Alcohol-related Liver Disease and sepsis.

OBJECTIVE: Sepsis is one of the most common complications and causes of death in patients with Alcohol-related Liver Disease. This narrative review will focus on several aspects of sepsis in the context of Alcohol-related Liver Disease. The pathophysiology of the increased susceptibility to infections consists mainly of impaired innate and adaptive immunity, changes in gut microbiota with consequent gut translocation of bacteria due to both alcohol abuse and the underlying liver disease. The diagnosis of sepsis in the context of Alcohol-related Liver Disease is challenging. Moreover, the use of classical acute-phase serum proteins (e.g., C-reactive protein and procalcitonin) has several limitations in this setting. The early administration of an adequate antibiotic treatment is pivotal. Finally, measures of infection control and prevention are needed because the prognosis of sepsis in patients affected by Alcohol-related Liver Disease is poor.

Madelung's disease and acute alcoholic hepatitis: case report and review of literature.

Madelung's disease is a rare condition characterized by symmetric growth of fatty tumors (lipomas) around the neck, shoulders, upper arms, and trunk. It often affects men with a history of alcohol abuse. Here we report a review of the literature about this disease together with the description of a patient affected by Madelung's disease and acute alcoholic hepatitis.

Neurosurgical contribution within a complex NF1 supraregional service.

OBJECTIVES: The goal of this study was to review and present neurosurgical related activity within a multidisciplinary nationally commissioned specialty neurofibromatosis type I (NF1) center. PATIENTS & METHODS: We reviewed all NF1 Neurosurgical MDTs, NF1 Neurosurgical clinics and all neurosurgical procedures carried out in NF1 patients over an 8-year period. RESULTS: Since the inception of the service in 2009, 1505 cases were discussed at our NF-1 multidisciplinary meeting, 171 clinic appointments in complex NF1 patients with neurosurgical pathologies and 43(cranial and spinal) operations were performed. CONCLUSIONS: The formation of a supraregional multidisciplinary team allows for a better understanding of the disease, a comprehensive evaluation of neuroimaging findings and a steep learning curve in the management of NF1 surgical conditions. We provide holistic treatment for these patients via direct care, specialist advice and liaison with local units.

Acute HEV hepatitis: clinical and laboratory diagnosis.

OBJECTIVE: Hepatitis E Virus (HEV) is probably the most common cause of acute hepatitis worldwide. It has been regarded for a long time as a disease limited to developing countries. Recently, the refinement of diagnostic techniques, on the one hand, and migratory flows, on the other hand, have also led to the identification of an increased number of HEV infections in industrialized countries. Four HEV genotypes have been identified across the world, with different epidemiological burdens and a wide range of clinical presentations. Here, we report a case series of acute HEV hepatitis observed in the last three years in our hospital. PATIENTS AND METHODS: We performed a search for HEV IgM and IgG in all subjects admitted for acute hepatitis without evidence of other possible infectious, toxic or metabolic causes of liver damage. In subjects with HEV IgM positivity, the search for HEV-RNA was performed. RESULTS: We diagnosed eight acute HEV infections: 2 epidemic and 6 sporadic forms. HEV-RNA was detected in serum in 2 cases. CONCLUSIONS: HEV infection appears to be a cause of acute hepatitis that we must keep in mind even in developed countries.

Dental health in patients affected by alcohol use disorders: a cross-sectional study.

