Brain DSC MR Perfusion in Children: A Clinical Feasibility Study Using Different Technical Standards of Contrast Administration.

BACKGROUND AND PURPOSE: Dynamic susceptibility contrast MR perfusion imaging has limited results in children due to difficulties in reproducing technical standards derived from adults. This prospective, multicenter study aimed to determine DSC feasibility and quality in children using custom administration of a standard dose of gadolinium. MATERIALS AND METHODS: Eighty-three consecutive children with brain tumors underwent DSC perfusion with a standard dose of gadobutrol administered by an automated power injector. The location and size of intravenous catheters and gadobutrol volume and flow rates were reported, and local and/or systemic adverse effects were recorded. DSC was qualitatively evaluated by CBV maps and signal intensity-time curves and quantitatively by the percentage of signal drop and full width at half-maximum, and the data were compared with the standards reported for adults. Quantitative data were grouped by flow rate, and differences among groups were assessed by analysis of covariance and tested for statistical significance with a t test. RESULTS: No local or systemic adverse events were recorded independent of catheter location (63 arm, 14 hand, 6 foot), size (24-18 ga), and flow rates (1-5 mL/s). High-quality CBV maps and signal intensity-time curves were achieved in all patients, and quantitative evaluations were equal or superior to those reported for adults. No significant differences (P ≥ .05) were identified among the higher-flow-rate groups in the quantitative data. CONCLUSIONS: A custom administration of a standard dose of gadobutrol allows safe and high-quality DSC MR perfusion imaging in children.

Molecular adsorbents recirculating system treatment in acute-on-chronic hepatitis patients on the transplant waiting list improves model for end-stage liver disease scores.

BACKGROUND: The aim of our study was to show an improvement in Model for End-Stage Liver Disease (MELD) score after treatment with Molecular adsorbents recirculating system (MARS) in acute-on-chronic hepatitis (AoCHF) patients. MELD was adopted to determine the prognosis of patients with liver chronic desease. We evaluated the possibility to improve the MELD score of patients awaiting liver transplantation using a liver support device, namely, MARS. PATIENTS AND METHODS: From September 1999 to April 2006, we treated 80 patients whose diagnoses were hepatitis C, 41.25%; hepatitis B, 27.5%; alcholic, 17.5%; intoxication, 8.75%; primary biliary cirrhosis, 5%. The overall mean age was 45 years (23 to 62), the cohort included 56 men and 24 women. Inclusion criteria were bilirubin >15 mg/dL; MELD >20; encephalopathy >II; and International Normalized Ratio, >2.1. Other parameters evaluated included ammonia, creatinine, lactate, glutamic oxalic transminase, and guanosine 5'-triphosphate. All patients were treated with a mean of 6-hour cycles of MARS (range, 5 to 8 hours) for a minimum of three treatments and a maximum of 20 treatments over 3 months. Clinical conditions were evaluated by improved hemodynamic parameters, kidney function, liver function, coagulation, neurologic status using the SOFA score, Glasgow Coma Scale (GCS), and Acute Physiology and Chronic Health Evaluation II Criteria. RESULTS: The MELD score for all categories of living patients showed significant improvements at the end of treatment and at 3-months follow-up, but the small number of patients was a limitation to determine prediction of mortality. CONCLUSION: Our study shows that MARS treatment improved multiple organ functions-liver, renal, neurologic, and hemodynamic. The improved MELD score gave patients on the transplant waiting list longer survival, allowing them a greater opportunity for liver transplantation.

[Treatment of idiopathic thrombocytopenic purpura with danazol]. / Tratamiento con danazol en púrpura trombocitopénica idiopática (PTI).

PURPOSE: To assess the response to danazol in our patient population with ITP. PATIENTS AND METHODS: We evaluated 17 patients with ITP (11 women and 6 men) with average age 67.3 +/- 9.9 years (range 46-83): 10 patients with acute ITP without response to corticosteroids during 4-6 weeks; 4 patients with acute ITP without treatment; 2 patients with chronic ITP and 1 patient with recurrent ITP resistant to corticosteroids. The dose of danazol ranged between 100 and 600 mg/d. The patients were included in two groups: 1) Responders if they achieved complete or partial remission; 2) Non-responders if they achieved transient or fair response. RESULTS: The overall response rate was 58.8% and in patients previously treated 46.1%. The time to response was 35.6 +/- 48.5 days (range 14-180 days). Among the 10 responder patients 6 have remained in remission, 1 died 6 months after the remission for unrelated disease, and 3 relapsed after discontinuation of treatment with a good response after the reinstitution of therapy. Two patients resistant to treatment with danazol were splenectomized with transient response, and they responded favorably to the reinstitution of danazol. The treatment had a good tolerance. CONCLUSION: 1) The treatment with danazol in ITP is a good alternative therapeutical approach as initial treatment in ITP and ITP patients without response to corticosteroids. 2) ITP patients resistant to danazol may response to this treatment after splenectomy.

[Idiopathic myelofibrosis with extramedullary hematopoiesis foci in the skin and testicles. Report of a case]. / Mielofibrosis idiopática con focos de hematopoyesis extramedular en piel y testículo. Presentación de un caso.

Idiopathic myelofibrosis (IMF) is a clonal chronic myeloproliferative syndrome characterized by the proliferation of the three haemopoietic series and the marrow connective tissue and by the development of extramedullary haemopoiesis in the liver, spleen and lymph nodes. Cutaneous extramedullary haemopoiesis is an uncommon event and we could not find any reported cases of testicular involvement in this disease. We report the case of a 28 year-old male with diagnosis of idiopathic myelofibrosis in November 1988. During the course of the disease, three years later, he developed a tumor on his right testis. Histologic examination showed extramedullary haemopoiesis with cells of the myeloid, erythroid and megakaryocyte series, in the interstice. Eight months later, numerous red-purple papules and nodules developed on the patients's trunk. The biopsy of a skin lesion revealed an infiltration of the dermis by myeloid, erythroid cells and few megakaryocytes. The patient's clinical condition worsened, and he died in February 1993 following progressive deterioration of the general condition. We describe a case of IFM with extramedullary hemopoiesis involving the skin and the testis pointing out the rarity of these localization.