[Severe forms of chikungunya virus infection in a pediatric intensive care unit on Reunion Island]. / Infections graves a virus chikungunya en réanimation pédiatrique a l'ile de La Réunion.

UNLABELLED: In 2005-2006, an unexpected, massive outbreak of chikungunya occurred on Reunion Island, a French overseas territory in the Indian Ocean. This arboviral infection transmitted by a mosquito of the Aedes genus is usually benign. A surprising feature of the Reunion Island epidemic was the occurrence of rare severe forms involving adults as well as children. OBJECTIVES: The purpose of this report is to describe severe forms of chikungunya observed in children hospitalized in a pediatric intensive care unit. PATIENTS AND METHODS: This retrospective single-center study was conducted from January 1st to April 30th, 2006. Children between 1 month and 15 years admitted to the pediatric intensive care unit with proven chikungunya infection were included. RESULTS: A total of 9 children were included. The main manifestations were extensive skin blisters in 5 cases, neurological symptoms (encephalopathy) in 4, cardiac complications (myocarditis, hemodynamic disorders) in 5 and bleeding in 1. Two children died. The causes of death were circulatory failure associated with coma and massive hemorrhage in one case and post-infectious encephalitis in the other. Three survivors present long-term neurologic or dermatologic sequels. DISCUSSION: Severe cases of chikungunya in children provide a stark reminder of the cardiac and neurological tropism of the virus and its hemorrhagic forms with high potential mortality and morbidity. These cases underline the need for personal protection measures and for research to develop specific antiviral therapy and vaccines to prevent potentially lethal forms of the disease.

[Chikungunya in paediatrics: epidemic of 2005-2006 in Saint-Denis, Reunion Island]. / Aspects pédiatriques de l'épidémie de Chikungunya à l'île de la Réunion.

UNLABELLED: During the southern-hemispheric 2005-2006 summer, Reunion Island was struck by an epidemic of Chikungunya (Chik), which affected more than a third of the overall population. OBJECTIVES: Our objective was to describe pediatric cases of Chick. MATERIAL AND METHODS: We conducted a retrospective descriptive monocentric study of confirmed pediatric cases of Chik recruited at Saint-Denis' departmental hospital during the peak of the epidemic (January 1st to April 30th 2006). RESULTS: Eighty-six children aged 10 days to 18 years were included. In addition to the typical clinical presentation, we observed other phenotypes. Well-known complicated forms with neurologic, cardiac, gastro-intestinal (plus dehydration) involvement were thoroughly investigated using modern medical technology. We observed 2 fatal cases of acute disease in 9-year-old children: death resulted from a central nervous system insult in one case, and multisystemic neurological, cardiac, haemorrhagic involvement in another. Severe acute presentations requiring hospital admission involved mainly children aged less than 6 months, and those with cardiac, skin and neurologic impairment. The study identified 3 cases of epidermolysis bullosa, which to our knowledge, have never been described previously. CONCLUSION: Chik in children warrants further research in order to propose early and appropriate treatments to avoid complications.

[Fryns syndrome. Report on 3 new cases]. / Syndrome de Fryns. Présentation de 3 nouvelles observations.

BACKGROUND: Fryns syndrome is a lethal autosomal recessive syndrome which includes congenital diaphragmatic hernia (CDH), pulmonary hypoplasia, cranio-facial manifestations including a coarse face, a cleft palate / lip and cloudy corneae, distal limb hypoplasia and internal malformations including central nervous system, cardiovascular, gastrointestinal, urogenital and skeletal anomalies. CASES REPORT: We report on 3 patients with Fryns syndrome in the french Indian Ocean islands (La Réunion and Mayotte islands). Pregnancies were complicated by polyhydramnios. All patients had coarse face, cloudy corneae, cleft lip / palate or high-arched palate, hypoplastic nails and brachytelephalangy. They died in the neonatal period. Two had CDH and 1 did not have a diaphragmatic involvement but a severe respiratory distress syndrome with refractory hypoxemia. Cytogenetic studies of blood lymphocytes and skin fibroblasts were normal. CONCLUSIONS: Fryns syndrome is the most common multiple congenital anomaly associated with CDH. The diagnosis is strongly suggested when CHD and brachytelephalangy are associated. Phenotypes with CDH similar to Fryns syndrome have been described with cytogenetic aberrations with G-banded chromosome analysis and submicroscopic chromosome deletions detected by high resolution karyotyping or array comparative genomic hybridization (CGH). Exclusion of chromosome aberrations (including isochromosome 12p in skin fibroblasts) is needed prior to making the diagnosis of Fryns syndrome. In sporadic cases, high resolution karyotyping or array CGH should be performed for correct diagnosis and genetic counselling.

