scholarly journals Physical growth in thalassemic children of 2-12 years with multiple transfusions

2022 ◽  
Vol 4 (4) ◽  
pp. 132-140
Author(s):  
Kalpana Datta ◽  
Subhayan Mukherjee ◽  
Kalyanbrata Mandal ◽  
Asraf Uz Zaman ◽  
Oishik Roy

The hemoglobinopathies are the most common single-gene defect in man. The thalassemia syndromes are a heterogeneous group of hereditary disorder due to decreased synthesis of either α or β globin chain of Hb A. There are 3 phases of growth disturbances and have three different etiologies. First phase: growth disturbances is mainly due to hypoxia, anaemia, ineffective erythropoiesis and nutritional factors; the Second phase: During late childhood, growth retardation is mainly due to iron overload affecting the GH-IGF-1 axis and other endocrinal complications. Third phase: after the age of 10-11 years, delayed or arrested puberty is an important contributory factor to growth failure in adolescents thalassemic who does not show any growth spurt. Cross-sectional, observational, single-centre, tertiary hospital-based study. Children of thalassemia major of 2-12 years with multiple transfusions was taken over 1 year. Study population was divided into 2 groups: Group1-irregularly transfused; Group 2-regularly transfused. Clinical settings, anthropometry, laboratory tests like serum ferritin, pre-transfusion haemoglobin, total leucocyte count etc. were taken into consideration. Thalassemia children with other comorbidities like tuberculosis, chronic kidney disease, chronic heart diseases etc. were excluded from the study.Among the 200 children, 143 (71.5%) were taking regular (2-4 weekly) transfusion therapy and 57 (28.5%) were taking irregular transfusion (>4weekly). Mean age of diagnosis was 18.66 ± 7.443months in Group 1 (Irregularly transfused) and 18.93 ± 7.218 months in Group 2 (Regularly transfused). Among the regularly transfused thalassemic 17.7% children had W/A < 3 percentile and among the irregularly transfused children it was 15%. Among the irregularly transfused children, 27. 1% and among the regularly transfused children 21.6% had H/A <3rd percentile. In the present study children 61% had normal BMI and only 5.4 % had BMI less than 3rd percentile overall. Among irregularly transfused thalassemic children >10years of age, 86.7% have not attained puberty yet. Among the regularly transfused thalassemic children 96.7% have not attained puberty yet. US and LS individually affected resulting in stunting but it was proportionate innature so US: LS ratio was according to age. A positive correlation between pre-transfusion haemoglobin and W/A and H/A suggested that with decreasing pre-transfusion haemoglobin concentration more child had growth retardation. Mean value of serum Ferritin was 941 ± 608.490 ng/ml in Group 1(Irregularly transfused) and Mean value of serum Ferritin was 1403 ± 685.584ng/ml in Group 2(Regularly transfused). MUAC in the present study was 12.44cm suggesting mild-moderate malnutrition. Extremely variable clinical and haematological findings were observed in these patients. Growth retardation has found in both regularly and irregularly transfused patients. These findings are almost comparable to other Indian studies. Appropriate knowledge regarding prenatal counselling, early diagnosis, regular transfusions and overall treatment can help better management of this group of patients.

