Rickets - Standard Treatment Guidelines

Rickets is a softening of bones in children due to deficiency or impaired metabolism of vitamin D, phosphorus or calcium, leading to fractures and deformity. Rickets is among the most frequent childhood diseases in many developing countries. The predominant cause is a vitamin D deficiency, but lack of adequate calcium in the diet may also lead to rickets (cases of severe diarrhea and vomiting may be the cause of the deficiency). Although it can occur in adults, the majority of cases occur in children suffering from severe malnutrition

Ministry of Health and Family Welfare, Government of India has issued the Standard Treatment Guidelines for Rickets. Following are the major recommendations :

Case Definition:

The primary cause of rickets is a vitamin D deficiency. Vitamin D is required for proper calcium absorption from the gut. Sunlight, especially ultraviolet light, lets human skin cells convert Vitamin D from an inactive to active state. In the absence of vitamin D, dietary calcium is not properly absorbed, resulting in hypocalcaemia, leading to skeletal and dental deformities and neuromuscular symptoms

Types:

• Nutritional Rickets


• Vitamin D Resistant Rickets


• Vitamin D Dependant Rickets

o Type I

o Type II

• Congenital Rickets

Incidence of Condition In Our Country

In developed countries, rickets is a rare disease (incidence of less than 1 in 200,000). Children ages 6 months to 24 months are at highest risk, because their bones are rapidly growing.Mother's milk gives adequate calcium and vitamin-D so nutritional rickets develops once breast feeding is stopped. Renal or vitamin-D resistant rickets develops in children of 5-8 years of age.

Differential Diagnosis

Differential diagnosis of reickets include

Hypophosphatasia

Metaphyseal dysplasia

Blounts disease

Prevention And Counselling

A sufficient amount of ultraviolet B light in sunlight each day and adequate supplies of calcium and phosphorus in the diet can prevent rickets. Recommendations are for 400 international units (IU) of vitamin D a day for infants and children.

Optimal Diagnostic Criteria, Investigations, Treatment & Referral Criteria

SITUATION 1: At Secondary Hospital / Non Metro situation : Optimal standards of Treatment in situations where technology and resources are limited

Clinical diagnosis:

Signs and symptoms of rickets include:

1. Bone pain or tenderness, dental problems, muscle weakness (rickety myopathy or "floppy baby syndrome" or "slinky baby", increased tendency for fractures


2. Skeletal deformity o Genu varum, Genu Valgum, Cranial, spinal, and pelvic deformities


3. Growth disturbance


4. Tetany, Craniotabes, Costochondral swelling "rickety rosary", Harrison's groove


5. Double malleoli sign due to metaphyseal hyperplasia


6. Widening of wrist raises early suspicion, it is due to metaphysial cartilage hyperplasia.

Investigations:

Alkaline Phosphatase

Serum Calcium

Serum Phosphorus

X rays of the deformed part

Treatment:

The goals of treatment are to relieve symptoms and correct the cause of the condition.

Replacing calcium, phosphorus, and vitamin D, Exposure to moderate amounts of sunlight is encouraged. Positioning or bracing may be used to reduce or prevent deformities. Skeletal deformities may require corrective surgery later on.

Standard Operating Procedure

In Patient : For corrective surgery

Out Patient : supplementation and bracing

Day Care: Injectable form of Vit D

Referral criteria:

Vitamin D resistant Rickets

Hypophosphatemic Rieckets

SITUATION 2: At Super Specialty facility in Metro Location where higher end technology is available

Clinical diagnosis:

Signs and symptoms of rickets include:

1. Bone pain or tenderness, dental problems, muscle weakness (rickety myopathy or "floppy baby syndrome" or "slinky baby", increased tendency for fractures


2. Skeletal deformity o Genu varum, Genu Valgum, Cranial, spinal, and pelvic deformities


3. Growth disturbance


4. Tetany, Craniotabes, Costochondral swelling "rickety rosary", Harrison's groove


5. Double malleoli sign due to metaphyseal hyperplasia


6. Widening of wrist raises early suspicion, it is due to metaphysial cartilage hyperplasia.

Investigations:

Alkaline Phosphatase

Serum Calcium

Serum Phosphorus

X rays of the deformed part

25 hydroxy – Vit D level

1,25 – dihydroxy- Vit D level

24 hours urinary Ca and Phosphorus levels

Treatment:

not applicable

Standard Operating Procedure

In Patient : For corrective surgery and Recombinant Growth hormone therapy for Hypophosphatemic rickets

Out Patient : supplementation and bracing

Day Care: Injectable form of Vit D

Referral criteria:

not applicable

WHO DOES WHAT? AND TIMELINES

Doctor

Early diagnosis and appropriate treatment. Counsel the patient for prevention and dietary advice.

Nurse

Counselling the patient

Technician

Appropriate bracing manufacturing and application Physiotherapy

Resources Required For One Patient / Procedure (Patient Weight 60 Kgs)

(Units to be specified for human resources, investigations, drugs and consumables and equipment. Quantity to also be specified)

Guidelines by The Ministry of Health and Family Welfare :

Dr. P.K. DAVE, Rockland Hospital, New Delhi, Dr. P.S. Maini, Fortis Jessa Ram Hospital, New Delhi

Reviewed By

Dr. V.K. SHARMA Professor Central Instiute of Orthopaedics Safdarjung Hospital New Delhi