Recent developments in pulse oximetry.
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Biomedical subjects
Publications and source records attributed to J F Kelleher.
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We present a case of a child with iron-deficiency anemia, folic acid deficiency, and scurvy. His anemia proved refractory to treatment with iron until he received both folic acid and vitamin C supplementation. This case illustrates the importance of the evaluation of ascorbic acid and folate status in treating iron-deficiency anemia initially refractory to iron supplementation, because multiple nutrient deficiencies may coexist.
Previous studies have indicated a high prevalence of nonanemic iron deficiency in female high school aged endurance athletes. It is not clear, however, whether these adolescents are at more risk for iron depletion than their nonathletic peers. We have previously reported declining serum ferritin levels in response to running but not swimming training in competitive adolescents. In this study we compared these findings with serum ferritin levels and hematologic parameters in a group of nonathletic females from the same community. Mean serum ferritin levels were not significantly different among the groups. A greater percentage of the swimmers and runners had ferritin levels less than 12 ng/ml at the beginning of the season (46.7 and 40%, respectively, compared to 26.7% in the nonathletes), but the differences were not statistically significant. These findings suggest that the high prevalence of hypoferritinemia at the beginning of a competitive season in female high school athletes is similar to that of nonathletes. Other studies have indicated, however, that some sports, particularly running, increase the incidence of iron depletion with training.
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A 9-month-old boy with known familial neurofibromatosis type I (NF-1) presented with a clinical and laboratory picture suggestive of juvenile chronic myelomonocytic leukemia (JCMMoL). Chromosomal studies obtained from the bone marrow indicated, however, that he had monosomy 7 syndrome. We believe this is the first reported case of monosomy 7 syndrome in a child with NF in the United States, and that this case complements a recent report of two cases of NF, JCMMoL, and monosomy 7 in Japanese children. Since monosomy 7 syndrome is very difficult to differentiate from JCMMoL or acute nonlymphocytic leukemia (ANLL) unless appropriate chromosomal studies are obtained, we believe it is possible that monosomy 7 may occur with increased frequency in patients with NF-1. Monosomy 7 syndrome might therefore be a significant cause of the known association between NF-1 and nonlymphoid leukemia.
In physical systems, a 'critical phenomenon' is a macroscopic occurrence which arises from a change in the relative magnitudes of two or more physical influences whose action is expressed purely in microscopic terms. This concept is potentially valuable in describing the clinical symptomatology of dystonia musculorum deformans (DMD) and other movement disorders. The lateral inhibitory network of Spiny I caudate cells and the profuse neostriatal projection of dopaminergic neurons from the substantia nigra are proposed as anatomic substrates for two short-range neostriatal influences whose interaction contributes to the onset and progression of global dystonic spasm in DMD. Such spasm may be explainable as a critical phenomenon, suggesting new directions for research into the etiology and treatment of this disorder.
Histiocytosis X of the female genital tract is unusual. Thirty-two cases have been reported to date in the world literature. An additional case is reported herein, presenting as a vulvar ulcer in a 2.5-year-old child with osteolytic lesions of the skull, splenomegaly, and otitis media. The diagnosis of histiocytosis X may be established by identifying the Langerhans histiocyte, characterized by nuclear grooves, immunoreactivity for S-100 protein, and pentalamellar cytoplasmic structures seen by electron microscopy. Prognosis is difficult to determine with certainty. However, age of less than 2 years at presentation, multi-organ involvement, and/or organ dysfunction appear to be associated with a less favorable prognosis. The patient presented herein is currently receiving vinblastine chemotherapy for recurrence of disease, manifested as an osteolytic lesion in the skull.
Previous studies have indicated a high incidence of nonanemic iron deficiency in high school runners, but little is known regarding participants in other sports. The hematologic and iron status of 30 high school swimmers was assessed at the beginning and end of a competitive swim season. Iron depletion (serum ferritin level less than 12 micrograms/L) was initially present in 46.7% of the girls and none of the boys, yet among those subjects with hypoferritinemia there was no evidence of impaired red blood cell production. Serum haptoglobin levels were normal and did not correlate with serum ferritin levels. No significant change was observed in ferritin, hemoglobin, or haptoglobin concentrations during the course of the season. Dietary intake of iron was poor, particularly in the girls, in whom it averaged 43% of the recommended dietary allowance. Menstrual histories suggested an inverse relationship between the amount of menstrual flow and the serum ferritin level. These findings indicate a high prevalence of nonanemic iron deficiency in female high school swimmers that bears no relationship to swim training. Dietary inadequacies and menstrual blood flow appear to be important contributors to the hypoferritinemia observed in these athletes.
The pulse oximeter, a widely used noninvasive monitor of arterial oxygen saturation, has numerous applications in anesthesiology and critical care. Although pulse oximetry is considered sufficiently accurate for many clinical purposes, there are significant limitations on the accuracy and availability of pulse oximetry data. This article reviews both the clinical uses of the pulse oximeter and the limitations on its performance. The pulse oximeter is generally acknowledged to be one of the most important advances in the history of clinical monitoring.
