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Biomedical subjects

K N Shaw

Publications and source records attributed to K N Shaw.

15 recordsLinked to original sources

Outpatient assessment of infants with bronchiolitis.

Two hundred thirteen infants younger than 13 months with bronchiolitis were prospectively followed up to identify the historical, physical, and laboratory clues at initial emergency department evaluation that would help to predict disease severity. Based on their total course of illness, the patients were classified as having mild (139 patients) or severe (74 patients) disease, and the initial emergency department evaluation findings of these two groups were compared. Six independent clinical and laboratory findings were identified that were strongly associated with more severe illness: (1) "ill" or "toxic" general appearance; (2) oxygen saturation less than 95%, as determined by pulse oximetry; (3) gestational age, younger than 34 weeks; (4) respiratory rate, 70/min or greater; (5) atelectasis on a chest roentgenogram; and (6) age, younger than 3 months. The infant's oxygen saturation as determined by pulse oximetry was the single best objective predictor of more severe disease.

Age Factors

Indigent children who are denied care in the emergency department.

We conducted a six-month prospective study of the diagnoses and outcomes of 588 children who were denied care in our emergency department under a new primary-care case management health system for 100,000 indigent patients. The mean patient age was 4.7 years (39% were less than 2 years old). The most common presenting complaints were colds, earaches, rash, vomiting, and diarrhea. Nine percent of children presented for trauma, and 10% had fever of more than 38.2 C. Follow-up was available from the primary care physician for 388 children (66%). Of the 60% of patients who kept their arranged appointment, 42% received antibiotics, 3% were referred for further evaluation, and two children were hospitalized. Follow-up was available from the parents for 125 children (21%). No follow-up information of any kind was available for 111 children (19%), and no follow-up regarding the health of the child was available for 265 children (45%). This last group included 10% with a chief complaint of trauma and 6% with temperature of more than 39 C. Forty-nine percent of patients in this group were less than 2 years old.

Appointments and Schedules

Submersion injuries: drowning and near-drowning.

Submersion injuries are a major cause of morbidity and mortality in children and young adults. The Emergency Department physician should be familiar with the epidemiology, pathophysiology, modes of therapy, and prognostic indicators in order to provide optimum resuscitation to the near-drowning victim, correct referral, and counseling to their families.

Emergencies

Hyperlysinemia with saccharopinuria due to combined lysine-ketoglutarate reductase and saccharopine dehydrogenase deficiencies presenting as cystinuria.

A 7-year-old boy with speech delay, hyperactive behavior, and minor neurologic abnormalities had been found in the past to have "intermittent cystinuria." A more detailed investigation revealed hyperlysinemia and hyperlysinuria, with lesser increases in urinary excretion of arginine and cystine. The plasma and urine abnormalities increased on a diet of 3 gm of protein/kg body weight/day. Saccharopine, a normal metabolite of lysine not found in the body fluids of normal people, was present in plasma, cerebrospinal fluid, and urine of the patient. Lysine-ketoglutarate reductase and saccharopine dehydrogenase activities were not detectable in extracts of cultured skin fibroblasts. Re-examination of the urine of previously studied cases of this double enzyme deficiency suggests that saccharopinuria of variable degree is the rule and not the exception.

Cells, Cultured

Ultrastructural studies on neuroblastoma: evaluation of cytodifferentiation and correlation of morphology and biochemical and survival data.

Fifteen cases of neuroblastoma, ganglioneuroblastoma and ganglioneuroma were studied by electron microscopy. Ultrastructural features of cytodifferentiation, including numbers of dense core neurosecretory granules (NSG) and neuritic processes, were used to evaluate variation within the neuroblastoma group to determine whether differences in cytodifferentiation exist where light microscopic variations are not evident. These studies revealed that undifferentiated neuroblastomas do show ultrastructural variations not evident by light microscopy. The ultrastructural findings for each case were compared with initial urinary catecholamine excretory patterns, the latter having recently been shown to have valuable prognostic significance. There was a positive correlation, in the undifferentiated neuroblastomas, between increased numbers of NSG and prognostically favorable biochemical excretory patterns. Conversely, low numbers of NSG were associated with an unfavorable biochemical pattern and fatal clinical course. These correlations between ultrastructural differentiation and the biochemical secretory pattern indicate that ultrastructural evaluation of undifferentiated neuroblastomas would appear to have prognostic value, particularly in cases lacking initial biochemical data or as an adjunct to biochemical studies.

Abdominal Neoplasms

Initial urinary catecholamine metabolite concentrations and prognosis in neuroblastoma.

Initial urinary catecholamine metabolite and amino acid excretion patterns were examined in 54 children with neuroblastoma. The relationships between prognosis and age at diagnosis, stage of disease, primary site, and histologic grade of tumor were similar in this population to those found in previous studies, but only age and stage were found to be independent prognostic variables. Prognosis in disseminated disease was found to correlate directly with the urinary vanilmandelic acid (VMA)/homovanillic acid (HVA) ratio but not with the absolute levels of HVA. The presence of the dopa metabolite, vanillactic acid, as well as increased amounts of cystathionine and/or low levels of VMA indicated poor prognosis. These results are consistent with the hypothesis that biochemically primitive neuroblastomas deficient in dopamine beta-hydroxylase are move virulent than their mature analogues which produce epinephrine, norepinephrine, and their metabolites.

Adrenal Gland Neoplasms

Hyperargininemia.

A 7 1/2-year-old boy had progressive psychomotor retardation, behavior disturbance, and spasticity, and had growth arrest from age three. Plasma arginine on a self-selected protein-poor diet was increased (4.05 mg/dl; nl 0.4 to 2.6), whereas urinary amino acid excretion was normal. Red blood cell arginase was less than 1% of normal in the patient and was half normal in both parents, in two normal siblings, and in his paternal grandfather. Three hours after a meal providing 2 gm protein/kg body weight, the plasma arginine value rose to 13.2 mg/dl, dibasic aminoaciduria was seen clearly for the only time, but blood ammonia concentration remained normal. We conclude that arginase deficiency in the red blood cells and probably in the liver is inherited in an autosomal recessive manner and is responsible for the clinical syndrome in this patient.

Amino Acid Metabolism, Inborn Errors

Teaching technical skills in pediatrics.

Technical skills are an important and difficult part of managing a very sick or injured child. However, technical procedures are more often learned from experience than practice. This article reviews a variety of technical skills and procedures commonly performed in a pediatric emergency department. Each technical procedure can be practiced using live or artificial model for simulation.

Clinical Competence