#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

‏السلام عليكم ورحمة الله وبركاته،
احنا طالب وطالبة من جامعة الملك فيصل الحمدالله حصلنا على درجة 100 في قسم الأطفال في اختبار نوفمبر.

‏حبينا نسوي ‏ملف نجمع فيها الاسئلة اللى جاتنا ونرسلها لكم.

وهذه المبادره بإذن الله راح تستمر لسنوات عديده بحيث كل سنة يمسكون القناة الي جابوا ١٠٠٪؜ بقسم الاطفال.

واي شخص جاب ١٠٠٪؜ بقسم الاطفال، اكتب لنا حسابك بالتلقرام بحيث نجمع اكبر عدد ممكن من الاشخاص الي جابوا ١٠٠٪؜ بقسم الاطفال.

Telegram: @SMLE_pediatric

وبالتوفيق يا رب 🙏

☝🏻كفو والله ❤️‍🔥 أحب الصنف هذا من البشر 🌹🫡

تجربة أحد المختبرين:

بالنسبة للي يسألون عن مصادر المذاكره لقسم الأطفال:
١. ملف د. مهدي المصحح (للاسف فيه كم خطأ مثال سؤال 177 جوابه A + سؤال 204 جوابه B)
٢. MG
٣. توب جيز (اعتمدوا الاجابات الاخيره لان بعض الاسئله يغيرون اجابتهم)


AA: ما فتحت ملفات د. صفدر ابدًا

AH: فتحتها كبدايه مذاكرتي للاختبار في جولاي ولو رجع فيني الزمن ماذكرت ملف د.صفدر لانه دسم وياخذ وقت طويل
الافضل من وجهه نظري مذاكره الاسئله اليوميه بعد ملف مهدي.

المراجعه قبل الاختبار: الاسئله الصعبه الي تحددونها من الاسئله اليوميه + MG + المايلستون والفاكسين تبع شهر اختباركم.

السلام عليكم كتبت بعض النوت بما ان ملف د مهدي الله يجزيه الخير تكرر منه كثير , ممكن يستفيدون منه
لاتنسوني ووالداي من دعواتكم