Epidemiology, pathogenesis, clinical presentation and management of TB in patients with HIV and diabetes.

Caused by Mycobacterium tuberculosis, TB is the leading cause of death from an infectious disease. HIV and diabetes are recognised risk factors for progression of TB disease and both have a strong impact on the diagnosis and management of TB, threatening efforts to end TB globally. Here we provide the latest data on the complex interplay between these conditions. TB patients with HIV present systemic immune activation, increased HIV viral load, more severe clinical presentations and reduced success of TB therapy. Similarly, TB patients with diabetes are characterised by an exaggerated adaptive immunity, worsening of the clinical presentations and a higher risk for multidrug resistance and treatment failure. It is important to strengthen resources to prevent these comorbidities from occurring and to implement screening, early diagnosis and appropriate management strategies.

Multidisciplinary consensus recommendations from a European network for the diagnosis and practical management of patients with incontinentia pigmenti.

BACKGROUND: Incontinentia pigmenti (IP) is a rare multisystemic X-linked dominant genetic disorder characterized by highly diagnostic skin lesions. The disease can be misdiagnosed in infants, and complications affecting the eyes and/or the brain can be severe. Our objective was to highlight the urgency of an appropriate diagnosis and management strategy, as soon as the first symptoms appear, and the need for a well-codified monitoring strategy for each child. METHODS: An in-depth literature review using a large number of databases was conducted. The selection criteria for articles were literature review articles on the disease, case series and retrospective studies based on the disease, clinical studies (randomized or not) on treatment, articles discussing patient care and management (treatment, diagnosis, care pathways), and recommendations. The research period was from 2000 until 2018. A group of multidisciplinary experts in IP management was involved, issued from different healthcare providers of the European Network for Rare Skin Diseases (ERN-Skin). The final recommendations have been submitted to two patient representative associations and to a general practitioner and a neonatal specialist prior to their finalization. RESULTS AND CONCLUSION: The diagnosis of IP must be promptly performed to detect potential extracutaneous manifestations, thus allowing the timely implementation of specific therapeutic and monitoring strategies. Eye involvement can be a therapeutic urgency, and central nervous system (CNS) involvement requires a very rigorous long-term follow-up. Assessments and patient support should take into account the possible co-occurrence of various symptoms (including motor, visual and cognitive symptoms).

COVID-19 among healthcare workers in a specialist infectious diseases setting in Naples, Southern Italy: results of a cross-sectional surveillance study.

A coronavirus disease 2019 (COVID-19) surveillance study was performed in March-April 2020 among asymptomatic healthcare workers (HCWs) at a specialist infectious diseases hospital in Naples, Italy. All HCWs underwent two rounds of molecular and serological testing for severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). One hundred and fifteen HCWs were tested; of these, two cases of infection were identified by reverse transcriptase polymerase chain reaction and two HCWs were SARS-CoV-2 immunoglobulin G seropositive. The overall prevalence of current or probable previous infection was 3.4%. The infection rate among HCWs was reasonably low. Most of the infected HCWs had been asymptomatic for the preceding 30 days, which supports the need for periodic screening of HCWs for COVID-19.

Incontinentia Pigmenti. / Incontinencia pigmenti.

Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a rare neuroectodermal dysplasia. It is an X-linked dominant disorder caused by mutations in the IKBKG/NEMO gene on Xq28. Approximately 80% of patients have a deletion of exons 4 to 10. Incontinentia pigmenti has an estimated incidence of 0.7 cases per 100,000 births. In hemizygous males, it is usually lethal, while in females, it has a wide spectrum of clinical manifestations. Incontinentia pigmenti is a multisystemic disease that invariably features skin changes. These changes are the main diagnostic criteria and they evolve in 4 stages, in association with other abnormalities affecting the central nervous system, eyes, teeth, mammary glands, hair, nails, skin, and other parts of the body. The aim of this brief review is to highlight the clinical features of this genodermatosis and underline the importance of case-by-case interdisciplinary management, including genetic counseling.

Transportation capacity for patients with highly infectious diseases in Europe: a survey in 16 nations.

