"Starry liver" - Von Meyenburg complex clinical case presentation and differential diagnosis discussion: A case report.

BACKGROUND: Von Meyenburg complex (VMC) (i.e., biliary hamartoma) is a rare congenital disorder characterized by multiple dilated cystic bile ducts, without clear trends in sex or age predominance. Due to the low number of published cases and the lack of recognized guidelines, the management of such patients remains a clinical challenge. CASE SUMMARY: We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations. Considering the patient's condition, a conservative treatment strategy was chosen. Instrumental, laboratory, and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment. CONCLUSION: VMC is a potentially non-life threatening condition, but its recognition is crucial for the management of patients.

Spectroscopic/Computational Characterization and the X-ray Structure of the Adduct of the VIVO-Picolinato Complex with RNase A.

The structure, stability, and enzymatic activity of the adduct formed upon the reaction of the V-picolinato (pic) complex [VIVO(pic)2(H2O)], with an octahedral geometry and the water ligand in cis to the V═O group, with the bovine pancreatic ribonuclease (RNase A) were studied. While electrospray ionization-mass spectrometry, circular dichroism, and ultraviolet-visible absorption spectroscopy substantiate the interaction between the metal moiety and RNase A, electron paramagnetic resonance (EPR) allows us to determine that a carboxylate group, stemming from Asp or Glu residues, and imidazole nitrogen from His residues are involved in the V binding at acidic and physiological pH, respectively. Crystallographic data demonstrate that the VIVO(pic)2 moiety coordinates the side chain of Glu111 of RNase A, by substituting the equatorial water molecule at acidic pH. Computational methods confirm that Glu111 is the most affine residue and interacts favorably with the OC-6-23-Δ enantiomer establishing an extended network of hydrogen bonds and van der Waals stabilizations. By increasing the pH around neutrality, with the deprotonation of histidine side chains, the binding of the V complex to His105 and His119 could occur, with that to His105 which should be preferred when compared to that to the catalytically important His119. The binding of the V compound affects the enzymatic activity of RNase A, but it does not alter its overall structure and stability.

Asymptomatic portal vein aneurysm: Three case reports.

BACKGROUND: Portal vein aneurysm (PVA) is an uncommon vascular dilatation, showing no clear trend in sex or age predominance. Due to the low number of published cases and the lack of management guidelines, treatment of this condition remains a clinical challenge. CASE SUMMARY: We present three cases of asymptomatic PVA; the first and second involve an extrahepatic manifestation, of 48 mm and 42.3 mm diameter respectively, and the third involves an intrahepatic PVA of 27 mm. All were diagnosed incidentally during routine check-up, upon ultrasonography scan. Since all patients were asymptomatic, a conservative treatment strategy was chosen. Follow-up imaging demonstrated no progression in the aneurysm dimension for any case. CONCLUSION: As PVA remains asymptomatic in many cases, recognition of its imaging features is key to favourable outcomes.

Evaluation of a diagnostic therapeutic educational pathway for asthma management in youth.

BACKGROUND: In spite of some evidence for positive effects of patient's education to asthma self-management by randomized clinical trials, there are few studies on the impact of patient' s educational programs in the real world. We aimed to assess the impact of a diagnostic therapeutic educational pathway (DTEP) on asthma control of children and adolescents by comparing frequency of outcomes indicative of asthma control before and after attending the pathway. METHODS: This is a retrospective cohort study including all patients aged 6-11 and 12-17 years who attended the DTEP in 2007-2014. The DTEP includes 3 specialist's evaluations at 8- to 12-week intervals and two follow-up visits. Patients and their parents receive an educational course concerning prevention measures, early recognition of symptoms, and appropriate use of drugs and devices. The rates of hospitalizations, outpatient services, emergency room visits, and drug prescriptions were considered as outcomes and computed as number of events divided by person-time. RESULTS: A total of 806 patients were enrolled. A statistically significant decrease in rates from before to after DTEP was observed for almost all outcomes, in both age groups, with relative risks ranging from 0.12 to 0.60. The rates of drug prescription showed a statistically significant decrease, from before to after DTEP, for each type of medicine for asthma, in both age groups, from percent difference of -66% to -24.3%. CONCLUSIONS: The positive impact of this program on the outcomes indicative of asthma control in both children and adolescents suggests that it may be valuable for asthma management.

