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Biomedical subjects

M Koshy

Publications and source records attributed to M Koshy.

51 records · Page 3Linked to original sources

Leg ulcers in patients with sickle cell disease.

During the entry examination, leg ulcers were present in 2.5% of 2,075 patients 10 years of age and older with sickle cell disease who entered into the Cooperative Study of Sickle Cell Disease (CSSCD) between 1979 and 1986. Prevalence rates were highest among patients with sickle cell anemia and sickle cell anemia with thalassemia genotypes. Among sickle cell anemia patients free of ulcers at entry, the overall incidence was 5.73 per 100 person years in those having associated alpha-thalassemia and 9.97 for those without. Among sickle cell anemia patients with two alpha genes, the estimated incidence of leg ulcers is 2.38 per 100 person years and 6.12 per 100 person years among sickle cell anemia patients with three alpha genes (P less than .05). In both groups, the incidence was highest among those patients over 20 years of age and considerably higher among males than females (P less than .001). Leg ulcers were nonexistent in patients with sickle beta plus thalassemia and sickle hemoglobin C disease. Low steady-state hemoglobin is associated with a higher incidence of ulcer formation (P less than .0001) in sickle cell anemia patients. The protective effect of hemoglobin F is apparent at all levels of total hemoglobin among sickle cell anemia patients and those with associated alpha-thalassemia.

Adolescent↗

Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study.

Prophylactic blood transfusion has come to be regarded as necessary in the treatment of patients with sickle cell disease during pregnancy. Because of the risks associated with blood products and reports of successful outcomes without the use of blood transfusion, we conducted a prospective randomized controlled study of this issue. Seventy-two pregnant patients with sickle cell anemia were randomly assigned to one of two treatment groups: 36 received prophylactic transfusions of frozen red cells, and 36 received red-cell transfusions only for medical or obstetric emergencies. Twenty-eight patients with sickle cell anemia who did not qualify for randomization (mainly because they had other medical disorders), 66 with sickle cell-hemoglobin C disease, and 23 with sickle cell-beta-thalassemia were also followed and received transfusions only for emergencies. There was no significant difference in perinatal outcome between the offspring of mothers with sickle cell disease who were assigned to treatment with prophylactic transfusions and those who were not (15 vs. 5 percent). The occurrence of a perinatal death in a previous pregnancy and the presence of twins in the present pregnancy were two major risk factors for an unfavorable outcome; when they were present, perinatal mortality was 50 percent. Perinatal mortality was somewhat higher in the two groups that were randomized than in the three groups that were not. Prophylactic transfusion significantly reduced the incidence of painful crises of sickle cell disease (P less than 0.01) and substantially reduced the cumulative incidence of other complications of this disorder (P = 0.07). Other medical and obstetric complications occurred with nearly equal frequency in the two randomized groups. Increases in costs, the number of hospitalizations, and the risk of alloimmunization were disadvantages of prophylactic transfusion. We conclude that the omission of prophylactic red-cell transfusion will not harm pregnant patients with sickle cell disease or their offspring.

Adult↗

Recruitment in the Cooperative Study of Sickle Cell Disease (CSSCD).

The Cooperative Study of Sickle Cell Disease (CSSCD) is a multiinstitutional investigation of the natural history of clinical course of sickle cell disease from birth through adulthood. The study is not a trial; rather, it involves data collection at 23 institutions in a uniform, standardized fashion on 3800 patients. Recruitment aspects that were addressed include issues related to recruitment of different age groups, ranging from newborns to pregnant women to patients over 50 years of age; the need to include mildly affected patients to ensure that the study would not reflect only a severe hospital-based population; recruitment from rural populations; and the need to screen and enter a newborn population at birth. The recruitment goal of entering 3200 patients, including 2100 patients with SS hemoglobinopathy, over a 24-month period was accomplished after 27 months.

Age Factors↗

Cooperative Study of Sickle Cell Disease: Demographic and socioeconomic characteristics of patients and families with sickle cell disease.

