Summarise all key exam aspects from long answer to short answer amd mcq aspects of sickle cell disease in paediatrics.include patjophysiology as a haematologist expert.Make a ppt
sickle cell disease red blood cells sickling pathophysiology
mkdir -p /home/daytona/workspace/scd-paediatrics && ls /home/daytona/workspace/scd-paediatrics/
~/scd-paediatrics/build_scd_ppt.js
const pptxgen = require("pptxgenjs");
const { execSync } = require("child_process");
// ── Fetch images ──────────────────────────────────────────────────────────────
const imgUrls = [
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"https://cdn.orris.care/cdss_images/HARRISON_1763036071170_b52af39c-ae90-4f8b-9826-2f200ccfcfd6.png",
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let imgs = [];
try {
const raw = execSync(
`node /home/daytona/skills/shared/scripts/fetch_images.js ${imgUrls.map(u => `"${u}"`).join(" ")}`,
{ maxBuffer: 50 * 1024 * 1024 }
).toString();
imgs = JSON.parse(raw);
} catch(e) {
console.warn("Image fetch failed:", e.message);
imgs = imgUrls.map(u => ({ url: u, base64: null, error: e.message }));
}
// ── Color Palette ─────────────────────────────────────────────────────────────
const C = {
bg: "0D1B2A", // deep navy
accent1: "C0392B", // crimson (sickle/blood)
accent2: "E67E22", // amber
accent3: "27AE60", // green
accent4: "2980B9", // blue
white: "FFFFFF",
offwhite: "ECF0F1",
grey: "BDC3C7",
darkcard: "1A2940",
midcard: "14243A",
};
// ── Helpers ───────────────────────────────────────────────────────────────────
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// ─────────────────────────────────────────────────────────────────────────────
const pres = new pptxgen();
pres.layout = "LAYOUT_16x9";
pres.title = "Sickle Cell Disease in Paediatrics";
const TOTAL = 18;
// ══ SLIDE 1 — TITLE ══════════════════════════════════════════════════════════
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const sl = pres.addSlide();
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sl.addText("SICKLE CELL DISEASE", {
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sl.addText("Expert Haematologist Perspective • Pathophysiology to Management", {
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sl.addText("Sources: Tintinalli's Emergency Medicine · Rosen's Emergency Medicine · Henry's Clinical Lab Methods · Swanson's Family Medicine Review", {
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}
// ══ SLIDE 2 — OUTLINE ════════════════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl); hdr(sl, "Lecture Outline", "Key exam themes covered in this deck");
footerSlide(sl, 2, TOTAL);
const topics = [
["01", "Definition, Genetics & Epidemiology", C.accent4],
["02", "Molecular Pathophysiology (HbS mutation → polymerisation)", C.accent1],
["03", "Vaso-occlusion cascade & endothelial dysfunction", C.accent1],
["04", "Clinical Types & Haemoglobin Genotypes", C.accent2],
["05", "Acute Crises — VOC, ACS, Splenic Sequestration, Aplastic, Stroke", C.accent2],
["06", "Chronic Complications (Organ damage)", C.accent3],
["07", "Diagnosis — Newborn Screening, Electrophoresis, Lab Findings", C.accent4],
["08", "Management — Analgesia, Hydroxyurea, Transfusion, HSCT", C.accent3],
["09", "Prevention — Penicillin, Vaccines, TCD Screening", C.accent3],
["10", "MCQ Bank + Short Answer Key Points", C.accent2],
];
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}
// ══ SLIDE 3 — DEFINITION & GENETICS ══════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Definition, Genetics & Epidemiology", "Long Answer — Foundational Concepts");
footerSlide(sl, 3, TOTAL);
card(sl, 0.2, 1.2, 4.65, 2.1, "🔬 Genetic Basis", [
"Autosomal RECESSIVE — both β-globin alleles mutated",
"Point mutation: adenine→thymine in β-globin gene",
"Amino acid change: Glutamic acid → Valine at position 6 of β-chain",
"HbSS (homozygous) = most severe disease; >85% HbS",
"Other genotypes: HbSC, HbS-β⁺thal, HbS-β⁰thal"
