6. Rickets of vitamin D deficiency

8. Rickets of vitamin D deficiency is a common nutritional disorder under 3 yrs of age , is the term signifying a failure in mineralization of growing bone or osteoid tissue . Definition

9. Physical and metabolic property and source of VD

10. Characteristics Fat-soluble stable to heat, acid, alkali and oxidation bile is necessary for absorption.

11. 7-dehydrocholesterol in skin irradiated by ultraviolet rays converts into cholecalciferol (endogenous VD 3 ) fish-liver oils; egg yolks; butter; VD fortified milk; margarine Sources

12. Cod fish Mackerel salmon Sardines

13. Metabolism and regulation Dietary source 7-Dehydrocholesterol in skin Either vitamin D 2 or D 3 ultraviolet radiation (296 -- 310 nm ) Vitamin D 3 (--) Liver 25-hydroxylase 25(OH)D 3 Kidney 1- hydroxylase Hypocalcemia (+) Hypophosphatemia (+) (+) Parathyroid hormone Hyperphosphatemia (--) (--) Calcitonin (+) Hypercalcemia 1,25(OH) 2 D 3

14. 296—310nm

15. Promotes intestinal calcium and phosphate absorption, presumably by affecting permeability of intestinal membrane Promotes bone dissolution and mineralization Promotes reabsorption of calcium and phosphorus from renal tubules Physical property ( 1, 25(OH) 2 VD 3 )

17. Inadequate direct exposure to ultraviolet rays in sunlight Inadequate intake of VD Rapid growth Malabsorption Drugs Etiology

19. 日光照射不足

21. the abundant laying down of osteoid tissue epiphyseal cartilage cells grow very rapidly widening of the epiphysis line Long bone Rachitic rosary Bracelets of wrist and ankle flat bone Craniotabes Square head disorder of new bone formation the bones are soft Bowlegs and knock-knees Pigeon breast Pathology

22. di c aphysis

23. Mechanism of rickets

24. VD deficiency reduction of intestinal absorption of calcium and phosphate Hypocalcemia Parathyroid hormone hyperfunction hypofunction Excretion of urinary P↑ decalcification of mobilization of bone old bone↑ Calcium into blood Serum p ↓ Ca normal or slightly↓ Ca X p↓ Failure of Calcification of Osteoid tissue Ca++↓ Accumulation of osteoid tissue Rickets Tetany

25. skeletal deformities muscular relaxation psychoneurological symptoms Clinical manifestation

27. Psychoneurological symptoms irritability restlessness night startle increased sweating occipital baldness Early active stage

30. occipital baldness

31. skeletal changes retardation of motor development Active stage

32. Craniotabes . 3-6 month old infants A pingpong ball sensation Square head over 5-9 months old saddle shaped or cross shaped head Delayed closure of anterior fontanel till 2-3 yrs old, or enlarged fontanel Delayed dentition till 10 months or 1 year old. defects of the enamel caries Head

33. Craniotabes

34. Active stage

35. Active stage

36. Frontal bossing Active stage

37. Vitamin D Deficiency: Rickets 1-1.5

38. Rachitic rosary Costochondral prominence or beading of the ribs the sides of the thorax become flattened the longitudinal grooves develop posterior to the rosary Harrison’s groove Along the lower border of the chest develops a horizontal depression, corresponds with the costal insertions of the diaphragm Pigeon breast deformity The sternum with its adjacent cartilage appears to be projected forward. . Thorax

39. Rachitic rosary

40. Rachitic rosary

41. Harrison’s groove

43. Funnel deformity

44. Bracelets of wrist and ankle The enlarged epiphysis can be seen or palpated Bowlegs and knock-knees bending of the softened shafts of the femur tibia and fibula. “ X” or “O” shaped legs coxa variation and greenstick fractures Extremities

45. Bracelets of wrist

46. Bracelets of ankle

50. Scoliosis kyphosis The pelvis entrance is narrowed by a forward projection of the promontory; The exit , by a forward displacement of the caudal part of the sacrum and the coccyx. Spinal column and pelvis

51. Scoliosis

52. kyphosis

53. relaxation of the ligaments the muscles are poorly developed and lack tone potbelly weakness Others the child is indifferent delayed speaking and dentition. Abdominal muscles Gastric and intestinal walls Ligaments and muscles

54. potbelly

55. Biochemical changes Serum calcium low < 2.2 mmol/l sometimes normal with the help of parathormone Serum phosphate below 1.5mmol/l calcium multiply phosphate low AKP elevated Active stage

56. The distal ends appear widened, concave, frayed. The distance from the distal ends of the ulna and radius to the metacarpal bones is increased doesn’t appear on the X-ray. The density of the shafts is decreased . X-ray changes Active stage

58. metacarpal

59. The symptoms disappear after treatment. Child becomes more active, normal muscular tone. Recovery stage

62. diaphyseal

63. Clinical symptoms disappear blood biochemistry normal x-ray examination different degrees of skeletal deformity Sequelae stage

65. Congenital rickets Pregnant women suffer from severe osteomalacia or VD deficiency due to less outdoor activity and poorer in dietary intake. The baby may be born with congenital rickets. two clinical types besides typical rickets

67. Late rickets It occurs over 10-14 yrs. Females>males, with the symptoms of pain in both lower extremities i.e. ankle or knees, restlessness, increased sweating, delayed dentition, but less signs. X-ray reveals typical rachitic changes. two clinical types besides typical rickets

