NLE Paediatric Nursing — Paediatric GI, Genitourinary & Neurologic DisordersStudy Notes

Infants and young children are far more vulnerable to fluid imbalance than adults due to several anatomical and physiological differences. Understanding these principles is essential for managing vomiting, diarrhoea, and renal disease in children.

Why Infants Are Vulnerable to Fluid Loss:

• Higher percentage of total body water (70–75% in infants vs. 60% in adults)
• Greater body-surface-area-to-mass ratio (higher insensible losses)
• Higher metabolic rate (increased fluid turnover)
• Immature kidneys with limited concentrating ability
• Inability to communicate thirst or regulate their own fluid intake

The 4-2-1 Rule: Calculating Maintenance Fluids (Holliday-Segar)
The 4-2-1 formula is used to calculate daily maintenance fluid requirements and is tested on the NLE:

• First 10 kg: 100 mL/kg/day = (10 kg × 100) = 1000 mL/day
• Next 10 kg (11–20 kg): 50 mL/kg/day = (additional kg × 50) mL/day
• Each kg above 20 kg: 20 mL/kg/day

Example: A 7-year-old child weighing 20 kg:

• First 10 kg: 10 × 100 = 1000 mL
• Next 10 kg: 10 × 50 = 500 mL
• Total daily maintenance: 1500 mL/day or approximately 63 mL/hour

For a 5 kg infant (common in paediatric GI disorders):

• 5 kg × 100 = 500 mL/day or approximately 21 mL/hour

Assessing Hydration Status in Infants & Children:
Because infants cannot communicate thirst and compensatory mechanisms are immature, nurses must use objective indicators:

Most Reliable Indicators (in order of sensitivity):

1. Daily weight (most sensitive)—loss of 5% = mild dehydration, 10% = moderate, >15% = severe
2. Urine output: aim for 1–2 mL/kg/hour (e.g., a 5 kg infant should produce 5–10 mL/hour)
3. Fontanelle tension (anterior fontanelle in infants): normally soft and flat; bulges with ↑ICP or dehydration from fluid loss may cause depression
4. Mucous membranes: dry or sticky indicates dehydration
5. Skin turgor: pinched skin returns slowly in dehydration
6. Capillary refill: should be <2 seconds; delayed in hypovolaemia

Clinical Pearl for NLE: In the Philippines where many paediatric patients present late with dehydration (due to healthcare access challenges), nurses in primary care settings must quickly assess these signs and refer appropriately. Weight is your most sensitive tool—always compare to the last recorded weight.

Fluid Deficit Replacement:
When a child is dehydrated, you must replace the deficit plus ongoing losses plus maintenance fluid:

Deficit fluid = % dehydration × body weight (in kg) × 10

Example: A 10 kg child with 10% dehydration (moderate):
Deficit = 10 × 10 × 10 = 1000 mL must be replaced

Then add daily maintenance (1000 mL for 10 kg) plus ongoing losses from vomiting/diarrhoea. This is critical in pyloric stenosis and Hirschsprung disease where fluid loss is significant.

Three conditions dominate the paediatric GI section of the NLE: pyloric stenosis, Hirschsprung disease, and intussusception. Each has a recognisable age of onset, clinical presentation, and critical nursing priority.

Pathophysiology:
The pyloric sphincter muscle undergoes hypertrophy (thickening and enlargement), creating a mechanical obstruction at the gastric outlet (pylorus). Food cannot pass from the stomach into the duodenum, so it accumulates and causes vomiting.

Epidemiology & Age of Presentation:

• Age: 2–8 weeks (classically 3–5 weeks)—this timing is very specific and frequently tested
• Gender: 4:1 ratio, males > females (especially firstborn males)
• Race: Caucasians > other groups (less common in African and Asian populations, but still occurs in the Philippines)
• May be familial (10–15% family history)

Classic Clinical Presentation (Know This for NLE):

1. Projectile vomiting: vomiting occurs forcefully with such velocity that it may project across a room; typically occurs shortly after feeding
2. NON-bilious vomiting: this is crucial—the obstruction is above the duodenum (before the bile duct enters), so bile is not present; vomitus is just gastric contents (milk/formula and stomach acid)
3. Hungry infant syndrome: the infant appears hungry immediately after vomiting and eagerly takes another feed—characteristic of this disorder
4. Visible peristaltic waves: you may observe waves of peristalsis moving left-to-right across the epigastrium as the stomach attempts to push food past the obstruction
5. Olive-shaped mass: palpation of the right upper quadrant (below the costal margin, medial to midclavicular line) may reveal a firm, olive-sized mass (the hypertrophied pylorus); best felt after vomiting when the stomach is empty
6. Weight loss and failure to thrive: persistent vomiting means no calories are retained

Fluid & Electrolyte Consequence (Critical for NLE):
Repeat loss of gastric acid (HCl) leads to a specific acid-base disorder:

• Hypochloremic (low chloride), hypokalemic (low potassium), metabolic ALKALOSIS
• Serum pH is ↑, HCO3− is ↑, Cl− is ↓, K+ is ↓
• Paradoxically, a child with persistent vomiting can become alkalotic (not acidotic as many students initially think)
• Clinical Pearl: Contraction of blood volume → kidneys reabsorb Na+ and H+ to compensate → paradoxical aciduria (acidic urine despite alkalemic blood)

Diagnosis:

• Ultrasound is the gold standard in most centres—shows a pyloric channel >3 mm (normal ~1.5 mm) and gastric wall thickness
• X-ray (barium series) may show the "string sign" (narrow pyloric channel) and retained gastric fluid

Nursing Management & Critical Do's/Don'ts:

Before Surgery (Pre-operative):

• PRIORITY 1: Correct fluid and electrolyte IMBALANCE BEFORE surgery—this is a medical emergency. Do not rush to OR until child is euvolemic
• IV rehydration: typically isotonic saline (0.9% NaCl) with added potassium (usually 20 mEq/L) once urine output is confirmed
• NPO (nothing by mouth) after diagnosis; allow ice chips for comfort
• Insert nasogastric tube to decompress the stomach and prevent aspiration
• Monitor weight, I&O, daily weights, urine output (should improve to 1–2 mL/kg/hr once fluids begin)
• Monitor serum electrolytes (especially K+, Cl−, pH) until corrected
• Manage vomiting: position upright, have suction available, offer mouth care (child experiences hunger)

Post-operative (After Pyloromyotomy/Fredet-Ramstedt Surgery):

• Position: upright, on the right side after feeds (promotes gastric emptying by gravity)
• Feed advancement: begin with small, frequent feeds of clear liquids (5–10 mL), then advance to formula/breast milk as tolerated (usually can resume full feeds within 24–48 hours post-op)
• DO NOT rush feeding—feed small amounts frequently (e.g., 15 mL every 2 hours, advancing by 5–10 mL per feed)
• Monitor surgical incision for redness, swelling, drainage, separation
• Watch for complications: paralytic ileus (rare), infection
• Pain management and gentle handling

Prognosis: Excellent with early surgical correction; full recovery expected.

Pathophysiology:
During fetal development, neural crest cells migrate from the proximal to distal colon to form the enteric (intrinsic) nervous system. In Hirschsprung disease, this migration stops prematurely, leaving a segment of the distal colon WITHOUT ganglion (nerve) cells. This aganglionic segment has no parasympathetic innervation and cannot relax or perform peristalsis, creating a functional obstruction. Stool accumulates proximal to the obstruction, and the proximal colon dilates (megacolon).

