Introduction
Effective physiotherapy interventions are required for haemophilic arthropathy (HA) of the elbow due to its biomechanical differences and contribution to upper limb functionality.
Aim
To ...investigate the effects of manual therapy & exercises on bleeding frequency, pain, range of motion (ROM), strength, joint health, functionality and quality of life (QoL) in HA of the elbow.
Methods
Seventeen participants with HA of the elbow were randomized as Manual Therapy & Exercises Group (MTEG = 9) and Home Exercises Group (HEG = 8). Soft tissue mobilization, joint traction (grade I‐II) and Mulligan's mobilization with movement as manual therapy, and stretching/strengthening exercises were applied to MTEG, while HEG had only same exercises as home programme. The interventions lasted 3 days/weekly for 5 weeks. Bleeding frequency was evaluated with patients’ records; pain with Numeric Pain Scale; ROM with goniometer; strength with digital dynamometer; joint health with HJHS; functionality with Quick‐Disability of Arm, Shoulder and Hand questionnaire; and QoL with Oxford Elbow Score.
Results
Bleeding frequency and activity pain were decreased, while elbow ROMs and flexor strength were increased in MTEG (P ˂ 0.05). Also significantly improvements were seen in joint health, functionality and QoL in MTEG. HEG showed improvements in activity pain, QoL and some ROMs. MTEG had better results in ROMs, joint health and functionality (P ˂ 0.05).
Conclusion
Manual therapy & exercises may be used without causing bleeding and pain to increase the functionality, joint health and QoL, and is superior to home exercise for joint health and functionality. Home exercises may be ameliorated in pain, QoL and some ROMs.
Introduction
The effects of manual therapy (MT) on joint health and functional level in haemophilic arthropathy (HA) have relatively under‐explored.
Aim
To investigate the effects of MT in HA of ...lower limb joints on musculoskeletal system, functional level, Functional Independence Score in Hemophilia (FISH), Hemophilia Joint Health Score (HJHS) and kinesiophobia.
Methods
Seventeen patients were randomized to the control group (CG = 9) and manual therapy group (MTG = 8). The number of haemarthrosis (NoH) was recorded from the diaries. Range of motion (ROM), strength and pain intensity were measured using goniometer, digital dynamometer and visual analogue scale, respectively. Joint health was evaluated with HJHS, functional independence with FISH, and kinesiophobia with Tampa Scale. Functional Reach (FR), Timed Up‐Go (TUG) and 5‐Times Sit‐to‐Stand (5*STS) tests were used to evaluate functional level. Home exercises were given to both groups. Additionally, myofascial release techniques and mobilizations were applied to MTG. Interventions were applied 3 d/weekly for 5 weeks. Assessments were performed at baseline and after treatment.
Results
ROM, strength, activity pain, HJHS and FISH were improved in MTG (p˂0.05). Ankle NoH, 5*STS and FR were developed in both groups (p˂0.05), but kinesiophobia, TUG and knee NoH were not significant. Although improvements were observed in favour of MTG in HJHS, FR, activity pain and ankle strength/ROM (p˂0.05), no significant difference was found in FISH, kinesiophobia, TUG, 5*STS, resting pain and knee strength.
Conclusion
Reduction in ankle NoH indicated that both interventions were safe. The use of MT in HA of lower limb joints was an effective physiotherapy approach to improve functional level, joint health and functional independence.
Introduction
Episodes of bleeding in patients with haemophilia (PwH) are associated with haemophilic arthropathy, limitations in physical performance, reduced quality of life (QoL), and gait ...disorders.
Aim
This non‐randomized, controlled, interventional, prospective, single‐centre pilot study aimed to assess the effects of an 8‐week supervised therapeutic exercise program on musculoskeletal health, gait kinematic parameters (GKP), functional capacity, and QoL in adult PwH.
Methods
Nineteen PwH were allocated to an exercise group (n = 10) or a control group (n = 9). The patients in the exercise group followed an 8‐week supervised therapeutic exercise program. The Haemophilia Joint Health Score (HJHS), a two‐dimensional video‐based gait kinematic analysis (2D‐GKA), the 6‐min walking test (6MWT), and the Haemophilia Quality of Life Questionnaire for Adults (Haem‐A‐Qol) were used as the outcome measures at baseline, after the exercise program (at the 8th week), and at the 6th‐month follow‐up.
Results
A significant improvement was observed in the exercise group in the HJHS‐Total and Haem‐A‐Qol Total scores and the 6MWT value after the exercise program. Moreover, the 2D‐GKA revealed improvement in most of the GKP (knee extension during the midstance and late swing phases, ankle dorsiflexion during the midstance phase, and ankle plantar flexion during the preswing phase). However, the gain obtained by the exercise program was not maintained at the 6th‐month follow‐up for the HJHS‐Total and Hem‐A‐QoL‐Total scores and GKP.
