Recurrent intrahepatic pigmented stones after liver transplantation in a patient with hemoglobin SC disease: case report and review of the literature

Patients with hemoglobinopathies may have hepatic involvement, which if severe, can lead to chronic liver disease and a need for liver transplant. Here, we present a case of a 16-yr-old female adolescent who presented to our center with hemoglobin SC disease, obstructive jaundice because of pigmented intrahepatic biliary stones, and progressive liver disease. She underwent a successful liver transplant but a few years later, she developed recurrent cholangitis and graft dysfunction because of recurrent intrahepatic biliary stones. Recurrent formation of intrahepatic stones after liver transplant is a rare and severe complication in patients with hemoglobinopathies. We recommend hypertransfusion therapy and surveillance imaging studies after liver transplant for early detection and prevention of this complication.     

Liver and combined lung and liver transplantation for cystic fibrosis: analysis of the UNOS database

A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.     

Liver transplantation in children weighing 5 kg or less: analysis of the UNOS database

Arnon R, Annunziato R, Miloh T, Sogawa H, Van Nostrand K, Florman S, Suchy F, Kerkar N. Liver transplantation in children weighing 5 kg or less: Analysis of the UNOS database.
Pediatr Transplantation 2011: 15: 650–658. © 2011 John Wiley & Sons A/S. Abstract: LT is a major medical and surgical challenge in very small patients. Aim of the study is to determine the outcomes after LT in infants ≤5 kg at transplant in a large cohort of patients. Methods: Infants ≤5 kg who had LT between 10/1987 and 5/2008 were identified from the UNOS database. Risk factors for death and graft loss were analyzed by multivariate logistic regression. Results: Of 11 467 children, 570 (5%) were ≤5 kg at LT. Mean age and weight at LT were 0.11 ± 0.48 yr, 4.32 ± 0.74 kg, respectively. One‐ and five‐yr patient and graft survival were 77.7%, 72.2% and 66.1%, 57.6%, respectively. The primary cause of death was infection (25.9%). Recipient age was a predictor of graft loss. Patient and graft survival have improved over time. Life support at transplant was identified as a risk factor for both death and graft loss (p < 0.02, p < 0.01, respectively). Conclusion: LT recipients ≤5 kg have high mortality and graft loss. Over time, graft survival has improved, although it is still inferior to the overall reported outcomes of pediatric LT. Being on life support at transplant is a significant risk factor for death and graft loss in very small recipients.     

Heterogeneity in the ultrastructure of the mucous (goblet) cells of the rabbit palpebral conjunctiva

Aim: The purpose of this study was to assess objectively the ultrastructure of the secretory granules in rabbit conjunctival mucin‐producing ‘goblet’ cells. Method: The upper eyelids from five young adult dioestrous female rabbits were dissected out, stretched onto a cardboard support and prepared for transmission electron microscopy by repeated application of an isotonic two per cent glutaraldehyde fixative at room temperature. Post‐fixation treatment included osmium tetroxide and staining with uranyl acetate and lead citrate. Low magnification micrographs were taken of the goblet cells of the conjunctiva, printed at a magnification of approximately 6,000 and the number, size and features of the secretory granules assessed. Results: Across the entire palpebral conjunctiva of ail five rabbits, the majority of mucous cells displayed a goblet shape and the secretory granules were uniformly pale in staining. The average width of the goblet cells was 10.8 ± 1.1 μm and the diameter of the secretory granules was 0.82 ± 0.16 μm. However, in localised regions across the palpebral conjunctiva of two of the rabbits, some goblet cells were different in that the secretory granules had either a denser‐staining core, in which some of the granules were densely staining (while others were pale) or most of the granules were densely staining. These mucous cells had an average diameter of 10.3 ± 1.7 μm and the granule diameters averaged 0.88 ± 0.01 μm. For these abnormal goblet cells, inflammatory cells were found in their immediate vicinity. Occasionally, goblet cells were seen to be in the process of degranulation with associated apparent cell necrosis and the mucin granule diameter was close to 1 μm. Conclusions: The ultrastructure of the mucin‐containing secretory granules of the conjunctival mucous cells is not necessarily homogeneous in character and further attention needs to be given to the effects of localised inflammation in the tissue and to possible hormonal influences.     

