Esophageal stenosis in epidermolysis bullosum: a challenge for the endoscopist

Background/Purpose

Esophageal stenosis is a severe complication in dystrophic epidermolysis bullosa (EB). Endoscopic dilations may cause mucosal injury with stricture recurrence. Our aim was to describe our referral EB-center experience on safety and long-term efficacy of fluoroscopically guided balloon dilation without endoscopy.

Methods

Over 14 years, 34 patients with EB, previously evaluated with barium esophagogram for dysphagia, underwent balloon esophageal dilation. Under fluoroscopy, a guide wire was introduced via a nostril into the stomach. A 12-mm pneumatic balloon, which passed over the wire, was filled using radio-opaque contrast, dilating the stricture. Orotracheal intubation was avoided. Antibiotics, dexamethasone, and proton-pump inhibitors were administered. Study approval was obtained from our ethical board.

Results

Ninety-three dilations were performed. Seventeen patients had a single stenosis. The mean age of onset was 18 years (range, 3-47 years). Thirteen patients underwent one dilation. In 6 cases, endoscopy was necessary to visualize the esophageal lumen. Complications included cervical esophageal perforation (2) and transitory dysphagia (10). Thirty patients were feeding within 24 hours. During the follow-up, 2 patients required a gastrostomy, and 2 patients underwent fundoplication for gastroesophageal reflux disease.

Conclusions

Fluoroscopically guided balloon dilation in EB is a safe and well-tolerated procedure. An experienced endoscopy team is necessary in certain cases.     

Bile leak from the hepatic bed after laparoscopic cholecystectomy

The aim of our study was to identify the best treatment for bile leakage from the gallbladder or hepatic bed as a result of laparoscopic cholecystectomy. Two hundred and fifty laparoscopic cholecystectomies were performed in our department from January 1997 to January 1999 and bile leak was identified in 5 cases (2%). In one case, a right subphrenic collection was detected and resolved with a percutaneous drainage. At ERCP all cases showed a small leak from an accessory hepatic duct (2 pts.) or from the hepatic bed (3 pts.), successfully managed with an immediate endoscopic sphincterotomy, with placement of a nasobiliary tube or a biliary endoprosthesis. The incidence of leakage from an accessory hepatic or from Luschka's duct is not well known. This complication can be successfully managed with endoscopic treatment.     

Atypical Lipomatous Tumors: Should They be Treated Like Other Sarcoma or Not? Surgical Consideration from a Bi-Institutional Experience

Background

To identify the best surgical approach to atypical lipomatous tumors we reviewed 171 patients who underwent surgery at two sarcoma referral centers with different surgical policies.

Methods

Of the 151 patients (88 %) with primary tumors, 95 were treated at Institution A and 76 were treated at Institution B. At Institution A, a wide surgical resection, including a slight cuff of soft tissue around the mass, was adopted, which was defined as marginal resection (MR) according to the Enneking classification. At Institution B, a simple tumor resection (SR), according to the Enneking classification, was employed. En bloc surgical resection was the goal in both centers. The primary outcomes of the study were local recurrence-free survival (LRFS), incidence of secondary dedifferentiation at recurrence, and presence of residual tumor after re-excision.

Results

Sixteen patients (9 %) had local recurrence. The 10-year LRFS was 82 %. No cases of secondary dedifferentiation were observed. Residual tumor after re-excision was found in 46 % of cases. In univariate analysis, sclerosing subtype, tumor rupture, and SR were unfavorable prognostic factors for LRFS. Sclerosing subtype and tumor rupture were independent prognostic factors for LRFS in multivariate analysis. SR was significantly associated with tumor rupture.

Conclusions

Sclerosing subtype and tumor rupture are unfavorable prognostic factors for local recurrence. MR is associated with a lower risk of tumor rupture than SR. Neurovascular and major muscle resections are not necessary in principle. Re-excision after unplanned surgery is not always mandatory. A preoperative core needle biopsy could be useful in identifying the sclerosing subtype.     

