scholarly journals Nephrotic Syndrome Following Resection of an Adrenal Incidentaloma:A Case Report

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A144-A145
Author(s):  
Maria Batool ◽  
Eamon Leen ◽  
Siobhan Glavey ◽  
Seamus K Sreenan ◽  
John McDermott
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Congo Red ◽  
Amorphous Material ◽  
Adrenal Lesion ◽  
Primary Amyloidosis ◽  
Secondary Amyloidosis ◽  
Congo Red Staining ◽  
T2 Hyperintensity ◽  
Al Amyloid

Abstract A 69 year old man had a 5 cm right adrenal lesion discovered incidentally while being investigated for a deterioration in previously well-controlled hypertension. Routine investigations including serum albumin were normal. Further investigation confirmed a non-functioning adrenal lesion. MRI revealed a ‘non-fat-containing T1 hyperintense indeterminate adrenal lesion with speckling of T2 hyperintensity, not typical for adenoma, hyperplasia, myelolipoma, haemangioma or pheochromocytoma’. An uncomplicated laparoscopic adrenalectomy was performed. Histology revealed a 118 g adrenal neoplasm, modified Weiss score 0, with abundant hyaline deposits.3 months later the patient complained of peripheral oedema. Investigations revealed a serum albumin of 24 g/L and 14 g of proteinuria in 24 hours. Serum protein electrophoresis revealed a monoclonal IgA type lambda band. Renal biopsy revealed amorphous material displaying apple green birefringence on staining with Congo Red, which stained with antibodies to lambda light chains, confirming AL amyloid. Therefore the patient’s resected adrenal specimen was retrieved and stained with Congo Red, revealing apple green birefringence in the walls of the blood vessels, confirming the presence of amyloidosis. Although adrenal gland involvement in secondary amyloidosis is common, adrenal involvement in primary amyloidosis is less well described. This case illustrates the indolent nature of primary amyloidosis, prior to the development of often catastrophic symptoms. Consideration should be given to Congo Red staining of resected pathologic specimens containing hyaline deposition, to potentially allow for earlier recognition of this devastating disease. A pathophysiologic link between the patient’s incidentaloma, adrenalectomy, and onset of nephrotic syndrome remains a matter for conjecture.

scholarly journals Gastroparesis in a Patient with Gastric AL Amyloidosis

2018 ◽  
Vol 12 (2) ◽  
pp. 317-321 ◽  
Author(s):  
Matthew Hoscheit ◽  
Afrin Kamal ◽  
Michael Cline
Keyword(s):  
Gastrointestinal Tract ◽  
Gastric Emptying ◽  
Light Chain ◽  
Congo Red ◽  
Al Amyloidosis ◽  
Primary Amyloidosis ◽  
Secondary Amyloidosis ◽  
Complex Disorders ◽  
Clinical Presentations ◽  
Congo Red Staining

Systemic amyloidosis is a group of complex disorders characterized by the extracellular deposition of nonbranching fibrils in various tissues, ultimately leading to a variety of clinical presentations including isolated or multiorgan dysfunction. Amyloid involvement of the gastrointestinal tract is common depending on the subtype of this disease; light chain primary amyloidosis (AL) and secondary amyloidosis (AA) affect the gastrointestinal tract in unique ways due to differing pathophysiology. A case is reported of gastroparesis associated with AL amyloidosis diagnosed by esophagogastroduodenoscopy and study of gastric emptying, then subsequently confirmed by Congo red staining performed on endoscopic biopsies.

scholarly journals Diagnosis and the treatment of primary amyloidosis

2010 ◽  
Vol 67 (9) ◽  
pp. 781-785 ◽  
Author(s):  
Irena Djunic ◽  
Dragica Tomin ◽  
Maja Perunicic ◽  
Ana Vidovic ◽  
Vesna Cemerikic ◽  
...  
Keyword(s):  
Congo Red ◽  
Rectal Biopsy ◽  
Diagnostic Procedures ◽  
Primary Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Biopsy Specimens ◽  
Plasma Cell Disorders ◽  
History Of ◽  
Congo Red Staining ◽  
Urine Proteins

Backgraund. Primary amyloidosis belongs to a group of monoclonal plasma cell disorders, characterized by extracellular deposition of immunoglobulin light chain fibrils in various tissues and subsequent multiorgan dysfunction. Case report. We present a 51-year-old female with 2-years history of fatigue on exertion, oedema of face, abdomen and legs, bone pain and obstipation. After diagnostic procedures such as electrophoresis and immunoelectrophoresis of serum and urine proteins, immunohistohemical staining of bone marrow biopsy specimens and Congo red staining of rectal biopsy specimens, the patient received misdiagnosis of multiple myeloma and was referred to our hospital for further treatment. We reevaluated and complemented diagnostic procedures (ehocardiosonography and biopsy of subcutaneaus tissue with Congo red staining), and established diagnosis of primary amyloidosis. The therapy had started with intravenous (iv) melphalan and dexamethasone (totally eight cycles) and continued with peroral melphalan and iv dexamethasone. Stabilization of the disease was achieved after 35 months of the treatment. Conclusion. The case of this rare and often fatal disease emphasizes significance of early diagnosis and, consequently, initiation of specific therapies which are indispensable to improve the disease prognosis.