Highly infectious diseases (HIDs) are defined as being transmissible from person to person, causing life-threatening illnesses and presenting a serious public health hazard. In most European Union member states specialized isolation facilities are responsible for the management of such cases. Ground ambulances are often affiliated with those facilities because rapid relocation of patients is most desirable. To date, no pooled data on the accessibility, technical specifications and operational procedures for such transport capacities are available. During 2009, the 'European Network for HIDs' conducted a cross-sectional analysis of hospitals responsible for HID patients in Europe including an assessment of (a) legal aspects; (b) technical and infrastructure aspects; and (c) operational procedures for ground ambulances used for HID transport. Overall, 48 isolation facilities in 16 European countries were evaluated and feedback rates ranged from 78% to 100% (n = 37 to n = 48 centres). Only 46.8% (22/47) of all centres have both national and local guidelines regulating HID patient transport. If recommended, specific equipment is found in 90% of centres (9/10), but standard ambulances in only 6/13 centres (46%). Exclusive entrances (32/45; 71%) and pathways (30/44; 68.2%) for patient admission, as well as protocols for disinfection of ambulances (34/47; 72.3%) and equipment (30/43; 69.8%) exist in most centres. In conclusion, the availability and technical specifications of ambulances broadly differ, reflecting different preparedness levels within the European Union. Hence, regulations for technical specifications and operational procedures should be harmonized to promote patient and healthcare worker safety.

Highly infectious diseases in the Mediterranean Sea area: Inventory of isolation capabilities and recommendations for appropriate isolation.

Epidemics such as viral haemorrhagic fevers, severe acute respiratory syndrome, Middle East respiratory syndrome coronavirus or yet unknown ones have few chances of disappearing. Globalization, worldwide travel, climate change, social conflicts and wars, among others, are likely to favor the emergence of epidemics. Preparedness of hospitals to prevent the spread of these outbreaks is among the prioritized political programmes of many countries. The EuroNHID network has in the past drawn a map of features and equipment of hospitals across Europe to take care of highly contagious patients. We update the data regarding isolation capabilities and recommendations, with an emphasis on Mediterranean countries.

Long-term results after stapled hemorrhoidopexy: a survey study with mean follow-up of 12 years.

BACKGROUND: Hemorrhoidal prolapse is a common benign disease. The introduction of circular-stapled hemorrhoidopexy as an alternative to the conventional hemorrhoidectomy led to a new spectrum of postoperative outcomes and complications. The aim of the present study was to evaluate long-term results after stapled hemorrhoidopexy. METHODS: All the patients who had stapled hemorrhoidopexy using a PPH03 stapler, from January 2003 to December 2005, were retrospectively collected in a dedicated database. Between March and May 2016, all the patients were asked by phone to complete a questionnaire. The study evaluated anatomical recurrence, symptom recurrence and frequency, and satisfaction after surgery. The postoperative complications recorded were hemorrhage, hematoma, urinary retention, anastomotic stenosis, persistent anal pain, tenesmus, and impaired anal continence evaluated also with the Faecal Incontinence Severity Index score. RESULTS: One hundred and ninety four patients were identified and 171 completed the questionnaire. The mean follow-up was 12 ± 0.8 years (range 11-13 years). Anatomical self-reported prolapse recurrence was 40.9% (n = 70). In 75.6% (n = 129) of patients, the severity and frequency of symptoms improved. The overall complication rate was 56.7% (n = 40) with a serious adverse event rate of 8.7% (n = 15). The overall tenesmus rate was 38.2% (n = 65) and the overall impaired continence rate was 39.1% (n = 67). Medical therapy was still required occasionally by 40.3% (n = 69) of the patients and 9.3% (n = 16) of the patients underwent surgery for recurrence. Patient satisfaction rate was good (≥ 3 on a scale of 1 to 5) in 81.2% (n = 139) of cases. CONCLUSIONS: The study showed that stapled hemorrhoidopexy using the first-generation devices is safe and feasible but associated with a high recurrence and incontinence rate. More stringent selection criteria in association with the use of large volume devices can lead to better results in the future.

Correction to: Long-term results after stapled hemorrhoidopexy: a survey study with mean follow-up of 12 years.

Unfortunately, the 7th author's family name was incorrectly published in the original publication. The complete correct name should read as follows.

Occupational transmission of an Orthopoxvirus infection during an outbreak in a colony of Macaca tonkeana in Lazio Region, Italy, 2015.

Orthopoxviruses spill over from animal reservoirs to accidental hosts, sometimes causing human infections. We describe the surveillance and infection control measures undertaken during an outbreak due to an Orthopoxvirus occurred in January 2015 in a colony of Macaca tonkeana in the province of Rieti, Latio, Italy, which caused a human asymptomatic infection. According to the epidemiological investigation, the human transmission occurred after an unprotected exposure. The contacts among wild, captive and domestic animals and humans, together with decreased immunity against Orthopoxviruses in the community, may put animal handlers at risk of infection, especially after the cessation of smallpox vaccination. To reduce these threats, standard precautions including respiratory hygiene and transmission-based precautions should be carefully applied also in veterinary medicine.

Localization of neuroglobin in the brain of R6/2 mouse model of Huntington's disease.

