Aminoglycoside prescription: compliance with national guidelines in a pediatric hospital.

OBJECTIVE: Our study aimed to describe the use of aminoglycosides (AGs) in the pediatric population in acute settings and to assess its compliance with the most recent national recommendations. METHODS: A single-center retrospective study conducted over a 5-month period. Pediatric patients who received at least one dose of AGs in emergency or intensive care unit were included. Compliance with the 2011 French recommendations was assessed. RESULTS: A total of 153 AG prescriptions (120 with gentamicin and 33 with amikacin) for 139 patients (median age of patients = 10 months [IQR: 3-36]) were analyzed. Most of the AG prescriptions were initiated in the emergency department (n = 117, 76%) and, overall, compliance with national guidelines was met in half (n = 77) of the prescriptions. In the emergency department, cases of misuse concerned the indication, mostly for patients with pyelonephritis. In the pediatric intensive care unit setting, the misuse concerned underdosing and a low rate of pharmacological monitoring. CONCLUSION: AGs are still misused in pediatric acute settings. In order to limit drug resistance and to be more efficacious, higher doses should be used and monitoring should be performed, in particular in pediatric intensive care units. In the emergency department, more objective criteria should be used to initiate AGs.

Has the introduction of high-flow nasal cannula modified the clinical characteristics and outcomes of infants with bronchiolitis admitted to pediatric intensive care units? A retrospective study.

BACKGROUND: This study aimed to assess how the emergence of high-flow nasal cannula (HFNC) has modified the demographic and clinical characteristics as well as outcomes of infants with bronchiolitis admitted to a pediatric intensive care unit (PICU). METHODS: This was a single-center retrospective study including infants aged 1 day to 6 months with bronchiolitis requiring HFNC, noninvasive ventilation (NIV), or invasive ventilation on admission. RESULTS: A total of 252 infants (mean age 53±36 days) were included in the study. The use of HFNC increased from 18 (21.4%) during 2013-2014 to 53 infants (55.2%) during 2015-2016. The length of stay in the PICU decreased over time from 4.7±2.9 to 3.5±2.7 days (P<0.01) but the hospital length of stay remained similar (P=0.17). On admission, patients supported by HFNC as the first-line therapy were older. The PICU length of stay was similar according to the type of respiratory support (P=0.16), but the hospital length of stay was longer for patients supported by HFNC (P=0.01). CONCLUSION: The distribution of respiratory support has significantly changed over time for patients with bronchiolitis and HFNC is increasingly used. The demographic and clinical characteristics of the have not changed over time. However, the PICU length of stay decreased significantly.

Enzyme-replacement therapy in perinatal hypophosphatasia: Case report and review of the literature.

Hypophosphatasia (HPP) is a rare disease resulting from alterations of the ALPL gene encoding tissue-nonspecific alkaline phosphatase (TNSALP). Perinatal HPP is mainly characterized by bone hypomineralization and severe respiratory insufficiency. We describe a full-term boy diagnosed with perinatal HPP after birth, showing dramatic improvement after treatment with Asfotase Alfa, an enzyme-replacement therapy (ERT) prescribed in HPP cases. He initially presented with respiratory insufficiency due to bone hypomineralization, and severe pulmonary hypoplasia that required tracheostomy and invasive ventilation for 8 months. He was taken off ventilation at 41 weeks of age. He also presented complications including hypercalcemia, craniosynostosis, nephrocalcinosis, hypotonia, and a severe feeding disorder. He is still alive at 30 months of age, and his respiratory status and tonus is steadily improving. This case reflects the progression of HPP patients with specific therapy added to symptomatic management. Some aspects of the disease are now well known, such as nephrocalcinosis and craniosynostosis, related to the natural course of the disease, which persisted despite the ERT. The long-term prognosis and outcome for this newborn child remain unknown.

Maxillary sinusitis complicated by stroke.

