Role of single nucleotide polymorphism rs2383206 on coronary artery disease risk among Saudi Population: a case-control study.

OBJECTIVE: We aim to investigate the relationship between genetic variation and biological function on a genomic scale, focusing on identifying genes responsible for complex diseases using single nucleotide polymorphisms. Specifically, the study explores the association between the rs2383206 gene located on chromosome 9p21.3 and the development of coronary artery disease (CAD) in a specific Saudi population. PATIENTS AND METHODS: This case-control study was conducted between September 2013 and May 2015 at King Abdullah Medical City (KAMC) and Al-Noor Specialist Hospital targeting the Saudi Population residing in the western region of Saudi Arabia. The study enrolled 315 cases with documented CAD and 205 controls with normal coronary arteries on coronary angiography. Genomic DNA was extracted from peripheral blood samples of both groups, and genotyping of rs2383206 was performed using the tetra-primer amplification-refractory mutation system-polymerase chain reaction (ARMS-PCR) method. RESULTS: In this study, the prevalence of the GG genotype in rs2383206 was found to be higher in patients with CAD than in controls, with an odds ratio of 1.997 [95% confidence interval (CI): 1.176-3.394, p = 0.007]. Additionally, individuals with the GG genotype who had sedentary lifestyles, hyperlipidemia, and smoked were found to be at a higher risk for developing CAD (p = 0.003, 0.009, and 0.003, respectively). The G allele also increased the risk of CAD with an odds ratio of 1.413 (95% CI: 1.099-1.817; p = 0.004). CONCLUSIONS: In conclusion, this study demonstrated a significant association between the rs2383206 variant located on chromosome 9p21 and the development of CAD. The findings of this study provide valuable insights into the genetic susceptibility to CAD and highlight the potential of this variant as a target for future functional studies.

Microscopic colitis, a forgotten condition: a clinical case and review of the literature.

OBJECTIVE: Microscopic colitis is a not uncommon chronic inflammatory disease of the colon, characterized by watery, non-bloody diarrhea, which is often forgotten and misdiagnosed. CASE PRESENTATION: In this paper, we present a puzzling case of relapsing chronic diarrhea triggered by non-steroidal anti-inflammatory drug (NSAID) abuse, smoking, inappropriate antibiotic use, and secondary Clostridium Difficilis infection. Several tests were performed during hospitalization, all of which were negative apart from fecal calprotectin (> 6,000 mg/kg, normal values < 50 mg/kg) and a positive Clostridium Difficilis toxin test. Since Vancomycin treatment did not bring about the expected response, colonoscopy was performed, which led to diagnosis, targeted therapy, and clinical resolution. Targeted therapy with budesonide and probiotics was initiated leading to resolution of the diarrhea. CONCLUSIONS: This case study shows how actual diagnosis may be delayed not only due to having to perform differential diagnosis with chronic inflammatory diseases, but also because certainty can only come from histological evidence, which takes time to obtain, especially when the disease's multifactorial nature is considered (smoking, NSAID abuse, oral proton pump inhibitors, inappropriate antibiotic use, and Clostridium difficilis infection).

The complexity of immunology: a rare adverse event following a Pfizer-BioNTech vaccine booster shot.

OBJECTIVE: Several mRNA vaccines have been developed to tackle the global pandemic. Despite their remarkable clinical efficacy, they are not devoid of severe short- and long-term adverse events. CASE PRESENTATION: In this paper, we describe a rare delayed adverse event (arterial and venous renal thrombosis with myocardial injury) in an otherwise healthy adult female, which occurred three months after she received a booster shot of Pfizer COVID-19 vaccine.  The patient was successfully treated for subacute renal ischemia with intra-arterial urokinase, and her myocardial injury was diagnosed with imaging (contrast-enhanced thoracic CT and cardiac magnetic resonance) and percutaneous coronary intervention. Deferred post-vaccine myocarditis was diagnosed and resolved with steroid therapy. CONCLUSIONS: In this paper, we report a useful clinical case for the pharmacovigilance database. Although scientific evidence confirms that the benefits of vaccination far outweigh the risk of adverse events, we would like to point out how important watchful observation is in the medium and long term, especially when the subject belongs to a specific risk category.

Incidence and impact of anticoagulation-associated abnormal menstrual bleeding in women after venous thromboembolism.

