Sreedhar P. Rao

PRIAPISM IN CHILDREN WITH SICKLE CELL DISEASE

A review of hospital admissions during 80 months revealed only 8 patients with episodes of priapism of approximately 400 pediatric male patients with sickle cell disease. The patients, who ranged in age from 5 to 19 years, underwent a 99mtechnetium penile scan, and 4 had a low and 4 had a high flow scan. Three cases resolved with hydration alone. Five patients received exchange transfusion of whom 3 subsequently underwent shunt procedures. One patient with a 5-year history of recurrent stuttering episodes was placed on transfusion therapy for 6 months and stuttering episodes have not recurred. One patient had a cerebrovascular accident 1 day after hospital discharge and another had priapism while on chronic transfusion therapy for a cerebrovascular accident. Each postpubertal patient had a severe clinical course; 1 had temporary impotence for 3 months and another had impotence at 2 weeks but was lost to followup. While 99mtechnetium penile scans may help clarify the severity of vascular stagnation, in our small group they were not helpful in predicting clinical course.

Less intensive long-term transfusion therapy for sickle cell anemia and cerebrovascular accident

To determine the efficacy of a less intensive transfusion regimen in preventing recurrent cerebrovascular accidents and reducing transfusion requirements in patients with sickle cell anemia, we offered to 14 patients who had been undergoing aggressive transfusion therapy (sickle hemoglobin concentration kept less than 30% of total) for a mean of 9 years the option of either diminishing or stopping transfusion therapy. Thirteen patients chose to continue a modified transfusion regimen to maintain sickle hemoglobin concentration less than 60%; 10 of these patients have now been followed for 1 year or more (12 to 27 months, mean 15.5 months). There have been no recurrent neurologic events, although two patients have died of complications of hemochromatosis. All patients had a reduction in donor exposure, and there was a mean reduction in net transfusion requirement of 31.4% during the first year after modification. The greatest reduction was achieved in the single patient managed by small-volume (5 ml/kg) simple transfusion rather than partial packed cell exchange. We conclude that although long-term consequences of less aggressive transfusion therapy are unknown, the use of such a regimen may be reasonable, particularly in patients with significant transfusional hemochromatosis.

Cerebrovascular accidents in children with sickle-cell disease and alpha-thalassemia.

Alpha-thalassemia occurs commonly in black Americans, ~ with approximately 30% lacking one a-glob{n gene (athai-2 genotype: aa/a-) and 4% lacking two genes (athai- 1 genotype: a-/a- or, rarely, aa/--). In individuals with sickle-cell disease, a-thalassemia attenuates some of the hematologic manifestations, 2-4 although the extent of clinical benefit remains controversial?. 5 Before routine implementation of long-term transfusion programs for children with sickle-cell disease and cerebrovascular accident, 6, 7 CVA was ~i significant cause of death in the young sickle-cell disease population? Because the favorable effects of a-thalassemia on hematologic manifestations are apparent by the age of 1 year, 9 it is possible that a reduction in incidence of CVA, which occurs at a mean age of 7 years, s might contribute to prolonged survivaP in patients with sickle cell-a-thalassemia. In fact, a reduced incidence of a-thalassemia has been reported in a small group of patients with sickle-cell disease who have had a CVA? ~ The a-globin gene status of 20 of our sickle-cell disease patients with a history of CVA has been ascertained and is the basis for this report. METHODS

Alveolar-arterial oxygen gradient in acute chest syndrome of sickle cell disease†

In 44 episodes of acute chest syndrome of sickle cell disease occurring in 37 children, simple clinical severity score, duration of hospital stay, transfusion data, and alveolar-arterial oxygen gradient were analyzed as indicators of severity of disease. The alveolar-arterial oxygen gradient, measured during breathing of room air, proved, on multivariate analysis, to be the strongest predictor of both clinical severity and the need for blood transfusion.

Human parvovirus infection in patients with sickle cell disease with and without hypoplastic crisis

Presque toutes les crises hypoplasiques chez les enfants avec anemie a hematies falciformes etaient associees a une infection aigue a parvovirus. Mais toutes les infections a parvovirus chez ces enfants ne provoquent pas des crises hypoplasiques cliniquement apparentes

Sickle cell-hemoglobin E disease: Clinical findings and implications

1. Tanner JM, Whitehouse RH, Takaishi M. Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. Part I, part II. Arch Dis Child 1966;41:454-71,613-35. 2. Food and Nutrition Board, Commission on Life Sciences. Recommended dietary allowances. 10th ed. Washington, D.C.: National Academy Press, 1989. 3. Berg K. Nutrition of children with reduced physical activity due to cerebral palsy. Nutr Diet 1973;19:12-20. 4. American Dietetic Association. Infant and child nutrition: concerns regarding the developmentally disabled. J Am Diet Assoc 1981;78:443-52. 5. Tolman KG, Jubiz W, Sanella J J, et al. Osteomalacia associated with anticonvulsant drug therapy in mentally retarded children. Pediatrics 1975;56:45-51. 6. Bandini LG, Schoeller DA, Fukagawa NK, Wykes L J, Dietz WH. Body composition and energy expenditure in adolescents with cerebral palsy or myelodysplasia. Pediatr Res 1991;29: 70-7. 7. Culley W J, Middleton TO. Caloric requirements of mentally retarded children with and without motor dysfunction. J PEDIATR 1969;75:380-4.

