Refractory sickle cell leg ulcer: is heparan sulphate a new hope?

Patients with sickle cell disease are known to have recurrent lower extremity ulcers that have a high pain score and are resistant to conventional means of wound therapy. This study reports the successful use of synthetic heparan sulphate (Cacipliq20^®, OTR3, Paris, France) in the treatment of a sickle cell ulcer that had failed to respond to several other means of treatment. Therapeutic success was assessed by complete wound coverage and vast improvement in pain score. This is the first study to report use of heparan sulphate in sickle cell ulcers.     

Laparoscopic or open cholecystectomy in patients with sickle cell disease: which approach is superior?

OBJECTIVE: To compare laparoscopic with open cholecystectomy in patients with sickle cell disease. DESIGN: Retrospective clinical study. SETTING: University hospital, Greece. SUBJECTS: 41 patients (22 men and 19 women) with sickle cell disease had laparoscopic cholecystectomy between September 1991 and June 1998. Each patient was matched for age, sex, year of operation, and number of preoperative transfusions with control patients with sickle cell disease who had open cholecystectomy. MAIN OUTCOME MEASURES: Duration of operation, postoperative stay in hospital, incidence of complications, and conversion to open operation. RESULTS: The mean operation time was 81.4 min (range 55-125) for open cholecystectomy and 64.2 min (range 45-90) for laparoscopic cholecystectomy (p < 0.01). Complications occurred in 5% (2/41) of the patients in the laparoscopic group and in 20% (8/41) of the patients in the open group (p = 0.04). The mean length of stay in hospital was 5.6 days (range 3-9) in the open group and 2.7 days (range 2-5) in the laparoscopic group (p < 0.01). Conversion to open operation was necessary in 2 (5%) patients. CONCLUSIONS: Laparoscopic cholecystectomy resulted in a shorter hospital stay with fewer postoperative complications than open operation in patients with sickle cell disease and may be the procedure of choice in the treatment of cholelithiasis in such patients.     

Paratesticular liposarcoma: What is the best therapeutic strategy?

Introduction  

Liposarcomas are neoplasms of mesodermal origin derived from adipose tissue and correspond to 10–14% of all soft tissue sarcomas. Paratesticular liposarcoma is very rare.     

Asymptomatic cholelithiasis in children with sickle cell disease: early or delayed cholecystectomy?

SUMMARY BACKGROUND DATA: Our study aimed to evaluate the role of elective laparoscopic cholecystectomy (LC) in children with sickle cell disease (SCD) and asymptomatic cholelithiasis and, furthermore, to determine whether the outcome is related to the operation timing. METHODS: The records of 30 children with SCD diagnosed with cholelithiasis from June 1995 to September 2005 were retraspectively reviewed. All 30 children were asymptomatic at the time of the first visit, and an elective LC was proposed to all of them. The operation was accepted in the period of study by 16 children and refused by 14. During medical observation, 10 of the 14 children who refused surgery were admitted for severe biliary colics. Acute cholecystitis was diagnosed by abdominal ultrasound in 3 cases and in 1 case choledocholithiasis, ultrasonographically suspected, was confirmed by magnetic resonance cholangiopancreatography (MRCP) and treated during endoscopic retrograde cholangiopancreatography (ERCP). All children, emergency admitted, underwent LC after the onset of symptoms. The patients were divided up into 2 groups (A: asymptomatic; B: symptomatic) depending on clinical presentation and operation timing and the respective outcomes were compared. RESULTS: Elective LC in asymptomatic children (group A) is safe with no major complications reported. During medical observation in children who refused elective surgery (group B), 6 biliary colics, 3 acute cholecystitis, and 1 choledocholithiasis were observed. Three sickle cell crises occurred in symptomatic children during biliary colics. The correlation between cholecystectomy performed in asymptomatic children (group A) and cholecystectomy performed in symptomatic children (group B) showed significant differences in the outcome. Morbidity rate and postoperative stay increased when children with SCD underwent emergency LC. CONCLUSIONS: Elective LC should be the gold standard in children with SCD and asymptomatic cholelithiasis to prevent the potential complications of biliary colics, acute cholecystitis, and choledocholithiasis, which lead to major risks, discomfort, and longer hospital stay.     