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Iron-deficiency anemia impairs exercise capacity, but whether nonanemic iron depletion decreases endurance performance is unclear. In 14 iron-deficient (serum ferritin level, less than 20 micrograms/L [less than 20 ng/L])nonanemic runners, hematologic and treadmill running values were followed up during a competitive season. Following a four-week control period, runners were treated for one month in a double-blind protocol with ferrous sulfate (975 mg/d) or placebo. During treatment, the mean ferritin level rose from 8.7 to 26.6 micrograms/L (8.7 to 26.6 ng/mL) in those patients taking iron and fell from 10.6 to 8.6 micrograms/L (10.7 to 8.6 ng/mL) in the placebo group. Treadmill endurance times improved significantly in the iron-treated runners compared with controls. Endurance time declined in all seven controls (range, 0.07 to 1.30 minutes), while six of seven iron-treated subjects improved their performance (range, 0.03 to 1.92 minutes). No significant differences in maximal or submaximal oxygen consumption, ventilation, or heart rate were observed between the groups except for a 4% increase in maximum oxygen consumption during placebo treatment. These data indicate that nonanemic iron deficiency impairs exercise performance but does not influence gas exchange or cardiac measures.
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Iron deficiency with or without anemia may impair athletic performance. Although previous reports suggest a high incidence of iron deficiency in adolescent athletes, the recommendations for routine screening are unclear. In this study, high school male and female cross-country runners were evaluated by determining serum ferritin, hemoglobin, and red blood cell indexes during an 11-week competitive season. At the beginning of the season one of 30 males and eight of 20 females had iron deficiency, defined as a ferritin level greater than or equal to 12 ng/ml. By the end of the season four additional males and another female became iron deficient, for an overall incidence of 17% in males and 45% in females. Of the 26 runners who were evaluated throughout the season, ferritin levels fell in all nine females and in 14 of 17 males. Iron-deficiency anemia was not observed in any subject. These findings suggest that nonanemic iron deficiency is common in adolescent runners. Although iron deficiency is more common in females, one of every five males was iron depleted by the close of the season. Preseason screening alone is shown to be inadequate for detecting iron-deficient athletes as five runners with low ferritin levels were not identified on the initial evaluation.
The risk of significant bleeding in patients with severe Factor VII (FVII) deficiency is well documented. However, controversy regarding management exists not only for the choice of FVII replacement product, but also for the desired hemostatic level of FVII. Two case reports are presented where fresh frozen plasma (FFP) and several Factor IX (FIX) complex concentrates were used to achieve hemostasis. Significantly higher FVII levels were achieved with Proplex (FIX Complex, Hyland Therapeutics) than with other products. Although this and other reports indicate that minor bleeding can be controlled with levels of FVII under 15%, life-threatening hemorrhages require higher FVII levels that can be attained only by using a product with a significant FVII content. It is important to know the FVII content of FIX concentrates in order to plan appropriate therapy for patients with FVII deficiency.
Hb Santa Ana (beta 88 Leu----Pro) was found at a level of 30% of total hemoglobin in red cells from a patient with congenital hemolytic anemia. Since no detailed functional studies of this hemoglobin have been done previously, we determined oxygen equilibrium curves of red cell suspensions, hemolysates, and isolated Hb Santa Ana. The P50 values obtained were 22.5 mm Hg (37 degrees C, pH 7.4) for red cell suspensions (normal mean = 27.0 mm Hg), 10.0 mm Hg (25 degrees C, pH 7.0) for hemolysate (normal = 13.5 mm Hg), and 1.3 mm Hg (25 degrees C, pH 7.0) for isolated Hb Santa Ana. Measurements of mechanical stability of Hb Santa Ana showed that the oxy-form of this hemoglobin was 10 times more unstable than that of Hb S and 100 times more unstable than that of Hb A. The deoxy-form of Hb Santa Ana was as unstable as its oxy-form, indicating that no stabilization (R----T conversion) takes place upon the deoxygenation of Hb Santa Ana.
An infectious agent has long been suspected as the cause of aplastic crisis in sickle cell disease, but no specific organism has been implicated. We studied six children with sickle cell disease in whom an aplastic crisis developed, looking for evidence of parvoviruslike virus (SPLV) infection using a new radioimmunoassay. All six patients had conclusive evidence of acute infection with SPLV. We postulated that SPLV is the predominant cause of aplastic crises in patients with hemolytic anemia.
Vigorous exercise is known to increase VIII:C and VIIIR:Ag levels transiently in normal individuals. Although exercise programs are frequently advocated in the management of hemophilia, the effects of exercise on coagulation parameters in these patients have not been well studied. Eleven hemophiliacs were exercised on a bicycle ergometer to maximum voluntary effort as evidenced by an increase in pulse, blood pressure, and plasma catecholamine (norepinephrine and epinephrine) levels. The effects of this exercise on coagulation parameters, including functional and antigenic components of the factor VIII molecule, were determined. The entire group demonstrated a decrease in mean prothrombin time (11.7 to 11.2 sec). Four mild hemophiliacs demonstrated an increase in mean VIII:C (14.5% to 17.3%), and VIII:CAg (12% to 17.8%). Changes in VIII:C and VIII:CAg were not noted in the seven severe hemophiliacs. Both severe and mild patients demonstrated significant changes in fibrinogen, factor II, and factor VII after exercise. This study indicates that submaximal exercise modifies coagulation parameters in patients with hemophilia.