Good outcome after liver transplantation for ALD without a 6 months abstinence rule prior to transplantation including post‐transplant CDT monitoring for alcohol relapse assessment – a retrospective study

Alcoholic liver disease (ALD) is the second most common indication for liver transplantation (LT). The utility of fixed intervals of abstinence prior to listing is still a matter of discussion. Furthermore, post‐LT long‐term observation is challenging, and biomarkers as carbohydrate‐deficient transferrin (CDT) may help to identify alcohol relapse. We retrospectively analyzed data from patients receiving LT for ALD from 1996 to 2012. A defined period of alcohol abstinence prior to listing was not a precondition, and abstinence was evaluated using structured psychological interviews. A total of 382 patients received LT for ALD as main (n = 290) or secondary (n = 92) indication; median follow‐up was 73 months (0–213). One‐ and five‐year patient survival and graft survival rates were 82% and 69%, and 80% and 67%, respectively. A total of 62 patients (16%) experienced alcohol relapse. Alcohol relapse did not have a statistically significant effect on patient survival (P = 0.10). Post‐transplant CDT measurements showed a sensitivity and specificity of 84% and 85%, respectively. In conclusion, this large single‐center analysis showed good post‐transplant long‐term results in patients with ALD when applying structured psychological interviews before listing. Relapse rates were lower than those reported in the literature despite using a strict definition of alcohol relapse. Furthermore, post‐LT CDT measurement proved to be a useful supplementary tool for detecting alcohol relapse.     

High 123I-MIBG uptake in neuroblastic tumours indicates unfavourable histopathology

Purpose

Scintigraphy using ¹²³I-metaiodobenzylguanidine (¹²³I-MIBG) is widely used for the detection of neuroblastic tumours. The aim of this study was to identify a possible correlation between the uptake intensity on ¹²³I-MIBG SPECT and histopathology of neuroblastic tumours.

Methods

¹²³I-MIBG SPECT examinations were performed in 55 paediatric patients with neuroblastic tumour and compared to histopathology after surgical resection or biopsy at a mean of 2 weeks after SPECT. For each lesion International Neuroblastoma Pathology Classification System (INPC) stage, mitosis karyorrhexis index (MKI), location and a semiquantitative tumour-to-liver count-rate ratio (TLCRR) were determined. Also, the presence or absence of MYCN amplification, p1 deletion, urine catecholamine and neuron-specific enolase blood levels at the time of scanning were recorded.

Results

In the 55 patients, 61 lesions were evaluated with ¹²³I-MIBG SPECT and corresponding histopathological findings were reviewed (11 ganglioneuroma, 11 ganglioneuroblastoma and 39 neuroblastoma). TLCRR was significantly higher in the neuroblastoma group (mean TLCRR 2.7) than in the ganglioneuroblastoma group (mean TLCRR 1.0) and ganglioneuroma group (mean TLCRR 0.7) at the time of primary diagnosis (p?<?0.001) and at follow-up (p?=?0.039). Intense ¹²³I-MIBG uptake was found in tumour tissue with a high mitotic activity (MKI-high or MKI-intermediate) after treatment. Four ganglioneuromas (36 %), three ganglioneuroblastomas (27 %) and six neuroblastomas (15 %) were ¹²³I-MIBG-negative.

Conclusion

In paediatric patients with peripheral neuroblastic tumours, strong ¹²³I-MIBG uptake indicates unfavourable histopathology. High uptake was seen in neuroblastomas and in tumours with a high mitotic activity.     

Ureteroscopic endoureterotomy

OBJECTIVE

To highlight the current status of ureteroscopic endoureterotomy (UE) by reporting extensive experience with the endoscopic management of ureteric strictures, with special emphasis on factors determining success, and by reviewing publications on the minimally invasive management of ureteric strictures.

PATIENTS AND METHODS

The study comprised 50 patients (mean age 53 years, range 18–85, equal sex distribution) with ureteric strictures of varying causes; all had their stricture treated endoscopically. The follow‐up was 0.5–9 years; 10 patients with recurrent strictures had two ipsilateral stents placed to try to improve the outcome, and eight patients with completely obliterating strictures were treated by ureteroscopic re‐canalization.