Fryns syndrome without diaphragmatic hernia. Report on a new case and review of the literature.

Fryns syndrome is an autosomal recessive multiple congenital anomaly syndrome characterized by coarse facies, diaphragmatic hernia, distal limb hypoplasia and malformations of the cardiovascular, gastrointestinal, genitourinary and central nervous systems. Diaphragmatic hernia is a leading diagnostic feature in Fryns syndrome, recorded in more than 80% of cases. We report a newborn with clinical features of Fryns syndrome except the diaphragmatic hernia. Cases of Fryns syndrome without diaphragmatic hernia are reviewed. Even in the absence of diaphragmatic hernia, pulmonary anomalies are described in Fryns syndrome, especially pulmonary hypoplasia. Fetal mice, exposed to nitrofen, have a high incidence of congenital diaphragmatic hernia and other malformations similar to that seen in Fryns syndrome. Nitrofen might target molecular mechanisms similar to those involved in Fryns syndrome.

[Bronchopulmonary dysplasia and cytomegalovirus pneumonia]. / Dysplasie bronchopulmonaire et pneumopathie à cytomégalovirus.

Hyaline membrane disease (HMD) of premature newborn can lead to bronchopulmonary dysplasia (BPD). We report the observation of a 33 weeks premature newborn with HMD, treated with exogenous surfactant and mechanical ventilation. The patient developed respiratory distress with oxygen dependency initially related to BPD. Because of worsening of respiratory condition after 2 months, despite corticosteroid therapy, further investigation was performed. Cytomegalovirus (CMV) was found in urine and in the pharynx and CMV-pneumonia was diagnosed. Treatment with gancyclovir allowed a rapid regression of symptoms. Association between BPD and CMV-pneumonia has been previously reported but the causal relationship is controversial. In premature newborn, CMV-pneumonia can appear clinically and radiologically like a BPD. When evolution is atypical, with persistence of respiratory distress despite BPD treatment, CMV-pneumonia must be considered as a specific antiviral therapy may be discussed.

[Malignant pertussis in the young infant]. / La coqueluche maligne du petit nourrisson.

UNLABELLED: Pertussis remains in France the first cause of bacterial, infectious death in infant aged 10 days to 2 months. It is especially in this age group that malignant pertussis occurs. CASE REPORT: A 40-day-old infant was admitted in the intensive care unit with symptoms of bronchiolitis along with a 200 bpm permanent tachycardia. He presented a marked leukocytosis with lymphocytosis. On the second day, convulsions and coma occurred, followed rapidly by respiratory failure, with a subsequent deterioration due to the development of severe pulmonary hypertension. Circulatory failure caused the infant's death on the beginning of the 5th day. Pertussis was confirmed by PCR on nasopharyngeal swab. Intra-familial contamination was most likely. COMMENTS: Malignant pertussis is characterized by the very young age of patients, permanent tachycardia sine materia, dyspnea with early respiratory failure, frequent neurological symptoms, severe hyperleukocytosis and hyperlymphocytosis, and deep hyponatremia with oliguria and edema. Mortality remains superior to 75% despite the various treatments and life support measures that have been attempted. Adult pertussis, which represents one third of the cases of prolonged cough in this age group, is the main source of contamination of non-immunized young infants. This mode of transmission stresses the importance of the generalization of pertussis vaccine booster in early adolescence, which is recommended in France since 1998. Its extension towards the adult age is under study.

[Warfarin fetopathy]. / Foetopathie coumarinique.