Conclusion
The 8‐week supervised therapeutic exercise program was successful in achieving improvement in joint health, GKP, functional capacity, and QoL in PwH.
In children with high‐risk childhood acute leukemia who undergo allogeneic hematopoietic stem‐cell transplantation (allo‐HSCT), relapse is still the leading cause of treatment failure. The prognosis ...is poor, yet prospective studies have only limited data on risk factors and outcomes. We aimed to understand the outcomes and prognostic factors for patients with acute lymphoblastic leukemia (ALL) who relapsed following allo‐HSCT. We analyzed retrospectively 46 children with childhood acute lymphoblastic leukemia who had relapsed after receiving their first alloHSCT. All these patients received salvage chemotherapy which consisted of fludarabine, cytarabine, and idarubicin before performing a second alloHSCT. The median follow‐up of the 46 patients after the first transplantation was 366 days. The median time from first allo‐HSCT to relapse was 278.4 ± 238.4 days. Forty‐six patients received salvage chemotherapy before the second alloHSCT, and CR was achieved in 32 of 46 patients. However, only 17 (37%) of 46 patients received a second allo‐HSCT, and 15 of 46 patients died from disease progression, infections, and bleeding. Twelve patients are still alive after the second allo‐HSCT. Two‐year overall survival (OS) was 38.9%. Local therapy was given to 10 (21.8%) patients, either as part of systemic therapy or alone. In multivariate analyses, the time of relapse and curative salvage therapy with a second allo‐HSCT were identified as significant prognostic factors for OS. Children with leukemia who had relapsed after the first allo‐HSCT received salvage chemotherapy. Our statistical analysis showed that the second HSCT could be beneficial for outcomes if patients relapsed beyond 180 days of the first allo‐HSCT.
Oral mucositis is one of the side effects developed post-hematopoietic stem cell transplant. This retrospective study aimed to assess the efficacy of a mouthwash mixture (lidocaine, sodium alginate, ...sucralfate, pheniramine) versus hyaluronic acid and a solution of sodium bicarbonate in terms of healing time and weight gain in the treatment of oral mucositis in pediatric patients undergoing allogeneic hematopoietic stem cell transplantation with hemato-oncological malignancies.
A total of 171 patients that received chemotherapy for the hematopoietic stem cell transplant were divided into three groups; group 1, treated with a mixed mouthwash of lidocaine, sodium alginate, sucralfate, and pheniramine; group 2, treated with hyaluronic acid; and group 3, treated with an aqueous solution of 5% sodium bicarbonate. Weight and mucositis scale scores derived from medical records of patients.
There was a statistically significant difference in the mucositis scale scores between the groups on the transplant day and days 5, 10, 15 and 20 after the transplantation. At these measurement points, Group 2 (receiving hyaluronic acid) had a lower score, and Group 3 (who received sodium bicarbonate) had a higher score, especially on days 5 and 10 after the transplantation.
The results suggest that hyaluronic acid is a more effective treatment option than the other oral care solutions that are frequently used for prophylaxis and treatment of oral mucositis.
Fanconi aplastic anaemia is a rare genetic disease which causes bone marrow failure. As a multisystem disease affecting almost every organ system, it demonstrates a wide spectrum of clinical ...manifestations. Renal malformations which are leading to end stage renal disease can be one of the major causes of morbidity and mortaility in these patients. So it is very important to investigate these children for genitourinary tract anomalies.Eleven patients with fanconi aplastic anaemia with renal anomalies were analized. Five of them were girls and six of them were boys. Of these 11 patients, 4 patients (63.6%) had unilateral vesicoureteral reflux (VUR) and 3 of them had higher grade bilateral (IV-V) VUR. Two patients (18%) had ectopic kidney, 4 patients (36%) had unilateral renal agenesis. Three patients (27%) needed clean intermittent catheterization for neurogenic bladder. One of the patients had penil deformity and urethral stricture. Four of the patients has predicting glomerular filtration rate lower than 60 ml/dk/1.73 m2 which demonstrates advanced stage chronic renal failure. One of them had end stage renal failure and followed on chronic peritoneal dialysis treatment.Genitourinary anomalies are frequently encountered in patients with fanconi aplastic anaemia. It is important to follow up of patients with fanconi aplastic anaemia in terms of the risk of chronic kidney disease.