Optimized contrast-enhanced ultrasonography for characterization of focal liver lesions in cirrhosis: A single-center retrospective study.

BACKGROUND: Hepatocellular carcinoma (HCC) is the leading cause of death amongst cirrhotic patients. Its diagnosis and discrimination from non-HCC malignant lesions in cirrhosis includes contrast enhanced computed tomography (CECT), contrast enhanced magnetic resonance imaging (CEMRI), or, in selected cases, liver biopsy. The role of contrast-enhanced ultrasonography (CEUS) is still controversial. AIMS: To evaluate whether, by selecting an appropriate 'time to wash-out' cut-off value, CEUS capability of discriminating between HCC and non-HCC malignancies in cirrhotic patients may be enhanced. METHODS: We enrolled 282 cirrhotic patients who underwent CEUS at our institute, from January 2008 to January 2012, for focal liver lesions (FLLs) detected at ultrasound (US). We used liver biopsy and subsequent histological evaluation as the gold standard for correct classification of FLLs. We calculated the area under receiver operator characteristic curves for CEUS to distinguish patients with HCC from those with non-HCC malignancies. The best 'time to wash-out' cut-off values were selected. RESULTS: HISTOLOGICAL DIAGNOSIS OF FLLS WAS AS FOLLOWS: 34 benign lesions (i.e. 25 regenerative nodules and 9 dysplastic nodules) and 248 malignant lesions (223 well-to-moderately differentiated HCCs; 7 poorly-differentiated HCCs; 5 intrahepatic colangiocellular carcinomas (ICCs); 5 primary non-Hodgkin B-cell lymphomas (NHBLs); and 8 metastatic liver tumors). A time to wash-out > 55 s identified patients with HCC with the highest level of accuracy (92.7%). Similarly, a time to wash-out ≤ 55 s correctly identified the vast majority of the non-HCC malignancies (100% sensitivity, 98.2% specificity and diagnostic accuracy of 98.3%). CONCLUSIONS: CEUS is an accurate and safe procedure for discriminating FLLs in cirrhotic patients, especially when a cut-off time to wash-out of 55 s is chosen as a reference value.

Ultrasound-guided percutaneous biopsy for diagnosis of gastrointestinal lesions.

BACKGROUND AND AIMS: Endoscopical examination is not always sufficient for the diagnosis of gastrointestinal masses. This study assessed the diagnostic accuracy and safety of ultrasound-guided percutaneous biopsy of gastrointestinal lesions. METHODS: This retrospective study evaluated 114 patients who underwent ultrasound-guided biopsy of gastrointestinal masses with a 18G needle. Thirty-two of these patients underwent a 22G fine-needle biopsy for cytology. Histology was compared with a composite standard of reference for diagnosis (i.e. post-surgery histological evaluation in 73 cases and computed tomography or magnetic resonance scan findings, together with a compatible clinical follow-up for at least 24 months, in the remaining 41 cases). Safety was assessed by recording side effects for up to 4h after the procedure. RESULTS: Of the 114 lesions evaluated, 112 were malignant (98.2%) and 2 benign (1.8%). Specimens were adequate for histology in all but one case. Specimens were obtained from the stomach (n=38; 33.3%), small bowel (n=36; 31.6%) and colon (n=40; 35.1%). Diagnosis was correct in 113/114 cases (99.1%). Sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy were 99.1%, 100%, 100%, 66%, and 99%, respectively. One of the 114 patients (0.9%) bled from a gastric GIST. CONCLUSIONS: Ultrasound-guided percutaneous biopsy of gastrointestinal lesions is a valid alternative when diagnosis of a gastrointestinal mass cannot be obtained with an endoscopical procedure.