Epidemiology:

• Incidence: 1 in 5000 live births in developed countries (may vary in Philippines)
• Gender: 4:1 ratio, males > females
• Length involved: 80% have short-segment disease (only rectosigmoid), 15% long-segment, 5% total colonic aganglionosis
• Associated conditions: Down syndrome (10–15%), familial clustering

Classic Clinical Presentation:

Neonatal Presentation (Most Common—Appears in First 24–48 Hours of Life):

• Failure to pass meconium within 24–48 hours (most specific neonatal sign)—normal newborns pass meconium within 12–24 hours
• Abdominal distension (increasing girth)
• Bilious vomiting (because the obstruction is in the colon, stool backs up and bile is present)
• Signs of enterocolitis (if not recognised early): explosive diarrhoea, fever, lethargy, septic appearance—this is a medical emergency
• May appear to improve briefly when passed a rectal tube (the tube relieves the pressure)

Infancy & Childhood Presentation (If Not Caught in Neonatal Period):

• Chronic constipation (severe, difficult to relieve)
• Ribbon-like or narrow, foul-smelling stools
• Abdominal distension and enterocolitis episodes (fever, explosive diarrhoea, severe cramping)
• Failure to thrive (poor growth, malnutrition)
• Anal fissures from straining

Diagnosis (Gold Standard):

• Rectal biopsy: definitive test—shows absence of ganglion cells in the submucosa and muscularis; you will see normal nerve plexuses above the transition zone and none below it
• Barium enema (contrast enema): shows the transition zone (where the narrow aganglionic segment meets the dilated proximal colon); may see a "funnel" or "cone" appearance; often the first diagnostic test ordered due to cost and availability in the Philippines
• Anorectal manometry: absent relaxation of the internal anal sphincter

Nursing Management & Critical Priorities:

Pre-operative (Acute Management):

• NPO (nothing by mouth) to rest the bowel
• Nasogastric tube for decompression
• IV fluids and electrolyte replacement (similar to pyloric stenosis—vomiting and bowel obstruction cause significant loss)
• Do NOT give enemas or laxatives (causes perforation risk in megacolon)

• Monitor for enterocolitis (fever, explosive diarrhoea, shock, lethargy)—this is LIFE-THREATENING:

  • If enterocolitis develops: rectal tube placement for decompression, IV antibiotics (broad-spectrum), aggressive fluid resuscitation, possible surgery for perforation
  • Enterocolitis is the leading cause of death in untreated Hirschsprung disease

Surgical Management:

• Initial surgery: often a temporary colostomy proximal to the aganglionic segment (to divert stool and allow inflammation to resolve)
• Definitive surgery: pull-through procedure (usually Soave or Duhamel technique) in which the proximal, normal (ganglionic) bowel is pulled down and anastomosed to the rectum, bypassing the aganglionic segment

Post-operative Care:

• Colostomy care (if colostomy placed): skin care around stoma, pouching, monitoring output
• Position: after pull-through, position on right side to promote bowel motility
• Diet advancement: gradual, starting with clear liquids, advancing to soft, then regular diet
• Monitor for stricture formation (post-op complication): if child cannot defecate or has bilious vomiting weeks/months post-op, stricture may have formed
• Monitor for enterocolitis (can still occur post-op): fever, explosive diarrhoea, abdominal distension

Long-term Outlook:

• With early diagnosis and surgery, prognosis is good
• Many children achieve continence with bowel training
• Risk of enterocolitis persists even after surgery (though reduced)

NLE Focus: The NLE loves testing recognition of:

• Failure to pass meconium within 24–48 hours as the key neonatal sign
• Rectal biopsy as the DEFINITIVE diagnostic test
• Enterocolitis as a life-threatening complication requiring urgent intervention
• Why you must NOT give enemas/laxatives (perforation risk)

Pathophysiology:
One segment of bowel (usually the terminal ileum) telescopes or invaginates into the segment immediately distal to it (usually the colon)—like one end of a telescope sliding into another. This causes obstruction and, if not reduced, ischemia and necrosis of the invaginated segment. The mesentery of the invaginated loop is also trapped, compressing blood vessels.

Epidemiology & Age:

• Peak incidence: 5–9 months of age (range 3 months to 3 years)
• Gender: 1.5–2:1 male predominance
• Classic scenario: a previously healthy infant (unlike Hirschsprung or pyloric stenosis, which have earlier or more insidious onset)
• Often preceded by a viral illness (may cause lymphoid hyperplasia that serves as the lead point)

Types (Important for Pathophysiology):

• Ileocolic (75%): ileum prolapses into the colon—most common, worst prognosis if not reduced
• Ileoileal (15%)
• Colocolic (10%)

Classic Clinical Presentation (Know This Triad for NLE):

The Classic Triad:

1. Sudden, severe, colicky abdominal pain (infant draws knees to chest, screams inconsolably for 15–20 seconds, then quiets and appears well between episodes—classic intermittent pattern)
2. Palpable "sausage-shaped" mass (usually in the right upper quadrant or midepigastrium)—from the invaginated bowel loop
3. "Currant-jelly" stools (blood-stained, mucoid stools resembling currant jelly)—a LATE sign indicating mucosal ischemia; not present in early cases

Additional Features:

• Infant appears well between episodes (unlike peritonitis where pain is constant)
• Vomiting (often bilious)
• Palpable abdominal mass on exam (if bowel has not started to reduce)
• If untreated: abdominal distension, shock, fever (peritonitis/perforation)

Diagnosis & Treatment (Unique Feature: Both Diagnostic AND Therapeutic):

• Air (pneumatic) enema or hydrostatic (barium/ultrasound-guided) enema: The enema is used to confirm the diagnosis (shows the "target" or "doughnut" sign on X-ray—invaginated bowel) AND attempt to reduce the intussusception mechanically
• Success rate: 60–90% (varies by technique and timing—earlier reduction has better success)
• Contraindications to enema reduction: signs of perforation (free air on X-ray, peritonitis), shock that doesn't respond to fluids

Clinical Pearl for Nurses:

• Passage of a normal brown stool signals successful reduction—the invaginated segment has prolapsed back into normal position. This is an important sign to report immediately.
• Many cases reduce spontaneously with enema procedure

Nursing Management & Critical Priorities:

Pre-reduction (Acute Management):

• NPO (nothing by mouth) until intussusception ruled out
• IV access and fluids (rehydrate if dehydrated)
• Nasogastric tube if vomiting is severe
• Pain management (after diagnosis confirmed, pain relief aids in reduction)
• Prepare for enema procedure: explain to parents, ensure bladder is empty, have resuscitation equipment available
• Have surgical equipment ready in case enema fails or perforation occurs

During Enema Procedure:

• Gentle technique (high pressures increase perforation risk)
• Fluoroscopy guidance (optimal, but not always available in provincial centres in the Philippines)
• Monitor vital signs continuously
• Watch for signs of perforation: sudden severe pain, free air on X-ray, cardiovascular instability

After Successful Reduction:

