Septic shock revealing boerhaave's syndrome a case report.

INTRODUCTION AND IMPORTANCE: Boerhaave's syndrome, recognized as spontaneous esophageal rupture, is an uncommon and perilous medical condition marked by the spontaneous tearing of the esophagus. This paper highlights the importance of an early diagnosis and its correlation to better outcomes for a rare pathology with high mortality. CLINICAL PRESENTATION: A 67-year-old female presenting with unexplored vomiting and hypertension, presented to the ER with a septic shock. The patient's clinical deterioration prompted emergency exploration, revealing a dilated esophagus with a 3-cm perforation. Despite surgical intervention, including suturing with a T-tube and esophageal exclusion, the patient succumbed to multiorgan failure. CLINICAL DISCUSSION: Boerhaave's syndrome, triggered by forceful vomiting, presents diverse clinical manifestations, making accurate diagnosis challenging. The characteristic triad of vomiting, pain, and subcutaneous emphysema is observed in a minority of cases, often overshadowed by acute respiratory distress. Diagnostic modalities include chest X-rays, contrast esophagography, and computed tomography, aiding in visualizing contrast leakage and confirming the diagnosis. The choice of surgical technique, ranging from esophageal suturing to esophagectomy, depends on the duration between rupture and surgery initiation. In this case, a bipolar esophageal exclusion was performed due to the patient's critical condition. CONCLUSION: Boerhaave's syndrome demands consideration in patients presenting with thoracic pain and vomiting, particularly in those with a pathological esophagus. Early diagnosis and surgical intervention remain pivotal in improving outcomes. Identification of hydro-pneumothorax in radiographic studies should prompt consideration of spontaneous esophageal rupture, highlighting the need for heightened clinical suspicion in nonspecific clinical scenarios.

Primary giant hydatid cyst of the retroperitoneum: Diagnosis and therapeutic approaches.

INTRODUCTION AND IMPORTANCE: Primary hydatid cyst of the retroperitoneum is an exceedingly rare manifestation of hydatid disease. Diagnosis proves challenging due to nonspecific symptoms, and the condition is typically not suspected when facing a retroperitoneal cystic mass, necessitating awareness among clinicians and surgeons, particularly in endemic regions. CASE PRESENTATION: A 45-year-old male with a three-month history of progressive abdominal enlargement and pain. Living in a rural area, he exhibited a 30 cm, well-defined retroperitoneal cyst, with no guarding confirmed by CT-scan, with characteristic daughter cysts. The diagnosis of primary retroperitoneal hydatid cyst was supported by positive hydatid serology and eosinophilia. Surgical intervention was crucial, and a complete pericystectomy, with 4 cm of pericyst on the aorta due to safety concerns, was performed after three months of preoperative albendazole-based treatment. The postoperative course was uneventful, and a two-year follow-up revealed no recurrence. CLINICAL DISCUSSION: The prevalence of hydatid disease in North Africa is high, yet retroperitoneal cases are rare. The difficulty to diagnosis retroperitoneal masses, underscores the importance of precise patient evaluation and detailed imaging analysis. Percutaneous puncture is contraindicated due to the risk of dissemination, highlighting even more the significance of accurate preoperative diagnosis. Surgery, coupled with Albendazole treatment, remains the gold-standard, associated with meticulous intraoperative precautions to prevent disease dissemination. CONCLUSION: Primary retroperitoneal hydatid cyst is rare. Diagnosis is difficult. Precise determination of patient's background and detailed analysis of imaging findings are mandatory. Percutaneous puncture is forbidden as it leads to disease spreading or even anaphylactic shock. Surgical excision is the gold-standard.

Intrauterine device migration resulting in acute appendicitis: A case report.

