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A K Brown

Publications and source records attributed to A K Brown.

At least 19 recordsLinked to original sources

Pregnancy in sickle cell disease: experience of the Cooperative Study of Sickle Cell Disease.

OBJECTIVE: To determine the maternal and fetal outcomes of pregnancy in women with sickle cell disease. METHODS: The subjects were part of a cohort recruited from 19 centers for a prospective study of the clinical course of sickle cell disease. Each participant was evaluated using a structured protocol in which steady-state data and information on both sickle- and non-sickle-related events were collected. The rates of antepartum and intrapartum complications were tallied for pregnancies carried to delivery. Fetal outcome was assessed according to gestational age, birth weight, and Apgar score. Differences among genotypes in event rates were assessed using Fisher exact test. Differences in gestational age and birth weight, and predictors of these outcomes, were assessed using analyses of covariance. RESULTS: Two hundred eighty-six of the 445 reported pregnancies proceeded to delivery. Non-sickle-related antepartum and intrapartum complication rates were comparable with those of African-American women who did not have sickle cell disease. One of the two deaths observed during this study was directly related to the presence of sickle cell disease. Rates of maternal morbidity from sickle cell disease were the same during pregnancy as during the nonpregnant state. Ninety-nine percent of those pregnancies carried to delivery resulted in a live birth. Twenty-one percent of the infants born to women of the SS genotype were small for gestational age (SGA). Preeclampsia and acute anemic events were identified as risk factors for SGA infants. CONCLUSIONS: Those caring for women with sickle cell disease should support them if they desire to have children.

Adult

Measurement of gap length in esophageal atresia: a simple predictor of outcome.

BACKGROUND: Previous classifications of esophageal atresia (EA) and tracheoesophageal fistula (TEF) have concentrated on associated medical conditions that influence survival. We propose a classification based on gap lengths to define the magnitude of the surgical problems in EA and TEF and correlate them with outcome. STUDY DESIGN: Gaps between the esophageal ends were classified as long (greater than 3 cm), intermediate (greater than 1 cm but less than or equal to 3 cm) or short (less than or equal to 1 cm). A series of 66 consecutive patients with EA and TEF were studied. RESULTS: The outcomes of patients with long (n = 16), intermediate (n = 16), and short (n = 34) gaps were respectively: death (18 percent, 6 percent, 3 percent), anastomotic leak (31 percent, 25 percent, 6 percent), stricture (44 percent, 31 percent, 17 percent), recurrent TEF (6 percent, 6 percent, 6 percent), gastroesophageal reflux (56 percent, 37 percent, 36 percent), and failure to thrive (56 percent, 43 percent, 18 percent). The mean hospital stay at first admission was (97, 54, 24 days, respectively) and the mean number of readmissions was (6.9, 5.2, 3.4, respectively). CONCLUSIONS: This classification, which is based on easily measurable criteria, provides a useful method to predict morbidity, long-term outcome, and health costs associated with EA and TEF surgery.

Anthropometry

Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases.

From 1978 to 1988, The Cooperative Study of Sickle Cell Disease observed 3,765 patients with a mean follow-up of 5.3 +/- 2.0 years. One thousand seventy-nine surgical procedures were conducted on 717 patients (77% sickle cell anemia [SS], 14% sickle hemoglobin C disease [SC], 5.7% S beta zero thalassemia, 3% S beta zero + thalassemia). Sixty-nine percent had a single procedure, 21% had two procedures, and the remaining 11% had more than two procedures during the study follow-up. The most frequent procedure was abdominal surgery for cholecystectomy or splenectomy (24% of all surgical procedures, N = 258). Of these, 93% received blood transfusion, and there was no association between preoperative hemoglobin A level and complication rates (except reduction in pain crisis). Overall mortality within 30 days of a surgical procedure was 1.1% (12 deaths after 1,079 surgical procedures). Three deaths were considered to be related to the surgical procedure and/or anesthesia (0.3%). No deaths were reported in patients younger than 14 years of age. Sickle cell diseases (SCD)-related complications after surgery were more frequent in SS patients who received regional compared with general anesthesia (adjusted for risk level of the surgical procedure, patient age, and preoperative transfusion status, P = .058). Non-SCD-related postoperative complications were higher in both SS and SC patients who received regional compared with those who received general anesthesia (P =.095). Perioperative transfusion was associated with a lower rate of SCD-related postoperative complications for SS patients undergoing low-risk procedures (P = .006, adjusted for age and type of anesthesia), with crude rated of 12.9% without transfusion compared with 4.8% with transfusion. In SC patients, preoperative transfusion was beneficial for all surgical risk levels (P = .009). Thus, surgical procedures can be performed safely in patients with SCD.

