Prepubertal Growth and Skeletal Maturation in Children With Sickle Cell Disease

PEDIATRICS ◽  
1986 ◽  
Vol 78 (1) ◽  
pp. 124-132
Author(s):  
Michael C. G. Stevens ◽  
Gillian H. Maude ◽  
Lena Cupidore ◽  
Helen Jackson ◽  
Richard J. Hayes ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Skeletal Maturity ◽  
Pubertal Development ◽  
Bone Age ◽  
Skeletal Maturation ◽  
Similar Data ◽  
Cell Disease ◽  
Normal Children ◽  
Normal Mean ◽  
Benign Nature

In a longitudinal study of 298 children with homozygous sickle cell (SS) disease and 157 children with hemoglobin SC disease, between birth and 9 years of age, observations of weight and height were made. These were compared with similar data derived from an age- and sex-matched group of 231 children with a normal hemoglobin (AA) genotype. Growth in children with SC disease was not significantly different from that in normal children, but children with SS disease had statistically significant, and progressive, deficits in both weight and height before 2 years of age. The average deficit approached 1 SD below the normal mean for age by 9 years. Observations of skeletal maturity, based on radiologic assessment of bone age at the wrist, were made on a proportion of these children at 5 and 8 years of age. Children with SS disease were significantly retarded at 8 years but not 5 years, which is consistent with increasing deficit in height. These observations confirm the early impact of SS disease on physical development and provide standards from which clinical expectations of growth may be derived. The relevance of these findings and their relationship to the characteristic delay in pubertal development is discussed together with a review of possible etiologic factors. The benign nature of SC disease is endorsed by the absence of an effect on growth in the prepubertal child.

scholarly journals Variation in fetal hemoglobin parameters and predicted hemoglobin S polymerization in sickle cell children in the first two years of life: Parisian Prospective Study on Sickle Cell Disease

Blood ◽  
1994 ◽  
Vol 84 (9) ◽  
pp. 3182-3188 ◽  
Author(s):  
M Maier-Redelsperger ◽  
CT Noguchi ◽  
M de Montalembert ◽  
GP Rodgers ◽  
AN Schechter ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Oxygen Saturation ◽  
Cell Population ◽  
Sickle Cell ◽  
Clinical Manifestations ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Normal Children ◽  
Hemoglobin S ◽  
F Cells

Abstract Intracellular hemoglobin S (HbS) polymerization is most likely to be the primary determinant of the clinical and biologic manifestations of sickle cell disease (SCD). Fetal hemoglobin (HbF) does not enter the HbS polymer and its intracellular expression in sickle erythrocytes inhibits polymerization. HbF levels, high at birth but decreasing thereafter, protect the newborn from the clinical manifestations of this hemoglobinopathy. We have measured the sequential changes in HbF, F reticulocytes, and F cells in the first 2 years of life in 25 children with SCD and compared the results with those obtained in 30 normal children (AA). We have also calculated HbF per F cell (F/F cell), the preferential survival of F cells versus non-F cells, as measured by the ratio F cells versus F reticulocytes (FC/FR) and polymer tendency at 40% and 70% oxygen saturation. HbF levels decreased from about 80.4% +/- 4.0% at birth to 9.2% +/- 2.9% at 24 months. During this time, we observed a regular decrease of the F reticulocytes and the F cells. The kinetics of the decline of F/F cell was comparable with the decline of HbF, rapid from birth (mean, 27.0 +/- 3.6 pg) to 12 months of age (mean, 8.5 +/- 1.5 pg) and then slower from 12 to 24 months of age (mean, 6.2 +/- 1.0 pg) in the SCD children. In the AA children, the decrease in HbF, due to changes in both numbers of F cells and F/F cell, was more precipitous, reaching steady-state levels by 10 months of age. Calculated values for mean polymer tendency in the F-cell population showed that polymerization should begin to occur at 40% oxygen saturation at about 3 months and increase progressively with age, whereas polymerization at 70% oxygen saturation would not occur until about 24 months. These values correspond to HbF levels of 50.8% +/- 10.8% and 9.2% +/- 2.9%, respectively, and F/F cell levels of 15.6 +/- 4.5 pg and 6.2 +/- 1.0 pg, respectively. In the non--F-cell population, polymerization was expected at birth at both oxygen saturation values. Three individuals had significantly greater predicted polymerization tendency than the remainder of the group because of early decreases in HbF. These individuals in particular, the remainder of the cohort, as well as other recruited newborns, will be studied prospectively to ascertain the relationship among hematologic parameters, which determine polymerization tendency and the various clinical manifestations of SCD.

scholarly journals Growth and Pubertal Development in Elite Female Rhythmic Gymnasts