Prognostic significance of estrogen receptors in 405 primary breast cancers: a comparison of immunohistochemical and biochemical methods

Over the last few years, estrogen receptor determinationby means of immunohistochemistry has been extensively used.The aim of this study was to comparethis technique with estrogen receptor determination by meansof dextran-coated charcoal, and to evaluate whether oneof the two methods is more predictive ofprognosis. Estrogen receptors were determined by means ofboth the dextran-coated charcoal method and immunohistochemistry in405 patients with primary breast cancer; age, pathologicaltumor size, nodal status, and progesteron receptors bydextran-coated charcoal method were also recorded. The disease-freeand overall survival probabilities were estimated using theproduct-limit method; Cox's proportional hazard model was usedto evaluate the prognostic role of estrogen receptorsas determined by the two methods.There appears to be a close association betweenestrogen receptor determination by the two methods (81.5%of concordant results) and their prognostic role wassimilar, even when the patients were divided intodifferent groups (on the basis of their estrogenreceptor status) and adjustments for the effect ofother prognostic variables were taken into account. Ourstudy shows that the two methods can beused indifferently to evaluate estrogen receptor status asa prognostic factor in breast cancer patients.     

Macromolecular permeability of chick wing microvessels: an intravital confocal study

The development of the vertebrate limb requires the formation of a normal vasculature to nurture the soft and hard tissue phenotypes. The pattern of embryonic limb bud vessels has been extensively studied, but little is known about the permeability characteristics of the developing circulation. In the present study, the microvascular endothelial cell phenotype was examined by in vivo confocal microscopy following the systemic injection of a graded series of fluorescent dextrans (40,000, 70,000, 150,000 molecular weight) into chick embryos at stages 21–23 in order to determine how selective is the endothelial lining of microvessels as a partition between the blood vessels and the interstitium. Videodensitometry, over a gray scale range of 0–255, was used to quantitate the amount of tracer found within the interstitial compartment of the limb. The tracers of larger molecular weight (70,000, 150,000) were confined exclusively to the vascular lumina, whereas that of smaller molecular weight (40,000) was found to cause perivascular brightening due to extravasation into the surrounding interstitium. The reported differences in permeability were not dependent upon the stage of the embryo used in this study, but were due to the size of the tracer. These data indicate that embryonic wing microvessels demonstrate permselectivity to macromolecular efflux across the endothelium. The present results provide a basis for additional studies concerned with the dynamic characteristics of the limb microvasculature and challenge our concepts about the role of diffusible morphogens in vertebrate limb development.     

Risk of neoplastic transformation in asymptomatic radial scar. Analysis of 117 cases

Background Radial scar (RS) is a benign breast lesion but a variable percentage of cases are associated with atypical epithelial proliferations and cancer. Previous studies have shown that patient age and the size of RS are correlated to a potential neoplastic transformation. Method We collected 117 asymptomatic patients with suspected RS following a mammogram, histologically confirmed. The clinical, pathological and immunophenotypical analysis is reported. The cases are subdivided into three different groups: (1) RS “Pure”, without epithelial atypia; (2) RS associated with epithelial atypical hyperplasia; (3) RS with cancer. Results “Pure” RS was detected in 55 patients (47%); the mean age was 48.1 years and the mean size 0.94 cm. RS associated with atypical epithelial hyperplasia was identified in 25 cases (21%) with a mean age of 53.1 years and a mean size of 0.98 cm. Carcinoma in RS was observed in 37 cases (32%); the mean age was 55.5 years and the mean size was 1.16 cm. The mean age was statistically significant (P = 0.004) in separating RS with cancer from the two other RS groups. The size of RS was not sufficiently statistically significant (P = 0.2) to differentiate the risk. Atypical lesions and cancers showed a morphology and marker of low-grade aggressiveness. Conclusion RS seems to represent a natural model of carcinogenesis starting from a proliferative lesion in patients of less than 50 years of age and developing into an atypical and later into a carcinomatous lesion. The fact that most carcinomas arising in RS are low grade also favors this hypothesis. All RS should be excised.     