What is the role of decorin in diabetic kidney disease?

The small proteoglycan decorin may intercept the activity of the TGF-beta system. Decorin administration has been advocated as potential therapy in renal fibrotic diseases, because of the findings of a relative deficiency of decorin and a relative excess of TGF-beta in acute glomerulonephritis. Does a similar situation pertain in diabetic kidney disease? Activation of TGF-beta seems to be crucial to tissue injury in diabetic nephropathy, but until recently it has not been established whether decorin plays any role in the manifestations of this disease. We review evidence that a surfeit rather than a deficit in decorin expression exists in diabetic renal disease, and that there exists a negative feed-back loop whereby TGF-beta1 induces down-regulation of decorin expression. Rat and mouse mesangial cells as well as mouse proximal tubular cells in culture exhibit increased decorin mRNA levels in high ambient glucose. Decorin mRNA level in the kidney of streptozotocin-induced diabetes in mice is rapidly and significantly increased following the induction of diabetes. Thus, the available evidence suggests that renal decorin is not deficient in this disorder and hence decorin supplementation does not seem to be warranted. Rather, interception of the effects of TGF-beta seems to be an approach most likely to yield beneficial results in diabetic nephropathy.     

Multifocal hepatoblastoma: What is the risk of recurrent disease in the remnant liver?

PurposeMultifocal hepatoblastoma (HB) is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease.MethodsWe undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed.ResultsSixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver — both from the unifocal group. There were equivalent distant (lung) recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12.ConclusionMultifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development.Level of evidenceLevel III — Limited cohort analysis.     

Cholecystectomy in sickle cell disease patients: is there more acute chest syndrome after laparoscopy? A case controlled study

Laparoscopy is increasingly being recommended in order to reduce postoperative complications among sickle cell disease patients undergoing cholecystectomy. Acute chest syndrome is the most deadly of these complications. The purpose of this study was to assess if laparoscopic approach reduces postoperative complications related to sickle cell disease after cholecystectomy. A retrospective study of records of sickle cell patients who underwent cholecystectomy for cholelithiasis, from January 1990 to December 2005 was conducted. 136 sickle cell patients underwent surgery: 47 (34.5%) by laparoscopy and 89 (63.5%) by laparotomy. The mean operative time was 71.4+/-18.9 min in the laparoscopy group and 61.2+/-15.3 min in the laparotomy group (non-significant difference). The mortality was not significantly different between the two groups: one patient died in the open cholecystectomy group but no death occurred in the laparoscopic one. The morbidity related to the sickle cell disease was significantly higher in the laparoscopy group [n=5 (10.5%)] than in the laparotomy group [n=4 (4.5%)] (p=0.04). This difference was associated with a higher rate of acute chest syndrome in the laparoscopy group [n=4 (8, 5%)] compared to that in the laparotomy group [n=1 (1.1%)] (p=0.01). There were more complications related to sickle cell disease in the laparoscopy group due to acute chest syndrome. Thus, these data should be confirmed in further randomized studies which must be undertaken.     

Acinar cell carcinoma of the pancreas: is resection justified even in limited metastatic disease?

Background

Acinar cell carcinoma (ACC) of the pancreas is characterized by better long-term survival compared with the more common ductal adenocarcinoma, and prognosis is better in resected compared with nonresected patients. The aim of the present study was to investigate the role of surgery in ACC with limited metastatic disease.

Methods

All patients with histologically confirmed ACC treated at the investigators' institution between October 2001 and September 2009 were identified from a prospective database. Clinicopathologic details, perioperative results, and follow-up results were analyzed.

Results

Seventeen patients with nonmetastatic and metastatic ACC were identified. Initially, localized, locoregional, and metastatic disease was present in 5, 7, and 5 patients, respectively. Pancreatic resections were performed in 15 patients. In limited metastatic disease, additional liver resection was performed in 3 patients and omentectomy in 1 patient. In 2 patients, metachronous liver metastases were resected. With a median follow-up period of 36.5 months, overall 1-year, 2-year, and 3-year survival rates were 88%, 65%, and 47%, respectively. Survival of resected patients with metastatic and nonmetastatic disease showed no differences between the 2 groups.

