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BACKGROUND Previous reports showed some beneficial effect of oral vancomycin treatment (OVT) in children with primary sclerosing cholangitis; conversely, the experience in patients with other autoimmune liver diseases (AILD), including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), is scant. AIM To assess the response to immunosuppressive treatment (IS) and to OVT in children diagnosed with AILD. METHODS Retrospective study of children diagnosed with AIH (normal biliary tree at cholangiography) and ASC (abnormal biliary tree at cholangiography) in the last 10 years. All underwent standard immunosuppressive therapy (IS), but nonresponders received also OVT. Biochemical remission [normal aspartate aminotransferase (AST)] and immunological remission (normal IgG and negative autoantibodies) rates and Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) index were assessed and compared during the follow up. RESULTS 75 children were included [69% female, median age 10.5 years (5.6-13.4 years), AIH = 54, ASC= 21]. Sixty-three patients (84%, AIH = 52, ASC = 11) were treated with standard IS and 61 achieved biochemical remission, whereas 12 not responding to IS [16%, F = 75%, median age 13.5 years, (12.2-15.7), 10 with ASC] required OVT and 8 achieved biochemical remission. Overall OVT increased the biochemical remission rate of the whole group of AILD patients from 81% (61/75) to 92% (69/75). Median values of AST, alanine aminotransferase (ALT) and gamma-glutamyl transferase (GGT) decreased significantly after OVT start (P < 0.05). Complete normalization of livers enzymes (AST, ALT and GGT) was observed in 6/12 patients (50%). Decrease in SCOPE index score was reported in 5/12 patients (42%). At last follow up (median of 4.4 years, range 0.6-13.8 years) all 75 patients are alive, 6 (8%, 1 with ASC) successfully discontinued medications, 1 (with ASC) required liver transplantation. CONCLUSION Children with AIH and ASC respond well to IS treatment

Non-syndromic primary ovarian insufficiency due to ovarian dysgenesis in 46,XX patients is an uncommon finding in the general population, even though several monogenic variants have been reported as causative factors. Here, we describe a 15-year-old patient diagnosed with gonadal dysgenesis possibly due to the interaction of three potentially pathogenic variants of genes involved in ovarian maturation, namely factor in the germline alpha (FIGLA), newborn ovary homeobox-encoding (NOBOX) and nuclear receptor subfamily 5 group A member 1 (NR5A1). We also describe a different degree of residual ovarian function within the proband's family, whose female members carry one to three demonstrated variations in the aforementioned genes in a clinical spectrum potentially dependent on the number of alleles involved. Our results support the hypothesis that the severity of the clinical picture of the proband, resulting in complete ovarian dysgenesis, may be due to a synergic detrimental effect of inherited genetic variants.

Non-syndromic primary ovarian insufficiency due to ovarian dysgenesis in 46,XX patients is an uncommon finding in the general population, even though several monogenic variants have been reported as causative factors. Here, we describe a 15-year-old patient diagnosed with gonadal dysgenesis possibly due to the interaction of three potentially pathogenic variants of genes involved in ovarian maturation, namely factor in the germline alpha (FIGLA), newborn ovary homeobox-encoding (NOBOX) and nuclear receptor subfamily 5 group A member 1 (NR5A1). We also describe a different degree of residual ovarian function within the proband's family, whose female members carry one to three demonstrated variations in the aforementioned genes in a clinical spectrum potentially dependent on the number of alleles involved. Our results support the hypothesis that the severity of the clinical picture of the proband, resulting in complete ovarian dysgenesis, may be due to a synergic detrimental effect of inherited genetic variants.

We propose an on-line fault detection and diagnosis framework in sensor networks, called Inspect, which is based on a hybrid tiered approach to integrity checking. Inspect tiered design, combines the benefits of distributed and centralized approaches, thus improving the responsiveness and efficiency of the fault detection. More precisely, Inspect consists of a local tier built at each sensor node and capable of detecting anomalies, and a centralized tier built at the sink and capable of distinguishing between sensor faults and unexpected temporal-spatial variations in the phenomenon, and detecting more complex types of faults.Inspects offers several desirable features: it provides online detection, it is not designed for a specific application or statistical distribution of the phenomenon, and it is more robust to temporal-spatial variations of the phenomenon. Moreover, Inspect provides confidence bounds that can be dynamically tuned according to the user requirements for a better resource management, and addresses the problem of detecting more complex types of faults, which is a challenging and yet unexplored problem.

We propose an on-line fault detection and diagnosis framework in sensor networks, called Inspect, which is based on a hybrid tiered approach to integrity checking. Inspect tiered design, combines the benefits of distributed and centralized approaches, thus improving the responsiveness and efficiency of the fault detection. More precisely, Inspect consists of a local tier built at each sensor node and capable of detecting anomalies, and a centralized tier built at the sink and capable of distinguishing between sensor faults and unexpected temporal-spatial variations in the phenomenon, and detecting more complex types of faults.Inspects offers several desirable features: it provides online detection, it is not designed for a specific application or statistical distribution of the phenomenon, and it is more robust to temporal-spatial variations of the phenomenon. Moreover, Inspect provides confidence bounds that can be dynamically tuned according to the user requirements for a better resource management, and addresses the problem of detecting more complex types of faults, which is a challenging and yet unexplored problem.

We propose a distributed digital time--stamping service (TSS) based on absolute timestamps and resilient to Byzantine failures. In contrast with previous solutions based on linking schemes, our TSS provides \emph{high scalability}, \emph{better response time}, and \emph{fine granularity}. It works in asynchronous systems

We propose a distributed digital time--stamping service (TSS) based on absolute timestamps and resilient to Byzantine failures. In contrast with previous solutions based on linking schemes, our TSS provides \emph{high scalability}, \emph{better response time}, and \emph{fine granularity}. It works in asynchronous systems