The effect of fruits and vegetables on urinary stone risk factors

BACKGROUND: The overall effect of fruit and vegetable intake on urinary stone risk profile is not yet known. METHODS: We studied the effect of a two-week period of fruit and vegetable elimination on urinary stone risk profile in 12 normal adults, and of supplementing the diet with a fair quantity of low-oxalate fruits and vegetables in 26 idiopathic calcium stone formers characterized by hypocitraturia and a very low fruit and vegetable intake in their usual diet. RESULTS: In the normal subjects, the elimination of fruits and vegetables from the diet decreased the urinary excretion of potassium (-62%), magnesium (-26%), citrate (-44%) and oxalate (-31%), and increased that of calcium (+49%) and ammonium (+12%) (P < 0.05 for all). The relative saturation for calcium oxalate and calcium phosphate increased from 6.33 to 8.24 (P = 0.028), and from 0.68 to 1.58 (P = 0.050), respectively. In the hypocitraturic stone formers, the introduction of these foods in the diet increased urinary volume (+64%), pH (from 5.84 to 6.19), excretion of potassium (+68%), magnesium (+23%), and citrate (+68%), while it decreased the excretion of ammonium (-18%) (P < 0.05 for all). The relative saturation for calcium oxalate and uric acid fell from 10.17 to 4.96 (P < 0.001), and from 2.78 to 1.12 (P = 0.003), respectively. CONCLUSION: The total elimination of fruits and vegetables in normal subjects brings about adverse changes in the urinary stone risk profile that are only partially counterbalanced by a reduction in oxalate. In contrast, the addition of these foods to the diet of hypocitraturic stone formers not used to eating them not only significantly increases citrate excretion without affecting oxalate excretion, but also decreases calcium oxalate and uric acid relative saturation.     

Are low plasma levels of 25-(OH)vitamin D a major risk factor for hyperparathyroidism independent of calcitriol in renal transplant patients?

BACKGROUND: Recently, some studies have emphasized the role of plasma 25-(OH)vitamin D (25OHD) levels in mineral metabolism dysregulation in chronic kidney diseases (CKDs). However, to date little attention has been paid to 25OHD metabolism abnormalities after renal transplantation (Tx). This cross-sectional study aimed to focus on its role in mineral metabolism dysregulation in functioning Tx. METHODS: Twenty-eight out of 75 Caucasian Tx patients were selected following strict inclusion and exclusion criteria. Two blood samples were effected at the end of the winter for the measurements of plasma 25OHD and calcitriol levels. Serum creatinine (Cr), alkaline phosphatase (SAP), immunoreactive intact parathyroid hormone (PTH), electrolytes and 24-hr proteinuria were also determined. The Kolmogorov-Smirnov test was used to evaluate the data distribution: serum Cr, Cr clearance, dialysis duration and PTH levels were non-normally distributed and were log-transformed. Values of p<=0.01 were assumed as statistically significant. RESULTS: Median serum Cr and PTH levels were, respectively, 1.0 mg/dL and 90.0 pg/mL (range 27-420; normal range 10-65); most of our Tx patients (78.5%) had serum PTH levels above the upper limit of normal values. Mean plasma 25OHD concentration was 19.6 +/- 8.9 SD ng/mL (range: 6-36). None had levels <5 ng/mL (severe deficiency); 10 patients (35.7%) had mild deficiency (5-15 ng/mL); 14 patients (50%) had vitamin D insufficiency (16-30 ng/mL); and only four patients (14.3%) had target levels (>30 ng/mL). Mean plasma calcitriol levels were 69.7 +/- 19.0 pg/mL (range 47-105; normal range 35-85). They were not significantly correlated to plasma 25OHD levels. Proteinuria (292.6 +/- 147.0 mg/24 hr) inversely correlated to plasma 25OHD levels (r=-0.480; p<0.01). The bivariate correlation analysis between logPTH and the other parameters showed a significant correlation for SAP (r=0.494; p=0.008), plasma 25OHD levels (r=-0.442; p=0.01), proteinuria (r=0.452; p=0.01), log serum Cr (r=0.551; p=0.002) and log Cr clearance (r=-0.534; p=0.003). The other parameters did not correlate significantly with logPTH, notably plasma calcitriol and serum phosphate levels. Only the parameters significantly correlated to logPTH in the bivariate correlation analysis were included in the back stepwise multiple linear regression analysis as independent variables (model: p<0.0001; R2=0.54): among them, only plasma 25OHD levels (Beta=-0.486; p=0.001) and log serum Cr levels (Beta=0.589; p=0.0002) were the dependent variable logPTH predictors. CONCLUSIONS: This cross-sectional study demonstrated that plasma calcitriol levels in a highly selected group of Tx patients were normal and not significantly correlated to either plasma 25OHD or serum PTH levels. Most patients (85.7%) had plasma 25OHD levels below the target value of 30 ng/mL; the latter were inversely correlated with serum PTH levels. Therefore, our study strengthens the suggestion that low plasma 25OHD levels are a major risk factor for secondary hyperparathyroidism (sHPTH) in Tx patients and stresses the importance of monitoring these patients.