The congenital cystic dilatation of the liver and bile ducts (CDB): our experience

The congenital cystic dilatation of the liver and bile ducts (CDB) is a very uncommon disease. It often appears in females and most frequently in paediatric patients. Its etiopathogenesis is not yet completely defined. Its evolution includes, together with several complications, the malignant transformation. The Authors present the outcomes of their experience based on a series of 5 patients observed from 1991 till today. Apart from the rarity of this disease, their series is so interesting because 4 out of 5 are adult patients and the fifth one is over 15. In addition, differentiating from the literature trend, the group included only male patients. Are also discussed the most important aspects referring to nosology, epidemiology, etiopathogenesis, clinical pattern and its evolution of CDB, looking over a wide review too. The Authors extensively examine the diagnostic problems; owing to that the patients were observed in the period 1991-1999 it is necessary to clarify that some imaging methods, i.e. CSTscan and the bile duct MNR, were not yet introduced. The surgical treatment has been investigated as well, developed during its historical evolution until nowadays and our solutions for the 5 cases have been presented and discussed. In their series the Authors haven't ever observed during hospitalisation a malignant transformation.     

Small bowel tumours in emergency surgery: specificity of clinical presentation

BACKGROUND: Despite advances in diagnostic modalities, small bowel tumours are notoriously difficult to diagnose and are often advanced at the time of definitive treatment. These malignancies can cause insidious abdominal pain and weight loss, or create surgical emergencies including haemorrhage, obstruction or perforation. The aim of the present study was to describe the clinical presentation, diagnostic work-up, surgical therapy and short-term outcome of 34 patients with primary and secondary small bowel tumours submitted for surgical procedures in an emergency setting and to look for a correlation between clinical presentation and the type of tumours. METHODS: From 1995 to 2005, 34 consecutive surgical cases of small bowel tumours were treated at the Department of Emergency Surgery of St Orsola-Malpighi University Hospital, Bologna, Italy. Clinical and radiological charts of these patients were reviewed retrospectively from the department database. RESULTS: All patients presented as surgical emergencies: intestinal obstruction was the most common clinical presentation (15 cases), followed by perforation (11 cases) and gastrointestinal bleeding (eight cases). Lymphoma was the most frequent histologic type (nine patients), followed by stromal tumours (eight patients), carcinoids (seven patients), adenocarcinoma (seven patients) and metastasis (three patients). Of the nine patients with lymphoma, eight were perforated, all patients with stromal tumours had bleeding, and all carcinoids patients had bowel obstruction. There were two patients with melanoma metastasis, both had bowel intussusception. Resection of the neoplasm was carried out in 32 patients and two patients were deemed unresectable and received a palliative procedure. CONCLUSIONS: The present study shows that there is a correlation between small bowel tumours and clinical emergency presentation: gastrointestinal stromal tumours (GIST) mostly bleed; carcinoids make an obstruction; lymphomas cause a perforation; and melanoma metastasis causes intussusception.     

Blunt liver trauma: efficacy of non-operative management

All haemodynamically stable patients with blunt abdominal trauma can be managed conservatively by non-operative management which is the treatment of choice in 80% of liver trauma. Non-operative management, when feasible, yields very good results in terms of survival, need for blood transfusions, morbidity and reduction of hospital stay. The Authors retrospectively analyse their recent experience with liver trauma. Of 53 patients with blunt liver trauma, 36 underwent surgical treatment whereas 17 haemodynamically stable patients received non-operative management. After CT scans, all patients on non-operative management underwent haemodynamic monitoring, US and blood examinations. A percentage of 47.1 % had a > or = grade III trauma. The mortality and morbidity rates were 0% and no blood transfusions were needed. The efficacy of non-operative management was 100% and none of the patients needed subsequent surgical treatment. Our clinical experience demonstrates that non-operative management is feasible and effective even for higher grade traumas, but always requires strict clinical, haemodynamic and US monitoring and careful patient selection. Current progress in the field and recent evidence reported in the literature indicate the likelihood of further developments and more widespread utilisation of non-operative management in liver trauma.     

Left ventricular assist device implantation in a patient with congenitally corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare anomaly characterized by atrioventricular and ventriculo-arterial discordance and several other malformations that eventually lead to heart failure. We describe the case of a 53-year-old woman with ccTGA and aortic insufficiency who was a candidate for heart transplantation due to end-stage congestive heart failure. Her condition deteriorated before a suitable donor heart could be found; therefore, we placed a left ventricular assist device in the right (systemic) ventricle. Concomitantly, we removed the aortic (systemic) valve, closed the aortic annulus with a bovine pericardial patch, and repaired the mitral valve. The patient recovered uneventfully and was discharged from the hospital 2 months postoperatively. She underwent cardiac transplantation approximately 6 months later and continued to do well after 18 months.     

