• Title/Summary/Keyword: Hemophilia A

Search Result 55, Processing Time 0.036 seconds

Comparative Study on Parenting Stress, Guilt, Parenting Attitude, and Parenting Satisfaction Between Mothers with a Hemophilic Child and a Healthy Child (혈우아동과 일반아동 어머니의 양육스트레스, 죄책감, 양육태도 및 부모역할만족도 비교)

  • Kim, Won-Ok;Kang, Hyun-Sook;Cho, Kyoul-Ja;Song, Young-A;Ji, Eun-Sun
    • Women's Health Nursing
    • /
    • v.14 no.4
    • /
    • pp.270-277
    • /
    • 2008
  • Purpose: Although Hemophilia is a relatively rare hereditary disease, and is treatable with blood products, the parenting stress and guilt of hemophilic patients and their mothers are always high. This study was done to assess the extent of parenting stress, guilt, parenting attitude and parenting satisfaction of mothers with a hemophilic child. Method: The participants in this study were 119 mothers with a hemophilic child who were registered members of the Korea Hemophilia Foundation, and 287 mothers with a healthy child. In order to measure the dimensions related to parenting stress, guilt, parenting attitude and parenting satisfaction, the Questionnaire on Parenting Stress Index, Maternal Guilt Scale, Parenting Attitude Scale & Parenting Satisfaction Scale were administered. We analysed the differences between mothers with a hemophilic child and a healthy child in the questionnaire scores using the SPSS program. Result: Parenting stress and guilt of mothers with a hemophilic child were higher than with a healthy child. Parenting attitude and parenting satisfaction of mothers with a hemophilic child were lower than with a healthy child. Conclusion: The results may help medical professionals understand mothers with a hemophilic child and give basic assistance to develop a nursing intervention by exploring possible ways to alleviate such parenting stress and guilt.

  • PDF

Comparative evaluation of the safety and efficacy of recombinant FVIII in severe hemophilia A patients

  • Abolghasemi, Hassan;Panahi, Yunes;Ahmadinejad, Minoo.;Toogeh, Gholamreza;Karimi, Mehran;Eghbali, Aziz;Mirbehbahani, Nargess Bigom;Dehdezi, Bighan Keikhaei;Badiee, Zahra;Hoorfar, Hamid;Eshghi, Peyman;Maghsoudi, Nader;Sahebkar, Amirhossein;Gholami-Fesharaki, Mohammad
    • Journal of Pharmacopuncture
    • /
    • v.21 no.2
    • /
    • pp.76-81
    • /
    • 2018
  • Objective: This study compared the safety and efficacy of $Safacto^{(R)}$ versus $xyntha^{(R)}$ in patients with severe hemophilia A. Methods: Thirty-three male patients with severe hemophilia A were randomly divided into two groups. Seventeen patients received $Safacto^{(R)}$ and 16 patients received $Xyntha^{(R)}$ for four consecutive times. The dosage of FVIII was 40-50 IU/kg for each injection. Plasma level of FVIII activity was evaluated before every injection, 15 minutes after the injection and one month after the start of the trial. The rate of factor VIII activity, pain and joint motion were also assessed before and after the treatment. Results: Plasma level of FVIII clotting activity in $Safacto^{(R)}$ and $Xyntha^{(R)}$ were $1.96{\pm}0.5IU/dl$ and $1.63{\pm}0.5IU/dl$ and increased to $88.84{\pm}25.2IU/dl$ and $100.09{\pm}17.8IU/dl$, respectively (P<0.001). Pain score and range of motion improvement were $9.3{\pm}0.9$ and $8.7{\pm}0.1$ in $Safacto^{(R)}$ (P=0.17); and $9.4{\pm}0.8$ and $8.8{\pm}0.3$ in $Xyntha^{(R)}$ (P=0.35), respectively. No allergic or other unfavorable reactions was observed with either of the preparations. Conclusion: This study showed that $Safacto^{(R)}$ has a favorable efficacy and safety profile.