], C.accent1);
card(sl, 0.2, 3.4, 4.65, 2.0, "🌍 Epidemiology", [
"~100,000 people in USA affected; 1/365 African-American births",
"1 in 13 African-Americans carries sickle cell trait (HbAS)",
"Also: Mediterranean, Indian, Middle Eastern, Caribbean",
"~250,000 new births/year globally",
"Heterozygous trait (HbAS) confers partial malaria protection"
], C.accent4);
card(sl, 5.15, 1.2, 4.65, 2.1, "🧬 Haemoglobin Types", [
"HbA (α²β²) — normal adult, >95% in adults",
"HbF (α²γ²) — fetal Hb; NO β-globin → NO sickling in newborns",
"HbS (α²βˢ²) — abnormal sickle Hb",
"Symptoms begin as HbF declines (after ~6 months of age)",
"HbA2 (α²δ²) — minor adult component"
], C.accent2);
card(sl, 5.15, 3.4, 4.65, 2.0, "📋 Exam Key Points", [
"Inheritance: autosomal recessive (both parents must carry gene)",
"Trait (HbAS): ~40% HbS, usually asymptomatic",
"MCQ: glutamic acid REPLACED by valine (not lysine, not Hb C)",
"HbF protective — fetal period is symptom-free",
"Screening: neonatal Hb electrophoresis (universal in many countries)"
], C.accent3);
}
// ══ SLIDE 4 — MOLECULAR PATHOPHYSIOLOGY ═════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Molecular Pathophysiology", "Long Answer — The HbS Polymerisation Cascade");
footerSlide(sl, 4, TOTAL);
// Flow diagram: 5 boxes in a cascade
const steps = [
{ label: "Deoxygenation", desc: "HbS exposed to low O₂ tension", col: C.accent1 },
{ label: "HbS Polymerisation", desc: "Hydrophobic Val-6 exposes valine pocket → HbS tetramers aggregate into long rigid fibres (Hb polymer)", col: C.accent1 },
{ label: "RBC Sickling", desc: "Flexible biconcave disc → rigid crescent/sickle cell; membrane damage; irreversible sickling with repeated episodes", col: C.accent2 },
{ label: "Vascular Effects", desc: "↑ blood viscosity, sludging, RBC-endothelial adhesion, microvascular obstruction → tissue ischaemia", col: C.accent2 },
{ label: "Haemolysis", desc: "RBC lifespan 20 days (normal 120 days); chronic anaemia; free Hb scavenges NO → endothelial dysfunction", col: C.accent3 },
];
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}
// ══ SLIDE 5 — VASO-OCCLUSION MECHANISM ══════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Vaso-Occlusion — The Multi-Hit Model", "Long Answer — Beyond Simple Polymerisation");
footerSlide(sl, 5, TOTAL);
card(sl, 0.2, 1.2, 4.6, 3.7, "Cellular Mechanisms of VOC", [
"1. Sickled RBC rigidity & adhesion to endothelium",
"2. Leukocyte (neutrophil) activation & rolling on endothelium",
"3. Activated vascular endothelium → ↑ VCAM-1, ICAM-1, P-selectin",
"4. Altered NO metabolism: Hb scavenges NO → vasoconstriction",
"5. Hypercoagulable state (↑ thrombin generation, microparticles)",
"6. Ischaemia-reperfusion injury: ROS generation → oxidative stress",
"7. Inflammatory cytokines: IL-8, IL-1, TNF-α → NF-κB cascade",
"⚠️ MCQ: VOC involves leukocytes + endothelium + NO + hypercoagulability (NOT hypocoagulability)"
], C.accent1);
if (imgs[0] && imgs[0].base64) {
sl.addImage({ data: imgs[0].base64, x: 5.0, y: 1.2, w: 4.8, h: 3.7 });
sl.addText("HbS polymerisation → vaso-occlusion (SEM)", {
x: 5.0, y: 4.85, w: 4.8, h: 0.25, fontSize: 8, color: C.grey, fontFace: "Calibri", italic: true, align: "center"
});
} else {
card(sl, 5.0, 1.2, 4.8, 3.7, "Precipitating Factors", [
"Dehydration → ↑ HbS concentration → polymerisation",
"Hypoxia → deoxygenation → sickling",
"Acidosis → Bohr effect → O₂ offloading → sickling",
"Cold exposure → vasoconstriction → stasis",
"Infection → systemic inflammatory state",
"Stress / trauma / overexertion"
], C.accent3);
}
}
// ══ SLIDE 6 — HAEMOGLOBIN GENOTYPES TABLE ═══════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Haemoglobin Genotypes & Clinical Severity", "Short Answer / MCQ — Know the Genotypes");
footerSlide(sl, 6, TOTAL);
sl.addTable([
[
{ text: "Genotype", options: { bold: true, color: C.bg, fill: C.accent1, fontSize: 11, fontFace: "Calibri" } },