Preliminary data and clinical experience have suggested an increased risk of abnormal uterine bleeding (AUB) in women of reproductive age treated with anticoagulants, but solid data are lacking. The TEAM-VTE study was an international multicenter prospective cohort study in women aged 18 to 50 years diagnosed with acute venous thromboembolism (VTE). Menstrual blood loss was measured by pictorial blood loss assessment charts at baseline for the last menstrual cycle before VTE diagnosis and prospectively for each cycle during 3 to 6 months of follow-up. AUB was defined as an increased score on the pictorial blood loss assessment chart (>100 or >150) or self-reported AUB. AUB-related quality of life (QoL) was assessed at baseline and the end of follow-up using the Menstrual Bleeding Questionnaire. The study was terminated early because of slow recruitment attributable to the COVID-19 pandemic. Of the 98 women, 65 (66%) met at least one of the 3 definitions of AUB during follow-up (95% confidence interval [CI], 57%-75%). AUB occurred in 60% of women (36 of 60) without AUB before VTE diagnosis (new-onset AUB; 95% CI, 47%-71%). Overall, QoL decreased over time, with a mean Menstrual Bleeding Questionnaire score increase of 5.1 points (95% CI, 2.2-7.9), but this decrease in QoL was observed only among women with new-onset AUB. To conclude, 2 of every 3 women who start anticoagulation for acute VTE experience AUB, with a considerable negative impact on QoL. These findings should be a call to action to increase awareness and provide evidence-based strategies to prevent and treat AUB in this setting. This was an academic study registered at www.clinicaltrials.gov as #NCT04748393; no funding was received.

Midodrine and albumin as a possible "winning pair" in managing paracentesis-induced circulatory dysfunction: a clinical case report.

BACKGROUND: Paracentesis-induced circulatory dysfunction (PICD) is a "silent killer syndrome" occurring after large volume paracenteses (LVPs). We here report an unusual case of PICD induced by right heart failure recognized and managed successfully. CASE PRESENTATION: A 60-year-old woman was admitted to our Emergency Department for worsening dyspnea and hypoxia. Her medical history enclosed a chronic heart failure with reduced ejection fraction and post-stroke dysarthria associated to right hemiplegia. Clinical and laboratory examination defined a severe right-heart failure unresponsive to high-dose diuretic therapy. Diagnostic and therapeutic paracentesis was thus performed determining, initially, a progressive normalization of the abdominal volume, followed, subsequently, by a severe hypotension associated with an acute kidney injury (AKI) combined with severe hyponatremia associated with a normal cardiac output. In the hypothesis of a PICD, abdominal drainage and diuretic therapy were interrupted, reninemia sampling was performed, resulting in diagnostic, and treatment with albumin and norepinephrine was started. The latter was tapered and then replaced with Midodrine that conferred the possibility to reach clinical and laboratory stability, allowing relocation in a cardiological rehabilitation. PICD represents an independent predictor of mortality. Midodrine's prophylactic use in PICD has been suggested as a cheaper alternative to albumin, as it appears to improve renal perfusion and reduce ascites with better clinical handling, as demonstrated in our patient. CONCLUSIONS: Our clinical case wants to show how not all PICDs are secondary to hepatic dysfunctions with Midodrine playing a possible therapeutic role by counteracting the pathophysiological mechanism in a rapid and non-invasive way, representing a valid therapeutic option in adjunction to albumin.

Emergency department reorganisation to cope with COVID-19 outbreak in Milan university hospital: a time-sensitive challenge.

BACKGROUND: In March 2020 we faced a huge spread of the epidemic of SARS-CoV2 in northern Italy; the Emergency Departments (ED) and the Emergency Medical Services (EMS) were overwhelmed by patients requiring care. The hospitals were forced to reorganize their services, and the ED was the focal point of this challenge. As Emergency Department in a metropolitan area of the region most affected, we saw an increasing number of patients with COVID-19, and we made some structural and staff implementations according to the evolution of the epidemic. METHODS: We analysed in a narrative way the weaknesses and the point of strength of our response to COVID-19 first outbreak, focusing point by point on main challenges and minor details involved in our ED response to the pandemics. RESULTS: The main stems for our response to the pandemic were: use of clear and shared contingency plans, as long as preparedness to implement them; stockage of as much as useful material can be stocked; training of the personnel to be prepared for a fast response, trying to maintain divided pathway for COVID-19 and non-COVID-19 patients, well-done isolation is a key factor; preparedness to de-escalate as soon as needed. CONCLUSIONS: We evaluated our experience and analysed the weakness and strength of our first response to share it with the rest of the scientific community and colleagues worldwide, hoping to facilitate others who will face the same challenge or similar challenges in the future. Shared experience is the best way to learn and to avoid making the same mistakes.