Adolescent

Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease.

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

Anemia, Sickle Cell

Glucose-6-phosphate dehydrogenase deficiency and carboxyhemoglobin concentrations associated with bilirubin-related morbidity and death in Nigerian infants.

Our objective was to determine whether glucose-6-phosphate dehydrogenase (G6PD) deficiency and elevated carboxyhemoglobin (COHb) levels correlated with bilirubin-related morbidity and mortality rates. For this purpose, we studied 55 clinically jaundiced infants admitted to a rural mission hospital in southern Nigeria. Total serum bilirubin levels (range, 80 to 1016 mumol/L (4.7 to 59.4 mg/dl)) correlated with the percentage COHb concentrations (COHb = 0.45 + 0.08 Total serum bilirubin; r = 0.72). Infants were divided into two groups of equal size around the median COHb concentration (COHb range, 0.43% to 5.93% (median = 1.40%), with ambient carbon monoxide of 0.65 +/- 0.03 microL/L). The COHb levels > or = 1.40% were associated with the need for exchange transfusion (15/28, or 54%, vs 5/27, or 19%; p < 0.01) and with an increased incidence of clinical findings compatible with kernicterus (9/28, or 32%, vs 0/27, or 0%; p < 0.01). Mortality rate was 29% (8/29) among infants with higher COHb levels, and 7% (2/28) in those with lower levels (p = 0.08). Thirty-one percent (14/45) of the clinically jaundiced infants tested had G6PD deficiency. Thirty-six percent of the infants with G6PD deficiency died with presumed kernicterus, compared with only 3% (1/31) of the infants with a normal G6PD screening test result (p < 0.01). These data suggest that G6PD deficiency and increased bilirubin production, as indexed by COHb, are associated with jaundice-related morbidity and death in Nigerian infants.

Bilirubin

Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease.

OBJECTIVE: To examine hematologic changes from birth to 5 years of age and establish hematologic reference values for infants and children with sickle cell disease. RESEARCH DESIGN: Prospective natural history study. SETTING: Nineteen pediatric sickle cell centers across the United States. PATIENTS: Six hundred ninety-four infants with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and sickle-beta-thalassemia) who were enrolled in the Cooperative Study of Sickle Cell Disease at younger than 6 months of age. Median follow-up time through 5 years of age was 4.1 years. MEASUREMENTS AND RESULTS: We present longitudinal analyses of total hemoglobin concentration, percent fetal hemoglobin values, mean corpuscular volumes, total bilirubin concentration, and red blood cell (RBC), "pocked" RBC, white blood cell, platelet, and reticulocyte counts. Anemia was apparent by 10 weeks of life in infants with sickle cell anemia (SS infants). This anemia was associated with a rising reticulocyte count consistent with a hemolytic process. The reticulocyte count of SS infants increased steadily, exceeding 12% at 5 years of age. The fetal hemoglobin concentration of SS infants declined more slowly than that of infants with sickle cell hemoglobin C disease (SC infants). Pocked RBC counts rose sharply after 6 months of age, and by 1 year, 28% of SS infants had abnormal counts, above 3.5%, indicating poor splenic function. At 3 years of age, 78% of SS patients and 32% of SC patients had abnormal pocked RBC counts. The SS patients with concurrent alpha-thalassemia had, after 6 months of age and throughout early childhood, a slightly higher mean total hemoglobin concentration and lower mean pocked RBC and reticulocyte counts than SS patients without alpha-thalassemia. The hematologic profile of SC infants more closely resembled that of normal black infants, but there was mild anemia (10.5 g/dL) and slightly elevated mean values for reticulocytes (3%) and fetal hemoglobin (3%) during early childhood.

Age Factors

The influence of infant and maternal sickle cell disease on birth outcome and neonatal course.

