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Reflection Questions Rachitic X-ray changes include flaring of the mother and foetus from Vitamin D deficiency. Postnatal costochondral junctions and frayed, cupped long bone administration of Vitamin D to Vitamin D deficient lactating 1. Are there any of the high risk groups metaphyses, with generalised osteopaenia. The typical mothers needs to be approximately 6 times the adult RDI for Vitamin D deficiency amongst the radiological appearances of rickets are shown in Figure 2. to correct their infants Vitamin D deficiency, indicating that c p r Community Pædiatric Review clients that you see? These radiological changes represent ongoing osteoid it is preferable to give Vitamin D replacement to the being laid down in bone that is then poorly mineralised. mother and infant separately. Vitamin D deficiency may be A NATIONAL PUBLICATION FOR COMMUNITY CHILD HEALTH NURSES AND OTHER PROFESSIONALS 2. Consider the issues that you would Radiologically gross metaphyseal changes are frequently prevented in at risk groups, by providing mothers with the not seen in infants under 3 months of age, however RDI of Vitamin D 800iu during pregnancy and lactation, have to discuss with a very fair st generalised osteopaenia remains characteristic, and and 200iu at other times. Vitamin D supplementation of www.rch.org.au/ccch Rickets in the 21 century skinned mother about obtaining occasionally “periosteal reactions” are also seen in the 400iu daily to exclusively breast fed “at risk” infants, or VOL 14 NO 2 MAY 2005 Introduction Causes and risks factors adequate Vitamin D for herself, and long bone X-rays, giving rise to the radiological Vitamin D supplemented infant formula feeds totally Rickets may have been thought to be a “thing World-wide the most common cause of rickets her equally fair skinned breast-fed differential diagnosis of osteomyelitis or scurvy, which prevents the development of Vitamin D deficiency rickets An initiative of the may be excluded on clinical grounds. in otherwise normal infants. Currently the only Centre for Community of the past”. In fact at the beginning of the is due to Vitamin D deficiency. Individuals at infant. Characteristic biochemical features include: commercially available preparation of Vitamin D syrup is Child Health, 20th century, it has been estimated that 85% risk for Vitamin D deficiency include those with: of children living in northern hemisphere urban 1. limited sunlight exposure, 3. What alternatives are available to • raised alkaline phosphatase (bone specific alkaline the multivitamin preparation “Infant Pentavite” which Royal Children’s phosphatase, BSAP), due to increased bone turnover contains 400iu Vitamin D per dose. Hospital, Melbourne industrialised cities had rickets. Major public 2. highly pigmented skin, or those who do not get adequate health initiatives were then introduced to tackle 3. malabsorption syndromes. • raised parathyroid hormone, in response to Food type Vitamin D content – hypocalcaemia this problem. exposure to sunlight to meet the iu per 100gm EXECUTIVE INDEX Specific social and cultural settings that may • low or undetectable 25 OHD3 In “sunny Australia” rickets has not been predispose individuals to Vitamin D deficiency requirements for the body to Cod liver oil 8,000 – 28,000 Other biochemical changes such as serum 1,25 OHD , Rickets in the 21st 1 considered a significant health problem until include: manufacture Vitamin D? 3 Oily fish (e.g. salmon, sardines) 200 – 480 calcium and phosphate levels are variable, and century the last decade or so, when a rising incidence • reduced sun exposure in institutionalised Margarine 200 – 240 dependent on the course of the disease. 1,25 (OH) D of Vitamin D deficiency rickets occurring in and “house bound” individuals, especially 2 3 Eggs 40 – 80 may often be within the upper end of the reference range southern states in particular, has been the chronically disabled, Breast milk 12 – 60 iu/l or frankly elevated. Both serum calcium and serum observed, however as yet there has been no • the practice of Hajjab in the Islamic Infant formulas 400 iu/l phosphate may be low or within the normal range, hence systematic approach to prevention. This article tradition, where women are almost entirely covered for reasons of modesty, measurement of these parameters is unhelpful in Conclusion will outline both the pathophysiology and diagnosis. Serum phosphate is characteristically clinical features of rickets, highlighting • highly pigmented individuals who are Although Vitamin D deficiency rickets almost exclusively decreased in long standing rickets, due to the recognition of infants and children at risk. socially isolated, particularly apartment occurs in a very small at risk group, it remains a major phosphaturic action of PTH. dwellers, who spend minimal time exposed cause of morbidity in affected individuals, with Rickets is broadly defined as undermineralised to direct sunlight. Sources and preparations of Vitamin D bone, particularly at the metaphyseal growth Note from the Editor hypocalcaemic seizures