Tratamiento endoscópico de lesiones quísticas supratentoriales en pediatría / Endoscopic treatment of supratentorial cystic lesions in pediatrics

Introducción: La endoscopía neuroquirúrgica es una técnica mínimamente invasiva, utilizada desde principios del siglo XX para dar solución a las patologías localizadas en el sistema ventricular. En la actualidad las indicaciones de esta técnica se han ampliado notablemente. El objetivo de este trabajo consiste en presentar el tratamiento endoscópico de quistes cerebrales supratentoriales de diferentes etiologías en pediatría. Materiales y métodos: Se realizó un estudio transversal retrospectivo, desde enero de 2016 hasta diciembre de 2019, de pacientes pediátricos con lesiones quísticas supratentoriales tratados endoscópicamente en el Hospital de Niños de La Plata. Para definir el éxito se utilizó la clasificación en 5 grados de Ross et al. Resultados: Se practicaron 14 procedimientos en 12 pacientes, con edades comprendidas entre los 2 meses y los 9 años. Del total, 6 fueron quistes intraventriculares, 3 quistes de línea media, 5 quistes paraventriculares. Todos presentaban algún signo o síntoma al momento de la consulta, predominando entre ellos la alteración del estado neurológico y los vómitos. Luego de practicarse la fenestración endoscópica, presentaron una evolución clínica favorable en 12 de los 14 procedimientos y una mejoría en al menos un criterio imagenológico en 10 del total de los procedimientos.Basados en la categorización de Ross et al. se obtuvo un grado I en el 57% de los casos, lo que implica una mejoría completa permanente. La tasa de complicación global fue del 7%, presentando en solo un caso infección post endoscopia. Conclusión: La neuroendoscopía debería ser considerada como una opción de primera línea para el tratamiento en las lesiones quísticas supratentoriales. Demostró ser un método poco invasivo, con el cual se obtuvieron buenos resultados y una baja tasa de complicaciones.

Introduction: Neurosurgical endoscopy is a minimally invasive technique, used since the beginning of the 20th century to solve pathologies localized in the ventricular system. Currently the indications for this technique have been greatly expanded. The objective of this work is to present the endoscopic treatment of supratentorial brain cysts of different etiologies in pediatrics. Material and methods: We carried out a retrospective cross-sectional study, from January 2016 to December 2019, of pediatric patients with supratentorial cystic lesions treated endoscopically at the Hospital de Niños of La Plata City. To define success, we used the 5-degree classification of Ross et al. Results: 14 procedures were performed in 12 patients, aged between 2 months and 9 years. Of the total, 6 were intraventricular cysts, 3 midline cysts, 5 paraventricular cysts. All presented any signs or symptoms at the time of the consultation, prevailing among them the alteration of the neurological state and vomiting. After endoscopic fenestration was performed, they presented a favorable clinical evolution in 12 of the 14 procedures and an improvement in at least one imaging criterion in 10 of all procedures. Based on the categorization of Ross et al. we obtained a grade I in 57% of the cases, which implies a permanent complete improvement. The overall complication rate was 7%, presenting post-endoscopy infection in only one case. Conclusion: Neuroendoscopy should be considered as a first-line option for the treatment of supratentorial cystic lesions. It proved to be a non-invasive method, with which we obtained good results and a low complication rate

Lavado endoscópico con colocación de catéter trans acueducto de Silvio en paciente lactante con ventriculitis de difícil manejo / Endoscopic lavage with placement of a trans-aqueduct of Silvio catheter in a nursing patient with difficult-to-manage ventriculitis

Introducción: La ventriculitis representa una emergencia infectológica, generalmente asociada a un procedimiento neuroquirúrgico. La incidencia es desconocida debido a la variación de los criterios diagnóstico. Descripción del caso: Presentamos una paciente de sexo femenino de 6 meses de edad con antecedente de hidrocefalia post hemorrágica, con diagnóstico de ventriculitis por Enterobacter complex asociada a sistema de derivación ventrículo peritoneal (DVP) de difícil manejo. La misma realizó tratamiento combinado de antibiótico con Meropenem y Colistin endovenoso e intraventricular asociado a tratamiento endoscópico que consistió en lavados, aspiración del contenido purulento intraventricular, tercer ventriculostomía endoscópica (TVE), coagulación bilateral del plexo coroideo y acueductoplastia con colocación de catéter de derivación ventricular externa (DVE) entre el tercer y el cuarto ventrículo con el objetivo de mantener la permeabilidad de la misma y de esta manera asegurar la llegada de antibiótico intraventricular al cuarto ventrículo. Discusión: La ventriculitis se asocia a múltiples complicaciones y una elevada tasa de morbi-mortalidad. El tratamiento de las ventriculitis de difícil manejo, es aún controvertido, actualmente existe bibliografía que reporta buenos resultados del tratamiento con lavado endoscópicos asociado al tratamiento combinado endovenoso e intraventricular para lograr mayores concentraciones de antibiótico intraventricular. Conclusión: Consideramos que el tratamiento combinado es una herramienta frente a las ventriculitis por patógenos resistentes a los tratamientos convencionales. En los casos con obstrucción del acueducto de Silvio recomendamos realizar acueductoplastia y colocación de catéter multifenestrado; para mantener la permeabilidad y la llegada de antibiótico al cuarto ventrículo.

