[Macrophage activation syndrome and Kawasaki disease: Four new cases]. / Syndrome d'activation macrophagique et maladie de Kawasaki : à propos de quatre observations.

Macrophage activation syndrome (MAS) is a rarely reported complication of Kawasaki disease (KD). It must be sought during KD with unusual clinical signs, such as enlargement of the liver or spleen, cytopenia (including thrombocytopenia), and elevated serum triglycerides. Here, we report four cases from a single center. The first is the description of a case of KD occurring in an 11-month-old child 15 days after the occurrence of infectious spondylitis. For the second, acute myocarditis heralded KD in a 5-year-old child. A 15-year-old boy had multiorgan failure with shock. The last case describes an infant with atypical KD. All had hepatosplenomegaly, thrombocytopenia, and increased serum ferritin. For all of them, hemophagocytosis was present on the bone marrow smears. The condition rapidly evolved favorably with intravenous immunoglobulins or steroids. The extensive microbiological work-up was unrevealing. None had any coronary sequelae after the episode. KD should be added to the list of inflammatory conditions that may be complicated by MAS that modifies the presentation mimicking toxic shock or infection-associated activation syndrome and makes the diagnosis difficult. This association does not appear to have a prognostic impact on the course of the disease in the literature, confirmed by the analysis of this small series.

[Ecstasy poisoning in a 10-month-old infant]. / Intoxication par ecstasy chez un nourrisson de 10 mois.

UNLABELLED: We describe an accidental ecstasy poisoning in a 10-month-old girl. CASE REPORT: A 10-month-old infant suddenly exhibited behavior disorders with restlessness, hypertonia, and repetitive movements. She was immediately taken to the Emergency Department. Cardiovascular (tachycardia and hypertension) and neurological disorders (major agitation, mydriasis, and nystagmus) were the predominating clinical features. The most common differential diagnoses including febrile convulsion, cranial trauma, or encephalitis were progressively ruled out. The urinary toxicological screen was positive for methamphetamine. MDMA, its main metabolite, and caffeine were subsequently detected in the infant's urine using gas chromatography/mass spectrometry. The child had completely recovered the next morning. COMMENT: Pediatric poisoning with ecstasy remains uncommon. To date, about 10 cases have been reported. All were due to accidental ingestion. Today, the use of ecstasy is no longer restricted to recreational purposes (rave parties, etc.) and it seems to be developing in private parties and in the home. The intrusion of this amphetamine-like drug into the domestic environment may expose young children to the accidental ingestion of ecstasy tablets.

Epstein-Barr virus-associated Kikuchi disease in two children.

The authors describe two children with Kikuchi necrotizing lymphadenitis, the main manifestations of which were cervical lymphadenopathy, fatigue, and fever. The diagnosis was based on histopathologic findings after open biopsy. Results of serologic studies, immunoperoxidase staining for Epstein-Barr virus (EBV) latent membrane protein, in situ hybridization for Epstein-Barr encoded RNAs, and polymerase chain reaction amplification of EBV Epstein-Barr nuclear antigen-1 (EBNA) DNA suggested that EBV was the causative agent in both patients. The disease was mild and subsided after complete surgical resection in one patient, with a follow-up of 1 year. In the other patient, a short course of corticosteroids led to complete clinical remission within 2 months, but the child still has biologic signs of persistent EBV infection. He experienced relapse with a large cervical mass and fever 28 months after the initial onset. Histologic findings were identical to those at initial presentation. Symptoms again resolved spontaneously within 2 weeks, but the follow-up was short (12 mos) and the child's EBNA antibodies are still absent. No evidence of immunodeficiency was found in either child. The cause of Kikuchi disease is unknown, but a viral or postviral hyperimmune reaction has been proposed. Malignant lymphoma and systemic lupus erythematosus are differential diagnoses. Early recognition of Kikuchi disease minimizes potentially harmful and unnecessary investigations and treatments. These findings add Kikuchi disease to the protean manifestations of chronic EBV infection.

[Hereditary neuropathy with tendency to pressure palsies (HNPP) in a child: clinical and biological diagnosis. A case report]. / La neuropathie héréditaire avec paralysie à la pression chez l'enfant: diagnostic clinique et biologique. A propos d'un ças.

UNLABELLED: HNPP occurs rarely but not exceptionally during childhood. CASE REPORT: José M., seven years old, presented an isolated radial nerve palsy of progressive onset with no other clinical feature. There was no family history of neurological disorder. The electromyogram (EMG) showed a 'mosaic-like' pattern of extended sensitive and motor alterations, with diminished conduction velocities and amplitudes and increased distal latencies. HNPP was considered through the association of an isolated radial nerve palsy with an asymptomatic polyneuropathy, and was confirmed by molecular biology. CONCLUSION: HNPP is generally considered on clinical features, heredity and EMG findings. Molecular biology confirms the diagnosis in most of the cases, even in apparently sporadic patients, and is of great interest in atypical cases.

