Browsing by Author "Sahebjam, Farhad"

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    A Case of Macroenzyme Aspartate Aminotransferase Mimicking Hepatic Injury
    Schimming, Sarah; Bansal, Jash; Sahebjam, Farhad (Cureus, 2021-08-19)
    Macroenzymes are high molecular weight complexes that are formed by the binding of normal serum enzymes with circulating immunoglobulins - primarily IgG, IgA, and IgM. These high molecular weight complexes are difficult to clear through the kidneys, therefore they remain persistently elevated in the serum leading to inaccurate diagnoses and unnecessary workup. The prevalence of macroenzymes is relatively rare in the general population; however, it is an important phenomenon to be aware of when working up isolated elevations in serum enzymes. A quick and efficient test for the detection of macroenzymes is the polyethylene glycol precipitation test. Here, we present a case of macro-aspartate aminotransferase masquerading as an underlying hepatobiliary disease ultimately leading to an extensive evaluation before arriving at the correct diagnosis. This case highlights the importance of an accurate and efficient diagnosis of macroenzymes in the serum in order to prevent unnecessary healthcare utilization and also to decrease the psychological burden on the patient.
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    Persistent diarrhea with petechial rash - unusual pattern of light chain amyloidosis deposition on skin and gastrointestinal biopsies: A case report
    Bilton, Shawna E.; Shah, Nikhil; Dougherty, Diana; Simpson, Sarah; Holliday, Alex; Sahebjam, Farhad; Grider, Douglas J. (Baishideng, 2022-10-06)
    BACKGROUND Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils. Gastrointestinal involvement in systemic amyloidosis is common, but is often subclinical or presents as vague and nonspecific symptoms. It is rare for gastrointestinal symptoms to be the main presenting symptom in patients with systemic amyloidosis, causing it to be undiagnosed until late-stage disease. CASE SUMMARY A 53 year-old man with diarrhea, hematochezia, and weight loss presented to a community hospital. Colonoscopy with biopsy at that time was suspicious for Crohn disease. Due to worsening symptoms including nausea, vomiting, and a new petechial rash, an abdominal fat pad biopsy was done. The biopsy showed papillary and adnexal dermal amyloid deposition, in a pattern usually seen with cutaneous amyloidosis. However, Cytokeratin 5/6 was negative, excluding cutaneous amyloidosis. The patterns of nodular amyloidosis, subcutaneous amyloid deposits and perivascular amyloid were not seen. Periodic Acid-Schiff stain was negative for lipoid proteinosis, Congo red was positive for apple green birefringence on polarization and amyloid typing confirmed amyloid light chain amyloidosis. Repeat endoscopic biopsies of the gastrointestinal tract showed amyloid deposition from the esophagus to the rectum, in a pattern usually seen in serum amyloid A in the setting of chronic inflammatory diseases, including severe inflammatory bowel disease. Bone marrow biopsy showed kappa-restricted plasma cell neoplasm. CONCLUSION Described is an unusual presentation of primary systemic amyloidosis, highlighting the risk of misdiagnosis with subsequent significant organ dysfunction and high mortality.
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    Vanishing Bile Duct Syndrome Associated With Estrogen
    Benfield, John; Shah, Raj A.; Grider, Douglas J.; Sahebjam, Farhad (Lippincott Williams & Wilkins, 2022-05)
    Vanishing bile duct syndrome (VBDS) refers to a form of cholestatic liver disease with many etiologies. Vanishing bile duct syndrome is characterized by biliary ductopenia and chronic cholestasis. This is a challenging condition for clinicians because of its rarity and unclear pathophysiology. Presented is an 18-year-old woman who developed cholestatic liver injury and intrahepatic biliary ductopenia after a course of oral contraceptives and intravenous estrogen for uterine bleeding. A year later, this patient did not have significant improvement in liver biomarkers and was referred for transplantation.