1999 ◽  
Vol 84 (12) ◽  
pp. 4525-4530 ◽  
Author(s):  
N. Georgopoulos ◽  
K. Markou ◽  
A. Theodoropoulou ◽  
P. Paraskevopoulou ◽  
L. Varaki ◽  
...  
Keyword(s):  
Body Fat ◽  
Adult Height ◽  
Pubertal Development ◽  
Optimal Growth ◽  
Bone Age ◽  
Skeletal Maturation ◽  
Chronological Age ◽  
Average Height ◽  
Limited Information ◽  
Rhythmic Gymnasts

Optimal growth depends upon both environmental and genetic factors. Among environmental factors that could alter growth and sexual maturation are stress and intensive physical training. The influence of these factors has been documented in a variety of sports, but there is limited information on rhythmic gymnasts, who have entirely different training and performance requirements. The study was conducted during the 13th European Championships in Patras, Greece, and included 255 female rhythmic gymnasts, aged 11–23 yr. The study included measurement of height and weight, assessment of breast and pubic hair development, estimation of body fat and skeletal maturation, and registration of menarcheal age and parental height. Gymnasts were taller than average height for age, with mean height above and mean weight below the 50th percentile. Actual height sd score was positively correlated to weight sd score (P < 0.001), number of competitions (P = 0.01), and body mass index (BMI; P < 0.001). Predicted adult height sd score was positively correlated to weight sd score (P < 0.001) and negatively to body fat (P = 0.004). There was a delay in skeletal maturation of 1.3 yr (P < 0.001). Pubertal development was following bone age rather than chronological age. The mean age of menarche was significantly delayed from that of their mothers and sisters (P = 0.008 and P = 0.05, respectively), was positively correlated to the intensity of training and to the difference between chronological age and bone age (P < 0.001 and P = 0.002, respectively), and was negatively correlated to body fat (P < 0.001). In the elite female rhythmic gymnasts, psychological and somatic efforts have profound effects on growth and sexual development. Despite these aberrations, adult height is not expected to be affected.

scholarly journals Management of osteonecrosis of the femoral head in children with sickle cell disease: results of conservative and operative treatments at skeletal maturity

2018 ◽  
Vol 12 (1) ◽  
pp. 47-54
Author(s):  
C. Mallet ◽  
A. Abitan ◽  
C. Vidal ◽  
L. Holvoet ◽  
K. Mazda ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Skeletal Maturity ◽  
Weight Bearing ◽  
Long Term Results ◽  
Harris Hip Score ◽  
Cell Disease ◽  
Femoral Head Osteonecrosis

Purpose Sickle cell disease (SCD) is the most common cause of femoral head osteonecrosis (ONFH) during childhood with an overall prevalence of 10%. In children, spontaneous revascularization can occur, as in Legg-Calve-Perthes disease. Consequently, the aim of treatment is to restore proper hip containment to prevent joint arthritis. This is the first study reporting long-term results at skeletal maturity of non-operative and surgical treatments for ONFH in SCD children. Methods All children with ONFH due to SCD were retrospectively reviewed. At initial evaluation, extension of osteonecrosis was radiographically defined using Catterall, lateral pillar Herring and Ficat classifications. Subluxation of the femoral head with Reimers migration index > 30% required surgical treatment including femoral varus osteotomy and/or pelvic osteotomies. Conservative treatment including non-weight bearing and physiotherapy was performed in the remaining cases. Outcomes were assessed at skeletal maturity using the Harris Hip Score (HHS) and the Stulberg classification. Total hip arthroplasty and Stulberg 5 were defined as failures. Results A total of 25 hips in 17 patients were included (mean follow-up 7.5 years SD 3.4). Mean age at diagnosis was 11.4 years SD 2.9. In all, 15 hips (60%) were classified Catterall 3 and 4 and Herring B and C. A total of 13 patients (52%) underwent surgical treatment. At skeletal maturity, mean HHS was good (81 SD 17), 12 hips (48%) were classified Stulberg 1 and 2, seven hips (28%) were classified Stulberg 3 and 4. Conclusion Both treatments led to good functional results with 75% of congruent hips at skeletal maturity. Level of Evidence IV

scholarly journals Objective Evaluation of Cervical Vertebral Bone Age— Its Reliability in Comparison with Hand-Wrist Bone Age: By TW3 Method

2013 ◽  
Vol 14 (5) ◽  
pp. 806-813
Author(s):  
Vamsi Nilay Reddy ◽  
Gojja Sreedevi ◽  
CMS Krishna Prasad ◽  
Swaroopa Rani Ponnada ◽  
K Padma Priya ◽  
...  
Keyword(s):  
Skeletal Maturity ◽  
Cervical Vertebrae ◽  
Bone Age ◽  
Objective Evaluation ◽  
Ease Of Use ◽  
Skeletal Maturation ◽  
Potential Growth ◽  
Vertebral Bone ◽  
Therapeutic Decisions ◽  
Lateral Cephalograms

