[Kikuchi-Fujimoto disease mimicking malignant lymphoma in adolescents]. / La maladie de Kikuchi-Fujimoto ; un diagnostic différentiel méconnu du lymphome chez l'adolescent.

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare cause of lymphadenopathy in children. This benign disease can mimic lymphoma and misleads doctors. It was first described in Asia, where it occurred especially in young women. Recent publications show that it can also affect teenagers and young adults in Caucasian populations. The pathophysiology remains unknown. Three hypotheses have been raised for this disease: the role of viruses (in particular HHV-8), genetic predisposition (two alleles in HLA class II genes were found more frequently in patients with Kikuchi disease), and an autoimmune cause because of the correlation with lupus erythematosus. Few cases have been reported in Europe so far. In this article, we report three cases of Kikuchi disease observed in less than 2 months in a single hospital in France. All three patients were teenagers who presented with lymphadenopathy, either isolated or combined with fever, weakness, and weight loss. In all of them, the hypermetabolic activity of the lymph node on the PET scanner misled us to suspect lymphoma. The diagnosis of Kikuchi disease was finally made, for all patients, after 2 weeks in the hospital based on lymph node biopsy. Based on this report, we highlight that early biopsy in presence of lymphadenopathy can avoid unnecessary extensive investigations. Moreover, in this rare disease, it is very surprising to come across three cases that are not family-related, in such a short period of time. This strengthens the hypothesis of the possible implication of an environmental factor in the pathophysiology of Kikuchi disease.

[Oral administration of medications to infants: Implementation of a preliminary risk analysis in a hospital service of pediatrics]. / Administration orale de médicaments au nourrisson : réalisation d'une analyse préliminaire des risques dans un service d'hospitalisation pédiatrique.

PURPOSE: The objective of this study was to reduce the risk of errors when administering oral medications to infants aged 28 days to 2 years. MATERIAL AND METHODS: The method of the preliminary risk analysis (PRA) was implemented by a multidisciplinary group in a hospital service of pediatrics. The study focused on the phase of preparation of drugs by nurses before administration. RESULTS: This analysis revealed 41 scenari, 16 were criticality unacceptable. In particular, their analysis highlighted the impact of the drug dosage form, the lack of scientific information and the human factor on this preparation. Eleven action sheets have been written. DISCUSSION: The risk management requires significant human investment, material resources and organizational solutions: formations, information, i.e. computerized prescribing, dispensing and administering system, centralized drug preparations, automated drugs cabinets or unit drug daily dispensing system. CONCLUSION: Control these risks means to get specific actions at pediatric wards, enhance dispensing system by the hospital pharmacist and the support of the pharmaceutical industry to get commercially available pediatric drugs.

Advanced paternal age and de-novo complex chromosomal rearrangement in offspring.

We report one case of a de-novo complex chromosomal rearrangement (CCR), t(1;5;13)ins(14;13), in an abnormal 19-month-old boy. Clinical features associated were a mild facial dysmorphy and a psychomotor retardation. Parental ages were, respectively, 29 years for the mother and 60 years for the father. We point out the usefulness of fluorescence in-situ hybridization in elucidating CCRs, and discuss the possible correlation between the existence of a chromosomal aberration and advanced paternal age.

[Fulminant encephalopathy due to Shigella flexneri]. / Encéphalopathie fulminante à Shigella flexneri.

BACKGROUND: In some rare cases, Shigella flexneri infection may be associated with fulminating encephatopathy. CASE REPORT: A 6-year-old girl was admitted with fever, diarrhea and consciousness abnormalities. Brain CT scan was normal. Shigella flexneri type 2 was isolated from the stools. Despite antibiotic therapy, the encephalopathy was lethal. Two siblings were also infected, the first with only diarrhea, the second with headache and mild consciousness abnormalities: both children had a favorable course. CONCLUSIONS: Shigella flexneri encephalopathy is associated with high mortality rate in developed countries. Presence of headache could be a poor prognostic factor.

[Treatment of migraine in children]. / Traitement des migraines de l'enfant.

Simple migraine attacks are usually controlled by rest and an analgesic (acetylsalicylic acid or paracetamol), eventually associated with metoclopramide. More severe cases with failure of these measures may benefit from antimigraine medications such as ergotamine derivatives. Preventive treatment is only indicated in case of frequent (> or = 3 per month) and complicated attacks.

The Wiedemann-Rautenstrauch neonatal progeroid syndrome: a case report and review of the literature.

We present the case of a post-term newborn with intrauterine growth retardation, pseudohydrocephalus, a tiny face and mouth, thin wrinkled skin, an aged appearance, lipoatrophy and a normal cranial CT scan, suggestive of the Wiedemann-Rautenstrauch neonatal progeroid syndrome. He developed hypothyroidism on day 18 due to a partial organification disorder as did a later born sib. His mental development remains normal at age 2 with delayed growth at -2.5 SD. The case is presented and discussed and the literature is reviewed.

