Unusual presentation of acquired thrombotic thrombocytopenic purpura (TTP) versus catastrophic antiphospholipid syndrome in a patient with Moya-Moya disease, case report, and literature review.

Coincidences in medicine are not so common. We are presenting a case of a patient diagnosed with Moya-Moya disease and antiphospholipid syndrome (APS) who presented with clinical and laboratory characteristics of catastrophic APS versus TTP. The diagnosis was a challenge because characteristics were overlapping. Nevertheless, a decision to treat the patient for TTP was made with afterward improvement. MMD has been associated with multiple immune disorders; however, only one case of acquired thrombotic thrombocytopenic purpura has been documented in association with this disease. None has been associated with catastrophic antiphospholipid syndrome. We are presenting a challenging case where all these three medical conditions were present at the same time.

Prognostic modeling in biliary diseases.

PURPOSE OF REVIEW: To discuss the prognostic models for the cholestatic diseases focusing on primary sclerosing cholangitis and primary biliary cholangitis. RECENT FINDINGS: Noninvasive prognostic models that outperform alkaline phosphatase and Mayo Risk Score have been developed to predict clinically significant events, such as transplant free survival or hepatic decompensation. Models for primary sclerosing cholangitis (PSC) include UK-PSC, Primary Sclerosing Cholangitis Risk Estimate Tool, and Amsterdam Oxford models. Models for primary biliary cirrhosis (PBC) include UK-PBC, Global primary biliary cholangitis group score (GLOBE) and Paris II scores. Other models have incorporated elastography with or without findings on magnetic resonance imaging. SUMMARY: Noninvasive prognostic models can inform patients about their risk for clinical outcomes and serve as surrogate intermediate outcomes to determine efficacy of novel agents in clinical trials.

Gastrointestinal involvement in systemic sclerosis: An updated review.

The gastrointestinal tract (GI) is the second most affected organ system in individuals suffering from systemic/localized scleroderma (SSc) or localized scleroderma. SSc can affect any part of the GI, between the oral cavity and anorectum. The annual incidence of SSc in the United States is estimated to be 19.3 cases per million adults, with the highest incidence reported in people aged 44 to 55. Females are 5 times more likely than males to suffer from SSc. Morbidity and mortality rates associated with SSc are predominantly elevated among patients with GI manifestations. Esophageal and intestinal manifestations impact 90% and 40% to 70% of patients with systemic scleroderma, respectively. SSc patients are known to suffer from small bowel hypomotility and small intestinal bacterial overgrowth, which cause malabsorption and malnutrition, ultimately contributing to the 50% mortality rate. Fecal incontinence is a common symptom of SSc that can lead to depression. SSc patients may suffer from gastrointestinal complications that can negatively impact their quality of life on a daily basis. Multidisciplinary approaches are necessary for systematically managing gastrointestinal complications associated with SSc. A prospective study should focus on developing targeted therapies to improve recovery patterns and prognosis in cases of SSc. This article summarizes the epidemiology, commonly reported clinical manifestations, complications, and available treatments for treating GI pathology in SSc patients.

Biliary Anastomosis Using T-tube Versus No T-tube for Liver Transplantation in Adults: A Review of Literature.

The T-tube-directed biliary anastomosis in orthotopic liver transplantation (OLT) aims to minimize preventable biliary complications, including bile leaks and strictures. Biliary complications in patients with OLT increase the risk of morbidity and mortality. This review paper evaluated the current evidence on the routine use of T-tube reconstruction in OLT cases. A review of prospective, retrospective, observational, cohort studies as well as systematic reviews, meta-analyses, review papers, and opinion papers has been conducted to evaluate the therapeutic potential of T tube-based biliary anastomosis in cases of OLT. Our finding showed a bile leak incidence of 16.6% and 6.6% in T-tube and non-T-tube groups, respectively. The results indicated a lower incidence of anastomotic fistulae in the non-T-tube group (0.6%) compared to the T-tube group (4%). The findings negated statistically significant differences in the three-year actuarial survival rates based on biliary anastomosis with and without T-tube intervention (62.5% vs. 69.8%). The studies revealed a 6-11% and 2-11% incidence of cholangitis in OLT patients with T-tube-based reconstruction and those without a T-tube, respectively, and 26% and 20% incidence of total biliary complications in OLT patients with and without T-tube, respectively. In addition, the findings ruled out the influence of a T-tube on the incidence of perioperative complications, endoscopies, and reoperations in OLT cases. The current evidence correlates the increased incidence of bile leaks, cholangitis, and overall biliary complications with the use of a T-tube during OLT. In addition, T-tube-guided reconstruction has no impact on perioperative complications, overall survival, endoscopies, and reoperations in OLT cases.