Introduction: Ventriculitis represents an infectious emergency which is normally associated with neurosurgical procedures. The incidence is unknown due to the variation of the diagnostic criteria. Case description: We present a 6-month-old female patient with a history of post-hemorrhagic hydrocephalus. The patient was diagnosed with Enterobacter complex ventriculitis difficult to manage associated with peritoneal ventricular shunt (VP). The patient received combined antibiotic treatment with Meropenem intravenous and intravenous-intraventricular Colistin associated with endoscopic treatment. This endoscopic treatment consisted of washes, aspiration of the intraventricular purulent content, third endoscopic ventriculostomy (ETV), bilateral coagulation of the choroid plexus and aqueductoplasty with external ventricular drain catheter (EDV). This EDV was placed between the third and fourth ventricle in order to maintain its permeability so as to ensure the arrival of intraventricular antibiotics to the fourth ventricle. Discussion: Ventriculitis is associated with multiple complications and a high morbidity and mortality rate. The treatment of ventriculitis that is difficult to manage is still controversial. Currently several authors show good results of endoscopic lavage treatment associated with combined intravenous/intraventricular antibiotic treatment. This leads to greater intraventricular antibiotic concentrations. Conclusion: We consider that combined treatment has been successful for ventriculitis difficult to manage. In those cases, with obstruction of the Silvio aqueduct, it is recommended to perform aqueductoplasty and placement of a multi-fenestrated catheter; to maintain patency and the arrival of antibiotics in the fourth ventricle.

Efficacy and safety of expectant management in the treatment of tubal ectopic pregnancy: a systematic review and meta-analysis.

STUDY QUESTION: Is expectant management (EM) of tubal ectopic pregnancy (EP) an effective and safe treatment strategy when compared to alternative interventions? SUMMARY ANSWER: There is insufficient evidence to conclude EM yields a difference in the resolution of tubal EP, the avoidance of surgery or time to resolution of tubal EP when compared to intramuscular methotrexate in stable patients with ß-hCG <1500 IU/l. WHAT IS ALREADY KNOWN: The utilisation of medical and surgical management for EP is well established. EM aims to allow spontaneous resolution of the EP without intervention. STUDY DESIGN SIZE AND DURATION: We performed a systematic review and meta-analysis, searching Ovid MEDLINE, Embase, PsycINFO, CINAHL, Web of Science, OpenGrey.eu, Google Scholar, cross-referencing citations and trial registries to 15 December 2019. There were no limitations placed on language or publication date. Search terms included tubal EP and EM as well as variations of these terms. PARTICIPANTS/MATERIALS SETTING AND METHOD: We considered studies that included patients with tubal EP, EM as a comparator, and that were randomised controlled trials (RCTs). The primary outcome was resolution of tubal EP. Secondary outcomes included avoidance of surgery and the time to resolution of EP. Two reviewers independently selected the studies, assessed bias and extracted data. Relative risk (RR) and mean difference with 95% CI were assessed using a random effects model. The certainty of evidence was scored according to Grading of Recommendations Assessment, Development and Evaluation guidelines. MAIN RESULTS AND THE ROLE OF CHANCE: In total, 920 studies were screened. Five studies were eligible for inclusion in the systematic review. Two RCTs comparing methotrexate to EM were identified as being eligible for inclusion in meta-analysis. No RCTs comparing surgery to EM were identified. Compared with EM, there was insufficient evidence that methotrexate yields a difference on resolution of tubal EP (RR 1.04, 95% CI 0.88-1.23, P = 0.67; two RCTs, moderate-certainty evidence), avoiding surgery (RR 1.10, 95% CI 0.94-1.29, P = 0.25; two RCTs, low-certainty evidence) or the time to resolution of tubal EP (-2.56 days (favouring EM), 95% CI -7.93-2.80, P = 0.35; two RCTs, low-certainty evidence). LIMITATIONS REASONS FOR CAUTION: Only two RCTs with a total of 103 patients were eligible for inclusion in this meta-analysis. Further RCTs comparing EM to medical and surgical management are needed and these should also report adverse events. Patient preference should also be evaluated. WIDER IMPLICATIONS OF THE FINDINGS: We found insufficient evidence of differences in terms of resolution, avoidance of surgery and time to resolution between expectant and medical management. Given the imprecision in the effect estimates as demonstrated by the wide CIs, resulting in the downgrading of certainty of evidence for all outcomes in this meta-analysis, larger RCTs comparing interventions for tubal EP are needed. Caution should be exercised when trying to decide between EM and methotrexate to treat tubal EP. STUDY FUNDING/COMPETING INTERESTS: There was no funding for this study. NICM receives funding from various sources; none specifically supported this research. M.L. reports grants from Australian Women and Children's Research Foundation, outside the submitted work. M.A.: As a medical research institute, NICM Health Research Institute receives research grants and donations from foundations, universities, government agencies and industry. Sponsors and donors provide untied and tied funding for work to advance the vision and mission of the Institute. This systematic review was not specifically supported by donor or sponsor funding to NICM. M.A. reports a partnership grant with Metagenetics outside the submitted work. G.C. reports grants from Australian Women and Children's Research Foundation, personal fees from Roche and GE Healthcare, outside the submitted work. The remaining authors report no conflicts of interest. PROSPERO REGISTRATION NUMBER: CRD42020142736.