2021 ◽  
pp. 1-7
Author(s):  
Emre Erdem ◽  
Ahmet Karatas ◽  
Tevfik Ecder

<b><i>Introduction:</i></b> The effect of high serum ferritin levels on long-term mortality in hemodialysis patients is unknown. The relationship between serum ferritin levels and 5-year all-cause mortality in hemodialysis patients was investigated in this study. <b><i>Methods:</i></b> A total of 173 prevalent hemodialysis patients were included in this study. The patients were followed for up to 5 years and divided into 3 groups according to time-averaged serum ferritin levels (group 1: serum ferritin &#x3c;800 ng/mL, group 2: serum ferritin 800–1,500 ng/mL, and group 3: serum ferritin &#x3e;1,500 ng/mL). Along with the serum ferritin levels, other clinical and laboratory variables that may affect mortality were also included in the Cox proportional-hazards regression analysis. <b><i>Results:</i></b> Eighty-one (47%) patients died during the 5-year follow-up period. The median follow-up time was 38 (17.5–60) months. The 5-year survival rates of groups 1, 2, and 3 were 44, 64, and 27%, respectively. In group 3, the survival was lower than in groups 1 and 2 (log-rank test, <i>p</i> = 0.002). In group 1, the mortality was significantly lower than in group 3 (HR [95% CI]: 0.16 [0.05–0.49]; <i>p</i> = 0.001). In group 2, the mortality was also lower than in group 3 (HR [95% CI]: 0.32 [0.12–0.88]; <i>p</i> = 0.026). No significant difference in mortality between groups 1 and 2 was found (HR [95% CI]: 0.49 [0.23–1.04]; <i>p</i> = 0.063). <b><i>Conclusion:</i></b> Time-averaged serum ferritin levels &#x3e;1,500 ng/mL in hemodialysis patients are associated with an increased 5-year all-cause mortality risk.


2013 ◽  
Vol 82 (4) ◽  
pp. 411-414
Author(s):  
Richard Chaloupka ◽  
Milan Dvořák ◽  
František Tichý ◽  
Jiří Veselý ◽  
Alois Nečas

The aim of this experimental study was to assess the spine development in growing rats following pinealectomy or partial sensorimotor cortical area damage. A total of 68 Wistar albino rats (Rattus norvegicus v. alba f. domestica) aged 3–4 weeks were divided into four groups. In group 1 (n = 22) pinealectomy was performed, in group 2 (n = 24) the sensorymotor cortical area 2 × 1 × 1 mm below the coronal suture was removed. Sham operation consisted of a craniotomy (n = 11) and a craniotomy with a durotomy (n = 11). All surgeries were performed from the left side. The rats were killed four months after surgery and radiography was then made. Scoliosis, C2-T7 lordosis and T7-S1 kyphosis were measured.The brains of rats after sensorimotor cortical area removal were isolated and investigated including histological examination (light microscope). Scoliosis of 9–14 degrees (mean value 10.8) was developed in five animals after pinealectomy; in rats after removal of the sensorimotor cortical area scoliosis of 10–24 degrees (mean value 15.9) was observed in eight animals. The scoliotic curves were non structural. Our results indicate the importance of cortical area damage, together with craniotomy and durotomy in the development of growing rat spine. These damages could cause a disorder of balance between smaller inhibitory and greater facilitating area of central nervous system, controlling the muscular tone and resulting in the development of increased lordosis and kyphosis and non structural scoliosis due to muscle imbalance. Thus the new hypothesis of scoliosis aetiology was introduced.


Author(s):  
С.В. Журавель ◽  
Н.К. Кузнецова ◽  
В.Э. Александрова ◽  
П.В. Гаврилов ◽  
А.М. Талызин ◽  
...  