INTRODUCTION: Neurological complications of acute sinusitis are exceptional, but potentially serious. CASE REPORT: The authors report the case of a 6-year-old diabetic girl who presented with middle cerebral artery ischemic stroke secondary to inflammatory arteritis of the left internal carotid artery in a context of bilateral acute maxillary sinusitis. MRI confirmed ischaemic stroke associated with carotid arteritis and complete obstruction of the maxillary sinuses. A favourable outcome was observed after endoscopic drainage of the sinuses associated with broad-spectrum antibiotic therapy. DISCUSSION: This complication was probably due to spread of an infectious inflammatory reaction of the intrapetrosal carotid artery and its branches via the pterygoid venous plexus. To our knowledge, this is the first published case report of maxillary sinusitis complicated by stroke.

[Acute epiglottitis due to Haemophilus influenzae b: A severe consequence of increased skepticism about vaccination]. / Épiglottite aiguë à Haemophilus influenzae b : conséquence grave du scepticisme vaccinal.

Acute epiglottis is a life-threatening disease in relation with the occurrence of an acute upper airway obstruction. Its incidence has fallen dramatically since the widespread introduction of Haemophilus influenzae type b (Hib) conjugate vaccine. We report the case of a 26-month-old child who was not fully immunized and presented acute upper airway respiratory distress with fever. The symptoms quickly evolved to a respiratory arrest condition with bradycardia, revealing epiglottitis due to Hib. Despite high immunization coverage with great efficacy and occurrence of herd immunity, this entity still exists because of the French population's skepticism of the routine vaccination schedule.

[Pediatric management by mobile intensive care units in the northern French Alps emergency network]. / Activité pédiatrique des SMUR du réseau nord-alpin des urgences.

INTRODUCTION: The northern French Alps emergency network (RENAU) manages emergent care and patient treatment pathways in a three-county area in the French Alps. The aim of the study was to describe the pediatric activity in mobile intensive care units (MICUs). METHOD: This retrospective observational study was conducted from 1 January to 31 December 2012. Data were obtained from patients' medical records in seven representative MICUs of the RENAU. Consecutive patients between 1 month and 18 years of age were enrolled. RESULTS: During the study period, MICUs carried out 11,951 primary transports and 3087 secondary transfers. A total of 1325 patients were enrolled: 1087 primary transports and 238 secondary transfers. In primary interventions, 531 (48.9%) patients were managed for a trauma, 118 (11%) children were discharged and stayed home, 427 (39.9%) underwent an intravenous cannulation and 27 (2.5%) on-scene intubation. A pediatrician was in charge of 64 (26.9%) secondary transfers. Among 1649 (18%) mountain rescues, 296 (18.7%) involved a child. CONCLUSION: Emergency physicians in MICUs are involved with children's prehospital care and need dedicated materiel and special training.

[YEL-AND meningoencephalitis in a 4-year-old boy consecutive to a yellow-fever vaccine]. / Méningo-encéphalite après vaccination anti-amarile : syndrome de YEL-AND.

Yellow fever is a vector-borne disease transmitted by an endemic mosquito in sub-Saharan Africa and tropical South America. It causes fever and possibly liver and renal failure with hemorrhagic signs, which may be fatal. The yellow-fever vaccine is an attenuated vaccine that is recommended for all travelers over the age of 9 months in high-risk areas. Adverse effects have been reported: minor symptoms (such as viral syndrome), hypersensitivity reactions, and major symptoms such as viscerotropic disease (YEL-AVD) and neurotropic disease (YEL-AND). The yellow-fever vaccine-associated autoimmune disease with central nervous system involvement (such as acute disseminated encephalomyelitis) associates fever and headaches, neurologic dysfunction, seizures, cerebrospinal fluid (CSF) pleocytosis, and elevated protein, with neuroimaging consistent with multifocal areas of demyelization. The presence of antibodies or virus in CSF, within 1-30 days following vaccination, and the exclusion of other causes is necessary for diagnosis. We describe herein the case of a 4-year-old child who presented with severe encephalitis consecutive to a yellow-fever vaccine, with favorable progression. Diagnosis is based on the chronology of clinical and paraclinical signs and the presence of yellow-fever-specific antibodies in CSF. The treatment consists of symptomatic treatment and immunoglobulin injection.

[A child with sepsis-associated encephalopathy]. / Encéphalopathie associée au sepsis (EAS), un cas pédiatrique.