INTRODUCTION AND IMPORTANCE: The Intrauterine Contraceptive Device (IUD), a widely used contraceptive since 1965, has demonstrated efficacy but is associated with complications such as bleeding, pain, and rare occurrences of perforation. This case report details an IUD migration into the peritoneal cavity, leading to acute appendicitis. CASE PRESENTATION: A 33-year-old woman, with a history of IUD insertion 16 months prior, presented with pelvic pain. Gynecological examination and computed tomography, revealed the IUD intraperitoneal migration. The patient underwent laparoscopic extraction of the IUD which was embedded in the appendix and appendectomy, with an uneventful recovery. CLINICAL DISCUSSION: This case emphasizes the complexity of IUD migration and its rare association with acute appendicitis, underscoring the importance of vigilant monitoring and prompt intervention. We also explored factors contributing to IUD perforation risk, imaging modalities for detection, and emphasizes the necessity of surgical removal upon confirmation. We highlight the fact that despite the atypical presentation with minimal symptoms, we should always consider emergency situations. Surgical intervention, particularly laparoscopy, may be the standard approach for managing migrated IUDs. CONCLUSION: We insist about the critical need for thorough assessment and vigilance in managing IUD-related complications, emphasizing timely intervention to ensure patient safety. This case contributes valuable insights into the complexities surrounding IUD migration, urging healthcare professionals to remain attentive to potential injuries in patients with a history of IUD insertion and abdominal pain.

Pleural migration of biliary stent 10 years following treatment of hepatic hydatic echinococcosis: A case report.

INTRODUCTION: Echinococcosis, caused by larval stages of taeniid cestodes, primarily affects the liver and is commonly treated surgically. However, a complication post-treatment is biliary fistula, necessitating interventions like biliary stents. While stent complications are recognized, proximal migration leading to pneumonia is exceptionally rare. This case report details an unusual occurrence of biliary stent migration years after hepatic hydatid echinococcosis treatment. CASE PRESENTATION: A 42-year-old patient underwent 2014 surgery for a large hydatid cyst, resulting in a biliary fistula. Endoscopic sphincterotomy and biliary stent placement led to a successful outcome. Lost to follow-up, the patient reappeared in 2022 with basithoracic pain, fever, and a thoracic CT scan revealing transdiaphragmatic stent migration causing basal pneumonitis. Antibiotic therapy and endoscopic stent removal ensued with an uncomplicated recovery. CLINICAL DISCUSSION: This report emphasizes a rare complication that is proximal migration of a biliary stent 10 years post-initial placement for biliary fistula management. Despite the absence of typical risk factors. We managed a successful endoscopic retrieval. This highlights the importance of vigilance and follow-up for potential complications associated with biliary stent. Unusual presentations, like pneumonitis, underscore the need for awareness and a cautious approach. CONCLUSION: The primary complication following surgical intervention for hepatic hydatid cysts is the development of an external biliary fistula, necessitating the use of biliary stents for treatment. Given the rarity of complications observed in our case, the removal of stents post-treatment for biliary fistula becomes crucial, underscoring the significance of vigilant follow-up care.

Surgical management of a proximal splenic artery aneurysm: A case report.

INTRODUCTION AND IMPORTANCE: Splenic artery aneurysms (SAA's) pose a rare yet clinically significant challenge, characterized by the weakening and ballooning of the splenic artery, potentially leading to severe complications such as rupture and hemorrhage. CASE PRESENTATION: A 52-year-old female presenting with biliary colic. Diagnostic imaging revealed a saccular lesion closely associated with gallstones. A multidisciplinary approach guided the decision for surgery due to the size and location of the aneurysm. A bi sub costal laparotomy was performed, after the resection of the aneurysm, an arterial anastomosis with pds 5/0 suture was performed. CLINICAL DISCUSSION: SAA's treatment modalities are tailored based on aneurysm localization and size. Imaging modalities such as Doppler ultrasound and CT angiography play a crucial role in accurate diagnosis, providing essential information for treatment planning. Treatment options include endovascular embolization, and surgical intervention. Traditionally open surgical techniques, including ligation of the splenic artery, aneurysmectomy, and splenectomy. Surgical treatment, especially for proximal aneurysms, is highlighted, with the presented alternative approach of resection with end-to-end anastomosis, showcasing an alternative surgical technique aimed at reducing the risk of spleen infarction. CONCLUSION: SAA's are a rarity that emphasizes the need for early detection and intervention. We are urged to maintain a high index of suspicion, particularly in high-risk individuals. We report an alternative surgical technique that we hope will contributes to the expanding repertoire of approaches, calling for further research to optimize SAA management strategies in the quest for improved patient outcomes.

