pediatrics, clinical meeting, endocrinology, osteoporosis, fractures, vertebral fractures

1. “All roads don’t
lead to Rome!!”
Dr. Arun George
Paediatric endocrinology

2. Presenting a case series of adolescents
reporting with varying symptoms and
also having significant back pain !!
-- which required imaging

3. Case 1
• AP view • Lateral view

4. Normal Spine XR in adolescence

5. Vertebral compression fractures
Osteoporosis vs trauma
Osteoporosis
• Severe osteopenia
• Usually non-displaced,
compression fracture.
Trauma
• No osteopenia
• Can be displaced.

6. Case 2

7. Case 3

8. Case 4

9. When is back pain significant enough for evaluation?
• Persistent back pain interfering with daily activities, night time pain interfering with sleep
• Radicular pain, positive straight leg raising test result
• Evidence of any neurological involvement – lower limbs, bowel and bladder
• Numbness and paresthesia of lower limb
• Fever, malaise, weight loss, generalised lymphadenopathy
• Muscle tenderness without radiation/ Hamstring tightness
• Morning stiffness, rigid kyphosis

10. Case 1 Case 2 Case 3 Case 4
14 years old girl –
presented to Paediatric
Orthopaedics OPD
Back pain for 2 years
Abdominal pain for 2 years
Blood in stools and joint
pains for 1 year
LOW - 10 Kg over 2 years
No history of fever, rashes,
oral ulcers, breathlessness,
chest pain, photosensitivity,
hair loss, other bleeding
manifestations, vomiting.
10 year old boy, 1st born of
non consanguineous
parentage
Facial puffiness and weight
gain for 4 months
Back pain for 2 months
Normal antenatal, perinatal
and neonatal period
Development normal
13 year old boy with high
grade fever, right knee and
shoulder pain for 3 months
Back pain for 2 months
Significant Loss of weight
Normal antenatal, perinatal
and neonatal period
Development normal
11 year old boy, Back pain
for 6 months.
Severe pain in the left
metatarsal following a
trivial trauma.
There was no history of
fever, loss of weight, loss of
appetite, bleeding
manifestation, recurrent
fractures, contact with
tuberculosis.

11. Case 1 Case 2 Case 3 Case 4
Active, alert, afebrile
Weight: 37.3 Kgs
Length: 148 cm
Head circumference: 52 cm
Pallor present, Grade 2
clubbing present
Vitals stable
Systemic examination -
unremarkable
Active, alert, afebrile
Weight: 31.8 Kgs
Length: 128 cm
Vitals stable
Truncal obesity,
dorsocervical fat pad,
striae
Systemic examination
normal.
Active, alert, febrile
Vitals stable
Swelling, warmth and
tenderness over right knee
and shoulder
Systemic examination
normal.
Afebrile, awake and alert.
Vitals stable.
Kyphosis and scoliosis.
He had antalgic gait.
Bilateral flat feet present.
Systemic examination
normal.

12. Case 1 Case 2 Case 3 Case 4
Cushing’s disease

13. Case 1 Case 2 Case 3 Case 4
CT Abdomen– Skip lesions
s/o Crohn’s disease.
Multiple wedge
compression of the
vertebral bodies,
osteoporosis.
X ray Spine- Diffuse
osteoporosis with
multilevel secondary
compression fractures –
thoracic and lumbar
vertebral bodies.
Chest X ray – collapse of
the D11 vertebrae with
lateral wedging, features of
osteoporosis.
CT scan – features of
osteoporosis, multiple lytic
lesions of the appendicular
skeleton
Chest X ray- Increased
thoracic kyphosis, diffuse
osteopenia

14. Case 1 Case 2 Case 3 Case 4
CBC – Normal
CALCIUM 8.46
PHOSPHORUS 5.4
ALKALINE
PHOSPHATASE 127
TSH 3.521
CREATININE 0.34
VITAMIN D(25 OH) 36.7
PARATHYROID HARMONE
(PTH) 26.0
UCA/UCR RATIO 0.25
CBC- Normal
CALCIUM 9.60
PHOSPHORUS 6.4
ALKALINE
PHOSPHATASE 255
CREATININE 0.39
VITAMIN D(25 OH) 42.1
CBC – Normal
CALCIUM 9.05
PHOSPHORUS 4.6
ALKALINE
PHOSPHATASE 442
CREATININE 0.46
VITAMIN D(25 OH) 10.7
PARATHYROID HARMONE
(PTH) 26.0
CBC – Normal
CALCIUM 10.20 mg%
ALKALINE
PHOSPHATASE 158 U/L
VITAMIN D(25 OH) 20.4
CREATININE 0.37 mg%
PARATHYROID HARMONE
(PTH) 24.7 pg/ml

