Multiple pilocytic astrocytomas of the cerebellum in a 17-year-old patient with neurofibromatosis type I

Objective Approximately 10% of patients with neurofibromatosis I (NFI) patients will have central nervous system (CNS) tumors. The most common of these are hypothalamic–optic gliomas, followed by brainstem and cerebellar pilocytic astrocytomas. While isolated pilocytic astrocytomas in NFI are well described, the appearance of multiple pilocytic astrocytomas in an individual patient is less common. The most frequent combination in NFI patients with more than one pilocytic astrocytoma is optic tract/hypothalamic and brainstem. Other combinations are exceedingly rare; multiple pilocytic astrocytomas have only been reported once in the cerebral hemispheres in a patient with NFI. This report presents the first documented case, to our knowledge, of multiple pilocytic astrocytomas in the cerebellum of a patient with NF1. Methods Case report. Conclusion The finding of multiple cerebellar pilocytic astrocytomas in a patient with NF1 is important because it expands the spectrum of presentations for patients with NF1 and also highlights specific diagnostic and therapeutic challenges faced by the treating physicians. The genetic and molecular basis of NF1 is reviewed. Strategies of diagnosis and treatment outlined here are relevant to both patients with NF1 and all patients with multiple posterior fossa tumors.     

A Prospective Randomized Trial of Steroid-Free Maintenance Regimens in Kidney Transplant Recipients—An Interim Analysis

We compared three maintenance immunosuppressive regimens in a rapid discontinuation of prednisone protocol. From March 1, 2001, through December 31, 2003, 239 first and second kidney transplant recipients (166 LD; 73 DD) were randomized. All recipients were treated with Thymoglobulin; all received steroids intraoperatively and for 5 days postoperatively. Randomization was to cyclosporine-mycophenolate mofetil (n = 85); high-level tacrolimus (TAC) (8-12 ng/mL)-low-level sirolimus (SRL) (3-7 ng/mL) (n = 72); or low-level TAC (3-7 ng/mL)-high-level SRL (8-12 ng/mL) (n = 82). We found no difference at 24 months between groups in patient, graft, death-censored graft, or acute rejection-free graft survival, or in kidney function. Wound complications were more common in SRL-treated recipients (p = 0.02); we found no other differences between groups in complication rates. Our data suggest that excellent patient and graft survival and low rejection rates can be obtained using a variety of maintenance protocols without prednisone.     

Powerlessness Regarding Health-Service Barriers: Construction of an Instrument

PURPOSE. The purpose of the study was to develop a scale to calculate college students' powerlessness regarding health service barriers (PHSB). METHODS. Scale items were generated to represent two domains of interest: powerlessness and college students' powerlessness regarding health service barriers. The final 20-item instrument was subjected to multiple measures of validity with college students and panels of nurse experts in the area of nursing diagnosis. Reliability was assessed by two samples (n = 92 and n = 33) of college-age students. A final administration was conducted with 197 college students. FINDINGS. The PHSB scale was determined to be an accurate and consistent measure. CONCLUSIONS. This instrument will provide a reliable and valid measure to conduct research.     

Histidine, histamine, and the neuroregulation of food intake: a review and hypothesis

Feeding, a behavior regulated by the central nervous system (CNS), includes the acquisition of specific essential nutrients and the maintenance of energy balance. Modulation of feeding behavior is a normal part of survival, but certain pathological conditions interrupt or modify regulatory aspects of feeding, thereby leading to inappropriate intake. This review examines aspects of metabolism associated with the anorexia seen in animals suffering from protein-energy malnutrition (PEM). The main focus is the indispensable amino acid histidine (His), the biosynthetic precursor of the neurotransmitter histamine (HA). In kwashiorkor-like PEM, His is elevated in plasma and brain, whereas all other indispensable amino acids are decreased. The elevation of His in the brain is to concentrations five times normal. Because the rate of HA synthesis in the brain is a function of the His concentration, His elevation raises the possibility of a profound direct effect of CNS function. In children, PEM consistently produces the symptoms of depressed food intake, edema, growth failure, and psychomotor changes. One known central effect of HA is the stimulation of ACTH and corticosteroid release. Based on these observations, the hypothesis being examined is as follows: one component of the pathophysiological neuroregulation of food intake involved the His-induced variation of HA concentration in the hypothalamus and the subsequently altered neurochemical activity at the corticotropin-releasing factor (CRF) neurons o the paraventricular nucleus (PVN).     

Theophylline and status epilepticus in children

We studied the role of theophylline on outcome of status epilepticus (SE) in children. During a two-year-period, 16 of 114 episodes of SE occurred in children receiving theophylline. At the onset of SE, theophylline blood levels were elevated in 8 episodes, and were therapeutic or subtherapeutic in 8 episodes. In the 8 episodes of SE with elevated theophylline levels, one child died and three suffered permanent new neurologic deficits. In the 8 episodes of SE with normal or low theophylline levels, only one child had a transient deficit. The occurrence of death or disability in 4 of 8 episodes of SE with elevated theophylline was considerably higher than the 23% incidence of death or persistent CNS deficit in the overall series of 114 episodes of SE. We conclude that theophylline, at toxic levels, is a significant factor in increased morbidity. We suspect that the hypoxia from the respiratory disorder for which theophylline was used, and the reduced cerebral blood flow known to occur with theophylline led to a failure to compensate for the increased cerebral metabolic rate of SE, thus increasing the risk of a poor outcome.