scholarly journals Growth and puberty in a prospective cohort of patients with sickle-cell anaemia: an assessment over ten years abstract

2017 ◽  
Vol 27 (1) ◽  
pp. 91 ◽  
Author(s):  
Ingrid Cristiane Pereira Gomes ◽  
Hugo Nivaldo Melo ◽  
Suyaluane Italla Amana Melo ◽  
Nelmo Vasconcelos de Menezes ◽  
Tulio Vinicius Paes Dantas ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Pubertal Development ◽  
Hereditary Diseases ◽  
Control Group ◽  
Age At Menarche ◽  
Prospective Longitudinal Study ◽  
Cellular Expression ◽  
Z Scores ◽  
Prospective Longitudinal

Introduction: Hereditary haemoglobinopathies are the most common group of monogenic hereditary diseases in the world. Erythrocytes in sickle form, cellular expression of polymerization of deoxygenated HbS, cause intermittent vascular obstruction, leading to tissue ischaemia and consequent chronic damage in organs and endocrine glands. Objective: The evaluation of the growth pattern and pubertal development of a group of patients with sickle-cell anaemia (SCA) from childhood to adulthood. Method: Thirty patients with SCA between the ages of 10 and 23 years were evaluated in a prospective longitudinal study at three points in time (Te1: 2005; Te2: 2010 and Te3: 2015) and compared with controls. Anthropometric, pubertal and hormonal evaluations were carried out. Age- and gender-specific Z-scores for weight, height and BMI (body mass index) were calculated according to the reference growth standards. Results: Thirty patients with SCA (mean age = 13.93 years) were evaluated at Te1 and 26 patients (mean age = 25.08 years) at Te3. The SCA group lower showed Z-scores for weight (p = 0.0002), height (p = 0.0184) and BMI (p = 0.0011) than the control group at Te1. At Te3, there was no difference in height, but weight (p = <0.0001) and BMI (p = <0.0001) were lower in the SCA group. Men showed greater weight commitment than women at the three study times (Te1: p = 0.0340, Te2: p = 0.0426 and Te3: p = 0.0387) and lower BMI in Te3 (p = 0.0155) in the SCA group. There was a significant increase in weight when comparing Te1 with Te3 (p = 0.0009) and in height when comparing Te1 with Te2 (p = 0.0292) and with Te3 (p = 0.0003) in the SCA group. There was a significant increase in weight when comparing Te1 and Te3 (p = 0.0009) and in height when comparing Te1 and Te2 (p = 0.0292) and Te3 (p = 0.0003) in the SCA group. At Te1, 14 cases and 2 controls were prepubertal. Bone age was delayed in 12 patients. Age at menarche was delayed and lower in the SCA group (mean = 15 years). Five patients had gestated, but no patient had experienced fatherhood. At Te1, TSH levels were higher (p = 0.0080) and T3 levels were lower (p = 0.0020) in the SCA group. At Te3, LH and FSH levels were higher in men with SCA (p = 0.0014; p; 0.0002). IGF-I levels were lower in cases both at Te1 (p = 0.0002) and at Te3 (p = 0.0032). Conclusions: Patients with SCA showed growth impairment and pubertal delay compared with healthy controls. However, albeit belatedly, they reached normal sexual maturation and height in adulthood. Women with SCA showed no fertility problems. The findings highlight the need to investigate the intention of paternity and fertility among men with SCA.