TRANSIENT NEUTROPENIA OF CHILDHOOD

While neutropenia (ANC<1500) related to acute infections in children is well known, natural histroy and appropriate management are unclear. Clinical data on 22 children referred for neutropenia over a 13 mo. period were reviewed. Twenty patients were age 3 mos.-3 yrs. and 2 were age 11 yrs. ANC was <500/cmm in 18 patients. All patients were febrile at some point in their illness; the majority (13) had respiratory tract infection. Infectious agents were isolated in 6 patients (S. pneumo. 2, H.flu, 1, E.coli 1, RSV 2). Neutropenia may have been drug-related in 5 patients (carbamazepine 1, chloramphenicol 2, penicillin 1, ampicillin 1). Bone marrow aspiration, performed in 14 patients, was normal in 7; seven patients demonstrated maturation arrest.Recovery from neutropenia was documented in 20 patients; one child has remained neutropenic for 12 months and one was lost to follow-up. Ten recovered within 1 wk. of referral, whereas 10 remained neutropenic from 1 to 6 wks. Time to recovery could not be predicted based on any clinical or laboratory parameters available.These cases of childhood neutropenia appear to have been infection-related and 20/21 patients recovered. Superimposed bacterial infection did not occur even with ANC<500/cmm; empiric treatment with broad spectrum antibiotics was not instituted. Bone marrow evaluation, while useful in excluding infiltrative disorders and aplastic anemia, was not predictive of the rate of recovery.

BONE INFARCTION, OR OSTEOMYELITIS IN CHILDREN WITH SICKLE CELL DISEASE: BONE AND BONE MARROW SCAN FINDINGS

Bone infarction (INF) occurs commonly in patients with sickle cell disease (SCD), and is frequently associated with infarction of the bone marrow (INF). Clinically and radiographically it is difficult to distinguish INF from Osteomyelitis (OSTEO). Bone scan using Tc99 diphosphonate &/or bone marrow scan using Tc99 Sulfur Colloid were done in 33 children (1-16 yrs.) with SCD (SS 32, S-C 1) who had localized bone pain and fever. Twenty-six of 33 patients had INF and 7 had bacteriologically proven OSTEO (Salmonella 5, S. Pneumo 2).As shown in table, while bone scan uniformly showed increased uptake in OSTEO, uptake was also frequently increased in INF. In contrast, marrow scan demonstrated normal uptake in all four OSTEO patients evaluable; uptake was diminished or absent in 10/11 patients with INF. Thus, in a patient with SCD who has localized bone pain and fever, a normal marrow uptake at the site of pain strongly suggests OSTEO and the need for diagnostic bone aspiration and appropriate therapy. Bone scan appears to be less useful than marrow scan in distinguishing between INF and OSTEO.

PLATELET ASSOCIATED IMMUNE GLOBULIN|[lpar]|PAIg|[rpar]| IN THE COURSE OF CHILDHOOD IDIOPATHIC THROMBOCYTOPENIC PURPURA

Platelet associated immune globulin was studied during the course of ITP in 15 children; the microtiter solid phase immunoassay was used.Values in 10 patients with acute ITP were compared with those in 5 children with chronic ITP.At the onset,PAIgG was elevated in 9/10 children with acute ITP;8/10 had levels>70fg/pl (median PAIgG=91.7;normal=<5.2fg/pl) The one child with acute ITP whose PAIgG was normal recovered within two weeks.At recovery PAIgG levels had fallen below 10fg/pl(median 3.7 fg/pl) in 7/8;5/7 had normal levels.In contrast, 4/5 children whose disease became chronic, had lower levels of PAIgG (<16.7fg/pl).Only 1/5 chronic ITP patients had markedly elevated PAIgG. In 2 patients post-splenectomy, levels were normal even in transient periods of thrombocytopenia. In one patient,PAIgG rose dramatically to 75 fg/pl during an intercurrent viral illness. Another patients PAIgG levels rose transiently following treatment with IV gamma globulin; no change in the platelet count occurred.These findings suggest that PAIgG is usually higher at the onset of classic childhood ITP (acute) than in those children whose course becomes chronic although the levels are not individually predictive. The observed drop in PAIgG after splenectomy as well as the sharp rises noted during infection and gammaglobulin treatment deserve further study to help understand the relationship between thrombocytopenia and PAIgG.