Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.
The progress of a female child with African type Fanconi anemia that evolves in time into paroxysmal nocturnal hemoglobinuria is described. Modern diagnostic methods are used to confirm this process. A discussion of possible mechanisms ensues.
Background:
Children with cancer are immunocompromised with increased susceptibility to infections. We evaluated the burden of tuberculosis in children with cancer.
Methods:
Children with cancer were enrolled and screened for Mycobacterium tuberculosis infection using the tuberculin skin test and enzyme-linked immune absorbent spot (T-SPOT.TB; Oxford Immunotec Ltd, Oxford, United Kingdom). Children with physician-suspected tuberculosis were investigated for M. tuberculosis using microscopy and culture on sputum or gastric washings.
Results:
We enrolled 169 children; 10.7% were living with HIV. The tuberculin skin test was positive in 2.9% of patients, who were treated for tuberculosis and excluded from further analysis. The enzyme-linked immune absorbent spot (T-SPOT.TB) was either negative or indeterminate in the first 100 children screened. The incidence of tuberculosis was 7.6 per 100 child-years; 35.3% were culture-confirmed. Tuberculosis was diagnosed at a mean of 5.5 months from cancer diagnosis. A greater proportion of children living with HIV (44.4%) developed tuberculosis than those without (17.2%; adjusted P = 0.042). Children treated for high-risk acute lymphoblastic leukemia, advanced stage non-Hodgkin lymphoma and acute myeloid leukemia (P = 0.009) and those with a higher exposure-period (per 100 child-years) to corticosteroids courses (350 vs. 29.4; P < 0.001) had a higher incidence of tuberculosis. Twenty-six of 34 children (76.5%) with tuberculosis died; multiple infections were identified at the time of death.
Conclusions:
Screening children for tuberculosis infection at cancer diagnosis was of limited value. The high rate of tuberculosis and poor outcomes emphasize the need for a high index of suspicion to diagnose tuberculosis and consideration for antituberculosis treatment, especially for those with identified risk factors.
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