Ganglioneuroma presenting with differentiated skeletal metastases. Report of a case.

The authors report the case of a child with retroperitoneal ganglioneuroma and cytodifferentiated skeletal metastases. The primary tumor was surgically resected, and the child is alive and well 2 years later without additional therapy. This rarely documented phenomenon can be explained by spontaneous cytomaturation within both primary and metastatic tumor.

Epidemic and endemic HLA-B and DR associations in mucocutaneous lymph node syndrome.

HLA-A,B and DR antigens were evaluated in 51 patients with mucocutaneous lymph node syndrome (Kawasaki disease) during both endemic and epidemic periods in the Boston area. Although 80% of patients were HLA-B5 (70% HLA-Bw51) during endemic periods, there were no HLA-B5 individuals during the epidemic period. There was, however, an increased incidence of HLA-Bw44 during the Boston epidemic. Such different associations in endemic versus epidemic periods suggest possible different, as yet unknown, inciting agents.

Stretch reflexes of triceps surae in patients with upper motor neuron syndromes.

Electromyographic responses of triceps surae to dorsiflexion stretch were studied in 47 patients with a variety of lesions producing an upper motor neuron syndrome. The short latency spinal reflexes, both when the patient was at rest and when he was exerting a voluntary plantarflexion, were frequently enhanced in magnitude and the rate of increase with acceleration was also enhanced. Long-latency reflexes were uncommon at rest. With background force long-latency reflexes were present unless the short latency reflex was very large. Long latency reflexes often were normal, but in some patients they were either excessively larger or even of abnormal shape with prolonged continuous activity. The clinical assessment of the ankle jerk correlated with the magnitude of the short latency reflex. The clinical assessment of tone correlated with the magnitude of the short latency reflex, the magnitude of the long latency reflex and the duration of the long latency reflex. There appear to be multiple physiological mechanisms underlying the clinical phenomenon of spasticity.

Stretch reflexes of triceps surae in normal man.

In order to learn more about stretch reflex behaviour of triceps surae, normal human subjects sat in a chair with one foot on a platform attached to a torque motor that produced phasic dorsiflexion displacements on the ankle. EMG activity was recorded from triceps surae and responses were obtained for various conditions. When the subjects's foot was relaxed, stretch of triceps surae produced a single EMG component at short-latency which increased in magnitude with increasing velocity of stretch. The response was not altered if the subject was asked to plantarflex or dorsiflex the ankle voluntarily when he felt the perturbation. It was reduced by vibration of the Achilles tendon. If the triceps surae was stretched while the subject plantarflexed his ankle, the short-latency response was followed by one and sometimes two long-latency responses. Like the short-latency reflex when the foot was relaxed, none of these responses was altered by the subject's planned movement after feeling the perturbation. All of the responses were suppressed to a similar degree by vibration. The long-latency reflexes depended on long-duration of stretching and relatively slow acceleration of stretch. The reflexes persisted after anaesthesia to the foot suggesting that muscle afferents were responsible. Interactions between H-reflexes and stretch-reflexes revealed that the afferent volley producing a stretch reflex acted like the afferent volley producing a small H-reflex. Responses at an interval of 30 ms to both an electrical stimulus for an H-reflex and a stretch stimulus were possible if the electrical stimulus produced only a small H-reflex and if the subject had been plantarflexing the ankle. The short-latency reflex when the foot was relaxed or exerting a background force appears to be the monosynaptic, Ia mediated stretch reflex. The physiological properties of the long latency reflexes are similar to those of the short-latency reflex, and they may represent, at least to a certain extent, response of the motor neuron pool to successive Ia bursts.

HLA antigens in mucocutaneous lymph node syndrome in New England.

HLA antigens were evaluated in 27 patients with mucocutaneous lymph node syndrome (Kawasaki disease) in the Boston area. In contrast to previous Japanese studies, no increase of HLA-Bw22 was found. A significant increase (P less than .002), however, in HLA-Bw51 was found in the patients with mucocutaneous lymph node syndrome as compared to an appropriate control population. To our knowledge, this is only the second disease associated with HLA-B5 specificity. The observations illustrate the lack of unified genetic predisposition for a such disease in two ethnic groups, white and Japanese. Such findings may have genetic implications regarding interhuman variation in immune responsiveness.

Poisoning in children under age 5: identification and treatment.

A rational approach to management of poisoning in young children requires assessment of what, when, and how much drug was ingested. Emesis, if indicated, is followed by general supportive measures, enhanced removal of absorbed poison, and antidote therapy.

Neonatal meningitis. The ventricle as a bacterial reservoir.

We reviewed autopsies of neonates who had died of meningitis. Plexitis and ventricular exudate were usually present. While the pathogenetic relationship between these two phenomena is uncertain, it is possible that the glycogen-rich choroid plexus of the lateral ventricles not only facilitates local bacterial growth but also acts as a bacterial reservoir relatively resistant to antimicrobial agents. We suggest that more drastic approaches than parenteral antimicrobial therapy may be indicated to reduce further the morbidity and mortality of neonatal meningitis.

Measuring the quality of medical care. A clinical method.

We outline the implementation of a new method of measuring the quality of medical care that counts cases of unnecessary disease and disability and unnecessary untimely deaths. First of all, conditions are listed in which the occurrence of a single case of disease or disability or a single untimely death would justify asking, "Why did it happen?" Secondly, we have selected conditions in which critical increases in rates of disease, disability, or untimely death could serve as indexes of the quality of care. Finally, broad categories of illness are noted in which redefinition and intensive study might reveal characteristics that could serve as indexes of health. We describe how these inth of the general population and the effects of economic, political, and other environmental factors upon it, and to evaluate the quality of medical care provided both within and without the hospital to maintain health and to prevent and treat disease.

Myelofibrosis-myeloid metaplasia in childhood.

A child with idiopathic myeloid metaplasia is described and her course contrasted with another child having myeloid metaplasia secondary to reticulum cell sarcoma. Despite prior reports the idiopathic form of illness may pursue a relatively indolent course in children. A thorough search should be made for underlying disease when myeloid metaplasia is first diagnosed.