OBJECTIVE: Chronic alcohol abuse represents a risk factor for oral diseases, in particular, oral cancer. Periodontal disease has been showed to be involved in the pathophysiology of cardiovascular and metabolic diseases, such as atherosclerosis and liver steatosis. The role of chronic alcohol consumption on periodontitis is still controversial. The aim of the study was to evaluate the effect of chronic alcohol abuse on oral health. PATIENTS AND METHODS: Twenty-three alcohol use disorders (AUD) patients and twenty-three healthy social drinkers underwent an oral examination by trained oral clinicians in order to evaluate oral and dental health. A questionnaire assessing oral hygiene was administered together with the evaluation of DMFT (decayed, missing, filled teeth), SLI (Silness-Loë plaque index) and CPI (community periodontal index of treatment needs) scores. RESULTS: Alcoholic patients showed significantly lower oral hygiene scores compared to controls. Alcoholic patients showed significantly poorer scores at DMFT, SLI and CPI tests. Moreover, among alcoholics, smokers showed a significantly poorer oral health than non-smokers. CONCLUSIONS: Chronic alcohol abuse increases the risk of dental and periodontal diseases. Smoking represents a significant co-factor. The practice of basic oral hygiene and the access to professional dental care should be encouraged among AUD patients in order to reduce oral diseases.

Review article: Alcohol and gut microbiota - the possible role of gut microbiota modulation in the treatment of alcoholic liver disease.

BACKGROUND: Alcohol abuse represents the most common cause of liver disease in the Western countries. Pre-clinical and clinical studies showed that alcohol consumption affects amount and composition of gut microbiota. Moreover, gut flora plays an important role in the pathogenesis of alcoholic liver injury. AIM: To review the relationship between alcohol administration and changes on gut microbiota, its involvement in the pathogenesis of alcoholic liver disease, and how gut microbiota modulation could be a target for the treatment of alcoholic liver disease. METHODS: Articles were identified using the PubMed database with the search terms 'Alcohol', 'Gut Microbiota', 'Alcoholic liver disease', 'Probiotic', 'Prebiotic', 'Symbiotic' and 'Antibiotic'. English-language articles were screened for relevance. Full review of publications for the relevant studies was conducted, including additional publications that were identified from individual article reference lists. RESULTS: Alcohol abuse induces changes in the composition of gut microbiota, although the exact mechanism for this alteration is not well known. The translocation of bacterial products into the portal blood appears to play a key role in alcohol-induced liver damage. Several studies show that the modulation of gut microbiota seem to be a promising strategy to reduce alcohol-induced liver injury. CONCLUSIONS: Further studies are needed to better understand the relationship between alcohol administration and changes in gut microbiota, and its involvement in alcoholic liver disease. Moreover larger studies are needed to confirm the preliminary results on the therapeutic effects of gut microbiota modulation.

Supporting parents following childhood traumatic brain injury: a qualitative study to examine information and emotional support needs across key care transitions.

INTRODUCTION: Traumatic brain injury (TBI) is the leading cause of death and acquired disability in childhood. Research has demonstrated that TBI can lead to long-term physical, cognitive, emotional and behavioural difficulties for children and parental stress. Less is known about how parents experience a childhood brain injury and their information and support needs. This study aimed to examine parents' experiences and support needs following a childhood TBI from the time of the accident to their child's discharge home. METHODS: Qualitative semi-structured interviews were conducted with 29 parents/carers of children who had experienced a severe TBI. Participants were recruited from one children's tertiary centre in the UK. Data were analysed using the Framework approach. RESULTS: Parents had unmet information and emotional support needs across the care trajectory from the time of the accident to their child's return home. Information needs related to the impact of the TBI on their child; current and future treatment/rehabilitation plans; helping their child and managing their behaviour; accessing services/support. They lacked information and support for care transitions. In different settings parents faced particular barriers to having their information needs met. Parents' felt they needed emotional support in coming to terms with witnessing the accident and the loss of their former child. Lack of community support related not only to service availability but to a general lack of understanding of the impact of TBI on children, particularly when this was invisible. Overall parents felt unsupported in coping with children's behavioural and psychological difficulties. DISCUSSION: Taking a holistic approach to examining parents' experiences and support needs has enabled their changing needs to be highlighted across key care transitions within hospital and community settings and the service implications identified. Improvements in care co-ordination across care transitions are needed to ensure continuity of care and integration of support.

Baclofen in the treatment of persistent hiccup: a case series.

