Amorphous Proteinaceous Material Research Articles

Crystallizing galactocele: A rare case report and review of literature.

Crystallizing galactocele is an uncommon condition that produces a viscous, chalky substance on fine needle aspiration cytology. (FNAC). Both the diagnosis and the management of this illness include the use of FNAC. Here, we discuss the case of a 25-year-old nursing woman who experienced left breast edema lump for two years. The upper outer quadrant of the leftt breast was involved by the hard, small, non-tender, and movable enlargement. The lesion's FNAC produced a thick, milky, and chalky substance. Numerous semi-transparent crystals of various sizes and shapes with angulated edges could be seen in cytological smears against a background of granular and amorphous proteinaceous material. A diagnosis of crystallizing galactocele was made on the basis of the patient's clinical history of lactation and characteristic cytological findings. Due to the rarity of this condition-to the best of our knowledge, less than ten cases of crystallizing galactocele have been documented in medical literature.

In hospital mortality and outcomes of patients with acute decompensated diastolic heart failure with and without amyloidosis

Abstract Introduction The incidence of heart failure has exponentially increased over the last few decades and acute decompensated diastolic heart failure is one of the leading causes of hospitalization and readmission. Cardiac amyloidosis is one of the rapidly progressing heart conditions. It occurs due to amorphous proteinaceous material called amyloid into the extracellular space of the heart. The infiltration of the heart from amyloid protein has a broad spectrum of presentation, including diastolic heart failure. Purpose Heart failure due to amyloidosis is characterized by diastolic dysfunction resulting from restrictive cardiomyopathy. The outcomes of hospitalized patient with acute decompensated diastolic heart failure in amyloidosis patients compared to those without amyloidosis is not well defined. Methods We conducted a retrospective cohort study by utilizing the National Inpatient sample database from 2017. Using International Classification of disease (ICD)-10 codes, patients with the diagnosis of acute and acute on chronic diastolic heart failure were enrolled in the study. They were further stratified based on the presence of amyloidosis. The primary outcome was to measure in-hospital mortality, while secondary outcomes included development of acute kidney injury (AKI), Acute respiratory failure (ARF), shock and arrhythmias. Results Out of the 915,694 patients with Acute Decompensated diastolic heart failure, about 2270 had amyloidosis as secondary diagnosis. 6.1% of ADHF and amyloidosis died in hospital, compared to 4.2% in those without amyloidosis (aOR=1.35 CI=0.89–2.05, p=0.197). On multivariate analysis, patients with Amyloidosis had increased odds of developing AKI (aOR=1.40 CI 1.13–1.72, p=0.001), Cardiogenic shock (aOR=2.67 CI 1.56–4.55, p<0.001) and arrhythmias (aOR=1.34, CI 1.10–1.64, p=0.004). The incidence of ARF was however lower in patients without amyloidosis compared to those with it (aOR=0.60, CI 0.47–0.75, p<0.001). Conclusion Amyloidosis is one of the underappreciated and underdiagnosed causes of heart failure. Our study shows an increased risk of complications in acute decompensated heart failure with the presence of amyloidosis. Thus, physicians must be aware of this clinical entity for early diagnosis as patients with advanced disease are likely to have poor prognoses. Funding Acknowledgement Type of funding sources: None.

Efficacy of Iopamidol for Sealing an Injured Thoracic Duct: Pilot Experiments in a Large Animal

Chylothorax can be spontaneously healed by lymphangiography using lipiodol, but pulmonary or systemic embolization is a potential complication. We determined the efficacy of iopamidol for treating chylous leakage in an animal model. Twelve pigs were randomly divided into two groups. After inducing thoracic duct damage, pigs from groups A and B were injected with iopamidol and lipiodol, respectively. At 5, 10, and 30 min after damage induction, the drug effects were monitored by video-assisted thoracoscopy and lymphangiography. In vitro, chyle samples from the pigs were incubated with iopamidol and lipiodol. The damaged thoracic duct was harvested and examined using microscopy. In group A, four and two pigs did not show chylous leakage after 5 and 10 min, respectively. In group B, none showed chylous leakage after 5 min. Nevertheless, the p value was 0.46, and there was no statistically significant difference between groups A and B. In vitro, both iopamidol- and lipiodol-treated chyle samples adhered after 5 min and solidified at 30 min. Our findings confirmed that the damaged thoracic duct was clogged with an amorphous proteinaceous material (iopamidol). Therefore, iopamidol is potentially a new therapeutic agent for chylous leakage. Thoracic duct embolization failures or systemic embolization risks from lipiodol injection may be avoided by injecting iopamidol via the thoracic duct, and this warrants further investigation.