Bouveret Syndrome: A rare form of gallstone ileus a case report.

INTRODUCTION AND IMPORTANCE: Bouveret Syndrome, a rare form of gallstone ileus, involves the migration and impaction of a gallstone in the duodenum or stomach, causing gastric outlet obstruction. Early intervention and a comprehensive care plan are essential for favorable outcomes. CASE PRESENTATION: This article presents a case of an 82-year-old female with a history of coronary artery disease and untreated gallstones. The patient experienced nausea, vomiting, and abdominal pain for two weeks. Diagnostic procedures revealed a cholecystoduodenal fistula with a 4 cm stone lodged at the duodenojejunal angle. For our patient the gallstone was moved to the jejunum, followed by enterotomy and a latero_lateral gastroenteroanastomosis. CLINICAL DISCUSSION: The rarity of Bouveret Syndrome and its nonspecific symptoms make diagnosis challenging, necessitating differentiation from other gastrointestinal disorders. Esophagogastroduodenoscopy (EGD) and imaging, such as computed tomography (CT), play crucial roles in diagnosis. In this case, the EGD did not show gallstones up to the second part of the duodenum. Management involves a multidisciplinary approach, with supportive care for stabilization and the primary goal of removing the impacted stone. Treatment options include endoscopic, surgical, or lithotripsy techniques. Bouveret Syndrome poses challenges due to its rarity, leading to delayed diagnosis. Prognosis varies based on factors such as stone size, location, and overall patient condition. CONCLUSION: Through this case we emphasizes the importance of awareness, timely diagnosis, and appropriate management, with EGD and CT scan playing key roles in diagnosis. Surgical intervention remains a viable treatment option when endoscopic approaches are unavailable. The article highlights the controversial nature of fistula repair in Bouveret Syndrome.

Importance of Dipole Orientation in Electrostatic Aerosol Filtration.

Electrostatic charge is a major part of modern-day aerosol filtration media (e.g., N95 respirators and surgical facemasks) that has remained poorly understood due to its complicated physics. As such, charging a fibrous material has relied on empiricism in dire need of a mathematical foundation to further advance product design and optimization. In this concern, we have conducted a series of numerical simulations to improve our understanding of how an electrostatically charged fiber captures airborne particles and to quantify how the fiber's dipole orientation impacts its capture efficiency. Special attention was paid to the role of Coulomb and dielectrophoretic forces in the capture of particles of different charge polarities (e.g., particles having a Boltzmann charge distribution). Simulation results were compared with the predictions of the popular empirical correlations from the literature and discussed in detail. Predictions of the empirical correlations better agreed with the simulation results obtained for fibers with a dipole perpendicular to the flow direction rather than for fibers with a dipole parallel to the flow. This indicates that such empirical correlations are more suitable for filters charged via contact electrification (friction charging), where the dipoles are mostly perpendicular to the flow direction, and less suitable for corona-charged media, where the fiber dipoles are generally parallel to the flow direction.

Adult intussusceptions: Clinical presentation, diagnosis and therapeutic management.

