A Unique Case of Perforated Appendicitis in a Giant Incarcerated Right-Sided Inguinal Hernia: Challenges and Surgical Management.

BACKGROUND Amyand hernia is a rare condition described as the presence of the appendix within an inguinal hernia. The clinical presentation of can be atypical, depending on the length of the defect's history and the size of the hernia. As inguinal hernia repair is considered a routine surgical procedure, giant hernias are mostly encountered in countries with limited medical care or with patient rejection of surgical management.

Normal B cells express ZAP70 in chronic lymphocytic leukemia: A link between autoimmunity and lymphoproliferation?

ZAP70 has a prognostic value in chronic lymphocytic leukemia (CLL), through altered B-cell receptor signaling, which is important in CLL pathogenesis. A good correlation between ZAP70 expression in CLL cells and the occurrence of autoimmune phenomena has been reported. Yet, the great majority of CLL-associated autoimmune cytopenia is due to polyclonal immunoglobulin (Ig) G synthesized by nonmalignant B cells, and this phenomenon is poorly understood.

Reverse Abdominoplasty in a Patient Presenting with Subcostal Nephrectomy Scar and Transverse Muscle Incisional Hernia.

Abdominoplasty and its different approaches have been widely described to improve abdominal wall contour. However, the role of reverse abdominoplasty, a less commonly performed technique, and its indications are not fully understood. Recent advances in the understanding of the vascular supply to the anterior abdominal wall have made this technique more popular, but there is still limited information available in the literature.

High-throughput microfluidic blood testing to phenotype genetically linked platelet disorders: an aid to diagnosis.

Linking the genetic background of patients with bleeding diathesis and altered platelet function remains challenging. We aimed to assess how a multiparameter microspot-based measurement of thrombus formation under flow can help identify patients with a platelet bleeding disorder. For this purpose, we studied 16 patients presenting with bleeding and/or albinism and suspected platelet dysfunction and 15 relatives.

One-step totally robotic Hartmann reversal and complex abdominal wall reconstruction with bilateral posterior component separation: a technical note.

Aim: This paper describes a robotic approach to combined gastrointestinal continuity restoration and complex abdominal wall reconstruction after Hartmann's procedure complicated by large midline and parastomal hernias.

Methods: A robotic Hartmann reversal is performed, followed by robotic retromuscular abdominal wall reconstruction of all ventral defects with bilateral posterior component separation using the double-docking approach. Surgical steps are thoroughly described, and the accompanying video highlights critical steps of the procedure, anatomical landmarks and technical details relevant to successful completion.

Discriminating young platelets on human leukocyte antigen-I expression highlights their extremely high reactivity potential.

Background: The platelet population is heterogeneous, with different subsets that differ on the basis of their function and reactivity. An intrinsic factor participating in this difference of reactivity could be the platelet age. The lack of relevant tools allowing a formal identification of young platelets prevents so far to draw solid conclusions regarding platelet reactivity.

Anti-HLA Class I alloantibodies in platelet transfusion refractoriness: From mechanisms and determinants to therapeutic prospects.

Patients with hematological disorders and severe thrombocytopenia require extensive and iterative platelet transfusion support. In these patients, platelet transfusion refractoriness represents a serious adverse transfusion event with major outcomes for patient care. Recipient alloantibodies against the donor HLA Class I antigens expressed at the cell surface of platelets result in a rapid removal of transfused platelets from the circulation and thus, therapeutic and prophylactic transfusion failure leading to a major bleeding risk.

Full-scale simulations to improve disaster preparedness in hospital pharmacies.

Purpose: Assess whether full-scale simulation exercises improved hospital pharmacies' disaster preparedness.

Methods: Swiss hospital pharmacies performed successive full-scale simulation exercises at least four months apart. An interprofessional team created two scenarios, each representing credible regional-scale disasters involving approximately fifty casualties (a major road accident and a terrorist attack).

A gain-of-function variant in the Wiskott-Aldrich syndrome gene is associated with a MYH9-related disease-like syndrome.

While loss-of-function variants in the WAS gene are associated with Wiskott-Aldrich syndrome and lead to microthrombocytopenia, gain-of-function variants of WAS are associated with X-linked neutropenia (XLN) and the absence of microthrombocytopenia. Only a few XLN families have been reported so far, and their platelet phenotype was not described in detail. To date, no renal involvement was described in XLN.

Mutations in the most divergent α-tubulin isotype, α8-tubulin, cause defective platelet biogenesis.

Background: In the panel of genes commonly associated with inherited macrothrombocytopenia, an important fraction encodes key cytoskeletal proteins such as tubulin isotypes, the building blocks of microtubules. Macrothrombocytopenia-causing mutations have been identified in the TUBB1 and TUBA4A genes, emphasizing their importance in the formation of platelets and their marginal band, a unique microtubule ring-like structure that supports the platelet typical disc-shaped morphology. This raised the hypothesis that other tubulin isotypes normally expressed in platelets could play a similar role in their formation.

