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Scurvy: From a Selective Diet in Children with Developmental Delay
THE JOURNAL OF PEDIATRICS • www.jpeds.com
INSIGHTS AND IMAGES
Scurvy: From a Selective Diet in Children with Developmental Delay
A
5-year-old boy with developmental delay presented with painful knees and difficulty walking for a month. He had been diagnosed with autism spectrum disorder and had a selective diet, only consuming bread, snacks, and water. Radiograph of the knee demonstrated a radiodense band at the chondroosseous junction and a translucent band in the metaphysis of the distal femora and the proximal tibiae (Figure, A), which raised a possibility of leukemic bands. However, the child’s complete blood count result was unremarkable. Prior to the scheduled bone marrow biopsy, magnetic resonance imaging (MRI) was obtained, which demonstrated ill-defined T2 hyperintensities in the metaphyses and juxtaosseous soft tissue (Figure, B). The diaphysis was spared. These findings led us to another possible diagnosis of scurvy. His serum vitamin C level was undetectable (<0.2 mg/dL). Vitamin supplementation improved his symptoms over the next 2 months. Scurvy or vitamin C deficiency is an ancient condition that has been known since the “Age of Discovery.”1 In modern developed countries, scurvy has become rare; however, it can still be seen in children with a very selective diet.2 In particular, children with developmental delay are at an increased risk for selective diet, both from the children’s choice and from the parents’ offering a limited variety of food. Affected children may have only musculoskeletal symptoms without classical
hemorrhagic diathesis.3 Radiography may show only some of the classic signs of scurvy (generalized osteoporosis with Wimberger ring, Pelkan spur, Frankel line, and Trummerfeld zone).4 Recently, several reports about MRI findings of scurvy have been published.4,5 They recapitulate classic histologic features of scurvy at bone ends, such as disruption of the chondroosseous junction and metaphyseal reparative changes. Diaphyseal involvement is less common. MRI usually, but not always, enables clinicians to distinguish scurvy from other bone marrow infiltrative disorders, such as leukemia or metastatic neuroblastoma. Clinicians should be aware of scurvy as a differential diagnosis of children with painful limbs, particularly those with developmental delay and selective diet. ■ We thank Dr Gen Nishimura for help with radiologic diagnosis, and Dr Jay Starkey for English editing and helpful comments.
Megumi Seya, MD Department of Pediatrics Atsuhiko Handa, MD Department of Radiology St. Luke’s International Hospital Tokyo, Japan MassGeneral Hospital for Children Harvard Medical School Boston, Massachusetts Daisuke Hasegawa, MD, and Toshihiro Matsui, MD Department of Pediatrics Taiki Nozaki, MD Department of Radiology St. Luke’s International Hospital Tokyo, Japan
Figure. A, Radiograph of the knee demonstrates a radiodense band at the chondroosseous junction (Frankel line; long arrows) and a translucent band in the metaphysis (Trummerfeld zone; short arrows) of the distal femora and the proximal tibiae. B, Magnetic resonance imaging (coronal fat saturation T2weighted imaging) of the knee demonstrates ill-defined T2 hyperintensities in the metaphyses and juxtaosseous soft tissue. The diaphysis was spared.
J Pediatr 2016;■■:■■ 0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved. http://dx.doi.org10.1016/j.jpeds.2016.06.018
References 1. Rajakumar K. Infantile scurvy: a historical perspective. Pediatrics 2001;108:e76. 2. Noble JM, Mandel A, Patterson MC. Scurvy and rickets masked by chronic neurologic illness: revisiting ‘psychologic malnutrition’. Pediatrics 2007;119:e783-90. 3. Alqanatish JT, Alqahtani F, Alsewairi WM, Al-kenaizan S. Childhood scurvy: an unusual cause of refusal to walk in a child. Pediatr Rheumatol Online J 2015;13:23. 4. Weinstein M, Babyn P, Zlotkin S. An orange a day keeps the doctor away: scurvy in the year 2000. Pediatrics 2001;108:e55. 5. Gulko E, Collins LK, Murphy RC, Thornhill BA, Taragin BH. MRI findings in pediatric patients with scurvy. Skeletal Radiol 2014;44:291-7.
