ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review.

Leukocyte chemotactic factor-2 amyloidosis (ALECT2) is a recently described subtype of amyloidosis. IgG4-related disease is a rare fibroinflammatory condition characterized by dense interstitial lymphoplasmacytic infiltrates and fibrosis. Membranous nephropathy and diabetic nephropathy are common causes of nephrotic syndrome. Here we report a 49-year-old Hispanic male patient with diabetes mellitus who presented with jaundice and pruritus. IgG4-related autoimmune pancreatitis was diagnosed through laboratory workup and ampulla biopsy. He subsequently presented with marked lower extremity edema and nephrotic syndrome. Kidney biopsy showed severe interstitial IgG4-positive plasma cell-rich inflammatory infiltrates and interstitial storiform fibrosis. Immunofluorescence microscopy revealed diffuse and finely granular glomerular capillary wall staining for IgG and the glomeruli were negative for anti-phospholipase A2 receptor. Congo red stain was positive for birefringent deposits in the interstitium, arteriolar walls, and glomeruli. Electron microscopy demonstrated subepithelial immune complex-type electron-dense deposits, thickening of glomerular basement membranes (GBM), and randomly oriented fibrils in the mesangium, GBM, and interstitium. Mass spectrometry identified a peptide profile consistent with ALECT2 amyloidosis. This is the first report of a case with concurrence of ALECT2 amyloidosis, IgG4-related disease involving the kidney, membranous nephropathy, and early diabetic kidney injury.

Novel Presentation of Pulmonary Atypical Carcinoid Tumor as Acute Pancreatitis.

Pulmonary neuroendocrine tumors (NETs) are a group of rare tumors that pose a high financial burden on patients and the United States healthcare system. The usual presenting symptoms include cough or wheezing, hemoptysis, or chest pain. Due to bronchial obstruction, patients may also present with recurrent pneumonia. Acute pancreatitis has yet to be documented as the initial manifestation of this disease. Atypical carcinoids - a subtype of NETs - are heterogeneous regarding their site of origin, biological behavior, and malignant potential. Studies show that the most common primary tumor site varies by race, with the lung being the most common in white patients and the rectum being the most common in Asian/Pacific Islander, American Indian/Alaskan Native, and African American patients. Certain carcinoid tumors, such as those of the rectum, are over-represented among the Black and Asian populations within the United States, suggesting the role of genetics in the development of this intriguing disease. Furthermore, the pancreas is not a usual site of metastasis for primary lung NET. Our case study describes the rare occurrence of a primary pulmonary NET (atypical carcinoid) metastasizing to the pancreas and presenting as acute pancreatitis.

A Rare Case Study About Necrotizing Granulomatous Sarcoidosis.

Sarcoidosis is a granulomatous disorder with an elusive etiology and pathogenesis. Classically, sarcoidosis is associated with non-caseating granulomas composed of mononuclear phagocytes, lymphocytes, and multinucleated giant cells. Necrotizing granulomas can also be associated with sarcoidosis but is scarcely reported in the medical literature. Necrotizing sarcoid granulomatosis is challenging to diagnose due to its rarity and similarity with other necrotizing disorders. Therefore, it is mainly considered a diagnosis of exclusion. We report one such case study, which could prompt further research to lay the course of treatment strategies for this disease. Moreover, our patient had a family history of sarcoidosis, which raises questions regarding possible genetic predisposition, and future work might help solve this medical mystery.

Vaping-Induced Lung Injury: An Uncharted Territory.

Vaping-associated lung injury (VALI) presents with symptoms ranging from lower respiratory tract involvement (shortness of breath, fever, and cough) to gastrointestinal involvement (vomiting and diarrhea). Based on the longitudinal analysis, VALI is associated with increased risk for respiratory disease, making it paramount for medical professionals to understand this disease process and be familiar with its varied presentations. Our case study is a presentation of two relatively young patients with VALI, with a varied clinical course and distinct levels of severity. VALI still remains uncharted territory. Case reports, such as ours, have the potential to invoke randomized controlled clinical trials to better understand the disease etiology, pathology, and management.

Delayed Recurrence of Chromophobe Renal Cell Carcinoma Presenting as Metastatic Duodenal Ulcer.

Renal cancers are one of the common causes of cancer-related morbidity and mortality worldwide. Most primary cases are localized at presentation and are treated with partial or radical nephrectomy with curative intent. However, renal cell carcinoma (RCC) is known for its potential recurrence, sometimes several years after initial management. Many of these recurrent cases commonly metastasize to the liver, kidney, or bone and herald a poor prognosis. We present a case study of nonclear cell RCC, which recurred 33 years after nephrectomy and masqueraded as a duodenal ulcer -- an extremely rare site for metastasis. This is unique as it describes a presentation only sparingly documented in the medical literature and highlights a more extended period of recurrence than currently reported. Moreover, our patient's tumor was chromophobe cell variety, a rare sub-type of nonclear cell RCC, which to our knowledge has never been known to cause duodenal metastasis. Studies have implicated a prognostic role of lymph node involvement at the time of initial diagnosis to predict future recurrence. This case is a drop in the mighty ocean to prompt further investigation on the utility of life-long surveillance protocols and further research evaluating the role of lymph node dissection in preventing such recurrences and high mortality.

Exogenous Lipoid Pneumonia Complicated by Mineral Oil Aspiration in a Patient With Chronic Constipation: A Case Report and Review.