The effect of sickle cell anemia on the linear growth of Nigerian children

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Ugo Nnenna Chikani ◽  
Adaobi Bisi-Onyemaechi ◽  
Ijeoma Ohuche ◽  
Justus Onu ◽  
Shalewa Ugege ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Linear Growth ◽  
Clinical Care ◽  
Growth Failure ◽  
Age At Diagnosis ◽  
Height Velocity ◽  
Prospective Longitudinal Study ◽  
High Prevalence ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Objectives Despite the high prevalence of children with sickle cell anaemia (SCA) in West Africa, there is paucity of data on the height velocity and prevalence of growth failure in SCA patients. With advances in clinical care of SCA patients, could there be a spatial and secular trend in the growth pattern of these children? Hence, the compelling needs to embark on this study. The objectives of the study were to determine the prevalence of growth failure among patients with SCA and its correlation with age, gender and age at diagnosis. Methods A Prospective longitudinal study of a cohort of sickle cell anaemic paediatric patients from Pediatrics SCA Clinic, University of Nigeria Teaching Hospital, Ituku Ozalla. Patients were enrolled over a period of two years using a non-parametric convenient sampling method. Their heights were measured at baseline, three months, six months and at 12 months intervals and subsequently plotted on a standard WHO growth chart. The height velocities at different monthly intervals were calculated and compared with the WHO standard normal linear growth rates) for children (used as control) to identify those with GF. (i.e. <10th percentile). The main outcome measures were the mean height velocities at different months' intervals calculated and compared using the repeated measurement analysis of variance (ANOVA) and the Wilcoxon signed test. Results A cohort of 316 children aged 1–18 years with SCA was evaluated with a male preponderance of 161 (57.4%). The mean age and age at diagnosis were 11.04 ± 5.56 and 4.2 ± 1.7 years, respectively. The prevalence of growth failure and short stature was 84.7%. The burden of GF was highest among post-pubertal participants (94.1%). The most important predictor of growth velocity deficit was age (R2=0.045, standard β coefficient = −0.22, t=−03.51, p=0.001). Conclusions The study demonstrated high prevalence of growth failure in children and adolescents with SCA which intensified with advancement in age and older age at diagnosis.

scholarly journals Impact of early extraction of the deciduous canine on relief of severe crowding:

2021 ◽  
Author(s):  
Mhanna A. Aljabab ◽  
Muteb Algharbi ◽  
Jan Huggare ◽  
Farhan Bazargani
Keyword(s):  
Orthodontic Treatment ◽  
Treatment Time ◽  
Permanent Teeth ◽  
Control Group ◽  
Fisher Exact Test ◽  
Prospective Longitudinal Study ◽  
Treatment Need ◽  
Exact Test ◽  
Number Of Visits ◽  
Prospective Longitudinal

ABSTRACT Objectives To explore whether there were any differences in orthodontic treatment need, treatment complexity, treatment time, or the number of visits between a group of children receiving early intervention (extraction of upper and lower deciduous canines) and an age- and condition-matched control group without intervention. Materials and Methods Patient records and study casts in the late mixed or early permanent dentitions of 46 subjects (20 from the extraction group and 26 from the control group) of an earlier prospective longitudinal study were retrieved. Orthodontic treatment need and complexity were assessed by the index of complexity, outcome, and need (ICON). Statistical calculations were performed by t-test for parametric outcome variables (treatment time, number of visits, and orthodontic treatment need) and Fisher exact test for the categorical variable (tooth extractions). Results There were no statistically significant differences between the groups in ICON scores of orthodontic treatment need (extraction group, mean score 59.8; control group, mean score 52.8), number of visits (mean of about 15 visits for both groups), or treatment time (extraction group, mean 21.5 months; control group, mean 20.3 months). The extraction of permanent teeth was more prevalent in the deciduous canine extraction group (59%) as compared with the control group (28%); however, this was not statistically significant (P = .07) but showed a tendency toward worsening the crowding and the future need of orthodontic extractions. Conclusions Early removal of deciduous primary canines will reduce neither the need for later orthodontic treatment nor its complexity, nor will it shorten the treatment time.

scholarly journals Evaluation of a relationship between malocclusion and idiopathic scoliosis in children and adolescents

2019 ◽  
Vol 13 (6) ◽  
pp. 600-606
Author(s):  
M. Laskowska ◽  
D. Olczak-Kowalczyk ◽  
M. Zadurska ◽  
J. Czubak ◽  
M. Czubak-Wrzosek ◽  
...  
Keyword(s):  
Idiopathic Scoliosis ◽  
Cobb Angle ◽  
Clinical Symptoms ◽  
Open Bite ◽  
Control Group ◽  
Level Of Evidence ◽  
Prospective Longitudinal Study ◽  
Stomatognathic System ◽  
Main Thoracic ◽  
Prospective Longitudinal

