A Case of Catastrophic Antiphospholipid Syndrome with Acute Multiorgan Thrombosis and Concerns for Re-Emergence.

BACKGROUND Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome characterized by widespread thrombotic complications leading to multiorgan ischemia and failure. Although there are no standard treatment guidelines for CAPS, it often involves triple therapy with anticoagulation, corticosteroids, and plasma exchange. Recently, biologics such as rituximab and eculizumab have also shown promise as potential new therapies for CAPS, as observed in our case. CASE REPORT We describe a 59-year-old female patient who presented with altered mental status and diffuse weakness. Imaging studies revealed multiorgan thrombosis along with thrombocytopenia that markedly improved with plasma exchange therapy, steroids, and a heparin drip. While the exact etiology of CAPS remained unknown, it was likely precipitated by her warfarin discontinuation and confirmed Haemophilus influenzae infection. The patient's hospital course was complicated by hemorrhagic shock after a renal biopsy, followed by an acute drop in thrombocytopenia and new embolic infarcts in the brain that raised concern for CAPS re-emergence. To address the refractory nature of her condition, the patient underwent a trial of rituximab, which remarkably improved her clinical picture and platelet count by an 8-fold increase within 1 week. CONCLUSIONS This case highlights the importance of early recognition and diagnosis of catastrophic antiphospholipid syndrome, a true rheumatological emergency that requires aggressive treatment to prevent irreversible complications. Our patient's presentation and response to treatment also underscores the complexity of managing CAPS and the use of newer biological therapies in refractory cases.

Co-Presentation of Lupus Nephritis with Autoimmune Hepatitis.

BACKGROUND Systemic lupus erythematosus (SLE) is a multiorgan immunologic disease which commonly results in systemic manifestations by involving joints, kidneys, skin, heart, hematologic cell lines, pulmonary and central nervous systems. The hepatic involvement of lupus is relatively less common, which creates diagnostic challenges, as the clinical presentations of lupus hepatitis and autoimmune hepatitis (AIH) are similar. CASE REPORT A 51-year-old woman presented for multiple joint pain that began 2 years ago. Her work-up, including kidney biopsy, was consistent with a diagnosis of class V lupus nephritis. Subsequently, within a few months, she was admitted with acute elevation of liver enzymes and high immunoglobulin IgG level, and a liver biopsy demonstrated impressive interface hepatitis with many plasma cells and lymphocytes, suggestive of chronic hepatitis with high histological activity. This case illustrates the co-presentation of lupus nephritis and AIH, which is a rare association. The patient was managed with a tapering dose of prednisone, hydroxychloroquine initially, and later with mycophenolate mofetil, with complete resolution of liver enzyme abnormalities by 4-month follow-up. CONCLUSIONS Lupus hepatitis is hepatic involvement of SLE, which should be distinguished from AIH. Accurate diagnosis is important, as management and prognosis of these immunologic conditions can differ. Although both entities share clinical and biochemical markers, the presence of anti-ribosomal P antibodies and liver histology features of predominant lymphoid infiltrates with lobular inflammation favor lupus hepatitis. A multidisciplinary approach involving rheumatologists, hepatologists, and pathologists can improve disease outcomes by properly differentiating the 2 entities and guiding the selection of appropriate immunosuppressive therapy.

Cerebrospinal fluid liver pseudocyst: A bizarre long-term complication of ventriculoperitoneal shunt: A case report.

BACKGROUND: Ventriculoperitoneal (VP) shunt placement has become a standard of care procedure in managing hydrocephalus for drainage and absorption of cerebrospinal fluid (CSF) into the peritoneum. Abdominal pseudocysts containing CSF are the common long-term complication of this frequently performed procedure, mainly because VP shunts have significantly prolonged survival. Of these, liver CSF pseudocysts are rare entities that may cause shunt dysfunction, affect normal organ function, and therefore pose therapeutic challenges. CASE SUMMARY: A 49-year-old man with history of congenital hydrocephalus status post bilateral VP shunt placement presented with progressively worsening dyspnea on exertion, abdominal discomfort/distention. Abdominal computed tomography (CT) scan revealed a large CSF pseudocyst in the right hepatic lobe with the tip of VP shunt catheter into the hepatic cyst cavity. Patient underwent robotic laparoscopic cyst fenestration with a partial hepatectomy, and repositioning of VP shunt catheter to the right lower quadrant of the abdomen. Follow-up CT demonstrated a significant reduction in hepatic CSF pseudocyst. CONCLUSION: A high index of clinical suspicion is required for early detection of liver CSF pseudocysts since their presentation is often asymptomatic and cunning early in the course. Late-stage liver CSF pseudocysts could have adverse outcomes on the treatment course of hydrocephalus as well as on hepatobiliary dysfunction. There is paucity of data to define the management of liver CSF pseudocyst in current guidelines due to rare nature of this entity. The reported occurrences have been managed by laparotomy with debridement, paracentesis, radiological imaging guided fluid aspiration and laparoscopic-associated cyst fenestration. Robotic surgery is an additional minimally invasive option in the management of hepatic CSF pseudocyst; however, its use is limited by lack of widespread availability and cost of surgery.