Serum albumin level accurately reflects antioxidant potentials in idiopathic nephrotic syndrome

2011 ◽  
Vol 16 (3) ◽  
pp. 411-414 ◽  
Author(s):  
Kazunari Kaneko ◽  
Takahisa Kimata ◽  
Shoji Tsuji ◽  
Tomohiko Shimo ◽  
Masaya Takahashi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Albumin ◽  
Serum Albumin Level ◽  
Idiopathic Nephrotic Syndrome ◽  
Albumin Level

SYSTEMIC AMYLOIDOSIS IN YOUNG MICE INDUCED BY HUMAN SALIVA ADMINISTRATION

2021 ◽  
pp. 40-56
Author(s):  
Vadim A. Kozlov ◽  
Vera Yu. Aleksandrova ◽  
Yulia V. Vasilyeva ◽  
Sergey P. Sapozhnikov ◽  
Pavel B. Karyshev
Keyword(s):  
Visual Field ◽  
Congo Red ◽  
Healthy Person ◽  
Human Saliva ◽  
Systemic Amyloidosis ◽  
Chronic Tonsillitis ◽  
Relative Area ◽  
Congo Red Staining ◽  
Number Of Cells ◽  
Young Mice

The urgency of the study lies in the fact that for the first time it was proved in the experiment that intraperitoneal administration of human saliva to young mice causes the development of systemic amyloidosis, comparable to hereditary systemic amyloidosis in humans, induced by mutagenic changes in saliva lysozyme. The aim of the research: to test human saliva as an amyloidogen in a model experiment on young mice in comparison with the albumin model of amyloidosis that we developed earlier. White male mice weighing 20.0–25.0 g were divided into six groups: Intact mice (5); 30 days 1 time every other day were intraperitoneally administered to groups: 1 (5) soy cream substitute TU 9199-004-58706213-10 15 10 g/100 ml water 0.1 ml/10 g; 2 (3) saliva of a healthy person (SHP) 0.5 ml; 3 (3) SHP and Ciprofloxacin (C) 0.05 mg/10 g of weight; 4 (3) saliva of a person with chronic tonsillitis (remission, SCT); 5 (3) SCT+C. Kidney weight changed from 176.0±21 mg in intact mice (IM) to 197±43,0, 195,0±18,0, 195,0±18,0, 183,0±44,0, 153,0±25,0 mg, respectively. The number of cells per visual field of the tubular epithelium decreased by 1.3, 1.2, 1.6, 1.4, 1.4 times from the number of cells per visual field in IM 380.0±84.0 (p = 0.00), glomerular cells – by 1.4, 1.1, 1.3, 1.2, 1.2 times from the number of glomerular cells in IM 34.0±11.0 (p = 0.02), in groups 1-5, respectively. The glomerular area decreased by 2.4, 2.7, 2.3, 2.4, 3.0 times from the average glomerular area in IM (3163.7±832.7 mm2, p = 0.0000). The relative area of amyloid lesion when stained with Congo red: 18.4±8.2% (group 1), 26,7±10,6, 35,0±11,9, 45,7±13,2, 63,6±14,0% (groups 1-5). The spleen mass and length increased from 133.0±16.0 mg (IM) by 1.7, 1.7, 1.8, 2.1, 1.7 times and from 17.0±2.0 mm (IM) by 1.2, 1.2, 1.3, 1.4, 1.2 times, the number of cells per visual field decreased from 1318±205 (IM) by 1.7, 2.0, 3.3, 2.1, 2.0 times, groups 1-5, respectively (p < 0.01). The relative area of amyloid lesion in Congo red staining: 11,4±9,4%, 28,2±16,0, 27,9±20,8, 20,9±12,2, 16,5±8,3% (groups 1-5). The liver mass changed from 1.4±0.15 g (IM) to 1,57±0,26, 1,45±0,18, 1,53±0,1, 1,71±0,3, 1,36±0,4 g, the number of cells per visual field decreased from 67.0±15.0 (IM) by 1.9, 1.4, 1.8, 2.5, 1.9 times, the area of hepatocyte nuclei – from 54.0±12.3 µm (IM) by 3.1, 3.6, 4.2, 3.1, 3.3 times (groups 1-5, respectively, p < 0.01). The relative area of amyloid lesion in Congo red staining: 6,8±4,6%, 15,3±11,0, 24,5±12,8, 9,8±8,5, 12,6±2,3% (groups 1-5). Conclusions: 1) human saliva when administered intraperitoneally causes severe systemic amyloidosis in young mice with damage to the liver, kidneys and the spleen, which manifests more by cytotoxic effect than the volume of amyloid deposition; 2) human saliva contains a more active amyloidogen than albumin in combination with fillers of the soy cream substitute formulation; 2) human saliva of a person with chronic tonsillitis in remission is more amyloidogenic than the saliva of a healthy person; 3) Ciprofloxacin, administered in a therapeutic dose during the amyloid model formation, moderately increases the severity of amyloid organ damage; 4) human saliva can be used to simulate amyloidosis in an experiment on young mice; 5) the liver may contain enzymatic systems that perform the function of amyloidoclasia.

