Prepubertal Growth and Skeletal Maturation in Children With Sickle Cell Disease

Stevens, Michaël C.G.; Maude, G. H.; Cupidore, Lena; Jackson, Harris; Hayes, Richard; Serjeant, G. R.

doi:10.1542/peds.78.1.124

Cited by 86 publications

(3 citation statements)

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“…It can therefore be argued that development of IDA in children with SCD would exacerbate neurocognitive impairment. Additionally, IDA is associated with impaired growth (3) and this may further worsen the growth deficit observed in SCD (32). In summary, IDA is a clinical problem, which affects children with SCD in Jamaica.…”

Section: Discussionmentioning

confidence: 99%

Iron deficiency anaemia in Jamaican children, aged 1-5 years, with sickle cell disease

King¹,

Reid²,

Te³

2005

West Indian med. j.

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Objective:The aim of this study was to determine, using a combination of measures, the prevalence of iron deficiency anaemia (IDA) in children under five years-of-age who have sickle cell disease (SCD) and attend the Sickle Cell Clinic (SCU) of the Tropical Medicine Research Institute. Materials and Methods: Children with homozygous sickle cell anaemia (Hb SS) or doubly heterozygous for Hb S and Hb C (Hb SC) disease who had not received a blood transfusion within three months prior to the iron measurements, were enrolled. The diagnosis of IDA was made if transferrin saturation was less than 16% with serum iron less than 10.7 µmol/l and a low mean corpuscular volume (MCV) for age. Results: Twelve children (8.5%), seven with Hb SS and five with Hb SC had IDA. Adjusting for genotype, children with IDA had significantly higher red blood cell (RBC) counts (4.3 x10 9 /l vs 3.0 x 10 9 /l, p < 0.001) and total iron binding capacity (TIBC) (65.6 µmol/l vs 55.2 µmol/l, p < 0.004) but significantly lower reticulocyte (retic) counts (7.8 % vs 12.2%, p = 0.001) than children without IDA. Conclusion: Iron deficiency anaemia is a clinical problem which affects children with SCD in Jamaica.The higher RBC counts in the IDA group may be due to decreased haemolysis and increased red cell survival whilst the lower reticulocyte counts may be due to impaired erythropoiesis. These observations need to be extended by clinical studies to establish improved diagnostic measures for IDA in SCD. Additionally, clinical trials are needed to determine whether treatment of IDA in children with SCD reduces morbidity and is associated with clinical benefits such as improvements in neurocognitive function.

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Section: Discussionmentioning

confidence: 99%

Iron deficiency anaemia in Jamaican children, aged 1-5 years, with sickle cell disease

King¹,

Reid²,

Te³

2005

West Indian med. j.

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“…Furthermore, IGF‐I levels did not increase after transfusion. Although apparently unrelated to each other, it is a distinct possibility that elevated REE and decreased IGF‐I may both contribute to the linear growth failure in children with SCA (23,24).…”

Section: Discussionmentioning

confidence: 99%

Effects of Red Blood Cell Transfusion on Resting Energy Expenditure in Adolescents with Sickle Cell Anemia

Harmatz,

Heyman,

Cunningham

et al. 1999

J. pediatr. gastroenterol. nutr.

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Background:Previous studies indicate that resting energy expenditure is elevated in children with sickle cell anemia, possibly caused in part by hemolysis and increased erythropoietic activity. The purpose of the present investigation was to determine whether erythrocyte transfusion normalizes resting energy expenditure in sickle cell anemia.Methods:Five adolescents with sickle cell anemia (12‐16 years old; 4 boys, 1 girl) were studied before and 1 week after erythrocyte transfusion before elective surgery or at the initial transfusion for growth failure. Resting energy expenditure was measured by indirect calorimetry, and laboratory measures were determined by routine, validated methods. Data comparisons were by nonparametric analysis.Results:After erythrocyte transfusion, total hemoglobin levels increased (difference (D = 15 g/l; p < 0.05), whereas hemoglobin S (D = ‐0.36; p < 0.05) and reticulocyte count (D = ‐0.12; p < 0.05) decreased. Mean pretransfusion resting energy expenditure was elevated to 124% above predicted levels (p < 0.05) and increased further to 134% above prediction (p < 0.05) vs. pretransfusion levels). Plasma triiodothyronine (T3) levels increased (D = 0.17 nmol/l; p < 0.05), reverse T3 (rT3) levels tended to decline (D = ‐0.04 nmol/l; p = 0.14), and rT3/T3 decreased (D = ‐0.03; p < 0.05). Plasma insulin‐like growth factor‐I (IGF‐I) levels were low‐normal before transfusion and did not change, despite the change in resting energy expenditure.Conclusions:The results confirm that resting energy expenditure is elevated in patients with sickle cell anemia. However, resting energy expenditure further increased after transfusion, despite decreased erythropoietic activity. A posttransfusion decrease in rT3/T3 may contribute to the increased resting energy expenditure. That there was no change in IGF‐I implies that the growth hormone‐IGF system is not involved in posttransfusion regulation of resting energy expenditure. Therefore, our data are not consistent with the hypothesis that increased resting energy expenditure in sickle cell anemia is directly related to erythropoietic activity. The mechanisms by which resting energy expenditure increases after transfusion in sickle cell anemia require additional investigation.

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“…It is important to note that impairment of growth in children and adolescents with sickle cell disease is a well-recognized complication of this disease condition [ 40 , 41 , 42 , 43 ]. Studies have shown that the main cause of growth impairment in this patient population is due to bone marrow hyperplasia [ 44 ].…”

Section: Common Bone Involvement In Patients With Sickle Cell Diseasementioning

confidence: 99%

Management of Salter–Harris Type 1 Fracture Complicated with Osteomyelitis in a Sickle Cell Disease Patient: A Case Report and Review of Literature

2022

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Salter–Harris fractures may occur due to a single injury or repetitive stress fractures on the extremities. Type I to III fractures are managed medically, while types IV and V, which are rare, are treated surgically. In the pediatric population, Salter–Harris I fractures of the distal tibia are commonly seen, and management of such fractures are well established in the literature. Despite the availability of a wide range of treatment for such fractures, osteonecrosis or avascular necrosis of the proximal femur can subsequently develop. Avascular necrosis is cell death secondary to metabolic disturbances, trauma, adverse effects of certain medications, or sickle cell disease. Avascular necrosis commonly affects the talus, humerus, or tibia in addition to the femoral head. Radiographic images are essential for prompt diagnosis and to minimize negative health outcomes in these patients. However, Salter–Harris I fracture in sickle cell patients can be very challenging due to these patients’ vulnerability to bone infections and sickle cell crisis. In this case report, our patient with a history of sickle cell disease and with a diagnosis of Salter–Harris I fracture was treated with surgical intervention as type V, which is discussed in this article, and responded well to treatment. Thus, this case suggests a new approach to managing Salter–Harris I fractures complicated with osteomyelitis in sickle cell patients.

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Prepubertal Growth and Skeletal Maturation in Children With Sickle Cell Disease

Cited by 86 publications

References 0 publications

Iron deficiency anaemia in Jamaican children, aged 1-5 years, with sickle cell disease

Iron deficiency anaemia in Jamaican children, aged 1-5 years, with sickle cell disease

Effects of Red Blood Cell Transfusion on Resting Energy Expenditure in Adolescents with Sickle Cell Anemia

Management of Salter–Harris Type 1 Fracture Complicated with Osteomyelitis in a Sickle Cell Disease Patient: A Case Report and Review of Literature

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