Sacro-coxxygial hygiene, a key factor in the outcome of pilonidal sinus surgical treatment?

Background: Surgical wound infection contributes to prolonged recovery time after pilonidal sinus excision. As a standard procedure after surgery, we recommend our patients to perform water irrigations in the intergluteal cleft 4 to 6 times a day during the post-operative period. Our hypothesis is that this should reduce healing time and complication rates.

Atypical focal segmental glomerulosclerosis associated with a new PODXL nonsense variant.

Background: Podocalyxin (PODXL) is a highly sialylated adhesion glycoprotein that plays an important role in podocyte's physiology. Recently, missense and nonsense dominant variants in the PODXL gene have been associated with focal segmental glomerulosclerosis (FSGS), a leading cause of nephrotic syndrome and kidney failure. Their histologic description, however, was superficial or absent.

Surgical management of vascular Ehlers-Danlos syndrome and its challenges: a case report.

Background: Ehlers-Danlos syndrome (EDS) is a heterogeneous group of rare inherited diseases involving connective tissue. Vascular EDS (vEDS) is associated with abnormal type III collagen, which is an essential component of skin, hollow organs and arterial walls, and causes potentially fatal visceral and arterial complications. The surgical management of these patients is challenging and should be limited to life-saving procedures.

Platelet δ-Storage Pool Disease: An Update.

Platelet dense-granules are small organelles specific to the platelet lineage that contain small molecules (calcium, adenyl nucleotides, serotonin) and are essential for the activation of blood platelets prior to their aggregation in the event of a vascular injury. Delta-storage pool diseases (δ-SPDs) are platelet pathologies leading to hemorrhagic syndromes of variable severity and related to a qualitative (content) or quantitative (numerical) deficiency in dense-granules. These pathologies appear in a syndromic or non-syndromic form.

Pathogenic and likely pathogenic variants in at least five genes account for approximately 3% of mild isolated nonsyndromic thrombocytopenia.

Background: Thrombocytopenia has a variety of different etiologies, both acquired and hereditary. Inherited thrombocytopenia may be associated with other symptoms (syndromic forms) or may be strictly isolated. To date, only about half of all the familial forms of thrombocytopenia have been accounted for in terms of well-defined genetic abnormalities.

[Ventral hernia surgery : New minimally invasive approaches].

Ventral hernia surgery has undergone major changes over the past decade with the emergence of new minimally invasive techniques. They merge fundamental concepts of parietal reconstruction of open surgery into a laparoscopic approach, aiming to reduce surgical site complications and to enhance recovery. The spread of robotic assistance systems in the field of abdominal wall surgery facilitates access to this type of procedures and allows their application in increasingly complex cases.

Robotic versus open primary ventral hernia repair: A randomized controlled trial (Robovent Trial).

Background: The objective of the present study is to compare the outcomes open PVHR and robotic PVHR.

Methods/design: The present study will be a randomized single-blinded controlled trial with intention-to-treat analysis comparing robotic PVHR to open PVHR in adult patients undergoing elective PVHR with a defect ranging between 1-5 cm. Patient refusing to participate, not able to give informed consent, with history of intra-abdominal surgery contraindicating a robotic surgical approach will be excluded.

Chronic Pain After Gastric Bypass: Another Argument to Support Mesenteric Windows Closure.

Chronic pain is frequent after Roux-en-Y gastric bypass (RYGB). Recurrent internal hernias (IHs) may be responsible for chronic abdominal pain. Physical examination and computed tomography are often inconclusive.

Platelet preparation for function testing in the laboratory and clinic: Historical and practical aspects.

Laboratory tests of platelet function are instrumental in studying platelet physiology and inherited or acquired platelet abnormalities. Light transmission aggregometry, developed in the early 1960s, is still considered the gold standard for the identification and diagnosis of platelet function disorders. Since then, novel techniques have been developed, including flow-based assays and flow cytometry.

Antithrombotic prophylaxis for surgery-associated venous thromboembolism risk in patients with inherited platelet disorders. The SPATA-DVT Study.

Major surgery is associated with an increased risk of venous thromboembolism (VTE), thus the application of mechanical or pharmacologic prophylaxis is recommended. The incidence of VTE in patients with inherited platelet disorders (IPD) undergoing surgical procedures is unknown and no information on the current use and safety of thromboprophylaxis, particularly of low-molecular-weight-heparin in these patients is available. Here we explored the approach to thromboprophylaxis and thrombotic outcomes in IPD patients undergoing surgery at VTE-risk participating in the multicenter SPATA study.

Gangrenous gas necrosis of the spleen: a case report.

Background: Splenic abscess usually arises from hematogenous spread. Causative pathogens are various and anaerobic pathogens are rarely reported.

Case Presentation: We report the case of a 50-year-old male patient who was admitted for sepsis due to gangrenous necrosis of the spleen associated with bacteremia.