Candida Bezoar of the Bladder Resulting in Obstructive Uropathy: A Case Report.

Candida bezoar is a rare pathologic entity characterized by the colonization of a cavity by an aggregate or mass of mycelia due to local or systemic infections with Candida spp. Candida bezoar is commonly seen in immunocompromised individuals and can often present in the context of symptomatic urinary tract infection or urosepsis. The implicated risk factors for the development of Candida bezoars are anatomical urinary tract abnormalities, diabetes mellitus, indwelling urinary catheters, increased use of broad-spectrum antibiotics, and corticosteroids. Early clinical suspicion is essential for diagnosis to prevent the dissemination of disease and for a favorable prognosis. We report a case of a 49-year-old diabetic male who presents with hematuria, abnormal urinary flow, and left-sided flank pain for four days caused by a Candida bezoar of the urinary bladder resulting in unilateral obstructive uropathy despite the appropriate placement of a ureteral stent. Treatment with left nephrostomy tube, oral fluconazole, and amphotericin bladder irrigation for three days was successful. The patient's condition improved, and he was discharged on fluconazole and was recommended to follow up with urology as an outpatient.

Comparison of mean pain score of oral non-steroidal anti-inflammatory agents and locally injectable steroid for the treatment of plantar fasciitis.

OBJECTIVE: To compare the efficacy of oral non-steroidal anti-inflammatory drugs with locally injectable steroids in the treatment of plantar fasciitis. METHODS: This comparative study was conducted from 10 May 2013 to 10 December 2013 at the Department of Orthopaedic Surgery, Punjab Medical College/Allied Hospital, Faisalabad, Pakistan, and comprised patients of either gender aged 26-60 years having unilateral plantar fasciitis who were not on any prior treatment and had moderate to severe pain intensity. The patients who received diclofenac sodium 50mg and acetaminophen 500mg twice a day for 4 weeks were assigned as Group-A, and those who received a single of 40mg (1ml) of methylprednisolone combined with 2ml of 0.5% bupivacaine into the tender most point of the inflamed plantar fascia were assigned as group - B. Pain was assessed using visual analogue scale at baseline and after 2 months of regular follow-up. Data was analysed using SPSS 10. RESULTS: Of the 140 patients, there were 70(50%) in each of the two groups. There were 102(72.9%) males and 38(27.1%) females, with an overall mean age of 42.24±9.30 years. While pain intensity decreased in both the groups, it was significant in group B (p=0.0001), but non-significant in group A (p=0.723). CONCLUSIONS: Locally injectable steroid was found to be a better modality for the management of plantar fasciitis in reducing mean pain score and sustained pain relief compared to non-steroidal anti-inflammatory drugs.

Erratum to "Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage".

[This corrects the article DOI: 10.1155/2021/5555961.].

Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage.

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucosal surfaces characterized by acantholysis (loss of adhesion between epidermal cells). Esophageal involvement of PV is an underdiagnosed entity as routine diagnostic endoscopy is not recommended in asymptomatic patients. Dysphagia and odynophagia are common presenting symptoms; however, upper gastrointestinal bleeding (UGIB) associated with esophageal involvement of PV without a history of mucosal blistering is extremely uncommon. We present a case of esophageal involvement of PV associated with active UGIB that was diagnosed on endoscopic evaluation. This case illustrated the importance of early endoscopy to identify the esophageal involvement of PV especially in patients with preexisting disease who present with gastrointestinal symptoms such as dysphagia, odynophagia, and hematemesis. Early recognition of esophageal involvement of PV and initiation of corticosteroid and/or immunosuppressant therapy may improve the outcome of the disease.