Socioeconomic data on 3538 black patients enrolled in the Cooperative Study of Sickle Cell Disease (CSSCD) from 1979 to 1981 are summarized and compared with the U.S. black population (USBP) where appropriate. The following contrasts between the patients and families with Sickle Cell Disease (SCD) and the USBP were found: family structure differed, with fewer two-parent families (40 vs 54%) and more single female heads-of-household (53 vs 42%) within the SCD population; of those employed, twice as many of the SCD patients of both sexes worked in white collar positions; a higher percentage of patients were unemployed and disabled; and male SCD patients had a lower median personal income than USBP males. Several similarities were also noted: the percentage of high school graduates was 71% for SCD patients and 75% for the USBP; female heads-of-household employed full time earned approximately the same salary as USBP females. These data will help health professionals who counsel SCD patients and their families.

Adolescent↗

Management of pain in sickle cell disease using biofeedback therapy: a preliminary study.

Fifteen patients with a history of painful episodes of sickle cell disease were given training in progressive relaxation, thermal biofeedback, cognitive strategies, and self-hypnosis to help them develop self-management skills to relieve pain. Results show a 38.5% reduction in the number of emergency room visits, a 31% reduction in the number of hospitalizations, and a 50% reduction in the inpatient stay during the 6 months since the beginning of therapy compared to 6 months prior to therapy. Analgesic intake was reduced by 29% for those who were using it regularly. This is a preliminary study, and the results are considered only as suggestive of the potential use of biofeedback therapy and behavioral management in alleviating painful episodes in sickle cell disease.

Adult↗

Continuous electrocardiographic monitoring in patients with sickle-cell anemia during pain crisis.

Electrocardiographic studies in patients with sickle-cell anemia have been performed during the normal resting state with routine twelve-lead ECGs. We studied 30 patients with sickle-cell disease in acute crisis with 24-hour continuous electrocardiographic monitoring. The standard ECG demonstrated a high incidence of abnormalities, but only three patients had arrhythmias. These findings contrasted sharply with the results of continuous monitoring, during which arrhythmias were detected in 24 of 30 patients. They were fairly evenly split between atrial (60%) and ventricular (67%). Nine of the patients had "complex arrhythmias" including two with episodes of ventricular tachycardia. Seventeen patients subsequently underwent equilibrium gated-blood pool scans. Eight patients had abnormal contractility and tended to have more arrhythmias on monitoring than those with normal contractility. Thus, continuous electrocardiographic monitoring of sickle-cell patients during crisis revealed a higher incidence of arrhythmias than previously thought.

Adolescent↗

Hemoglobin F Lódź (G gamma I 44 Ser replaced by Arg). A newly identified variant from an American infant of Polish descent.

An electrophoretically slowly migrating hemoglobin variant was identified in a neonate of Polish parents from a cord blood hemoglobin survey. Structural analysis of the gamma chain of the mutant hemoglobin, with fractionation of the tryptic peptides by means of high-performance liquid chromatography, demonstrated a substitution of serine-44 by arginine. Glycine was identified at position 136 of the gamma chain and isoleucine at position 75. Serine residue 44 appears not to be a heme contact site, but as a result of the substitution in this mutant hemoglobin, the possibility exists for formation of a salt bridge between the arginine and a heme propionate group. The infant's hematologic findings suggested the possibility of mild hemolysis, but these changes may have been due to isoimmune disease.

Amino Acids↗

Post-transfusion alloimmunization in patients with sickle cell disease.

The transfusion histories over a 33-month period of 50 patients with sickle cell disease were reviewed to determine the frequency of alloimmunization to red cell antigens following transfusion in these patients. There were 30 females and 20 males, aged 19--49 years. Eighteen (36%) were immunized of which thirteen were females. Five of the patients have formed only one antibody so far, while the other 13 have formed two or more. Thirty-six antibodies were identified: 16 against various Rh antigens, 12 anti-Lewis, 5 anti-Kell and one each of anti-Jka, -Fya and -M. The immunized patients received, on the average, more transfusions although there was a considerable degree of overlap between the immunized and nonimmunized groups. An approach to the hemotherapy of patients with sickle cell disease (SCD) is discussed.