Long-term follow-up of psychological distress following earthquake experiences among working Italian males: a cross-sectional analysis

This report details relationships between earthquake exposures in 1980 and 1983 to 1984 and psychological distress reported in 1994. Participants are 555 Italian male factory workers from Naples, Italy. Those men who experienced damage from the 1980 quake reported higher levels of psychological distress (across several dimensions of the Symptom Checklist) than those without damage; additionally, 30% of these men reported symptoms of posttraumatic stress disorder (PTSD). While men evacuated as a result of the 1983 to 1984 Bradyseism earthquakes did not report higher distress levels (Symptom Checklist) than their nonevacuated colleagues, they did report more PTSD-like symptoms than those not evacuated. Financial loss from the Bradyseism quakes was associated with higher distress across all measures (seven Symptom Checklist dimensions and presence of PTSD symptoms). Additionally, social network disruptions following 1983 to 1984 evacuation were associated with greater distress (not all measures). These findings suggest that psychological distress from natural disasters may be very long lasting.     

Pathogenesis of some neurological immune ultrastructural and morphometrical observations on rat thymus

Numerous studies on neuro-immuno-modulation indicate that the thymus is involved in many neurological diseases, including experimental allergic encephalomyelitis (EAE). Twenty Lewis rats were induced for EAE. At X, XII, XX and XXX days post-inoculation the animals were killed, and the thymus was recovered and harvested. Specimens of thymus were submitted to morphological light microscopy analysis (1% toluidine blue) and ultra-structural analysis (transmission electron microscopy). Significant morphometric data were collected by examining the images quantitatively and by statistically analysing the values. Our results show that the microenvironment of the thymus is severally involved in acute EAE. Thymocytes and reticular epithelial cells show many changes which are closely related to the pathogenesis of EAE. In particular we observed: (1) inside the cell an increase in intra-cytoplasmic vacuoles, and changes in the thickness of the nuclear membrane, mitochondria, rough endoplasmic reticulum, cellular inter-digitations and cellular electron-density; (2) outside the cell an increase in pericellular translucent halo, intercellular spaces, intercellular contacts and apoptotic and necrotic figures. The evidence of a thymic role in MS may suggest the intriguing therapeutic concept of thymectomy in the management of this neurological disease.     

Fibroblast growth factor 10 (Fgf10) invalidation results in anorectal malformation in mice

Background/purpose

Anorectal malformations occur in 1 per 4,000 live births and represent a surgical challenge. Although critically important, the basic mechanisms of normal anorectal union are incompletely understood. Fgf10 signaling is known to serve a key role in mesenchymal/epithelial interactions in many organ systems including the gastrointestinal tract (GIT). The authors therefore hypothesized that Fgf10 signaling has a central role in normal anorectal development.

Methods

Fgf10 expression in wild-type (Wt) embryos was evaluated using whole-mount in situ hybridization. Wt and Fgf10^−/− embryos were harvested from timed pregnant mothers at E12.5 through E17.5 and were analyzed for anorectal phenotype.

Results

Wt development of union between anorectal structures is completed between E12.5 and E13.5 with luminal communication between distal rectal epithelium and anus. Fgf10 is discreetly expressed at E12.5 in the distal rectum. Fgf10^−/− mutants show failure of union of the rectum and anus at an early stage (E13.5) and near term (E17.5).

Conclusions

Fgf10 is expressed in the rectum at the time when anorectal continuity is established, indicating a role in normal anorectal development. Fgf10 invalidation (Fgf10^−/− mutant) results in a genetically reproducible anorectal malformation phenotype. Fgf10 function is critical for normal anorectal development.     

Non-invasive diagnosis of Helicobacter pylori infection: simplified 13C-urea breath test, stool antigen testing, or DNA PCR in human feces in a clinical laboratory setting?

OBJECTIVES: (1) To compare two stool antigen EIAs (HpSA, FemtoLab) and PCR of ureaseA and cagA in feces, with (13)C-urea breath test (UBT). (2) To ascertain whether a simplified UBT (breath collection time = 10 min) is as reliable as the standard assay (30 min). DESIGN AND METHODS: Helicobacter pylori status was recorded in Group 1 (n = 187) by UBT, H. pylori stool antigen, ureA and cagA PCR in feces. UBT with 10, 20 and 30 min sampling was performed in Group 2 patients (n = 283). RESULTS: The sensitivity and specificity of HpSA, FemtoLab, and ureA were 67% and 99%, 90% and 96%, 35% and 98%, respectively. cagA results were positive in 16/48 H. pylori-positive, and in 5/100 H. pylori-negative patients. The results of UBT with a 10- and 30-min sampling strictly overlapped. CONCLUSION: UBT with 10 min breath collection and FemtoLab stool antigen assay are the most reliable non-invasive tests to diagnose H. pylori infection.     

Blood lead levels, clinico-pathological findings and erythrocyte metabolism in dogs from different habitats

Biological markers of lead exposure were measured in 20 dogs from five different habitats chosen on the basis of the degree of anthropogenic influence. None of the dogs had clinical signs of lead poisoning. Compared to controls, blood lead concentrations were significantly higher in dogs from industrial areas, confirming the role of lead emissions in environmental pollution and the possible role of dogs as biomonitors of lead exposure in these areas. Whole blood lead concentrations were similar in dogs living in urban and rural areas, probably due to "indirect" lead sources and due to decreased urban lead contamination. As in humans, individual variability was detected. No significant correlation between clinico-pathological changes (hematology, clinical chemistry, Delta-aminolevulinic acid dehydratase activity and other intra-erythrocytic metabolic parameters) and lead concentration were observed. Our findings suggest dogs can be useful as sentinels of environmental lead exposure.