RESULTS

The site of stricture had no bearing on the eventual outcome. Patients with uretero‐enteric and malignant strictures did not fare so well. The most important predictor of failure was the length of the stricture, with failure in all seven patients with strictures of > 2 cm. In the 10 patients treated with two ipsilateral stents, eight were successful, which was very promising considering that these patients had recalcitrant strictures and placing one stent had previously failed. The overall success rate was 74%.

CONCLUSION

UE has become the procedure of choice for the initial management of ureteric strictures. Simple balloon dilatation is also effective in certain situations. The characteristics of the stricture often govern the eventual outcome. In properly selected cases success rates of ≈ 75% can be expected.

     

Is Hepatic Resection for Large or Multinodular Hepatocellular Carcinoma Justified? Results From a Multi-Institutional Database

Background The role of surgical resection in patients with large or multinodular hepatocellular carcinoma (HCC) remains unclear. This study evaluated the long-term outcome of patients with hepatic resection for large (>5 cm in diameter) or multinodular (more than three nodules) HCC by using a multi-institutional database.Methods The perioperative and long-term outcomes of 404 patients with small HCC (<5 cm in diameter; group 1) were compared with those of 380 patients with large or multinodular HCC (group 2). The prognostic factors in the latter group were analyzed.Results The postoperative complication rate (27% vs. 23%; P = .16) and hospital mortality rate (2.4% vs. 2.7%; P = .82) were similar between groups. The overall survival rates were significantly higher in group 1 than group 2 (1 year, 88% vs. 74%; 3 years, 76% vs. 50%; 5 years, 58% vs. 39%; P < .001). Among patients in group 2, five independent prognostic factors were identified to be associated with a worse overall survival: namely, symptomatic disease, presence of cirrhosis, multinodular tumor, microvascular tumor invasion, and positive histological margin.Conclusions Hepatic resection can be safely performed in patients with large or multinodular HCC, with an overall 5-year survival rate of 39%. Symptomatic disease, the presence of cirrhosis, a multinodular tumor, microvascular invasion, and a positive histological margin are independently associated with a less favorable survival outcome.     

Presentation of idiopathic retroperitoneal fibrosis in the pediatric population

Idiopathic fibrosis of the retroperitoneum is rare in childhood. The authors describe an 11-year-old boy who presented with progressive renal failure, bilateral hydronephrosis, hypertension, and elevated erythrocyte sedimentation rate (ESR) owing to retroperitoneal fibrosis. Ureterolysis was performed with improvement in his creatinine level and blood pressure. The soft tissue mass consisted of dense collagenous fibers consistent with retroperitoneal fibrosis. Postoperatively, he received steroids and azathioprine. Retroperitoneal fibrosis in the pediatric population is rare with only 23 cases reported in the English-language literature. Treatment includes pulsed steroid regimens, ureteral catheterization, and retroperitoneal exploration with ureterolysis. If allowed to progress, renal failure can result and lead to death. The etiology of retroperitoneal fibrosis in the pediatric patient may include autoimmune diseases, infection, and neoplasm, but most cases are idiopathic. Retroperitoneal fibrosis should be considered in patients with an elevated ESR, hypertension, renal failure, and hydronephrosis. Evaluation also should include a search for autoimmune diseases and malignancy.     

Carcinoid tumor in a Meckel's diverticulum: hypothesis on mutual embryological origin

Congenital defects presenting at an adult age are subject to diagnostic errors because of their relative rarity and often odd clinical presentation. We illustrate a 63-year-old male patient with multiple ileal carcinoid tumors along with a carcinoid tumor in Meckel's diverticulum. The Meckel's diverticulum is a congenital abnormality arising from a patent vitelline duct and is found at the anti-mesenteric side of the ileum. In the Meckel's diverticulum, ectopic tissue or neoplasms are sometimes found, which may lead to intussusception, hemorrhage, or inflammation. The carcinoid tumor is a neuroendocrine neoplasm originating from the neural crest and is diagnosed at virtually all ages. It may exhibit malignant behavior but generally has a mild clinical course. It is most often found in endodermally derived organs, especially the digestive and pulmonary tracts, and coexists frequently with malignant tumors, predominantly of endodermal origin such as colorectal carcinoma. The carcinoid tumor and Meckel's diverticulum are known to coincide, whereas the carcinoid is not really considered to be an embryologically determined defect. We hypothesize that both lesions are derived from local erroneous interaction among the neural crest and the endoderm.     

