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1.
Health Sci Rep ; 7(4): e2003, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38567187

RESUMO

Background and Aims: Knee support, frequently made from sponge, is used to reduce injury. Sponge has less elasticity and durability compared with natural rubber. To our knowledge, there was no study that demonstrated the effectiveness of natural rubber and sponge in prevention of injury in children with bleeding disorders. The study aimed to demonstrate the effectiveness and satisfaction of natural rubber knee support compared with sponge knee support among children with bleeding disorders. Methods: The study consisted of three phases: (I) measuring reduced compression force, (II) producing size-appropriate knee support prototypes, and (III) conducting a randomized crossover trial, including 8 weeks wearing natural rubber knee support and sponge knee support with a 4-week wash-out period. The number of knee bleeds and user satisfaction were recorded. Results: A better compression force reduction in natural rubber (60%) than sponge (12%) was demonstrated. Knee support comprised a body part, made from natural-stretchable cotton and a protection part, made from either natural rubber or sponge. They were produced in four sizes: S, M, L, and XL and appropriately applied to 42 patients (21 hemophilia, 21 platelet disorders) with a mean (SD) age of 7.0 (2.9) years. The results from randomization showed no significant difference in the number of knee bleeds between the two knee support groups (10 vs. 7, p = 0.37). In terms of satisfaction score, the natural rubber knee supports were more durable (45.2% vs. 23.8%, p = 0.04) and easier to use (28.5% vs. 14.3%, p = 0.03). In addition, a higher percentage of parents chose natural rubber knee support when compared with sponge knee supports (71.0% vs. 29.0%, p = 0.006). Conclusion: Natural rubber knee support showed comparable effectiveness in the prevention of knee bleeding but was superior to sponge knee support in compression force reduction and satisfaction.

2.
Expert Rev Hematol ; 16(12): 1063-1076, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38100503

RESUMO

INTRODUCTION: Inherited bleeding disorders (IBDs) including hemophilia, von Willebrand disease, platelet disorders, mucocutaneous bleeding disorders and coagulation factor deficiencies are rarely found and under-recognized in low and lower-middle-income countries. Some patients succumbed to serious bleeding without diagnosis and treatment during childhood. AREA COVERED: Diagnosis, management, and prevention should be integrated into the existing health care system. Although some countries have not implemented appropriate health care infrastructure, an initiative plan should be set up by cooperation of experienced experts and health care providers. Identification of patients with IBDs should be started in the antenatal setting to search for females at risk of carrier state. The investigations include bleeding assessment, mixing venous clotting time, coagulogram, coagulation factor assay and mutation detection. Genotypic analysis is helpful for confirming the definite diagnosis, carrier detection as well as prenatal diagnosis for females at risk of bearing an offspring with severe bleeding manifestations. Management involves replacement therapy ranging from blood component to virus-inactivated factor concentrate. Appropriate research is an essential backbone for improving patients' care. EXPERT OPINION: Effective national strategic advocacy to manage patients with IBDs requires intensive collaboration among policy makers, health care providers, patients, and family members.


Assuntos
Transtornos Herdados da Coagulação Sanguínea , Hemofilia A , Doenças de von Willebrand , Humanos , Feminino , Gravidez , Países em Desenvolvimento , Transtornos Herdados da Coagulação Sanguínea/diagnóstico , Transtornos Herdados da Coagulação Sanguínea/genética , Transtornos Herdados da Coagulação Sanguínea/terapia , Hemofilia A/terapia , Hemorragia/diagnóstico , Hemorragia/etiologia , Hemorragia/prevenção & controle , Fatores de Coagulação Sanguínea
4.
Int J Hematol ; 117(2): 293-306, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36151351

RESUMO

OBJECTIVES: The study aimed to determine the incidence of femoral neuropathy in patients with haemophilia exhibiting iliopsoas haemorrhage. METHODS: Patients with iliopsoas haemorrhage confirmed by ultrasonography or CT scan were studied retrospectively. RESULTS: A total of 44 episodes of iliopsoas haemorrhage occurred in 20 patients with haemophilia (A17, B3). Most episodes in patients without inhibitors (14/16 = 87.5%) were adequately treated followed by prophylaxis. However, 11 of 28 episodes (39.3%) in patients with inhibitors were adequately treated and no prophylaxis was provided. An appropriate rehabilitation programme was arranged during hospitalisation and follow-up. Femoral neuropathy was observed in 28 of 44 episodes, while 16 episodes of persistent femoral neuropathy from previous bleeding were excluded. As a result, 11 of 28 episodes (39.3%) of femoral neuropathy were similarly found amongst patients with and without inhibitors. The mean time of onset and resolution of femoral neuropathy were 3.7 (1.8) and 23.4 (20.5) days after the onset of iliopsoas haemorrhage, respectively. Patients receiving inadequate and delayed replacement had a significantly higher rate of femoral neuropathy than those who received adequate and prompt replacement. CONCLUSION: Femoral neuropathy following iliopsoas haemorrhage was common in haemophilia patients with and without inhibitors.


Assuntos
Neuropatia Femoral , Hemofilia A , Humanos , Hemofilia A/complicações , Neuropatia Femoral/complicações , Estudos Retrospectivos , Hemorragia/etiologia , Pesquisa
5.
Orthop Res Rev ; 13: 209-214, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34819756

RESUMO

Distal upper extremity weakness is a rare complication after herpes zoster, which can be easily misdiagnosed by other nerve entrapment syndromes. We present a 31-year-old immunocompromised woman who developed a claw-like deformity of her right hand after full medical treatment and resolution of herpes zoster. The electrodiagnostic finding was compatible with right multiple mononeuropathies of the median and ulnar nerves, ongoing axonal loss, unlike the nerve entrapment patterns. The early recognition and early electrodiagnosis of herpes zoster-induced distal motor weakness, especially in the upper extremity, is necessary to exclude the nerve entrapment syndrome and to be the baseline for functional motor recovery prediction. The prognosis of functional motor recovery is considered good but may take months to years to accomplish. Rehabilitation management plays an important role after full medical treatment.

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