Введение. Терапевтическое использование раствора человеческого альбумина у пациентов в периоперационном периоде трансплантации печени (ТП) представляет интерес в контексте осложнений и исходов операции. Цель исследования: оценить влияние интраоперационной трансфузии 25% раствора человеческого альбумина на течение раннего послеоперационного периода при ортотопической ТП от посмертного донора. Материалы и методы. В исследование включены 47 пациентов, которым была выполнена трупная ТП. Были сформированы 2 группы: пациенты группы 1 (n = 21) получали трансфузию 25% раствора человеческого альбумина в конце операции ТП и через 24 ч после операции; пациенты группы 2 (n = 26) получали трансфузию 25% раствора человеческого альбумина в первые сутки послеоперационного периода. Осуществляли контроль лабораторных параметров крови пациента перед началом оперативного вмешательства, через 24 ч и через 48 ч после операции. Интраоперационно оценивали значения систолического (САД) и диастолического (ДАД) артериального давления, частоту сердечных сокращений (ЧСС), дозу вазопрессоров, объем инфузионно-трансфузионной терапии, кровопотерю и диурез. В послеоперационном периоде фиксировали возможные осложнения, проведенные сеансы заместительной почечной терапии (ЗПТ), а также количество дней в стационаре. Результаты. Оценка значений САД, ДАД и ЧСС в начале и конце операции показала достоверно лучшие показатели гемодинамики и снижение дозировок вазопрессорной поддержки в конце вмешательства в группе 1 по сравнению с группой 2 (p < 0,05). В послеоперационном периоде инфекционные осложнения зарегистрированы у одного пациента группы 1 и у трех пациентов группы 2. Проведение ЗПТ потребовалось двум пациентам из группы 2. Все пациенты обеих групп были выписаны из стационара, при этом число дней госпитализации в группе 2 было статистически значимо больше по сравнению с пациентами группы 1: 26,9 ± 3,9 против 17,2 ± 4,3 (p < 0,05). Заключение. Интраоперационная инфузия 25% раствора альбумина позволяет стабилизировать показатели гемодинамики в конце операции ТП, снизить потребность в кардиотонической поддержке и сократить время госпитализации у пациентов после ТП. Background. The therapeutic use of human albumin solution in patients in the perioperative period of liver transplantation (LT) is of interest in the context of complications and outcomes of surgery. Objectives: to assess the effect of intraoperative transfusion of 25% human albumin solution on the early postoperative period in orthotopic LT from a postmortem donor. Patients/Methods. The study included 47 patients who underwent cadaveric LT. Two groups were formed: patients in group 1 (n = 21) received transfusion of 25% human albumin solution at the end of LT and 24 hours after surgery; patients in group 2 (n = 26) received transfusion of 25% human albumin solution on the first day of the postoperative period. The laboratory parameters of the patient’s blood were monitored before surgery, 24 hours later, and 48 hours after surgery. Intraoperatively, the values of systolic (SBP) and diastolic (DBP) blood pressure, heart rate (HR), dose of vasopressors, volume of infusion- transfusion therapy, blood loss and diuresis were assessed. In the postoperative period, possible complications, renal replacement therapy (RRT) sessions performed, and the number of days in the hospital were recorded. Results. Assessment of SBP, DBP and HR values showed significantly better hemodynamic parameters and reduced dosages of vasopressor support at the end of surgery in group 1 compared to group 2 (p < 0.05). In the postoperative period, infectious complications were registered in one patient of group 1 and in three patients of group 2. Two patients from group 2 required RRT. All patients were discharged from the hospital, while the number of hospitalization days in group 2 was statistically significantly greater than in patients in group 1: 26.9 ± 3.9 versus 17.2 ± 4.3 (p < 0.05). Conclusions. Intraoperative infusion of 25% albumin solution allows stabilizing hemodynamic parameters at the end of LT surgery, reducing the need for cardiotonic support and shortening the hospitalization time in patients after LT.


2022 ◽  
Vol 15 (6) ◽  
pp. 695-704
Author(s):  
E. A. Orudzhova

Aim: to study the role of antiphospholipid antibodies (AРA) and genetic thrombophilia as a potential cause of the development or a component in the pathogenesis of early and late fetal growth retardation (FGR).Materials and Methods. There was conducted a prospective randomized controlled trial with 118 women enrolled. The main group consisted of 83 patients, whose pregnancy was complicated by FGR degrees II and III, stratified into two groups: group 1 – 36 pregnant women with early FGR, group 2 – 47 pregnant women with late FGR. Women were subdivided into subgroups according to the FGR severity. The control group consisted of 35 pregnant women with a physiological course of pregnancy. АРА were determined according to the Sydney antiphospholipid syndrome criteria by enzyme immunoassay (ELISA): against cardiolipin, β2 -glycoprotein 1, annexin V, prothrombin, etc. (IgG/IgM isotypes); lupus anticoagulant – by the three-stage method with Russell's viper venom; antithrombin III and protein C levels – by chromogenic method; prothrombin gene polymorphisms G20210A and factor V Leiden – by polymerase chain reaction; homocysteine level – by ELISA.Results. AРA circulation (medium and high titers), genetic thrombophilic defects and/or hyperhomocysteinemia were detected in 40 (48.2 %) patients with FGR, which was significantly higher than that in the control group (p < 0.05): in group 1 (41.7 % of women) AРA (30.6 %) and AРA with genetic thrombophilia or hyperhomocysteinemia (11.1 %) were revealed; in group 2 (51.1 % of women) AРA (21.3 %), AРA with hyperhomocysteinemia (4.3 %), genetic thrombophilia (25.5 %), and due to hyperhomocysteinemia (2.1 %) were found. No differences in prevalence of thrombophilia rate in patients were observed related to FGR severity, but a correlation between the FGR severity and AРA titers was found.Conclusion. Testing for the presence of AРA, genetic thrombophilia and hyperhomocysteinemia should be recommended for patients with FGR (including those with FGR in medical history), especially in the case of its early onset. It is recommended to determine the full AРA spectrum.


