Sharing features of uncommon respiratory syncytial virus complications in infants.

We describe 4 nonconsecutive cases of infants admitted to Catholic University pediatric intensive care unit (PICU) because of complicated respiratory syncytial virus (RSV) infection during winter RSV outbreaks from the year 2000 to the year 2003. A hyponatremic epileptic status (as in the first case) has been reported by several authors as a rare RSV complication, potentially leading to death. The second infant developed a serious pulmonary edema after a subglottic obstruction (croup) associated with RSV infection. The remaining 2 infants developed a pneumothorax and subcutaneous emphysema while breathing spontaneously during an RSV bronchiolitis. In all infants, a full recovery and PICU discharge was achieved despite the need for mechanical ventilation in cases 1 and 2. Increased intrapleural negative pressure or its combination with hypoxia/hypercapnia has been suggested as the common factor possibly joining these different clinical pictures.

Lung injury from "liquid ecstasy": a role for coagulation activation?

We describe our second clinical observation of pulmonary injury after a "liquid ecstasy" ingestion/inhalation by a 3-year-old girl. Apart from the deep coma state, a markedly asymmetric pulmonary compromise was recorded. A transient coagulation activation was detected, possibly triggered off by the toxic effect on the lung alveolar-capillary membrane.

Intraventricular nerve growth factor infusion: a possible treatment for neurological deficits following hypoxic-ischemic brain injury in infants.

OBJECTIVE AND IMPORTANCE: Hypoxic-ischemic brain injuries in childhood are associated with poor neurological outcome. Unfortunately, no new therapeutic approaches have been proposed. Recently, animal studies show that nerve growth factor (NGF) can reduce neurological deficits following hypoxic-ischemic brain injuries. The objective of this study is to demonstrate the therapeutic effects of intraventricular NGF infusion in severe post-ischemic damage. CLINICAL PRESENTATION: Two infants, aged 9 and 8 months, with hypoxic-ischemic brain damage, secondary to prolonged cardiorespiratory arrest and stabilized after the conventional treatment, were treated with intraventricular NGF infusion. Before the therapy both infants were comatose with asymmetrical tetraparesis; their Glasgow Coma Scale (GCS) was 4 and 5, respectively. One month after the treatment, their GCS was 8 and 9, respectively. EEG examinations performed after the NGF infusion showed an increased alpha/theta ratio. MRI showed a reduction of malacic areas in the brain. A SPECT study, performed only in one infant, demonstrated that the NGF treatment resulted in an improvement of regional cerebral perfusion in right temporal and occipital cortices. INTERVENTION: The drug utilized was 2.5S NGF purified and lyophilized from male mouse submaxillary glands. NGF infusion was started about 30 days after the hypoxic-ischemic brain injury. 0.1 mg NGF was administered via the external drainage catheter into the right cerebral ventricle once a day for 10 days consecutively. CONCLUSION: Our observations are interesting, but further studies are necessary to confirm the effects of NGF in hypoxic-ischemic brain injuries in infants.

Neurotrophic factor expression in three infants with Ondine's curse.

This study investigates the expression of some neurotrophic factors (brain-derived neurotrophic factor, glial-derived neurotrophic factor, and nerve growth factor) in the cerebrospinal fluid of infants suffering from idiopathic congenital central hypoventilation syndrome and determines their correlations with this syndrome. Cerebrospinal fluid samples were collected from three infants suffering from idiopathic congenital central hypoventilation syndrome and 15 control subjects with obstructive hydrocephalus to measure the expression of brain-derived neurotrophic factor, glial-derived neurotrophic factor, and nerve growth factor using an immunoenzymatic assay. In the cerebrospinal fluid of patients, analysis of neurotrophic factors expression indicated a reduction, not statistically significant, of brain-derived neurotrophic factor compared with the mean level of the control group (1554 pg/mL, 1509 pg/mL, and 1582 pg/mL respectively, in comparison to 1954 +/- 103 pg/mL), whereas nerve growth factor and glial-derived neurotrophic factor did not undergo significant variations in either group. Neurotrophic factors, namely brain-derived neurotrophic factor, regulate the maturation and differentiation of respiratory neurons. The reduced expression of brain-derived neurotrophic factor in the cerebrospinal fluid samples of infants with Ondine's curse, although not statistically significant, is suggestive of a dysregulation in the brain-derived neurotrophic factor synthesis that could play an important role in the breathing disorders observed in patients with idiopathic congenital central hypoventilation syndrome.