ABSTRACT Aim The aim of this study was to establish the validity of a new method for evaluating skeletal maturation by assessing the 3rd and 4th cervical vertebrae seen in the cephalometric radiograph. Materials and methods This study consisted of a sample of 50 patients in the age group of 8 to 14 years of age. Chronologically, they were divided into six groups, based on the age consisting of a minimum of six to a maximum of 10 subjects. All the patients included in the study were females. The selected subjects were clinically examined and then age and date of birth of the patient in years and months was noted. Then lateral cephalograms and hand-wrist radiographs of the patient were taken on the same day with good clarity and contrast. Results The results suggested that cervical vertebral bone age on cephalometric radiographs calculated with this method is as reliable at estimating bone age as is the Tanner-Whitehouse 3 (TW3) method on hand-wrist radiographs. By determining the cervical vertebral bone age, skeletal maturity can be evaluated in a detailed and objective manner with cephalometric radiographs. Conclusion The ability to accurately appraise skeletal maturity from cervical vertebral maturation, without the need for additional radiographs, has the potential to improve orthodontic diagnostic and therapeutic decisions. The technique's simplicity and ease of use should encourage this method as a first level diagnostic tool to assess skeletal maturation. Clinical significance This study revealed that the timing and sequence of ossification of the bones in hand and wrist and cervical vertebrae were able to relate the skeletal development of the various skeletal maturity indicators to a child's development. This method provided a mean with which one can determine the skeletal maturity of a person and thereby determine whether the possibility of potential growth existed. How to cite this article Prasad CMSK, Reddy VN, Sreedevi G, Ponnada SR, Priya KP, Naik BR. Objective Evaluation of Cervical Vertebral Bone Age—Its Reliability in Comparison with Hand-Wrist Bone Age: By TW3 Method. J Contemp Dent Pract 2013;14(5):806-813.

SKELETAL MATURATION IN THE CURRENT PEDIATRIC MEXICAN POPULATION

2020 ◽  
Vol 26 (10) ◽  
pp. 1053-1061
Author(s):  
Miguel Klünder-Klünder ◽  
Montserrat Espinosa-Espindola ◽  
Desiree Lopez-Gonzalez ◽  
Mariana Sánchez-Curiel Loyo ◽  
Pilar Dies Suárez ◽  
...  
Keyword(s):  
Middle Childhood ◽  
Skeletal Maturity ◽  
Bone Age ◽  
Mexican Population ◽  
Growth Potential ◽  
Skeletal Maturation ◽  
Chronological Age ◽  
Greulich And Pyle ◽  
Reference Curves ◽  
End Of Puberty

Objective: The most commonly used methods for bone age (BA) reading were described in the Caucasian population decades ago. However, there are secular trends in skeletal maturation and different BA patterns between ethnic groups. Automated BA reading makes updating references easier and more precise than human reading. The objective of the present study was to present automated BA reference curves according to chronological age and gender in the Mexican population and compare the maturation tempo with that of other populations. Methods: The study included 923 healthy participants aged 5 to 18 years between 2017 and 2018. A hand radio-graph was analyzed using BoneXpert software to obtain the automated BA reading according to Greulich and Pyle (G&P) and Tanner-Whitehouse 2 (TW2) references. We constructed reference curves using the average difference between the BA and chronological age according to sex and age. Results: The G&P and TW2 automated reference curves showed that Mexican boys exhibit delays in BA during middle childhood by 0.5 to 0.7 (95% confidence interval [CI], −0.9 to −0.2) years; however, they demonstrate an advanced BA of up to 1.1 (95% CI, 0.8 to 1.4) years at the end of puberty. Mexican girls exhibited a delay in BA by 0.3 to 0.6 (95% CI, −0.9 to −0.1) years before puberty and an advanced BA of up to 0.9 (95% CI, 0.7 to 1.2) years at the end of puberty. Conclusion: Mexican children aged <10 years exhibited a delay in skeletal maturity, followed by an advanced BA by approximately 1 year at the end of puberty. This may affect the estimation of growth potential in this population. Abbreviations: BA = bone age; CA = chronological age; G&P = Greulich and Pyle; TW2 = Tanner-Whitehouse 2

scholarly journals Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study

2018 ◽  
Vol 2 (3) ◽  
pp. 285-291 ◽  
Author(s):  
Riten Kumar ◽  
Joseph Stanek ◽  
Susan Creary ◽  
Amy Dunn ◽  
Sarah H. O’Brien
Keyword(s):  
Risk Factors ◽  
Retrospective Study ◽  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Administrative Database ◽  
Similar Data ◽  
Cell Disease ◽  
Database Study ◽  
Key Points

Key Points Adults with SCD have an increased incidence of VTE, but similar data in children are lacking. In this 7-year, multicenter retrospective study, 1.7% of children with SCD developed VTE.

