Sinus Venosus ASDs: Imaging and Percutaneous Closure.

PURPOSE OF THE REVIEW: Percutaneous closure of sinus venosus atrial septal defects (ASD) using covered stent implantation is a new and promising minimally invasive technique. New imaging tools are used to ensure preoperative anatomical characterization and preoperative guidance, which are key procedural success factors. Here we will describe and analyze these recent developments. RECENT FINDINGS: Sinus venosus ASDs present a wide variety of anatomical features which must be described and analyzed using various imaging tools, including 3D technology. Percutaneous closure is challenging, but can hasten clinical recovery compared to the gold-standard conventional open-heart surgery. The feasibility of percutaneous closure relies on precise preoperative anatomical study and on real-time guidance using a multimodal fusion imaging process. Three-dimensional modeling of sinus venosus ASD is essential to understand the large anatomical panel encountered in this pathology. Multimodal fusion imaging guidance is very useful for performing sinus venosus ASD percutaneous closure in selected patients.

[Respiratory syncytial virus brainstem encephalitis in a 7-year-old boy]. / Rhombencéphalite liée au virus respiratoire syncytial chez un garçon de 7 ans.

The literature reports that neurological complications of childhood respiratory diseases due to respiratory syncytial virus (RSV) fluctuate between 1 and 40% of cases. They mostly involve central apnea - often the first symptom of infection - anoxia, and ischemic brain damage due to severe sudden weakness in infants, and seizures and consciousness disorders more or less associated with focalized neurological deficiency proving an encephalitis lesion. We report the case of brainstem encephalitis in a 7-year-old boy with RSV A nasopharyngitis, with meningitis, positive polymerase chain reaction in cerebrospinal fluid and magnetic resonance imaging (MRI) abnormalities, which was explained by viral replication encephalitis. Based on a literature review, we discuss the main aspects of epidemiology and physiopathology of the main neurological complication of RSV. Most of them have not been fully investigated and only a few articles report encephalitis. As far as central apnea is concerned, an animal experimental hypothesis surprisingly suggests a peripheral mechanism.

[Value of lumbar puncture after a first febrile seizure in children aged less than 18 months. A retrospective study of 157 cases]. / Utilité de la ponction lombaire lors de la première convulsion fébrile chez l'enfant de moins de 18 mois. Étude rétrospective de 157 cas.

AIM: Because meningitis symptoms are not very specific under the age of 18 months, lumbar puncture (LP) was widely recommended in children presenting a febrile seizure (FS). Recent retrospective studies have challenged this age criterion. In 2011, the American Academy of Pediatrics updated its guidelines for the first episode of simple FS: LP is indicated if signs suggestive of meningitis are present and remains "an option" in case of prior antibiotic treatment or between the age of 6 and 12 months if the child is not properly vaccinated against Haemophilus and Streptococcus pneumoniae. Because the meningitis epidemiology and the vaccination coverage are different, the objective of this study was to evaluate whether these new guidelines were applicable in France. PATIENTS AND METHODS: Between 2009 and 2010, we conducted a retrospective single-center study including 157 children aged less than 18 months admitted to the pediatric emergency department (Children's Hospital, Toulouse, France) for their first febrile seizure. The data collected were: type of seizure, knowledge of prior antibiotic treatment, neurological status, signs of central nervous system infection, and biological results (LP, blood cultures). RESULTS: Lumbar puncture was performed in 40% of cases (n=63). The diagnosis of meningitis/encephalitis was selected in eight cases: three cases of viral meningitis, three bacterial meningitis (Streptococcus pneumoniae), and two non-herpetic viral encephalitis. The incidence of bacterial meningitis in our study was 1.9%. The risk of serious infection, bacterial meningitis or encephalitis, was increased when there was a complex FS (14% versus 0% with a simple FS, P=0.06). The presence of other suggestive clinical symptoms was strongly associated with a risk of bacterial meningitis/encephalitis (36% in case of clinical orientation versus 0% in the absence of such signs, P<0.001). DISCUSSION: All severe clinical presentations were associated with complex FS (prolonged, focal, and/or repeated seizures) and the presence of other suggestive clinical signs (impaired consciousness lasting longer than 1h after the seizure, septic aspect, behavior disorders, hypotonia, bulging fontanel, neck stiffness, petechial purpura). The risk of bacterial meningitis or encephalitis associated with a simple FS and followed by a strictly normal clinical examination is extremely low. CONCLUSION: After a simple febrile seizure without any other suggestive signs of meningitis, systematic lumbar puncture is not necessary even in children younger than 18 months. LP remains absolutely indicated if clinical symptoms concentrate on central nervous system infection and should be discussed in case of complex seizures, prior antibiotic treatment, or incomplete vaccination.