occurring in young infants, bone Vitamin D metabolism (see Figure 1) involves plates, in growing children. The skeleton is a Vitamin D preparations used for Vitamin D deficiency pain with or without gross motor delay in older children, multiple organ systems, namely the skin, liver, • We hope that you continue to gain value from this complex structure comprising mineral ions include ergocalciferol (D ) and cholecalciferol (D ) and and osteomalacia with bone pain and increased risk of kidney, placenta, gut and bone. For this publication. If you are looking for any back copies 2 3 which precipitate on a predominantly collagen either preparation is effective in restoring Vitamin D osteoporotic fractures in at risk mothers. Cultural reaction to take place there must be direct go to: www.rch.org.au/ccch/pub framework of bone matrix proteins, to form the stores in Vitamin D deficiency. The active form of Vitamin sensitivity towards and clinical awareness of this readily exposure to sunlight or other sources of UV B, • Please feel free to let us know of any topics/issues preventable medical condition is needed to ensure that rigid, strong structure characteristic of normal D, 1,25 (OH)2 Vitamin D3 (calcitriol) should not be used as this wavelength of UV radiation is not that you would like to have addressed in this bone. Reinforced concrete may be considered to treat Vitamin D deficiency as it “bypasses” the Vitamin D deficiency rickets once again become “a transmitted through glass or clothing. Both publication. an analogy of bone structure, as the steel regulating step of 1 alpha hydroxylation in the kidney, and disease of the past”. dietary Vitamin D and D , and skin provides tensile strength, analogous with the 2 3 • A parent information sheet is supplied with this may lead to hypercalcaemia. Furthermore, calcitriol does Author synthesized Vitamin D are hydroxylated in the bone collagen and the concrete provides 3 edition. This master copy is designed for you to not build up hepatic stores of 25 OH Vitamin D. Dr Christine Rodda. PhD, FRACP liver to form 25 hydroxyvitamin D3 (25OH D3), photocopy and distribute to parents. Another rigidity analogous with bone mineral ion Expectant and lactating mothers have an increased Head, Paediatric Endocrinology and Diabetes which is the storage form of Vitamin D, and original copy can be accessed from deposition. In growing infants and children, Vitamin D requirement, 2 to 4 times the RDI for healthy Monash Medical Centre, Clayton this hydroxylation step is largely unregulated. www.rch.org.au/ccch/pub or contact bone growth, involving laying down of a adults. Identification of pregnant mothers at risk for Honorary Senior Lecturer Further hydroxylation of 25OH D3 to form 1,25 Wyeth Nutrition on 1800 552 229. collagen framework, which is subsequently Department Biochemistry and Molecular Biology dihydroxyvitamin D (1,25(OH) D ), occurs in Vitamin D deficiency and providing Vitamin D mineralised, predominantly occurs at the meta- 3 2 3 Email: [email protected] Monash University, Clayton the kidney, through the stimulation of 1 alpha supplementation during pregnancy, will protect both the physeal growth plates, within the long bones. hydroxylase by parathyroid hormone (PTH), Unmineralised osteoid, as occurs in rickets in hypocalcaemia (probably indirectly via PTH) c p r Community Pædiatric Review Editors Production Editor SUPPORTED BY AN children, results in soft, pliable bones. Professor Frank Oberklaid Vicki Attenborough Raelene McNaughton EDUCATIONAL GRANT FROM SUPPORTED BY AN EDUCATIONAL GRANT FROM Sharon Foster Carolyn Briggs CENTRE FOR COMMUNITY CHILD HEALTH Michele Meehan Jenny Donovan ROYAL CHILDREN’S HOSPITAL, MELBOURNE Dr Jane Redden-Hoare Libby Dawson TEL 1800 55 2229 For further information contact the Centre for Community Child Health, Royal Children’s Hospital, Melbourne. Phone 03 9345 6150 or Fax 03 9347 2688 www.rch.org.au/ccch TEL 1800 55 2229 © COPYRIGHT 2005. THIS PUBLICATION IS COPYRIGHT. APART FROM ANY FAIR DEALING FOR THE PURPOSE OF PRIVATE STUDY, RESEARCH, CRITICISM OR REVIEW AS PERMITTED UNDER THE COPYRIGHT ACT, NO PART MAY BE REPRODUCED BY ANY CENTRE FOR COMMUNITY CHILD HEALTH, ROYAL CHILDREN’S HOSPITAL, MELBOURNE PROCESS OR PLACED IN COMPUTER MEMORY WITHOUT WRITTEN PERMISSION. ENQUIRIES SHOULD BE MADE TO THE PRODUCTION EDITOR. In populations where there is no Vitamin D supplemented receives 25 OHD3 from transplacental transfer from the foodstuffs, approximately 90% of Vitamin D is obtained maternal circulation. Hence neonatal 25 OHD3 levels from Vitamin D3 synthesised in the epidermis from 7- reflect maternal stores. Premature infants and infants of dehydrocholesterol (7DHC), in a reaction catalysed by Vitamin D deficient mothers are at high risk of Vitamin D ultraviolet B light with a wave-length of 288nm. The deficiency, if Vitamin D supplementation is not instituted remaining 10% of Vitamin D is derived from dietary routinely in the neonatal period. sources such as cod liver oil, eggs or oily fish. Clinical characteristics To maintain normal mineral ion homeostasis, particularly The clinical