1 CRP 5.4.0 DTD ■ YMPD8442_proof ■ July 15, 2016
INSIGHTS AND IMAGES
Scurvy: From a Selective Diet in Children with Developmental Delay
A
5-year-old boy with developmental delay presented with painful knees and difficulty walking for a month. He had been diagnosed with autism spectrum disorder and had a selective diet, only consuming bread, snacks, and water. Radiograph of the knee demonstrated a radiodense band at the chondroosseous junction and a translucent band in the metaphysis of the distal femora and the proximal tibiae (Figure, A), which raised a possibility of leukemic bands. However, the child’s complete blood count result was unremarkable. Prior to the scheduled bone marrow biopsy, magnetic resonance imaging (MRI) was obtained, which demonstrated ill-defined T2 hyperintensities in the metaphyses and juxtaosseous soft tissue (Figure, B). The diaphysis was spared. These findings led us to another possible diagnosis of scurvy. His serum vitamin C level was undetectable (<0.2 mg/dL). Vitamin supplementation improved his symptoms over the next 2 months. Scurvy or vitamin C deficiency is an ancient condition that has been known since the “Age of Discovery.”1 In modern developed countries, scurvy has become rare; however, it can still be seen in children with a very selective diet.2 In particular, children with developmental delay are at an increased risk for selective diet, both from the children’s choice and from the parents’ offering a limited variety of food. Affected children may have only musculoskeletal symptoms without classical
hemorrhagic diathesis.3 Radiography may show only some of the classic signs of scurvy (generalized osteoporosis with Wimberger ring, Pelkan spur, Frankel line, and Trummerfeld zone).4 Recently, several reports about MRI findings of scurvy have been published.4,5 They recapitulate classic histologic features of scurvy at bone ends, such as disruption of the chondroosseous junction and metaphyseal reparative changes. Diaphyseal involvement is less common. MRI usually, but not always, enables clinicians to distinguish scurvy from other bone marrow infiltrative disorders, such as leukemia or metastatic neuroblastoma. Clinicians should be aware of scurvy as a differential diagnosis of children with painful limbs, particularly those with developmental delay and selective diet. ■ We thank Dr Gen Nishimura for help with radiologic diagnosis, and Dr Jay Starkey for English editing and helpful comments.
Megumi Seya, MD Department of Pediatrics Atsuhiko Handa, MD Department of Radiology St. Luke’s International Hospital Tokyo, Japan MassGeneral Hospital for Children Harvard Medical School Boston, Massachusetts Daisuke Hasegawa, MD, and Toshihiro Matsui, MD Department of Pediatrics Taiki Nozaki, MD Department of Radiology St. Luke’s International Hospital Tokyo, Japan
Figure. A, Radiograph of the knee demonstrates a radiodense band at the chondroosseous junction (Frankel line; long arrows) and a translucent band in the metaphysis (Trummerfeld zone; short arrows) of the distal femora and the proximal tibiae. B, Magnetic resonance imaging (coronal fat saturation T2weighted imaging) of the knee demonstrates ill-defined T2 hyperintensities in the metaphyses and juxtaosseous soft tissue. The diaphysis was spared.
J Pediatr 2016;■■:■■ 0022-3476/$ - see front matter. © 2016 Elsevier Inc. All rights reserved. http://dx.doi.org10.1016/j.jpeds.2016.06.018
References 1. Rajakumar K. Infantile scurvy: a historical perspective. Pediatrics 2001;108:e76. 2. Noble JM, Mandel A, Patterson MC. Scurvy and rickets masked by chronic neurologic illness: revisiting ‘psychologic malnutrition’. Pediatrics 2007;119:e783-90. 3. Alqanatish JT, Alqahtani F, Alsewairi WM, Al-kenaizan S. Childhood scurvy: an unusual cause of refusal to walk in a child. Pediatr Rheumatol Online J 2015;13:23. 4. Weinstein M, Babyn P, Zlotkin S. An orange a day keeps the doctor away: scurvy in the year 2000. Pediatrics 2001;108:e55. 5. Gulko E, Collins LK, Murphy RC, Thornhill BA, Taragin BH. MRI findings in pediatric patients with scurvy. Skeletal Radiol 2014;44:291-7.
1 CRP 5.4.0 DTD ■ YMPD8442_proof ■ July 15, 2016