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Therapy for asthma is being prescribed more frequently. However, asthma mortality remains high in many countries. Fatal outcome is not always related to inadequate follow-up. In this article we report that children with mild to moderate symptoms may present a fatal attack. Many factors are responsible for such an outcome: asphyxia +3 due to ventilation/perfusion mismatch and/or bronchospasm, cardiac failure, cardiac arrhythmia, intrinsic positive expiratory pressure, or metabolic disturbances (hypokalemia, for example). Such problems can occur in predisposed patients: it has been shown that the chemosensitivity to hypoxia and the perception of dyspnoea are altered in certain patients with near-fatal asthma. It is very important to identify children at risk of severe asthma and to organize care so as to optimize the management of such children.

We report the case of a child with 22q11 microdeletion who presented with abdominal lymphatic dysplasia resulting in exsudative enteropathy. This primitive and localized lymphatic malformation is consistent with the vascular theory in the velocardiofacial syndrome.

Oesophageal atresia usually occurs without any genetic background. Three cases associated with Fanconi's anaemia are reported. One neonate had growth retardation and numerous malformations including oesophageal atresia and four other components of the VACTERL association. In the two others, oesophageal atresia was isolated. In patients with such malformations an early diagnosis of Fanconi's anaemia may have important genetic and therapeutic implications.

Severe community-acquired pneumonia (CAP) is still a serious disease with a high mortality rate, especially in developing countries. Children under 5 years are more prone to severe CAP. In this article, the authors review the definition and clinical criteria used in the initial evaluation and decision to hospitalize patients. The diagnosis approach requires only a limited number of laboratory tests before initiating the supportive measures and wide spectrum antimicrobial therapy. If the patient fails to respond favorably 48-72 hours after initiating therapy, more invasive investigations are indicated. After resolution, an immunological evaluation is warranted.

We conducted a prospective study in the multidisciplinary pediatric intensive care unit (pediatric ICU) of a tertiary-care university hospital in order to determine the incidence, risk markers, risk factors, and complications related to bacterial nosocomial pneumonia (BNP) and tracheitis (BNT) in children. A cohort of 1,114 consecutive admissions to the pediatric ICU was enrolled over a 56-wk period; 154 cases were excluded mostly (75%) because they already had a respiratory infection at entry. The final sample included 960 admissions (831 patients). Diagnosis of BNP or BNT was based on Centers for Disease Control of Atlanta criteria using a consensus method involving three experts, who also attributed complications to BNP and BNT. A total of 29 BNP and BNT (3.0%; 95% CI: 1.1 to 4.1%) were diagnosed (BNP: 1.2%, 95% CI: 0.7 to 1.9%; BNT: 1.8%, 95% CI: 0.8 to 2.6%). Three factors were retained by multivariate analysis as independent risk factors or markers for BNP (immunodeficiency, immunosuppression, and neuromuscular blockade), and two for BNT (head trauma and respiratory failure). Gram-negative bacteria and Staphylococcus aureus were the microorganisms most frequently found in the tracheal aspirates. Prescription of antibiotics was commonly attributable to BNP (75%) and BNT (59%). Death, as well as multiple organ system failure, resulted from BNP in 8% of cases, but never from BNT. In BNT, the reintubation rate was 24%. Nosocomial bacterial respiratory infections are rare in critically ill children. However, BNP causes significant complications, and more attention should be focused on BNT in the critically ill child.

Study Objectives: To determine the cumulated incidence and the density of incidence of systemic inflammatory response syndrome (SIRS), sepsis, severe sepsis, septic shock, and multiple organ dysfunction syndrome (MODS) in critically ill children; to distinguish patients with primary from those with secondary MODS., Design: Prospective cohort study., Setting: Pediatric ICU of a university hospital., Patients: One thousand fifty-eight consecutive hospital admissions., Interventions: None., Measurements and Results: SIRS occurred in 82% (n=869) of hospital admissions, 23% (n=245) had sepsis, 4% (n=46) had severe sepsis, 2% (n=25) had septic shock; 16% (n=168) had primary MODS and 2% (n=23) had secondary MODS; 6% (n=68) of the study population died. The pediatric risk of mortality (PRISM) scores on the first day of admission to pediatric ICU were as follows: 3.9 +/- 3.6 (no SIRS), 7.0 +/- 7.0 (SIRS), 9.5 +/- 8.3 (sepsis), 8.8 +/- 7.8 (severe sepsis), 21.8 +/- 15.8 (septic shock); differences among groups (p=0.0001), all orthogonal comparisons, were significant (p<0.05), except for patients with severe sepsis. The observed mortality for the whole study population was also different according to the underlying diagnostic category (p=0.0001; p<0.05 for patients with SIRS and those with septic shock, compared with all groups). Among, patients with MODS, the difference in mortality between groups did not reach significance (p=0.057). Children with secondary MODS had a longer duration of organ dysfunction (p<0.0001), a longer stay in pediatric ICU after MODS diagnosis (p<0.0001), and a higher risk of mortality (odds ratio, 6.5 [2.7 to 15.9], p<0.0001) than patients with primary MODS., Conclusions: SIRS and sepsis occur frequently in critically ill children. The presence of SIRS, sepsis, or septic shock is associated with a distinct risk of mortality among critically ill children admitted to the pediatric ICU; more data are needed concerning children with MODS. Secondary MODS is much less common than primary MODS, but it is associated with an increased morbidity and mortality; we speculate that distinct pathophysiologic mechanisms are involved in these two conditions.