Monochorionic Triplet Gestation after Single Blastocyst Transfer Using Donor Oocytes: Case Report and Review.

We report a case of a 43-year-old patient with a monochorionic triamniotic triplet pregnancy after IVF with donor oocytes. After failed IVF attempts, the patient chose to undergo treatment with donor oocytes. Her 22-year-old oocyte donor underwent standard controlled ovarian hyperstimulation. The retrieved oocytes were fertilized in vitro, and one embryo was transferred at the blastocyst stage. At 6 weeks and 5 days of gestation, an ultrasound revealed monochorionic triamniotic (MCTA) triplets. The risk of monozygotic twinning in women undergoing in vitro fertilization (IVF) is reported to be higher than that in natural conception, although the causes of the phenomenon have not yet been clarified. Efforts still must be made in order to prevent monozygotic multiple pregnancies, associated with much greater chances of obstetric and perinatal morbidity and mortality.

Efectividad y técnica quirúrgica de la neuroendoscopía para el tratamiento de la hidrocefalia en menores de un año / Effectiveness and technique Surgical neuroendoscopy for the treatment of hydrocephalus in children under one year

Objetivos: Demostrar la efectividad y describir la técnica quirúrgica de la tercer ventriculostomía endoscópica (TVE) asociada a la coagulación de plexos coroideos (CPC) como alternativa terapéutica en pacientes menores de un año con hidrocefalia. Materiales y métodos: Se realizó un estudio prospectivo observacional, desde junio del 2013 a septiembre del 2016, que incluyó a 34 pacientes con hidrocefalia. Fueron criterios de inclusión pacientes menores de 12 meses de edad, cuyo tratamiento de la hidrocefalia fue una TVE con CPC. Los pacientes fueron clasificados de acuerdo al origen de su hidrocefalia. Hidrocefalia posinfección (HPI), hidrocefalia no posterior a infección (HNPI), hidrocefalia posterior a hemorragia intraventricular (HPHIV), hidrocefalia en pacientes portadores de mielomeningocele. Se confeccionó una tabla de puntuación inicial de éxito de TVE dependiendo de la edad del paciente, la etiología de la hidrocefalia y si poseía o no sistema de derivación de LCR previo. Resultados: Según el origen de la hidrocefalia, se encontraron MMC 13 (38,5%), HNPI 12 (35%), HPHIV 8 (23,5%) y HPI 1 (3%). El porcentaje de éxito, independientemente de la etiología, fue del 63,8% para todos los procedimientos realizados. El mejor resultado se observó en los recién nacidos con mielomeningocele (69%), HNPI (63,6%), HPHIV (60%) y HPI (0%). Conclusiones: La TVE-CPC puede ser considerada como una opción primaria en el tratamiento de la hidrocefalia, en pacientes menores de un año en centros con experiencia