UNLABELLED: We report a case of warfarin embryopathy. This disease affects more than 6% of fetuses exposed in utero to a vitamin K antagonist. OBSERVATION: A child whose mother was treated with acenocoumarol because of a mechanical heart valve presented with signs of warfarin embryopathy. He showed chondrodysplasia punctata with telebrachydactyly, facial dysmorphism with nasal hypoplasia, a cataract, and a bilateral pyeloureteral junction syndrome. COMMENTS: Characteristics of this drug induced embryopathy are reminded, while bearing in mind the conflict of interests between the mother and the fetus. The mechanisms of this embryopathy are debated in light of the recent knowledge concerning fetal metabolism of vitamin K.

[27-gauge percutaneous catheters: use in nutritional management of very-low-birth-weight and severely premature newborns in a neonatal intensive care unit]. / Cathéters épicutanéocaves 27 gauge: utilisation chez les nouveau-nés de très faible poids de naissance et les grands prématurés dans un service de réanimation néonatale.

UNLABELLED: Percutaneous central venous catheterization for parenteral feeding is a permanent problem in the management of very low birth weight neonates. Usually, 23-gauge catheters (diameter: 0.6 mm) are used. In our unit, we have started to use 27-gauge catheters (diameter 0.35 mm) since 1995. The aim of this study was to report our experience with this materiel. SUBJECT AND METHODS: Between September 1, 1997 and January 30, 2000, 352 catheter implantations were performed using 27-gauge infusing sets in 200 premature infants (gestational age less than or equal to 33 weeks [mean 29.2], weight less than or equal to 1,500 g [mean: 1, 152.5 g]). Data were reviewed retrospectively with the view to determine the modality of use and complications. Peculiar insertion modalities were prospectively evaluated in the 50 last included infants (92 catheters). RESULTS: In 97% of cases, the physician needed no help to insert the catheter. In 99.5% of cases (199 neonates), the insertion was successful (the procedure failed in one case). The mean duration procedure was 26 min (range 10 to 85 min). The mean age at insertion of the first catheter was 29 hours (range 0 to 216). Mean catheter maintenance duration was 15 days (range 1 to 53). In 31 cases, documented sepsis related to the catheter were noted (8.8% or 5.8 infections for 1,000 catheter-days). Endocarditis was observed in one case. A pericardial effusion was diagnosed in two cases. No death related to the catheter was noted. CONCLUSION: In our experience, 27-gauge catheter implantation is an easy and safe procedure. We noted no major maintenance problems. Complications were not observed more frequently than those usually encountered with 23-gauge catheters. We conclude that 27-gauge catheter utilization is an attractive alternative for percutaneous central venous catheterization, in very low birth weight neonates.

[Feingold syndrome]. / Syndrome de Feingold.

BACKGROUND: Feingold syndrome is a combination of hand and foot anomalies, microcephaly, tracheo-esophageal fistula, esophageal/duodenal atresia, short palpebral fissures and developmental delay. The most frequent physical findings are clinodactyly of the second and fifth fingers with absent or hypoplastic middle phalanges, and syndactyly of the toes. Inheritance is autosomal dominant (MIM number 164280) with full penetrance concerning hand anomalies, variable expressivity and great intrafamilial variability. CASE REPORT: We report the case of an African boy with duodenal atresia, microcephaly, brachymesophalangy of the second and fifth fingers, unilateral thumb hypoplasia, bilateral syndactyly of toes 2-3 and amesophalangy of the toes. Karyotype was normal. No other member of the family was affected. A fresh mutation is possible. CONCLUSION: Brachymesophalangy affecting mainly the second and fifth fingers and amesophalangy of the lateral toes are cardinal clinical findings in Feingold syndrome. These clinical findings define the syndrome of brachydactyly A4-Temtamy type (MIM 112800), suggesting that brachydactyly A4 would be a partially expressed Feingold syndrome. The incidence of esophageal/duodenal atresia is 29%, including all the known cases. All karyotypes of reported patients were normal.

[Angiostrongylosis in infants in Reunion and Mayotte. Apropos of 3 cases of eosinophilic meningitis including 1 fatal radiculo-myeloencephalitis with hydrocephalus]. / L'angiostrongylose chez le nourrisson à la Réunion et à Mayotte. A propos de trois méningites à eosinophiles dont une radiculomyéloencéphalite fatale avec hydrocéphalie.

