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Medical Student Forums => Problem-Based Learning Center => Topic started by: Diagnostic on April 16, 2008, 11:22:52 PM

Title: X-ray Case (6)
Post by: Diagnostic on April 16, 2008, 11:22:52 PM

(http://myweb.lsbu.ac.uk/dirt/museum/margaret/438-651-1081151.jpg)

This is X-ray of 12 months old male with an inherited, autosomal recessive trait condition and tender swelling of hands and feet Questions: 1- What is your diagnosis? 2- Why this condition appear around 1 year old babies? 3- What are other findings you would like to see in this child and triggered by the same mechanism behind this disorder? 4- How to treat this condition? Diagnostic

Title: Re: X-ray Case (6)
Post by: Doctoor on April 18, 2008, 10:19:36 PM

1- What is your diagnosis?
Hand - Foot Syndrome (dactylitis)
This the earliest clinical manifestation of sickle-cell disease (Autosomal recessive trait condition) in infancy which start as a painful swelling of the dorsa of the hands
or feet or both.

2- Why this condition appear around 1 year old babies?
Hemoglobin F (also called fetal hemoglobin) is the form of hemoglobin that exists in the fetus and small infants.
Fetal hemoglobin is able to block the sickling action of red blood cells so that infants with sickle cell disease do not develop symptoms of the illness while they still have hemoglobin F.
Fetal hemoglobin is nearly completely replaced by adult hemoglobin by approximately the twelfth week of postnatal life.
Because of that, the skeletal manifestations of sickle cell disease are the result of changes in bone and bone marrow caused by the chronic tissue hypoxia that is exacerbated by episodic occlusion of the microcirculation by the abnormal sickle cells.
Also this condition is not seen after age 5 years because hematopoiesis in the small bones of the hands and feet ceases at this age.

3- What are other findings you would like to see in this child and triggered by the same mechanism behind this disorder?
Infarction of bone and bone marrow in patients with sickle cell disease can lead also to: Osteolysis, Osteonecrosis, Articular disintegration, Myelosclerosis & Periosteal reaction.

4- How to treat this condition?
Adequate fluid therapy to minimize the sequestration and aggregation of sickled cells inside the bone with pain medication.

Hope this is the answer.

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Doctoor

Title: Re: X-ray Case (6)
Post by: Diagnostic on April 22, 2008, 05:43:50 PM

Thanks Doctoor, that was a good answer