Adult↗

Sickle cell syndromes. II. The sickle cell anemia-alpha-thalassemia syndrome.

Five American black patients, ages 1 to 16 years, with the sickle cell anemia-alpha-thalassemia syndrome are described. Each patient had persistent microcytosis not explained by iron deficiency, and in each family the presence of alpha-thalassemia in combination with sickle cell trait was demonstrated in one of the parents. In one patient, in whom the diagnosis of sickle cell anemia was established at birth, an elevated level of Barts (gamma4) hemoglobin was also found. In these patients levels of alkali-resistant hemoglobin and reticulocyte counts were similar to those of sickle cell anemia patients of comparable age; however, stained smears of their peripheral blood rarely showed the presence of irreversibly sickled cells. No major ameliorative effect of the alpha-thalassemia on the clinical expression of the sickle cell disease of these patients was evident.

Adolescent↗

Pregnancy in patients with hemoglobinopathies and thalassemias.

The availability of sophisticated laboratory procedures has made the diagnosis of many hemoglobinopathies and thalassemias simple. Structural alteration of the polypeptide chains and defects in the rate of synthesis of any of the polypeptide chains are the most common abnormalities: hemoglobins S, C and D are examples of the former, and the thalassemias are examples of the latter. The pathophysiology, clinical manifestations and individual variation of each abnormality are significantly different. Early diagnosis and the knowledge of the pathophysiology together with careful and frequent follow-up are necessary for providing better medical care. Pregnancy in a patient with a diagnosis of hemoglobinopathy or thalassemia certainly speaks for risks to both the mother and fetus. Management of each case has to be planned individually to provide optimal medical and supportive care. We have found a special combined hematology-obstetrics clinic to be helpful in the follow-up of these high-risk patients.

Anemia, Sickle Cell↗

Renal papillary necrosis in sickle cell hemoglobinopathies.

Approximately 65 per cent of patients with clinical manifestations of S-hemoglobinopathies show urographically demonstrable changes of renal papillary necrosis, regardless of the presence of urinary symptoms. With a more precise excretory urographic technique these changes can be diagnosed with greater accuracy without the need for sophisticated equipment. Excretory urography should be performed routinely in the evaluation of all patients with manifestations of S-hemoglobinopathy in order to diagnose papillary necrosis before the onset of urological symptoms.

Adolescent↗

Hospital utilization patterns and costs for adult sickle cell patients in Illinois.

OBJECTIVES: To determine population size, demographic characteristics, hospital utilization patterns, the specialties of physicians providing care, and costs for hospitalized adult sickle cell patients in Illinois. METHODS: A statewide, administrative dataset for the two-year period from january 1992 through December 1993 was analyzed retrospectively. RESULTS: There were 8403 admissions among 1189 individual sickle cell patients for the two-year period. Eighty-five percent of patients resided in the Chicago metropolitan area. The median age of the 1189 patients was 29; two-thirds had Medicaid or Medicare coverage. Emergency departments were the primary source of admissions (85.7%). The most common admitting diagnosis was painful crisis (97.4%), and average length of stay was four days. The median number of admissions per patient was three; most patients (85%) used only one or two hospitals. A small group used more than four hospitals and accounted for 23% of statewide admissions. Primary care physicians cared for most patients, and total hospitalization charges were more than $59 million. CONCLUSIONS: In Illinois the adult sickle cell population is concentrated in major urban centers, primarily the Chicago metropolitan area. These patients accounted for approximately 8400 admissions and more than $59 million in hospital charges during the two-year study period. A small group of patients used multiple hospitals and accounted for more than 23% of total hospitalization charges. This study shows the necessity of and provides a useful framework for developing targeted programs for adult sickle cell patients as well as for training physicians to efficiently provide comprehensive health care services for this population.

Adolescent↗