characteristics of rickets include: in growing children, the Recommended Daily Intake (RDI) of both calcium and Vitamin D (Table 1) must be met. • hypocalcaemic seizures However, the RDI for Vitamin D remains controversial • slowing of linear growth given the wide variation in direct sun exposure around • rachitic rosary the world due to both latitude and lifestyle factors. In • wrist and ankle swelling Australia there are no national RDI’s for Vitamin D, as it is • craniotabes (softening of the skull bones) assumed that Australians have adequate sunlight • a widely patent anterior fontanelle exposure for their Vitamin D requirements. The RDI’s for Vitamin D cited are based on a number of published • frontal bossing recommendations by other authors. • reluctance to weight bear/delayed walking Table 1: Recommended Daily Intake (RDI) for calcium for Hypocalcaemic seizures are a common presenting women and children (Australian National Health and feature of rickets in infants aged less than 9 months, but Medical Research Council 1991) and RDI for iron (from are seen less frequently in older infants and toddlers. other published reports) Older infants and toddlers tend to present with gross motor delay, leg bowing, or occasionally fragility An 18 month old boy with vitamin D deficiency rickets, Category and age Australian RDI RDI for fractures. However, the diagnosis may be made showing moderate skin pigmentation and wrist for calcium Vitamin D incidentally on biochemical testing or on chest X-ray swelling. performed for other reasons, such as investigation for Children 700mg 400iu failure to thrive or fever of unknown origin. Figure 1: Vitamin D/calcium metabolism – PTH: parathyroid hormone, produced by the parathyroid glands; [Ca]: serum 1 – 3 yrs 800mg 400iu calcium; [P]: serum phosphate; UCa: urinary calcium; UP: urinary phosphate 4 – 7 yrs Rachitic bones are characteristically soft and pliable, as a Women 800mg 200iu result of growth of unmineralised bone osteoid. and hypophosphataemia, with feedback inhibition of PTH 20 minutes direct sun exposure of face and forearms 19 – 54 yrs Consequently, wrist and ankle swelling, and leg bowing secretion by 1,25(OH) D . every day or so over summer should prevent the become more marked as infants start weight bearing 2 3 Pregnant women 1100mg 800iu development of Vitamin D deficiency. through crawling, cruising and walking. Rachitic rosary Skin pigmentation also influences the efficiency of Lactating women 1200mg 800iu corresponds to the flaring of the costochondral junctions Vitamin D3 production from sunlight exposure, and In Australia, sun avoidance is increasing due to the Vitamin D production in the skin is inversely proportional development of the “ozone hole” in the earth’s seen radiologically, and almost invariably may be In the absence of Vitamin D fortified foods or Vitamin D to melanin production, as melanin competes with 7DHC atmosphere, resulting in increased exposure to ultraviolet palpated anteriorly along the “nipple line” in infants and supplements, individuals living in polluted cities, at high for UV B protons. Consequently black skinned individuals light and consequent increased risk of skin melanoma young children with rickets, and is sometimes also visible latitudes are at increased risk of Vitamin D deficiency, need approximately 6 times the sunlight exposure due to exposure to UV B. This has prompted the on the anterior chest wall in infants and children in the and child bearing women and infants and young children compared with fair skinned individuals, to maintain development of “broad spectrum” sunscreens which setting of failure to thrive. It is important also to be aware are at greatest risk. In industrially developed countries equivalent Vitamin D status. The amount of UV B block both UV A and UV B from sunlight, and when used that other dietary deficiencies may co-exist in prolonged, today, rickets most commonly occurs in exclusively exposure from sunlight is also inversely proportional to assiduously, these may also reduce skin exposure to UV essentially exclusively breast fed infants and toddlers breast fed infants born in industrialised cities at high latitude, so that UV B exposure is greater in equatorial B sufficiently to cause Vitamin D deficiency, raising the through the second and into the third year of life, latitudes, to mothers who are also at risk of Vitamin D regions compared with that in regions at higher latitudes. issue that very fair skinned individuals at highest risk of including iron deficiency anaemia, and if the mother is a deficiency due to poor sunlight exposure, unfortified food Furthermore, UV B exposure is greater during summer melanoma and other skin cancers may more safely vegan, consuming no dairy products, Vitamin B12 Figure 2: Wrist X-rays of an 18 month old boy with sources (such as milk), social isolation, increased skin than winter at higher latitudes, so that the greatest risk maintain their Vitamin D stores through long term deficiency may also be present. Older infants and vitamin D deficiency rickets. Note the expanded pigmentation, or cultural clothing practices, for Vitamin D deficiency in such regions is late winter and supplementation. children may also have abnormal dentition, due to metaphyses particularly of the radius and ulnar at the independently or in combination. In utero, the foetus early spring. For lightly pigmented individuals, just 10 – enamel hypoplasia. wrist, and generalised osteopaenia.