BACKGROUND: Adult intussusception is a rare clinical entity. It is an uncommon cause of intestinal obstruction in adult. It often presents with nonspecific symptoms and preoperative diagnosis remains difficult. The purpose of this study was to determine the clinical entity and surgical approach of adult intussusception. METHODS: We have conducted a retrospective descriptive study starting from 2006 until 2014. We reviewed data for all patients that had been admitted to our department for intestinal intussusception. RESULTS: Eight consecutive patients were admitted to our department. The mean age was 48 years old (20-71). The sex ratio was 0,6. The clinical presentation was acute in 5 cases. A computed tomography was performed in 6 cases. The diagnosis of gastrointestinal intussusception was made preoperatively in 100% of patients. All patients underwent surgery. An organic lesion was identified in 100% of the cases. In all cases, resection of the intussuscepted intestinal loop was done without intestinal reduction. All patients were well followed up and recurrences have been documented. CONCLUSION: In adults, intussusception is usually secondary to an organic cause. In the absence of signs of severity, etiologic diagnosis based on CT allows the diagnosis of the intussusception and sometimes can detect the causal lesion. Therapeutic sanction of intussusception is surgery and there is more emphasis towards resection without reduction.

Triazene compounds in the treatment of acute myeloid leukemia: a short review and a case report.

Acute myeloid leukemia (AML) is a highly lethal disease, especially in old patients. Chemoresistance and the absence of host immune responses against autochthonous malignancy play a major role in the poor prognosis of AML. The triazene compounds Dacarbazine and Temozolomide are monofunctional alkylators that donate methyl groups to many sites in DNA, including the O(6)-position of guanine producing O(6)-methylguanine (O(6)-MeG). If not repaired, O(6)-MeG frequently mispairs with thymine during DNA duplication. O(6)-MeG:T mismatches can be recognized by the mismatch repair (MMR) system which activates a cascade of molecular events leading to cell cycle arrest and cell death. If MMR is defective, cells continue to divide and GC → AT transition mutations occur. In preclinical models, such mutations can lead to the appearance of abnormal proteins containing non-self peptides ("chemical xenogenization" CX) that can be recognized by host cell-mediated immunity. Repair of O(6)-MeG is achieved by the DNA repair protein, O(6)-methylguanine-DNA methyltransferase (MGMT), which removes the methyl adduct in an autoinactivating stoichiometric reaction. High MGMT levels attenuate the pharmacodynamic effects of triazenes. In the last few years, triazenes, alone or with MGMT inhibitors, have been tested in AML. In view of their potential activity as CX inducers, triazenes could offer the additional advantage of host anti-leukemia immune responses. The present paper describes several studies of leukemia treatment with triazenes and a case of acute refractory leukemia with massive skin infiltration by malignant cells. Treatment with Temozolomide and Lomeguatrib, a potent MGMT inhibitor, produced a huge, although transient, blastolysis and complete disappearance of all skin lesions.

Expression of the soluble vascular endothelial growth factor receptor-1 in cutaneous melanoma: role in tumour progression.

BACKGROUND: Vascular endothelial growth factor (VEGF)-A, placenta growth factor (PlGF) and their corresponding membrane receptors are involved in autocrine and paracrine regulation of melanoma growth and metastasis. Besides the membrane receptors, a soluble form of the VEGF receptor (VEGFR)-1 (sVEGFR-1) has been identified, that behaves both as a decoy receptor, sequestering VEGF-A and PlGF, and as an extracellular matrix (ECM) molecule, promoting endothelial cell adhesion and migration through the interaction with α5ß1 integrin. OBJECTIVES: To analyse whether sVEGFR-1 plays a role during melanoma progression. METHODS: sVEGFR-1 expression was evaluated in a panel of 36 melanoma cell lines and 11 primary human melanocyte cultures by quantitative real-time polymerase chain reaction analysis and in specimens of primary or metastatic melanoma lesions from 23 patients by immunohistochemical analysis. RESULTS: sVEGFR-1 expression was highly upregulated in melanoma cell lines with respect to human melanocytes. Interestingly, cell lines obtained from cutaneous metastases showed a significant reduction of sVEGFR-1 expression, as compared with cell lines derived from primary tumours. These results were confirmed by immunohistochemical analysis of sections from primary skin melanomas and the corresponding cutaneous metastases, suggesting that modulation of sVEGFR-1 expression influences ECM invasion by melanoma cells and metastasis localization. Moreover, we provide evidence that adhesion of melanoma cells to sVEGFR-1 is favoured by the activation of a VEGF-A/VEGFR-2 autocrine loop. CONCLUSIONS: Our data strongly suggest that sVEGFR-1 plays a role in melanoma progression and that low sVEGFR-1/VEGF-A and sVEGFR-1/transmembrane VEGFR-1 ratios might predict a poor outcome in patients with melanoma.