BACKGROUND: Persistent hiccup is a worrying symptom both for patients, because of reduced quality of life, and for physicians, because of frustration for unsuccessful treatments. AIM: To test baclofen administration for the treatment of persistent hiccup. METHOD: We report a series of seven patients affected by persistent hiccup successfully treated with baclofen. RESULTS: Hiccup stopped in all patients after a single administration of the drug. CONCLUSIONS: Baclofen is a GABA(B) receptor agonist. It is conceivable that the reduction of dopamine release by GABA(B) receptor stimulation is able to interrupt hiccup's reflex arc.

Clinical variability and characteristic autoantibody profile in primary C1q complement deficiency.

OBJECTIVES: C1q deficiency is a rare inherited defect in the early part of the complement cascade. In this report, we describe the varied clinical features of patients with this condition as well as the characteristic autoantibody profile. METHODS: A large Pakistani family with a high degree of consanguinity is described in which the father and five sons have C1q deficiency, all with different clinical manifestations. RESULTS: Clinical features of C1q deficiency can vary from almost no disease to fulminant bacterial infection and localized lupus-like skin, renal or CNS disease. Autoantibodies to ribonucleoproteins such as anti-Sm and Ro, but not dsDNA, were present. CONCLUSIONS: Awareness of the spectrum of clinical disease, autoantibody profiles and tests required to confirm the diagnosis of C1q deficiency are important if this life-threatening immunodeficiency disease is to be managed correctly.

ALK positive lymphohistiocytic variant of anaplastic large cell lymphoma in an adult.

BACKGROUND AND OBJECTIVES: The lymphohistiocytic (LH) variant of anaplastic large cell lymphoma (ALCL) has, for a long time, been considered typical of children and adolescents. The aim of this study is a detailed characterization of a case of this peculiar ALCL subtype affecting an adult patient. DESIGN AND METHODS: A 36-year old male presented with diffuse adenopathy and systemic symptoms (high fever, anorexia, asthenia); a diagnosis of CD30+/ALK+ ALCL, LH variant, was morphologically suspected and corroborated by immunohistochemistry that was crucial for the definitive diagnosis and subtyping. RESULTS: The neoplastic population consisted of cells highly variable in size and shape but more often isolated and largely obscured by a predominant reactive cellular infiltrate of histiocytes and plasma cells. The lymphoma cells exhibited a null non-B non-T antigenic profile, but reacted strongly for the Ber-H2/CD30, EMA, ALKc anti-TIA-1 monoclonal antibodies. The patient underwent chemotherapy plus bone marrow transplantation and, one year after diagnosis, he is well and in complete remission. INTERPRETATION AND CONCLUSIONS: Our findings provide additional evidence that: a) ALK+ lymphoma represents a single disease with a broad spectrum of morphology; b) clinicians and pathologists should be aware of the possible occurrence of LH variant of ALK+ ALCL also in adults in whom a favorable response to therapy may be expected despite systemic disease and an aggressive clinical presentation.

Anti-CEA immunoscintigraphy and computed tomographic scanning in the preoperative evaluation of mediastinal lymph nodes in lung cancer.

BACKGROUND: Thoracic computed tomography (CT) provides most of the staging information needed before operation for lung cancer and can reduce the number of exploratory thoracotomies. In recent years a new immunoscintigraphic technique with anti-carcinoembryonic antigen (CEA) monoclonal antibodies has been shown to be effective in lung cancer staging. This study compares the yields of CT scans and immunoscintigraphy in the preoperative evaluation of the medistinal lymph nodes of patients with non-small cell lung cancer. METHODS: One hundred and thirty one patients believed on clinical grounds to have a operable non-small cell lung cancer were photoscanned with the indium-111 labelled F(ab')2 fragments of the antibody FO23C5. Both planar and single photoemission computed tomography (SPECT) thoracic views were recorded. CT scan of the thorax, abdomen, and brain were obtained in all patients. Seventy of the patients eventually underwent surgery, an additional seven underwent mediastinoscopy or mediastinotomy, and a further 10 had both cervical exploration and thoracotomy. Pathological evaluation of the mediastinal nodes was available in all 87 patients, but in only 80 of them was the diagnosis of lung cancer eventually confirmed. RESULTS: The diagnostic accuracy of planar immunoscintigraphy, SPECT immunoscintigraphy, and CT scanning for N2 disease was 76%, 74%, and 71%, respectively. The corresponding sensitivity and specificity rates were 45%, 77%, 64% and 88%, 72%, and 74%. These were not significantly different. CONCLUSIONS: This study shows that anti-CEA immunoscintigraphy has no advantage over conventional CT scanning in assessing mediastinal lymphoadenopathy in patients with lung cancer. CT scanning remains the gold standard test in these patients.

