemporal Spikes)
• Most common form of benign epilepsy in children
• Sx: usually during sleep 😴, facial twitching and numbness
• Dx: EEG: Centrotemporal Spikes
• Mx:
- Anticonvulsants are only recommended in high frequency/severity

Infantile Spasms (West Syndrome)
• Sx: Multiple neck flexion followed by neck extension
• EEG: Very heterogeneous, hypsarrhythmia
Tx: ACTH, Steroid

Status Epilepticus
• Mx:
1. IV lorazepam (given again if no response) or IV/Rectal diazepam or Buccal midazolam
2. IV phenytoin (recurrent in ER > Fosphenytoin)
3. General anesthesia
• Absence seizure > Ethosuximide
• Generalized epilepsy > Sodium valporate

Guillian Barre Syndrome GBS
• Sx:
- Acute ascending areflexic paralysis and parasthesia
- Hx of URTI/GI inf (Cambylobacter jeujeni)
• Dx: clinically
• Mx: 1. IVIG or 2. Plasma exchange
• Excellent prognosis w full recovery ❤️‍🩹
• To monitor ventilatory impairment🫁 -> Bedside measurement of vital capacity

Cerebral Palsy
• Heterogeneous group of disorders affecting muscle tone and the development of movement and posture, non-progressive
• RF:
- Birth wt <1.5 kg
- Prematurity⚠️
- TORCH infection
• Red flags
🚩 Definite hand preference before 1 yo
🚩Persistent moro reflex (>6 mo)
• Types:
- Spastic (75%): Can result in hemiplagia (one side of the body), diplegia (both legs 🦵), quadriplegia (all limbs)
- Dyskinetic/Athentoid (Basal ganglia affected, maybe associated w kernicterus)
- Ataxic

Duchenne Muscular Dystrophy
• Type of Progressive Muscular Dystrophies
• X-linked recessive disorder
• Screening: Creatinine kinase
• Sx: Calf pseudohypertrophy, Grower maneuver (pt supports himself on his thighs and uses his hands to walk up his body until he’s standing)

#Hematology 🩸

IDA
• Screening for anemia:
- W/o risk factors -> At 12 mo
- W risk factors (Prematurity, LBW) -> At birth and 4 mo
• Primary causes of IDA in pediatrics -> Dietary issues
• Exclusively breastfeeding 🤱-> Iron supplements should be given from 4 mo
• No cow milk 🐮 in the first year of life
• Physiologic anemia:
- Term: 2-3 mo
- Preterm: 1-2 mo

Sickle Cell Anemia
• AR, Hallmarks in SCD is Vaso-occlusive phenomena, Hemolytic anemia
• Dx:
- Markers of hemolysis: high indirect hyperbilirubinemia, LDH, AST, reticulocyte
- Peripheral boold film: Sickle cell, Howell-Jolly body, Target cell
- Confirm: Hb electrophoresis

• Mx:
- Hydroxyurea: prevent cx (vaso-occlusive crises) by inc HbF
- Vaccines: pneumococcal, meningococcal, Hemophilus influenza type b
- Daily pencillin prophylaxis (until 5 y)

• Cx:

Acute:

Painful episode
- Triggered by: inf, dehydration, cold weather, high altitude
- Site: legs, arms, back
- Mx: Regydration & Pain management, If fever: Abx

Acute Chest Syndrome ACS
- Sx: fever, chest pain, hypoxemia, wheezing, cough, new pulmonary infiltrate
- Mx: broad spectrum Abx, Transfusion

Aplastic crisis
- Arrest of erythropoiesis
- Dx: dec reticulocytes, Acute drop of Hb
- Organism: Parovirus B-19

Splenic Squestration Crisis
- Life-threatening
- Dx: Acute drop of Hb typically two below baseline, reticulocytosis

Chronic:
- Stroke
- Renal impairment & HTN
- Pigmented gallstones

Hereditary Spherocytosis
- AD
- Family hx of gallbladder stone/Splenectomy
- Can presents as neonatal jaundice
Dx:
- Blood film: Spherocytes
- Gold standard: Erosin 5 Malemide (EMA) binding test
- Osmotic fragility test
- To differentiate from AHA: Coombs test (negative)
Tx:
- EPO, iron and folate
- Splenectomy (curative)