BACKGROUND: Eosinophilic meningitis caused by Angiostrongylus cantonensis is widespread in Southeast Asia and Pacific islands. Adults develop a transient meningitis with a benign course but severe or fatal disease may occur in pediatric patients. CASE REPORT: Case 1. A 11-month-old boy living in Mayotte island was hospitalized a few days with fever and skin rash following by seizure, coma, flaccid quadraplegia, absence of deep tendon reflexes, urinary retention and anal incontinence. Eosinophilia was observed in peripheral blood and cerebrospinal fluid. He further developed a triventricular hydrocephalus treated by ventriculoperitoneal shunt. The child died 3 weeks later. A serodiagnosis of angiostrongylus infestation was restrospectively established. Case reports 2 and 3.-Two infants, 10 and 11-month-old-boys, living in Reunion island, developed fever and vomitings, irritability and, for one of them, unilateral sixth cranial nerve palsy. There was eosinophilia in the peripheral blood and in the cerebrospinal fluid. All symptoms progressively disappeared with complete recovery. The suspected diagnosis of angiostrongylus infestation was confirmed by the serology. CONCLUSION: We report the first case of Angiostrongylus cantonensis infection in the French island of Mayone (Comoro Islands) and we confirm the presence of this disease in Reunion island. In this Indian Ocean area, eosinophilic meningitis occurs most of the time in infants with sometimes severe radiculomyeloencephalitic forms.

[Angiostrongylus in the infant at Reunion and Mayotte. Apropos of 3 cases of eosinophilic meningitis including 1 fatal radiculomyeloencephalitis with hydrocephalus]. / L'angiostrongylose chez le nourrisson à la Réunion et à Mayotte. A propos de trois méningites à éosinophiles dont une radiculomyéloencéphalite avec hydrocéphalie fatale.

BACKGROUND: Eosinophilic meningitis caused by Angiostrongylus cantonensis is widespread in Southeast Asia and Pacific islands. Adults develop a transient meningitis with a benign course but severe or fatal disease may occur in pediatric patients. CASE REPORT 1: A 11 months old boy living in Mayotte island was hospitalized some days after fever and skin rash with seizure status, coma, flaccid quadriplegia, absence of deep tendon reflexes, urinary retention and anal incontinence. Eosinophilia was observed in peripheral blood and in the cerebrospinal fluid. Secondary, he developed a triventricular hydrocephalus treated by a ventriculoperitoneal shunt. After 3 weeks, the child died. Retrospectively, the diagnosis of angiostrongylus infection was established with the help of serology. CASE REPORTS 2 AND 3: Two infants, 10 and 11 months old boys, living in Reunion island, developed fever and vomitings, irritability and, for one of them, a unilateral sixth cranial nerve palsy. There was eosinophilia in the peripheral blood and in the cerebrospinal fluid. All symptoms progressively disappeared with complete recovery. The suspected diagnosis of angiostrongylus infection was confirmed by the serology. CONCLUSION: We report the first case of Angiostrongylus cantonensis infection in the French island of Mayotte (Comoro Islands) and we confirm the presence of this disease in Reunion island. In this Indian Ocean area, eosinophilic meningitis occurs most of the time in infants with sometimes severe radiculomyeloencephalitic forms. The origin of these occasionally massive infections is the giant African snail Achatina fulica. For a child with meningitis living in Reunion or Mayotte, or coming back from these 2 islands, Angiostrongylus cantonensis infection must be evoked, especially if there is a blood eosinophilia. Since efficiency of antiparasitic treatment is nowadays not proved, information must be given to people living in exposed areas in view to limit incidence of this disease.

Orthotopic heart transplantation with preservation of the left heart.

In December 1992, cardiac transplantation (for right heart failure with partially occluded pulmonary arteries but with a normal left ventricle) was accomplished with partial resection of right atrium and ventricle but with preservation of the left heart, obtaining a three-ventricle heart. The short-term result (27 months) is good, with normal native and grafted heart rhythms and functions, allowing us to conceive of the possible application of this procedure in some other selected cases.

[Outcome of Wolff-Parkinson-White syndrome in children. Transesophageal study of anterograde permeability of the accessory pathway and of atrial vulnerability]. / Evolution du syndrome de Wolff-Parkinson-White chez l'enfant. Etude transoesophagienne de la perméabilité antérograde de la voie accessoire et de la vulnérabilité atriale.