Detecting Activated Thrombin Activatable Fibrinolysis Inhibitor (TAFIa) and Inactivated TAFIa (TAFIai) in Normal and Hemophilia A Plasmas

  • Hulme, John P.;An, Seong Soo A.
    • Bulletin of the Korean Chemical Society
    • /
    • v.30 no.1
    • /
    • pp.77-82
    • /
    • 2009
  • Thrombin activatable fibrinolysis inhibitor (TAFI) also known as plasma procarboxypeptidase B or U is a 60 kD glycoprotein, which is the major modulator of fibrinolysis in plasma. TAFI is a proenzyme, which is activated by proteolytic cleavage to an active carboxypeptidase B-like enzyme (TAFIa, 35.8 kD) by thrombin/thrombomodulin and plasmin. Modulation of fibrinolysis occurs when TAFIa enzymatically removes C-terminal lysine residues of partially degraded fibrin, thereby inhibiting the stimulation of tissue plasminogen activator (t-PA) modulated plasminogen activation. TAFIa undergoes a rapid conformational change at $37{^{\circ}C}$ to an inactive isoform called TAFIai. Potato tuber carboxypetidase inhibitor (PTCI) was shown to specifically bind to TAFIa as well as TAFIai. In this study, a novel immunoassay TAFIa/ai ELISA was used for quantitation of the two TAFI activation isoforms TAFIa and TAFIai. The ELISA utilizes PTCI as the capture agent and a double antibody sandwich technique for the detection. Low levels of TAFIa/ai antigen levels were detected in normal plasma and elevated levels were found in hemophilia A plasmas. TAFIa/ai antigen represents a novel marker to monitor fibrinolysis and TAFIa/ai ELISA may be a valuable assay for studying the role of TAFI in normal hemostasis and in pathological conditions.

Experience of Hemophilic Pseudotumor Developed at the Fracture Site of the Proximal Phalanx of the Hand (수부 근위지골 골절 수술 후 발생한 혈우병성 가성종양의 치험례)

  • Park, Ji Ung;Kim, Chang Yeon;Shin, Jong In;Cho, Sang Hun
    • Archives of Plastic Surgery
    • /
    • v.34 no.4
    • /
    • pp.516-519
    • /
    • 2007
  • Purpose: Hemophilia is one of the most common bleeding disorder, and circulating levels of factor VIII or IX are closely related to the severity and frequency of the bleeding. The severity of hemophilia is classified to be severe, moderate, and mild when the factor level is less than 1%, between 1% and 5%, and between 5% and 25%, respectively. Hemophilic pseudotumor is a rare complication occurring in 1 - 2% of patients with factor VIII or IX deficiency. If the treatment is delayed, it would cause disabling and life threatening results. Methods: We experienced hemophilic pseudotumor developed at the fracture site of the proximal phalanx of the hand in a hemophilic B patient. Hemophilic pseudotumor was successfully treated with perioperative factor replacement and surgical intervention included excision and autologous bone graft. Results: Hemophilic pseudotumor was healed with complete regression, and no specific complication was developed. Conclusion: When we accounter hematoma like lesion after surgery unpredictably, we must consider hemophilic pseudotumor and make a accurate diagnosis with preoperative hematologic screening and various imaging study. Subsequently, adequate perioperative supplement of concentrate and surgical intervention brings to the satisfactory result without recurrence.

Improvement of the Sequelae of Peripheral Facial Nerve Palsy in a Patient with Hemophilia Mainly Treated with Facial Chuna Manual Therapy: A Case Report

  • Geumm Mi Lee;Jae Hyung Kim;Ga Young Choi;Jung Hee Lee;Jae Soo Kim;Hyun Jong Lee
    • Journal of Acupuncture Research
    • /
    • v.41
    • /
    • pp.143-148
    • /
    • 2024
  • A patient with sequelae of peripheral facial nerve palsy (FNP) was mainly treated with facial chuna manual therapy (FCMT) for over 6 months. The patient was diagnosed with hemophilia B and C, so invasive treatments, such as acupuncture and pharmacopuncture, were precluded. The facial nerve grading system 2.0 (FNGS) and the House-Brackmann Grading Scale (HBGS), Peitersen, Murata, Mehta, and numeric rating scale (NRS) systems were used to measure treatment effectiveness. Each scale showed improvement: FNGS, 3 to 2; HBGS, 3 to 2; Peitersen scale, 2 to 1; Murata scale, 9 to 4; Mehta scale, 14 to 1; and NRS, 8.5 to 2.5. An overall improvement was evident in facial muscle strength, particularly in synkinesis. If acupuncture and pharmacopuncture are unavailable, FCMT alone may be effective in treating FNP sequelae.