2016 ◽  
Vol 62 (5) ◽  
pp. 62-63
Author(s):  
Tatyana Y. Tselovalnikova ◽  
Alla E. Yudina ◽  
Maria G. Pavlova ◽  
Alexey V. Zilov ◽  
Nadezhda A. Mazerkina ◽  
...  

Background. Endocrine consequences such as growth hormone deficiency (GHD), growth disturbances and metabolic disorders are common in childhood cancer survivors.Aim: to evaluate and compare the prevalence of growth disturbances and metabolic disorders in childhood posterior cranial fossa tumors (cPCFT) and acute lymphoblastic leukemia (cALL) survivors.Materials and methods. 40 subjects (21 men, 19 women) who had undergone treatment for cPCFT (group 1) and 25 subjects (9 men, 16 women) after treatment for cALL (group 2) were assessed. Group 1 underwent surgery, chemotherapy (CT) and craniospinal irradiation in a dose of 34.9 ± 1.6 Gy with a boost to the PCF 51.3 ± 9.2 Gy. Group 2 underwent CT (23 subjects were treated with ALL-BFM-90 protocol; 2 subjects were treated with ALL-MB-2002 protocol). All subjects of the group 2 received cranial irradiation in a dose 12,7±2 Gy. Age at the time of the survey in a group 1 and 2 – 19.8 ± 3.05 and 21.2±3.9 years; age at the time of treatment – 10.9 ± 3.4 and 6.9±3.4 years; follow-up – 7.2 ± 4.2 and 13.8±4.9 years, respectively. 16 age and sex matched healthy controls were included. Patient’s anthropometric and laboratory parameters were measured, GHD was diagnosed in group 1 by 2 tests – insulin tolerance test (ITT) and glucagon stimulation test (GST). In group 2 these tests didn't perform. At the time of the survey no one in both groups received GH replacement therapy. Only 5 subjects (3 boys and 2 girls) in group 1 were treated with recombinant human GH during childhood.Results.Final height SDS in the group 1 was significantly less than in the group 2 (p=0.001) and in healthy controls (p<0.001). In the group 1 and 2 there were significantly less patients reached target height compared to healthy controls (p<0.001). Subjects of group 1 rarely reached their target height in comparison to the group 2 (p=0.006). IGF-1 SDS was significantly less in the group 1 (-1.37±1.24) than in the group 2 (0.5±1.24, p<0.001). In group 1 GHD according to GST was diagnosed in 60% of subjects, according to ITT in 82.1% of subjects. Waist circumference was significantly bigger in group 2 compared to the group 1 (p=0.046) and to healthy controls (p=0.001). Overweight was registered in 10% of patients in group 1 and in 16% - in group 2. Dyslipidemia was diagnosed in 50% in group 1, in 19% in group 2 (p=0.226). In group 1 16.7% and in group 2 66.7% of subjects were insulin resistant.Conclusions. After treatment for cPCFT growth disturbances occurred more often than after cALL therapy. Metabolic disorders were diagnosed with different frequency in both cPCFT and cALL survivors. These patients need endocrinologist’ observation.