Purpose Idiopathic scoliosis is a developmental deformation of the vertebral column of an unknown aetiology. Its clinical symptoms and hypothetical causative factors may affect the stomatognathic system. The aim of this study was to analyse the relationships between the prevalence and type of malocclusions, and the presence of idiopathic scoliosis, its location and severity. Methods This was a prospective longitudinal study. The study group consisted of 80 patients with idiopathic scoliosis and the control group of 61 healthy individuals. Standard standing long-cassette radiographs were taken of all of the patients in the idiopathic scoliosis group in order to confirm diagnosis, to determine localization and the Cobb angle of the curve. Both groups underwent standard clinical dental examination. Results The most commonly observed types included right main thoracic (R-MT) and thoracolumbar or left lumbar scoliosis (Cobb angle 11° to 125°). In the idiopathic scoliosis group, prevalence of malocclusions was greater than in the control group (95% versus 82%). In the idiopathic scoliosis group more than one type of malocclusion was observed with a higher incidence than that in the control group (63.8% versus 37.7%; p = 0.002). A correlation between the left proximal thoracic (L-PT) curve with anterior partial open bite was demonstrated (p = 0.323), between thoracic dextroscoliosis main thoracic with lateral partial cross bite (p = 0.230) and a correlation between scoliosis severity and malocclusion in the event of L-PT and anterior partial open bite (p = 0.330) and R-MT and scissors bite (p = 0.248). Conclusion The incidence of malocclusions is greater in children with idiopathic scoliosis than in their healthy peers Level of Evidence III

Tinzaparine in the Management of Painful Vaso-Occlusive Crisis of Sickle Cell Anaemia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2340-2340 ◽  
Author(s):  
Mohamad H. Qari ◽  
Shaker Mousa ◽  
Mohamad A. Alsaigh ◽  
Panagiotis Zografos ◽  
Suad K. Aljaouni ◽  
...  
Keyword(s):  
Pain Intensity ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Prospective Trial ◽  
Control Group ◽  
Study Group ◽  
Minor Bleeding ◽  
Efficacy And Safety ◽  
Hospitalization Period ◽  
Painful Crisis

Abstract Introduction Several components of the haemostatic system are altered in sickle cell anaemia(SCA). The sum of these alterations is manifested as a state of hypercoagulability. Many therapeutic approaches including anticoagulants has been tried in managing the acute painful crisis of SCA along with the analgesics. Aim This trial aimed at assessing the efficacy and safety of low molecular weight heparin Tinzaparine (innohep®) as an adjuvant therapy in the management of acute painful vaso-occlusive crisis. Material and Methods This two arm pilot, randomised multi-centre controlled prospective trial has incorporated 253 patients admitted through emergency in acute painful crisis, with no other complications of SCA. Subjects were consecutively randomized to the study or control group. In the study group 127 patients (mean age 22,83 years) received tinzaparine 175 i.u, s.c once daily along with analgesics, while in the control group 126 patients (mean age 21,64 years) received placebo and the same analgesics. Subjects were followed for improvement using the numerical pain scale (NMS), assessing the number of days in which the patient experienced the severest degree of pain on the NMS, the hospitalization period, the duration of painful crisis, and the rate at which the pain intensity declined over time. The maximal treatment period was seven days. The complications encountered are treated and estimated in both groups. The results were analysed using “SSPS-V10” statistical tests Results The results displayed in the table.1 showed statistically significant reduction in the duration of morbidity in the study group. The drop in the pain intensity is sharper, and the complications experienced in the study group were two events of minor bleeding treated conservatively. Patient Control No. of days with severest pain score on the NMS (Days) 1,28 1,74 Duration of painful crisis(Days) 2,57 4,35 Total duration of hospitalization(Days) 7.08 12,06 Conclusion The results indicate a favourable outcome in the study group compared to the control group. The use of tinzaparine in the acute painful crisis of SCA as an adjuvant to the strong analgesics is recommended due to its proven efficacy and safety in shortening the period of the agonizing pain at presentation, the period of painful crisis and the hospital stay.