{ text: "HbS %", options: { bold: true, color: C.bg, fill: C.accent1, fontSize: 11, fontFace: "Calibri" } },
{ text: "Severity", options: { bold: true, color: C.bg, fill: C.accent1, fontSize: 11, fontFace: "Calibri" } },
{ text: "Key Features", options: { bold: true, color: C.bg, fill: C.accent1, fontSize: 11, fontFace: "Calibri" } }
],
[
{ text: "HbSS (Sickle Cell Anaemia)", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } },
{ text: ">85%", options: { color: C.accent1, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Most Severe", options: { color: C.accent1, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Classic disease; all complications", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } }
],
[
{ text: "HbSC", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } },
{ text: "~50% each", options: { color: C.accent2, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Moderate", options: { color: C.accent2, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Retinopathy, avascular necrosis prominent; less anaemia", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } }
],
[
{ text: "HbS-β⁰ thalassaemia", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } },
{ text: ">90%", options: { color: C.accent1, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Severe (like HbSS)", options: { color: C.accent1, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "No HbA produced; clinically = HbSS", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } }
],
[
{ text: "HbS-β⁺ thalassaemia", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } },
{ text: "~70%", options: { color: C.accent2, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Mild-Moderate", options: { color: C.accent2, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Some HbA produced; milder course", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } }
],
[
{ text: "HbAS (Sickle Cell Trait)", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } },
{ text: "~40%", options: { color: C.accent3, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Benign/Carrier", options: { color: C.accent3, bold: true, fontSize: 10, fontFace: "Calibri" } },
{ text: "Usually asymptomatic; haematuria, splenic infarct at altitude", options: { color: C.offwhite, fontSize: 10, fontFace: "Calibri" } }
],
], {
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rowH: 0.6,
border: { type: "solid", color: "2C3E50", pt: 1 }
});
sl.addText("⭐ MCQ TIP: HbS-β⁰ thal is clinically indistinguishable from HbSS. HbAS trait is usually benign.", {
x: 0.25, y: 5.18, w: 9.5, h: 0.25,
fontSize: 9.5, color: C.accent2, bold: true, fontFace: "Calibri", margin: 0
});
}
// ══ SLIDE 7 — ACUTE CRISES OVERVIEW ═════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Acute Crises in Sickle Cell Disease", "Long Answer — Recognise, Diagnose, Treat");
footerSlide(sl, 7, TOTAL);
const crises = [
{ name: "Vaso-Occlusive Crisis (VOC)", col: C.accent1, desc: "Most common; bone/joint/back pain; tissue ischaemia; precipitated by cold, dehydration, infection" },
{ name: "Acute Chest Syndrome (ACS)", col: C.accent1, desc: "New CXR infiltrate + fever/cough/hypoxia/chest pain; leading cause of death; infection, fat embolism, in-situ sickling" },
{ name: "Splenic Sequestration", col: C.accent2, desc: "Sudden trapping of blood in spleen → acute anaemia, ↓ Hb >2g/dL from baseline; massive splenomegaly; haemodynamic collapse; common <5 yrs" },
{ name: "Aplastic Crisis", col: C.accent2, desc: "Parvovirus B19 → transient red cell aplasia; reticulocytopenia; severe anaemia; self-limiting (7-10 days)" },
{ name: "Stroke / CVA", col: C.accent4, desc: "Ischaemic >> haemorrhagic; peak age 2-10 yrs; TCD velocity >200 cm/s = high risk; 11% lifetime risk untreated" },
{ name: "Hyperhemolytic Crisis", col: C.accent3, desc: "Abrupt ↑ haemolysis rate; Hb drops acutely with ↑ reticulocytes; differentiate from aplastic (latter has low reticulocytes)" },
];