Outcomes of heart transplantation in cardiac amyloidosis: an updated systematic review.

Cardiac amyloidosis is one of the most common infiltrative cardiomyopathies that is characterized by the extracellular deposition of misfolded fibrillar protein. Several studies have previously found that patients with amyloid in the past have performed poorly after heart transplantation. Recent advancements in treatments have been made that have significantly improved outcomes in these patients. The study aimed to evaluate the outcomes of heart transplantation in cardiac amyloidosis. We systematically searched EMBASE, PubMed/MEDLINE, and Cochrane Library databases on 30 December 2021 following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. We identified 22 studies that examined 42,951 patients with cardiac amyloidosis of which only 1,329 patients underwent isolated heart transplantation. Seven studies reported individual patient data. The results of 123 patients have been pooled for analysis. There were 70 male patients, 45 female patients, and eight patients who did not report their gender. Among the types of amyloids, 63 (51%) patients were found to have light chain amyloidosis (AL) and 33 (27%) patients had transthyretin amyloidosis (ATTR). Only 41 patients (33.3%) reported a monoclonal component. There were 30 patients with AL that underwent autologous hematopoietic stem cell transplant (ASCT). The mean survival of 24 out of 30 patients was 4.33 years. In addition, the reported data include 13 patients requiring intra-aortic balloon pump (IABP), six with cardiac resynchronization therapy (CRT), and four with implantable cardioverter defibrillator (ICD). With the current advancements in treatments in combination with a multidisciplinary approach and careful patient selection, patients undergoing heart transplantation for amyloidosis may have encouraging results in the current era. Further studies will be needed to evaluate the outcomes of heart transplantation in amyloidosis patients now that several advances have been made in the field.

Hepatic sarcoidosis resembling primary sclerosing cholangitis.

We report the case of a 29-year-old man who presented with progressive weight loss, night sweats, abdominal pain and pruritus who was found to have obstructive jaundice and cholestatic pattern of liver injury on laboratory workup. Though findings on magnetic resonance cholangiopancreatography were initially concerning primary sclerosing cholangitis, he was ultimately diagnosed with biliary sarcoidosis after a liver biopsy. This case brings attention to the rare phenomenon of hepatic sarcoidosis causing hyperbilirubinemia and highlights the importance of reaching the correct diagnosis early, as the patient's symptoms improved after initiation of steroids.

Brown Tumor With Spine Involvement at Multiple Levels in a Hemodialysis Patient.

Brown tumor of the bone or osteitis fibrosa cystica is a rare manifestation of hyperparathyroidism, most seen nowadays in association with secondary and tertiary hyperparathyroidism. Chronic kidney disease (CKD) and end-stage renal disease (ESRD) are the major culprits of secondary hyperparathyroidism (sHPTH). CKD is known to cause phosphate retention and a decrease in 1,25-dihydroxyvitamin D and ionized calcium levels, which in turn trigger the PTH secretion. Brown tumor can affect the jawbones, femur, sternum, ribs, and rarely the spine. We present the case of a 60-year-old male with ESRD on hemodialysis who was found to have lytic bone lesions in the thoracic and lumbar spine. Initially, malignancy was suspected. Blood work revealed markedly elevated PTH at 3,563 pg/mL, hypocalcemia, and hyperphosphatemia. Biopsy of the L5-S1 lesion was consistent with reactive changes due to sHPTH. Once a diagnosis of the brown tumor was confirmed, the patient was started on cinacalcet and was referred for parathyroidectomy.

Phlegmasia Cerulea Dolens: A New Perspective on Management.