2 COMMUNITY PAEDIATRIC REVIEW COMMUNITY PAEDIATRIC REVIEW 3 In populations where there is no Vitamin D supplemented receives 25 OHD3 from transplacental transfer from the foodstuffs, approximately 90% of Vitamin D is obtained maternal circulation. Hence neonatal 25 OHD3 levels from Vitamin D3 synthesised in the epidermis from 7- reflect maternal stores. Premature infants and infants of dehydrocholesterol (7DHC), in a reaction catalysed by Vitamin D deficient mothers are at high risk of Vitamin D ultraviolet B light with a wave-length of 288nm. The deficiency, if Vitamin D supplementation is not instituted remaining 10% of Vitamin D is derived from dietary routinely in the neonatal period. sources such as cod liver oil, eggs or oily fish. Clinical characteristics To maintain normal mineral ion homeostasis, particularly The clinical characteristics of rickets include: in growing children, the Recommended Daily Intake (RDI) of both calcium and Vitamin D (Table 1) must be met. • hypocalcaemic seizures However, the RDI for Vitamin D remains controversial • slowing of linear growth given the wide variation in direct sun exposure around • rachitic rosary the world due to both latitude and lifestyle factors. In • wrist and ankle swelling Australia there are no national RDI’s for Vitamin D, as it is • craniotabes (softening of the skull bones) assumed that Australians have adequate sunlight • a widely patent anterior fontanelle exposure for their Vitamin D requirements. The RDI’s for Vitamin D cited are based on a number of published • frontal bossing recommendations by other authors. • reluctance to weight bear/delayed walking Table 1: Recommended Daily Intake (RDI) for calcium for Hypocalcaemic seizures are a common presenting women and children (Australian National Health and feature of rickets in infants aged less than 9 months, but Medical Research Council 1991) and RDI for iron (from are seen less frequently in older infants and toddlers. other published reports) Older infants and toddlers tend to present with gross motor delay, leg bowing, or occasionally fragility An 18 month old boy with vitamin D deficiency rickets, Category and age Australian RDI RDI for fractures. However, the diagnosis may be made showing moderate skin pigmentation and wrist for calcium Vitamin D incidentally on biochemical testing or on chest X-ray swelling. performed for other reasons, such as investigation for Children 700mg 400iu failure to thrive or fever of unknown origin. Figure 1: Vitamin D/calcium metabolism – PTH: parathyroid hormone, produced by the parathyroid glands; [Ca]: serum 1 – 3 yrs 800mg 400iu calcium; [P]: serum phosphate; UCa: urinary calcium; UP: urinary phosphate 4 – 7 yrs Rachitic bones are characteristically soft and pliable, as a Women 800mg 200iu result of growth of unmineralised bone osteoid. and hypophosphataemia, with feedback inhibition of PTH 20 minutes direct sun exposure of face and forearms 19 – 54 yrs Consequently, wrist and ankle swelling, and leg bowing secretion by 1,25(OH) D . every day or so over summer should prevent the become more marked as infants start weight bearing 2 3 Pregnant women 1100mg 800iu development of Vitamin D deficiency. through crawling, cruising and walking. Rachitic rosary Skin pigmentation also influences the efficiency of Lactating women 1200mg 800iu corresponds to the flaring of the costochondral junctions Vitamin D3 production from sunlight exposure, and In Australia, sun avoidance is increasing due to the Vitamin D production in the skin is inversely proportional development of the “ozone hole” in the earth’s seen radiologically, and almost invariably may be In the absence of Vitamin D fortified foods or Vitamin D to melanin production, as melanin competes with 7DHC atmosphere, resulting in increased exposure to ultraviolet palpated anteriorly along the “nipple line” in infants and supplements, individuals living in polluted cities, at high for UV B protons. Consequently black skinned individuals light and consequent increased risk of skin melanoma young children with rickets, and is sometimes also visible latitudes are at increased risk of Vitamin D deficiency, need approximately 6 times the sunlight exposure due to exposure to UV B. This has prompted the on the anterior chest wall in infants and children in the and child bearing women and infants and young children compared with fair skinned individuals, to maintain development of “broad spectrum” sunscreens which setting of failure to thrive. It is important also to be aware are at greatest risk. In industrially developed countries equivalent Vitamin D status. The amount of UV B block both UV A and UV B from sunlight, and when used that other dietary deficiencies may co-exist in