crises.forEach((c, i) => {
const col_ = i % 2, row_ = Math.floor(i / 2);
const x = 0.2 + col_ * 4.9, y = 1.22 + row_ * 1.38;
sl.addShape("rect", { x, y, w: 4.7, h: 1.25, fill: { color: C.darkcard }, line: { color: c.col, width: 1.5 } });
sl.addShape("rect", { x, y, w: 4.7, h: 0.36, fill: { color: c.col } });
sl.addText(c.name, { x: x + 0.12, y: y + 0.06, w: 4.46, h: 0.28, fontSize: 11.5, bold: true, color: C.white, fontFace: "Calibri", margin: 0 });
sl.addText(c.desc, { x: x + 0.12, y: y + 0.42, w: 4.46, h: 0.78, fontSize: 9.5, color: C.offwhite, fontFace: "Calibri", valign: "top", margin: 0 });
});
}
// ══ SLIDE 8 — ACS DETAIL ════════════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Acute Chest Syndrome (ACS)", "Long Answer — Management Protocol");
footerSlide(sl, 8, TOTAL);
card(sl, 0.2, 1.2, 4.6, 4.15, "Definition & Diagnosis", [
"NEW pulmonary infiltrate on CXR PLUS ≥1 of:",
" • Fever (>38.5°C)",
" • Respiratory symptoms: cough, wheeze, dyspnoea",
" • Chest/rib/bone pain",
" • SpO₂ drop from baseline",
"Most common cause of death in SCD",
"Occurs in 30% after VOC hospitalisation",
"Causes: infection (Chlamydia, Mycoplasma, RSV), fat/bone marrow embolism, in-situ sickling, pulmonary infarction",
], C.accent1);
card(sl, 5.05, 1.2, 4.75, 2.0, "Investigations", [
"CBC + reticulocyte count (baseline Hb essential)",
"Blood cultures, sputum cultures",
"CXR — new infiltrate required for diagnosis",
"ABG if SpO₂ <95% or severe respiratory distress",
"Cross-match — if transfusion likely",
"Bronchoalveolar lavage: fat-laden macrophages (fat embolism)"
], C.accent2);
card(sl, 5.05, 3.28, 4.75, 2.07, "Treatment (Mnemonic: OAHATE)", [
"O — Oxygen (titrate to SpO₂ ≥95%)",
"A — Analgesics (opioids; incentive spirometry mandatory)",
"H — Hydration: D5 0.45%NaCl ×1–1.5 maintenance",
"A — Antibiotics: cover atypicals (azithromycin ± ceftriaxone)",
"T — Transfusion: simple (Hb <6) or exchange (severe/worsening)",
"E — Exchange transfusion if: prior ventilation, multiorgan failure, neurological signs, no improvement"
], C.accent3);
}
// ══ SLIDE 9 — SPLENIC SEQUESTRATION & APLASTIC ══════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Splenic Sequestration & Aplastic Crisis", "Short Answer / MCQ — Age-Specific Crises");
footerSlide(sl, 9, TOTAL);
card(sl, 0.2, 1.22, 4.65, 3.6, "Splenic Sequestration Crisis", [
"Pathogenesis: Sickled cells pool in splenic sinusoids → sudden ↓ circulating Hb",
"Definition: Hb falls >2 g/dL from baseline + splenomegaly",
"Age: Most common <5 years (before autosplenectomy)",
"Clinical: Lethargy, pallor, LUQ mass/pain, shock, tachycardia",
"Hb may fall to 1–3 g/dL; haemodynamic collapse possible",
"Reticulocytes: ELEVATED (distinguishes from aplastic crisis)",
"Treatment: Urgent IV fluids, simple transfusion (cautious — avoid over-transfusion)",
"Recurrence: 50% → consider splenectomy",
"Prevention: Parents taught spleen palpation at home"
], C.accent1);
card(sl, 5.15, 1.22, 4.65, 3.6, "Aplastic Crisis", [
"Pathogenesis: Parvovirus B19 infects erythroid progenitors → transient arrest of RBC production",
"Duration: Self-limited 7–10 days (bone marrow recovers)",
"Clinical: Severe anaemia, fatigue, pallor; fever +/−",
"KEY DIFFERENTIATOR: Reticulocytes LOW/ABSENT (vs. high in sequestration)",
"Platelet/WBC count typically preserved initially",
"Diagnosis: Parvovirus B19 IgM or PCR; bone marrow shows absent erythroblasts",
"Treatment: RBC transfusion (may require repeated); supportive care",
"Isolation: Parvovirus B19 infectious — avoid pregnant contacts",
"⭐ MCQ: Aplastic crisis = Parvo B19 + LOW reticulocytes"
], C.accent2);
}
// ══ SLIDE 10 — STROKE & NEUROLOGICAL ════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Cerebrovascular Disease & Stroke", "Long Answer — TCD Screening Protocol");
footerSlide(sl, 10, TOTAL);
card(sl, 0.2, 1.22, 4.65, 3.9, "Stroke in SCD", [
"11% of HbSS children have stroke before age 20 (untreated)",