15. Case 1 Case 2 Case 3 Case 4
FECAL CALPROTECTIN BY
ELISA TEST- POSITIVE -
(>2010 µg/g)
SURG PATHOLOGY
SPECIMEN
Mild to moderate chronic
ileitis with mild
activity and focal
microgranulomata, mucosal
biopsy. Minimal to mild
chronic colitis with edema
and minimal to mild
eosinophilia.
Note: Crohn's disease is a
histological possibility.
CORTISOL SERUM [11
PM] 26.0
CORTISOL SERUM [8.00
AM] 23.8
ACTH (ICED SAMPLE) [11
PM] <5.00
DHEAS 58.6
BIOPSY CMCH VELLORE-
Adrenocortical adenoma,
Modified Weiss score
0, right adrenal gland.
Biopsy from the swelling on
right shoulder – f/o High
grade T cell NHL

16. Case 1 Case 2 Case 3 Case 4
Crohn’s disease Cushing’s disease T cell NHL Juvenile Idiopathic
Osteoporosis

17. Thus
• All 4 patients had compression fractures secondary to osteoporosis
• However, their underlying diagnosis was totally different.

18. Paediatric
Osteoporosis

19. Osteoporosis
• Osteoporosis is defined as follows: a systemic skeletal disease
characterized by low bone mass and micro architectural deterioration
of bone tissue, resulting in increased bone fragility and susceptibility
to fracture
• A child or teen with one or more vertebral compression fractures
occurring without local bone disease or high-energy trauma meets
criteria for paediatric osteoporosis.

20. Distinguish with other terms:
• Osteomalacia is defective mineralisation of the bones
• RICKETS is defective mineralisation of a growing bone
• Osteopenia – early stage: is when your bones are weaker than
normal but doesn’t have risk of fractures ( d/d which is the hallmark
of osteoporosis)

21. Osteoporosis
• Is a consequence of imbalance between bone formation and bone
resorption in favor of bone resorption and a failure of normal bone gain
• Histologic sections of bone in all forms of osteoporosis reveal a normal
degree of mineralization but a reduction in the volume of bone,
especially trabecular bone (vertebral bone)
• In contrast in osteomalacia, there is undermineralization and normal
bone volume

22. Criteria for paediatric osteoporosis
• The diagnosis of osteoporosis requires the presence of both a clinically
significant fracture history and low bone mineral content (BMC) or bone
mineral density (BMD)
• A chronic disease process negatively affects longitudinal growth and cause a
delay in pubertal development
• Therefore, the interpretation of the bone mineral density assessment should be
adjusted for body size and pubertal development
• The ISCD 2013 Pediatric Official Positions, Journal of Clinical Densitometry: Assessment & Management of
Musculoskeletal Health, vol. 17, no. 2, 2014

23. • A clinically significant fracture history is one or more of the following:
Long bone fracture of the lower extremities.
Vertebral compression fracture.
Two or more long bone fractures of the upper extremities.
The ISCD 2013 Pediatric Official Positions, Journal of Clinical Densitometry: Assessment
& Management of Musculoskeletal Health, vol. 17, no. 2, 2014

24. Measurement of Bone Density
Dual energy X-ray absorptiometry (DEXA)
• Direct measurement of mineral content/ Indirect assessment of density
• Results are affected by body size and skeletal maturity
• Does not measure trabecular density separately
• Must be evaluated with pediatric software
• Results must be obtained as Z-score (subject’s results compared to age and
gender – matched controls)/ DO NOT USE T-scores
• Should be done after Vit D sufficient

25. Peripheral computer tomography (pQCT)
• is less widely available than DXA.
• assesses volumetric BMD and bone geometry, commonly of the
forearm
• the result of the patient is compared to age, gender and ethnicity
matched reference data and expressed in Z-score
• the advantage is that volumetric bone mineral density is assessed
• the disadvantage is that only appendicular bone can be measured

26. • Low BMC or BMD is defined as a BMC or a real BMD Z-score that is
less than or equal to 2.0, adjusted for age, gender, and body size, as
appropriate.
• The ISCD 2013 Pediatric Official Positions, Journal of Clinical Densitometry: Assessment &
Management of Musculoskeletal Health, vol. 17, no. 2, 2014

27. Bone anatomy
• Bone is a multi composite material that consists of
cells (osteocytes, osteoblasts, and osteoclasts),
extracellular organic components (collagen and noncollagenous
matrix proteins), and
 nonorganic components (calcium hydroxyapatite).

29. Osteoporosis - Types
• Primary and Secondary
• Primary osteoporosis:
Heritable disorders of connective tissue- including osteogenesis
imperfecta, Bruck syndrome, osteoporosis pseudoglioma syndrome,
Ehlers-Danlos syndrome, Marfan syndrome, homocystinuria, and
idiopathic juvenile osteoporosis.