Noninvasive ventilation options in pediatric myasthenia gravis.

A 10-month-old female infant with congenital myasthenic syndrome suffering from acute respiratory failure was supported using face mask positive pressure ventilation until definitive diagnosis and specific treatment was achieved. A 12-year-old girl suffering from seronegative myasthenia gravis was treated by helmet-delivered noninvasive ventilation during recurrent myasthenic episodes. Noninvasive support was really beneficial in the myasthenic crisis with respiratory muscle weakness, whereas a shift to tracheal intubation was necessary when pulmonary infection and multiple atelectasis occurred. The new helmet interface for noninvasive positive pressure ventilation can represent a valuable means of respiratory support in the early phase of respiratory failure in older children.

Fatal coronary artery anomaly presenting as bronchiolitis.

UNLABELLED: During winter outbreaks of respiratory syncytial virus bronchiolitis from 2002 to 2004, three infants presented with a presumptive diagnosis of lower respiratory tract infection and wheezing. The clinical condition in two cases was rapidly progressive and precipitated into intractable shock; clinical and instrumental examinations revealed a cardiac origin of their illness. A subacute presentation permitted a cardiological assessment and a proper treatment in the third infant. An abnormal origin of the left coronary artery from the pulmonary trunk was demonstrated in all cases. The concurrent acute airway infection had a catastrophic effect on the underlying cardiovascular anomaly leading to refractory cardiogenic shock and death. CONCLUSION: Admission chest X-ray film and arterial gas analysis can raise the suspicion of cardiac involvement when treating a severe wheezing episode in young infants. Paediatric cardiological evaluation with two-dimensional echocardiography may eventually reveal this rare condition, whereas cardiac catheterisation with aortography remains the standard means of diagnosis.

Neurotrophin overexpression in lower airways of infants with respiratory syncytial virus infection.

RATIONALE: Nerve growth factor and its receptors are upregulated in the lungs of weanling rats with lower respiratory tract infection caused by the respiratory syncytial virus (RSV), and this is a major mechanism of the inflammatory response against the virus. However, no information is available in humans. OBJECTIVES: We sought to determine whether the expression of neurotrophic factors and receptors is increased in human infants infected with RSV. METHODS: We used a highly sensitive immunoassay to measure the concentrations of nerve growth factor and brain-derived neurotrophic factor proteins in serum samples and in the supernatants and cell fractions of nonbronchoscopic bronchoalveolar lavage fluid. We also used immunofluorescence to detect expression in airway cells of the tyrosine kinase receptor TrkA, which binds nerve growth factor with high affinity. Samples were obtained at 24 and 48 hours postintubation from 31 mechanically ventilated infants: 15 patients with RSV infection, 5 patients with respiratory infection negative for RSV, and 11 postsurgical patients without respiratory infection. MAIN RESULTS: Data show significantly higher concentrations of both neurotrophins in the cell fractions of bronchoalveolar lavage samples obtained from infants with RSV infection compared with control infants, whereas no significant difference was found in supernatants or serum samples. We also detected tyrosine kinase receptor immunoreactivity only in cells from airways infected with RSV. CONCLUSIONS: We conclude that neurotrophic factors and receptors are overexpressed in human airways infected by RSV, and may contribute to airway inflammation and hyperreactivity.

Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients.

BACKGROUND: Cases of respiratory failure at presentation in children with anterior mediastinal malignancies can be very challenging for clinicians. Seven consecutive children presenting with superior mediastinal syndrome are reported needing urgent critical care approach. PATIENTS AND METHODS: Seven children (age range from 0.8 to 14 years; mean, 4.5 years) suffered from critical mediastinal neoplasms. Clinical presentation, laboratory findings, treatment, and outcome are discussed. SETTING: a tertiary-care 6-bed medical and surgical pediatric intensive care in a university hospital. Interventions included emergency management, resuscitation and intensive care admission, and diagnostic and therapeutic procedures. RESULTS: All cases showed a respiratory compromise and underwent ventilatory and/or cardiovascular support. Two patients needed renal replacement therapy. Pediatric Intensive Care Unit discharge was achieved in all patients. CONCLUSIONS: Critical or extreme presentation of mediastinal neoplasms does not preclude a good clinical outcome: an intensive care approach is essential to allow patient recovery and effective antineoplastic therapy administration.

