1. RICKETS DR. MUHAMMAD ABBAS ASSTT. PROFESSOR DEPTT. OF PEDIATRICS SIMS/SERVICES HOSPITAL LAHORE

2. DEFINITIONS RICKETS: Failure of mineralization of growing bone. OSTEOMALACIA: Failure of mature bones to mineralize. OSTEOPOROSIS: Decreased mineralization and osteoid tissue.

3. V ITAMIN ‘D’ METABOLISM 7-DEhydrocholesterolDietary Vit.D2 & D3 UV radiation Vit.D3 Hepatic microsomal 25-Hydroxylase 25 (OH)D3 Kidney-1alpha hydroxylase Low Ca Low PO4 PTH 1,25 (OH)2D3

4. EFFECTS OF VIT. ‘D’ DEFFICIENCY Decreased absorption of calcium and phosphate. Secretion of PTH due to low calcium. Release of calcium from bones. Phosphate excretion from kidneys. Decreased calcification of epiphyseal cartilage. Proliferation of epiphyseal cartilage.

5. ETIOLOGY OF RICKETS  Vit. D disorders  Nutritional deficiency  Congenital deficiency  Secondary - Mal-absorption - Inadequate sun-light o Vit. D dependent type – I o Vit. D dependent type – II o CRF & CLD Calcium deficiency Low in-take Mal-absorption – CD, Dietary inhibitors

6. ETIOLOGY …..  Phosphorus deficiency  In-adequate in-take  Aluminum containing antacids  Renal losses  Vit. D resistant rickets  Fanconi syndrome

7. CLASSIFICATION 1. Hypocalcemia with secondary hyperparathyroidism Vit. D deficient rickets Vit. D dependent Type – 1: Defect in renal 1-alpha hydroxylase Normal 25-OH-D3, low 1, 25 (OH)2-D3 Renal rickets Hepatic rickets Chronic anti-convulsant therapy

8. 2. Primary phosphate deficiency Vit. D resistant rickets (X-linked hypophosphatemia) Most common non-nutritional rickets. Impaired tubular reabsorption of phosphate Fanconi syndrome RTA type – II Oncogenic hypophosphatemia Phosphate deficiency – TPN, low intake 3. Vit. D dependent Type – II: End organ resistance to 1, 25 (OH)2-D3 4.Metaphyseal dysostosis

9. CLINICAL FEATURES  Peak incidence is between 6 months to 2 yrs  Early manifestations Irritability, profuse sweating, hypotonia and respiratory tract infections.  SIGNS OF FLORID RICKETS  General  FTT, listlessness, protruding abdomen, muscle weakness, fractures  Head  Craniotabes, frontal bossing, delayed fontanelle closure, delayed dentition, abnormal head shape  Chest  Rachitic rosary, Harrison groove

10.  Back  Scoliosis, kyphosis, lordosis  Extremities  Enlargment of wrists & ankles  Valgus or varus deformity  Winds wept deformity  Hypocalcemic symptoms  Tetany, seizures, stridor

11. DIAGNOSIS  History  Dietary history  Sunlight exposure  Maternal risk factors  Medications - anti-convulsants, antacids  Liver or intestinal disease  Renal disease  Family history  Examination

12. DIAGNOSIS …..  Lab. investigations  Biochemical changes S/Ca, PO4, ALP, PTH, Vit. D levels  Radiological changes  Active rickets  Healing rickets  Renal function & LFTs  Urinalysis

13. TREATMENT Nutritional rickets: Oral Vitamin D – 2000 - 6000 IU/day for 4 wks OR Intra-muscular – 600,000 IU single dose Vitamin D dependendent rickets: Physiological doses of 1,25(OH)2-D3 (1-2 mcg/day ) Vitamin D resistant rickets: Oral phosphate (0.5-1 g/day) & 1,25 (OH)2-D3 (0.05 mcg/kg/day)

14.  Hepatic Rickets: Oral vitamin D daily with calcium.  Renal Rickets: Controlling hyperphosphatemia & 1,25(OH)2- D3.  Renal tubular Acidosis

15. PROGNOSIS  Healing begins within a few days with Vit. D therapy.  Bone deformities usually disappear within 1-2 years with maintenance dose of Vit. D.  In advanced cases permanent deformity may persist.

16. PREVENTION  Exposure to sunlight  Oral Vit.D 400 IU/day  Vit.D supplementation of premature or breast fed babies whose mothers are not exposed to adequate sunlight.  Vit.D supplementation of pregnant and lactating mothers.

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