Racial and Ethnic Disparities in Idiopathic Pulmonary Fibrosis: A UNOS/OPTN Database Analysis

We previously reported poorer survival among non-Hispanic blacks and Hispanics with idiopathic pulmonary fibrosis (IPF) compared to non-Hispanic whites at our center. In the current study, we hypothesized that these disparities would exist in a nationwide cohort of wait-listed patients with IPF. We performed a retrospective cohort study of 2635 patients with IPF listed for lung transplantation between 1995 and 2003 at 94 transplant centers in the United States. The age-adjusted mortality rate was higher among non-Hispanic blacks [hazard ratio (HR) = 1.24, 95% confidence interval (CI) 1.06-1.45, p = 0.009] and Hispanics (HR = 1.29, 95% CI 1.06-1.56, p = 0.01) compared to non-Hispanic whites. These findings persisted after adjustment for transplantation, medical comorbidities and socioeconomic status. Worse lung function at the time of listing appeared to explain some of these differences (HR for non-Hispanic blacks after adjustment for forced vital capacity percent predicted = 1.16, 95% CI 0.98-1.36, p = 0.09; HR for Hispanics = 1.21, 95% CI 0.99-1.48, p = 0.056). In summary, black and Hispanic patients with IPF have worse survival than whites after listing for lung transplant.     

The floating hip injury: which should we fix first?

In floating hip injuries, the priority of the steps in fixation has not been well described. We have treated these patients in accordance with the following protocols. In patients with pelvic ring fractures, the external fixation should be performed first, and in patients with acetabular fractures, the femoral fractures should be internally fixated first. The purpose of the present study was to review our experience with these fracture treatments to evaluate our strategy. For 8 years, 31 patients with femoral fractures associated with ipsilateral fractures: 26 unstable pelvic ring fractures and 7 unstable acetabular fractures were surgically treated at our level 1 trauma center. We reviewed the admission, operation, and follow-up records retrospectively and evaluated the order of the fixation and the complications. Two patients with acetabular fractures failed to follow our protocol outlined at the preoperative planning stage. There were no surgical difficulties other than two patients with the hemipelvis dislocated proximally who required traction of the fixated femur. The complications that were thought to be related to our protocol were one fat embolism syndrome and one pubic fracture displacement. One heterotopic ossification seemed to occur due to using a single approach for both fractures. We believe our protocol to be generally effective, however, in patients with acetabular and vertical shear type pelvic ring fractures, the internal or external fixation of the femur should be performed before the internal fixation of the pelvis.     

Subclinical course of adult visceral Niemann–Pick type C1 disease. A rare or underdiagnosed disorder?

Summary  We present the third case of Niemann–Pick disease type C without neurological symptoms. The patient was a 53-year-old woman without significant prior health problems who died of acute pulmonary embolism. Autopsy findings of hepatosplenomegaly, lymphadenopathy and ceroid-rich foam cells raised the suspicion of the visceral form of acid sphingomyelinase deficiency (Niemann–Pick disease type B; NPB) or a much rarer disorder, variant adult visceral form of Niemann–Pick disease type C (NPC). To verify the histopathological findings, SMPD1, NPC1 and NPC2 genes were analysed. Two novel sequence variants, c.1997G>A (S666N) and c.2882A>G (N961S) were detected in the NPC1 gene. No pathogenic sequence variants were found either in the SMPD1 gene mutated in NPB or in NPC2 gene. The pathogenicity of both NPC1 variants was supported by their location in regions important for the protein function. Both variations were not found in more than 300 control alleles. Identified sequence variations confirm the diagnosis of the extremely rare adult visceral form of Niemann–Pick disease type C, which is otherwise dominated by neurovisceral symptoms. Although only three patients have been reported, this (most probably underdiagnosed) form of NPC should be considered in differential diagnosis of isolated hepatosplenomegaly with foam cells in adulthood. Electronic supplementary material  Supplementary material is available for this article at     

p53 expression and disease outcome of breast cancer patients undergoing primary chemotherapy with anthracycline-containing regimens

Primary chemotherapy administered to breast cancer patientsis the best model to identify baseline features able to predictwhich patients may be most likely to benefit or not from a cytotoxicregimen. In the March issue of Annals of Oncology two papersevaluated the predictive role of immunohistochemical p53 expressionon     

Avascular osteonecrosis after hyperthermia in children and adolescents with pelvic malignancies: a retrospective analysis of potential risk factors.

PURPOSE: In children with locally advanced or recurrent malignant tumours, prognosis can be improved by regional deep hyperthermia (RHT) in combination with platin-based chemotherapy. However, because of the increasing number of patients that achieve long-time remission with this therapy, it is necessary to evaluate long-term sequelae of thermochemotherapy. During the years 1993-2004 one has observed avascular osteonecrosis (AON) of the femoral head after RHT in seven children with pelvic germ cell tumours or rhabdomyosarcomas. METHODS: Although AON may develop in patients with malignancies treated with chemo- or radiotherapy alone, RHT might nevertheless contribute to the occurrence of AON. In order to determine potential risk factors for AON after RHT, this study analysed the relationship of AON to the patient's age, medical history and treatment parameters such as thermal dose equivalent and power output. RESULTS AND CONCLUSIONS: In the present study AON was associated with young age as well as intensity of hyperthermia indicated by high power levels that exceed 20 W per kg body weight and/or application of eight or more heat sessions as well as additional radiotherapy. Based on this observation, it was assumed that an optimized three dimensional thermal field modelling may be helpful to avoid hazardous temperatures in the femoral heads during RHT treatment and to reduce AON of the femoral heads.