Author(s):  
Abhishek Salwan ◽  
Shakeen Singh

Background: Cystoid macular edema (CME) is the formation of fluid-filled cystoid spaces between the outer plexiform and inner nuclear layers of the retina. It may present as a complication of routine cataract surgery including phacoemulsification (PHACO) and small incision cataract surgery (SICS).Methods: An observational study of uncomplicated cataract surgery with assessment by ophthalmoscopy, slit Lamp, Snellen chart and OCT during pre- and post-operative period. 100 patients were selected by inclusion criteria and using convenient sampling technique and were divided into two groups of 50 each in PHACO and SICS groups.Results: During preoperative observation in SICS/group-1, mean value and SD of macular thickness was 223.38±12.61 and in PHACO/group-2 it was 224.14±12.69. Mean value, mean difference and p value of visual acuity in group 1 was 0.258, during 12 weeks 0.788 with mean difference -0.53 and p=0.000 and in group 2 it was 0.269, during 12 weeks 0.844 with mean difference -0.58 and p=0.000. Mean value, SD, mean difference and p value of macular thickness in SICS during 1 week was 238.28±12.29, during 12 weeks 227.04±12.58 with mean difference 11.24000 and p=0.000 and in PHACO mean value, SD, mean difference with p value during 1 week was 231.90±12.42, during 12 weeks was 225.02±11.74 with mean difference 6.88000 and p=0.000.Conclusions: A subclinical increase in post-operative macular thickness was recorded which returned nearly to baseline values during the 12 weeks follow up and did not affect visual outcome.


2021 ◽  
Vol 10 (1) ◽  
pp. 33-38
Author(s):  
Rajeev Raj Manandhar ◽  
Krishna Raj Khanal ◽  
Himal Khanal ◽  
Saroj Gautam

Background: The pain and limitation of shoulder function can disrupt daily activities of patients for months to years. Adhesive capsulitis is considered a self-limiting disease but the duration remains uncertain. The brunt of the disease is focussed on the inflamed joint capsule. On this basis, use of corticosteroid injection is justified. However, injection method is not conclusive. Objectives: To compare clinical benefits of intra-articular injection alone versus combined intra-articular and subacromial injections in management of adhesive capsulitis. Methods: Fifty-nine patients with diagnostic criteria for adhesive capsulitis were included in the study from March 2019 to September 2020. Patients were divided into two groups; patients who underwent intra-articular (IA) injection alone (Group 1) and those who received both intra-articular and sub-acromial (IA+SA) injection (Group 2). The injections were landmark guided. Patients were followed up at three, six, and 12 weeks. Pain was recorded using visual analogue scale (VAS) and subjective function using Constant-Murley score. Results: Twenty-eight patients were included in Group 1 (IA) and 31 in Group 2 (IA+SA). Thirty-six patients were female (18 each in Group 1 and Group 2) and 23 patients were male (Group 1 = 13; Group 2 = 10). In the twelfth week, VAS score was reduced in both the groups. On comparing the mean value of Constant-Murley score between the two groups there is significant difference in value recorded at the sixth and twelfth week. Conclusion: The IA+SA injection provides significant reduction in pain and better function in the short term over the IA injection.


Author(s):  
Bhupender Yadav ◽  
Puja Malhotra ◽  
Harish Yadav ◽  
Sumit Singh Phukela

ABSTRACT Retention in mandibular dentures has always been a challenge for the treating dentist. Denture adhesives are known to improve the adhesive bond between the denture and the underlying tissues. However, denture adhesives still remain unexplored and not much is known about the efficacy of these materials in mandibular dentures especially in patients with poor foundations. The present study was carried out to compare the efficacy of different commercially available denture adhesives in relation to their retentive ability in patients with good well formed mandibular ridges and in patients with resorbed and flat mandibular ridges. Seventy edentulous patients, age range between 50 to 70 years, were selected. The patients were divided into 2 groups; group 1 comprised of 35 patients with good well formed mandibular ridges and group 2 consisted of 35 patients having low well rounded mandibular ridges. The adhesion and cohesion that developed between the dentures and the underlying tissues when the various materials were interposed between them was evaluated with the help of a test apparatus in newtons. Mean value of retention in denture without adhesive was 9.93 N and 24.53 N in resorbed and well formed ridges respectively. Use of denture adhesive materials led to a higher value of retention as opposed to when the dentures were used without adhesives. This was true for patients with good well formed mandibular ridges as well as for patients with poor foundations. Among the various denture adhesive powders tested, Fittydent showed better results, i.e. 29.56 and 33.05 in resorbed and well-formed ridges respectively as opposed to fixon which showed values of 16.47 and 27.70 respectively. How to cite this article Malhotra P, Yadav B, Yadav H, Phukela SS. A Clinical Study to assess the Efficacy of Commonly Available Denture Adhesive Materials in Mandibular Dentures. Int J Prosthodont Restor Dent 2013;3(4):125-130.