Incorporating a service improvement project into an undergraduate nursing programme: A pilot study

Although service improvement projects have been incorporated into some postregistration nurse education modules in the UK, they have not been generally available to pre‐registration students. This paper reports on the implementation and evaluation of a pilot project in pre‐registration nurse education at one university in eastern England. It concludes that the nursing students found the project to be a useful addition to their course. Supportive reactions were obtained from nurse managers, mentors and academics. A need to improve communication with clinical areas through the use of web‐based materials was highlighted.     

A novel tool for improving the interpretation of isotope bone scans in metastatic prostate cancer

Objectives:The isotope bone scan (IBS) is the gold-standard imaging modality for detecting skeletal metastases as part of prostate cancer staging. However, its clinical utility for assessing skeletal metastatic burden is limited due to the need for subjective interpretation. We designed and tested a novel custom software tool, the Metastatic Bone Scan Tool (MetsBST), aimed at improving interpretation of IBSs, and compared its performance with that of an established software programme.Methods:We used IBS images from 62 patients diagnosed with prostate cancer and suspected bone metastases to design and implement MetsBST in MATLAB by defining thresholds used to identify the texture and size of metastatic bone lesions. The results of MetsBST were compared with those of the commercially available automated Bone Scan Index (aBSI) with regression analysis.Results:There was strong agreement between the MetsBST and aBSI results (R² = 0.9189). In a subregional analysis, MetsBST quantified the extent of metastatic disease in multiple bone sites in patients receiving multimodality therapy (radium-223 and external beam radiotherapy) to illustrate the differences in bone metastatic response to different treatments.Conclusion:The results of MetsBST and the commercial software aBSI were highly consistent. MetsBST introduces novel clinical utility by its ability to differentiate between the responses of different bone metastases to multimodality therapies.Advances in knowledge:MetsBST reduces the variability in assessment of tumour burden caused by subjective interpretation. Therefore, it is a useful aid to physicians reporting nuclear medicine scans, and may improve decision-making in the treatment of metastatic prostate cancer.     

Partial internal biliary diversion for Alagille syndrome: case report and review of the literature

This is a case report of the first patient with Alagille syndrome (AGS) to undergo a partial internal biliary diversion (PIBD) for the treatment of symptoms refractory to medical therapy. Alagille syndrome is a hereditary disease resulting in chronic cholestasis and hypercholesterolemia that can lead to severe and intractable pruritus and disfiguring and debilitating xanthomas. PIBD has proven to be an effective treatment option for other causes of cholestatic liver disease. This report reviews the immediate and 2-year follow-up of a patient after this surgical procedure. The results suggest that PIBD has potential to provide relief of intractable symptoms and improve the quality of life in patients with AGS while avoiding an external stoma. It does not, however, appear to prevent the progression of liver disease. Long-term follow-up is still needed.     

Analysis of T-cell receptor beta chain (T beta) gene rearrangements demonstrates the monoclonal nature of T-cell chronic lymphoproliferative disorders

We investigated the rearrangement patterns of the gene coding for the beta chain of the T cell receptor (T beta) in 11 patients with T-cell derived chronic lymphoproliferative disorders, including T-cell prolymphocytic leukemia (T-PLL) and T-cell chronic lymphocytic leukemia (T-CLL). We found that all five cases of T-PLL, and five of six cases of T-CLL, displayed T beta-gene rearrangements, clearly establishing their monoclonal nature. Clonality could not be determined in one case of T-CLL where the T beta gene was found unrearranged. Our results demonstrate that the majority of cases of both clinically aggressive T- PLL and clinically indolent T-CLL are monoclonal. These results suggest that the analysis of T beta gene rearrangements represents a valid tool for the differential diagnosis and clinical monitoring of T-cell derived chronic lymphoproliferative diseases.