This is a case of phlegmasia cerulea dolens (PCD) with unexpected but complete resolution of symptoms with short-term administration of heparin products, despite falling into category IIb according to the Rutherford limb ischemia scale, which regularly requires aggressive surgical intervention. We present a case of a 58-year-old Hispanic female with a past medical history of gastritis who arrived at the emergency room (ER) with acute onset severe pain on the left leg associated with discoloration of the leg. The patient was quickly diagnosed with PCD affecting the left lower extremity, which quickly resolved after administration of heparin infusion for one hour, despite the degree of limb ischemia. There is no consensus for the treatment of this condition. The most interesting feature of this case is the prompt resolution of symptoms with short-term administration of anticoagulation with total resolution without the need for thrombolysis or thrombectomy. This may suggest that prompt pharmacologic treatment in patients eligible for anticoagulation may successfully restore venous flow negating the need for further intervention.

Heroin-Induced Acute Pancreatitis.

Heroin-induced pancreatitis (HIP) is rare with only a few cases reported previously in the literature and the pathophysiology mechanism is yet to be investigated. We present two cases of acute pancreatitis (AP) in the setting of acute heroin (diacetylmorphine) intoxication. Both patients presented with nausea, vomiting and severe abdominal pain after intranasal heroin use. On laboratory analysis were found to have elevated serum lipase, positive urine toxicology for opioids, without any other obvious causes for AP. Both patients had a full recovery with supportive treatment. As a general approach, drug-induced pancreatitis is a diagnosis of exclusion and a high index of suspicion is required when the most common etiologies are ruled out.

A Case of Primary Mediastinal Seminoma Associated With Testicular Microlithiasis and Liver Injury.

Mediastinal seminomas are rare neoplasms that can be found incidentally in asymptomatic patients. However, a few cases may present in the emergency room with mild to severe respiratory and/or cardiovascular symptoms. This can occur when the tumor grows large enough to cause compression and obstruction of the various structures present in the thorax. Here we present a case of a large medium mediastinal seminoma that grew to the extent of causing pulmonary artery compression which led to chronic passive backflow through the hepatic veins and hepatic congestion. This case was remarkable as well for the presence of testicular microlithiasis, a rare feature with unknown significance to date.

Osseointegration of zirconium dental implants three months after insertion in rabbit femur. Histopathological study.

Implantology is a highly researched field with a constant concern in finding and studying new implant materials. Lately, zirconium has become a very attractive alternative to the detriment of titanium, but the research results were sometimes contradictory. Thus, we considered as opportune to study the osseointegration of zirconium dental implants in rabbit femur, three months after insertion. The biological material was represented by five rabbits and the experimental protocol was approved by the Ethics Committee of the University of Oradea, Romania. The implants (zirconium ceramic dental implants: 5 mm length, 2.6 mm diameter) were inserted in the femur under controlled conditions, after creating a bone defect. The animals received the appropriate postoperative care. Three months later, the implantation area was harvested and processed for histological examination. The assessment of the osseointegration process of the zirconium implants showed that they were very well tolerated by the host organism that did not trigger any rejection processes. Approximately 80% of the compromised bone was replaced with newly formed bone in advanced stages of remodeling and consolidation. The proliferated bone near the implants acquired a structure similar to the rabbit diaphyseal bone, but with higher density and size of the osteons. The stage reached by the osseointegration process three months after the insertion of the implants, ensures a good consolidation of the implants that supports the prosthetic structures, which are to be built on them.

Acinic cell carcinoma of minor salivary glands - case report.

Salivary gland tumors have a high incidence (90%) within the parotid gland. The acinic cell carcinoma (ACC) represents only 1-3% of the salivary gland tumors, most frequently affecting the parotid. The minor salivary glands rarely develop ACC and when the ACC is localized in these glands, generally, it has a less aggressive evolution. The main criterion in the diagnostic of ACC is the histological examination with the regular Hematoxylin-Eosin (HE) staining and additionally Periodic Acid-Schiff (PAS) special staining. Immunohistochemical (IHC) examination confirms the origin of the tumor cells and the cellular proliferation index. Therapeutic management consists of surgical excision and radiotherapy. Left jugular tumor was the clinical diagnosis in the case we presented. The computed tomography (CT) examination revealed a voluminous expansive process of the left cheek. Surgery was performed with tumor resection and the skin defect was threatened. The histopathological (HP) and IHC exams have established the diagnosis of minor salivary glands adenocarcinoma with increased tumor proliferation index. The evolution was unfavorable to tumor recurrence in a short time of one year.