Sepsis-associated encephalopathy (SAE) is a diffuse brain dysfunction due to a systemic response to infection. We report the case of a 4-year-old girl with fever and vomiting for 48h, brought to the university hospital of Grenoble because of vigilance disorders, loss of verbal fluency, and a cerebellar syndrome. She had a biological infectious syndrome. Infectious encephalitis was suggested first, but the cerebral scan and the lumbar punction were normal. Magnetic resonance imaging (MRI) showed a diffuse brain edema with extended involvement of cortical and basal ganglia. The electroencephalogram was globally slow. The infectious syndrome was explained by perforated appendicitis with peritonitis, treated by surgery and antibiotic therapy. Other infectious explorations were negative. No metabolic or autoimmune diseases were found. Hence, our final diagnosis was sepsis-associated encephalopathy. After 1 year of follow-up care, her clinical exam, MRI, and EEG were normal. Sepsis-associated encephalopathy has been increasingly described in the adult population, but until today only three pediatric cases have been published. It is diagnosed when the patient has a severe infectious syndrome associated with neurologic symptoms, mostly vigilance or consciousness disorders, no signs of shock, and only when other potential reasons have been ruled out. The MRI shows non-specific diffuse lesions with vasogenic edema on the subcortical substance or on the basal ganglia and the thalami. The electroencephalogram is slowed down on the whole. The main differential diagnoses are infectious encephalitis, acute disseminated encephalomyelitis, and cerebral vasculitis. Posterior reversible encephalopathy syndrome is an MRI diagnosis that presents characteristics similar to SAE. In the future, it could be discovered that it is the same physiopathology. At the moment, we only treat the symptoms and the causative infection. Most of the time, patients have neurologic sequelae that affect their verbal fluency. It can persist from a few months up to 6yrs. Although quite slow, the neurologic progression is good. The mechanisms are studied and there are hopes for specific treatments. The main explanation seems to be immune with alterations of the blood-brain barrier. Cytokines and activated leukocytes may attack the cerebral substance.

[Unusual presentation of a severe Mycoplasma pneumoniae infection: report of 2 cases]. / Présentation inhabituelle d'une infection grave à Mycoplasma pneumoniae, à propos de 2 observations.

Mycoplasma pneumonia is responsible for multisystemic infection. Pulmonary symptoms are most common in children. We describe herein two unusual severe forms of M. pneumoniae infection without initial pulmonary symptoms. The first case is an 8-month-old boy who was hospitalized in the pediatric intensive care unit with severe sepsis. There were no initial pulmonary symptoms, nor obvious clinical infection. Initial blood tests and x-ray did not aid the diagnosis. The blood tests came back positive for M. pneumonia. Pulmonary symptoms eventually appeared 24h later, and there was a pneumonia outbreak on the chest radiograph. The boy was given josamycin and improved quickly. The second case concerns an 8-year-old child who was hospitalized in the pediatric intensive care unit with toxic shock. No clinical infectious origin was found. A broad-spectrum antibiotic therapy was started with ceftriaxone and josamycin. The M. pneumoniae blood test came back positive, which confirmed the diagnosis of septic shock in M. pneumoniae, requiring adjustment of the antibiotic therapy. Current guidelines for the choice of probabilistic antibiotic therapy in case of severe sepsis do not include the case of M. pneumoniae. The early initiation of antibiotic therapy plays a major role in the prognosis of these patients. It seems useful to search for M. pneumoniae in cases of severe atypical infections, particularly in the absence of pulmonary symptoms.

[One magnet is better than two]. / Un aimant vaut mieux que deux.

Accidental foreign body ingestion by children is not exceptional and occurs in most cases in children less than 5 years old. The impact is not well known. We present the case of a 31-month-old boy affected by peritonitis caused by several bowel perforations after swallowing three magnets at different times. A single foreign body magnetic ingestion does not often cause trouble, but if the ingestions are spaced out, then consequential complications can occur. This is why, in case of stomachaches in a child less than 3 years or a child with behavior problems, it is important to perform standard abdominal X-ray examination.

