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1.
Pediatr Blood Cancer ; 70(2): e30119, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36495235

RESUMO

McLeod phenotype-caused by the missing Xk protein-is a very rare red cell phenotype, one characteristic of McLeod syndrome, and sometimes associated with X-linked chronic granulomatous disease (CGD). Diagnosis of McLeod phenotype is important for appropriate transfusion management, because red blood cells from all healthy donors will have the Xk protein with its Kx antigen and can lead to red cell antibody formation without the ability to find compatible McLeod phenotype blood for transfusion. We offer a review and approach to diagnosis of the McLeod phenotype and special transfusion considerations.


Assuntos
Doença Granulomatosa Crônica , Neuroacantocitose , Humanos , Neuroacantocitose/genética , Transfusão de Sangue , Doença Granulomatosa Crônica/genética , Fenótipo
2.
Transfusion ; 62(9): 1699-1705, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35815552

RESUMO

BACKGROUND: Whole blood (WB) is an attractive product for prehospital treatment of hemorrhagic shock and for initial in-hospital resuscitation of patients likely to require massive transfusion. Neither our regional blood provider nor our hospital blood bank had recent experience collecting or using WB, so we developed a stepwise process to gather experience with WB in clinical practice. METHODS: When our Transfusion Committee suggested a WB program, we worked with our regional blood provider to collect cold-stored, leukoreduced, low-titer anti-A, and anti-B group O RhD positive WB (low-titer group O WB [LTOWB]) and worked with our city Fire Department to integrate it into prehospital care. This work required planning, development of protocols, writing software for blood bank and electronic medical records, changes in paramedic scope of practice, public information, training of clinicians, and close clinical follow-up. RESULTS: Between June 2019 and December 2021, we received 2269 units of LTOWB and transfused 2220 units; 24 (1%) were wasted, two were withdrawn, and 23 were in stock at the end of that time. Most (89%) were transfused to trauma patients. Usage has grown from 48 to 120 units/month, covers all 5 Fire Districts in the county, and represents about » of all hospital trauma blood product use. CONCLUSIONS: Developing a WB program is complex but can be started slowly, including both pre-hospital and hospital elements, and expanded as resources and training progress. The investments of time, effort, and funding involved can potentially improve care, save blood bank and nursing effort, and reduce patient charges.


Assuntos
Choque Hemorrágico , Ferimentos e Lesões , Bancos de Sangue , Transfusão de Sangue/métodos , Hospitais , Humanos , Ressuscitação/métodos , Choque Hemorrágico/terapia
3.
Transl Androl Urol ; 10(3): 1342-1346, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33850768

RESUMO

Leiomyosarcoma (LMS) is one of the most common forms of soft tissue sarcoma with approximately 2,500 cases per year in the United States. The symptoms LMS vary depending upon the location, size, and spread of the tumor. In early stages, it may not be associated with any obvious symptoms so diagnosis and treatment may be delayed. In some cases, it can grow quickly and behave aggressively. Most types of LMS occur in the abdomen or in the uterus; although, scrotal LMS can be a very rare presentation of the disease. Here we present our case of a large, ulcerated scrotal LMS originating from subcutaneous tissue but not invading spermatic cord or tunica. Radical orchiectomy with high ligation of spermatic cord was performed, and patient had an uneventful postoperative course. This disease entity remains rare in the literature, and warrants larger studies in order to better understand treatment and oncologic outcomes. When LMS is identified early and is removed by surgical excision, prognosis can be good and full recovery quite likely. When LMS is already large or has spread to other parts of the body, treatment is relatively more complex and the prognosis poor. Hence, prompt diagnosis and treatment of genitourinary LMS require prompt attention, referral to tertiary, referral center should be strongly considered.

6.
Autops. Case Rep ; 8(4): e2018050, Oct.-Dec. 2018. ilus
Artigo em Inglês | LILACS | ID: biblio-986542

RESUMO

Herpes Simplex Virus infections (HSV) are ubiquitous. The neonatal HSV infection (NHSV) is rare. The incidence is estimated globally at only 10.3 per 100,000 births, but it can cause devastating disease in premature infants. Both HSV-1 and HSV-2 can be the etiologic agents in this type of vertically transmittted NHSV infection. Here we describe the pathological findings from a complete autopsy of a very low birth weight infant who succumbed to the infection despite early institution of antiviral treatment. We urge more awareness of this disease with continued surveillance; every effort should be taken to make an early diagnosis and thus prevent this devastating disease.


Assuntos
Humanos , Feminino , Recém-Nascido , Complicações Infecciosas na Gravidez , Herpes Simples/patologia , Autopsia , Gravidez , Evolução Fatal , Doenças Raras , Nascimento Prematuro , Sepse Neonatal
7.
Autops Case Rep ; 8(4): e2018050, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30775325

RESUMO

Herpes Simplex Virus infections (HSV) are ubiquitous. The neonatal HSV infection (NHSV) is rare. The incidence is estimated globally at only 10.3 per 100,000 births, but it can cause devastating disease in premature infants. Both HSV-1 and HSV-2 can be the etiologic agents in this type of vertically transmittted NHSV infection. Here we describe the pathological findings from a complete autopsy of a very low birth weight infant who succumbed to the infection despite early institution of antiviral treatment. We urge more awareness of this disease with continued surveillance; every effort should be taken to make an early diagnosis and thus prevent this devastating disease.

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