Benign osteoblastoma of the mastoid part of the temporal bone: case report.

Osteoblastoma is a benign bone lesion that mainly affects the long bones and rarely the temporal bones. Very few cases have been reported in the literature. This paper reviews the literature, discusses the differential diagnosis, clinical presentation, and CT scan findings of such a condition and details our experience with a young patient who had a temporal bone (mastoid process) osteoblastoma.

Giant cell tumor of the lateral skull base: a case report.

Giant cell tumors of the temporal bone are very rare lesions. They should be differentiated from other giant cell lesions of bone, mainly reparative granulomas. A case with a very huge giant cell tumor of the temporal bone extending to the infratemporal fossa, temporomandibular joint, and greater wing of the sphenoid has been presented. Because of their tendency for recurrence, total removal is the treatment of choice for these tumors and was accomplished through the infratemporal fossa approach type B. A follow-up of 2 years and 9 months revealed no evidence of tumor recurrence.

[Nonocclusive intestinal infarct and necrotizing enterocolitis in the adult]. / Infarto intestinale non occlusivo ed enterocolite necrotizzante dell'adulto.

A case of full-thickness necrosis of the small bowel, and colon, which required partial resection of the jejunum and total resection of the ileum and colon is reported. The case gives the chance for a review of the Literature on intestinal necrosis not caused by vascular occlusion. Nonocclusive intestinal ischemia, acute neonatal necrotizing enterocolitis and adult necrotizing enterocolitis including the Pig-bel disease, common in Papua-New Guinea, are examined. Resemblances and differences in etiology, pathophysiology and clinical findings are discussed. The hypothesis that the process of "bacterial translocation" plays a central role in the pathogenesis of bowel infarction, representing therefore a possible link between infective and vascular mechanisms, is emphasized. Important suggestions on massive intestinal necrosis management are also reported.

Imaging lung cancer by scintigraphy with Indium 111-labeled F(ab')2 fragments of the anticarcinoembryonic antigen monoclonal antibody FO23C5.

BACKGROUND: Anticarcinoembryonic (CEA) monoclonal antibodies are able to react specifically with the antigen and have the potential for the detection of CEA-bearing tumors. METHODS: The authors photoscanned with indium 111 (111In)-labeled F(ab')2 fragments of the murine CEA monoclonal antibody FO23C5 63 patients with a newly diagnosed and pathologically documented bronchogenic carcinoma. Planar dual views of the thorax, abdomen, and brain were acquired between the 24th and 144th hour after the radiotracer injection. Patients had a complete pretreatment workup, which included a routine multiorgan computed tomography (CT) scan, and the determination of the serum and tissue CEA concentration. All patients were followed up clinically and radiologically. Nineteen needle aspirations and biopsies, 23 surgical explorations, and 4 mediastinoscopic studies yielded 121 pathologically documented sites of reference. RESULTS: Fifty-seven of 63 scans were positive for the primary tumor (sensitivity, 0.90). The uptake of the radiotracer correlated significantly with the intensity of tissue CEA expression (Spearman R [Rs], 0.25; P less than 0.05), but not with the serum CEA level or with the histotype. Overall, the sensitivity of the anti-CEA immunoscintigraphy (IS) for the N1, N2, N3, T3, T4, and M1 disease (1987 International Union Against Cancer [UICC] staging classification) was 0.67, 0.64, 0.62, 0.31, 0.29, and 0.86, respectively. Corresponding values of specificity were 0.67, 0.81, 0.90, 1, 1, and 0.93; accuracy values were 0.67, 0.71, 0.85, 0.71, 0.76, and 0.92. The authors limited the analysis to all of the pathologically documented sites and obtained slightly superior values but no meaningful differences. The stage derived from IS readings was correct in 33 patients. The same figure was obtained after an initial clinical workup, which included physical examination, laboratory routine tests, chest radiographs, bronchoscopy, and any diagnostic procedure indicated by those tests. CONCLUSIONS: Anti-CEA FO23C5-F(ab')2 fragments are not yet "magic bullets" for perfect diagnoses; however, their staging potential seems to be remarkable. Technical improvements, single-photo emission CT, and the use of such fragments in combination with other imaging techniques might enable researchers to further improve the current results.