Objectives: To demonstrate the effectiveness and describe the surgical technique of the Endoscopic third ventriculostomy (ETV) associated with coagulation choroid plexus (CPC) as a therapeutic alternative in patients younger than one year with hydrocephalus.Materials and Methods: A prospective observational study was conducted from June 2013 to September 2016 that included 34 patients with hydrocephalus. The inclusion criteria were patients younger than 12 months old, whose treatment of hydrocephalus was ETV with CPC. Patients were classified according to the origin of their hydrocephalus. Hydrocephalus post infection (HPI), not after infection Hydrocephalus (HNPI), Hydrocephalus following intraventricular hemorrhage (HPHIV), hydrocephalus in patients with myelomeningocele. An initial score table showing success of TVE depending on the patient's age, etiology of hydrocephalus and whether or not they had a prior CSF shunt system was made. Results: According to the origin of hydrocephalus MMC 13 (38.5%), HNPI 12 (35%), HPHIV 8 (23.5%) and HPI 1 (3%) were found. The success rate regardless of etiology was 63.8% for all procedures performed, the best result was observed in newborns with myelomeningocele (69%), HNPI (63.6%), HPHIV (60 %) and HPI 0%. Conclusions: ETV-CPC can be considered as a primary option in the treatment of hydrocephalus in patients younger than one year in experienced centers. Keywords: hydrocephalus, third ETV, coagulation choroid plexus

Use of platelet rich plasma in human infertility.

Successful embryo implantation requires good quality embryo but also needs a receptive endometrium site. In our clinical practice, we daily verify that an adequate endometrial growth is reached for successful implantation. To understand whether platelet rich plasma (PRP) can improve endometrium thickness and performance, PRP treatment was carried out after at least three of the classic medical protocols currently in use had been unsuccessfully adopted. Eight patients with more than 3 cryo-transfers cancelled because of failure of endometrial growth, defined as endometrium less than 6 mm, with negative hysteroscopic screening for endometrial pathology, and with negative bacteriologic screening, before present and all previous treatment, were selected to undergo PRP treatment. In 7 out of 8 treatments, an endometrial thickness greater than 6.5 mm (mean 6.9 mm) was reached, with endometrial three-layer pattern, before progesterone administration and embryo transfer was performed. In 6 out of 7 patients, who underwent embryo transfer, beta-HCG were positive, with 2 biochemical abortions, one miscarriage at 6-week pregnancy, two babies born and one drop-out. In this study, 8 patients had extraordinarily poor endometrial quality, and the endometrium was non-responsive to conventional estrogenic therapy, resulting in cycle cancellation. After application of PRP, the endometrial thickness was satisfactory in all the patients except one. Of these, beta-HCG was positive in 6 women, the pregnancy was progressing normally in 2 women, and one had an early miscarriage. We can suppose that the multiple implantation failures were caused by inefficient expression adhesion molecules, which can hypothetically be more represented after PRP application.

Postoperative complications in cirrhotic pediatric deceased donor liver transplantation: Focus on transfusion therapy.

Intraoperative transfusions seem associated with patient death and graft failure after PLTx. A retrospective analysis of recipients' and donors' characteristics and transplantation data in a cohort of patients undergoing PLTx from 2002 to 2009 at the Bergamo General Hospital was performed. A two-stage hierarchical Cox proportional hazard regression with forward stepwise selection was used to identify the main risk factors for major complications. In addition, propensity score analysis was used to adjust risk estimates for possible selection biases in the use of blood products. Over the 12-year period, 232 pediatric cirrhotic patients underwent PLTx. One-year patient and graft survival rates were 92.3% and 83.7%, respectively. The Kaplan-Meier shows that the main decrease in both graft and patient survival occurs during the first months post-transplantation. At the same time, it appears that most of the complications occur during the first month post-transplantation. One-month and 1-year patient complication-free survival rates were 24.8% and 12.1%, respectively. Our study shows that intraoperative red blood cells and platelet transfusions are independent risk factors for developing one or more major complications in the first year after PLTx. Decreasing major complications will improve the health status and overall long-term patient survival after pediatric PLTx.

Histamine release positive test associates with disease remission in chronic spontaneous urticaria: a proof-of-concept study.

