Low,fixed dose defibrotide in management of hepatic veno-occlusive disease post stem cell transplantation

Objective/background

Hepatic veno-occlusive disease (VOD) is well recognized potentially serious regimen-related toxicity seen after stem cell transplantation. Severe VOD is associated with poor long-term outcomes with very high mortality. Besides supportive care, only defibrotide has been found to be effective in the management of VOD. The recommended dose of defibrotide is 25 mg/kg/d but there has been no classical dose finding study done for this drug. A higher dose of defibrotide is associated with increased risk of bleeding and this drug is prohibitively expensive. We report our experience of using fixed low dose of defibrotide in patients with VOD.

Simplified Technique for Orbital Prosthesis Fabrication: A Clinical Report

Loss of orbital content can cause functional impairment, disfigurement of the face, and psychological distress. Rehabilitation of an orbital defect is a complex task, and if reconstruction by plastic surgery is not possible or not desired by the patient, the defect can be rehabilitated by an orbital prosthesis. The prosthetic rehabilitation in such cases depends on the precisely retained, user‐friendly removable maxillofacial prosthesis. Many times, making an impression of the orbital area with an accurate record of surface details can be a difficult procedure. The critical areas are making a facial moulage, mold preparation, and attaching the retention device, particularly when eyeglass frames are used. This case focuses on these hindrance factors. A simple basket was used for the impression tray to obtain the facial moulage. A putty mold was used, and attachment of the prosthesis to a retention device was accomplished with positional distance. This method proves to be an economical and simple way of making an orbital prosthesis.     

Imaging signs in idiopathic intracranial hypertension: Are these signs seen in secondary intracranial hypertension too?

Background:

The purpose of this study was to evaluate the difference in the occurrence of the various “traditional” imaging signs of intracranial hypertension (IIH) on magnetic resonance imaging (MRI) in patients with idiopathic (IIH) and secondary intracranial hypertension.

Materials and Methods:

In a retrospective analysis, the MRI findings of 21 patients with IIH and 60 patients with secondary intracranial hypertension (41 with tumors; 19 with intracranial venous hypertension) were evaluated for the presence or absence of various “traditional” imaging signs of IIH (perioptic nerve sheath distention, vertical buckling of optic nerve, globe flattening, optic nerve head protrusion and empty sella) using the Fisher’s exact test. Odds ratios were also calculated. Statistical Package for the Social Sciences version 17.0 was used for statistical analysis. Subgroup analysis of the IIH versus tumors and IIH versus venous hypertension were performed.

Results:

Optic nerve head protrusion and globe flattening were significantly associated with IIH. There was no statistically significant difference in the occurrence of rest of the findings. On subgroup analysis, globe flattening and optic nerve head protrusion occurred significantly more often in IIH than in tumors. However, there was no statistically significant difference in the occurrence of any of these findings in patients with IIH and venous hypertension.

Conclusions:

IIH is a diagnosis of exclusion. While secondary causes of raised intracranial pressure (ICP) have obvious clinical findings on MRI, some conditions like cerebral venous thrombosis may have subtle signs and differentiating between primary and secondary causes may be difficult. In the absence of any evident cause of raised ICP, presence of optic nerve head protrusion or globe flattening can suggest the diagnosis of IIH.     

Spinal MR imaging in Vitamin B12 deficiency: Case series; differential diagnosis of symmetrical posterior spinal cord lesions

We report three cases of Vitamin B12 deficiency with symmetrical posterior spinal cord lesions and discuss the differential diagnosis, some of which are not well known. Because the degree of resolution of the clinical symptoms in subacute combined degeneration depends on early detection, MRI findings should not be missed.     