Hemophilia (A&B)
• A: Vlll, B: lX, X-linked
• Sx:
- Hemarthrosis (knee)
- Intracranial bleeding
- IM/Retroperitoneal hematoma
- Hematuria, hemospermia
• Dx:
- Prolonged aPTT
- Normal PT, thrombin time, bleeding time

Infantile Vitamin K-Deficient Bleeding
• Sx: presents at 2-7 d of life, easy bruising, umbilical/mucosal/GI bleeding, Intracranial hemorrhage
• Dx: prolonged PT
• Tx:
- IV vit K
- Unstavle -> FFP
• Prevention: IM vit K at birth
• Vit K dependent factors: II, VII, IX, X (2,7,9,10)

Immune Thrombocytopenia Purpra ITP
- Superficial bleeding, can be preceded by viral inf
- Dx: isolated thrombocytopenia (<100,000)
- Tx:
Cutaneous sx only -> Observe
Bleeding -> CS, IVIG, or Anti-D
- Tx in adults:
No bleeding, Plt 50,000-30,000 -> No tx
Minor bleeding, Plt 50,000-30,000 -> CS (prendnisone)
Severe bleeding or Plt less than 10,000 -> IVIG or Anti-Rho (anti-D)
Recurrent/Failed -> Splenectomy

Thrombotic Thrombocytopenic Purpura TTP
• Severe deficiency in ADAMTS13
• Sx: fever, neurological sx
• Dx:
CBC:
- Thrombocytopenia
- Low Hb
- Film: schistocytes (Microangiopathic hemolytic anemia)
Hemolysis:
- High reticulocytes
- High LDH
- Low Haptoglobin
Coagulation profile
- Normal/mildly prolonged PT, aPTT
- Normal/mildly elevated D-dimer
Liver chemistry
- High indirect bilirubin
BMP
- High BUN, Cr
Urinalysis
- Hematuria, proteinuria
• Tx: Plasma Exchange


Hemolytic Uremic Syndrome HUS
• Causes: E. Coli (O157:H7, O104:H4)
• Sx: Renal impairment
• Dx:
- Thrombocytopenia
- Film: schistocytes (Microangiopathic hemolytic anemia)
- AKI: ⬆️BUN and Cr
• Tx:
- Typical HUS > Supportive
- Atypical HUS > Eculizumab (causes fulminant meningococcal inf, screen for vaccin.)
⚠️Abx are C/I -> Injury to bacterial wall releasing large anounts of toxins!

#Nephrology

UTI
• RF:
- Females
- Uncircumcised boys
- Fever >=39
- Nitrate
- Age <12 mo
- Duration >=48 hr
- Vesicouretral reflux
- Constipation
• Sx: Fever, poor feeding, dec urine output
• Tx:
Mild sx (cystitis):
- Oral amoxicillin
- TMP/SMX
- Nitrofurantoin
Unstable UTI or Pyelonephritis:
- Inability to tolerate oral -> IV Ceftriaxone, Cefepime, or Ceftaxime

• Indications for Imaging:

Renal US
- First febrile UTI in <2 yo
- Recurrent febrile UTI
- UTI + Family hx of renal disease, poor growth, or HTN
- Not responsive to Abx

Voiding Cystourethrogram (look for vesicuretral reflux)
- Child <6 mo w recurrent UTI, atypical UTI, or UTI + US showing hydronephrosis
- Child 6 mo - 3 yo w atypical UTI or US showing hydronephrosis

Renal Scintiography (DMSA scan)
- To detect acute pyelonephritis and renal scarring

Glomerular Diseases

Nephrotic vs Nephritic syndrome
Sx:
• Nephrotic: generalized edema and peri-orbital edema
• Nephritic: HTN
Dx:
• Protein in 24 h urine:
- Nephrotic >3.5 gm
- Nephritic <3.5 gm
• Urine cast:
- Nephrotic: Fatty cast🟡
- Nephrtitic: RBCs cast🔴
• Hyperlipidemia and hypoalbuminemia
- Nephrotic: Yes
- Nephritic: No