Cells deficient in the base excision repair protein, DNA polymerase beta, are hypersensitive to oxaliplatin chemotherapy.

A significant proportion of human cancers overexpress DNA polymerase beta (Pol beta), the major DNA polymerase involved in base excision repair. The underlying mechanism and biological consequences of overexpression of this protein are unknown. We examined whether Pol beta, expressed at levels found in tumor cells, is involved in the repair of DNA damage induced by oxaliplatin treatment and whether the expression status of this protein alters the sensitivity of cells to oxaliplatin. DNA damage induced by oxaliplatin treatment of HCT116 and HT29 colon cancer cells was observed to be associated with the stabilization of Pol beta protein on chromatin. In comparison with HCT116 colon cancer cells, isogenic oxaliplatin-resistant (HCT-OR) cells were found to have higher constitutive levels of Pol beta protein, faster in vitro repair of a DNA substrate containing a single nucleotide gap and faster repair of 1,2-GG oxaliplatin adduct levels in cells. In HCT-OR cells, small interfering RNA knockdown of Pol beta delayed the repair of oxaliplatin-induced DNA damage. In a different model system, Pol beta-deficient fibroblasts were less able to repair 1,2-GG oxaliplatin adducts and were hypersensitive to oxaliplatin treatment compared with isogenic Pol beta-expressing cells. Consistent with previous studies, Pol beta-deficient mouse fibroblasts were not hypersensitive to cisplatin treatment. These data provide the first link between oxaliplatin sensitivity and DNA repair involving Pol beta. They demonstrate that Pol beta modulates the sensitivity of cells to oxaliplatin treatment.

Molecular alterations at chromosome 9p21 in melanocytic naevi and melanoma.

BACKGROUND: The chromosome 9p21 and its CDKN locus, with the p16 tumour suppressor gene (CDKN2A), are recognized as the genomic regions involved in the pathogenesis of melanoma. OBJECTIVES: To elucidate further the role of such regions during the different phases of melanocytic tumorigenesis. METHODS: Tissue sections from naevi, primary and metastatic melanomas were investigated by fluorescence in situ hybridization for allelic loss at the 9p21 chromosome and by immunochemistry for p16CDKN2A expression. RESULTS: Dysplastic naevi and primary or secondary melanomas were found to carry hemizygous deletions within the entire 9p21 region at similar frequencies (varying from 55% to 62%). Allelic deletion spanning the CDKN locus was observed at significantly increased rates moving from early (7%) to advanced (28%) primary melanomas and to secondary melanoma lesions (37%) (P=0.018). Also, inactivation of the p16 gene (CDKN2A) was absent in naevi and present at steadily increasing rates moving from primary melanomas (7% early lesions to 17% advanced lesions) to melanoma metastases (62%) (P=0.004). CONCLUSIONS: Our findings indicate that, in a model of sequential accumulation of genetic alterations, 9p21 deletions may play a role in melanocytic transformation and tumour initiation whereas rearrangements at the CDKN locus, and p16 gene (CDKN2A) inactivation may contribute to tumour progression.

Nemaline rod myopathy: a rare form of myopathy.

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Fibrous dysplasia localized to spine: a diagnostic dilemma.

Fibrous dysplasia of the spine is uncommon, especially in monostotic form. Isolated vertebral involvement in polyostotic form is very rare. We report a case of polyostotic fibrous dysplasia with lesions localized to dorso-lumbar spine in a 45-year-old rheumatoid arthritis patient. No associated appendicular lesions, cutaneous manifestations or endocrinopathies were seen. The extreme rarity of this type of lesion can pose a diagnostic dilemma, and biopsy is required for diagnosis. The association with rheumatoid arthritis in our case seems to be a chance occurrence.