[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent]. / Déficit homozygote en C8 révélé par des infections récidivantes à Neisseria meningitidis chez un adolescent.

BACKGROUND: Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adolescence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. CASE REPORT: A 14 year-old gypsy boy was admitted because of a febrile meningococcal meningitis that was complicated by a rapidly extensive and necrotic purpura, obnubilation and clotting abnormalities without hemodynamic anomalies. The patient was given symptomatic therapy and a 12-day course of antibiotics that resulted in rapid and complete recovery. Medical history of this patient showed that he had been admitted at the age of 3 years for a severe febrile purpura with septic shock and clotting abnormalities followed by rapid and complete recovery after symptomatic and antibiotic therapy. No germ had been then isolated. The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examination of the terminal pathway-revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin prophylaxis. He remained healthy during the ensuing 4 years. CONCLUSIONS: Meningococcal infections associated with late complement component deficiency are generally uncomplicated but they remain potentially severe. Early screening for this late complement component deficiency should be considered after severe clinical manifestations.

[Hyperglycemia revealing neonatal infection]. / Hyperglycémie révélatrice d'une infection néonatale.

BACKGROUND: Hyperglycemia as the first manifestation of neonatal sepsis is rare. CASE REPORT: A breast-fed neonate was admitted at the age of 6 days because of vomiting. Group B streptococci had been isolated in secretions of the ear at birth but the neonate had not been treated. At admission, physical examination was normal but glucosuria, without ketonuria, and hyperglycemia (9.7 mmol/l) were noted. Because hyperglycemia was not explained by usual causes, the baby was systemically given antibiotics. The next day, blood, spinal fluid and urine cultures taken on admission were positive for group B streptococci while blood fibrinogen and C-reactive protein were increased. Hyperglycemia and glycosuria were normal after 24 hours of antibiotic therapy and follow-up was uneventful. Subsequently, the same bacteria was isolated from the mother's milk. CONCLUSION: Isolated hyperglycemia may reveal an infection; therefore its discovery might contribute to early diagnosis and treatment.

Bacillus Calmette-Guérin infection after vaccination of human immunodeficiency virus-infected children.

The use of Mycobacterium bovis/Bacillus Calmette-Guérin (BCG) to vaccinate against tuberculosis remains controversial. The development of tuberculosis in human immunodeficiency virus (HIV)-infected children demands specific evaluation of the risk/benefit ratio of BCG vaccination in this situation. In our institution 9 of 68 HIV-infected children vaccinated with BCG before the diagnosis of HIV infection was suspected developed vaccine-related complications: 7 of these children had a large satellite adenopathy with or without skin fistulae, whereas the other 2 had disseminated BCG infection beyond the satellite ganglion (involvement of the spleen and mesenteric and mediastinal lymph nodes in one case and the liver and lungs in the other). The children were vaccinated soon after birth; no particular problems were observed at that time, but complications appeared 3 to 35 months later. All but one of these children had a rapidly progressive form of HIV disease. The possibility of delayed local or disseminated BCG infection must be considered in analysis of the risk/benefit ratio of vaccination of HIV-infected children. The prognosis of HIV infection must be taken into account, even if the child is asymptomatic when vaccination is being considered.

[Neonatal cardiac failure secondary to hypocalcemia caused by maternal vitamin D deficiency]. / Insuffisance cardiaque néonatale secondaire à une hypocalcémie par carence maternelle en vitamine D.

BACKGROUND: Heart failure is a rare manifestation of neonatal hypocalcemia. This paper describes such a case resulting from maternal vitamin D deficiency. CASE REPORT: A full-term boy, born in December after a normal pregnancy, was admitted at the age of 6 weeks because of dyspnea that appeared during suckling. Examination showed heart failure. Electrocardiogram showed that the corrected QT-interval was lengthened (0.54 s, normal < 0.45 s). Echocardiogram showed dilated, hypokinetic myocardiopathy. His serum calcium concentration was low (1.40 mmol/l) and phosphate was high (2.8 mmol/l); his alkaline phosphatase was 513 Ul/l. His blood PTH concentration was high (120 pg/ml) and his 25 (OH) D was low (5 ng/ml). The patient was given calcium (1 g/m2/day) and 1.25 (OH)2 D (2 micrograms/day orally). His serum calcium returned to normal within 4 days, and his cardiac abnormality was resolved within 3 months. His mother's blood 25 (OH) D concentration was very low (3 ng/ml), 6 weeks after birth. CONCLUSION: Neonatal hypocalcemia appears to have been compounded in this case by a maternal vitamin D deficiency. Hence, all pregnant women at risk of deficiency should be given vitamin D.