Neuroglobin (Ngb) is expressed in the central and peripheral nervous system, cerebrospinal fluid, retina, and endocrine tissues where it is involved in binding O2 and other gasotransmitters. Several studies have highlighted its endogenous neuroprotective function. Huntington's disease (HD), a dominant hereditary disease, is characterized by the gradual loss of neurons in discrete areas of the central nervous system. We analyzed the expression of Ngb in the brain tissue of a mouse model of HD, in order to define the role of Ngb with respect to individual cell type vulnerability in HD and to gender and age of mice. Our results showed different expressions of Ngb among neurons of a specific region and between different brain regions. We evidenced a decreased intensity of Ngb at 13 weeks of age, compared to 7 weeks of age. The double immunofluorescence and fluorescence resonance energy transfer (FRET) experiments showed that the co-localization between Ngb and huntingtin at the subcellular level was not close enough to account for a direct interaction. We also observed a different expression of Ngb in the striatum, depending on the sex and age of animals. These findings provide the first experimental evidence for an adaptive response of Ngb in HD, suggesting that Ngb may exert neuroprotective effects in HD beyond its role in reducing sensitivity to oxidative stress.

Giant endometrial cyst of the liver: a case report and review of the literature.

Endometriosis is a benign condition described as the presence of endometrial- like tissue found outside the uterine cavity. Hepatic endometriosis is one of the rarest localization of extrapelvic endometriosis, only 22 cases have been reported in the literature. The preoperative diagnosis of hepatic endometriosis is rather difficult because in about the half of the patient affected they had no history of endometriosis. Moreover radiological images reveal no characteristic findings for hepatic endometriosis. It is often described as cystic mass with or without solid component, difficult to distinguish from hepatic abscess, hematoma, cystoadenoma or malignant neoplasia. We report a case of a 27-year-old female with a large cystic mass involving the left lobe of the liver. The patient underwent laparoscopic exploration and converted to laparotomy for resection of giant hepatic endometriosis.

Effects of alpha1-blockers on urodynamic parameters of bladder outlet obstruction in patients with lower urinary tract symptoms suggestive of benign prostatic enlargement: a review.

α1-adrenergic receptors blockers (ABs) are recommended as first-line medical therapy in men with Lower Urinary Tract Symptoms suggestive of Benign Prostatic Enlargement (LUTS/BPE). Available ABs include: terazosin, doxazosin, tamsulosin, naftopidil, alfuzosin and silodosin. These agents have different profiles of selectivity for α1-adrenergic receptors subtypes. All these agents are efficacious in improving both storage and voiding LUTS. In recent years the efficacy of ABs in improving urodynamic parameters of bladder outlet obstruction (BOO) has been questioned. We reviewed literature evidences about the effects of available ABs on invasive urodynamic parameters of BOO in men with LUTS/BPE. The impact of ABs therapy on urodynamic parameters indicative of BOO has been evaluated for all currently approved drugs. Available data demonstrate improvements in terms of both free uroflowmetry and pressure-flow parameters. While the impact of ABs on maximum urinary flow is clinically modest, the improvement of detrusor pressure at maximum urinary flow is more robust. Only few studies exist that directly compare the urodynamic effects of a small number of ABs. According to these studies there are no differences among ABs in terms of urodynamic efficacy. Indirect comparison of ABs suggests greater effectiveness of silodosin in terms of detrusor pressure at maximum urinary flow reduction. Studies that stratified populations based upon the degree of obstruction at baseline demonstrated greater urodynamic changes in patients with baseline BOO with respect to the unobstructed patients. Globally, the quality of studies available is low and there is considerable heterogeneity among studies.

Rare mendelian primary immunodeficiency diseases associated with impaired NF-κB signaling.

Mendelian primary immunodeficiency diseases (MPIDs) are rare disorders affecting distinct constituents of the innate and adaptive immune system. Although they are genetically heterogeneous, a substantial group of MPIDs is due to mutations in genes affecting the nuclear factor-κB (NF-κB) transcription pathway, essential for cell proliferation and cell survival and involved in innate immunity and inflammation. Many of these genes encode for crucial regulatory components of the NF-κB pathway and their mutations are associated with immunological and developmental signs somehow overlapping in patients with MPIDs. At present, nine different MPIDs listed in the online mendelian inheritance in man (OMIM) are caused by mutations in at least nine different genes strictly involved in the NF-κB pathway that result in defects in immune responses. Here we report on the distinct function of each causative gene, on the impaired NF-κB step and more in general on the molecular mechanisms underlining the pathogenesis of the disease. Overall, the MPIDs affecting the NF-κB signalosome require a careful integrated diagnosis and appropriate genetic tests to be molecularly identified. Their discovery at an ever-increasing rate will help establish a common therapeutic strategy for a subclass of immunodeficient patients.