68. history of inadequate intake of VD clinical observation classical radiological biochemical changes Low serum 25(OH)D 3 is more reliable Diagnosis

69. Degree mild moderate severe Skeletal deformity none or abnormal exist obvious Skull and thorax Serum calcium normal Serum phosphate normal/ Akp normal/ x-ray normal typical signs deformity fracture clinical degree

70. Congenital hypothyroidism Characteristic features : dry and coarse skin myxedema subnormal body temperature the lower segment is shorter than the upper retardation of physical and mental development The serum calcium and phosphate are normal X-ray shows a delay in appearance of ossification centers in bone. Differential diagnosis

71. Congenital hypothyroidism

72. Chondrodystrophy Special features Protrusion of forehead broad with bulging of metaphysis short and broad fingers lordosis of lumbar spine, kyphosis of hip X-ray: long bones are short, broad and curved metaphysis are broad. Differential diagnosis

74. Recurrent respiratory infection Associated with iron deficiency anemia and malnutrition. Hypocalcemic tetany Bony deformities and fractures Stunted growth Complication

75. Aim: Control the development of rickets Prevent malformation Treatment

76. General therapy Insist on breast feeding from birth Supply with diet rich in VD Stay outdoor for enough sunlight Don’t sit ,stand and walk too much early for prevention of deformities in active rachitic infants Treatment

77. food VitaminD( µ g) (40iu=1 µ g) Cod liver oil 1tb 34.5 Most fish, 3.5 oz 8 Egg, cooked 0.65 Beef, 3.5 oz 0.18

78. VD therapy Preventive dosage: 400-800 Iu/day ① Treatment

80. Calcium therapy Calcium gluconate 1-3g/d 10% calcium chloride active calcium Treatment

81. Genaral therapy Prevention therapy The daily requirement of VD is 400 Iu Prematurely born infants: The preventive dosage is 800 Iu/d in the first 3 m, then 400Iu/d No necessity for the child over 3 yrs old VD should also be pregnant and lactating mothers. Prevention

82. Tetany of Vitamin D deficiency

83. rachitic tetany infantile tetany rachitic hypocalcemic convulsion characteristic convulsion carpopedal spasms laryngeal stridor Definition

84. VD deficiency reduction of intestinal absorption of calcium and phosphate Hypocalcemia Parathyroid hormone hyperfunction hypofunction Excretion of urinary P↑ decalcification of mobilization of bone old bone↑ Calcium into blood Serum p ↓ Ca normal or slightly↓ Ca X p↓ Failure of Calcification of Osteoid tissue Ca++↓ Accumulation of osteoid tissue Rickets Tetany

85. Serum calcium is regulated by VD parathormone calcitonin normal lever 2.2-2.7 mmol/l. Chemical pathology

87. Ca ++ is regulated by: blood pH serum protein serum phosphate

88. In early stage of VD deficiency, the serum calcium falls as with failure of parathyroid compensation The child receives more sunlight in the summer and spring, or at the beginning of VD therapy, large amount of calcium deposits on the bone with less decalcification of the old bone Serum phosphate release from tissues during infection, fever and hunger, the increase of serum phosphate lowers the level of serum calcium Ca ++ may be low when the child is injected with an alkaline drug or albumin Etiology

89. Typical symptom Convulsion Carpopedal spasm Laryngospasm Clinical manifestation

91. Carpopedal spasm

93. Latent signs Chvostek’s sign Peroneal reflex Trousseau’s sign Clinical manifestation

95. Systolic diastolic ischemia Clinical manifestation

96. symptoms of tetany the combined presence of rickets low serum calcium lever (especially Ca ++ ) Diagnosis

97. Other afebrile convulsion Hypoglycemia blood glucose is below 2.2mmol/l the convulsion can be controlled by glucose injection. Differential Diagnosis

98. Hypomagnesemia insufficient intake ( artificial feeding) infantile diarrhea, stearrhea hereditary magnesium malabsorptive defect blood magnesium is below 0.58 mmol/l treatment with magnesium can stop the seizures. (25%Mgso 4 , oral or injection) Differential Diagnosis

99. Epilepsy repeated attacks extremities bend loss of consciousness Electroencephalographic(EEG) changes no response to calcium therapy Differential Diagnosis

100. CNS Infection meningitis, encephalitis, cerebral abscess fever, poisoning symptoms, coma, intracranial hypertension cerebrospinal fluid (CSF) changes Differential Diagnosis

101. Acute laryngitis accompanies with upper respiratory infection characteristic hoarseness of voice whooping cough air way obstruction in severe cases Differential Diagnosis

102. Emergent therapy Stop the convulsion a. 5% chloral hydrate 1-2 ml/kg po/pr b. Luminal 5-7mg/kg im c. Valium 0.2-0.3 mg/kg im/iv Control the laryngospasm a. Oxygen inhalation b. Endotracheal intubation or trache’ostomy if necessary. Treatment

103. Calcium therapy a. 10% calcium Gluconate 10ml bid i.v; or more b. 10% calcium chloride 5-10ml tid p.o Caution a. Injective of calcium must slow, >10 min b. Don’t mixed with milk c. Other elements supplement i.e. K+, Na+, Mg++ Treatment

104. VD therapy See the rachitic VD therapy It will begin at 1 week after hypocalcemia is controlled . Treatment

105. Sources of VD Metabolism and regulation of VD Etiology of Rickets Symptoms and signs of Rickets in active stage Treatment of Rickets Clinical manifestation of Tetany Emergent therapy of Tetany EMPHASIS

106. THANK YOU