Characteristics of Sickle Cell Disease Children Having Elevated Thrombin Potential

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4762-4762
Author(s):  
Denis F Noubouossie ◽  
Phu-Quoc Le ◽  
Laurence Rozen ◽  
Dominique Willems ◽  
Mahadeb Bhavna ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Cell Disease ◽  
Normal Children ◽  
Clotting System ◽  
Younger Age ◽  
The Mean ◽  
D Dimer ◽  
Age Category

Abstract Abstract 4762 Introduction: Abnormalities of the clotting system are often observed in sickle cell disease (SCD). In addition to activation of coagulation, increased thrombin generation (TG) has recently been demonstrated in SCD children Aim of the study: To characterize the group of SCD children with increased TG in terms of clinical and biological parameters. Material and methods: TG was performed in the platelet-poor plasma of 97 SCD children at steady-state and 80 controls aged between 2 and 20 years. TG was triggered using 1 pM tissue factor and 4μM synthetic phospholipids with thrombomodulin to activate the protein C/protein S anticoagulant pathway. The Endogenous Thrombin Potential (ETP) and the peak height were analyzed. As these parameters increase with age in normal children, controls were distributed in 4 categories of age: [2–5], [6–10], [11–15] and [16–20] years. The mean ETP and the mean peak were calculated for each age category. A ratio for ETP (r ETP) and a ratio for the peak (r Peak) were defined for each control and patient as follows: individual ETP/ mean ETP and individual peak/ mean peak according to age category. Overall mean of r ETP and r Peak was then calculated such as to eliminate bias due to age. This calculated mean was 1.00 (0.39 – 1.61) for r ETP and 0.99 (0.28 – 1.81) for r Peak in controls. TG was considered abnormal if r ETP or r Peak value was above the mean + 2SD of controls (r ETP≥ 1.62 and r Peak≥ 1.82). Clinical and laboratory parameters were compared between SCD children having normal or increased ratios using the Mann Whitney test for numbers or the Fisher's exact test for proportions. P < 0.05 was considered significant. Results and Discussion: Overall 48 (49.4 %) patients showed either high r ETP or high r Peak whereas both parameters were increased in 31 (31.9 %) patients. As shown in Tables I and II, SCD children with elevated ratios were characterized by a younger age, shorter duration of hydroxyurea (HU) treatment, lower total hemoglobin level, higher reticulocyte and monocyte counts, higher LDH and D-dimer levels and a trend to increased procoagulant microparticle level (PMP) as compared with those having normal ratios. The higher D-dimer level in the group with abnormal ratios indicates that these children also manifest a higher degree of coagulation activation than those with normal ratios. Differences observed with markers of hemolysis are consistent with other reports suggesting a link between hypercoagulability and high hemolytic rate in SCD. The borderline significance of PMP is probably due to the use of exogenous phospholipids in TG which reduces the sensitivity of the test to endogenous phospholipids at the surface of PMPs. Conclusion: According to our study, Elevated Thrombin Potential is more frequently encountered in SCD children of younger age, with a shorter duration of HU treatment and with increased rate of hemolysis. These observations together with elevated D-dimer level seem to characterize SCD children with a hypercoagulable state. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Breakthrough: new guidance for silent cerebral ischemia and infarction in sickle cell disease

Hematology ◽  
2014 ◽  
Vol 2014 (1) ◽  
pp. 438-443 ◽  
Author(s):  
Charles T. Quinn
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Normal Children ◽  
Total Burden ◽  
Neurologically Normal ◽  
Cerebral Ischemic ◽  
Pressure Headache ◽  
First Year Of Life ◽  
Ischemic Events

Abstract Silent cerebral infarction (SCI) is a highly prevalent and morbid condition in sickle cell disease (SCD). SCI can occur beginning in the first year of life and becomes more common with increasing age. Potentially modifiable risk factors for SCI include anemia and blood pressure. Headache does not appear to be associated with SCI, so neurologically normal children with headache do not necessarily warrant screening MRI for SCI. SCI does affect cognition, but biological determinants of cognition are not more important than socioeconomic factors. The recent identification of acute silent cerebral ischemic events indicates that the total burden of ischemic injury to the brain in SCD is far greater than previously realized. Acute anemic events appear to increase the risk of acute silent cerebral ischemic events and SCI dramatically. The medical management of SCI is not yet defined, but documentation of the presence of SCI may qualify affected individuals for special resources because comprehensive interventions are needed to optimize patients' academic and vocational outcomes.

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