Simple enucleation versus standard partial nephrectomy for clinical T1 renal masses: perioperative outcomes based on a matched-pair comparison of 396 patients (RECORd project).

OBJECTIVES: To compare simple enucleation (SE) and standard partial nephrectomy (SPN) in terms of surgical results in a multicenter dataset (RECORd Project). MATERIALS AND METHODS: patients treated with nephron sparing surgery (NSS) for clinical T1 renal tumors between January 2009 and January 2011 were evaluated. Overall, 198 patients who underwent SE were retrospectively matched to 198 patients who underwent SPN. The SPN and SE groups were compared regarding intraoperative, early post-operative and pathologic outcome variables. Multivariable analysis was applied to analyze predictors of positive surgical margin (PSM) status. RESULTS: SE was associated with similar WIT (18 vs 17.8 min), lower intraoperative blood loss (177 vs 221 cc, p = 0.02) and shorter operative time (121 vs 147 min; p < 0.0001). Surgical approach (laparoscopic vs. open), tumor size and type of indication (elective/relative vs absolute) were associated with WIT >20 min. The incidence of PSM was significantly lower in patients treated with SE (1.4% vs 6.9%; p = 0.02). At multivariable analysis, PSM was related to the surgical technique, with a 4.7-fold increased risk of PSM for SPN compared to SE. The incidence of overall, medical and surgical complications was similar between SE and SPN. CONCLUSIONS: Type of NSS technique (SE vs SPN) adopted has a negligible impact on WIT and postoperative morbidity but SE seems protective against PSM occurrence.

Health-related quality of life in patients with haemophilia and inhibitors on prophylaxis with anti-inhibitor complex concentrate: results from the Pro-FEIBA study.

Patients with haemophilia A and inhibitors are at high risk for severe bleeding, progression of joint disease and deterioration of health-related quality of life (HRQoL). To determine the impact of prophylaxis with an activated prothrombin complex concentrate (aPCC) on HRQoL, HRQoL was assessed using the Short-Form (SF)-36 Health Survey and the EQ-5D questionnaire in subjects ≥ 14 years participating in a prospective, randomized, crossover study comparing 6 months of aPCC prophylaxis with 6 months of on-demand therapy. Eighteen of 19 patients completed the survey or questionnaire before and after the on-demand therapy and prophylaxis periods. A general trend towards improved HRQoL after prophylaxis was observed for the 18 evaluable patients in all SF-36 dimensions except for vitality/energy and physical functioning. After prophylaxis, 'good responders,' defined as patients experiencing ≥ 50% reduction in bleeding, exhibited statistically and clinically significant differences in the physical component score (P = 0.021), role - physical (P = 0.042), bodily pain (P = 0.015), and social functioning (P = 0.036). Similarly, the EQ-5D health profile showed a trend towards improvement after prophylaxis in all evaluable patients. Among the good responders, improvements did not differ from those observed after on-demand treatment. EQ visual analogue scale values were slightly improved following prophylaxis for all evaluable patients and the EQ-5D utility index improved in the good responders only. During prophylaxis, patients missed significantly fewer days from school or work because of bleeding than during on-demand treatment (P = 0.01). In conclusion, by significantly reducing bleeding frequency in good responders, aPCC prophylaxis improved HRQoL compared with on-demand treatment.

Comparison between tungsten and steel polypectomy snares: evaluation of depth of colonic thermal wall injury in a pig model.

BACKGROUND AND STUDY AIMS: Thermal injury of the colonic wall during polypectomy may induce complications such as bleeding and perforation. To date, the role of the snare material in these injuries has not been examined. The aim of this study was to evaluate the depth of colonic post-polypectomy thermal wall injury induced by tungsten and steel endoscopic snares, in an effort to reduce electrosurgery-related complications. MATERIALS AND METHODS: This was a single tertiary center experimental study in a porcine model. A total of 90 polypectomies where performed in three live pigs under general anesthesia, using both steel and tungsten snares by cut, coagulation, and blend current modes. The pigs were then euthanized and their colons examined histologically. RESULTS: Steel snares induced significantly deeper tissue injury than tungsten snares in the pure cut mode (Pearson χ2 = 6.136, P = 0.013). The ordinal logistic regression analysis showed that the current mode and snare material were significantly associated with the ordinal score for the depth of injury. Thus, cut mode was positively associated with a lower score and coagulation mode with a higher score. In addition, tungsten was significantly associated with a lower depth of tissue injury. CONCLUSIONS: Due to its inherent electrical properties, tungsten is very well suited for manufacture into electrosurgical endoscopic devices. Thus, tungsten snares may be advantageous for routine use in endoscopic polypectomy, although further studies are needed to confirm these promising findings in human patients.