Background: A chronic intestinal pseudo-obstruction is sometimes seen in premature neonates who are fed early and subsequently suffer from digestive intolerance for several weeks., Patients: Seven premature babies (mean gestational age: 30.5 weeks, mean birthweight: 950 g) suffered from abdominal distension and failure to pass stools at a mean age of 2.5 days (extremes: 1 to 6 days); 2 of them also had vomiting. X-ray examination showed dilated loops of bowel throughout the abdomen without obstructive changes; barium or Gastrografin studies demonstrated inertia of the colon without obstructive changes or abrupt changes in caliber. Histological examination of enteric nerve cells in 2 cases showed normal maturation. Parenteral nutrition was necessary in all patients for 30 to 78 days (mean: 47), followed by continuous enteral feeding for 24 to 48 days (mean: 37). Septicemia complicated parenteral nutrition in 4 babies and was responsible for the death of 1 of them. Normal evacuation of stools occurred between day 27 and day 91 (mean day 46) allowing normal enteral feeding. All 6 patients, now aged 2.5 to 8 years (mean 3.5 years) are in good health, and have no digestive problems., Conclusions: Immaturity of intestinal motility may occur in some premature neonates suffering from intestinal ileus. Parenteral nutrition may be necessary for several weeks, but spontaneous recovery is usual. This transient intestinal pseudo-obstruction of premature babies is different from the classical chronic pseudo-obstruction for which surgery is often needed.

We report the case of an unusual form of Noonan's syndrome with multisystemic involvement identified in the early neonatal period. Apart from the common association of dysmorphia, cardiopathy and lymphatic vessel anomalies, a tumor-like naevoid cutis verticis gyrata of the scalp was observed. This type of cutaneous lesion has never been described in previous reports of the syndrome.

Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day). Histologically there were incomplete cirrhosis, microvesicular steatosis, major canalicular cholestasis with proliferative neocholangioles, and bile duct thrombi. There were also some iron pigments in the periportal area and partial glycogen depletion. By electron microscopy, mitochondria in numerous hepatocytes appeared abnormal with occasional cristae in a fluffy matrix, some containing dense inclusions. Study of respiratory chain activity showed a defect in cytochrome c oxidase (complex IV), revealed by oxygraphic measurement on fresh muscle biopsy and confirmed by spectrophotometric enzymatic assays performed on muscle and liver homogenates. The association of neonatal liver failure with hyperlactacidemia warrants investigation into a deficiency in oxidative phosphorylation.

Near-miss is the term used by English-speaking authors to define a sudden accident suggestive of imminent infant death. This is one of the most worrying problems, due to its frequency and its multiple possible causes, the most common of which are gastro-oesophageal reflux and vagal hypertonia. These accidents occur in the same age-group as the sudden infant death syndrome and in similar circumstances, even though they more often occur during waking. Near-miss therefore may constitute an abortive form of sudden infant death syndrome, which would make its study a good way for understanding the syndrome. Yet one should wait before making this assimilation as it might induce unwarranted medical procedures. The risk of recurrence (about 10%), sometimes lethal, exists, but is must be discussed after full investigation in search of a cause and a possible treatment. Electronic home monitoring is only one of the possible preventive measures; it must be decided upon and applied by a specialized medical team.

The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.

Two cases of spontaneous ileal perforation in neonates have been reported. The diagnosis is often difficult due to the lack of clinical or radiological signs. Other possible etiologies are excluded at laparotomy. Hypotheses concerning the etiology of spontaneous intestinal perforations have been discussed.

The Noonan syndrome is a multiple congenital anomalies syndrome with variable expressivity and autosomal dominant inheritance. We report an observation of a newborn with Noonan syndrome and an unusual molluscoid cutaneous excess over the scalp that might represent a new skin manifestation in Noonan Syndrome rather than a consequence of lymphatic dysplasia.

Eosinophilia is a common finding in premature babies during the neonatal period (75%). It has a variable correlation with several factors that may influence it: gestational age, birth weight, time required to regain birthweight, neonatal stress, caesarean birth, formula feeding, endotracheal intubation, positive pressure ventilation, parenteral nutrition, umbilical catheterization, blood transfusions, antibiotics, phototherapy, sex, neutropenia and sepsis. The possible mechanisms are discussed: resolution of a stress, anabolic state, reaction to antigens, normal granulopoietic maturation, transplacental transfer of a factor stimulating the eosinophil synthesis.