prolonged, today, rickets most commonly occurs in exclusively exposure from sunlight is also inversely proportional to assiduously, these may also reduce skin exposure to UV essentially exclusively breast fed infants and toddlers breast fed infants born in industrialised cities at high latitude, so that UV B exposure is greater in equatorial B sufficiently to cause Vitamin D deficiency, raising the through the second and into the third year of life, latitudes, to mothers who are also at risk of Vitamin D regions compared with that in regions at higher latitudes. issue that very fair skinned individuals at highest risk of including iron deficiency anaemia, and if the mother is a deficiency due to poor sunlight exposure, unfortified food Furthermore, UV B exposure is greater during summer melanoma and other skin cancers may more safely vegan, consuming no dairy products, Vitamin B12 Figure 2: Wrist X-rays of an 18 month old boy with sources (such as milk), social isolation, increased skin than winter at higher latitudes, so that the greatest risk maintain their Vitamin D stores through long term deficiency may also be present. Older infants and vitamin D deficiency rickets. Note the expanded pigmentation, or cultural clothing practices, for Vitamin D deficiency in such regions is late winter and supplementation. children may also have abnormal dentition, due to metaphyses particularly of the radius and ulnar at the independently or in combination. In utero, the foetus early spring. For lightly pigmented individuals, just 10 – enamel hypoplasia. wrist, and generalised osteopaenia.

2 COMMUNITY PAEDIATRIC REVIEW COMMUNITY PAEDIATRIC REVIEW 3 Reflection Questions Rachitic X-ray changes include flaring of the mother and foetus from Vitamin D deficiency. Postnatal costochondral junctions and frayed, cupped long bone administration of Vitamin D to Vitamin D deficient lactating 1. Are there any of the high risk groups metaphyses, with generalised osteopaenia. The typical mothers needs to be approximately 6 times the adult RDI for Vitamin D deficiency amongst the radiological appearances of rickets are shown in Figure 2. to correct their infants Vitamin D deficiency, indicating that c p r Community Pædiatric Review clients that you see? These radiological changes represent ongoing osteoid it is preferable to give Vitamin D replacement to the being laid down in bone that is then poorly mineralised. mother and infant separately. Vitamin D deficiency may be A NATIONAL PUBLICATION FOR COMMUNITY CHILD HEALTH NURSES AND OTHER PROFESSIONALS 2. Consider the issues that you would Radiologically gross metaphyseal changes are frequently prevented in at risk groups, by providing mothers with the not seen in infants under 3 months of age, however RDI of Vitamin D 800iu during pregnancy and lactation, have to discuss with a very fair st generalised osteopaenia remains characteristic, and and 200iu at other times. Vitamin D supplementation of www.rch.org.au/ccch Rickets in the 21 century skinned mother about obtaining occasionally “periosteal reactions” are also seen in the 400iu daily to exclusively breast fed “at risk” infants, or VOL 14 NO 2 MAY 2005 Introduction Causes and risks factors adequate Vitamin D for herself, and long bone X-rays, giving rise to the radiological Vitamin D supplemented infant formula feeds totally Rickets may have been thought to be a “thing World-wide the most common cause of rickets her equally fair skinned breast-fed differential diagnosis of osteomyelitis or scurvy, which prevents the development of Vitamin D deficiency rickets An initiative of the may be excluded on clinical grounds. in otherwise normal infants. Currently the only Centre for Community of the past”. In fact at the beginning of the is due to Vitamin D deficiency. Individuals at infant. Characteristic biochemical features include: commercially available preparation of Vitamin D syrup is Child Health, 20th century, it has been estimated that 85% risk for Vitamin D deficiency include those with: of children living in northern hemisphere urban 1. limited sunlight exposure, 3. What alternatives are available to • raised alkaline phosphatase (bone specific alkaline the multivitamin preparation “Infant Pentavite” which Royal Children’s phosphatase, BSAP), due to increased bone turnover contains 400iu Vitamin D per dose. Hospital, Melbourne industrialised cities had rickets. Major public 2. highly pigmented skin, or those who do not get adequate health initiatives were then introduced to tackle 3. malabsorption syndromes. • raised parathyroid hormone, in response to Food type Vitamin D content – hypocalcaemia this problem. exposure to sunlight to meet the iu per 100gm EXECUTIVE INDEX Specific social and cultural settings that may • low or undetectable 25 OHD3 In “sunny Australia” rickets has not been predispose individuals to Vitamin D deficiency requirements for the body to Cod liver oil 8,000 – 28,000 Other biochemical changes such as serum 1,25 OHD , Rickets in the 21st 1 considered a significant health problem until include: manufacture Vitamin D? 3 Oily fish (e.g. salmon, sardines) 200 – 480 calcium and phosphate levels are variable, and century the last decade or so, when a rising incidence • reduced sun exposure in institutionalised Margarine 200 – 240 dependent on the course of the disease. 