Syphilitic Aortic Aneurysm: A Rare Entity in the Era of Antibiotics.

A thoracic aortic aneurysm is a rare entity of tertiary syphilis in the era of antibiotics. The diagnosis of the aortic aneurysm due to tertiary syphilis may be challenging due to deceptive clinical presentation and rarity of the disease in the western world. We report the case of a 59-year-old man, who presents with worsening shortness of breath and was found to have a large ascending aortic aneurysm on computed tomography angiogram (CTA) of the chest. Further workup demonstrated a positive syphilis test. Untreated earlier stages of syphilis attribute to the development of the ascending aortic aneurysm. The patient was medically treated with IV penicillin and underwent surgical repair of the aortic aneurysm. Histopathology confirmed the diagnosis of syphilitic aortitis. Tertiary syphilis often presents several years after initial infection and usually after a latent phase, making it difficult to diagnose. Syphilitic aortic aneurysms may result in a high mortality rate in untreated patients. Therefore, a high index of suspicion is required for the early recognition of a syphilitic aortic aneurysm. Early treatment with antibiotic therapy and surgical repair of syphilitic aortic aneurysms can prevent life-threatening complications.

Endoscopic retrograde cholangiopancreatography guided interventions in the management of pancreatic cancer.

Pancreatic cancer is the leading cause of cancer-related morbidity and mortality with an overall five-year survival of less than 9% in the United States. At presentation, the majority of patients have painless jaundice, pruritis, and malaise, a triad that develops secondary to obstruction, which often occurs late in the course of the disease process. The technical advancements in radiological imaging and endoscopic interventions have played a crucial role in the diagnosis, staging, and management of patients with pancreatic cancer. Endoscopic retrograde cholangiopancreatography (ERCP)-guided diagnosis (with brush cytology, serial pancreatic juice aspiration cytologic examination technique, or biliary biopsy) and therapeutic interventions such as pancreatobiliary decompression, intraductal and relief of gastric outlet obstruction play a pivotal role in the management of advanced pancreatic cancer and are increasingly used due to improved morbidity and complication rates compared to surgical management. In this review, we highlight various ERCP-guided diagnostic and therapeutic interventions for the management of pancreatic cancer.

Gastric Mucormycosis: An Infection of Fungal Invasion into the Gastric Mucosa in Immunocompromised Patients.

Primary gastric mucormycosis is a rare but potentially lethal fungal infection due to the invasion of Mucorales into the gastric mucosa. It may result in high mortality due to increased risk of complications in immunocompromised patients. Common predisposing risk factors to develop gastric mucormycosis are prolonged uncontrolled diabetes mellitus with or without diabetic ketoacidosis (DKA), solid organ or stem cell transplantation, underlying hematologic malignancy, and major trauma. Abdominal pain, hematemesis, and melena are common presenting symptoms. The diagnosis of gastric mucormycosis can be overlooked due to the rarity of the disease. A high index of suspicion is required for early diagnosis and management of the disease, particularly in immunocompromised patients. Radiological imaging findings are nonspecific to establish the diagnosis, and gastric biopsy is essential for histological confirmation of mucormycosis. Prompt treatment with antifungal therapy is the mainstay of treatment with surgical resection reserved in cases of extensive disease burden or clinical deterioration. We presented a case of acute gastric mucormycosis involving the body of stomach in a patient with poorly controlled diabetes and chronic renal disease, admitted with acute onset of abdominal pain. Complete resolution of lesion was noted with 16 weeks of medical treatment with intravenous amphotericin B and posaconazole.

Association of splenic artery pseudoaneurysm with recurrent pancreatitis.

A pseudoaneurysm of the splenic artery (SAP) is a rare entity which is associated with pancreatitis in 52% of cases. In the presence of pancreatitis, the enzymatic damage to the wall of splenic artery results in pseudoaneurysm formation. The clinical presentation is variable and ranges from asymptomatic to hemodynamic instability. The diagnosis of SAP is challenging in the presence of peripancreatic fluid collection or pseudocyst, where CT abdomen can miss small pseudoaneurysms. Angiography is a useful modality to establish a definitive diagnosis. We present a 49-year-old male with a history of recurrent pancreatitis due to alcoholism who presented with acute abdominal pain and was found to have acute pancreatitis. Abdominal CT scan showed a peripancreatic fluid collection and hyperdense lesion at the splenic hilum, which was diagnosed as SAP on angiography. A transcatheter embolization was performed with complete resolution of symptoms thereafter.