Diseases in Pediatrics
Nephrotic Syndrome:
Minimal change disease
- Common in children👧
- Tx: steroid
- Confirm remission -> Urine dipstick negative or trace for 3 consecutive days

Nephritic Sybdrome:
Post-streptococcal glomerulonephritis PSGN
- 10 d after the infection
- Has Low C3

IgA nephropathy/Burger’s disease
- Presents within 3 d of URTI🤧

Alport syndrome
- Hearing loss👂❌, ocular :
findings👁️, +family hx of ESRD
- Cause: defect in collagen type 4

Primary Nocturnal Enuresis
• Urinary incontinence in >5 yo
• Caused by destrusor overactivity/instability
• Dx: >2 times per week for 3 mo in >5 yo patient
- Urinalysis to R/O other causes
• Mx:
- Lifestyle changes (minimize fluid intake before bed, void before bed, reward system like gold star chart)
- Enuresis alarm ⏰
- Desmopressin tx
• Bed-wetting is Normal behavior until 6 yo

Prevent progression of Nephrotic Syndrome -> Enalapril

Steroid resistant nephrotic syndrome is the Inability to induce a remission within 4 weeks of daily steroid therapy

#Endocrinology 🍰🍭

T1DM:
• Autoimmune
• Screen for celiac disease at diagnosis, every 2 y
• Screen for microvascular cx: after 5 y
• Tx: Full bolus insulin regimen w once-daily insulin glargine
- Honeymoon period: hypoglycemia attacks when the pt first start insulin

DKA
• Diagnostic Criteria:
- Glucose >200 mg (11.1 mmol)
- pH 7.3
- Ketones in urine or serum
• Mx:
- Fluid & electrolyte tx💦
- Gradual decline in glucose (to minimize the risk of cerebral edema)💦🧠
- Potassium repletion:
Hyperkalemia -> K not given
Normokalemia -> Give K w insulin
Hypokalemia -> Give K before insulin⚠️
- Insulin: regular insulin, continuous infusion of 0.1 U/kg/hr

Normal Puberty
Female: Onset 8-13 yo
1. Thelarche
2. Pubarche
3. Growth spurt
4. Menarche (mean: 12.5 yo)

Male: Onset 9-14 yo
1. Testicular enlargement
2. Penile enlargement
3. Pubarche
4. Growth spurt

Approach to precocious puberty
Early secondary sexual development: (F <8 yo, M <9 yo)

Bone age: 🦴

1. Advanced Bone Age:
• Low basal LH -> GnRH stimulation test:
- Low LH: Peripheral precocious puberty
- High LH: Central precocious puberty (Brain MRI)🧠
• High basal LH: Central precocious puberty (Brain MRI)🧠

2. Normal bone age
• Isolated breast development: Premature thelarche (Pelvic US)
• Isolated pubic hair development: Premature adrenarche (test DHEA) 💉


Nutritional Rickets (Vit D def)
• RF: exclusive breastfeeding 🤱, inadequate sun exposure☀️
• Sx:
- Craniotabes (ping-pong skull)
- Widening of wrists
- Delayed fontanel closure
- Frontal bossing
- Costochondral joints hypertrophy (Rachitic rosary)
- Femoral & tibial bowing
• Dx: Labs and Xray
Labs:
⬇️Ca, P, Vit D
⬆️Alk Phos, PTH
• Mx: Vit D & Ca suppl

Familial Short Stature vs Constitutional Growth Delay
Family hx
Familial: Short stature
Constitutional: Delayed puberty
Bone age
Familial: Normal
Constitutional: Less than chronological age

#Dermatology 🧴

Atopic Dermtitis
• Sx:
- Infants (<2 y): Itchy, red, scaly, crusted lesions on extensor surfaces, trunk, cheeks, and scalp
- Child/adult: lichenified plaques in flexural creases
• Mx: topic emollients, steroids
• Cx:
- Bacterial like S. Aureus (grape-like pattern🍇)
- Viral like HSV/eczema herpticup

Impetigo
Nonbullous
• Pathogen:
1. S. Aureus
2. GAS (S. Pyogenes)
• Sx: Honey-crusted lesions🍯
• A pt w imptigo 1-2 w with low complement -> Post-strept GN
Bullous
• Pathogen: S. Aureus
• Sx: Rapidly enlarging flaccid bullae w yellow fluid🟡
Tx for Impetigo:
- Limited to skin: Topic Abx (Mupirocin)
- Extensive: PO Abx (cephalexin or clindamycin)