OBJECTIVE: To compare the influence of maternal hemoglobin phenotype as well as that of the infant on birth outcome and neonatal complications. RESEARCH DESIGN: Prospective, natural history study with retrospective chart review for neonatal complications. SETTING: Nineteen pediatric sickle cell centers across the United States. PATIENTS: Four hundred eighty infants with sickle cell disease (SCD) who were enrolled in the Cooperative Study of Sickle Cell Disease at less than 6 months of age, as well as a comparison cohort of 118 infants with sickle cell trait born to women with sickle cell anemia in the Cooperative Study. RESULTS: In the SCD cohort, overall rates of preterm (< 37 weeks), low-birth-weight (< 2500 g), and small-for-gestational age births were 9%, 10%, and 8%, respectively; no significant differences were found according to infant hemoglobin phenotype. Term births accounted for 59% of the infants with low birth weight, significantly higher than the 41% US rate for black low-birth-weight infants (P = .014). Expectant mothers with sickle cell anemia are 2.5 times more likely to bear newborns who are small for gestational age than are women with other types of sickle cell disease, sickle trait, or C-trait. The most common prepartum and neonatal complications in infants with SCD were jaundice (25%), fetal distress (13%), anemia (10%), and respiratory distress (6%). Complication rates did not differ significantly by hemoglobin phenotype in the infants with SCD, but infants born to women with sickle cell anemia had higher rates of jaundice (P < .0001). CONCLUSIONS: Rates of adverse birth outcomes and neonatal complications in infants with SCD are similar to the rates for normal infants, although preterm birth accounts for fewer of the low-birth-weight outcomes among newborns with SCD relative to US black newborns. The hemoglobin phenotype of infants with SCD does not influence birth outcome and neonatal course, but infants born to women with sickle cell anemia are at greater risk of preterm birth, low birth weight, being small for gestational age, and neonatal jaundice.

Anemia, Sickle Cell

Effects of delayed reinforcement on infant vocalization rate.

Three previous studies have failed to demonstrate conditioning in infants using a 3-s delay of reinforcement. The effects of a delayed reinforcement schedule on vocalization rates therefore were explored in a single-subject repeated-reversal experimental design for 3 4- to 6-month-old normally developing infants. Each infant received delayed social reinforcement from his or her parent for vocalizing. The comparison condition was a schedule of differential reinforcement of behavior other than vocalizations to control for elicitation by social stimulation. An operant level of infant vocalizations was the initial condition, after which the differential reinforcement schedule was implemented in an across-subjects multiple baseline design. Infants' vocalization rates increased above levels measured during differential reinforcement following onset of the delayed reinforcement condition. Also, vocalization rates decreased during differential reinforcement compared to operant levels. The successful use of delayed reinforcement schedules with infants in this study, as opposed to others, is discussed in terms of procedural differences among them.

Arousal

Newborn screening for sickle cell disease. When is an infant 'lost to follow-up'?

Success of programs to screen newborns for sickle cell disease depends on timely follow-up. Education regarding fever and splenic palpation, and initiation of prophylactic penicillin therapy, will reduce morbidity and mortality and should occur prior to 4 months of age. However, contacting parents to permit implementation of care may be difficult, particularly in large urban populations; only nine (36%) of 25 infants recently identified as having sickle cell disease arrived at our institution for initial appointments. Medical providers must be aware of medical and legal obligations related to follow-up of newborns with sickle cell disease to prevent untoward events in "missed cases."

Follow-Up Studies

The use of radiotelemetry after discharge from the coronary care unit.

Forty-six of 110 consecutive admissions to the coronary care unit (CCU) were monitored by telemetry after discharge from the unit. One of three patients with sudden cardiac death was resuscitated successfully and four patients developed atrial fibrillation which resulted in investigation by echocardiography and treatment with anticoagulants. Significant ventricular ectopic activity was detected in four other patients. Arbitrary selection of patients resulted in those with complicated courses in the CCU being more likely to be monitored by telemetry after transfer to the ward than those which were uncomplicated. There were no cases of sudden cardiac death in patients who did not have telemetry. The cost of telemetry was 2,775 pounds per system. All 19 districts in the North-West region of the UK responded to a questionnaire. Five of the 19 used telemetry but only two of these used the technique routinely after discharge from the CCU whereas four used radiotelemetry to monitor patients on the ward when coronary care beds were unavailable. Eight districts wanted telemetry but needed funds. We conclude that radiotelemetry is an inexpensive way to detect serious arrhythmias in patients transferred from the CCU to the general ward. Monitored from the CCU, cardiac arrest can be immediately detected and treated by experienced staff. Asymptomatic atrial fibrillation leads to prompt investigation by echocardiography and possible treatment with anticoagulants. The importance of noticing major ventricular ectopic activity is likely to increase with the possibility of selecting patients with a high risk of life-endangering arrhythmias so that antiarrhythmic drugs or techniques such as automatic defibrillators can be used appropriately.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

Gunshot wounds then and now.

This review is a study of the development of the bullet and its effects, and the surgical treatment of gunshot wounds from John Hunter's time to the present day.

Art