Blue-emissive two-component supergelator with aggregation-induced enhanced emission

Two-component organogels offer several advantages over one-component gels, but their design is highly challenging. Hence, it is extremely important to design new approaches for the crafting of two-component organogels with interesting optical and mechanical properties. Herein, we report the design of a new class of two-component supergelators obtained from the assembly between acid functionalized tetraphenylethylene (TPE)-based dendrons and alkylated melamine. No gelation behaviour is observed for the individual components, but interestingly, remarkable gelation behaviour is observed for their hydrogen-bonded complex. The primary driving force responsible for the gelation is the strong π–π stacking interaction of TPE units. Because of the strong π-stacking of TPEs in the gel state, the C(sp²)–C(sp²) bond rotation of the TPE segment is completely arrested in the gel state, which results in intense fluorescence emission of the gels. Furthermore, excellent elastic response is observed for the gels as evident from their high storage modulus compared to loss modulus values. Our results clearly demonstrate that by the appropriate selection of the molecular components, this approach can be applied for the creation of functional nanomaterials with emergent properties absent in the individual blocks.

Design of a novel class of two-component, highly emissive, low molecular weight supergelator is reported.     

The surgical management of patients who achieve a complete pathological response after primary chemotherapy for locally advanced breast cancer.

AIMS: Our aim was to investigate the role of minimal surgery in patients with locally advanced breast cancer (LABC) who achieve a complete pathological (pCR) response to primary chemotherapy (PC) and evaluate subsequent local recurrence (LRR), disease free survival (DFS) and overall survival (OS). METHODS: Between January 2000 and April 2005, 101 patients with operable LABC (T2, T3, N0 or N1, M0) who were not suitable for conservation surgery were treated with PC. Patients were treated with doxorubicin and cyclophosphamide for four cycles (100 patients) then four cycles with paclitaxel (91 patients). Post-PC surgery consisted of multiple core biopsies and axillary clearance for patients with a complete clinical and radiological response. If a pCR was confirmed no further breast surgery was performed. The remaining patients were treated with breast conserving surgery or mastectomy and axillary clearance as appropriate. Adjuvant radiotherapy was given to all patients. RESULTS: Breast conservation was possible in 60% of patients. Overall, 20 patients achieved a pCR of which 16 were confirmed on core biopsies alone. All patients were followed-up for a mean of 33.5 months (95% CI, 30.3-36.7). There were 10 local recurrences, four following mastectomy, four after wide excision and two after core biopsies. There was no difference in DFS (chi square=0.18; p=0.67) or OS (chi square=0.67; p=0.41) between patients achieving a pCR and the remainder. CONCLUSIONS: The local recurrence rate of these poor prognosis patients is similar to other reported series but higher than in our previously reported series of patients managed according to the same protocol. Our current management therefore now includes pre-treatment marking and subsequent surgical excision.     

Effects of the restriction of food and water intake on the distribution and retention of radioiodine in mice

The effects of the restriction of food and water intakes on gastrointestinal absorption, distribution to organs and excretion of 131I were investigated in C3H/He mice. The animals were divided into four groups and administered orally 37 kBq carrier-free Na 131I in 0.25 ml normal saline. One group of animals was given food and water ad libitum throughout the experimental period. Food and water to the remaining groups were restricted before and/or after the administration of 131I. The animals in each group were sacrificed 4 h and 24 h after administration, and the activity of 131I in thyroid, blood, liver, kidney, gastrointestinal tract, urine, feces, and carcass was measured. There was a significant increase in the retention of 131I in the thyroid and the concentration of 131I in the blood due to the restriction of food and water after the administration of 131I. In contrast, a significant decrease in the urinary excretion was observed in these animals. In those animals, which fasted before administration only, the retention of 131I in the thyroid and other organs were decreased. Therefore, for an accurate diagnosis and effective therapy with radioiodine as well as effective radiation protection, the intake of food and water should be taken into account.     

Cyclospora cayetanensis--an emerging coccidian parasite

Over the last decade increasing number of enteritis cases have been attributed to infection with a new coccidian sp that was named Cyclospora cayetanensis in 1993. Diarrhoea caused by this agent is clinically indistinguishable from cryptosporidiasis, isosporiasis and microsporidiasis but cyclospora infection are often very prolonged (upto 15 weeks) and may cause severe weight loss. Diagnosis is important because unlike diarrhoea caused by cryptosporidium and microsporidium, treatment with co-trimoxazole is effective. We report here a case of cyclosporiasis, to increase awareness of possibility of cyclospora infection in patients with prolonged diarrhoea. It should be considered in assessment of patients with unexplained prolonged diarrhoeal illness.