S2935 Gastric Mass: A Case of Primary Amyloidosis

INTRODUCTION: We present a rare case of primary amyloidosis presenting as a bleeding gastric mass. Involvement of the gastrointestinal tract is very common in systemic amyloidosis (30-60%), but very rare in primary amyloidosis (8%). CASE DESCRIPTION/METHODS: An 81-year-old man presented to the hospital with anemia with a hemoglobin of 5.3 g/dL. Symptoms on presentation included severe generalized weakness and weight loss. Lab results showed MCV 94.3, Iron 26 ug/dl, Ferritin 15 ng/dl. Upper endoscopy revealed a large ill-defined mass in the gastric body with diffuse ulcerations, friable mucosa, and intramural hematoma (Figure 1). The mass was biopsied, and histology revealed Congo red positive amorphous proteinaceous material consistent with amyloid with apple-green birefringence under polarized light. (Figures 2A and 2B). Serum protein electrophoresis revealed a gamma monoclonal gammopathy. Serum immunofixation showed an IgG kappa and a free light chain lambda. A serum-free light chain assay was not obtained. A bone marrow biopsy revealed plasmacytosis, adjunct flow cytometry showed CD 38 + Plasma cell population with cytoplasmic kappa light chain excess/restriction. These findings where indictive of primary light chain amyloidosis (AL) and excluded the diagnosis of multiple myeloma or systemic amyloidosis (AA). DISCUSSION: Primary amyloidosis is a rare entity. The reported incidence in a population-based study was approximately 1 case per 100,000 population per year. Fatigue and weight loss are the most common clinical manifestations. Hemorrhagic manifestations, congestive heart failure, nephrotic syndrome, malabsorption, and constipation are all relatively common. The diagnosis is made by establishing the presence of amyloid in tissue. Amyloid is amorphous, waxy-appearing, and pink in H&E slides. The Congo red stain is the most used technique for confirming amyloid in a tissue biopsy. characteristic apple-green birefringence under polarized light is seen. Involvement of the gastrointestinal tract is very common in systemic amyloidosis (30–60%), but very rare in primary amyloidosis. The exact prevalence of primary amyloidosis involvement of the stomach is unknown, but the involvement of the upper gastrointestinal tract appears to be present in 8% of all cases. The mainstay treatment is chemotherapy. primary amyloidosis has a poor prognosis when compared to systemic amyloidosis with a predicted median survival of <2 years despite treatment.Figure 1.: Large ill-defined mass in the gastric body with diffuse ulcerations and friable mucosa.Figure 2A.: Congo red positive amorphous proteinaceous material.Figure 2B.: Apple green birefringence under polarized light.

