The syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia. Report of a new family and a review

Hasan A. Majeed, Mosleh Al-Tarawna, Hatem El-Shanti, Basem Kamel, Fatma Al-Khalaileh

Research output: Contribution to journalArticle

82 Citations (Scopus)

Abstract

A new autosomal recessive syndrome of chronic recurrent multifocal osteomyelitis (CRMO) and congenital dyserythropoietic anaemia (CDA) with microcytosis has recently been described in four children (two sibships) of one consangineous Arab family. In this report, we describe the clinical features and course of the syndrome of CRMO and CDA in two additional patients (one sibship) from another consanguineous Arab family and review the literature. The two patients (brother and sister), the products of a consanguineous marriage, developed the syndrome at an early age of 3 weeks and 2 months respectively. The diagnosis of CRMO was confirmed by radiological and technetium isotope bone scans. Bone marrow studies confirmed the diagnosis of CDA. Peripheral blood films showed hypochromia and microcytosis. The sites involved by CRMO were periarticular, mainly around the elbow, knee, wrist and small joints of the hand. The brother is now 21 years old and the sister 3.5 years old and CRMO is still active with frequent relapses. The brother developed flexion deformities at the age of 13 years. Both patients failed to thrive; weight and height were below the 5th percentile. Conclusion: this is the second report of the syndrome of chronic recurrent multifocal osteomyelitis and microcytic congenital dyserythropoietic anaemia, confirming it as a clinical entity, inherited as an autosomal recessive trait. The disease is characterised by an early onset, long clinical course of remissions and relapses, and seems to be different from the sporadic form of chronic recurrent multifocal osteomyelitis.

Original languageEnglish
Pages (from-to)705-710
Number of pages6
JournalEuropean Journal of Pediatrics
Volume160
Issue number12
Publication statusPublished - 2001
Externally publishedYes

Fingerprint

Siblings
Congenital Dyserythropoietic Anemia
Wrist Joint
Recurrence
Technetium
Elbow
Marriage
Isotopes
Knee
Hand
Bone Marrow
Majeed syndrome
Weights and Measures
Bone and Bones
Chronic recurrent multifocal osteomyelitis

Keywords

  • Autosomal recessive syndrome
  • Chronic recurrent multifocal osteomyelitis
  • Congenital dyserythropoietic anaemia
  • Microcytosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

The syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia. Report of a new family and a review. / Majeed, Hasan A.; Al-Tarawna, Mosleh; El-Shanti, Hatem; Kamel, Basem; Al-Khalaileh, Fatma.

In: European Journal of Pediatrics, Vol. 160, No. 12, 2001, p. 705-710.

Research output: Contribution to journalArticle

Majeed, Hasan A. ; Al-Tarawna, Mosleh ; El-Shanti, Hatem ; Kamel, Basem ; Al-Khalaileh, Fatma. / The syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia. Report of a new family and a review. In: European Journal of Pediatrics. 2001 ; Vol. 160, No. 12. pp. 705-710.
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