[Partial splenectomy in sickle cell disease].

Introduction: Total splenectomy in sickle cell disease is related to a high risk of fulminant sepsis and increased incidence of other events, which have not been reported in patients with partial splenectomy. In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients.

Material And Methods: We studied 54 patients with sickle cell disease who underwent partial splenectomy in childhood from 1986 until 2011 at the Institute of Hematology and Immunology.

[Primary amoebic meningoencephalitis: a case report].

Introduction: Infections of the central nervous system by free-living amoeba are a rare phenomenon around the world. They present as a necrotising haemorrhagic acute meningoencephalitis, with a fatality rate close to 100% or as granulomatous amoebic encephalitis with a chronic progression.

Case Report: We describe a fatal case of primary amoebic meningoencephalitis produced by Naegleria fowleri in an 8-year-old child, with a history of immersion in a freshwater reservoir 52 hours before death.

[Retroperitoneal fibrosarcoma. Apropos of a case].

Objectives: To report one case of retroperitoneal fibrosarcoma in a 45 year-old caucasian patient cared for at the Public Health System hospitals in Republic of Cuba.

Methods: Blood analysis showed increased erythrocyte sedimentation rate and moderate anemia. Intravenous urography showed that left kidney was displaced towards midline but presented a good contrast uptake and excretion.

[Embryonic rhabdomyosarcoma of the prostate. Report of a case and review of the literature].

Objectives: To report a new case of prostatic rhabdomyosarcoma in a child.

Methods: Blood analysis, urinalysis, radiologic tests (abdominal ultrasound, intravenous urography, cystography, barium enema, lower extremities X-rays), and prostatic biopsy were performed.

Results/conclusions: Neoadjuvant treatment with chemotherapy and radiotherapy were started prior to intended radical prostatectomy, but the patient died before due to disease progression with dissemination.

[Reflex anoxic cerebral crises in children].

Objective: Reflex anoxic cerebral crises are due to depression of nerve function caused by a vagotonic state or vagal hypersensitivity. In this paper we propose to review the physiopathology, clinical features and diagnostic procedures of these crises.

Development: There are three types of reflex anoxic cerebral crises: asphyxiating anoxic crises, ischemic anoxic crises and asphyxiating-ischemic anoxic crises.

[Trisomy 5p due to paternal translocation (4;5) (q35;p12)].

A ten month-old boy had a 5p trisomy secondary to a paternal t(4;5) (p35;p12) translocation. The main stigmata were macrocranium, hydrocephaly, coloboma of the right iris, atrial septal defect, talipes equinovarus, and psychomotor retardation. Overall, the clinical picture was characteristic of the 5p trisomy that includes the band 5p13.