Congenital neuroblastoma presenting as profound neonatal metabolic acidosis with systemic hypertension: Case report.

BACKGROUND: Clinical collapse in the newborn most often occurs secondary to sepsis, delivery complications, congenital cardiac defects, or inborn errors of metabolism. We report on a neonate with respiratory, cardiac, and hepatic failure, with disproportionate metabolic acidosis and systemic hypertension, found to be caused by a congenital neuroblastoma. CASE DESCRIPTION: A term infant presented to our Level IV NICU via emergent transport at 12 hours of life with clinical and laboratory findings consistent with respiratory, cardiac, and hepatic failure. Typical workup for sepsis and cardiac etiology was unrevealing. The infant was noted to have systemic hypertension despite prolonged capillary refill. A profound metabolic acidosis led the primary team to pursue a genetics consult. In the course of the workup for disorders of metabolism, a urine organic acids panel revealed an elevated HVA (homovanillic acid) and VMA (vanillylmandelic acid), the metabolites of the neurotransmitters epinephrine, norepinephrine and dopamine. Subsequent abdominal ultrasound and chest/abdomen CT revealed a large heterogeneous mass with internal vascular flow and scattered calcifications arising from the medial limb of the left adrenal gland, consistent with neuroblastoma. CONCLUSION: Although rare, neuroblastomas can present clinically in the perinatal period in a manner requiring immediate life-saving intervention. Providers should consider the diagnosis in the setting of a newborn with a sepsis-like syndrome or profound metabolic acidosis presenting along with systemic hypertension without clear underlying etiology.

Acute asthma management in children: factors identifying patients at risk for intensive care unit treatment.

The hospitalization and mortality rates incurred from childhood asthma continue to rise despite recent progress into the pathophysiology and treatment of reactive airway disease. We believe that there are specific factors that identify children at risk for death from asthma. The objective of the study was to determine those risk factors that identify children at increased risk for nonfatal, but life-threatening asthma exacerbations resulting in pediatric intensive care unit (PICU) admission. Patients aged 10 months-16 years admitted to Brenner Children's Hospital for status asthmaticus between April 1991 and December 1992 were evaluated with regard to the prevalence of eight different factors using two categories for asthma: (1) life-threatening asthma requiring PICU admission and (2) non-life-threatening asthma requiring routine hospitalization. Risk factors associated with an increased prevalence of non-fatal, but life-threatening asthma requiring PICU sensitivity state. The classification employed here seemed to provide some merits in delineating the features of adulthood asthma.

Outcome of neonatal strokes.

We examined the clinical outcome of 17 children, 1 to 11 years of age, who experienced major cerebral artery infarctions (strokes) as neonates. Nine of the 17 children had left middle cerebral artery (MCA) infarctions, five had right MCA infarctions, two had bilateral MCA infarctions, and one had a left posterior cerebral artery infarction. Fourteen of the 17 children developed seizures as neonates. Most of these children who developed seizures were neurologically abnormal as neonates, became seizure free and neurologically normal early in the first year of life, and their anticonvulsant therapies were discontinued. After a seizure-free period of one to eight years, three of the 14 patients again required anticonvulsant therapy for seizure control. Two of the 16 surviving children continue to be severely handicapped while 11 of the 16 are making apparently normal developmental progress. One of the two children presently attending school has cognitive deficits appropriate to the site affected by the original infarction. Most children with neonatally diagnosed strokes appear to have a good short-term outcome, but later onset of seizures and subsequent recognition of cognitive deficits may not be uncommon.