[Community-acquired Staphylococcus aureus necrotizing pneumonia secreting Panton-Valentine leukocidin in an infant]. / Un cas de pneumopathie nécrosante à Staphylococcus aureus sécréteur de toxine leucocidine de Panton Valentine chez un nourrisson.

Staphylococcus aureus (SA) necrotizing pneumonia secreting Panton-Valentine leukocidin (PVL) has a high mortality rate, approximately 50% according to recent data, mainly in children and young adults. Recently, 2 cases of the disease have been reported in newborn twins with a good outcome. We report a third case of community-acquired necrotizing pneumonia due to SA sensitive to methicillin (SASM) secreting Panton-Valentine leukocidin (PVL) in an infant, initially paucisymptomatic, in the context of familial furunculosis, with a fatal outcome. Cases of necrotizing pneumonia in this age group are emerging, and extensive information for clinicians is needed due to the high mortality rate of the disease.

[Problems and advantages of autopsy in the case of out-of-hospital childhood death: report of 2 cases]. / Difficultés et intérêt de l'autopsie dans le cas de décès d'enfant hors établissement de santé : à propos de 2 observations.

In France, the number of deaths remains high in the pediatric population. These deaths are rarely subject to a scientific or forensic autopsy. However, this medical act appears necessary to answer the many private and public questions (public health, prevention, judicial, or even institutional) that can arise. Two pediatric clinical observations raising these questions in the context of a household accident are presented. In the first one, the death led to the establishment of a forensic obstacle to the burial and a forensic autopsy. The autopsy results provided answers, both to the relatives and to the court. The second concerns a deceased child managed within the protocol for sudden infant death syndrome. The autopsy was not authorized by the parents and no answer on the causes of death could be determined. These 2 observations underline the importance and utility of this medical act.

[Lymphohistiocytic activation syndrome and Burkholderia cepacia complex infection in a child revealing chronic granulomatous disease and chromosomal integration of the HHV-6 genome]. / Syndrome d'activation lymphohistiocytaire associé à une infection àBurkholderia cepacia complex chez un nourrisson révélant une granulomatose septique et une intégration génomique du virus HHV-6.

Chronic granulomatous disease (GCD) is characterized by severe infections, notably with Burkholderia cepacia complex (BCC). GCD is rarely complicated by lymphohistiocytic activation syndromes, most often secondary to bacterial or viral infections, in particular human herpes virus 6 (HHV-6). We describe the case of a 10-month-old boy who suffered from multiple organ failure due to a BCC infection and a lymphohistiocytic activation syndrome, leading to diagnosis of GCD. The initial search for HHV-6 was positive and the infection was treated, but the progression and viral sample analysis led to the chromosomal integration of the HHV-6 genome. The child's clinical condition was normal after bone marrow transplantation. This case describes a rare association between GCD and lymphohistiocytic activation syndrome and raises questions about the role played by chromosomal integration of the HHV-6 genome.

[The EXIT procedure: principles and application in congenital cervical teratomas]. / La procédure EXIT modifiée: principes et application dans les tératomes cervicaux congénitaux.

INTRODUCTION: The ex utero intrapartum treatment (EXIT) procedure uses uteroplacental gas exchange after caesarean section for foetus oxygenation. This placental support allows establishing an airway in the newborn in case of obstruction. Maintenance of placental perfusion requires uterine relaxation. A halogenated agent is classically use while nitroglycerine is used in the modified EXIT procedure. CASE REPORT: We present the case of a newborn with a giant thyroid teratoma diagnosed on ultrasound at 20 weeks' gestation. At 32 weeks' gestation, a modified EXIT procedure was performed. The EXIT was successful and newborn was operated on the following day. Currently, the child is 32-month-old, has no sequelae and benefits from thyroid hormone substitution. DISCUSSION: The EXIT procedure allows managing airway obstruction, even when complex, at birth. Nevertheless, prolonged uterine relaxation increases the risk of flooding. Because of its short half-life, nitroglycerine reduces this risk. In this case report we chose the modified EXIT procedure because the mother was young and primipara and foetal prognosis was poor. CONCLUSION: Currently, the EXIT procedure is the technique of choice in the management of a foetal cervical mass. An exhaustive preoperative foetal workup is necessary to choose the classical or modified EXIT procedure.