Prognosis after hepatic resection for stage IVA hepatocellular carcinoma: a need for reclassification

OBJECTIVE: To evaluate whether the prognosis of the four categories of patients with hepatocellular carcinoma (HCC) classified as stage IVA in the tumor-node-metastasis (TNM) classification of the International Union Against Cancer (UICC) is homogeneous. SUMMARY BACKGROUND DATA: Hepatic resection has been proposed as the treatment of choice for patients with TNM stage IVA HCC, which consists of four different categories. It is unknown whether the prognosis of the four categories of patients is homogeneous. METHODS: Clinicopathologic and follow-up data of 106 patients with resection of stage IVA HCC from 1989 to 2000 were prospectively collected. Survival results of the four categories of stage IVA patients were compared. RESULTS: Among stage IVA patients, survival was significantly worse in those with tumors involving a major branch of the portal or hepatic veins than in those with tumors invading adjacent organs, bilobar multiple tumors, or perforated visceral peritoneum. There were no significant differences in survival among the latter three groups. By Cox regression analysis, invasion of the portal or hepatic veins and presence of cirrhosis were independent adverse prognostic factors of overall survival among stage IVA patients, and invasion of the portal or hepatic veins was the only significant adverse prognostic factor of disease-free survival. CONCLUSIONS: The prognosis of the four categories of patients with stage IVA HCC under the current UICC TNM staging was not homogeneous. A refined classification of stage IV HCC is needed to take into consideration the worse prognosis associated with tumor invasion of a major branch of the portal or hepatic veins.     

Graft injury in relation to graft size in right lobe live donor liver transplantation: a study of hepatic sinusoidal injury in correlation with portal hemodynamics and intragraft gene expression

OBJECTIVE: To investigate the degree and mechanism of hepatic sinusoidal injury in different graft sizes in right lobe live donor liver transplantation (LDLT). SUMMARY BACKGROUND DATA: Liver grafts from living donors are likely to be small-for-size for adult recipients. Graft injury after reperfusion is common, but the mechanism and degree of injury remain unclear. The hepatic sinusoidal injury in different graft sizes and its relationship with portal hemodynamics and intragraft gene response at the early phase after reperfusion have not been studied in right lobe LDLT. METHODS: From May 2000 to November 2001, 40 adults receiving right lobe LDLT had portal pressure measured continuously before and after reperfusion. Liver biopsies were taken before and after reperfusion for detection of vasoregulatory genes (endothelin-1 and endothelial nitric oxide synthase) and heat shock genes (heat shock protein 70 and heme oxygenase-1), and electron microscope examination. Blood samples from the portal vein and suprahepatic inferior vena cava were taken for the measurement of plasma nitric oxide level. RESULTS: The recipients were grouped according to the ratio of graft weight to estimated standard liver weight: group 1 (n = 10), less than 40%; group 2 (n = 21), 40% to 60%; and group 3 (n = 9), more than 60%. The portal pressures recorded after reperfusion in group 1 were significantly higher within 30 minutes of reperfusion than those in groups 2 and 3. After reperfusion, the intragraft endothelin-1 mRNA level in group 1 increased by 161% of the basal level but decreased by 31.5% and 62% of the basal level in groups 2 and 3, respectively. The intragraft mRNA level of heme oxygenase-1 in groups 1 and 2 decreased by 75.5% and 25.3% of the basal level respectively but increased by 41% of basal level in group 3. The intragraft protein level of heat shock protein 70 decreased by 50 ng/mL after reperfusion in group 1 but increased by 12.4 ng/mL and 0.6 ng/mL in groups 2 and 3, respectively. The portal vein plasma nitric oxide level decreased more significantly after reperfusion in group 1 than in group 2. Electron microscope examination of liver biopsies in group 1 showed tremendous mitochondrial swelling as well as irregular large gaps between the sinusoidal lining cells. There were two hospital deaths in group 1 and none in the other two groups. CONCLUSIONS: Patients implanted with grafts less than 40% of standard liver weight suffered from transient portal hypertension early after reperfusion. The phenomenon was accompanied by intragraft upregulation of endothelin-1 and ultrastructural evidence of sinusoidal damage. The transient portal hypertension after reperfusion, subsequent endothelin-1 overexpression, and plasma nitric oxide level reduction, together with downregulation of heme oxygenase-1 and heat shock protein 70, may account for the small-for-size graft injury.     