30. Secondary forms of osteoporosis
Neuromuscular disorders, chronic illness, endocrine disorders, and
drug-induced and inborn errors of metabolism, including lysinuric
protein intolerance and Gaucher disease.

31. Evaluation:
• Evaluation of bone mineral content and bone density by dual-energy
x-ray absorptiometry or, less often, quantitative CT shows markedly
reduced values.
• Review of chronic illness, medications, nutrition, activity, and fracture
history.
• A family history of hip fractures in elderly relatives or repeated
fractures in younger family members should be explored.

32. • Complete blood count, erythrocyte sedimentation rate, serum
calcium, phosphorus, alkaline phosphatase, intact PTH, total 25
hydroxy vitamin D, BUN, creatinine, and celiac screen, as well as
urinary calcium to creatinine ratio.
• Additional tests to consider depending upon the age, physical exam,
and history include luteinizing hormone, follicle stimulating hormone,
oestradiol, testosterone, insulin-like growth factor type I, free
T4, and TSH.

33. • Genetic screening for osteogenesis imperfecta
• Genetic disorders, such as inactivating mutations in low-density
lipoprotein receptor related protein 5
• Vertebral fractures may be asymptomatic and a lateral thoracolumbar
spine radiograph is indicated for those with back pain, low-trauma
fractures of the long bones, or chronic glucocorticoid therapy.

34. Secondary osteoporosis
• Chronic inflammatory diseases
In inflammatory bowel disease, juvenile idiopathic arthritis and
dermatomyositis, bone metabolism is negatively influenced by pro-
inflammatory cytokines, like interleukin 6. Immobility and corticosteroid
treatment has additive negative effects
• Immobility
Conditions that are associated with impaired mobility: cerebral palsy, spinal
cord injury, muscular dystrophy and spinal muscular atrophy have an
increased risk of fractures, especially of the femur. - Dynamic bone loading
stimulates bone formation.
Anticonvulsant treatment may contribute to the increased fracture risk in
patients with cerebral palsy.

35. • Leukemia
• In haematological disorders the bone marrow expansion may affect
trabecular bone
• A vertebral fracture can be the presenting symptom of acute lymphoblastic
leukemia.
• Chronic renal failure
• increased risk of renal osteodystrophy.
• secondary hyperparathyroidism cause a high bone turnover bone diseas
• excessive suppression of the pararathyroid gland is associated with adynamic
bone disease
• low levels of 1,25 dihydroxyvitamin D cause osteomalacia
• metabolic acidosis stimulates bone resorption.

36. Management
• The treatment of secondary osteoporosis is best achieved by treating the underlying
disorder when feasible .
• A number of measures influence bone mass: vitamin D (400-800 IU daily), calcium
intake (≥1,200 mg/day in adolescents), and weightbearing exercise throughout
childhood
• Weight- bearing exercise enhances bone formation
• Several modes of therapy with bisphosphonates have been used with success
• Use of alcohol and tobacco – prevent

37. • Hypogonadism should be treated with hormone replacement
therapy
• Calcium intake should be increased to 1,500-2,000 mg/day
• In glucocorticoid-induced osteoporosis- lowest possible dose to
prevent disease activity (inflammatory bowel disease)- alternate-day
or topical therapy and the use of inhaled glucocorticoids in asthma is
essential.

38. • Special diets for inborn errors of metabolism are also appropriate.
• Celiac disease may be overrepresented in adults with osteoporosis
and should be screened for and treated appropriately.
• Treatment with bisphosphonates that inhibit bone resorption in
certain secondary (glucocorticoid-induced) and adult-onset
osteoporosis has been successful. Bisphosphonate therapy is also
beneficial in osteogenesis imperfecta and cerebral palsy.

39. Case 1 Case 2 Case 3 Case 4
Crohn’s disease Cushing’s disease T cell NHL Juvenile Idiopathic
Osteoporosis
What happened to our patients ?

40. Take Home Message
• Low backache in a child is not always benign
• Osteoporosis in children are usually secondary to a systemic
pathology. Idiopathic osteoporosis is rare in children.
• Patient has to be evaluated for the underlying disease and treated
for the same in addition to the treatment of osteoporosis.
• Pamidronate infusion is the mainstay of therapy in Paediatric
osteoporosis. The vertebral fractures can be medically managed.

41. References
1. Nelson textbook of Paediatrics – 1st south Asian edition
2. Diagnosis and treatment of Pediatric osteoporosis, Laura K. Bachrach , Current Opinion in
Endocrinology, Diabetes & Obesity: December 2014- Vol 21 Issue 6
3. Fracture Prediction and the Definition of Osteoporosis in Children and Adolescents: The ISCD
2013 Pediatric Official Positions, Journal of Clinical Densitometry: Assessment & Management
of Musculoskeletal Health, vol. 17, no. 2, 2014