Twenty-nine children with the Wolff-Parkinson-White syndrome (WPW) were evaluated by transoesophageal electrophysiological studies to determine the quality of anterograde-conduction in the accessory pathway and the atrial vulnerability. The study group included 15 neonates, 1 to 30 days old, and 14 children from 5 to 15 years of age; Anterograde conduction through the bundle of Kent was tested by incremental transoesophageal atrial pacing and by the determination of the shortest conducted cycle with preexcited RR waves; bursts of atrial pacing were then used to try to trigger an atrial arrhythmia. In the group of the 15 neonates, 11 had accessory pathways capable of conduction to the ventricules at frequencies > 300/min (stimulation cycle < or = 2.00 ms) but no atrial arrhythmias could be induced. The older children had slower conduction in the accessory pathways with the shortest conducted cycle length > 200 ms in 11/14 cases; on the other hand, atrial fibrillation was easily induced in 4 children, all over 12 years of age. The risk of syncope by rapid conduction of an atrial arrhythmia through the accessory pathway is negligeable in young children, including those on digoxin. This study suggests that this low risk is explained more by the absence of atrial vulnerability than by the electrophysiological properties of the accessory pathways.

[Rhythmogenic cardiomyopathies of atrial origin in children. Myth or reality?]. / Myocardiopathies rythmiques d'origine atriale chez l'enfant: mythe ou réalité?

Incessant, rapid, supraventricular tachycardia may be complicated by cardiac failure with ventricular dilatation and hypokinetic wall motion on echocardiography: so-called tachycardia-induced cardiomyopathy. The diagnosis is simple when the cardiac rhythm is not sinus rhythm. The authors report the cases of 4 children aged 7 months to 12 years, referred for diagnosis and treatment of apparently primary cardiomyopathy. The findings of spontaneous or vagally-induced atrioventricular conduction defects, a permanently rapid atrial rhythm though influenced by 24 hour variations, or periodic abnormal rate increases, suggested myocardial dysfunction due to an ectopic atrial tachycardia. This was an essential step in management as the control of the tachycardia by amiodarone or betablocker therapy resulted in regression of symptoms and normalisation of left ventricular function. However, some atrial tachycardias are very resistant to medical treatment and, in such cases, there should be no hesitation in using more radical approaches, surgery or ablation, even and especially in patients with severe cardiac failure. In conclusion, apparently primary dilated cardiomyopathy in children may be due to chronic atrial arrhythmia and it is essential to perform at least Holter monitoring in order not to miss this diagnosis.

[Fatal acute cerebral edema in a diabetic child]. / Oedème cérébral aigu mortel chez un enfant diabétique.

A case is reported of fatal acute cerebral oedema occurring in a 15-year-old child suffering diabetic ketoacidosis. He had severe gastro-enteritis, with a weight lose of 8 kg over a period of 8 days (initial weight = 50 kg). He was admitted in a stupor with pH 7.15, 129 mmol.l-1 natraemia, and 31 mmol.l-1 blood glucose concentration. Blood osmolaity was calculated to be 310 mosmol.l-1. He was rehydrated with 416 ml.h-1 normal saline and 416 ml.h-1 of 1.4% sodium bicarbonate. At the same time a total dose of 75 i.u. of ordinary insulin was given. After 2 h, the patient's condition suddenly worsened with unreactive coma, bilateral fixed mydriasis, respiratory pauses, and impairment of haemodynamic state (heart rate 150 b.min-1, blood pressure 80/50 mmHg). The diagnosis of cerebral oedema with severe intracranial hypertension was confirmed by different investigations. Despite ventilatory support and continued intensive care, the patient died a few hours later. It is concluded that some degree of subclinical brain swelling could be common occurrence during diabetic ketoacidosis, present maybe even before the start of treatment. Such cases of cerebral oedema are often reported, but the pathophysiological mechanisms remain unclear. However, unlike this case, rehydration must be moderate (less than 41.m-2.day-1), especially in case of hyponatraemia. Insulin and sodium bicarbonate must be used with care. Early rigorous clinical and biological monitoring is essential. Treatment should aim at a progressive correction of the metabolic disturbances.