Presse Med
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Review
[Congenital hypopituitarism: when should transcription factor gene screenings be performed?].
THE GENETIC ORIGIN INCREASINGLY INCRIMINATED: Congenital pituitary hormone deficiencies represent conditions of hypopituitarism resulting from abnormal pituitary ontogenesis. This group of genetically determined diseases has considerably widened with the development of molecular biology. Many transcription factors playing a role in pituitary development have been identified, and their mutations reported as causes of isolated or multiple pituitary hormone deficiencies. ⋯ Multiple (or combined) pituitary hormone deficiencies result in the concomitant or sequential onset of several anterior pituitary hormone deficiencies. They are due to mutations of transcription factors involved in the early steps of pituitary development (RIEG, HesX1, LHX4, LHX3, Prop1, POU1F1/Pit-1), and are associated with various phenotypes. FOR BETTER MANAGEMENT: Long-term follow-up of these patients and functional studies of the mutations identified in specialized research centers will help to determine phenotype-genotype correlations, hence providing a valuable help to the management of these orphan diseases.
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Review Comparative Study
[Outpatient antibiotherapy in children with neutropenia and fever. A review of the literature].
NEW STRATEGIES: Fever in a neutropenic patient requires the rapid initiation of a broad spectrum antibiotic and continued until correction of the neutropenia. Several studies have been conducted recently in order to define the populations of children in whom the antibiotherapy could be suspended early without risk of relapse of fever and/or severe infection. Moreover, the high costs of hospitalisation and the limited number of beds in the departments of Paediatric Oncology Haematology have led to studies on the feasibility of an antibiotherapy at home. ⋯ The first studied the feasibility of an antibiotherapy at home following antibiotherapy in the hospital in order to reduce the costs and duration of hospitalisation. The others proposed an antibiotherapy at home from the start, either with the intravenous or the oral route. Following all these studies, it appeared that, in certain low-risk neutropenic children with fever, not only the antibiotics could be suspended before the complete correction of the neutropenia, but also a large spectrum oral antibiotherapy could replace the intravenous antibiotherapy and outpatient treatment would therefore be feasible.
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A NEW Keeping neutropenic patients with fever in their homes helps to preserve their quality of life and reduces the costs. However, it is important to specify the conditions and the means necessary for the organisation so that home treatment can be applied safely because of the high risk of morbidity due to infection. THE FUNDAMENTAL CONDITIONS FOR ITS MANAGEMENT: The patients who could potentially benefit from an outpatient treatment strategy when presenting with neutropenia and fever must not have a tumour progressing and must not exhibit signs of co-morbidity and be affected by neutropenia and fever at home. ⋯ The development of nursing networks ensure the continuity between the hospital and the town and the good coordination of the health workers caring for the patient. The steps to be taken during an episode of fever are debated: complete discharge from hospital for some, initial outpatient controls in the hospital for several hours for others and the initial hospitalisation for 24 to 72 hours for some others. Whatever the case, haemocultures must be performed before the initiation of any antibiotherapy.
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INFECTIOUS AGENT: The severe acute respiratory syndrome (SARS) is a febrile pneumonia initially observed in China at the end of 2002. The infectious agent has rapidly been identified as a new coronavirus, baptised SARS-associated coronavirus (CoV-SARS). Transmission is inter-human, via respiratory particles mainly. ⋯ During the first half of 2003, the spreading of the virus has been very fast, with a pandemic mode of evolution. More than 8,000 people were infected and 774 died. The reservoir of the virus, which may be animal, is still unknown. The epidemic seems to be controlled, but sporadic or epidemic re-emergences may occur and have been observed in China during January 2004.