Black esophagus: a syndrome of acute esophageal necrosis associated with active alcohol drinking.

Black esophagus, also known as acute esophageal necrosis (AEN) syndrome, is a rare entity characterized by patchy or diffuse circumferential black pigmentation of the esophageal mucosa from ischemic necrosis. It may present with life-threatening upper gastrointestinal hemorrhage resulting in high mortality in immunocompromised patients. Advanced age with multiple comorbidities compounded with compromised hemodynamic states are poor prognostic factors. Findings on laboratory work-up and radiological imaging are non-specific. After initial resuscitation, endoscopic evaluation and histological examination of esophageal biopsy are diagnostic. Early recognition and aggressive resuscitation are the fundamental principles for the management of AEN and better outcome of the disease. We report a case of a 56-year-old woman with diabetes mellitus, gastro-esophageal reflux disease, and active alcohol binging who presented with hematemesis and acute epigastric pain due to AEN. This case illustrates a rare etiology of AEN due to active alcohol drinking, which may be overlooked. Physician awareness about this etiology is important as early recognition and timely management may improve survival.

Glycogenic Hepatopathy: A Reversible Complication of Uncontrolled Diabetes Mellitus.

Glycogenic hepatopathy (GH) is a rare complication of long-standing uncontrolled type I diabetes mellitus (TIDM) resulting in liver dysfunction and hepatomegaly due to intrahepatic deposition of glycogen. Herein we present a 19-year-old male with a history of TIDM and multiple prior hospitalizations with diabetic ketoacidosis (DKA) who presented with nausea, vomiting, right upper quadrant pain, and massive hepatomegaly. Laboratory workup was consistent with DKA and revealed a greater than 10-fold increase in liver enzymes. Despite the resolution of DKA, his liver function was worsening, and further workup was indicated. Ultimately, he underwent a liver biopsy that showed swollen hepatocytes overloaded with intracytoplasmic glycogen consistent with glycogenic hepatopathy. It is an underestimated entity and physicians should have a high index of suspicion for GH in individuals presenting with liver dysfunction, hepatomegaly, and poor glycemic control in TIDM. Strict glycemic control may result in complete resolution of disease.

Biloma: A Rare Manifestation of Spontaneous Bile Leak.

A biloma is an intrahepatic or extrahepatic encapsulated collection of bile outside of the biliary tree and within the abdominal cavity. Hepatobiliary interventions and laparoscopic cholecystectomy are the most common etiologies of biloma followed by abdominal trauma, choledocholithiasis, and biliary dilation secondary to biliary stricture. We report a case of a 91-year-old female who presented to the emergency room with an acute onset of epigastric and right upper quadrant sharp pain for one day that radiated to the back, and was associated with two to three episodes of vomiting. Initial abdominal imaging including CT scan, ultrasound and magnetic resonance cholangiopancreatography (MRCP) of the abdomen and pelvis revealed a distended gallbladder with wall thickening, but without evidence of pancreatitis or gallstones. Hepatobiliary iminodiacetic acid (HIDA) scan findings were consistent with extrahepatic biliary leakage into the peritoneum. A cholangiogram demonstrated a perihepatic biloma. A combined approach using fluoroscopic-guided biloma drainage and endoscopic retrograde cholangiopancreatography (ERCP)-guided biliary stent placement across the site of the biliary leak resulted in the complete resolution of symptoms. Biloma should be included in the differential diagnosis of right upper quadrant abdominal pain. A high index of clinical suspicion is required for early diagnosis and treatment.

Endoscopic ultrasound (EUS) and the management of pancreatic cancer.

