Scurvy Rickets - orthopaedic metabolic bone diseases PDF

Preview

Summary

orthopaedic metabolic bone diseases...

Description

SCURVY Vitamin C (ascorbic acid) deficiency

Epidemiology 



bimodal age bracket o

infants 5-10months

o

men >60 years

location o

wrists, knees, sternal ends of ribs

Risk Factors 

elderly, especially men who live alone



alcoholic



smokers



malabsorptive conditions (Whipple's disease, inflammatory bowel disease, cancer chemotherapy)

Pathophysiology 

Involved in the hydroxylation of proline to hydroxyproline, which is necessary for the formation of collagen.



Defective collagen fibrinogenesis: o

Impairs wound healing

o

Affects bone formation

o

Fragile capillary abnormal bleeding

Symptoms 

Malaise and fatigue



bone pain



myalgia



gum bleeding and loosening of teeth



haematuria



hematemesis



iron deficiency

Physical Examination 

Petechiae and ecchymosis



Keratosis of hair follicle with ‘corkscrew’ hair



Perifollicular haemorrhage



Spontaneous bruising



Failure of wound healing

Investigation X-ray: 

The white line of Frankel o



Widened zone of provisional calcification between epiphysis and metaphysis

Trummerfeld zone o

Transvese radiolucent band in the metaphysis adjacent to the Frankel line

Management 

Medication : Ascorbic acid

Prevention 

Correction of patient diet : Fruits (e.g., citrus), Vegetables (e.g., cauliflower, broccoli)

RICKETS Vitamin D metabolism

Pathology 

Lack of vitamin D lead to inability to calcify the intercellular matrix in the growth plates.



Resulting in softening of the bones and development of deformities in growing bones of children

Causes 

Reduced serum 1,25-OHD (most common cause) with combination of nutritional deficiency and lack of sunlight exposure.



Other causes: o

intestinal malabsorption (eg: coeliac disease),

o

liver disease

o

renal disease

Clinical features 

Infants may present with tetany or convulsions. Later stage, failure to thrive and muscular flaccidity.



Craniotabes: deformity of the skull



Rachitic rosary: enlargement of costochondral junction



Broadening of the ends of long bones: wrists and knees



Harrison's sulcus: lateral indentation of the chest



Lower limb deformities: coxa vara and bowleg deformity

Investigations Biochemistry 

Serum calcium and phosphorus low



Serum alkaline phosphatase high



Calcidiol (25-hydroxyvitamin D) low



Parathyroid hormone increased

Radiological 

X-ray o

Widening of the epiphyseal plates

o

Cupping of the metaphysis

o

Splaying of the metaphysis

Management 

Advice balance diet



Adequate sun exposure



Medical treatment: o

Administration of Vit D in the form of calciferol 400-1000 IU/day with calcium supplements.



Special designed splints (mermaid splints) or orthopaedic shoes for correction of knee deformities.



Operative methods (eg: corrective osteotomies) for moderate or severe deformities....