The literature on the use of recombinant factor VIIa (rFVIIa), which was initially used in hemophiliac patients with inhibitors, for hemorrhages that cannot be managed with conventional methods or ...operations that cannot be performed safely is increasingly growing. This study presents a group of nonhemophiliac patients with hemorrhagic problems or hemorrhage risk for some interventions that were successfully resolved with the use of rFVIIa. The patient group was composed of 20 patients with different disorders resulting in similar results as hemorrhage or hemorrhage risk. Most of the patients were diagnosed with liver disorders primary or secondary to other diseases. The remaining cases were patients with leukemia, sepsis, intracranial hemorrhage, and burn. Some of the patients had multiple problems like a patient with liver disorder and intracranial hemorrhage or a leukemia patient with sepsis and disseminated intravascular coagulation. rFVIIa had been administered to the patients at dosages between 70 and 150 μg/kg up to 6 doses with 2-hour to 3-hour intervals. All the patients had benefited from the use of rFVIIa even though some of them died because of primary disease. This study shows that rFVIIa can be safely used in high-risk patients with a history of recurrent hemorrhage, for whom no improvement can be achieved in the hemostasis tests.
Purpose: Haemophilia causes musculoskeletal problems over many years secondary to recurrent hemarthrosis. In this study, the effects of water exercises on the musculoskeletal system of severe ...haemophilia A patients with muscle and joint problems were investigated. Material and Methods: Eleven severe haemophilia A patients on prophylaxis treatment participated in the study and following a regular exercise protocol. Results: Subjects displayed statistically significant increases in mid-thigh, upper thigh and calf circumference for right leg (42.0 +/- 2.4, 43.0 +/-2.1 ; 37.1 +/-1.9, 39.0 +/-1.8; 28.1 +/- 1.4, 28.9 +/-1.3 respectively) (mean +/- SE) in mid-thigh and upper thigh for left leg (36.9 +/- 1.5 , 38.9 +/- 1.5 ; 41.2 +/- 2.2 , 42.9 +/- 2) (p 0.05). Compared to pre-exercise values, leg extensor and flexor strength as well as range of motion were increased significantly (96.6 +/- 9 and #7506; vs 104.5+/- 8 and #7506;; before and after training for right knee, 98.5 +/- 7.6 and #7506; vs 104 +/- 7.9 and #7506; before and after training for left leg respectively) (p 0.05). In addition to that, post training serum level of growth hormone was found to be significantly higher than the pertaining value (p 0.05). Conclusion: These results show that some easily performed exercise protocols such as water exercises can promote muscle development and increase range of motion of the knee joint. Our findings indicate that appropriately designed water exercise may prevent muscle atrophy and joint deformities in haemopliliac patients. Cukurova Med J 2014; 39(3.000): 470-479
Sickle cell anemia is a disease which is characterized with hemolytic anemia, hypercoagulopathy and painful crisis. Microparticles are 0,1-1 µm sized little membrane particles which are derived ...during activation or apoptotic phase of cell cycle. It is reported that microparticles are increased in many systemic disease including sickle cell anemia.
In this study we aimed to investigate the role of microparticles on during crisis and non-crisis periods in sickle cell anemia patients.
Twenty nine patients, following by Cukurova University, Department of Pediatric Hematology, are included in this study. Blood samples were collected in 26 of these patients in non-crisis period. Control group formed with 18 healthy children without any systemic disease. Complete blood count, hemoglobin electrophoresis and biochemical parameters were studied in both groups. Also patients' total microparticle levels, erythrocyte (CD235a), endothelial (CD106), monocyte (CD14) particle levels and tissue factor expressing (CD142) microparticle levels were studied by flow cytometry and whole data was statistically analyzed.
Hemoglobin and hematocrit levels were significantly low in sickle cell anemia patients (p<0,001). Levels of HbS were significantly high during crisis period comparing with mean HbS levels during non-crisis period (p<0,001). Total microparticle levels were significantly high in sickle cell anemia patients with painful crisis comparing with control group (p<0,05). Erythrocyte and monocyte microparticle levels were significantly high in patients with painful crisis comparing with non-crisis periods (p<0,05). Endothelial and tissue factor expressing microparticle levels were high in patiens with crisis comparing to non-crisis period but this was not statistically significant (p>0,05). There was not any significant relation with frequency of crisis and microparticle levels (p>0,05). Microparticle levels were low in patients whose were taking hydroxiurea treatment comparing with non-hydroxiurea treatment but this data was not statistically significant (p>0,05).
As a result we found high levels of total microparticle, erythrocyte and monocyte microparticles in sickle cell anemia patients during painful crisis period. This important clue of crises was need further studies in order to understand the effect of microparticles on pathophysiology of sickle cell anemia.
No relevant conflicts of interest to declare.
PDF