The Effect of Tai Chi Self Help Group Program for Hemophilic Arthritis Patients (혈우병관절염 수술환자를 위한 타이치자조관리 프로그램 효과)

  • Bak, Won-Sook;Yoo, Myung-Chul;Kang, Hyun-Sook
    • Journal of muscle and joint health
    • /
    • v.19 no.1
    • /
    • pp.71-83
    • /
    • 2012
  • Purpose: The purpose of this study was to develop and evaluate the effects of Tai Chi Self-help program for Hemophilic Arthritis Patients. Method: A quasi-experimental design was used. The subjects were 48 patients who underwent orthopedic surgery (24 experimental group, 24 control group). The subjects of experimental group were participated in the Tai Chi self-help group program in which 16 times for 8 weeks. The program consisted of health education on hemophilia, Tai Chi exercise, and workshop and its outcomes have been evaluated on WOMAC(Western Ontario and McMaster scale), mobility, pain, fatigue, muscle strength, depression, and quality of life(SF-36). The obtained data were analyzed by using the t-test or Fisher's exact test of PASW 18.0. Results: 1) The score of WOMAC, pain, fatigue, and depression decreased significantly in the experimental group as compared to the control group. 2) The score of mobility, muscle strength, and quality of life increased significantly in the experimental group as compared to the control group. Conclusion: Considering these research results, the program could be useful to improve joint movement, strength and psychological condition for patient with hemophilic arthritis, particularly in those who underwent orthopedic surgery.

Femoral Neuropathy due to Iliacus Muscle Hematoma in a Patient on Warfarin Therapy

  • Kong, Woo-Keun;Cho, Keun-Tae;Lee, Ho-Jun;Choi, Jae-Sung
    • Journal of Korean Neurosurgical Society
    • /
    • v.51 no.1
    • /
    • pp.51-53
    • /
    • 2012
  • Spontaneous hematomas of the iliacus muscle are rare lesions and these are seen in individuals receiving anticoagulation therapy or patients with blood dyscrasias such as hemophilia. It can cause femoral neuropathy and resultant pain and paralysis. Although there is no clear consensus for the treatment of femoral neuropathy from iliacus muscle hematomas, delays in the surgical evacuation of hematoma for decompression of the femoral nerve can lead to a prolonged or permanent disability. We report here on a rare case of a spontaneous iliacus muscle hematoma that caused femoral neuropathy in a patient who was taking warfarin for occlusive vascular disease and we discuss the treatment.

Hemophiliac Patient with Hemarthrosis and Physical Therapy (혈우병 환자의 관절혈증과 물리치료)

  • Lee, Woo-Hyung
    • The Journal of Korean Academy of Orthopedic Manual Physical Therapy
    • /
    • v.16 no.1
    • /
    • pp.43-49
    • /
    • 2010
  • Purpose : The purpose of this study was to understanding of a hemophiliac with hemarthrosis and provided information of rehabilitative therapy. Methods : The General understanding and rehabilitative therapy of a hemophiliac with hemarthrosis considered the matter carefully using a related publications and a internet searching investigation. Results : From the physical therapeutic method for pain alleviation, decreasing edema, ability of muscular strength and control, range of motion(ROM) in joint, prevention of damage on hemorrhaged joint in the hemophiliac with hemarthrosis, rehabilitative therapy for exercise of ROM, muscular strength, proprioceptive sense was most effective for patients. Conclusion : Physical therapists known the rehabilitative therapy should helped a hemophiliac with hemarthrosis to prevent from permanent change.

  • PDF