Multicentric Castleman's disease in two cases.

Castleman's disease is an atypical lymphoproliferative disorder having two types of presentation--the localized and the multicentric form. The localized form presents as a slowly growing mass with a relatively benign course. Multicentric Castleman's disease has a more aggressive clinical course with diffuse lymph node enlargement and systemic illness. It is rarely seen in childhood and only thirteen cases have been reported in literature. This is the first report of 2 cases from the Indian subcontinent with a maximum follow-up of 44 months one of whom had asplenia.

O6-(4-bromothenyl)guanine (PaTrin-2), a novel inhibitor of O6-alkylguanine DNA alkyl-transferase, increases the inhibitory activity of temozolomide against human acute leukaemia cells in vitro.

Anti-tumour activity of triazene compounds of clinical interest [i.e. dacarbazine and temozolomide (TMZ)] relies mainly on the generation of methyl adducts to purine bases of DNA. Two DNA repair enzyme systems, i.e. the O6-guanine-alkyl-transferase (MGMT) and mismatch repair (MMR), play a predominant role in conditioning the cytotoxic effects of triazenes. In particular, high levels of MGMT associated with target cells are responsible of resistance to triazenes. On the contrary, the presence of MMR is required for the cytotoxic effects of these compounds. Previous studies performed by our group and a more recent clinical investigation reported by Karen Seiter, pointed out that triazene compounds could play an important role in the treatment of refractory acute leukaemia. Leukaemia blasts, especially of lymphoblastic leukaemia, show frequently high levels of MGMT activity. Therefore, it reasonable to hypothesize that combined treatment of leukaemia patients with triazene compounds along with MGMT inhibitors could lead to a better control of the disease. PaTrin-2 (O6-(4-bromothenyl)guanine, PAT) is a potent and scarcely toxic MGMT inhibitor recently introduced in clinical trials. This drug is used in combination with triazene compounds in order to augment their anti-tumour efficacy against neoplastic cells endowed with high MGMT activity. The present report describes, for the first time, pre-clinical in vitro studies on the cytotoxic activity of combined treatment with PAT+TMZ against long-term cultured leukaemia cells and primary leukaemia blasts obtained from patients with acute lymphoblastic leukaemia or acute myeloblastic leukaemia. The results point out that, both in long-term cultured leukaemia cell lines and in primary blast samples, PAT could improve dramatically the sensitivity of malignant cells to the cytotoxic effects of TMZ. This sensitizing effect is detectable when leukaemia cells show resistance mechanisms based on a MGMT-proficient phenotype. On the contrary, when resistance to TMZ is dependent on MMR deficiency, no influence of PAT can be detected in various experimental conditions. In conclusion, these results appear to provide disease-oriented rational basis to design novel clinical protocols for the treatment of acute leukaemia with combined administration of PAT and triazene compounds.

Preclinical studies on detection of circulating melanoma cells in patients: telomerase as a recognition marker of malignancy.

Preclinical studies based on a "simulation design", were performed with cultured melanoma cells prelabeled with 51Cr, added to normal blood and subjected to separation and recognition steps. Mononuclear cells (MNC) were isolated on ficollhypaque gradient, and melanoma cells were separated from lymphocytes using anti-CD45 immunomagnetic beads. Malignant cells were then recognized by measuring telomerase activity (TRAP and TRAP-ELISA assays). It was found that: (a)recovery of prelabeled cells present in MNC did not exceed 75%; (b) further recovery of prelabeled cells after separation from lymphocytes did not exceed 68%. Therefore, the overall recovery of prelabeled cells did not exceed 48%; (c) the entire procedure was able to reliably detect as few as 30 malignant cells added to normal blood, providing a telomerase signal significantly higher than that found in absence of melanoma cells. These results furnish the technical bases for developing a tumor detection assay in the blood of melanoma patients.