Interleukin 1beta and interleukin 6 relationship with paediatric head trauma severity and outcome.

BACKGROUND: Based on the known inflammatory role of interleukins (IL), we evaluated IL-1beta and IL-6 expressions and their association with the severity of traumatic brain injury (TBI; Glasgow Coma Scale [GCS]) and the outcome (Glasgow Outcome Score [GOS]) recorded in a paediatric population. DESIGN: The design was a perspective observational clinical study carried out in the paediatric intensive care unit of the University Hospital. METHODS: We measured the IL-1beta and IL-6 levels in 14 children with severe TBI (patients) and in 12 children with obstructive hydrocephalus (control group). Cerebrospinal fluid (CSF) and plasma samples were collected 2 h (T1) and 24 h (T2) after TBI. Interleukins were assayed using the immunoenzymatic method. RESULTS: The IL-1beta mean level was significantly lower than the IL-6 mean level both in the CSF and plasma of TBI children. In the CSF, the IL-1beta level increased from 55.71+/-72.79 pg/ml at T1 to 106.10+/-142.12 pg/ml at T2 and the IL-6 level increased from 405.43+/-280.28 pg/ml at T1 to 631.57+/-385.35 pg/ml at T2; a similar trend was observed in plasma. We found a statistically significant correlation between the increase in CSF and plasma interleukin levels between T1 and T2 and head injury severity (GCS

A case of acute lymphoblastic leukemia presenting as severe hypercalcemia.

A 9-year-old girl presented at the Pediatric Emergency Department with an acute onset of gastrointestinal symptoms due to hypercalcemia. Despite the absence of circulating blast, bone marrow biopsy was diagnostic of acute lymphoblastic leukemia. The hypercalcemia was initially treated with intravenous hydration and furosemide, and later on with bisphosphonates. However, the serum calcium levels normalized only after the beginning of specific chemotherapy. Hypercalcemia represents an emergency in children, and acute leukemia must be considered in its differential diagnosis, even when there are no circulating blasts.

Massive congenital intracranial teratoma: perioperative coagulation impairment.

Massive congenital intracranial teratoma is a rare neoplasm with a poor prognosis. Surgery may be curative only if complete resection can ben obtained. Several single case reports have been published in the pediatric literature, mostly focusing on prenatal diagnosis. The authors describe six patients with congenital intracranial teratoma treated at their institution in the past decade. Perioperatively, a marked hemostatic derangement was observed in three of them undergoing surgery: the pathophysiology of this complication is discussed. The surgical indication itself represents an ethical dilemma when treating a large intracranial tumor with partial destruction and replacement of brain structures.

Treatment of acute respiratory failure by helmet-delivered non-invasive pressure support ventilation in children with acute leukemia: a pilot study.

OBJECTIVE: To evaluate the feasibility of non-invasive ventilation (NIV) through a new interface-the helmet-in the treatment of acute respiratory failure (ARF) in hematologic children. DESIGN AND SETTING: Observational, non-randomized report of four consecutive cases. Pediatric Intensive Care Unit in a university hospital. PATIENTS AND METHODS: Four consecutive females (aged 9-17 years) affected by acute leukemia (3 acute lymphocytic leukemia [ALL], 1 acute myeloid leukemia [AML]) and with hypoxemic ARF (defined by severe dyspnea at rest, respiratory rate >30 breaths/min, PaO2:FiO2 <200 and active contraction of the accessory muscles). Pressure support ventilation was delivered via a helmet (CaStar,Starmed, Italy) by means of an ICU ventilator (Servo 300, Siemens Elema, Sweden). RESULTS: We evaluated the effect of pressure support ventilation delivered by helmet on blood gases, respiratory rate, hemodynamics, patient tolerance, complication rate and outcome. An improvement of oxygenation was uniformly observed within the first 3 h after admission. The helmet was well tolerated by all children. No complication was observed. Two patients were discharged from the PICU in stable clinical conditions, whereas the remaining two children overcame the respiratory distress but had non-respiratory complications and eventually died. CONCLUSION: Non-invasive ventilation via the helmet can offer effective ventilatory support and improve gas exchange in the treatment of ARF in pediatric hematologic patients. As already shown in adults, NIV may decrease the risk of life-threatening complications associated with invasive mechanical ventilation (MV), also in children with hematologic malignancies; moreover, it offers the possibility of an earlier approach to respiratory failure in this patient subset.