Cat Scratch Disease🐈😼
• Pathogen: Bartonella Henselae
• Sx: enlarged LNs
• Mx: Macrolides Abx (Azithromycin)

#Immunology

Wiskott-Aldrich Syndrome WAS
• Genetic condition characterized by impaired T cell function and thrombocytopenia
• Mutation in WAS gene 🧬 (X-linked recessive)
• Common in boys 👦
• Sx: Triad of:
1. Thrombocytopenia
2. Eczema
3. Recurrent bacterial infections
• Dx:
⬆️IgA & IgE

X-linked (Bruton) Agammaglobulinemia
• X-linked recessive disease causes a complete deficiency of mature B lymphocytes 🧬
• Common in boys👦
• Sx: starts at 3-6 mo after materal IgG starts to decline:
- Recurrent severe sinopulmobary and GI infections
- Small/absent lymphoid tissues (Tonils, adenoids, LNs)
• Dx: Low B Cells (CD19)
• Tx: IV immunoglobulins (IVIG)💉

#Rheumatology 🦴

Transient Synovitis vs Septic Arthritis
• Sx:
Transient: Well-appearing, Afebrile
Septic: ill-appearing, febrile🤒, non-wt bearing
• Dx:
Septic: synovial fluid WBCs >=50,000
• Tx
Transient: conservative
Septic: drainage & Abx

Juvenile Idiopathic Arthritis JIA
• Dx:
Persistent arthritis lasting for >6 w
• Females > Males
• All 4 types have negative RF, except in Seropositive Polyarticular JIA
• Uveitis screening:👁️
+ANA: every 3-6 mo (in <7 yo), every 6 mo (in >=7 yo)
-ANA: every 6 mo
Systemic JRA: every 12 mo

• Types:

Oligoarticular JIA (Most common)
• Arthritis: <=4 joints, asymmetrical, large joints
• Extra-articular sx: Anterior uveitis👁️
• Tx: NSAIDs💊

Seronegative polyarticular JIA
• Arthritis: >=5 joints
• Tx: MTX and NSAIDs💊

Seropostive polyarticular JIA
• Arthritis: >=5 joints, symmetrical
• Extra-articular sx: Rheumatoid nodules
• Dx: RF positive
• Tx: MTX and NSAIDs💊

Systemic JIA (Still’s disease)
• Arthritis: >=1 joint AND intermittent fever🤒 AND extra-articular sx
• Extra-articular sx:
- Transient migratory salmon-pink rash🍣
- Generalized lymphadenopathy
• Dx: Anemia, leukocytosis, thrombocytosis


Henoch-Schonlein Purpura HSP
• Most common vasculitis in children👧
• Often has Hx of URTI 1-3 w before sx onset
• Sx:
IgA deposits in small vessels of skin, joints, GI, and kidney
- Palpable purpura (buttocks, LL)
- Arthritis/arthralgia
- Abdominal pain, Intussusception
- Renal disease
• Dx:
Normal Plt
⬆️Cr, Hematuria🩸
• Tx
- Hydration & NSAIDs
- Severe: Systemic CS

Kawasaki Disease
• 90% age <5
• Diagnostic criteria:
Fever >=5 d + >=4 of the following:
- Conjunctivitis (bilateral👁️👁️, nonexudative)
- Mucositis: fissured lips/pharynx, strawberry tongue👅🍓
- Rash (erythematous generalized)
- Erythema and edema of hands/feet✋️🦶
• Tx: Aspirin & IVIG
• Cx: coronary artery aneurysms, MI🫀
• Poor prognostic factors (Kobayashi score):
- Na <=133
- CRP >=10 mg/dL (>=100 mg/L)
- Neutrophils >=80%

Juvenile Dermatomyositis
• Sx: muscle weakness, cutaneous features (Helitrope rash)
• Dx: High CK, ANA