1,25 (OH) D of Vitamin D deficiency rickets occurring in and “house bound” individuals, especially 2 3 Eggs 40 – 80 may often be within the upper end of the reference range southern states in particular, has been the chronically disabled, Breast milk 12 – 60 iu/l or frankly elevated. Both serum calcium and serum observed, however as yet there has been no • the practice of Hajjab in the Islamic Infant formulas 400 iu/l phosphate may be low or within the normal range, hence systematic approach to prevention. This article tradition, where women are almost entirely covered for reasons of modesty, measurement of these parameters is unhelpful in Conclusion will outline both the pathophysiology and diagnosis. Serum phosphate is characteristically clinical features of rickets, highlighting • highly pigmented individuals who are Although Vitamin D deficiency rickets almost exclusively decreased in long standing rickets, due to the recognition of infants and children at risk. socially isolated, particularly apartment occurs in a very small at risk group, it remains a major phosphaturic action of PTH. dwellers, who spend minimal time exposed cause of morbidity in affected individuals, with Rickets is broadly defined as undermineralised to direct sunlight. Sources and preparations of Vitamin D bone, particularly at the metaphyseal growth Note from the Editor hypocalcaemic seizures occurring in young infants, bone Vitamin D metabolism (see Figure 1) involves plates, in growing children. The skeleton is a Vitamin D preparations used for Vitamin D deficiency pain with or without gross motor delay in older children, multiple organ systems, namely the skin, liver, • We hope that you continue to gain value from this complex structure comprising mineral ions include ergocalciferol (D ) and cholecalciferol (D ) and and osteomalacia with bone pain and increased risk of kidney, placenta, gut and bone. For this publication. If you are looking for any back copies 2 3 which precipitate on a predominantly collagen either preparation is effective in restoring Vitamin D osteoporotic fractures in at risk mothers. Cultural reaction to take place there must be direct go to: www.rch.org.au/ccch/pub framework of bone matrix proteins, to form the stores in Vitamin D deficiency. The active form of Vitamin sensitivity towards and clinical awareness of this readily exposure to sunlight or other sources of UV B, • Please feel free to let us know of any topics/issues preventable medical condition is needed to ensure that rigid, strong structure characteristic of normal D, 1,25 (OH)2 Vitamin D3 (calcitriol) should not be used as this wavelength of UV radiation is not that you would like to have addressed in this bone. Reinforced concrete may be considered to treat Vitamin D deficiency as it “bypasses” the Vitamin D deficiency rickets once again become “a transmitted through glass or clothing. Both publication. an analogy of bone structure, as the steel regulating step of 1 alpha hydroxylation in the kidney, and disease of the past”. dietary Vitamin D and D , and skin provides tensile strength, analogous with the 2 3 • A parent information sheet is supplied with this may lead to hypercalcaemia. Furthermore, calcitriol does Author synthesized Vitamin D are hydroxylated in the bone collagen and the concrete provides 3 edition. This master copy is designed for you to not build up hepatic stores of 25 OH Vitamin D. Dr Christine Rodda. PhD, FRACP liver to form 25 hydroxyvitamin D3 (25OH D3), photocopy and distribute to parents. Another rigidity analogous with bone mineral ion Expectant and lactating mothers have an increased Head, Paediatric Endocrinology and Diabetes which is the storage form of Vitamin D, and original copy can be accessed from deposition. In growing infants and children, Vitamin D requirement, 2 to 4 times the RDI for healthy Monash Medical Centre, Clayton this hydroxylation step is largely unregulated. www.rch.org.au/ccch/pub or contact bone growth, involving laying down of a adults. Identification of pregnant mothers at risk for Honorary Senior Lecturer Further hydroxylation of 25OH D3 to form 1,25 Wyeth Nutrition on 1800 552 229. collagen framework, which is subsequently Department Biochemistry and Molecular Biology dihydroxyvitamin D (1,25(OH) D ), occurs in Vitamin D deficiency and providing Vitamin D mineralised, predominantly occurs at the meta- 3 2 3 Email: [email protected] Monash University, Clayton the kidney, through the stimulation of 1 alpha supplementation during pregnancy, will protect both the physeal growth plates, within the long bones. hydroxylase by parathyroid hormone (PTH), Unmineralised osteoid, as occurs in rickets in hypocalcaemia (probably indirectly via PTH) c p r Community Pædiatric Review Editors Production Editor SUPPORTED BY AN children, results in soft, pliable bones. Professor Frank Oberklaid Vicki Attenborough Raelene