Search

Hepatomegaly and alterations in hepatic function are common to all patients with sickle-cell disease. In these patients, hepatic sickling is a manifestation of severe intrahepatic vaso-oclusive crises, even at levels of 25 % HbS and hematocrits of more than 45-50 %, which in 10 % of cases can lead to acute hepatic failure (AHF). AHF can be due to a variety of causes, including hematologic malignancies, but T cell lymphoma, which is usually secondary to diffuse hepatic infiltration and ischemia, is an exceptional cause, although other mechanisms can be involved. Cytokines released by lymphomas have recently been implicated as a cause of AHF.We describe a black girl with sickle cell disease, who developed AHF due to T cell lymphoma without lymphomatous infiltration of the liver. The only mechanism found to explain the clinical findings was release of cytokines by lymphoma. In patients with AHF of unknown etiology we propose early liver biopsy, because prognosis depends on the presence or absence of hepatic tumour infiltration. If AHF develops in a patient with diagnosed malignant disease, cytokine release may be the cause of AHF. Consequently, early diagnosis of the underlying disease and provision of liver support, as well as direct removal of inflammatory mediators from the circulation by exchange transfusion or other methods, should be the main priorities.

Non-intentional burns are still a frequent cause of morbidity in children despite the implementation of preventive campaigns. Children with severe burns (more than 10 % of the body surface) may require iron supplementation, especially in the post-stress or recovery phase. We report the case of a 15-month-old boy, who suffered polytrauma and burns to 20 % of the body surface. A dermal graft was performed eight days after his admission to the pediatric burns unit, which required transfusion of packed red blood cells. On day 15 hemoglobin value was 9.3 g/dL, the reticulocyte hemoglobin content was reduced to 26.2 pg (normal value: 27.2-30.3 pg) and the reticulocyte mean corpuscular value was increased to 124 fl (normal value: 101-108 fl). These findings were interpreted as a functional iron insufficiency, despite the increased ferritin value (219 ng/mL). We highlight the satisfactory outcome of the different hematologic values, with the development of reticulocyte crisis and normalization of hematological parameters after administration of 0.5 mg/kg/day of ferrous sulfate, plus iron corresponding to the follow-up milk formula and a regular diet for the child's age.

Very few cases of children with cardiac tamponade from central venous catheterization have been reported. Four cases are described. In 3 patients the diagnosis was suspected, a pericardiocentesis was performed and they survived. The other patient died and, at autopsy, diagnosis was made. The available literature on cardiac tamponade from central venous catheters in children is reviewed, preventive measures are considered in detail and the need for a high index of suspicion is highlighted.