[Neonatal splenic injury: a difficult diagnosis]. / La rupture splénique en période néonatale: un diagnostic difficile.

Neonatal splenic injury is a rare but serious condition, due to the risk of haemorrhagic shock. We report on the case of a newborn infant with a neonatal respiratory distress that first evoked materno-fetal infection. Clinical deterioration, with anemia and abdominal distension, led then to the proper diagnosis. Dystocia seems to be the most likely cause of the splenic rupture in this report. Medical treatment is advocated as first line, while surgical treatment may be necessary in some cases. In the case surgery is inevitable, a conservative approach is preferable.

Fetal lung volume in congenital diaphragmatic hernia.

In a retrospective study of 22 neonates with congenital diaphragmatic hernia, fetal lung volume (FLV) measured by magnetic resonance imaging was associated with survival; the best FLV ratio cut-off to predict mortality was 30% of expected FLV. This study supports a correlation between FLV and the chances of survival.

[Outcomes of children after bronchial foreign body inhalation]. / Que deviennent les enfants ayant présenté un corps étranger bronchique ?

Bronchial foreign body inhalation is a frequent and potentially serious accident in children. It can provoke long-term complications such as bronchiectasis. These complications occur especially if the delay between inhalation and extraction is long (above 7 days) and in case of vegetal foreign body. To decrease the risk of complications, one must emphasize the importance of early diagnosis and the potential role of fiberoptic bronchoscopy to help the diagnosis in case of suspected inhalation without clinical or radiological signs. Rigid bronchoscopy is the only procedure that allows diagnosis and removal of the foreign body.

[Congenital nasal pyriform aperture stenosis: a case report]. / Sténose congénitale des orifices piriformes: à propos d'un cas.

Congenital nasal pyriform aperture stenosis is a rare and poorly understood pathology. It's etiology is unknown. In cases with severe nasal airway obstruction, this anomaly may produce newborn respiratory destress similar to those seen in bilateral posterior choanal atresia. It can occur as an isolated anomaly or it can be associated with a dental anomaly, craniofacial, ophthalmological or central nervous system malformation. The diagnosis is made by physical examination. Computed tomography confirms the diagnosis. Magnetic resonance imaging of the brain and pituitary endocrine axis must be performed. In patients with severe obstruction, surgical correction is necessary. We describe the case of a 1-month-old child who needed surgical treatment with a sublabial approach for a congenital nasal pyriform aperture stenosis associated with a solitary maxillary central incisor. The follow-up (4 years) reveals normal nasal airway and facial growth.

[Acute organophosphate intoxication after using a anti-lice insecticide shampoo]. / Intoxication aiguë par organophosphorés à la suite de l'utilisation d'un insecticide comme shampooing anti-poux.

CASE REPORT: Two children were admitted for poisoning by organophosphate pesticides applied as hair rinses against lice. These chemical agents inhibit the acetylcholinesterase enzyme at various sites. The resultant accumulation of the transmitter acetylcholine causes abnormal signs and symptoms. The diagnosis is based on a reduction in the blood cholinesterase activity. The specific treatment comprises the administration of atropine and pralidoxime. CONCLUSION: Accidental poisoning by organophosphate insecticides may occur, due to the misuse of such substances as shampoo against lice. An accurate information for users is necessary.

[Congenital long QT syndrome in newborns]. / Syndrome du QT long congénital de révélation néonatale.

UNLABELLED: The perinatal manifestations of the long QT syndrome are rare, but early diagnosis and therapy are necessary to prevent sudden death. CASE REPORTS: A long QT syndrome was diagnosed in two neonates who presented with foetal bradycardia. In one case, a mutation in the gene KCNQ1 was identified, and a long QT syndrome was diagnosed in the mother and two brothers of the neonate. On beta-blocker therapy, one infant became free of long QT syndrome related symptoms, but a sudden death of the second infant occurred. CONCLUSION: The long QT syndrome should be considered in the differential diagnosis of foetal bradycardia. Early treatment of the neonate and his family may prevent ventricular arrhythmias and sudden death.