• Observe for 4–6 hours for signs of re-intussusception (colicky pain returns, sausage mass reappears)
• Begin feeds cautiously (clear liquids, then advance)
• Discharge with strict follow-up and parent education: return immediately if colicky pain returns
• Recurrence rate: ~10% (most occur within 24–48 hours)

If Enema Fails or Perforation Occurs:

• Emergency surgical reduction or resection of necrotic bowel
• Position: pre-op same as for other GI obstructions (upright, minimal handling)

Long-term Prognosis:

• Excellent if reduced early (within 24 hours)
• Mortality is rare with modern management; mainly from delayed presentation in rural/underserved areas

NLE Focus:

• Recognition of the classic intermittent colicky pain pattern (child is well between episodes)
• The currant-jelly stool is a LATE sign (many exams ask: what's the early sign? Answer: colicky pain and palpable mass)
• Enema is both diagnostic AND therapeutic (a unique feature—other obstructions require surgery)
• Know the target sign and doughnut sign on X-ray
• Passage of normal brown stool = successful reduction

Pathophysiology:
Wilms tumour is an embryonal (paediatric) renal malignancy arising from primitive metanephric tissue. It is the most common renal cancer in childhood. The tumour is usually confined to the kidney by a pseudocapsule, but it can invade locally, metastasize to the contralateral kidney (4–5%), lungs (80% of metastases), liver, and bone.

Epidemiology:

• Age: Peak diagnosis 2–3 years (almost always diagnosed before age 7)
• Gender: slight male predominance
• Incidence: 1 in 10,000 live births
• Genetic associations: WAGR syndrome (Wilms, Aniridia, Genitourinary abnormalities, intellectual disability), Beckwith-Wiedemann syndrome, Denys-Drash syndrome
• Unilateral (90%), bilateral (10%) — bilateral disease requires more conservative treatment (avoid complete nephrectomy if possible)

Classic Clinical Presentation:

1. Firm, non-tender abdominal mass (often discovered by a parent during bathing or dressing; the child is usually asymptomatic)
2. Mass is usually on one side and does NOT cross the midline (important distinguishing feature from neuroblastoma, which often crosses midline)
3. Haematuria (15–20% of cases)—may be gross or microscopic; often the reason for referral
4. Hypertension (25% of cases)—from mass effect on renal vessels or production of renin
5. Abdominal distension/pain (if large)
6. Fever, weight loss (if advanced)
7. Respiratory symptoms (if lung metastases)

Diagnosis:

• Abdominal ultrasound or CT: first-line imaging (shows the renal mass, cystic vs. solid, involvement of surrounding structures)
• Chest X-ray or CT: to assess for lung metastases (most common site of spread)
• Lab: CBC (anaemia common), renal function, urinalysis

CRITICAL NURSING RULE—DO NOT PALPATE THE ABDOMEN:

• The tumour is encapsulated but fragile
• Palpation can rupture the capsule and spill tumour cells into the abdomen, upstaging the disease and worsening prognosis
• Post a sign at the bedside: "DO NOT PALPATE ABDOMEN"
• Handle the child gently; no vigorous bathing or manipulation
• If abdominal exam is necessary (e.g., by the surgeon), it is done very gently and minimally

Staging (TNM-based, modified by the Children's Oncology Group):

• Stage I: tumour confined to the kidney
• Stage II: tumour extends beyond the kidney but is completely resected
• Stage III: local residual tumour (cannot be completely resected)
• Stage IV: hematogenous metastases (lung, liver, bone, distant nodes) or positive lymph nodes outside the abdomen
• Stage V: bilateral renal involvement at diagnosis

Management (Multimodal):

Pre-operative:

• Do NOT palpate; handle gently
• Obtain baseline labs (CBC, renal function, urinalysis)
• Assess for associated syndromes (vision screening for aniridia, genitourinary anomalies)
• Preparation for surgery and possibly neoadjuvant chemotherapy (depending on stage/protocol)

Surgical:

• Radical nephrectomy (removal of the kidney, tumour, surrounding fascia, and ipsilateral adrenal gland)—usually within 24–48 hours of diagnosis
• For bilateral disease: attempt nephron-sparing approach if possible
• Careful handling to avoid capsule rupture

Adjuvant Therapy (Post-operative):

• Chemotherapy: all children (stages I–V) typically receive chemotherapy (regimens vary by stage; Stage I may receive less toxic therapy)

  • Common agents: dactinomycin, doxorubicin, vincristine
  • Course: weeks to months depending on stage
• Radiation therapy: for stages III, IV, V (to abdomen and/or metastatic sites)

Post-operative Nursing Care:

• Monitor surgical incision (retroperitoneal incision, so deep; watch for infection, bleeding)
• Monitor urine output and colour (haematuria may decrease post-op, or new-onset haematuria signals complication)
• Monitor blood pressure (can decrease post-op if renal artery was involved; conversely, hypertension may persist from remaining renal tissue)
• Side effects of chemotherapy: nausea, vomiting, alopecia, risk of infection, secondary malignancy (later in life)
• Emotional support: parents often shocked by cancer diagnosis in a young, previously healthy child

Prognosis:

• Overall survival: 90% at 5 years (with current multimodal treatment)
• Better prognosis for early-stage, unilateral, favourable histology
• Worse for Stage IV, bilateral, or unfavourable histology
• Secondary cancers (from radiation/chemotherapy) are a long-term concern in survivors

NLE High-Yield Points:

• Classic presentation: young child (2–3 years) with firm, one-sided, NON-crossing-midline mass
• DO NOT PALPATE—capsule rupture risks disease progression
• Surgery within 24–48 hours + chemotherapy ± radiation
• All children receive chemotherapy (even Stage I)
• Excellent prognosis with early diagnosis (90% 5-year survival)

Paediatric renal diseases are categorized by the kidney structures involved (glomerular vs. tubular), the mechanism (immune-mediated vs. structural), and the clinical presentation. Three conditions are high-yield for the NLE: nephrotic syndrome, acute glomerulonephritis, and UTI/vesicoureteral reflux.

Pathophysiology:
Nephrotic syndrome results from increased glomerular permeability to protein, most commonly from minimal change disease (MCD) in children. The glomerular basement membrane loses its selectivity and allows large-molecular-weight proteins (especially albumin) to pass into the urine.

The Classic Tetrad of Nephrotic Syndrome:

1. Massive PROTEINURIA (>3.5 g/day in adults; >40 mg/kg/day in children, often >100 mg/kg/day)
2. HYPOALBUMINEMIA (low serum albumin, typically <2.5 g/dL; normal ~3.5–5.5 g/dL)
3. GENERALIZED EDEMA (from loss of oncotic pressure due to hypoalbuminemia)
4. HYPERLIPIDEMIA (elevated cholesterol and triglycerides; mechanism not fully understood)

Epidemiology:

• Age: peak 2–7 years (most common glomerular disease in children)
• Gender: 2:1 male > female
• Race: varies (Caucasians and Asians more common in some studies)
• Minimal change disease (MCD): 90% of cases in children—excellent prognosis
• Other causes (focal segmental glomerulosclerosis, membranoproliferative GN, membranous GN): 10%, worse prognosis

Classic Clinical Presentation:

Onset (Often Acute Over Days to Weeks):

