RESUMEN
Kasabach-Merritt phenomenon (KMP) is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma. This condition primarily occurs in infants and young children, usually with acute onset and rapid progression. This review article introduced standardized recommendations for the pathogenesis, clinical manifestation, diagnostic methods and treatment process of KMP in China, which can be used as a reference for clinical practice.
Asunto(s)
Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/terapia , Niño , China/epidemiología , Diagnóstico Diferencial , Humanos , Síndrome de Kasabach-Merritt/epidemiología , Nivel de AtenciónRESUMEN
To assess the practicality of 3-D power Doppler angiography (3-D-CPA) for local drug perfusion dosage guidance of refractory infantile hemangioma (IH) treatment, 47 cases (48 lesions) of refractory IH were selected for local bleomycin infusion (once a month). Ultrasound was performed before treatment and 1 and 2 months after the first treatment. The 3-D volume (V) change of infantile hemangiomas and the ratio of bleomycin injection to 3-D V before treatment were calculated, and statistical analysis was performed. One month after percutaneous local drug perfusion, 37 participants (77.08%) exhibited significant improvement; controlled growth was observed on six lesions (12.5%); and treatment of the remaining 5 lesions (10.42%) failed to suppress growth. The calculated tolerable and effective dose of bleomycin for refractory IH was 0.34 ± 0.03 mL/cm3; the corresponding 3-D V decreased approximately 70.27 ± 6.27%. Three-dimensional CPA can provide abundant information on internal lesions. In particular, 3-D-CPA can quantitatively assess changes in lesion volume and guide the effective and rational use of interventional drugs.
Asunto(s)
Angiografía/métodos , Antibióticos Antineoplásicos/administración & dosificación , Bleomicina/administración & dosificación , Hemangioma/diagnóstico por imagen , Hemangioma/tratamiento farmacológico , Imagenología Tridimensional , Neoplasias Cutáneas/diagnóstico por imagen , Neoplasias Cutáneas/tratamiento farmacológico , Ultrasonografía Doppler , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Perfusión/métodos , Estudios RetrospectivosRESUMEN
Infantile hemangioma (IH) is one of the most common benign vascular tumors in children. A variety of treatment methods have been documented for the management of IH over the past years, including pharmacotherapy via oral administration or injection of corticosteroids, vincristine, alpha interferon and bleomycin; laser therapy, radionuclide therapy, cryotherapy and excisional surgery. The therapeutic efficacy of each treatment modality is variable, while adverse effects or complications are common and sometimes serious. Since the serendipitous discovery of propranolol, a nonselective beta-adrenergic receptor blocker, being very efficacious in treating IH in 2008, oral propranolol has earned a role as a first-line medical therapy for complicated IH. However, the appropriate drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects remains controversial. To standardize the use of propranolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese experts consensus on the use of oral propranolol for treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion.
Asunto(s)
Antagonistas Adrenérgicos beta/uso terapéutico , Hemangioma/tratamiento farmacológico , Propranolol/uso terapéutico , Administración Oral , Consenso , Femenino , Humanos , Lactante , Terapia por Láser , Masculino , Propranolol/administración & dosificación , Neoplasias Cutáneas , Resultado del TratamientoRESUMEN
Non-selective ß-blocker propranolol has been proved by FDA as the first-line agent for infantile hemangioma (IH) with dramatic response. To reduce the side effects caused by systemic administration of propranolol, timolol maleate treatment has been increasingly used as an alternative to systemic ß-blockers and watchful waiting for many IH patients in recent years. However, the appropriate indications, drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects still remains controversial. To standardize the use of topical timolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese expert consensus on the use of topical timolol treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion, which can be used to reduce inappropriate variations in clinical practice and to promote the delivery of high quality, evidence-based health care for IH patients.