[Severe hydrogen sulfide intoxication: a pediatric case of survival]. / Intoxication accidentelle grave à l'hydrogène sulfuré: un cas pédiatrique de survie.

We report a paediatric case of survival following severe hydrogen sulfide (H2S) gas intoxication. A 13-year-old boy was found submerged to the neck in a manure tank. He was hypothermic, unresponsive with bilateral mydriasis, and had poor oxygen saturation. After intubation, he was transferred to the paediatric intensive care unit of a tertiary care children's hospital. He developed acute respiratory distress syndrome (ARDS) requiring high frequency percussive ventilation. Cardiac evaluation was significant for myocardial infarction and left ventricular function impairment. He completely recovered from the respiratory and cardiac failure. Neurological examinations showed abnormal signals on MRI in the semi-oval center and in the frontal cortex. Follow-up detected partial impairment of axonal fibers of the right external popliteal sciatic nerve. Paediatric cases of survival after H2S intoxication have been rarely reported. Such exposures can evolve to severe ARDS and benefit from high frequency percussive ventilation. Hypothermia and other metabolic abnormalities are now better explained thanks to actual knowledge about endogenous H2S function. Lessons learned from paediatric accidents should result in better information about this threat for farmers and families living in houses with septic tanks, reducing the risk to their own and their children's safety.

[Guillain-Barré syndrome in a child with normal tendon reflexes]. / Un cas pédiatrique de syndrome de Guillain-Barré à réflexes conservés.

We describe the case of a 10-year-old child with the acute motor axonal neuropathy (AMAN) form of Guillain-Barré syndrome (GBS) with preserved tendon reflexes, 6 days after a bout of gastroenteritis. The child quickly showed weakness of the distal muscles of his four limbs, with preserved tendon reflexes and a raised CSF protein concentration with no cells. Nerve conduction studies showing motor axonal degeneration confirmed the diagnosis of GBS in spite of preserved tendon reflexes. The serum was positive for IgG antibodies to gangliosides GM1 and GD1b. The child received intravenous immunoglobulins, which resulted in a favorable progression. This case proves that GBS with normal tendon reflexes exists. The other cases of SGB with preserved tendon reflexes already described in the literature were the AMANs form with antibodies to gangliosides in the serum and only adults were affected.

[Chronic meningococcemia: 3 cases in the immunocompetent child]. / Méningococcémie chronique: trois cas chez l'enfant immunocompétent.

UNLABELLED: Chronic meningococcemia is a part of extra meningeal manifestations of meningococcal disease. Its diagnosis can be difficult because of lack of sensitivity of blood cultures. CASE REPORT: Three cases, concerning immunocompetent children, respectively aged of 14, 10 and 4 years are reported. The clinical course was characterized by recurrent fever, inflammatory joint manifestations and diffuse maculopapules secondary centered by petechiae. Microbiological findings revealed in one case a positive throat culture and presence of meningococcal soluble antigens in blood and urine. In the other two cases, diagnosis was done after done after positive blood culture at the 7th, and 13th days of course. CONCLUSION: The diagnosis should be considered in any children with a prolonged, recurrent fever and cutaneous and joint manifestations even if blood cultures remain negative. The response to therapy by usual antimeningococcal antibiotics is dramatic and curative while a prolonged untreated course may be complicated by metastatic infection.

[Clear cell meningioma: recurrent intraspinal tumor in a child]. / Méningiome à cellules claires: tumeur rachidienne récidivante chez un enfant.

BACKGROUND: Meningiomas represent 1.5% to 4.3% of cerebral and medullar primary tumors in children. CASE REPORT: A 9-year-old girl had a history of thoracolumbar scoliosis. An intracanalar and extramedullar tumor was confined to the lumbar region. Resection identified a clear cell meningioma. A symptomatic and tumoral recurrence occurred 5 months later in the same region. After a second resection, the patient received radiotherapy. At 8 months follow-up, no recurrence was documented. CONCLUSIONS: A stiff and painful scoliosis can be predictive of expansive intracanalar tumor. The recurrent or multifocal evolution of clear cell meningioma show the "aggressive behavior" of this histological type. A preventive radiotherapy could be proposed, depending on the age of the patient and the localization of the tumor.

Paradoxical effect of ethanol on liver lipogenesis in the genetically-obese Zucker rat.