1. Periorbital edema (around the eyes, worse in the morning upon waking—classic sign)
2. Progressive generalized edema (dependent areas: ankles, sacrum if bedridden, scrotum/labia)
3. Ascites (fluid in peritoneal cavity; abdomen appears distended)
4. Weight gain (from fluid retention, may be rapid—5–10 kg over days)
5. Frothy urine (proteinuria causes foaming; parents may report this)
6. Respiratory symptoms (if pulmonary edema develops)
7. Anorexia, abdominal pain (from ascites stretching the peritoneum)

Vital Signs & Hydration Status (Key Differences from Other Glomerular Disease):

• Blood pressure: NORMAL or LOW (unlike acute glomerulonephritis, where it's elevated)
• Urine output: reduced or normal (not markedly oliguria unless very severe)
• The child is edematous but not hypertensive—a key distinguishing feature

Diagnosis:

• Urinalysis: massive proteinuria (3+ or 4+, or semiquantitative >500 mg/dL), urine sediment is typically CLEAN (no RBCs, WBCs, casts)—this is important to note because it suggests glomerular proteinuria without glomerulonephritis
• 24-hour urine protein: quantifies the degree of proteinuria (>40 mg/kg/day in children = nephrotic range)
• Serum albumin, BUN, creatinine, lipid panel, complement levels (C3, C4)
• Renal biopsy: typically NOT done in children with classic MCD (because 90% respond to steroids); reserved for atypical presentations, frequent relapses, or steroid resistance

Management (Corticosteroid-Based in Most Cases):

First-Line Therapy—Corticosteroids (Prednisone):

• Majority of children (~90%) respond to steroids and achieve complete remission (no proteinuria, normal BP, resolution of edema)
• Typical regimen: prednisone 60 mg/m²/day (or 2 mg/kg/day, max 80 mg/day) for 4–6 weeks, then taper over 4–6 weeks
• Response: proteinuria usually disappears within 3–4 weeks; edema resolves more slowly
• Steroid-resistant cases (10%): require additional agents (cyclophosphamide, tacrolimus, rituximab)

Dietary Management:

• Normal protein intake during remission (some restrict protein during acute nephrosis, but normal intake is preferred)
• LOW-SODIUM diet during edema phase (restrict Na+ to <2 g/day) to reduce fluid retention
• Fluid restriction during acute edema (some centres restrict to 1000–1200 mL/day, others use a less stringent approach)
• Gradual return to normal diet as edema resolves and prednisone is tapered

Monitoring & Nursing Care:

1. Daily weight (most sensitive indicator of fluid balance)—weigh at the same time each day, ideally in the morning before breakfast
2. Abdominal girth measurement (circumference at midaxillary line at level of umbilicus) to assess ascites
3. Intake and output (I&O)—measure all fluid intake (IV, oral, feeds) and output (urine, stool, emesis, drains)
4. Urine protein assessment (dipstick each void, or 24-hour quantification)
5. Blood pressure monitoring (daily; should normalize with treatment)
6. Serum electrolytes (especially K+, Na+; monitor during diuresis phase—can develop electrolyte imbalances)
7. Skin care for edematous skin (at high risk for breakdown; use water-based lotion, reposition frequently, avoid pressure)

Prevention of Infection (CRITICAL—These Children Are Immunosuppressed):

• Increased susceptibility to infection: due to loss of immunoglobulins in urine + immunosuppression from corticosteroids and mycophenolate
• Risk of spontaneous bacterial peritonitis (SBP) if ascites is present—fever, abdominal pain, change in ascites character → suspect SBP → paracentesis for diagnosis → antibiotics
• UTI risk: ensure proper hygiene, encourage frequent voiding, maintain perineal dryness
• Respiratory infection risk: teach cough/deep breathing, limit visitors if ill, consider prophylactic antibiotics during high-risk periods (influenza season)
• Avoid live vaccines during immunosuppressive therapy

Complications During Acute Nephrosis:

• Hypovolemia (despite edema): the oedema is "third-space" fluid; intravascular volume may be depleted → risk of acute kidney injury (AKI)

  • Monitor for signs: tachycardia, hypotension, oliguria, elevated BUN/creatinine
  • May require IV albumin (25%) if hypovolaemia is severe or AKI develops
• Hypertension (from sodium retention and fluid overload; treated with sodium restriction and diuretics)
• Pulmonary edema (if severe fluid overload)
• Thromboembolism (hypercoagulable state from loss of anticoagulant proteins; rare but serious)

Relapse & Long-term Outlook:

• Frequent relapses (50% of children): may relapse multiple times over years; each relapse treated with steroids
• Steroid-dependent: some children relapse whenever steroids are tapered; may require steroid-sparing agents
• Long-term prognosis: excellent in MCD; normal renal function in majority even with multiple relapses
• Complications of prolonged steroid therapy: growth retardation, osteoporosis, increased infection risk

NLE Focus:

• Distinguish nephrotic syndrome (normal/low BP, massive proteinuria, severe edema) from acute glomerulonephritis (hematuria, hypertension, mild edema)
• Steroids are first-line therapy; 90% of children respond
• Daily weight is the most sensitive monitoring tool
• Infection prevention is critical (immunosuppression + immunoglobulin loss)
• These children are edematous but can be hypovolaemic intravascularly—a paradox

Pathophysiology:
Acute glomerulonephritis (most commonly post-streptococcal) is an immune-complex inflammatory disease of the glomeruli. Antigen-antibody complexes deposit in the glomerular basement membrane, activate complement, and trigger inflammation, leading to haematuria, proteinuria, and reduced glomerular filtration rate (GFR).

Epidemiology & Timing:

• Age: school-age children (6–12 years most common)
• Season: winter (respiratory strains) or summer (skin strains, pyoderma)

• Onset: typically 1–2 weeks after a streptococcal infection (Group A β-haemolytic streptococcus)

  • Throat infection (pharyngitis): latency ~10 days
  • Skin infection (impetigo, pyoderma): latency ~3 weeks
• Risk factors: untreated strep infection, poor hygiene, crowded living conditions (relevant in the Philippines where strep skin infections are common in tropical climate and limited water access)

Classic Clinical Presentation:

The Classic Tetrad (Different from Nephrotic Syndrome):

1. GROSS HEMATURIA (dark, cola-coloured or tea-coloured urine—a striking feature parents report immediately)
2. HYPERTENSION (often the first sign; systolic/diastolic elevated for age; risk of hypertensive encephalopathy if severe)
3. Mild periorbital edema (present but not the massive edema of nephrotic syndrome)
4. Oliguria (reduced urine output, though not always as severe as post-acute kidney injury)

Associated Symptoms:

• Malaise, fatigue
• Mild fever (not always present)
• Anorexia, nausea, vomiting
• Abdominal or flank discomfort
• Headache (may indicate hypertensive encephalopathy if severe)

Contrast with Nephrotic Syndrome (NLE Loves This Distinction):

Diagnosis:

• Urinalysis: brown, tea-coloured urine; gross hematuria (3+ or 4+ RBCs); protein (usually 1+ to 2+, not massive); RBC casts, WBCs, WBC casts (indicates glomerular inflammation)
• 24-hour urine protein: typically <1 g/day (unlike nephrotic syndrome)
• Serum creatinine, BUN: may be mildly elevated (reflects reduced GFR from glomerular damage)

• Streptococcal serology:

