ABSTRACT
Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.
Subject(s)
Coronary Aneurysm , Mucocutaneous Lymph Node Syndrome , Vasculitis , Child , Humans , Male , Infant , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/complications , Aspirin/therapeutic use , Fever/etiology , Vasculitis/complications , Coronary Aneurysm/diagnosis , Coronary Aneurysm/etiology , Coronary Aneurysm/therapy , Immunoglobulins, Intravenous/therapeutic useSubject(s)
Bone Diseases, Metabolic/etiology , Bone and Bones/pathology , Hepatolenticular Degeneration/diagnosis , Bone and Bones/diagnostic imaging , Brain/diagnostic imaging , Ceruloplasmin/analysis , Child , Copper/urine , Female , Hepatolenticular Degeneration/complications , Humans , Magnetic Resonance ImagingABSTRACT
BACKGROUND: Body composition (BC) analysis based on bioelectrical impedance analysis (BIA) provides conflicting results. The purpose of the study was to validate an equation specific for young patients with cystic fibrosis (CF), describe their BC and investigate its association with lung function. METHODS: Fifty-four young CF patients were evaluated by BIA and dual X-ray absorptiometry (DXA). An empirically derived CF-specific equation for fat-free mass (FFM) estimation by BIA was elaborated after stepwise multivariate regression and the agreement between BIA and DXA was assessed by Bland-Altman plots. The association between BC and lung function was investigated by regression analysis. RESULTS: The mean difference between the BIA and DXA assessment was close to zero. A total of 22.5% of patients (n=9) presented a FFM z-score≤-2. They had a worse pulmonary function and diaphragmatic impairment. Among these 9 patients, 7 had a normal BMI z-score>-1. CONCLUSIONS: BIA, based on a CF-specific equation, is a reliable method for BC assessment and allows the identification of patients at risk of nutritional degradation and bad respiratory prognosis.
Subject(s)
Body Composition , Cystic Fibrosis , Absorptiometry, Photon/methods , Adolescent , Body Mass Index , Child , Cystic Fibrosis/diagnosis , Cystic Fibrosis/physiopathology , Electric Impedance , Female , France , Humans , Male , Prognosis , Prospective Studies , Respiratory Function Tests/methods , Statistics as Topic , Young AdultABSTRACT
Cat scratch disease is the most common zoonosis in humans and its typical expression is a persistent benign regional adenopathy. In some rare cases, mono- or multifocal osteomyelitis is described. In this paper, we report the case of bone lesions in a 13-year-old girl infected with cat scratch disease. We have also undertaken a literature review and analyzed 60 other such cases. The manifestation of a bone lesion associated with cat scratch disease was characterized by a mono- or multifocal infectious osteomyelitis, fever, and a general alteration of the patient's health. The most frequent location of osteomyelitis was in the spine. Magnetic resonance imaging appeared the most sensitive test to highlight the bone lesions. Serological findings help reinforce the diagnosis of cat scratch disease caused by Bartonella henselae infection. Osteomyelitis in cat scratch disease is rare but not exceptional. Therefore, it is essential to think about this hypothesis in case of osteomyelitis associated with a general alteration of the patient's health, especially if the lesions are multifocal and if there is a known history of cat contact.