McNaughton EDUCATIONAL GRANT FROM SUPPORTED BY AN EDUCATIONAL GRANT FROM Sharon Foster Carolyn Briggs CENTRE FOR COMMUNITY CHILD HEALTH Michele Meehan Jenny Donovan ROYAL CHILDREN’S HOSPITAL, MELBOURNE Dr Jane Redden-Hoare Libby Dawson TEL 1800 55 2229 For further information contact the Centre for Community Child Health, Royal Children’s Hospital, Melbourne. Phone 03 9345 6150 or Fax 03 9347 2688 www.rch.org.au/ccch TEL 1800 55 2229 © COPYRIGHT 2005. THIS PUBLICATION IS COPYRIGHT. APART FROM ANY FAIR DEALING FOR THE PURPOSE OF PRIVATE STUDY, RESEARCH, CRITICISM OR REVIEW AS PERMITTED UNDER THE COPYRIGHT ACT, NO PART MAY BE REPRODUCED BY ANY CENTRE FOR COMMUNITY CHILD HEALTH, ROYAL CHILDREN’S HOSPITAL, MELBOURNE PROCESS OR PLACED IN COMPUTER MEMORY WITHOUT WRITTEN PERMISSION. ENQUIRIES SHOULD BE MADE TO THE PRODUCTION EDITOR. Reflection Questions Rachitic X-ray changes include flaring of the mother and foetus from Vitamin D deficiency. Postnatal costochondral junctions and frayed, cupped long bone administration of Vitamin D to Vitamin D deficient lactating 1. Are there any of the high risk groups metaphyses, with generalised osteopaenia. The typical mothers needs to be approximately 6 times the adult RDI for Vitamin D deficiency amongst the radiological appearances of rickets are shown in Figure 2. to correct their infants Vitamin D deficiency, indicating that c p r Community Pædiatric Review clients that you see? These radiological changes represent ongoing osteoid it is preferable to give Vitamin D replacement to the being laid down in bone that is then poorly mineralised. mother and infant separately. Vitamin D deficiency may be A NATIONAL PUBLICATION FOR COMMUNITY CHILD HEALTH NURSES AND OTHER PROFESSIONALS 2. Consider the issues that you would Radiologically gross metaphyseal changes are frequently prevented in at risk groups, by providing mothers with the not seen in infants under 3 months of age, however RDI of Vitamin D 800iu during pregnancy and lactation, have to discuss with a very fair st generalised osteopaenia remains characteristic, and and 200iu at other times. Vitamin D supplementation of www.rch.org.au/ccch Rickets in the 21 century skinned mother about obtaining occasionally “periosteal reactions” are also seen in the 400iu daily to exclusively breast fed “at risk” infants, or VOL 14 NO 2 MAY 2005 Introduction Causes and risks factors adequate Vitamin D for herself, and long bone X-rays, giving rise to the radiological Vitamin D supplemented infant formula feeds totally Rickets may have been thought to be a “thing World-wide the most common cause of rickets her equally fair skinned breast-fed differential diagnosis of osteomyelitis or scurvy, which prevents the development of Vitamin D deficiency rickets An initiative of the may be excluded on clinical grounds. in otherwise normal infants. Currently the only Centre for Community of the past”. In fact at the beginning of the is due to Vitamin D deficiency. Individuals at infant. Characteristic biochemical features include: commercially available preparation of Vitamin D syrup is Child Health, 20th century, it has been estimated that 85% risk for Vitamin D deficiency include those with: of children living in northern hemisphere urban 1. limited sunlight exposure, 3. What alternatives are available to • raised alkaline phosphatase (bone specific alkaline the multivitamin preparation “Infant Pentavite” which Royal Children’s phosphatase, BSAP), due to increased bone turnover contains 400iu Vitamin D per dose. Hospital, Melbourne industrialised cities had rickets. Major public 2. highly pigmented skin, or those who do not get adequate health initiatives were then introduced to tackle 3. malabsorption syndromes. • raised parathyroid hormone, in response to Food type Vitamin D content – hypocalcaemia this problem. exposure to sunlight to meet the iu per 100gm EXECUTIVE INDEX Specific social and cultural settings that may • low or undetectable 25 OHD3 In “sunny Australia” rickets has not been predispose individuals to Vitamin D deficiency requirements for the body to Cod liver oil 8,000 – 28,000 Other biochemical changes such as serum 1,25 OHD , Rickets in the 21st 1 considered a significant health problem until include: manufacture Vitamin D? 3 Oily fish (e.g. salmon, sardines) 200 – 480 calcium and phosphate levels are variable, and century the last decade or so, when a rising incidence • reduced sun exposure in institutionalised Margarine 200 – 240 dependent on the course of the disease. 