  • ASO titre (antistreptolysin O titre): elevation confirms recent strep infection (>320 Todd units suggests recent infection)
  • Anti-DNase B: alternative marker of recent strep
  • Throat/skin culture: may be positive if infection is ongoing
• Complement levels (C3): typically low (consumed by immune complex deposition); usually normalizes by 6–8 weeks
• Renal biopsy: rarely needed for diagnosis in typical post-streptococcal cases; used if atypical presentation or suspected membranoproliferative GN

Management (Largely Supportive):

Treatment of Hypertension (PRIORITY):

• Strict sodium restriction (restrict to <1 g/day during acute phase) to reduce blood volume and BP
• Fluid restriction (often to 600–800 mL/day during oliguria)
• Diuretics (furosemide 1 mg/kg/dose, 1–2 times daily) if hypertension persists or pulmonary edema develops
• Antihypertensive agents (rarely needed; if BP is severely elevated—e.g., >95th percentile by >20 mmHg—calcium channel blockers or ACE inhibitors may be used; never give ACE inhibitors if creatinine is >2 mg/dL due to risk of further GFR decline)

Monitoring for Hypertensive Emergency (CRITICAL):

• Hypertensive encephalopathy signs: severe headache, altered mental status, seizures, visual changes, pulmonary edema
• Response: urgent antihypertensive therapy (IV agents such as nicardipine, labetalol) and possible ICU monitoring

Infection Treatment:

• Penicillin course (if strep still present or to prevent other infections): penicillin V orally or penicillin G IM
• For penicillin-allergic: erythromycin or first-generation cephalosporin
• Rheumatic fever prophylaxis: if prior history of rheumatic fever, long-term penicillin prophylaxis is needed

Diet & Fluids During Acute Phase:

• Low-sodium diet (supports BP control)
• Fluid restriction (during oliguria; liberalize as urine output improves)
• Protein: normal intake (once acute phase resolves); during oliguria, may restrict slightly to reduce nitrogenous waste

Monitoring & Nursing Care:

1. Daily weight and vital signs (especially BP—measure standing and sitting, and compare to baseline for age)
2. Strict I&O (every void; note colour of urine—changes from cola to lighter brown indicates improvement)
3. Urine dipstick (daily; protein and blood should gradually decrease over weeks)
4. Serum electrolytes, BUN, creatinine (baseline, then at 1–2 weeks; should improve)
5. C3 levels (should normalize by 6–8 weeks; persistent low C3 suggests other forms of GN)
6. Streptococcal serology (to confirm recent strep)
7. Monitor for complications: hypertensive encephalopathy, acute kidney injury, pulmonary edema, heart failure

Activity Restriction:

• Rest during acute phase (2–4 weeks depending on severity)
• Gradual return to activity as haematuria resolves and BP normalizes
• Avoid strenuous activity until urinalysis is normal

Prognosis & Long-term Outlook:

• Excellent in most cases: >95% of children recover completely with normal renal function
• Haematuria gradually resolves over weeks to months (may take 6–12 months for microscopic hematuria to clear)
• Hypertension usually resolves within 1–2 weeks as GFR improves
• Proteinuria typically becomes negative within 2–3 weeks
• Chronic sequelae: very rare in children; some may develop chronic kidney disease if severe acute injury or multiple episodes occur

NLE Focus:

• Distinguish acute GN (hematuria + hypertension + mild edema) from nephrotic syndrome (proteinuria + severe edema + normal BP)
• Post-streptococcal AGN is the most common form in children; latency 1–2 weeks after strep infection
• Priority management: control hypertension (sodium/fluid restriction, diuretics, antihypertensives)
• Risk of hypertensive encephalopathy if BP is severely elevated
• ASO titre and anti-DNase B confirm recent strep infection
• C3 (complement) is low; normalizes by 6–8 weeks
• Self-limited; >95% recovery with good prognosis

Pathophysiology:
Urinary tract infections in children can involve the lower tract (cystitis—bladder infection) or upper tract (pyelonephritis—kidney infection). Vesicoureteral reflux (VUR)—abnormal backward flow of urine from the bladder into the ureters—is a major risk factor for recurrent UTIs and potential renal scarring.

Epidemiology:

• Incidence: 1–3% of children (varies by population)
• Gender: girls > boys (especially after infancy), due to shorter urethra and proximity of the meatus to the anus
• Neonatal period & infancy: boys > girls (especially uncircumcised males)
• Vesicoureteral reflux: present in 30–50% of children with febrile UTI; familial in some cases

Classic Presentation Varies by Age:

Neonates & Infants (<2 Years):
Signs are non-specific and subtle (often why UTIs are missed):

• Fever (often the only sign)
• Poor feeding, irritability
• Vomiting, diarrhoea
• Jaundice (UTI can cause hyperbilirubinaemia in neonates)
• Foul-smelling urine (if diapers are checked carefully)
• Failure to thrive
• Abdominal pain or distension
• No dysuria because the infant cannot communicate

Older Infants & Young Children (2–5 Years):

• Dysuria (pain/crying with urination)
• Frequency, urgency
• Foul-smelling urine
• Fever (hallmark of UTI in children)
• Suprapubic or abdominal pain
• Enuresis (new-onset bedwetting) or daytime frequency
• Irritability, malaise

School-Age Children:

• Dysuria, frequency, urgency
• Suprapubic pain
• Fever (if pyelonephritis)
• Flank pain (pyelonephritis)

Diagnosis:

Urine Specimen Collection (CRITICAL—Improper specimen = false positive/negative):

• Gold standard: Straight catheterization or suprapubic aspiration (eliminates contamination from perineal flora)

• Clean-catch midstream (acceptable if properly done):

  • Wash perineum with soap/water; dry
  • Begin voiding; discard initial stream
  • Collect midstream in sterile container
  • This method is prone to contamination if not done carefully—common source of false positives in the Philippines where education on collection technique may be variable
• Avoid: diaper bags, bottles, catheterized specimens not properly collected (these are highly contaminated)

Urinalysis:

• Nitrites: positive (indicates gram-negative organisms like E. coli; absent in some gram-positive infections)
• Leukocyte esterase: positive (indicates WBCs; suggests infection or inflammation)
• Pyuria (WBCs in urine): 5–10 WBCs per high-power field suggests infection
• Bacteriuria: presence of bacteria on Gram stain
• Casts: may indicate pyelonephritis if WBC casts present

Urine Culture (Most Definitive):

• Gold standard: >100,000 CFU/mL (colony-forming units) from a properly collected specimen indicates UTI
• >50,000 CFU/mL may be significant if symptoms present
• Culture allows identification of organism and antibiotic sensitivities
• Takes 24–48 hours; start treatment based on urinalysis + clinical suspicion while awaiting results

Other Tests:

• CBC: elevated WBC count (if pyelonephritis or systemic infection)
• BUN, creatinine: assess renal function; elevated if severe pyelonephritis or obstruction
• Renal ultrasound: screen for anatomical abnormalities (hydronephrosis, duplicate collecting system, posterior urethral valves)
• Voiding cystourethrogram (VCUG): diagnoses vesicoureteral reflux; done after the acute infection is treated
• Radionuclide imaging (DMSA scan): detects renal scarring; typically done 3–6 months after acute pyelonephritis

Management:

Antibiotic Therapy (Adjust Based on Culture Results):

