Pediatric patients with achondroplasia: CT evaluation of the craniocervical junction

Twenty-six patients (4 months to 6 years old) with achondroplasia complicated by sleep apnea and/or other neurologic manifestations underwent plain computed tomography (CT) of the craniocervical junction; six also underwent CT myelography. For objectification, multiplanar reconstruction was used to complement axial plane measurements by providing coronal and sagittal measurements; multiplanar reconstruction also improved perception of the longitudinal relationships between the brain stem and subarachnoid space. A narrow subarachnoid space was found in all 26 patients; marked cord compression was present in nine, six of whom underwent CT myelography. These six had marked focal obliteration of the subarachnoid space on both plain CT and CT myelography. Since the subarachnoid space immediately above and below the craniocervical junction is normally capacious, when marked constriction was present, no additional information could have been gained from CT myelography. Thus, plain CT was shown to be sufficient for surgical planning (suboccipital decompression) in nine patients with cord compression due to achondroplasia.     

Significant others benefit from preoperative information

Little attention has been given to the preparation of the patient's family prior to surgery, even though nurses' clinical experience suggests that family members are often more anxious than the patient. This study explored the knowledge and anxiety of spouses and significant others of patients preparing for cardiac surgery. The subjects were selected by convenience from a preoperative class offered at the hospital where surgery was to occur. Before and after the class, the subjects completed a cardiac-surgery knowledge test and an anxiety test. The significant others were significantly more anxious than the patients prior to the class. The anxiety level of significant others was significantly reduced after the class. There were no statistically significant differences between patients and significant others on the cardiac-surgery knowledge test. These results suggest that significant others may benefit from preoperative instruction.     

Attention-Deficit/Hyperactivity Disorder: A Neurodevelopmental Approach

TOPIC. Attention-deficitlhyperactivity disorder is frequently overdiagnosed when a complete evaluation by a knowledgeable clinician is not undertaken. Because of the recent negative media campaign regarding psychos t im ulan t medication, it becomes more imperative that a thorough and accurate evaluation be completed before starting children and adolescents on psychostimulant medications.
PURPOSE. To describe the neurodevelopmental perspective in the assessment and treatment of ADHD.
SOURCES. Published literature and clinical experience.
CONCLUSION. It is useful in practice to conceptualize the dmelopmental path of children with ADHD not as disordered but as delayed, and to build on each child's strengths. The neurodmeloprnental approach provides the child psychiatric nurse a child-focused framework for assessment and development of individualized interventions.     

Development of an assay for in vivo human neutrophil elastase activity. Increased elastase activity in patients with alpha 1-proteinase inhibitor deficiency.

Leukocyte extracts contain enzymes that digest fibrinogen and release a fibrinopeptide A-containing fragment. This study was undertaken to identify the responsible proteinase and to characterize the fibrinopeptide A-containing fragment so that it could be used as an index of enzyme activity. Both the fibrinogenolytic activity and the release of the fibrinopeptide A-containing fragment mediated by the leukocyte extracts were shown to be due to human neutrophil elastase (HNE) by the following criteria: activity was completely blocked by a specific HNE inhibitor or by adsorbing HNE from the extracts with a monospecific antibody and reconstitution with purified HNE restored the ability to release the fibrinopeptide A-containing fragment. This fragment was not released by a variety of other proteinases or by HNE-inhibitor complexes indicating that, at least with respect to the enzymes tested, it is a specific product of HNE and its release requires the free enzyme. By separating the products of HNE digestion of fibrinogen using high performance liquid chromatography, identifying the immunoreactive fractions and subjecting them to amino acid analysis, the fragment was identified as A alpha 1-21, indicating an HNE cleavage site at the Val(A alpha 21)-Glu(A alpha 22) bond. The mean plasma A alpha 1-21 level was markedly higher in patients with alpha 1-proteinase inhibitor deficiency as compared to healthy controls (0.2 nM vs. 7.9 nM; P less than 0.0001), consistent with increased in vivo HNE activity in these individuals.