scholarly journals Evaluation of Congo Red Staining Kit to Determine Proteinuria in Preeclampsia

2018 ◽  
Author(s):  
JL Rodríguez Chávez ◽  
EK Fuentes Gutiérrez ◽  
MJ AngelesVázquez ◽  
H Mendieta Zerón
Keyword(s):  
Congo Red ◽  
Congo Red Staining

Feline Insular Amyloid: Histochemical Distinction from Secondary Systemic Amyloid

1981 ◽  
Vol 18 (2) ◽  
pp. 181-187 ◽  
Author(s):  
B. L. Yano ◽  
K. H. Johnson ◽  
D. W. Hayden
Keyword(s):  
Sulfuric Acid ◽  
Potassium Permanganate ◽  
Congo Red ◽  
Staining Procedure ◽  
Domestic Cats ◽  
Tissue Sections ◽  
Dilute Sulfuric Acid ◽  
Congo Red Staining ◽  
Red Dye ◽  
Human Primate

Amyloid in islets of Langerhans from 48 domestic cats, one human, one non-human primate, and one raccoon was compared with secondary systemic amyloid from three domestic cats, one dog, one human, and one cow to determine affinity for Congo red dye after treatment of paraffin-embedded tissue sections with potassium permanganate and dilute sulfuric acid. Insular amyloid from all six species was resistant to pretreatment with potassium permanganate, i.e., affinity for Congo red was retained, whereas secondary systemic amyloid from all species was sensitive to the potassium permanganate pretreatment. Other stains did not distinguish between insular and secondary systemic amyloid. The potassium permanganate-Congo red staining procedure thus can be used to differentiate insular from secondary systemic amyloid in the cat and other species. The results also indicate that insular amyloid and secondary systemic amyloid are of different chemical composition and pathogenesis.

scholarly journals MP172TOCILIZUMAB TREATMENT FOR NEPHROTIC SYNDROME DUE TO SECONDARY AMYLOIDOSIS IN RHEUMATIC AND INFLAMMATORY DISEASES

2016 ◽  
Vol 31 (suppl_1) ◽  
pp. i398-i398
Author(s):  
Sheila Cabello Pelegrin ◽  
Miguel Giovanni Uriol Rivera ◽  
Julia Fernandez Melón ◽  
Javier Rascón Risco
Keyword(s):  
Nephrotic Syndrome ◽  
Inflammatory Diseases ◽  
Secondary Amyloidosis

scholarly journals Histochemical Differential Diagnosis and Polarization Optical Analysis of Amyloid and Amyloidosis

2006 ◽  
Vol 6 ◽  
pp. 154-168 ◽  
Author(s):  
M. Bély
Keyword(s):  
Differential Diagnosis ◽  
Congo Red ◽  
Early Recognition ◽  
Polarized Light ◽  
Optical Analysis ◽  
Early Start ◽  
Amyloid Deposits ◽  
Protein Fibrils ◽  
Congo Red Staining ◽  
Sensitive Method

Amyloidosis is characterized by extracellular deposition of protein fibrils of chemically heterogeneous composition. Early recognition and identification of amyloid deposits allows an early start of therapy, which may entail a better prognosis. Congo red staining according to Romhányi (1971) is a highly specific and sensitive method for early microscopic recognition of amyloidosis. The main and most important types of amyloidosis may be distinguished by classic histochemical methods of performate pretreatment according to Romhányi (1979), or by KMnO4oxidation according to Wright (1977) followed by Congo red staining and viewed under polarized light. Differences in the speed of breakdown (disintegration) of amyloid deposits according to Bély and Apáthy allow a more precise distinction of various types of amyloid.

Sign in / Sign up

Export Citation Format

Share Document