#Toxicology 💊

Toxicity agents & their antidotes:
Paracetamol: N-acetylcystine
TCA: Sodium bicarbonate
Aspirin: Sodium bicarbonate
Narcotics: Naloxone
Iron: Deferoxanine
Lead: D-penicillamine
Copper: D-penicillamine
Organophosphates: Atropine

Acetaminophen Toxicity Phases
Phase 1
• 0.5-24 hours after ingestion
• Sx: asymptomatic or report anorexia, nausea or vomiting, and malaise
• PE: reveal pallor, diaphoresis, malaise, and fatigue

Phase 2
• 18-72 h after ingestion
• Sx: right upper quadrant abdominal pain, anorexia, nausea, and vomiting
• PE: Right upper quadrant tenderness, Tachycardia and hypotension

Phase 3: Hepatic phase
• 72-96 h after ingestion
• Sx: continued nausea and vomiting, abdominal pain, and a tender hepatic edge, jaundice, coagulopathy, hypoglycemia, and hepatic encephalopathy
• Acute kidney injury

Phase 4: Recovery phase
• 4 d to 3 wk after ingestion
• Patients who survive critical illness in phase 3 have complete resolution of symptoms

#Fluid_Management 💦

Indications for IV fluid:
1) Resuscitation
2) Replacement
3) Maintenance

1) Resuscitation (in shock)
10-20 cc/kg over 20-60 min

2) Replacement
- Dehydration % x wt (kg) x 10
- Bolus given to moderate-severe dehydration
^Degree of dehydration:

Mild: 5% (<2 yo), 3% (>2 yo)
Pulse: N
BP: N
Urine output: Decreased
Oral mucosa: Slightly dry
Ant fontanelle: N
Eyes: N
Skin turgor: N
CR: N (<2 s)

Moderate: 10% (<2 yo), 6% (>2 yo)
Pulse: Rapid
BP: N to decreased
Urine output: Markedly Decreased
Oral mucosa: Dry
Ant fontanelle: Sunken
Eyes: Sunken
Skin turgor: Decreased
CR: N to increased

Severe: 15% (<2 yo), 9% (>2 yo):
Pulse: Rapid, weak
BP: Decreased in shock
Urine output: Anuria
Oral mucosa: Parched
Ant fontanelle: Markedly Sunken
Eyes: Markedly Sunken
Skin turgor: tenting
CR: Increased

3) Maintenance
Holliday-Segar Method: 100:50:20 rule (24 hr)
First 10 kg: 100 cc/kg
Second 10 kg: 50 cc/kg
Remaining kilos: 20 cc/kg

Osgood schlatter disease 🦵
• Traction apophysitis
• Age 9-14 yo
• Sx: Tender lump over tibial tuberosity
• Self-limiting

Infantile Colic
• In 3-6 months old babies
• Dx: crying lasts for >=3 hr/d, >=3 d/week, for >=3 weeks
• Mx: behavioral change

Sudden Infant Death Syndrome SIDS
• RF: prematurity, prone sleeping, smoking, family hx of SIDS
• Prevention:
- Sleep in supine position
- Use pacifier during sleep
- Firm mattress, no pillows/blankets/stuffed animals🛏️
- In the first 6 mo: sleeping in the same room w/o bed-sharing
- Smoke-free🚭🙅‍♀️
- Avoid overheating
- Breastfeeding 🤱 until at least 4 mo
- Placing an infant prone while they’re awake

Primary protein-energy malnutrition
Main types:

Marasmus:
- Sx: Muscle wasting, loss of subcutaneous fat, no edema
- Deficiency: All major nutrients

Kwashiorkor:
- Sx: Bilateral pitting edema, distended abdomen, hepatomegaly
- Deficiency: Protein

Infantile Beriberi
• Caused by vitamin B1 deficiency
• Sx: anorexia, edema, aphonia

Scurvy (Vitamin C Deficiency)
• Sx: Follicular hyperkeratosis, gingivial hypertrophy, enlarged bluish purple friable gums

Common cause of vomiting in infants -> UTIs

Maternal phenobarbital consumption causes low vitamin K in fetus -> Prenatal 1 mg vitamin K1

Tanner Stages

☝🏻Beginning of SMLE Notes (Pedia)

☝🏻من هنا أول رسالة في قناة البيديا

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