Estimation of Antiphospholipid Antibodies in a Prospective Longitudinal Study of Children with Migraine

Cephalalgia ◽  
2004 ◽  
Vol 24 (10) ◽  
pp. 831-837 ◽  
Author(s):  
T Avcin ◽  
G Markelj ◽  
V Niksic ◽  
Z Rener-Primec ◽  
S Cucnik ◽  
...  
Keyword(s):  
Antiphospholipid Antibodies ◽  
Tension Type Headache ◽  
Control Group ◽  
Healthy Children ◽  
Prospective Longitudinal Study ◽  
Glycoprotein I ◽  
Patient Groups ◽  
Unselected Group ◽  
Type Headache ◽  
Prospective Longitudinal

The aim of this study was to determine the prevalence and clinical significance of antiphospholipid antibodies (aPL) in children with migraine. The values of anti-cardiolipin (aCL) and antiβ2 glycoprotein I (antiβ2GPI) antibodies were assayed by an ELISA method in 52 children with migraine and 22 children with tension-type headache. The control group consisted of 61 apparently healthy children at regular preventive visits. Two monoclonal β2GPI dependent aCL (HCAL and EY2C9) were used as calibrators. Lupus anticoagulant (LA) was determined by a modified dilute Russell viper venom time test. Persistently positive aPL were observed during the follow-up in 16.3% of children with migraine (9.3% for aCL, 7.0% for antiβ2GPI and 0% for LA) and in 16.7% of children with tension-type headache (11.1% for aCL, 5.6% for antiβ2GPI and 0% for LA). The prevalence of aPL did not differ significantly between patient groups and healthy children. The prevalence of aPL does not appear to be increased in an unselected group of children with migraine, however, the possible role of aPL in individual cases of paediatric migraine can not be excluded.

scholarly journals Blood group and Human Leucocyte Antigen sub-type as determinants to keloid formation and Recurrence in Keloid Patients; A prospective longitudinal cohort study

2021 ◽  
Author(s):  
Ferdinand Nangole ◽  
Kelsey Ouyang ◽  
George Agak ◽  
Julius Ogeng'o ◽  
Anzala Omu
Keyword(s):  
Blood Group ◽  
Genetic Factors ◽  
Control Group ◽  
Prospective Longitudinal Study ◽  
Female Ratio ◽  
Blood Group A ◽  
Significant Difference ◽  
Group A ◽  
Surgical Conditions ◽  
Prospective Longitudinal

Abstract The role of genetic factors in keloid is a firmed by the fact that keloids have been shown to occur among members of the same family.. We undertook this study to determine whether there is any association between patients’ bloodgroup and HLA sub-types to keloids and keloid recurrence. This was a prospective longitudinal study of patients with keloids and a control of patients managed for other surgical conditions with no keloids. Blood was taken from each patient and analysed for blood group and HLA sub-types using the sequence specific primer geno-typing. Data captured were summarized and analysed using students T-test to compare means. Probability values significance was at 0.05. A total of 90 patients with keloids and 59 in a control group were followed up in the study. The male to female ratio of the patients was 2:1. The most common blood group for both groups was blood group O at 51.3% and 49.2%, followed by blood group A and B respectively. Patients with keloids had a significantly higher positive alleles with DQA*01, DQB1*05, DQB1*06 and DRB1*15. There was an association between blood group A and DQB1*06 and recurrence. In conclusion, this study demonstrates that there is significant difference in HLA sub-types among patients who form keloids and the non-keloid forming patients among our study cohorts. Salient differences were also noted in patients with keloid recurrence based on their blood group, a pointer to the significance of genetic factors in keloid pathogenesis and severity.

scholarly journals Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

2021 ◽  
pp. 53-64
Author(s):  
Barinaaziga S. Mbeera ◽  
Susanna O. Akwuebu ◽  
A. C. U. Ezimah ◽  
Nancy C. Ibeh ◽  
Evelyn M. Eze
Keyword(s):  
Steady State ◽  
Sample Size ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Sickle Cell Anaemia ◽  
Control Group ◽  
Port Harcourt ◽  
The Mean ◽  
The University ◽  
D Dimer

