A Case of Congenital Anaplastic Large Cell Lymphoma in a Very Preterm Low-Birth Weight Neonate.

A premature infant male was born at 30 weeks' gestation with severe coagulopathy and thrombocytopenia. Over the first days of his life, the patient developed evidence of immune hyperactivation with adenopathy, hepatosplenomegaly, and elevated ferritin. Although the patient met diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), flow cytometric based assays were not consistent with primary HLH. A lymph node and bone marrow biopsy eventually revealed the presence of anaplastic lymphoma kinase+anaplastic large cell lymphoma. To our knowledge, this is the earliest presentation of a lymphoma, and expands the known timeframe of lymphomagenesis.

Handheld, portable ultrasound in the neonatal intensive care nursery: a new, inexpensive tool for the rapid diagnosis of common neonatal problems.

The use of ultrasound imaging in the neonatal intensive care unit (NICU) has become an essential part of the evaluation and delivery of care for most neonates. Until recently, ultrasound machines were large, expensive, and often not immediately available, particularly at night and during weekends. Additionally, serial studies to define the evolution of an acute clinical situation were often not practical because of the dedicated time required and the expense involved. The recent introduction into our NICU of a high-quality, reasonably priced, and completely portable neonatal ultrasound unit (Sonosite, Bothell, WA) has now made it possible for neonatologists to rapidly obtain the hour-by-hour information that can be extremely helpful in the evaluation of a critically ill neonate. This paper illustrates some of the capabilities of this simplified device, and the value of having continuous on-site ultrasound availability in the NICU.

Poland syndrome with extracorporeal intercostal liver herniation and thoracic myelomeningocele.

Poland syndrome is characterized by hypoplastic unilateral chest wall structures. These chest wall deformities may be associated with upper extremity anomalies. The association of Poland syndrome with either intercostal liver herniation or a spinocerebral deformity has been described, but there is no report of both findings encountered simultaneously. This is the first report of a newborn child with Poland syndrome associated with an intercostal liver segment herniation and thoracic myelomeningocele with features of an Arnold-Chiari II cerebral malformation.

Cerebral function monitoring: a new scoring system for the evaluation of brain maturation in neonates.

OBJECTIVE: Cerebral function monitoring (CFM), using compressed single-channel amplitude-integrated electroencephalogram recorded from 2 biparietal electrodes, has been shown previously to be a simple bedside tool for monitoring neonatal central nervous system (CNS) status. As the pattern of the CFM changes with gestational age, the technique can be used to assess brain maturation in premature infants. We have developed a new scoring system for the interpretation of neonatal CFM recordings. The objective of this study was to evaluate CFM tracings at increasing gestational and postnatal ages to develop a scoring system to quantify CFM pattern changes. METHODS: Term and preterm neonates were studied with CFM at 12 to 24 hours of life, 48 to 72 hours of life, and then weekly or biweekly until hospital discharge. Each study comprised 8 to 24 hours of continuous CFM recording. CFM recordings were evaluated using the scoring system for record continuity, presence of cyclic changes in electrical activity, degree of voltage amplitude depression, and bandwidth. Each variable was scored for each recording. All variables were summed to yield a total score (minimum 0, maximum 13). Total scores were correlated with gestational and postconceptional ages. RESULTS: Thirty infants were studied with gestational ages at birth that ranged from 24 to 39 weeks and birth weights that varied between 450 and 3850 g. A total of 146 CFM tracings were analyzed. With advancing gestational and postconceptional age, scores for each variable as well as total scores progressively increased with CNS maturation. The highest scores were attained at 35 to 36 weeks' postconceptional age, which corresponded to previously reported subjective observations performed by visual description of CFM patterns. Of the 4 component variables that we analyzed, the most sensitive indicators of CNS maturity were 1) the presence of a cycling pattern, 2) the continuity of the record pattern, and 3) the CFM recording bandwidth. CONCLUSIONS: Our proposed scoring system may be a valuable tool to quantify changes during CFM more objectively, reflecting variations in CNS activity in newborn infants and allowing for better statistical comparisons between amplitude-integrated electroencephalogram tracings from different patients as well as from the same patient at different points of time.