1,25 (OH) D of Vitamin D deficiency rickets occurring in and “house bound” individuals, especially 2 3 Eggs 40 – 80 may often be within the upper end of the reference range southern states in particular, has been the chronically disabled, Breast milk 12 – 60 iu/l or frankly elevated. Both serum calcium and serum observed, however as yet there has been no • the practice of Hajjab in the Islamic Infant formulas 400 iu/l phosphate may be low or within the normal range, hence systematic approach to prevention. This article tradition, where women are almost entirely covered for reasons of modesty, measurement of these parameters is unhelpful in Conclusion will outline both the pathophysiology and diagnosis. Serum phosphate is characteristically clinical features of rickets, highlighting • highly pigmented individuals who are Although Vitamin D deficiency rickets almost exclusively decreased in long standing rickets, due to the recognition of infants and children at risk. socially isolated, particularly apartment occurs in a very small at risk group, it remains a major phosphaturic action of PTH. dwellers, who spend minimal time exposed cause of morbidity in affected individuals, with Rickets is broadly defined as undermineralised to direct sunlight. Sources and preparations of Vitamin D bone, particularly at the metaphyseal growth Note from the Editor hypocalcaemic seizures occurring in young infants, bone Vitamin D metabolism (see Figure 1) involves plates, in growing children. The skeleton is a Vitamin D preparations used for Vitamin D deficiency pain with or without gross motor delay in older children, multiple organ systems, namely the skin, liver, • We hope that you continue to gain value from this complex structure comprising mineral ions include ergocalciferol (D ) and cholecalciferol (D ) and and osteomalacia with bone pain and increased risk of kidney, placenta, gut and bone. For this publication. If you are looking for any back copies 2 3 which precipitate on a predominantly collagen either preparation is effective in restoring Vitamin D osteoporotic fractures in at risk mothers. Cultural reaction to take place there must be direct go to: www.rch.org.au/ccch/pub framework of bone matrix proteins, to form the stores in Vitamin D deficiency. The active form of Vitamin sensitivity towards and clinical awareness of this readily exposure to sunlight or other sources of UV B, • Please feel free to let us know of any topics/issues preventable medical condition is needed to ensure that rigid, strong structure characteristic of normal D, 1,25 (OH)2 Vitamin D3 (calcitriol) should not be used as this wavelength of UV radiation is not that you would like to have addressed in this bone. Reinforced concrete may be considered to treat Vitamin D deficiency as it “bypasses” the Vitamin D deficiency rickets once again become “a transmitted through glass or clothing. Both publication. an analogy of bone structure, as the steel regulating step of 1 alpha hydroxylation in the kidney, and disease of the past”. dietary Vitamin D and D , and skin provides tensile strength, analogous with the 2 3 • A parent information sheet is supplied with this may lead to hypercalcaemia. Furthermore, calcitriol does Author synthesized Vitamin D are hydroxylated in the bone collagen and the concrete provides 3 edition. This master copy is designed for you to not build up hepatic stores of 25 OH Vitamin D. Dr Christine Rodda. PhD, FRACP liver to form 25 hydroxyvitamin D3 (25OH D3), photocopy and distribute to parents. Another rigidity analogous with bone mineral ion Expectant and lactating mothers have an increased Head, Paediatric Endocrinology and Diabetes which is the storage form of Vitamin D, and original copy can be accessed from deposition. In growing infants and children, Vitamin D requirement, 2 to 4 times the RDI for healthy Monash Medical Centre, Clayton this hydroxylation step is largely unregulated. www.rch.org.au/ccch/pub or contact bone growth, involving laying down of a adults. Identification of pregnant mothers at risk for Honorary Senior Lecturer Further hydroxylation of 25OH D3 to form 1,25 Wyeth Nutrition on 1800 552 229. collagen framework, which is subsequently Department Biochemistry and Molecular Biology dihydroxyvitamin D (1,25(OH) D ), occurs in Vitamin D deficiency and providing Vitamin D mineralised, predominantly occurs at the meta- 3 2 3 Email: [email protected] Monash University, Clayton the kidney, through the stimulation of 1 alpha supplementation during pregnancy, will protect both the physeal growth plates, within the long bones. hydroxylase by parathyroid hormone (PTH), Unmineralised osteoid, as occurs in rickets in hypocalcaemia (probably indirectly via PTH) c p r Community Pædiatric Review Editors Production Editor SUPPORTED BY AN children, results in soft, pliable bones. Professor Frank Oberklaid Vicki Attenborough Raelene McNaughton EDUCATIONAL GRANT FROM SUPPORTED BY AN EDUCATIONAL GRANT FROM Sharon Foster Carolyn Briggs CENTRE FOR COMMUNITY CHILD HEALTH Michele Meehan Jenny Donovan ROYAL CHILDREN’S HOSPITAL, MELBOURNE Dr Jane Redden-Hoare Libby Dawson TEL 1800 55 2229 For further information contact the Centre for Community Child Health, Royal Children’s Hospital, Melbourne. Phone 03 9345 6150 or Fax 03 9347 2688 www.rch.org.au/ccch TEL 1800 55 2229 © COPYRIGHT 2005. THIS PUBLICATION IS COPYRIGHT. APART FROM ANY FAIR DEALING FOR THE PURPOSE OF PRIVATE STUDY, RESEARCH, CRITICISM OR REVIEW AS PERMITTED UNDER THE COPYRIGHT ACT, NO PART MAY BE REPRODUCED BY ANY CENTRE FOR COMMUNITY CHILD HEALTH, ROYAL CHILDREN’S HOSPITAL, MELBOURNE PROCESS OR PLACED IN COMPUTER MEMORY WITHOUT WRITTEN PERMISSION. ENQUIRIES SHOULD BE MADE TO THE PRODUCTION EDITOR.