Overview

Definition:
-Osteopenia of prematurity (OOP) is a metabolic bone disease characterized by a low bone mineral content in preterm infants, leading to increased risk of fractures and bone deformities
-It results from an inadequate supply of calcium and phosphorus to meet the demands of rapid skeletal growth, especially in infants born before 30 weeks of gestation or with a birth weight less than 1000 grams.
Epidemiology:
-Occurs in 20-40% of very low birth weight (VLBW) infants, with the incidence increasing with decreasing gestational age and birth weight
-Infants requiring prolonged parenteral nutrition, those with chronic lung disease, or malabsorption syndromes are at higher risk.
Clinical Significance:
-OOP poses significant challenges in neonatal intensive care units, impacting long-term skeletal development, growth, and potentially leading to rickets, fractures, and impaired motor development
-Optimal calcium and phosphorus management is crucial for improving bone health outcomes in this vulnerable population.

Risk Factors

Gestational Age: Infants born before 30 weeks gestation have insufficient intrauterine accretion of bone minerals.
Birth Weight: Very low birth weight (VLBW) infants (<1000g) have limited mineral reserves.
Nutritional Intake: Inadequate enteral or parenteral intake of calcium and phosphorus.
Medical Conditions: Chronic lung disease (requiring ventilation, steroids), gastrointestinal disorders (NEC, cholestasis, short bowel syndrome), infections, and prolonged immobilization.
Medications: Corticosteroids (inhibit osteoblast activity and calcium absorption), loop diuretics (increase urinary calcium and phosphorus excretion).

Clinical Presentation

Symptoms:
-Often insidious onset
-May present with poor weight gain
-Irritability
-Muscle weakness or hypotonia
-Fractures (pathological, often long bones like femur, humerus) identified incidentally or following minor trauma
-Respiratory distress may worsen due to rib fractures.
Signs:
-Skeletal deformities (rachitic rosary, bowing of long bones) may be subtle in neonates
-Epiphyseal enlargement is uncommon
-Tenderness on palpation of bones
-Signs of hypocalcemia (e.g., carpopedal spasm, stridor) if severe.
Diagnostic Criteria:
-Diagnosis is often based on a combination of clinical suspicion, risk factors, laboratory findings, and imaging
-Radiographic findings of decreased bone mineralization (generalized osteopenia, cortical thinning, fraying of metaphyses) are classic
-Biochemical markers are supportive but not definitive for diagnosis.

Diagnostic Approach

History Taking:
-Detailed history of gestational age, birth weight, postnatal feeding (enteral/parenteral), duration of ventilatory support, steroid use, and presence of gastrointestinal issues
-Family history of bone disease.
Physical Examination:
-Assess for signs of rickets, assess muscle tone and strength, palpate for bone tenderness
-Examine for any signs of trauma or fracture.
Investigations:
-Serum calcium (total and ionized), phosphorus, alkaline phosphatase (SAP), magnesium, and vitamin D levels (25-hydroxyvitamin D)
-Serial monitoring is essential
-Radiographs of long bones (e.g., tibia, femur) to assess bone mineralization, cortical thickness, and metaphyseal changes
-Bone mineral density (BMD) measurements using DXA or quantitative ultrasound (QUS) can be used in older infants but are less common in neonates.
Differential Diagnosis: Other causes of hypomineralization: Hypophosphatasia (genetic disorder with low SAP), severe malnutrition, chronic renal insufficiency, Albright's hereditary osteodystrophy, and calcium/phosphorus malabsorption syndromes.

Management

Initial Management:
-Early identification of at-risk infants and initiation of appropriate nutritional support
-Aim to provide adequate calcium and phosphorus to support bone growth, considering both enteral and parenteral routes.
Calcium And Phosphorus Supplementation:
-Target serum phosphorus levels: 1.5-2.5 mmol/L (4.6-7.8 mg/dL)
-Target serum calcium levels: 2.0-2.6 mmol/L (8-10.4 mg/dL) for total calcium, or 1.1-1.4 mmol/L (4.4-5.6 mg/dL) for ionized calcium
-Recommended daily intake for preterm infants is approximately 100-150 mg/kg/day for calcium and 50-100 mg/kg/day for phosphorus, adjusted based on gestational age, birth weight, and feeding route.
Enteral Supplementation:
-Provide calcium and phosphorus in fortified human milk or specialized preterm formula
-Common forms: calcium gluconate, calcium lactate, dibasic calcium phosphate, potassium phosphate
-Dosing is crucial to avoid precipitation in feeding tubes or gastrointestinal issues.
Parenteral Supplementation:
-Administered via peripheral or central venous access
-Calcium and phosphorus must be added to parenteral nutrition (PN) solutions carefully to prevent precipitation (calcium phosphate salt)
-The order of addition (e.g., phosphate before calcium) and pH of PN are critical
-Maximum solubility depends on PN formulation, osmolarity, and temperature
-Refer to established PN guidelines for safe limits.
Vitamin D Supplementation:
-Vitamin D is essential for calcium and phosphorus absorption
-Administer cholecalciferol (D3) or ergocalciferol (D2)
-Typical dose: 400-1000 IU/day
-Monitor serum 25-hydroxyvitamin D levels.
Supportive Care:
-Minimize corticosteroid use if possible
-Optimize nutrition
-Gentle handling of infants to prevent fractures
-Physiotherapy may be considered for infants with muscle weakness or fractures.

Complications

Early Complications:
-Fractures (especially long bones, ribs)
-Hypocalcemia (symptomatic)
-Hypophosphatemia
-Precipitation of calcium phosphate in PN lines or kidneys.
Late Complications:
-Delayed growth and development
-Impaired motor milestones
-Skeletal deformities (bowing of limbs)
-Increased risk of osteoporosis later in life.
Prevention Strategies:
-Proactive identification of high-risk infants
-Timely and adequate enteral/parenteral calcium and phosphorus supplementation
-Careful management of PN solutions to prevent precipitation
-Judicious use of corticosteroids
-Adequate vitamin D intake.

Prognosis

Factors Affecting Prognosis: Severity of OOP, gestational age at birth, presence of associated comorbidities (e.g., chronic lung disease), adequacy and timing of nutritional management, and development of fractures.
Outcomes:
-With appropriate management, bone mineralization improves, and fractures heal
-Long-term skeletal outcomes depend on the severity and duration of the disease and the effectiveness of interventions
-Most infants achieve normal bone density by term-equivalent age or later.
Follow Up:
-Regular monitoring of growth, bone mineral status (biochemical markers and serial radiographs), and skeletal development
-Continued nutritional support and vitamin D supplementation as needed
-Physiotherapy and orthopedic follow-up may be required for infants with significant deformities or fractures.

Key Points

Exam Focus:
-Risk stratification for OOP
-Biochemical markers: high alkaline phosphatase (SAP), low phosphorus
-Radiographic signs of osteopenia
-Principles of calcium/phosphorus supplementation in enteral and parenteral nutrition
-Prevention of calcium-phosphate precipitation in PN
-Role of vitamin D.
Clinical Pearls:
-Always consider OOP in VLBW infants with poor growth or fractures
-Monitor serum electrolytes and SAP closely
-Pay meticulous attention to the safe preparation of PN solutions to avoid calcium-phosphate precipitation
-Consider adjusting mineral intake based on infant's growth trajectory and metabolic status.
Common Mistakes:
-Underestimating the mineral needs of preterm infants
-Inadequate monitoring of serum calcium, phosphorus, and SAP
-Incorrect calculation or administration of PN solutions leading to precipitation
-Delayed diagnosis and initiation of treatment.