omneya magdy omar , amira hamed - espe...

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58ESPE Poster presented at: Omneya Magdy Omar, Amira Hamed Pediatrics, Alexandria University, Alexandria, Egypt Vitamin D deficiency is prevalent in infants and children in underdeveloped countries. Secondary myelofibrosis has been reported as a complication of severe rickets and in these children anemia, myeloid metaplasia and bone aplasia strongly suggested myelofibrosis Correspondence [email protected] No conflict of interest We report a case of myelofibrosis in two years old boy with severe vitamin D deficiency rickets and hepatosplenomegaly. He presented with grossly delayed gross milestones but his intellectual development was normal. The nutritional intake was very poor, comprising of breastfeeding and small quantities of home-cooked cereals. The child severely malnourished, with weight in -2.4 z score and length was -4.9 z score. he was anemic and had a wide open anterior fontanel and signs of florid clinical rickets. There were no neurological abnormalities except for mild generalized hypotonia. Radiological survey of the bony skeleton showed severe generalized osteopenia. extensive rickets of the thoracic cage and ends of long bones with splaying, cupping and fraying of metaphyses. No pathological features were noted. There was focal kyphosis in dorsolumbar region. Bone trephine clearly depicted replacement of hemopoiesis by fibroblasts with very occasional erythroid and myeloid precursors and no megakaryocytes were seen. Reticulin stain revealed significantly increased fibrosis, findings were consistent with myelofibrosis. 1. Cooperberg AA, Singer OP. Reversible myelofibrosis due to vitamin D deficiency rickets. Can. Med. Assoc. J., 1966;94:392. 2. Holick MF. Non-calcemic actions of 1,25- dihydroxyvitamin 03 and clinical applications. Bone. 1995. 3. Bhakhri BK & Debata PK. Nutritional Rickets Presenting with Myelofibrosis. Indian J Pediatr (2010) 77:1437–39 Rickets should be considered as one of the conditions that can lead to severe hematological disorders in infants P3-041 Omneya Magdy Omar DOI: 10.3252/pso.eu.58ESPE.2019 Bone, growth plate and mineral metabolism

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Page 1: Omneya Magdy Omar , Amira Hamed - ESPE Abstractsabstracts.eurospe.org/hrp/0092/eposters/hrp0092p3-41_eposter.pdf · Omneya Magdy Omar , Amira Hamed . I Alexandria I Alexandria, Egypt

58ES

PE

Poster presented at:

Omneya Magdy Omar, Amira Hamed

Pediatrics, Alexandria University, Alexandria, Egypt

Vitamin D deficiency is prevalent in infants

and children in underdeveloped countries.

Secondary myelofibrosis has been reported

as a complication of severe rickets and in

these children anemia, myeloid metaplasia

and bone aplasia strongly suggested

myelofibrosis

Correspondence [email protected]

No conflict of interest

We report a case of myelofibrosis in two

years old boy with severe vitamin D

deficiency rickets and hepatosplenomegaly.

He presented with grossly delayed gross

milestones but his intellectual development

was normal. The nutritional intake was very

poor, comprising of breastfeeding and small

quantities of home-cooked cereals. The child

severely malnourished, with weight in -2.4 z

score and length was -4.9 z score. he was

anemic and had a wide open anterior

fontanel and signs of florid clinical rickets.

There were no neurological abnormalities

except for mild generalized hypotonia.

Radiological survey of the bony skeleton

showed severe generalized osteopenia.

extensive rickets of the thoracic cage and

ends of long bones with splaying, cupping

and fraying of metaphyses. No pathological

features were noted.

There was focal kyphosis in dorsolumbar

region. Bone trephine clearly depicted

replacement of hemopoiesis by fibroblasts

with very occasional erythroid and myeloid

precursors and no megakaryocytes were

seen. Reticulin stain revealed significantly

increased fibrosis, findings were consistent

with myelofibrosis.

1. Cooperberg AA, Singer OP. Reversible myelofibrosis due to vitamin D deficiency rickets. Can. Med. Assoc. J., 1966;94:392.

2. Holick MF. Non-calcemic actions of 1,25-dihydroxyvitamin 03 and clinical applications. Bone. 1995.

3. Bhakhri BK & Debata PK. Nutritional Rickets Presenting with Myelofibrosis. Indian J Pediatr (2010) 77:1437–39

Rickets should be considered as one of the conditions that can lead to severe hematological disorders in infants

P3-041Omneya Magdy Omar DOI: 10.3252/pso.eu.58ESPE.2019

Bone, growth plate and mineral metabolism