"Ischaemic > haemorrhagic (ischaemic in younger children)",
"Mechanism: Large vessel vasculopathy (internal carotid, MCA) → thrombosis",
"Moyamoya: 30% of cerebral vasculopathy in paediatric SCD",
"Presentation: Focal neurological deficit, seizure, altered consciousness",
"Imaging: MRI + MRA preferred (may also use CT acutely)",
"Acute treatment: Emergency exchange transfusion (target HbS <30%)",
"Secondary prevention: Chronic transfusion programme to keep HbS <30%",
"Hx of stroke → keep HbS <30% lifelong"
], C.accent4);
card(sl, 5.15, 1.22, 4.65, 1.9, "TCD Screening", [
"Transcranial Doppler from age 2–16 years, annually",
"Time-averaged mean velocity (TAMV) >200 cm/s = HIGH RISK",
"TAMV 170–199 cm/s = CONDITIONAL (repeat in 3–6 months)",
"High risk → prophylactic chronic transfusion reduces stroke risk by 90%",
"⭐ TCD is a KEY paediatric intervention (STOP trial)"
], C.accent3);
card(sl, 5.15, 3.25, 4.65, 1.9, "Silent Cerebral Infarcts (SCI)", [
"MRI lesions WITHOUT clinical stroke symptoms",
"Present in 20–35% of children with HbSS",
"Associated with cognitive impairment, school difficulties",
"Diagnosed on MRI brain (routine screening recommended)",
"Management: hydroxyurea; consider transfusion if progressive"
], C.accent2);
}
// ══ SLIDE 11 — CHRONIC COMPLICATIONS ════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Chronic Organ Complications", "Long Answer — Systematic Review");
footerSlide(sl, 11, TOTAL);
const organs = [
{ organ: "Spleen", col: C.accent1, points: ["Autosplenectomy by age 5–8 years (repeated infarction)", "Functional asplenia → encapsulated organisms (Strep pneumo, H. influenzae, Neisseria)", "↑ Risk: sepsis, meningitis"] },
{ organ: "Lung", col: C.accent2, points: ["Recurrent ACS → restrictive lung disease", "Pulmonary hypertension (TRV >2.5 m/s)", "SpO₂ monitoring; annual echo for PH screening"] },
{ organ: "Brain", col: C.accent4, points: ["Overt stroke (11%), Silent cerebral infarcts (20–35%)", "Cognitive impairment, learning disabilities", "Moyamoya syndrome"] },
{ organ: "Bone", col: C.accent3, points: ["Avascular necrosis: femoral/humeral heads", "Dactylitis (hand-foot syndrome) — first crisis in infants", "Osteomyelitis (Salmonella, Staph aureus)"] },
{ organ: "Kidney", col: C.accent2, points: ["Sickle nephropathy: papillary necrosis, proteinuria, haematuria", "Hyposthenuria (inability to concentrate urine) — early sign", "CKD in adults; glomerulosclerosis"] },
{ organ: "Eye", col: C.accent3, points: ["Proliferative retinopathy (especially HbSC)", "Annual ophthalmology screening", "Can cause retinal detachment and blindness"] },
];
organs.forEach((o, i) => {
const col_ = i % 3, row_ = Math.floor(i / 3);
const x = 0.2 + col_ * 3.25, y = 1.22 + row_ * 2.05;
sl.addShape("rect", { x, y, w: 3.1, h: 1.88, fill: { color: C.darkcard }, line: { color: o.col, width: 1.4 } });
sl.addShape("rect", { x, y, w: 3.1, h: 0.35, fill: { color: o.col } });
sl.addText(o.organ, { x: x + 0.1, y: y + 0.06, w: 2.9, h: 0.26, fontSize: 12, bold: true, color: C.white, fontFace: "Calibri", margin: 0 });
const items = o.points.map((p, pi) => ({
text: p, options: { bullet: { type: "bullet" }, color: C.offwhite, fontSize: 9, fontFace: "Calibri", breakLine: pi < o.points.length - 1 }
}));
sl.addText(items, { x: x + 0.08, y: y + 0.4, w: 2.94, h: 1.42, valign: "top", margin: 0 });
});
}
// ══ SLIDE 12 — DIAGNOSIS ════════════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Diagnosis & Laboratory Findings", "Short Answer — Know Your Lab Values");
footerSlide(sl, 12, TOTAL);
card(sl, 0.2, 1.22, 4.65, 4.1, "Neonatal Screening & Confirmatory Tests", [
"Neonatal screening: Haemoglobin ELECTROPHORESIS (HPLC) — gold standard",
"Pattern HbSS: FS (Fetal + Sickle bands; no A band)",
"Pattern HbSC: FSC",
"Pattern HbAS (trait): FAS",
"Confirmatory: repeat electrophoresis at 3–6 months",
"Sickling test (Sickledex/sodium metabisulfite): cheap screening but does NOT distinguish SS from AS",