Aim: The aim of this study was to assess some haemostatic parameters in sickle cell anaemia subjects in Rivers and Bayelsa States. Study Design: This study is a cross-sectional observational study. Place and Duration of Study: This study was carried out at the University of Port Harcourt Teaching Hospital, Rivers State, and the Federal Medical Centre, Yenagoa, Bayelsa State, between the months of February and August, 2020.  Methodology: A total of four hundred and fifty (450) subjects with age range of 1-50 years were randomly selected. There were about 200 registered patients (adults and children alike) at the sickle cell clinics of the University of Port Harcourt Teaching Hospital, and the Federal Medical Centre, Yenagoa, with an average of 4 new patients per month. The sample size was obtained using a prevalence of sickle cell anaemia of 2% and the sample size was calculated using Cochran sample size formula. Five milliliters (5ml) of venous blood sample was withdrawn from the peripheral vein in the upper limb of subjects using a standard venipuncture technique. The sample was rocked gently to mix and kept at room temperature and the haemostatic parameters (vWF, FVIII, D-dimer, L-arginine, fibrinogen, ADAMTS13) were assayed quantitatively with Bioassay Technozym kit using Microplate Reader (Labtech microplate auto ELISA plate reader, an IS0 13485:2003 CE and WHO compliance Co., Ltd. Shanghai International Holding Corp. GmbH; Europe) calibrated to a wavelength of 450 nm with strict adherence to the manufacturer's instructions, while PT and APTT were analysed with Fortress reagent and Uniscope SM801A Laboratory using water bath.Data management and statistical analyses were conducted using Statistical Analyses System SAS 9.4 (SAS Institute, Cary, North Carolina, USA) and p values less than .05were considered statistically significant. Results: The results showed the mean comparison of haemostatic parameters in sickle cell anaemia and control subjects. The comparison of haemostatic parameters showed significant(p<.05) increasesand decreases inVaso-Occlusive Crisis (VOC) and steady state respectively compared with the control group. There was statistically significantreduction in the mean comparison of L-Arginine (p<.01) in VOC) condition than steady state in relation to the control group in our study population, while D-Dimer, ADAMTS13 were also significantly reduced statistically (p<.01) in VOC condition than steady state compared with the control group. However, the mean FVIII inhibitor, Fibrinogen, PT (INR) and APTT were significantly higher (p<.01) in VOC than steady state when compared to controls with normal haemoglobin (HbAA).Correlations of haemostaticparameters by sickle cell anaemia subjects’ condition showed more significant positive correlations in VOC than steady state. Conclusion: This study showed a heightened hypercoagulability in Sickle Cell Snaemia(SCA)subjects, and further pave way for better understanding particularly the diagnostic variables underlying SCA, specific to each subject condition (steady state and VOC). Subjects with SCA, particularly during VOC, undergo significant haemostatic alterations that increase their risk of developing coagulation activation-related complications. Thus, though selected markers of coagulation were significantly different between the subject conditions, they were often significantly higher in the SCA.

scholarly journals Temperament in preschool children with sickle cell anaemia

2018 ◽  
Vol 105 (9) ◽  
pp. 900-902
Author(s):  
Michelle Downes ◽  
Michelle de Haan ◽  
Tess O’Leary ◽  
Paul T Telfer ◽  
Fenella J Kirkham
Keyword(s):  
Preschool Children ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Hospital Admissions ◽  
Negative Affectivity ◽  
Parent Report ◽  
Future Research ◽  
Control Group ◽  
Significant Difference ◽  
Children’S Behaviour

AimsFew studies have investigated the potential impact of sickle cell anaemia (SCA) on temperament. The aim of the current study was to investigate temperament in preschool children with SCA and to establish the reliability of the Children’s Behaviour Questionnaire (CBQ) in this population.MethodsThe CBQ, a parent-report measure of temperament, was completed by parents of 21 preschool children with SCA and a control group of parents of typically developing children, matched for age, ethnicity and socioeconomic status.ResultsA significant difference between groups was identified for the dimension of negative affectivity only, with specific differences observed in the discomfort subdomain. Patients with a greater number of hospital admissions in the previous year were reported to have higher levels of discomfort.ConclusionsPreschool children with SCA are reported to have higher rates of negative affectivity, particularly discomfort. Future research is required to investigate the potential influence of dysregulated negative emotions and discomfort on disease management and quality of life throughout childhood.

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