Osgood-Schatter syndrome, short stature and Vit D deficiency

Question 1

What is Osgood-Schatter syndrome?

Answer 1

Extra-articular disease, due to external tibial tubercle apophyseal traction, leading to pain and inflammation.

Question 2

What is the aetiology of OSS?

Answer 2

Mostly traumatic, usually occurs in the year following a growth spurt.

Tension from the insertion of the quadriceps muscle into the tibial apophysis leads to a partial avulsion fracture in the ossificaiton centre. This leads to sclerosis of the apophyseal bone around the insertion, leading to a visibile lump.

Usually occurs in athletes, especially impact ones (i.e. running jumping).

DDX: tibial fracture, underlying tumor, quadriceps tendon avulsion. Chondromalacia patellae, osteomyelitis, patellar tendonitis.

Question 3

What is the epidemiology of OSS?

Answer 3

Most common cause of knee pain in adolescents.

Question 4

What would you find in the Hx and Ex in someone with OSS?

Answer 4

Pain in front of knee below, history of trauma. Localised. Increases when moving, decreases with rest. Pain for several months.

Look: soft tissue swelling over proximal tibial tuberosity.

Feel: tender over tibial tuberosity.

Move: pain reproducible when extending the knee against resistance, squatting.

Knee joint exam normal as this is extra articular.

Question 5

What investigations would you do for OSS?

Answer 5

XR knee, obvious fractures or ossicles the tibial tubercle

Question 6

What is the management of OSS?

Answer 6

Rest: avoid activity. Pain management with NSAIDS. Leg strap or knee immobiliser.

Removal surgically of separate ossicle rarely required and only to be done in adults.

Question 7

What are the complications and prognosis of OSS?

Answer 7

Non union of tibial tubercles, patellar tendon avulsion, pain, bony prominence.

Relatively benign condition

Question 8

What is the definition of short stature?

Answer 8

Height below second centile (<2SD below mean for gender and sex).

Question 9

What are the Ddx for short stature?

Answer 9

Familial: compare child height to mid parental height. Check bone age and growth velocity which should be appropriate. Make sure no bone disease in famiy.

IUGR: 33% of IUGR infants remain short.

Endocrine: decreased growth velocity and weight loss. Hypothyroidism, GH deficiency, corticosteroid excess.

Nutritional or chronic disease

Chromosomal disorders, dysproportion or bone disease

Psychological deprivation

Question 10

What is the epidemiology of short stature?

Answer 10

By definition 2% of children.

Question 11

What may you find in the history of a child with short stature?

Answer 11

General: birth records and growth charts, parental height and weight anf FHx.

Target for girl: (Mother height x Father height-13) /2.

For boy same but mother + 13.

Systemic review and detailed social hx necessary.

Question 12

What might you find in the examination of someone with short stature?

Answer 12

Measure height, weight, FO circumference. If can not stand, arm span can be used for height.

Growth velocity: over 6/12.

Specific: height of sitting body (short limbed dwarfism), thyroid examination, shortered fourth metacarpals (Turner’s), ulceratice stomatitis (Crohn’s)

Question 13

What investigations would you do for short stature?

Answer 13

Bloods: low Hb in nutritional deficiency/chronic disease, U&Es (renal failure) ESR/CRP (IBD) albumin (malnutrition), IGFB3 for GH deficiency.

Karyotype for genetic conditions, sweat test for CF, Ig assays for coeliac.

Hand/wrist XR for bone age. MRI if suspect neurological (i.e. GH def tumor)

Question 14

What is the management of short stature?

Answer 14

Dependent on cause

Optimise diet and psychosocial environment, rhGH for deficiency, thyroxine for hypothyroid, remove tumors if causative. Counsel.

Question 15

What are the complications and prognosis of short stature?

Answer 15

Familial persists, GH/TH def can attain height if starting replacement <5y, chronic disease depends on tx.

Question 16

What is vitamin D deficiency?

Answer 16

Low Vitamin D

Vitamin D = ergocalciferol, cholecalciferol, 25OHD3 and 1-25-OHD3 (calcitriol).

Question 17

What are the different levels of Vit D deficiency?

Answer 17

· Mild = 25-50nmol/l

· Moderate = 12.5=25nmol/l

· Severe= <12.5nmol/l

Question 18

What is the aetiology of vitamin D deficiency?

Answer 18

Intake

· Malabsorption: CF and coeliac (rare)

· Inadequate sunlight exposure: very nordic countries, for activation of vit D

· Inadequate intake: exclusive breastfeed, unsupervised exclusion diet, poor feeding.

Vitamin D metabolism

· Renal failure (low 1ahydrox)

· AEDs (especially PHT)

(low Ca -> high PTH -> high bone resorption to release Ca -> low mineralisation)

Question 19

What is the epidemiology of Vit D deficiency?

Answer 19

High risk in UK.

Question 20

What is in the history and examination of a child with Vit D deficiency?

Answer 20

Irritability and generalised seizures (hypocalcaemia)

Poor growth, delayed dentition, poor motor development.

Bowed legs, knock knees, swollen chostochrondral junction, thickened wrists, frontal bossing, pathological fractures.

In adolescents, carpopedal spasms.

Question 21

What investigations do you do for a child with Vit D deficiency?

Answer 21

Radiology: wrist XR, fraying of metaphyseal surfaces, widened epiphyseal plate.

High PTH, low P, low vit D, low Ca.

Question 22

What is the management of Vit D deficient?

Answer 22

Prevent: expose to sun, eat dairy. Increase fatty fish intake, fortified foods.

Supplement ergocalciferol if vit D deficient. ALFACALCIDIOL if ther eis renal/liver disease. Serial bloods taken, 2-4 month treatment.

Question 23

What are the complications and prognosis of Vit D deficiency?

Answer 23

Rickets. Low maternal vit.D ma cause impairment in brain development.

y abnormalities may take over 2y to resolve after rickets.