Exogenous lipoid pneumonia is a rare and frequently misdiagnosed lung disease. It occurs as an inflammatory reaction secondary to either aspiration or inhalation of lipids. Our patient had a history significant for recurrent pneumonia and the use of mineral oil for chronic constipation. A chest computed tomography showed multifocal consolidative opacities with areas of low attenuation, highly suspicious of exogenous lipid pneumonia. The diagnosis was confirmed with combined bronchoalveolar lavage and transbronchial lung biopsy that showed lipid-laden macrophages consistent with exogenous lipoid pneumonia. After thorough medication review, apart from mineral oil, no other contributing factors were found. A diagnosis of exogenous lipoid pneumonia associated with the use of mineral oil made and successfully managed by stopping the offending agent and supportive antibiotics.

Accidental Central Venous Catheter Placement in the Internal Thoracic Vein: A Case Report.

Central venous catheter (CVC) placement is an essential component of critical care medicine. CVC malposition is a known complication of internal jugular vein (IJV) cannulation. However, catheterization of the internal thoracic vein (ITV) is much rarer. Only a handful of case reports have been documented, and guidelines for management are therefore lacking. Our case study describes this rarely occurring ITV cannulation along with the discussion of risk factors, warning signs of malpositioning, and subsequent management plans to optimize patient safety. Previous studies have used fluoroscopy and agitated saline flush tests to confirm that agents administered through an ITV-located catheter would reach the right atrium. Considering this, it would follow that a catheter in this site could theoretically be used for medication administration, especially in emergency settings. This hypothesis remains the most novel part of our case study and might prompt further exploration of management strategies in this particular situation.

Calcifying Fibrous Tumor Complicated by Empyema.

Calcifying fibrous tumor (CFT) is a rare, benign proliferation of fibroblasts and inflammatory cells that is non-invasive and usually arises in deep tissue structures. Due to its overall paucity, no accurate incidence has been reported yet. We understand this disease via a handful of case studies published in the medical literature, first of them being from 1988. Earlier known as 'pseudotumor', it was recently given its name due to the potential of recurrence and multifocal involvement. We describe the case of a 43-year-old Hispanic male who presented with a large symptomatic pleural-based mass which turned out to be CFT and was later complicated by empyema. Our aim is to increase awareness about this rare disease and throw light upon its benign nature, despite an alarming and suspicious appearance on imaging. Due to the large size of our patient's mass (largest reported yet), he needed extensive chest wall reconstruction, leading to complications requiring additional invasive procedures. This underscores the importance of early diagnosis and treatment which can reduce the need for aggressive surgical manipulation and avoid postoperative complications, thereby providing high-value care. Treating physicians should be mindful of this, in order to prompt early recognition to ensure effective patient care.

Pulmonary Embolism in COVID-19 and the Unanswered Questions.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has ravaged the global society as we know it. As almost a dozen pharmaceutical agents go into randomized controlled clinical trials, multiple studies have surfaced trying to associate a hypercoagulable state to coronavirus disease 2019 (COVID-19) patients. We report two COVID-19 cases who presented with occlusive pulmonary embolism (PE) strongly supporting a hypercoagulable state incurred by SARS-CoV-2. This is significant as it is one of the early reports of such an initial presentation of COVID-19 in the USA. Through our report, we invite the medical community to share a perspective about long-term management guidelines for SARS-CoV-2 associated venous thromboembolism (VTE) and prompt future research.

An Unfortunate Case of Reactivation of Tuberculosis in a Postpartum Female.

Tuberculosis (TB) is a widely prevalent disease, especially in resource-limited settings. It poses a big burden to the community and is a significant cause of morbidity and mortality in pregnant females due to their immunosuppressed state. During pregnancy, the immune system is suppressed to prevent fetal rejection, and it gets reconstituted postpartum. During this reconstitution phase, reactivation of TB may occur, making it quintessential to test peripartum females for latent TB, especially those belonging to endemic regions. We describe an unfortunate case of reactivation of TB in a postpartum female from Central America.

Adenosine Deaminase Levels in CSF of Tuberculous Meningitis Patients.

BACKGROUND: Tuberculosis kills five lakh patients in India every year, out of which 7-12 % are with meningeal involvement. Delay in its diagnosis and in initiation of treatment results in poor prognosis and sequlae in up to 25% of cases. The aim of the present study is to look for a simple, rapid, cost effective, non-invasive and fairly specific test in differentiating tubercular etiology from other causes. METHODS: Forty patients between the age of 6 - 24 months attending hospital with symptoms and signs of meningitis were selected and divided into two groups: tubercular and non-tubercular, depending upon the accepted criteria. CSF was drawn and ADA estimated. RESULTS: Out of 19 tubercular patients, 18 had CSF ADA at or above the cutoff value while one had below. Out of 21 non-tuberculous patients, two had ADA levels at or above the cutoff value while 19 had below this value. Results of our study indicate that ADA level estimation in CSF is not only of considerable value in the diagnosis of TBM, CSF ADA level 10 U/L as a cutoff value exhibited 94.73% sensitivity and 90.47% specificity in differentiating tuberculous from non-tuberculous meningitis; it also has 90.00% positive predictive value and 95.00% negative predictive value. CONCLUSIONS: It can be concluded that ADA estimation in CSF is not only simple, inexpensive and rapid but also fairly specific method for making a diagnosis of tuberculous etiology in TBM, especially when there is a dilemma of differentiating the tuberculous etiology from non-tuberculous ones. For this reason ADA estimation in TBM may find a place as a routine investigation. KEYWORDS: Cerebrospinal fluid; Adenosine deaminase; Tuberculous meningitis.