Empirical Choice (While Awaiting Culture):

• Oral (for cystitis or mild pyelonephritis, non-toxic child):

  • Cephalexin 25 mg/kg/day divided QID (first-line due to good UTI penetration and low resistance in most regions)
  • Trimethoprim-sulfamethoxazole (TMP-SMX) 6–12 mg/kg/day of TMP component (if susceptibilities known)
  • Cefixime 8 mg/kg/day divided BID (good for gram-negatives)
  • Fluoroquinolone (e.g., ciprofloxacin): avoided in children due to cartilage toxicity concerns, but increasingly used in serious cases

• IV (for pyelonephritis, febrile, ill-appearing child, or unable to tolerate oral):

  • Ceftriaxone 50–80 mg/kg/day divided BID (broad-spectrum coverage)
  • Gentamicin 2.5 mg/kg/dose TID IV (good gram-negative coverage, but requires monitoring of drug levels; nephrotoxicity risk)
  • Ampicillin + gentamicin (for infants <3 months to cover group B strep)

Duration:

• Cystitis (lower UTI): 3–5 days of antibiotics (some guidelines allow 2–3 days if lower tract)
• Pyelonephritis (upper UTI): 7–14 days depending on severity and response

Fever Management:

• Acetaminophen or ibuprofen for comfort and fever reduction
• Hydration: encourage fluid intake to promote urine flow and dilution (reduces bacterial concentration)

Monitoring:

• Urine culture at follow-up (24–48 hours): should show sterilization if antibiotics are effective
• Repeat urinalysis after treatment completion: should be normal
• Renal ultrasound: ordered to exclude anatomical abnormalities, especially in boys (any UTI in a boy warrants imaging), infants, and those with recurrent UTIs

Prevention & Health Teaching (Critical Component of Nursing Role):

Behaviour & Hygiene Modifications:

1. Increased fluid intake (water, dilute fruit juice)—promotes urine flow and bladder emptying

2. Regular voiding habits:

   • Void every 2–3 hours (don't hold urine; full bladder increases infection risk)
   • Complete bladder emptying ("double void": void, wait a minute, void again)
   • Void before bedtime and after intercourse (in adolescents)

3. Perineal hygiene:

   • Girls: wipe front-to-back after toilet use (critical—back-to-front contamination is a major risk)
   • Wash hands after toilet
   • Avoid constipation (hard stool may obstruct the urethra)

4. Clothing:

   • Cotton underwear (absorbs moisture; nylon traps moisture and promotes bacterial growth)
   • Avoid tight pants/diapers

5. Avoid irritants:

   • Bubble baths (can irritate the urethra and bladder; avoid)
   • Perfumed soaps or douches
   • Chlorinated pools (less proven, but some recommend caution)

Vesicoureteral Reflux (VUR):

Pathophysiology:
Normally, a valve mechanism at the ureterovesical junction prevents backward flow of urine from the bladder to the ureters. In VUR, this valve is incompetent, allowing urine to reflux into the ureters and kidneys. This increases the risk of:

• Recurrent pyelonephritis
• Renal scarring (leading cause of hypertension and chronic kidney disease in children)
• Hydronephrosis (dilated ureters/renal pelvis)

Diagnosis:

• Voiding cystourethrogram (VCUG): definitive test; performed after acute infection is treated (to avoid introducing infection during the study); shows contrast refluxing into ureters during micturition

Grading (Modified International Reflux Study Committee Grading):

• Grade I: reflux into the ureters only
• Grade II: reflux into the ureters, renal pelvis, and calyces (no dilation)
• Grade III: mild dilation of the above
• Grade IV: moderate dilation
• Grade V: severe dilation with tortuous ureters

Management:

• Low-grade VUR (I–III): often managed conservatively with prophylactic antibiotics (low-dose TMP-SMX or nitrofurantoin at night) + monitoring for breakthrough UTIs; many resolve spontaneously with growth
• High-grade VUR (IV–V): may require ureteral reimplantation surgery to prevent recurrent pyelonephritis and renal scarring

Follow-up:

• Repeat VCUG: typically at 12–18 months to assess for resolution or persistence; may repeat annually
• Renal ultrasound: assess for scarring or hydroureteronephrosis
• Radionuclide imaging (DMSA): detects scarring; done 3–6 months after pyelonephritis

Prognosis:

• Most children with VUR resolve reflux by adolescence (spontaneous resolution in ~80%)
• Risk of renal scarring is highest in those with recurrent pyelonephritis or high-grade reflux

NLE Focus:

• Non-specific signs of UTI in infants (fever, poor feeding, irritability); high index of suspicion needed
• Proper urine collection (straight catheterization or careful clean-catch) is essential; diaper specimens are contaminated
• Girls: wipe front-to-back; boys: any UTI warrants imaging
• Antibiotic choice depends on organism sensitivities; empirical therapy based on local resistance patterns
• Recurrent UTIs or any UTI in a boy → renal ultrasound and VCUG
• Prevention through hygiene, frequent voiding, increased fluids, and cotton underwear

Paediatric neurologic emergencies and developmental disorders dominate the NLE examination. This section covers hydrocephalus, cerebral palsy, bacterial meningitis, spina bifida, and seizure disorders. Each requires rapid assessment, specific nursing interventions, and understanding of critical complications.

Pathophysiology:
Hydrocephalus is an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain resulting from one of three mechanisms:

1. Overproduction of CSF (rare; choroid plexus papilloma)
2. Obstruction to CSF flow (most common; e.g., stenosis of the aqueduct of Sylvius, posterior fossa tumour)
3. Impaired absorption of CSF (e.g., arachnoid adhesions from meningitis, subarachnoid haemorrhage)

The accumulation increases intracranial pressure (ICP), which compresses brain tissue and disrupts neurological function.

Epidemiology:

• Incidence: 1–2 per 1000 live births
• Congenital forms: spina bifida (70–90% develop hydrocephalus), Chiari malformation, aqueductal stenosis, Arnold-Chiari malformation
• Acquired forms: post-meningitis, post-intraventricular haemorrhage (in premature infants), post-trauma, posterior fossa tumours

Clinical Presentation Differs by Age of Onset:

Infants (Fontanelles & Sutures Still Open—Allow Expansion):
Infants can tolerate increased ICP better than older children because the skull is not yet rigid. Classic signs include:

1. Rapidly increasing head circumference (most important sign in infants; measure at the widest point above the ears at each visit; serial measurements often exceed the normal growth curve)
2. Bulging, tense anterior fontanelle (normally soft and flat; bulges when ICP is elevated; may be tense even with the infant upright)
3. Widely separated cranial sutures (palpable gaps between skull bones)
4. "Sunset eyes" (sclera visible above the iris; downward gaze; due to upward pressure on the brain)
5. High-pitched cry (often described as a "hydrocephalic cry" or "neurological cry")
6. Irritability and lethargy (poor feeding, vomiting, seizures)
7. Distended scalp veins (CSF pressure dilates vessels)

Older Children & Adults (Rigid Skull—No Fontanelles/Sutures):
Once the skull is rigid, ICP increases rapidly without compensatory expansion:

1. Headache (often morning headache, worsening with movement; not present in young infants because they cannot communicate)
2. Projectile vomiting (often worse in the morning or when head is moved; the "neurological vomiting" of increased ICP)
3. Altered consciousness: drowsiness, confusion, difficulty concentrating
4. Papilledema (swelling of the optic disc seen on fundoscopy; indicates chronic elevated ICP)
5. Sixth nerve palsy (lateral rectus weakness causing convergent strabismus; a "false localizing" sign of increased ICP—affected because CN VI is stretched as ICP rises)

CRITICAL: Cushing's Triad (LATE Sign of Severely Elevated ICP—Emergency):
When ICP becomes very high and there is brainstem compression:

• Bradycardia (severe slowing of heart rate)
• Hypertension with widening pulse pressure (elevated systolic and normal/low diastolic; e.g., 180/60 instead of normal 120/80)
• Irregular respirations (Cheyne-Stokes or ataxic breathing patterns)

This triad indicates impending herniation and is an EMERGENCY requiring immediate intervention.