SUMMARY: Background. Histamine release (HR) test has previously been shown to predict the presence of endogenous histamine-releasing factors in chronic spontaneous urticaria (CSU). Objectives and methods. Twenty CSU patients unresponsive to antihistamine treatment were enrolled in order to evaluate the correlations between HR test results and demographic features, quality of life, disease activity, clinical course, and autologous serum and plasma skin tests (ASST and APST). Results. All patients with positive HR test (9/9, 100%) had a more severe disease activity at onset (urticaria activity score, UAS > 2) when compared to negative HR test patients (5/11; p = 0.04). Quality of life questionnaire's results were not substantially different between HR positive and negative subgroups at baseline (p > 0.05), and results of HR test and ASST/APST did not co-segregate (p > 0.05). After 12 months, patients with a positive HR test had a significant reduction of disease activity (p = 0.003) whereas patients with a negative HR test did not (p > 0.05), leading to disease remission and antihistamine treatment withdrawal in 67% (6/9) of positive HR test patients versus 18% (2/11) of negative HR test patients (p = 0.027). Conclusions. Positive HR test may predict spontaneous CSU remission at 12 months.

Shrimp Allergy: Analysis of Commercially Available Extracts for In Vivo Diagnosis.

BACKGROUND AND OBJECTIVE: Skin prick testing (SPT) with commercial extracts is the first step in the diagnosis of shrimp allergy, although its clinical efficiency is unknown. Objective: To analyze the clinical usefulness of all commercial crustacean extracts available for SPT in Italy. METHODS: We performed a multicenter study of 157 shrimp-allergic patients who underwent SPT with 5 commercial crustacean extracts and with house dust mite (HDM) extract. Commercial extracts were analyzed using SDS-PAGE and compared with a freshly prepared in-house shrimp extract. IgE to Pen a 1/Pen m 1, Pen m 2, and Pen m 4 was determined, and immunoblot analysis was performed on a large number of sera. RESULTS: The skin reactions caused by commercial crustacean extracts were extremely heterogeneous, resulting in 32 clinical profiles, with marked differences in protein content and missing proteins at molecular weights corresponding to those of major shrimp allergens. Only strong Pen a 1/Pen m 1 reactors reacted to both HDM and all 5 commercial extracts in SPT. Most patients, including those who were tropomyosin-negative, reacted to HDM. Patients reacted to a large and variable array of proteins, and IgE reactivity was common at high molecular weights (>50 kDa). CONCLUSIONS: The in vivo diagnosis of shrimp allergy must continue to be based on SPT with fresh material. Shrimp-allergic patients frequently react to a number of ill-defined high-molecular-weight allergens, thus leaving currently available materials for component-resolved diagnosis largely insufficient. Mites and crustaceans probably share several allergens other than tropomyosin.

Shrimp allergy: analysis of commercially available extracts for in vivo diagnosis

Background: Skin prick testing (SPT) with commercial extracts is the first step in the diagnosis of shrimp allergy, although its clinical efficiency is unknown. Objective: To analyze the clinical usefulness of all commercial crustacean extracts available for SPT in Italy. Methods: We performed a multicenter study of 157 shrimp-allergic patients who underwent SPT with 5 commercial crustacean extracts and with house dust mite (HDM) extract. Commercial extracts were analyzed using SDS-PAGE and compared with a freshly prepared in-house shrimp extract. IgE to Pen a 1/Pen m 1, Pen m 2, and Pen m 4 was determined, and immunoblot analysis was performed on a large number of sera. Results: The skin reactions caused by commercial crustacean extracts were extremely heterogeneous, resulting in 32 clinical profiles, with marked differences in protein content and missing proteins at molecular weights corresponding to those of major shrimp allergens. Only strong Pen a 1/Pen m 1 reactors reacted to both HDM and all 5 commercial extracts in SPT. Most patients, including those who were tropomyosin-negative, reacted to HDM. Patients reacted to a large and variable array of proteins, and IgE reactivity was common at high molecular weights (>50 kDa). Conclusions: The in vivo diagnosis of shrimp allergy must continue to be based on SPT with fresh material. Shrimp-allergic patients frequently react to a number of ill-defined high-molecular-weight allergens, thus leaving currently available materials for componentresolved diagnosis largely insufficient. Mites and crustaceans probably share several allergens other than tropomyosin (AU)