Author(s):  
Mona Sohrabi ◽  
Sara Ghadimi ◽  
Bahman Seraj

Objectives: This study aimed to assess the microleakage of Pedo Jacket crowns compared to stainless steel crowns (SSCs) cemented with different luting cements. Materials and Methods: In this in-vitro experimental study, 80 primary molars were randomly divided into four groups of 20 each. Groups 1 and 2 were subjected to standard tooth preparation for SSC. Crowns in group 1 were cemented with glass ionomer (GI), and crowns in group 2 were cemented with a resin-modified glass ionomer (RMGI) cement. In groups 3 and 4, minimal tooth preparation was performed for Pedo Jacket crowns, and the crowns were cemented with RMGI and Panavia resin cement, respectively. Microleakage was measured at mesial and distal surfaces in micrometers (µm), and the mean value for each tooth was calculated. One-way analysis of variance (ANOVA) was applied to compare the microleakage of the four groups. Results: Group 3 (Pedo Jacket cemented with RMGI) showed the highest microleakage (1523.83±250.32 µm) with significant differences with the remaining three groups (P<0.001). Microleakage in group 4 (Pedo Jacket cemented with Panavia) was significantly lower than that in the other three groups (301.25±219.53 µm, P<0.001). Groups 1 (SSCs cemented with GI) and 2 (SSCs cemented with RMGI) were not significantly different in terms of microleakage (P=0.49) although group 1 showed slightly higher microleakage than group 2 (598.43±260.85 µm versus 500.25±124.74 µm). Conclusions: Pedo Jacket crowns can serve as an acceptable esthetic alternative to SSCs if cemented with resin cements.  


Genes ◽  
2022 ◽  
Vol 13 (1) ◽  
pp. 118
Author(s):  
Luis Alfredo Utria Acevedo ◽  
Aline Morgan Alvarenga ◽  
Paula Fernanda Silva Fonseca ◽  
Nathália Kozikas da Silva ◽  
Rodolfo Delfini Cançado ◽  
...  

Background: Hemochromatosis is a genetic condition of iron overload caused by deficiency of hepcidin. In a previous stage of this study, patients with suspected hemochromatosis had their quality of life (QL) measured. We observed that QL scores differed among genotypic groups of patients. In this reported final phase of the study, the aims were to compare QL scores after a treatment period of approximately 3 years and to analyze a possible association of the serum ferritin values with QL scores. Methods: Sixty-five patients were enrolled in this final phase and divided into group 1 (patients that showed primary iron overload and homozygous genotype for the HFE p.Cys282Tyr mutation) and group 2 (other kinds of genotypes). Short Form 36 (SF-36) was performed and consisted of eight domains with a physical and also a mental component. Results: Both groups had a significant decrease in serum ferritin concentrations: group 1 had a variation from 1844 ± 1313 ng/mL to 281 ± 294 ng/mL, and group 2 had a variation from 1216 ± 631 ng/mL to 236 ± 174 ng/mL. Group 1 had a smaller mean value for these six SF-36 domains compared with group 2, indicating a worse QL. Conclusions: In this final stage, six domains demonstrated a difference among genotypic groups (role emotional and mental health, adding to the four of the initial phase), reassuring the impact of the identified genotype on the QL of hemochromatosis patients. Furthermore, despite that both patient groups demonstrated similar and significant decreases in serum ferritin values, no association was found between the decrease in this biological parameter and the SF-36 domains.


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