Diagnosis:

• Neuroimaging (CT or MRI): shows enlarged ventricles and, if present, the cause (obstruction, mass, etc.)
• Lumbar puncture: may be contraindicated in acute hydrocephalus (risk of brain herniation from sudden CSF release)
• Ultrasound: in infants, can be done through the open fontanelle (bedside assessment)

Management:

Immediate (Acute Decompression):
If ICP is severely elevated and the child is deteriorating:

• Elevate head of bed 30 degrees (promotes venous drainage from head)
• Midline head positioning (avoid head rotation, which compresses jugular veins)
• Osmotic therapy: IV mannitol (0.25–1 g/kg IV bolus) or hypertonic saline (3% or 23.4%) to osmotically draw fluid from the brain into the intravascular space and reduce ICP
• Maintain normothermia, normoxia, normocarbia (fever, hypoxia, and hypercarbia all increase ICP)
• Prepare for emergency drainage: external ventricular drain (EVD) at the bedside for acute relief

Definitive Treatment: Ventriculoperitoneal (VP) Shunt:
The vast majority of infants with congenital hydrocephalus and children with acquired hydrocephalus are treated surgically with a VP shunt:

How It Works:

• A small catheter is placed in the lateral ventricle of the brain (the distal end)
• The catheter is tunnelled under the skin to the peritoneal cavity (abdominal cavity)
• CSF drains from the ventricle into the peritoneum, where it is absorbed
• Unidirectional valve in the shunt opens when ICP exceeds a set pressure (typically 5–15 cm H₂O), allowing drainage

Other Shunt Sites (Less Common):

• VA (ventriculoatrial) shunt: drains into the right atrium of the heart
• Ventriculopleural shunt: drains into the pleural cavity

Post-operative Management (Critical for NLE):

1. Positioning (CRUCIAL):

   • Keep the child FLAT in bed for the first 24–48 hours post-op (prevents too-rapid CSF drainage and the risk of subdural haematoma from rapid ventricular collapse)
   • Position on the NON-operative side (e.g., if the shunt is on the right, position on the left side) to reduce pressure on the incision
   • Head elevation is done GRADUALLY as ordered (e.g., raise head 15 degrees on day 2, 30 degrees on day 3, etc.) to avoid sudden fluid shifts

2. Monitor for Shunt Malfunction (Symptoms Suggest Returning Elevated ICP):

   • Bulging fontanelle (in infants)
   • Increased head circumference
   • Headache, lethargy
   • Projectile vomiting
   • Decline in pupillary response, vital sign changes
   • Report immediately; may require imaging (CT/ultrasound) to check shunt position and assess for obstruction

3. Monitor for Shunt Infection (Most Serious Post-op Complication):

   • Fever (especially in the first week post-op)
   • Poor feeding, irritability, lethargy
   • Erythema, warmth, or drainage along the shunt tract (can track from head incision down to abdomen)
   • Meningeal signs: nuchal rigidity, positive Kernig's/Brudzinski's (if ventriculitis develops)
   • CSF characteristics if tapped: elevated protein, pleocytosis, positive culture
   • Risk factors: infants <6 months, prolonged hospitalization, multiple previous surgeries
   • Treatment: removal of infected shunt, external ventricular drain (EVD) for temporary drainage, IV antibiotics (vancomycin ± gentamicin until sensitivities known), replacement of shunt after 48–72 hours of sterile CSF

4. Incision Care:

   • Keep incision clean and dry
   • Watch for signs of infection (redness, warmth, drainage)
   • Typical suture removal: 10–14 days post-op

5. Feeding & Comfort:

   • Begin feeds once the child is alert and able to swallow
   • Hold infant for feeds (supports bonding; flat position restriction is for initial recovery)
   • Pain management (opioids or non-opioids as appropriate)

6. Developmental Monitoring:

   • Early developmental intervention (physical, occupational, speech therapy) as needed
   • Many children with hydrocephalus have near-normal development if shunt is placed early and functions well

Long-term Issues:

• Shunt revision: shunts have a limited lifespan (average 8–10 years in infants; longer in older children)

  • Reasons for revision: malfunction, infection, outgrowth (child grows, ventricular size changes, requiring upsizing)
  • Symptoms of need for revision: same as malfunction (increasing ICP signs)
• Learning difficulties: some children develop subtle learning or attentional problems even with a functioning shunt
• Endocrine dysfunction: growth hormone deficiency or other endocrine disorders may develop (from hypothalamic-pituitary compression initially or from shunt-related issues)

NLE Focus:

• Infants: increasing head circumference + bulging fontanelle + "sunset eyes" + high-pitched cry
• Older children: headache + projectile vomiting + altered consciousness + papilledema
• Cushing's triad = LATE, OMINOUS sign (bradycardia, hypertension, irregular respirations)
• VP shunt is the definitive treatment for most cases
• Post-op positioning: FLAT + NON-operative side initially; head elevated gradually
• Shunt malfunction = returning ICP signs; requires imaging
• Shunt infection = fever + poor feeding + local signs along tract; highest risk in infants; serious complication requiring shunt removal/replacement

Pathophysiology:
Cerebral palsy is a non-progressive disorder of movement and posture resulting from an insult to the immature/developing brain (before age 2–3 years, before motor development is complete). The critical point is that the brain lesion does NOT progress, but the child's manifestations may change and evolve as the child grows (this is why early intervention is important—catch and treat complications early).

Etiology & Timing of Insult:

• Prenatal (40%): intrauterine infection (TORCH, cytomegalovirus), teratogenic exposures, genetic disorders, placental insufficiency
• Perinatal/Birth (40%): birth asphyxia, hypoxic-ischaemic encephalopathy (HIE), prematurity, intraventricular haemorrhage (IVH)
• Postnatal (20%): head trauma, infection (meningitis, encephalitis), stroke, near-drowning, shaken baby syndrome

Risk Factors:

• Prematurity (especially <32 weeks gestation)
• Birth weight <1500 g
• Maternal infection during pregnancy
• Asphyxia at birth
• Severe jaundice (bilirubin encephalopathy / kernicterus)
• Postnatal infection or trauma

Classification by Motor Type (Pyramidal vs. Extrapyramidal):

1. Spastic Cerebral Palsy (70–80% of cases)—Most Common

Pathophysiology: Damage to the motor cortex (pyramidal tract damage) → increased muscle tone (hypertonicity), especially in antigravity muscles.