Introducción: Las pruebas cutáneas con extractos comerciales representan el primer paso en el diagnóstico de alergia a gamba, si bien, su eficacia clínica no está bien definida. Objetivos: El objetivo de este estudio fue analizar la utilidad clínica de todos los extractos comerciales disponibles en Italia frente a crustáceos en pruebas cutáneas. Métodos: En un estudio multicéntrico, se incluyeron 157 pacientes alérgicos a gamba a los que se realizaron pruebas cutáneas con cinco extractos comerciales de crustáceos y con ácaros del polvo doméstico. Los extractos comerciales fueron analizados mediante SDS-PAGE y comparados con un extracto de gamba preparado en fresco. Se determinó IgE frente a Pen a 1/Pen m 1; Pen m 2, y Pen m 4; y el análisis mediante inmunoblotting se realizó en un amplio número de sueros. Resultados: Los extractos de gamba comercializados dieron lugar a reacciones cutáneas muy poco homogéneas en 32 perfiles clínicos diferentes; así mismo, mostraron grandes diferencias en contenido proteico y, en algunos casos, a falta de proteína a pesos moleculares correspondientes a alérgenos mayoritarios de gamba. Únicamente los reactores más fuertes a Pen a1 /Pen m 1 reaccionaron tanto a ácaros del polvo de casa como a los cinco extractos comerciales en pruebas cutáneas. La mayoría de los pacientes, incluyendo los negativos a tropomiosina, reaccionaron a los ácaros del polvo. Los pacientes reaccionaron a un amplio y variable array de proteínas y se detectó con frecuencia reactividad de IgE en pesos moleculares altos (>50 kDa). Conclusiones: El diagnóstico in vivo de alergia a gamba todavía debe estar basado en pruebas cutáneas prick con producto fresco. Los pacientes alérgicos a gamba a menudo reaccionan a un número de alérgenos de peso molecular alto poco definido, lo que hace que las moléculas disponibles hoy en día para el diagnóstico por componentes sean muy insuficiente. Ácaros y crustáceos probablemente comparten varios alérgenos además de la tropiomiosina (AU)

Nasal powders of thalidomide for local treatment of nose bleeding in persons affected by hereditary hemorrhagic telangiectasia.

In this work nasal powder formulations of thalidomide were designed and studied to be used by persons affected by hereditary hemorrhagic telangiectasia as a complementary anti-epistaxis therapy, with the goal of sustaining the effect obtained with thalidomide oral treatment after its discontinuation for adverse effects. Three nasal powders were prepared using as carriers ß-CD or its more hydrophilic derivatives such as hydropropyl-ß-CD and sulphobutylether-ß-CD and tested with respect to technological and biopharmaceutical features after emission with active and passive nasal powder devices. For all formulated powders, improved dissolution rate was found compared to that of the raw material, making thalidomide promptly available in the nasal environment at a concentration favouring an accumulation in the mucosa. The very limited transmucosal transport measured in vitro suggests a low likelihood of significant systemic absorption. The topical action on bleeding could benefit from the poor absorption and from the fact that about 2-3% of the thalidomide applied on the nasal mucosa was accumulated within the tissue, particularly with the ß-CD nasal powder.

Patients with breakthrough reactions to iodinated contrast media have low incidence of positive skin tests.

BACKGROUND: The term "breakthrough reactions" designates repeated hypersensitivity reactions to iodinated contrast media (ICM) despite premedication with glucocorticoids and antihistamines. We aimed to retrospectively evaluate the rate of positive skin test (STs) in our cohort of patients with previous breakthrough reactions to different ICMs. METHODS: A series of 35 patients, who experienced at least one breakthrough reaction to ICM and who underwent STs within 6 months from the reaction were studied, and results were compared to a control group of patients with a first hypersensitivity reaction occurred without premedication. Skin prick tests (SPT), intradermal tests (IDT) and patch tests (PT) at different dilutions, with a set of three to four ICM were performed. RESULTS: Of the 35 patients with prior breakthrough reactions, 57% had an immediate reaction (IR) and 43% had a non-immediate reaction (NIR). Patients who experienced the first hypersensitivity IR or NIR, later had one or more breakthrough IR or NIR, respectively. Overall, 29% (10/35) of patients with prior breakthrough reactions resulted positive to STs compared to 57% (16/28) of the control group (p < 0.05). No significant difference in allergy history, age, sex, other clinical / demographic features nor chronic use of ACE-inhibitor, beta-blockers or NSAIDs was observed. CONCLUSION: This preliminary finding suggests that patients with prior breakthrough reactions have significantly lower immunologically proven ICM reactions (positive STs) if compared to non-breakthrough patients. According to that, a considerable number of breakthrough reactions seems to be non-allergic hypersensitivity reactions or reactions which could be mostly prevented by a proper, well-timed skin testing. Larger prospective studies are needed to confirm these results, with a more careful analysis of patients' risk factors, a laboratory assessment that includes an in vitro allergy diagnostics, and hopefully a drug provocation test for selected cases.