"MRI brain: silent cerebral infarcts",
"TCD ultrasonography: stroke risk stratification",
"Echo: pulmonary hypertension screening",
"Urine: haematuria, hyposthenuria (inability to concentrate)",
], C.accent4);
card(sl, 5.15, 1.22, 4.65, 2.0, "Peripheral Blood Film", [
"Sickle cells (drepanocytes) — crescent/boat shapes",
"Target cells (HbSC, S-thal)",
"Polychromasia — ↑ reticulocytes (compensatory haematopoiesis)",
"Howell-Jolly bodies — evidence of functional asplenia",
"Nucleated RBCs in severe anaemia",
"⭐ Howell-Jolly bodies = functional asplenia (MCQ favourite)"
], C.accent2);
card(sl, 5.15, 3.28, 4.65, 2.1, "Routine Labs in SCD", [
"Hb: 6–9 g/dL (baseline in HbSS)",
"MCV: Normal or mildly ↓ (macrocytosis if on hydroxyurea)",
"WBC: Elevated (leukocytosis common — marker of inflammation)",
"Reticulocytes: 5–15% (elevated baseline)",
"Bilirubin: ↑ unconjugated (haemolysis)",
"LDH: ↑ (marker of haemolysis intensity)",
"Urinalysis: haematuria, proteinuria, low SG"
], C.accent3);
}
// ══ SLIDE 13 — MANAGEMENT OVERVIEW ═════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Management — Comprehensive Overview", "Long Answer — Acute + Chronic + Curative");
footerSlide(sl, 13, TOTAL);
card(sl, 0.2, 1.22, 3.05, 4.1, "Acute VOC Management", [
"Hydration: oral preferred; IV D5 0.45% NS ×1–1.5 maintenance",
"Analgesia: WHO ladder",
" • Mild: paracetamol + NSAID (ketorolac)",
" • Moderate-Severe: opioids (morphine)",
" • PCA morphine for inpatients",
"Oxygen ONLY if SpO₂ <95%",
"Incentive spirometry (prevents ACS)",
"Treat precipitating infection",
"Avoid over-hydration (risk of ACS)",
"Monitor resp rate hourly on opioids"
], C.accent1);
card(sl, 3.5, 1.22, 3.05, 4.1, "Hydroxyurea (HU)", [
"Mechanism: ↑ HbF → inhibits HbS polymerisation",
"Also: ↓ leukocytes, ↓ RBC adhesion, ↑ RBC hydration",
"Indications (paeds): ≥9 months all HbSS/HbS-β⁰thal (2014 ASH)",
"BABY HUG trial: HU in infants → ↓ VOC, ACS, dactylitis",
"Dose: 20 mg/kg/day, titrate to response (max ~35 mg/kg)",
"Monitor: CBC every 2–4 weeks initially (myelosuppression)",
"Side effects: neutropenia, thrombocytopenia, teratogenic",
"⭐ HU is disease-modifying — reduces mortality"
], C.accent3);
card(sl, 6.8, 1.22, 3.0, 4.1, "Transfusion Therapy", [
"Simple transfusion: ACS, splenic sequestration, aplastic crisis, preoperative",
"Chronic transfusion: stroke prevention (TCD >200 cm/s), recurrent ACS",
"Target: Hb ≤10 g/dL; HbS <30%",
"Exchange transfusion: acute stroke, severe ACS, multiorgan failure",
"Complications: alloimmunisation, iron overload (treat with deferasirox), transfusion reactions, infections",
"Iron chelation: deferasirox (oral) or desferrioxamine (SC) when ferritin >1000",
"Asymptomatic: do NOT transfuse for Hb level alone"
], C.accent2);
}
// ══ SLIDE 14 — PREVENTION & PROPHYLAXIS ══════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Prevention, Prophylaxis & Long-Term Care", "Short Answer — Paediatric Surveillance");
footerSlide(sl, 14, TOTAL);
card(sl, 0.2, 1.22, 4.65, 1.72, "Antibiotic Prophylaxis", [
"Penicillin V: start at 2–3 months (before spleen dysfunction complete)",
"Dose: 125 mg BD (<3 yrs), 250 mg BD (≥3 yrs)",
"Continue until at least age 5 years",
"Penicillin-allergic: erythromycin",
"⭐ Prevents pneumococcal septicaemia — most important early intervention"
], C.accent1);
card(sl, 0.2, 3.0, 4.65, 2.3, "Vaccination Schedule", [
"Pneumococcal: PCV-13 (routine childhood) + PPSV-23 (from age 2)",
"Haemophilus influenzae type b (Hib)",
"Meningococcal: MenACWY + MenB",
"Annual influenza vaccine",
"Hepatitis B (blood product exposure)",
"⭐ MCQ: Vaccinate against ENCAPSULATED organisms (Pneumococcus, Hib, Meningococcus)"
], C.accent2);
card(sl, 5.15, 1.22, 4.65, 1.72, "TCD & Stroke Prevention", [
"TCD screening: Annual from age 2–16 years",
"High risk (>200 cm/s) → chronic transfusion reduces stroke by 90%",
"Conditional (170–199) → repeat in 3–6 months",