Characteristics:

• Increased muscle tone (spasticity): muscles are "stiff" or "tight"; clasp-knife rigidity (resistance that suddenly gives way)
• Hyperreflexia (exaggerated deep tendon reflexes)
• Persistent primitive reflexes (e.g., Moro reflex, grasp reflex) beyond the age they should resolve (normally by 3–6 months)
• Scissoring of the legs (crossing one leg over the other due to adductor muscle spasticity; classic sign)
• Toe-walking (due to plantarflexor spasticity)

Distribution (By Limbs Affected):

• Hemiplegia (50%): one side affected (usually arm > leg); often follows a stroke or focal brain lesion
• Diplegia (30%): both legs affected more than arms; classic in premature infants with periventricular leukomalacia (PVL)
• Quadriplegia (10%): all four limbs severely affected

2. Ataxic Cerebral Palsy (10–15%)

• Affects cerebellar pathways
• Hypotonia (low muscle tone, "floppy baby")
• Poor coordination, balance, and fine motor control
• Wide-based gait (unsteady, staggering)
• Tremor and dysmetria (inability to judge distance/place limbs accurately)

3. Athetoid (Dyskinetic) Cerebral Palsy (10–15%)

• Affects basal ganglia (extrapyramidal)
• Involuntary, writhing movements (athetosis)
• Fluctuating muscle tone (low at rest, increases with effort)
• Oral-facial dyskinesia (difficulty with speech and feeding; tongue thrusting)
• Often associated with bilirubin encephalopathy (kernicterus) from severe unconjugated hyperbilirubinaemia

Early Signs (Detection in First 2 Years):

Delayed Motor Milestones:

• Failure to lift head by 3 months
• Inability to roll over by 6 months
• No sitting by 9 months
• Not walking by 18 months

Abnormal Movement Patterns:

• Persistent fisting (clenched fist beyond 3 months)
• Scissoring of legs
• Toe-walking
• Inability to track with eyes or respond to stimuli

Muscle Tone Abnormalities:

• Hypotonia (initially; "floppy baby" in some)
• Later, progressive spasticity

Feeding Difficulty & Aspiration Risk:

• Weak suck
• Difficulty chewing and swallowing
• Drooling
• Food/formula entering the lungs (aspiration)—major complication

Persistent Primitive Reflexes:

• Moro reflex present after 6 months
• Grasp reflex still strong (normal in newborns, should fade by 5–6 months)
• Asymmetrical tonic neck reflex (ATNR) present; normally integrates by 4–6 months

Diagnosis (Clinical):

• History of prenatal, perinatal, or postnatal insult
• Pattern of abnormal movement/tone on examination
• Neuroimaging (MRI preferred): shows the lesion (e.g., PVL in premature infants, basal ganglia damage in bilirubin encephalopathy, cortical infarct)
• Standardized developmental assessment (Bayley Scales, Gross Motor Function Classification System—GMFCS)
• Diagnosis is often not made before age 2 years (because younger infants have limited motor repertoire)

Management (Supportive, Multidisciplinary)—Goal Is to MAXIMIZE Function, Not "Cure":

1. Physical Therapy:

• Stretching exercises (prevent contractures)
• Positioning: proper posture during sleep and sitting to prevent deformities
• Gait training: teach optimal walking patterns; use of walkers, crutches as needed
• Starts early (often by 6–12 months if CP suspected)

2. Occupational Therapy:

• Activities of daily living (ADL) training: eating, dressing, hygiene
• Fine motor development: manipulation, grasp, coordination
• Adaptive equipment: special utensils, writing aids, splints

3. Speech-Language Pathology:

• Speech/language development (many children with CP have speech difficulties)
• Feeding/swallowing assessment (due to oral-motor dysfunction)
• Augmentative and alternative communication (AAC) devices if unable to speak (e.g., communication boards, computer-assisted speech)

4. Medications:

For Spasticity:

• Baclofen (oral or intrathecal): GABA-B agonist; reduces muscle tone; side effects include drowsiness
• Botulinum toxin (Botox) injections: weakens overactive muscles; given every 3 months; helps with specific muscle groups (e.g., tight hip flexors, calf muscles)
• Dantrolene: reduces muscle tone; rare, can cause hepatotoxicity

For Seizures:

• Phenytoin, phenobarbital, carbamazepine, levetiracetam (25–40% of children with CP develop seizures, especially in quadriplegic forms)
• The risk of seizures is highest in the first few years after the brain insult

5. Orthopedic Surgery:

• Tendon lengthening/transfer (if contractures develop and limit function)
• Spinal fusion (if significant scoliosis develops, especially in non-ambulatory children)
• Usually done only after maximal physical therapy

6. Nutritional Support:

• Many children with CP have feeding difficulties (oral-motor dysfunction, aspiration risk)
• May require modified diet (pureed, thickened liquids)
• Tube feeding (NG tube or G-tube/gastrostomy) if unable to safely take adequate nutrition by mouth
• Monitor growth carefully (many children with CP are nutritionally at-risk)

Nursing Care & Family Support (Critical for NLE):

Aspiration Precautions (MOST CRITICAL):

• Position during feeding: upright (head >45 degrees) or semi-upright; never feed a floppy infant in supine position
• Small, frequent bites/sips
• Allow extra time for chewing and swallowing
• Supervise feeding (don't leave child unattended with a spoon/bottle)
• Listen for wet cough or change in voice after swallowing (signs of aspiration)
• Monitor respiratory status (fever, cough, tachypnea may indicate aspiration pneumonia)

Preventing Contractures:

• Passive range-of-motion (ROM) exercises: move each joint through full range of motion, gently, several times daily
• Proper positioning: prevent scissoring by placing a pillow between the legs during sleep
• Splinting: may be recommended by physical therapist to hold joints in a neutral position at night

Developmental Support:

• Play therapy: use age-appropriate play to encourage development
• Early intervention programmes: available in most developed countries; in the Philippines, these may be through government health centres or NGOs
• School placement: mainstream schooling when possible; special education if needed
• Adaptive equipment: wheelchair, stander, walker—chosen based on child's abilities

Emotional & Family Support:

• Parents often experience grief and stress over the diagnosis
• Provide realistic information about the child's potential and limitations
• Connect families with support groups and resources
• Teach parents therapeutic exercises they can do at home
• Celebrate small milestones

Prognosis:

• Non-progressive: the brain lesion does not worsen, but manifestations change as the child grows
• Highly variable: depends on the severity of the initial insult, the type of CP, and the presence of other complications (seizures, vision/hearing loss, intellectual disability)
• Many children with CP attend regular school, some live independently as adults
• Lifespan: generally normal for spastic and ataxic types; may be shortened in severe quadriplegic forms with significant respiratory/feeding involvement

NLE Focus:

• CP = non-progressive brain insult; manifestations change over time
• Spastic type (70–80%): scissoring, toe-walking, increased tone, hyperreflexia, persistent primitive reflexes
• Early signs: delayed milestones, abnormal tone/movement, feeding difficulty
• Goal: maximize function, prevent complications (contractures, aspiration pneumonia, seizures)
• Aspiration precautions: upright feeding, small amounts, supervised
• Multidisciplinary team: PT, OT, speech therapy, orthopedic surgery as needed
• Seizures common; treat promptly
• Many children with CP live full, productive lives with early intervention and support