Upper cervical ossicles in Down syndrome

Six patients with upper cervical spine ossicles and Down syndrome from a study population of 184 are described. The average age was 20.6 years at the time of diagnosis. Three of the patients were involved in tumbling. Radiographic anatomy of the ossicles and the dens, as well as serial radiographs in three of the six patients, suggest that this represents an avulsion of the upper end of the dens rather than an occipital vertebrae, ossiculum terminale, or os odontoideum, as previously described. The authors advocate restriction of high-risk activities for all patients with Down syndrome.     

ENDOTHELIN RECEPTORS IN RAT ADRENAL GLAND VISUALIZED BY QUANTITATIVE AUTORADIOGRAPHY

1. The radioligand [125I]-endothelin was used to map receptors for endothelin in rat adrenal gland using in vitro autoradiography and computerized densitometry. 2. In the adrenal, a high density of binding was found in the adrenal medulla (binding affinity constant 0.18 +/- 0.11 X 10(9)M-1) and zona glomerulosa (binding affinity constant 0.18 +/- 0.07 X 10(9)M-1). Binding was low to undetectable in the zona fasciculata and zona reticularis. Unrelated peptides did not displace endothelin. 3. These results provide evidence of endothelin receptor distribution in adrenal gland and suggest that endothelin might exert multiple actions in the adrenal gland on catecholamine and aldosterone biosynthesis and secretion.     

Risks of neuroleptic drugs in children

We surveyed members of the Child Neurology Society to examine their attitudes about use of neuroleptic drugs in children and their experiences with neuroleptic-induced movement disorders. Sixty percent of the membership responded to our questionnaire. Of these responders, over 99% agreed that there were clinical indications for neuroleptics in children, but their criteria varied. Only a minority of the responders routinely incorporated drug holidays in the therapeutic plans of children treated with neuroleptics. The majority had not had clinical experience with patients who developed chronic movement disorders associated with neuroleptic treatment, but 35% (140/410) had encountered patients whose symptoms they considered typical of tardive dyskinesia. However, some of these patients did not meet strict criteria for diagnosis of this movement disorder. The results suggest that neuroleptic-induced movement disorders occur with significant frequency in children and that more detailed prospective studies of the risks and benefits of these drugs in children are warranted.     

Consequences of misfilling contemporary vaporizers with desflurane

Desflurane is a volatile anaesthetic that combines low blood gas solubility (blood/gas partition coefficient =0.42 at 37° C), moderate potency (MAC = 6–7%), and high volatility (vapour pressure =681 mmHg at 20° C, boiling point = 23.5° C). The volatility and potency of desflurane prevent its safe use in vaporizers of traditional design. We present a mathematical model which demonstrates the potential for desflurane overdose if contemporary vaporizers are misfilled with desflurane. The most hazardous filling error occurs if an enflurane vaporizer is misfilled with desflurane. The calculated desflurane output of a misfilled enflurane vaporizer at a dial setting of 1% and a temperature of 22° C is 57.8%, or 9.6 MAC. For misfilled enflurane, isoflurane, and halothane vaporizers at dial settings equivalent to one MAC at 22° C, the calculated desflurane output is 14.0, 10.2, and 7.8 MAC, respectively. We conclude that the safe delivery of desflurane will require engineering safeguards, additional monitoring, and education of the anesthesia community.     

Three-dimensional structure of a membrane-containing virus.

The structure of Sindbis virus was determined by electron cryomicroscopy. The virion contains two icosahedral shells of viral-encoded proteins separated by a membrane bilayer of cellular origin. The three-dimensional structure of the ice-embedded intact Sindbis virus, reconstructed from electron images, unambiguously shows that proteins in both shells are arranged with the same icosahedral lattice of triangulation number T = 4. These studies also provide structural evidence of contact between the glycoprotein and the nucleocapsid protein across the membrane bilayer. The structural organization of Sindbis virus has profound implications for the morphogenesis of the alphaviruses. The observed interactions confirm stoichiometric and specific protein associations that may be crucial for virion stability and predict a mechanism for assembly.     

Parental 'expressed emotion' as a predictor of schizophrenic relapse

Fifty-seven schizophrenic patients were initially assessed after admission to hospital, at which time their parents completed a Camberwell Family Interview, generating "expressed emotion" (EE) scores. Relapse over the next nine months was not predicted by household EE status or by individual EE scales. Multivariate analyses suggested that a poor course after hospitalization was best predicted by a poor course before admission and by living in a one-parent household. High household EE status was a predictor only in one multivariate analysis after course of illness and one-parent household status had been entered, suggesting an interaction effect. We query the causal proposition linking high EE and relapse and suggest instead that a poor illness course may elicit high EE in relatives, particularly in one-parent households, and, thus, may make the principal contribution to the proposed link.     

T lymphocytes and activated eosinophils in airway mucosa in fatal asthma and cystic fibrosis.

With the use of monoclonal antibodies (mAb) and immunohistology, the numbers of phenotypically distinct cells infiltrating lung tissue from 15 postmortem (PM) cases of fatal asthma were quantified and compared with 6 cases of cystic fibrosis (CF) (three postmortem, three transplant) and 10 nonasthmatic cases of sudden death matched for age and sex. Tissue eosinophilia was significantly greater (p less than 0.001) in the fatal asthma group than in the CF or sudden death controls. In asthma, approximately 40% of the eosinophilic infiltrate was EG2 positive (an indication of eosinophil activation and secretion of eosinophil cationic protein). The numbers of eosinophils and EG2 positive cells were significantly elevated in the subjects with acute severe asthma who had had a duration of terminal illness exceeding, as compared with less than, 24 h (p less than 0.05). When compared with the sudden death controls, there were increases in the numbers of Dako L C positive cells (i.e., CD45 positive "total leukocytes") in both fatal asthma and CF (p less than 0.01 and 0.05, respectively). The mean number of MT-1 positive (T) cells in the asthma and CF groups was approximately twice that of the control (p less than 0.05 and 0.01, respectively). The mean number of MB2 positive (B) cells was similar for both the asthma and sudden death control groups but was significantly increased in CF (p less than 0.05). The average T:B cell ratios were 6:1, 1:1, and 2:1 in the fatal asthma, CF, and control groups, respectively. The results support a role for the T lymphocyte in the pathogenesis of fatal asthma and CF.(ABSTRACT TRUNCATED AT 250 WORDS)     

Normal perfusion pressure breakthrough complicating surgery for the vein of Galen malformation: report of three cases

Three cases are described of infants who developed malignant brain swelling (and in one case hemorrhage) after surgery for vein of Galen malformations. The cause for the brain swelling was felt to be due to hyperperfusion, or the "normal perfusion pressure breakthrough" syndrome. Although well-described for cerebral parenchymal arteriovenous malformations, cases of this complication occurring in vein of Galen malformations have not previously been reported. It is concluded from these cases that infants with large arteriovenous shunts, as attested by cardiac failure and cerebral atrophy, have an increased risk of developing this complication.