"STOP & STOP 2 trials established TCD-based protocol",
"Hydroxyurea now shown non-inferior to transfusion (TWiTCH trial)"
], C.accent4);
card(sl, 5.15, 3.0, 4.65, 2.3, "Curative & Novel Therapies", [
"HSCT (Haematopoietic Stem Cell Transplant): only current CURE",
"Requires HLA-matched sibling donor; survival >90%",
"Gene therapy: lentiviral HbF-inducing vectors (FDA-approved 2023: Casgevy — CRISPR)",
"Voxelotor: HbS anti-polymerisation agent (increases O₂ affinity)",
"Crizanlizumab: anti-P-selectin antibody (reduces VOC frequency)",
"L-glutamine: reduces oxidative stress; FDA-approved supplement"
], C.accent3);
}
// ══ SLIDE 15 — DACTYLITIS & INFECTION ════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Dactylitis, Infection & Osteomyelitis", "Short Answer / MCQ — Infant Presentations");
footerSlide(sl, 15, TOTAL);
card(sl, 0.2, 1.22, 4.65, 3.8, "Dactylitis (Hand-Foot Syndrome)", [
"FIRST clinical manifestation of SCD in infants (age 6–24 months)",
"Cause: Vaso-occlusion in small bones of hands/feet → infarction",
"Presentation: Painful, non-pitting swelling of hands/feet; fever",
"Bilateral and symmetric common",
"Resolves spontaneously in 1–2 weeks",
"Differential: osteomyelitis (persistent fever, elevated WBC, CRP)",
"Treatment: analgesia, hydration, treat fever",
"⭐ Dactylitis = first crisis in infant → always test for SCD in infant with hand/foot swelling + fever",
"Radiograph: normal initially; periosteal reaction later"
], C.accent2);
card(sl, 5.15, 1.22, 4.65, 3.8, "Infection & Sepsis", [
"Functional asplenia → encapsulated organism susceptibility",
"Most feared: Streptococcus pneumoniae septicaemia (can be fulminant)",
"Also: Haemophilus influenzae, Neisseria meningitidis, Salmonella",
"FEVER in SCD child: MEDICAL EMERGENCY until proven otherwise",
" • Temp ≥38.5°C → blood culture + empiric IV antibiotics",
" • Ceftriaxone (covers pneumococcus + Gram-negatives)",
"Osteomyelitis: Salmonella spp. (most specific to SCD), Staph aureus",
"Differentiate AVN from osteomyelitis: MRI, bone scan, WBC, CRP",
"⭐ MCQ: Salmonella osteomyelitis = pathognomonic association with SCD"
], C.accent1);
}
// ══ SLIDE 16 — MCQ BANK ══════════════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "MCQ Bank — High-Yield Questions", "Exam Drill — Cover the answers first!");
footerSlide(sl, 16, TOTAL);
const mcqs = [
{ q: "Q1. Molecular basis of HbS?", a: "A. Glu→Val substitution at β-globin position 6", col: C.accent1 },
{ q: "Q2. First crisis in infant with SCD?", a: "A. Dactylitis (hand-foot syndrome)", col: C.accent2 },
{ q: "Q3. Aplastic crisis pathogen?", a: "A. Parvovirus B19 (low/absent reticulocytes)", col: C.accent3 },
{ q: "Q4. TCD cut-off for stroke high-risk?", a: "A. ≥200 cm/s TAMV → start chronic transfusion", col: C.accent4 },
{ q: "Q5. Most specific osteomyelitis organism in SCD?", a: "A. Salmonella spp.", col: C.accent1 },
{ q: "Q6. Blood film finding of functional asplenia?", a: "A. Howell-Jolly bodies", col: C.accent2 },
{ q: "Q7. Mechanism of hydroxyurea?", a: "A. ↑ HbF production → inhibits HbS polymerisation", col: C.accent3 },
{ q: "Q8. Key differentiator: splenic sequestration vs aplastic?", a: "A. Sequestration: HIGH reticulocytes; Aplastic: LOW reticulocytes", col: C.accent4 },
];
mcqs.forEach((m, i) => {
const col_ = i % 2, row_ = Math.floor(i / 2);
const x = 0.2 + col_ * 4.9, y = 1.22 + row_ * 1.04;
sl.addShape("rect", { x, y, w: 4.7, h: 0.95, fill: { color: C.darkcard }, line: { color: m.col, width: 1 } });
sl.addText(m.q, { x: x + 0.12, y: y + 0.07, w: 4.46, h: 0.3, fontSize: 10.5, bold: true, color: m.col, fontFace: "Calibri", margin: 0 });
sl.addText(m.a, { x: x + 0.12, y: y + 0.42, w: 4.46, h: 0.46, fontSize: 10, color: C.offwhite, fontFace: "Calibri", margin: 0 });
});
}
// ══ SLIDE 17 — SHORT ANSWER KEYS ════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
hdr(sl, "Short Answer Key Points", "Quick-Recall Summary for SAQ Questions");