Sperm retrieval procedures and intracytoplasmatic spermatozoa injection with epididymal and testicular sperms

INTRODUCTION: Male infertility caused by azoospermia due to non-reconstructable obstruction or non-obstructive azoospermia can be treated by microsurgical epididymal aspiration (MESA) or testicular sperm extraction (TESE) followed by an intracytoplasmatic spermatozoa injection (ICSI). MATERIAL AND METHODS: From 9/93 to 6/01, we carried out 1,025 ICSI procedures with aspirated epididymal or testicular sperms in 684 cases. 163 ICSI cycles were performed with epididymal sperms and 862 ICSI cycles with testicular sperms or spermatids. The TESE was carried out by open biopsy, frequently in a multilocular technique. The aspirated spermatozoas were used after cryopreservation (frozen) or immediately after aspiration (fresh). RESULTS: 538 patients had obstructive azoospermia or ejaculation failure. In 487 cases the underlying cause of azoospermia was an impaired spermatogenesis, following maldescensus testis, chemotherapy, radiotherapy, or caused by Sertoli-cell-only syndrome, a genetic disorder or an unknown etiology. The transfer rates, pregnancy rates and birth rates per ICSI cycle showed no statistically significant differences between testicular and epididymal sperms in the cases of seminal obstruction (28% average birth rates in both cases). However, highly significant was the difference in birth rates with regard to the underlying cause of infertility. In contrast, in treating non-obstructive azoospermia we observed a birth rate of 19% per cycle. In all patient groups the birth rate with fresh spermatozoas did not differ from those with cryopreserved spermatozoa. 40% of patients after multilocular TESE showed clinical signs of testicular lesion. CONCLUSION: The underlying cause of azoospermia is the most important factor for the outcome of ICSI using epididymal and testicular sperms. In cases of non-obstructive azoospermia, the pregnancy rate is low compared with the results in cases of obstructive azoospermia. There is no difference between fresh and cryopreserved sperms. TESE with ICSI is the most efficient treatment of azoospermia caused by hypergonadotropic hypogonadism. The morbidity of the TESE procedure is highly relevant and must be considered if this technique is indicated.     

Steady-state free-precession (SSFP) cine MRI in distinguishing normal and bicuspid aortic valves

PURPOSE: To assess the diagnostic sensitivity and specificity of double-oblique true fast imaging with steady-state precession (SSFP) cine MRI in distinguishing normal and bicuspid aortic valves. MATERIALS AND METHODS: Echocardiograms on patients referred for MRI of the heart and thoracic aorta over a four-year period were reviewed retrospectively. A total of 17 patients with bicuspid aortic valve were identified and compared to 21 randomly chosen control patients. All patients had double-oblique SSFP (True FISP) cine MRI of the aortic valve independently assessed by two radiologists in a blinded fashion, and graded as bicuspid or normal. Image quality was graded as 1, 2, or 3. Discordance was resolved by consensus. RESULTS: A total of 38 cases were reviewed (27 men, 11 women; age range = 15-67 years, mean = 25.33 years). Interobserver agreement was 0.97 (36/38 cases). One case of normal tricuspid valve was reported as bicuspid by both readers (false-positive). All cases in which disparity arose were rated suboptimal by both readers (grade 2 or 3). Consensus review yielded sensitivity = 100%, specificity = 95.2%, positive predictive value = 94.4%, and negative predictive value = 100%; overall diagnostic accuracy was 97.36%. Interobserver agreement was 0.97. CONCLUSION: Double-oblique True FISP cine MR imaging of the aortic valve is 100% sensitive and 95% specific in distinguishing normal and bicuspid aortic valves.