[Hepatic abscess due to Yersinia in a child with thalassemia]. / Abcès hépatique à Yersinia chez un enfant thalassémique.

A 15-year-old boy with thalassaemia major treated with desferrioxamine, experienced fever associated with digestive disorders. These symptoms led to the discovery of multiple liver abscesses. Therapy included antibiotics (third generation cephalosporin and aminoglycoside) and desferrioxamine discontinuation. Recovery occurred within one month approximately. Systemic yersiniosis was further confirmed by the finding of antibodies to Yersinia enterocolitica O5. The systemic diffusion of Yersinia is favoured by iron-overload and iron-chelating agents.

[Peri-ungual capillaroscopy in pediatric practice]. / La capillaroscopie péri-unguéale en pratique pédiatrique.

Nailfold capillaries may be visualized in vivo using a simple non invasive microscopic technic. The assessment of capillary flow disturbances and organic microangiopathies is therefore rendered possible through capillary microscopy. Three kinds of clinically significant morphological abnormalities can be easily recognized: megacapillaries are huge capillaries with a diameter greater than 50 microns. They are mainly found in progressive systemic sclerosis and dermatomyositis, together with avascular areas and pericapillary edema; ramified capillaries are neovessels, most frequently related to subclinical cutaneous vasculitis; capillary hemorrhages may be induced either by active microangiopathies, or by deep vasomotor changes. The semiological consistency of these abnormalities makes capillary microscopy a valuable procedure for the early recognition of connective tissue diseases and the evaluation of vasomotor disturbancies in children as well as in adults. Further investigation of other microangiopathies like purpura rheumatica and diabetes are needed.

[Acute chorea, systemic lupus erythematosus and antiphospholipid antibodies. Apropos of a case]. / Chorée aiguë, lupus érythémateux disséminé et anticorps antiphospholipides. A propos d'une observation.

A case of acute chorea in a 10 years old girl complicating a systemic lupus erythematosus associated with antiphospholipid antibodies is reported. The lupus anticoagulant was detected with a coagulation assay and the false serological reaction for syphilis by the RPR test. The child recovered with Prednisone therapy. The place of chorea in the context of neurological complications of SLE and the particularity of its association with anti-phospholipid antibodies are discussed.

[Lipoprotein lipase deficiency. Apropos of 2 cases]. / Déficit en lipoprotéine lipase. A propos de deux observations.

Lipoprotein-lipase deficiency is an uncommon disease, inherited as an autosomal recessive pattern. The authors report two cases: the first one is a fourteen years old girl. It is revealed by abdominal pain; the diagnosis is detected by a milky plasma and confirmed by the enzyme activity which is dramatically decreased; the other one is a seven year old boy, who shows several pancreatitis but in whom the enzyme activity is not so low, perhaps because of genotypical difference.

[Acute hemorrhagic edema in infants: role of adenoviruses? Apropos of a case]. / Cedème aigu hémorragique du nourrisson: rôle de l'adénovirus? A propos d'une observation.

A case of acute hemorrhagic oedema of the skin is reported. About it we discuss: Anatomo-clinical data, similar to immune complex type III hypersensitivity vasculitis. Antigen has never been defined. In our case adenovirus can be discussed. Evolution is usually benign, but lethal intestinal complications have been described. Nosologic relation with anaphylactoid purpura.

[Extrinsic allergic alveolitis in children. Apropos of 4 cases]. / Alvéolites allergiques extrinsèques chez l'enfant. A propos de 4 observations.

Four cases of extrinsic allergic alveolitis are reported. About them, we discuss: The difficulties of diagnosis that we can only affirm on the association of several clinical, biological, and radiological arguments. The physiopathology: association of immune complex hypersensitivity type III and immediate hypersensitivity type I. It's important to always search for a probable starting infectious factor (mycoplasma). The therapy: antigenic exclusion is the only way to avoid a lung fibrosis.

[A case of acute reversible dysautonomia in an adolescent]. / Un cas de dysautonomie aigüe réversible chez l'adolescent.

A 16 years old male presented with orthostatic hypotension with constant pulse, associated with severe constipation, decrease of sudoral secretions, weight loss and asthenia. This dysautonomia evolued during a period of 6 months and disappeared completely leaving no sequellaes. The symptoms appeared after a rubella. The authors discuss the originality of this observation among the pure pan-dysautonomia and the modes of exploration of the autonomic nervous system. Its mechanisms is probably auto-immune.