footerSlide(sl, 17, TOTAL);
card(sl, 0.2, 1.22, 4.65, 4.15, "Pathophysiology SAQ", [
"1. β-globin point mutation: adenine→thymine → Glu→Val (position 6)",
"2. Deoxygenation triggers HbS polymerisation into rigid fibres",
"3. Sickled RBC lifespan: ~20 days (normal: 120 days) → haemolytic anaemia",
"4. VOC involves: sickled RBCs + leukocytes + endothelium + ↓NO + hypercoagulability",
"5. HbF is protective — no β-globin chains, does not sickle",
"6. Autosplenectomy by age 5–8: repeated splenic infarction → functional asplenia",
"7. Free Hb from haemolysis scavenges NO → ↑ vasoconstriction, platelet aggregation",
"8. Ischaemia-reperfusion injury → reactive oxygen species → endothelial damage"
], C.accent1);
card(sl, 5.15, 1.22, 4.65, 4.15, "Clinical & Management SAQ", [
"1. Neonatal screening: HPLC/Hb electrophoresis — FS pattern = HbSS",
"2. Penicillin prophylaxis from 2–3 months of age, continue to age 5+",
"3. Fever in SCD = emergency: blood culture + IV ceftriaxone stat",
"4. VOC management: analgesia (opioids) + hydration + treat precipitant",
"5. ACS treatment: O₂, antibiotics, incentive spirometry, +/− transfusion",
"6. Hydroxyurea: ↑ HbF — 1st-line disease-modifying therapy",
"7. Transfusion target: Hb ≤10 g/dL, HbS <30%",
"8. Curative: HLA-matched sibling HSCT; gene therapy (Casgevy/CRISPR)",
"9. TCD >200 cm/s → 90% stroke reduction with chronic transfusion programme"
], C.accent3);
}
// ══ SLIDE 18 — SUMMARY ═══════════════════════════════════════════════════════
{
const sl = pres.addSlide(); bgFill(sl);
sl.addShape("rect", { x: 0, y: 0, w: 10, h: 1.1, fill: { color: C.accent1 } });
sl.addShape("rect", { x: 0, y: 0, w: 0.18, h: 1.1, fill: { color: C.accent2 } });
sl.addText("EXAM SUMMARY — 10 MUST-KNOW FACTS", {
x: 0.3, y: 0.2, w: 9.4, h: 0.7,
fontSize: 24, bold: true, color: C.white, fontFace: "Calibri", charSpacing: 2, margin: 0
});
const facts = [
["1", "Mutation: Glu→Val at β-globin position 6 (adenine→thymine DNA substitution)", C.accent1],
["2", "HbF is protective; symptoms begin as HbF falls after 6 months of age", C.accent2],
["3", "Autosplenectomy by age 5 → encapsulated organisms → penicillin prophylaxis + vaccines", C.accent1],
["4", "Dactylitis = FIRST crisis in infants (6–24 months); Parvovirus B19 = aplastic crisis", C.accent2],
["5", "Aplastic crisis: LOW reticulocytes; Splenic sequestration: HIGH reticulocytes", C.accent3],
["6", "ACS: NEW CXR infiltrate + fever/resp symptoms = most common cause of death", C.accent4],
["7", "TCD >200 cm/s → high stroke risk → chronic transfusion → 90% stroke reduction", C.accent4],
["8", "Hydroxyurea: ↑ HbF; BABY HUG trial → recommend from 9 months in all HbSS", C.accent3],
["9", "Howell-Jolly bodies = functional asplenia; Salmonella osteomyelitis = SCD-specific", C.accent2],
["10","VOC: multi-hit model (sickled RBCs + leukocytes + ↓NO + hypercoag + ischaemia-reperfusion)", C.accent1],
];
facts.forEach(([num, text, col], i) => {
const row_ = Math.floor(i / 2), col_ = i % 2;
const x = 0.2 + col_ * 4.9, y = 1.22 + row_ * 0.84;
sl.addShape("rect", { x, y, w: 4.7, h: 0.72, fill: { color: C.darkcard }, line: { color: col, width: 1 } });
sl.addShape("rect", { x, y, w: 0.42, h: 0.72, fill: { color: col } });
sl.addText(num, { x, y, w: 0.42, h: 0.72, fontSize: 16, bold: true, color: C.white, fontFace: "Calibri", align: "center", valign: "middle", margin: 0 });
sl.addText(text, { x: x + 0.5, y: y + 0.1, w: 4.16, h: 0.54, fontSize: 9.8, color: C.offwhite, fontFace: "Calibri", valign: "middle", margin: 0 });
});
footerSlide(sl, 18, TOTAL);
}
// ── Write File ────────────────────────────────────────────────────────────────
pres.writeFile({ fileName: "/home/daytona/workspace/scd-paediatrics/SickleCell_Paediatrics_Exam_Review.pptx" })
.then(() => console.log("✅ PPT saved successfully"))
.catch(e => { console.error("❌ Error:", e); process.exit(1); });
cd /home/daytona/workspace/scd-paediatrics && node build_scd_ppt.js 2>&1