Sixteen obese (fa/fa) Zucker rats, sixteen lean (Fa/-) Zucker rats and sixteen Wistar rats, all male rats aged 7-8 weeks, were given either a control (C) diet containing no ethanol or an ethanol (E) diet in which 36% of the energy was supplied by ethanol, for a period of 4 weeks. The activities of glucose-6-phosphate dehydrogenase (EC 1.1.1.49), glucose-6-phosphatase (EC 3.1.3.9) and glycerol kinase (EC 2.7.1.30) and the glycogen content in the livers of obese (fa/fa) rats were lower in animals given diet E than in those given diet C. As a result, hepatic lipogenesis and fatty degeneration of the liver were reduced in obese (fa/fa) rats given diet E.

Influence of chronic ethanol intake on obesity, liver steatosis and hyperlipidaemia in the Zucker fa/fa rat.

The effect of chronic alcohol intoxication on metabolic disturbances and fatty infiltration and degeneration was studied in genetically obese, hyperlipoproteinaemic, fa/fa Zucker rats. Sixteen obese Zucker (fa/fa) rats, sixteen lean Zucker rats (Fa/-) and sixteen Wistar rats, all male rats aged 7-8 weeks, were given either a control (C) diet (13% of energy from protein, 37% from fat, 50% from carbohydrate) or an ethanol (E) diet (13% of energy from protein, 37% from fat, 14% from carbohydrate, 36% from ethanol) for 4 weeks. The fa/fa rats given diet E consumed more energy than those given diet C, but after 4 weeks the weight gains and degrees of obesity were similar for both groups. With both diets, the developed hyperlipidaemia could be explained by the hyperinsulinaemia. Both hypertriglyceridaemia and hypercholesterolaemia were lower in fa/fa rats eating diet E than in those given diet C. Fatty infiltration of the liver, as assessed by hepatic triacyglycerol and cholesterol contents, was observed with both diets, but for fa/fa rats it was less extreme in those given diet E.

Effects of chronic ethanol intake on mobilization and excretion of cholesterol in baboons.

To investigate the effects of chronic ethanol administration on the mobilization and excretion of cholesterol, turnover and balance studies were carried out in baboons pair-fed cholesterol-free diets containing 50% of energy either as ethanol or as additional carbohydrate for several years. Ethanol feeding increased free cholesterol in all plasma lipoprotein fractions, and esterified cholesterol in very low density lipoprotein, intermediate density lipoprotein, and high density lipoprotein (HDL). The major increase occurred in HDL, mainly as esterified cholesterol. The latter was associated with decreased transfer of esterified cholesterol from HDL to low density lipoprotein. By contrast, the smaller increase in HDL-free cholesterol was associated with increased turnover in the plasma, increased splanchnic uptake, and increased fecal excretion of plasma cholesterol, mainly as neutral steroids. Cholesterol extraction predominated over release in the splanchnic vascular bed, suggesting that the excess of cholesterol excreted in the feces originated in extrasplanchnic tissues. Thus, these findings indicate that alcohol consumption favors mobilization of tissue free cholesterol for hepatic removal and excretion. By contrast the increase in HDL-cholesterol (mainly esterified) appears to be a poor indicator of cholesterol mobilization.

[Effects of a diet containing ethanol on the composition of fatty acids of liver, blood and adipose tissue in the Zucker fa/fa rat]. / Effets d'un régime contenant de l'éthanol sur la composition en acides gras du foie, du sang et du tissu adipeux du rat Zucker fa/fa.

Zucker fa/fa rats were subjected to chronic ethanol intoxication; (alcohol incorporated into the diet represented 36% of the energy of the control diet. The amount of endogenous hepatic fatty acids was lower with the alcohol diet than with the control diet. The incorporated alcohol had no significant influence on the fatty acid composition of adipose tissue. The variations in the fatty acid composition of the blood followed the same patterns as those of the rat liver.

[Lactase deficiency in the intestinal mucosa. Demonstration by hydrogen detection in the expired air after lactose loading (author's transl)]. / Déficit de la muqueuse intestinale en lactase. Mise en évidence par détection d'hydrogène dans l'air expiré après charge en lactose.

Lactose malabsorption can be demonstrated by detecting hydrogen (H2) in the expired air after oral loading with lactose. A comparative study was carried out on 24 subjects. Following an oral loading dose of lactose, the H2 eliminated during expiration was assayed by gas chromatography, and blood galactose levels were measured. The results showed that the test was reliable, well tolerated and reproductible. However, the method does not measure the amounts of lactase present in the intestinal mucosa. The lactose loading test seems to be valuable for studies on lactose and carbohydrate malabsorption.