Stepwise increase in plasma insulin and C-peptide concentrations in obese, in obese hypertensive, and in obese hypertensive diabetic subjects.

Several clinical and epidemiological evidences support the increased risk of cardiovascular disease (CVD) in pathological conditions as obesity, hypertension, non-insulin-dependent diabetes mellitus, which have hyperinsulinemia as a common feature. In this study, we assessed basal plasma insulin (IRI) and C-peptide (CPR) concentrations in 297 volunteers who participated in a survey concerning risk factors of CVD. We found a stepwise increase in fasting insulin and C-peptide levels in normal subjects (IRI 9.10 +/- 0.41 microU/ml; CPR 1.79 +/- 0.08 ng/ml), in obese subjects (IRI 11.31 +/- 0.38 microU/ml; CPR 2.54 +/- 0.07 ng/ml) in obese hypertensive subjects (IRI 14.17 +/- 0.72 microU/ml; CPR 2.64 +/- 0.09 ng/ml), in obese hypertensive diabetic subjects (IRI 22.57 +/- 2.62 microU/ml; CPR 3.33 +/- 0.27 ng/ml). Thus, we found increasing levels of IRI and CPR as normal conditions changed towards progressively more severe pathological conditions. Although several other factors contribute to determine CVD, we conclude that increasing levels of insulin and C-peptide could play an important role in causing CVD.

[Spontaneous pneumothorax. The therapeutic directions based on 117 cases]. / Il pneumotorace spontaneo. Orientamenti terapeutici sulla base di 117 casi.

117 cases of spontaneous pneumothorax are reviewed. The authors report their experience and consider surgical drain to be the best method in most cases to achieve a rapid recovery. Surgical management or conservative approach can also to be used in selected patients.

Multidirectional carcinoma of the thymus with neuroendocrine and sarcomatoid components and carcinoid syndrome.

We report a case of metastatic thymic neoplasm occurring in a 62-year-old Caucasian man. The tumor was polymorphic, with intimately admixed carcinoid, sarcomatous and undifferentiated components. Sarcomatous cells were spindle-shaped and immunoreactive for vimentin, actin and desmin. Carcinoid cells reacted for cytokeratin as well as with a panel of general neuroendocrine markers, including chromogranins A and B, synaptophysin, protein gene product (PGP) 9.5, neuron specific enolase (NSE), Leu 7 and Grimelius' silver. The endocrine nature of the latter cells was confirmed by ultrastructural evidence of abundant electron-dense granules 100 to 300 nm in size. Among the various hormones investigated immunohistochemically only the alpha chain of human chorionic gonadotropin (alpha-hCG) was detected in tumor cells. The neoplasm recurred after surgical resection and caused a typical carcinoid syndrome with skin flushing and increased urinary 5-hydroxyindolacetic acid (5-HIAA). The diagnosis of multidirectional thymic carcinoma with two lines of differentiation, of carcinoid and sarcomatoid type, was made.