Conclusions

ACC of the pancreas is a relatively rare tumor entity for which resection may result in long-term survival even in limited metastatic disease.     

Vitamin D in chronic kidney disease: is the jury in?

Vitamin D shows promise for improving diverse health outcomes among patients with chronic kidney disease. Observational studies of vitamin D medications have contributed important evidence for broad beneficial clinical effects of vitamin D beyond actions on bone. However, such studies are limited by the potential for confounding by indication. A large randomized controlled trial is now needed to test the hypothesis that vitamin D therapy improves clinical outcomes in patients with kidney disease.     

What is the place of internal urethrotomy in the treatment of urethral stricture disease?

As a treatment for male urethral stricture, internal urethrotomy (IU) has the advantages of ease, simplicity, speed and short convalescence. Various modifications of the single cold-knife incision in the 12 o'clock position have been proposed, but there are no prospective, randomized studies to prove their claims of greater efficacy. IU can be performed as an outpatient procedure using local anesthesia, with an indwelling silicone catheter for 3 days after the procedure. Complications of IU are usually minor, including infection and hemorrhage. The reported success rate of IU varies, mainly because of differences in the definition of success and the duration of follow-up. Strictures can recur, usually within 3-12 months of IU. There are several known risk factors for recurrence: a previous IU, penile and membranous strictures, long (>2 cm) and multiple strictures, untreated perioperative urinary infection and extensive periurethral spongiofibrosis. Repeated IU might be useful in patients who have a stricture recurrence more than 6 months after the initial procedure, but repeat IU offers no long-term cure after a third IU, or if a stricture recurs within 3 months of the first IU. Such patients should be offered urethroplasty. Repeated IU followed by long-term self-dilation is an alternative option for men with severe comorbidity and limited life expectancy, or those who have failed previous urethroplasty. Overall, IU has a lower success rate (+/-60%) than urethroplasty (+/-80-90%), but if used for selected strictures, the success rate of IU could approach that of urethroplasty.     

What is the impact of immunosuppressive treatment on the post-transplant renal osteopathy?

Although glucocorticoid therapy is considered to be the main pathogenic factor, a consistent body of evidence suggests that other immunosuppressants might also play an important role in the development of the post-transplant renal osteopathy (PRO) through their pleiotropic pharmacological effects. Glucocorticoids seem to induce osteoclasts’ activity suppressing the osteoblasts while data regarding other immunosuppressive drugs are still controversial. Mycophenolate mofetil and azathioprine appear to be neutral regarding the bone metabolism. However, the study analyzing any independent effect of antimetabolites on bone turnover has not been conducted yet. Calcineurin inhibitors (CNIs) induce trabecular bone loss in rodent, with contradictory results in renal transplant recipients. Suppression of vitamin D receptor is probably the underlying mechanism of renal calcium wasting in renal transplant recipients receiving CNI. In spite of an increased 1,25(OH)2 vitamin D level, the kidney is not able to reserve calcium, suggesting a role of vitamin D resistance that may be related to bone loss. More efforts should be invested to determine the role of CNI in PRO. In particular, data regarding the role of mammalian target of rapamycin inhibitors (mTORi), such as sirolimus and everolimus, in the PRO development are still controversial. Rapamycin markedly decreases bone longitudinal growth as well as callus formation in experimental models, but also lowers the rate of bone resorption markers and glomerular filtration in clinical studies. Everolimus potently inhibits primary mouse and human osteoclast activity as well as the osteoclast differentiation. It also prevents the ovariectomy-induced loss of cancellous bone by 60 %, an effect predominantly associated with a decreased osteoclast-mediated bone resorption, resulting in a partial preservation of the cancellous bone. At present, there is no clinical study analyzing the effect of everolimus on bone turnover in renal transplant recipients or comparing sirolimus versus everolimus impact on bone, so only general conclusions could be drawn. Hence, the use of mTORi might be useful in patients with PRO due to their possible potential to inhibit osteoclast activity which might lead to a decreased rate of bone resorption. In addition, it should be also emphasized that they might inhibit osteoblast activity which may lead to a decreased bone formation and adynamic bone disease. Further studies are urgently needed to solve these important clinical dilemmas.