Malignant hypertension and cerebral haemorrhage in Seckel syndrome.

UNLABELLED: Seckel syndrome is an autosomal recessive condition with severe short stature and facial and neurological anomalies. Intracranial haemorrhage, due to rupture of a cerebral aneurysm, is a very rare complication of this syndrome. Malignant hypertension may play an important role in the aetiology of the aneurysm and early detection is essential in order to prevent organ damage. CONCLUSION: we report a new case of Seckel syndrome associated with malignant hypertension and cerebral haemorrhage.

Correlation between neurotrophic factor expression and outcome of children with severe traumatic brain injury.

OBJECTIVES: We evaluated the neurotrophic factors [nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), glia-derived neurotrophic factor (GDNF)] expression and their association with the severity and outcome of children with traumatic brain injury. DESIGN: Prospective observational clinical study. SETTING: Pediatric intensive care unit. PATIENTS: Fourteen children with severe head injury; 12 controls with obstructive hydrocephalus. MEASUREMENT: Cerebrospinal fluid (CSF) and plasma samples were collected 2 h (T1) and 24 h (T2) after head injury. Neurotrophic factor levels were measured using an immuno-enzymatic assay. MAIN RESULTS: In patients, neurotrophic factor mean levels were significantly different in both CSF and plasma, showing high levels of BDNF compared to NGF and GDNF. Considering T1 and T2 expression, in the CSF the level of NGF increased from 3.5+/-0.4 pg/ml to 48.2+/-11.7 pg/ml ( p<0.001); BDNF decreased from 4854.0+/-1303.7 pg/ml to 593.0+/-114.8 pg/ml ( p<0.001), while GDNF did not undergo significant variations. In plasma, no significant changes were observed. Regarding severity and outcome, BDNF levels showed a sharp peak after head injury, but the only significant association was between NGF expression in the CSF and a good outcome versus a poor outcome ( p=0.007). CONCLUSIONS: The variations in neurotrophic factor levels reflect an endogenous attempt at neuroprotection against biochemical and molecular changes after traumatic head injury. BDNF represents an early marker of brain injury, while NGF expression in the CSF was indicative of a good outcome and the role of this neurotrophin in the treatment of children with severe head injury may be hypothesized.

The impact of initial management on the outcome of children with severe head injury.

BACKGROUND: Neurotrauma is one of the most important causes of death in the pediatric age group. Head injury is responsible for both primary and secondary brain damage. The outcome of children with brain injury depends on the nature of the primary damage and on how well secondary brain damage can be limited. Appropriate critical care management at the scene and in hospital can prevent secondary brain damage that would otherwise result from cerebral reactions. Hypoxia and hypotension are major early complications of aggravation of secondary brain damage that are amenable to emergency maneuvers at the scene. In the literature, the influence of initial critical care management and early complications on the outcome of children with severe head injury is not clearly documented. METHODS AND RESULTS: We have prospectively examined the impact of the management at the scene on outcome in 40 children admitted to our Pediatric Intensive Care Unit with severe head injury (Glasgow Coma Scale < or = 8). The outcome of these children was assessed using the Glasgow Outcome Score (GOS). The results were evaluated by univariate and multivariate analysis. In our series the length of time before admission to an intensive care unit appears to have influenced the outcome among survivors, while the severity of injury is the only factor statistically associated with early complications (hypoxia and hypotension). With regard to the impact of early complications on outcome, the multivariate analysis showed that hypoxia and hypotension were significantly associated with GOS, independently of the GCS. CONCLUSION: Our results confirm the need for a precise treatment strategy for the initial management of children with neurotrauma to decrease the incidence of secondary brain damage attributable to hypoxia and hypotension.