Pancreatic cancer is one of the leading causes of cancer-related mortality in western countries. Early diagnosis of pancreatic cancers plays a key role in the management by identification of patients who are surgical candidates. The advancement in the radiological imaging and interventional endoscopy (including endoscopic ultrasound (EUS), endoscopic retrograde cholangiopancreatography and endoscopic enteral stenting techniques) has a significant impact in the diagnostic evaluation, staging and treatment of pancreatic cancer. The multidisciplinary involvement of radiology, gastroenterology, medical oncology and surgical oncology is central to the management of patients with pancreatic cancers. This review aims to highlight the diagnostic and therapeutic role of EUS in the management of patients with pancreatic malignancy, especially pancreatic ductal adenocarcinoma.

Role of Radiofrequency Ablation in the Management of Unresectable Pancreatic Cancer.

Pancreatic cancer is one of the most aggressive malignancies of the digestive tract and carries a poor prognosis. The majority of patients have advanced disease at the time of diagnosis. Surgical resection offers the only curative treatment, but only a small proportion of patients can undergo surgical resection. Radiofrequency ablation (RFA) is a well-known modality in the management of solid organ tumors, however, its utility in the management of pancreatic cancer is under investigation. Since the past decade, there is increasing use of RFA as it provides a feasible palliation treatment in the management of unresectable pancreatic cancer. RFA causes tumor cytoreduction through multiple mechanisms such as coagulative necrosis, protein denaturation, and activation of anticancer immunity. The safety profile of RFA is controversial because of the high risk for complications, however, small prospective and retrospective studies have shown promising results in its applicability for palliative management of unresectable pancreatic malignancies. In this review, we discuss different approaches of RFA, their indications, technical accessibility, safety, and major complications in the management of unresectable pancreatic cancer.

The Pathophysiology of Gastrointestinal and Hepatic Manifestations of COVID-19.

Coronavirus disease 2019 (COVID-19), also known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is the sixth international public health emergency. While COVID-19 classically manifests as a respiratory illness, SARS-CoV-2 may infect multiple organ systems and cause a wide array of presentations. The gastrointestinal tract has become increasingly recognized as a site of SARS-CoV-2 infection with reports of diarrhea, nausea, and liver failure, with or without concomitant respiratory involvement. In this case series and literature review, we report three cases of SARS-CoV-2 infected patients that presented with predominantly gastrointestinal symptoms or laboratory abnormalities such as diarrhea, anorexia, and transaminitis. The receptor for SARS-CoV-2, angiotensin-converting enzyme 2 (ACE2), as well as the necessary protease to facilitate viral entry, transmembrane protease serine-2 (TMPRSS2), and to a lesser extent, cathepsins, have been demonstrated to be present throughout the gastrointestinal tract, thus facilitating viral entry and pathogenesis. Furthermore, multiple reports have demonstrated evidence of viral shedding outside the nasopharynx, including the stool, for prolonged time periods even in the absence of detection of viral RNA in the nasopharynx. As such, testing for SARS-CoV-2 in stool samples with reverse transcription polymerase chain reaction (RT-PCR) assays for detection of viral RNA could aid in identifying patients that lack classic respiratory symptoms, present with atypical symptoms, or in those with a high index of suspicion (e.g. elevated inflammatory markers), but test negative on the classic nasopharyngeal swab. Furthermore, this underscores the potential for atypical transmission, with a focus on fecal-oral transmission and the need for strict hand hygiene.

Hepatotoxicity associated with Garcinia cambogia: A case report.

BACKGROUND: Herbal supplements (HS) for weight loss are perceived to be "safe" and "natural", as advertised in ads, however, hepatotoxicity can be associated with consumption of some HS. Use of HS may be missed, as the patient may not report these unless specifically asked about these products, since they are often not thought of as medications with potential side effects or interaction potential. CASE SUMMARY: We reported a case of a 21-year-old female with morbid obesity who presented with abdominal pain for 1 wk associated with nausea, vomiting, anorexia and myalgias. She denied smoking tobacco, drinking alcohol, usage of illicit drugs, hormonal contraceptives, or energy drinks. There was no significant past medical or family illnesses. Her laboratory workup revealed acute liver failure. The workup for possible etiologies of acute liver failure was unremarkable. She was using a weight loss herbal supplement "Garcinia cambogia" for 4 wks. This case demonstrates the association of acute liver failure with Garcinia cambogia. CONCLUSION: Medical reconciliation of HS should be performed in patients with suspected acute liver failure and early discontinuation of HS can prevent further progression of drug induced hepatoxicity.