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Biomedical subjects

D L Wethers

Publications and source records attributed to D L Wethers.

10 recordsLinked to original sources

Febrile episodes in children with sickle cell disease treated on an ambulatory basis.

Children with sickle cell disease have a greatly increased potential for developing rapid and at times fatal sepsis from Streptococcus pneumoniae. Hospitalization and parenteral antibiotic treatment in all febrile children with sickle cell disease have thus become the standard of care at most sickle cell centers. As an alternative approach, we managed selected febrile children with sickle cell disease on an ambulatory basis with parenteral ceftriaxone to determine its safety and effectiveness in preventing sepsis and reducing the number of days of hospitalization. Twenty of 40 children who presented with significant fever met the study criteria and received ceftriaxone on an ambulatory basis. Three were subsequently hospitalized. Compared with a previous year, when all febrile children were admitted, ceftriaxone use reduced the days of hospitalization from 214 (6.3 +/- 1.6 days/patient) to 111 days (2.8 +/- 0.7 days/patient). The empiric use of ceftriaxone appears safe and effective, but it requires an expanded study over an extended period.

Adolescent

Management of acute splenic sequestration crisis in sickle cell disease.

Acute splenic sequestration crisis (ASSC) is a significant cause of early morbidity in children with sickle cell disease. With timely diagnosis and prompt transfusion, the outcome is good. However, recurrences are frequent, and both splenectomy and long-term transfusion therapy have been advanced as appropriate preventive approaches. We describe the diagnosis, management, and course of 15 patients with ASSC followed at the St. Luke's-Roosevelt Hospital Center Comprehensive Sickle Cell Program. Based on our experience, we recommend surgery after the first episode of ASSC in the child 5 years of age and older and the choice of a year or more of long-term transfusion therapy for the child under 3. With intensive education of the parents, and with close observation and individualized management of the patient, the overall prognosis may be improved.

Acute Disease

Spleen dysfunction in hemoglobinopathies determined by pitted red cells.

Splenic dysfunction measured by pitted red cells (pit) was studied in hemoglobinopathies (SS-, SC-, and S beta-type thalassemias and CC-type hemoglobinopathy) in relation to age, in steady state, and during certain significant events. Our experience revealed that the pit count rose with age during steady state in most children with SS disease. A marked increase in pit count was noted in patients with CC disease. The pit count in four patients with S beta+ thalassemia remained normal (i.e., less than 3.5%) at all ages. In children with homozygous SS disease tested at the time of pneumococcal sepsis, the pit count was universally elevated. The pit count was in the normal range in one child with SS disease and osteomyelitis but was elevated in all others. All children had normal pit counts (less than 3.5%) at the onset of acute splenic sequestration crisis, and the counts remained normal during transfusion therapy. No correlation was detected between the pit count and the size of the spleen in patients under 1 year of age.

Adolescent

Growth hormone response to growth hormone releasing factor in sickle cell disease.

Many children with sickle cell disease (SCD) have impaired growth during childhood and adolescence, with patterns of growth consistent with constitutional delay in growth and pubertal development (CDGD). We evaluated the growth hormone (GH) response to a rapid intravenous (i.v.) infusion of growth hormone releasing factor (GRF, 1-44, 1 microgram/kg) in six children with SCD whose growth patterns and bone ages were consistent with CDGD. The peak GH response of the SCD patients to GRF (29.2 +/- 14.3 ng/ml, mean +/- SD, n = 6) was not statistically significantly different from the peak GH response of the control children (29.0 +/- 6.3 ng/ml, mean +/- SD, n = 7). These findings suggest that pituicyte GH response to GRF is intact and is not the cause of the observed impaired growth in patients with SCD.

Adolescent

Reversibility of splenic function by transfusion in two young adults with sickle cell anemia.

A level of circulating "pitted" or vesiculated red blood cells higher than 3.5% was recently reported in studies in the Cooperative Study of Sickle Cell Disease to correlate with splenic dysfunction as shown by spleen scans. Reversal of splenic dysfunction by transfusion in children with sickle cell anemia (SS disease) is known to occur in the young child. We report two older patients with homozygous sickle cell disease, aged 17 and 21 years, whose spleen function, as measured by pit count, was restored to normal range after transfusion.

Adolescent