PULMONARY ALVEOLAR PROTEINOSIS PRESENTING AS INTRACEREBRAL NOCARDIOSIS

SESSION TITLE: Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 07:30 am - 08:30 am INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a rare disorder with an incidence of 0.5 / million people [1]. PAP is caused by the accumulation of surfactant in the alveoli, leading to hypoxemic respiratory failure and increased risk of opportunistic infections [2]. First-line therapy after infection eradication is whole-lung lavage. Treatment with exogenous granulocyte macrophage colony stimulating factor (GM-CSF) is under investigation. PAP can be a diagnostic challenge due to its low prevalence and nonspecific presentation. Here we describe a unique presentation that was diagnosed as PAP. CASE PRESENTATION: A 56 year old male farmer, never smoker, with no past medical history presented with right-sided weakness, two months of dyspnea, and dry cough. Brain MRI demonstrated multiple cerebral rim-enhancing abscesses (Fig 1A). He underwent a craniotomy and evacuation of cerebral abscesses with acid fast cultures positive for Nocardia farcinica. Chest CT showed a "crazy paving" pattern consistent with PAP (Fig 1B, C). Serum anti GM-CSF antibody was positive. Bronchoscopy showed opaque fluid on bronchoalveolar lavage, with negative cultures, and clumps of amorphous proteinaceous material consistent with PAP. Disseminated Nocardia was treated with ciprofloxacin for 1 year and sulfamethoxazole/ trimethoprim for 6 months. He underwent a whole lung lavage with resolution of symptoms. DISCUSSION: PAP is caused by surfactant accumulation due to incomplete removal by alveolar macrophages. Autoimmune PAP (primary PAP) accounts for the majority of cases [1]. Autoimmune PAP is caused by anti GM-CSF antibodies that disrupt macrophage activation, antigen presentation, and phagocytosis [2]. Secondary PAP is thought due to a relative deficiency of GM-CSF usually in the setting of hematologic diseases or dust exposures. The ensuing macrophage dysfunction leads to increased infection risk. Opportunistic infections including Nocardia, Mycobacterium, and fungi in otherwise immunocompetent patients with PAP have been previously reported [2]. In a report of 7 patients with disseminated Nocardiosis without PAP, 5 patients were found to have anti GM-CSF antibodies; two of these patients were also found to have radiographic characteristics of PAP [3]. CONCLUSIONS: Primary PAP is a rare autoimmune disorder that confers an immunocompromised state due to impaired alveolar macrophage activation. Opportunistic infections such as Nocardiosis are frequently seen with PAP. Eradication of the opportunistic infections, and targeted whole lung lavage are effective at relieving hypoxia in PAP. A high diagnostic suspicion is essential in the work-up of PAP. Reference #1: Clinics in Chest Medicine. 2016;37(3):431-440. https://doi.org/10.1016/j.ccm.2016.04.006 Reference #2: Journal of Infectio. 2012;65(2):173-179. https://doi.org/10.1016/j.jinf.2012.03.020 Reference #3: Clinical Infectious Disease. 2014;60(7):1017-1025. https://doi.org/10.1093/cid/ciu968 DISCLOSURES: No relevant relationships by David Dennis, source=Web Response No relevant relationships by Ulysses Magalang, source=Web Response No relevant relationships by Virgil Secasanu, source=Web Response

Lamb congenital lymphatic malformation - a case report

Lymphatic malformations have been rarely reported in literature either in humans or in animals. However, in recent years, concern about these malformations in humans has increased. A five-month-old Rasa Aragonesa male lamb was received at the Ovine Clinical Service of the Veterinary Faculty of Zaragoza, Spain, with a history of cervical protuberances coming from birth. The lamb showed three round swollen lumps (5–12 cm in diameter) parallel to the trachea on the left side of the neck. Clinical examination, haematology, ultrasonography, fluid examination and histopathology were performed. No abnormalities were found in blood samples and ultrasound confirmed a multicystic lesion with internal separations. Histological evaluation of the tissue revealed dilated lymphatic vessels and channels in the dermis and hypodermis; some lymphatic vessels were filled with amorphous proteinaceous material and occasional lymphocytes and macrophages. These protuberances were diagnosed as congenital lymphatic malformations. Most of the gross and microscopical lesions were very similar to those described in humans. To the authors’ knowledge, this is the first time that a congenital lymphatic malformation is described in sheep.

Primary localized amylodoisis of bladder: Is there a need for cystoscopic surveillance?

Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of amorphous proteinaceous material in various tissues. Amyloidosis of bladder is of significant clinical interest to the urologist because of its presentation as urothelial cancer. Transurethral resection and histology examination is essential to exclude malignancy and to establish the benign nature of amyloidosis. Apart from managing the localized bladder amyloidosis, it is important to exclude systemic amyloidosis. Here in we describe two cases of localized, primary amyloidosis and discuss briefly their management and follow-up.

Rapidly Progressive Thyroid Mass

Amyloidosis is a disease of unknown etiology characterized by the accumulation of an amorphous proteinaceous material in various organs and tissues of the body. Amyloid goiter is an exceedingly rare pathologic condition due to massive amyloid infiltration of the thyroid tissue. Amyloid goiter occurs in association with both primary and secondary systemic amyloidosis, more commonly in the latter. Preoperatively, it simulates a multinodular goiter, and surgical intervention is often necessary to establish a diagnosis and to relieve compressive symptoms of a neck mass. We present the case of an 85-year-old female patient who presented with a rapidly enlarging goiter. Histologic examination confirmed amyloid goiter.

Melanins and melanosomes from llama (Lama glama L.).

Analysis of melanins and melanosomes in eight hair and skin samples taken of adult pigmented Argentine llamas (Lama glama L.) has been carried out. In each sample, eumelanins, pheomelanins and alkali-soluble melanins were identified. The total amount of melanins and the amount of eumelanins both decreased from black to reddish brown colour, while pheomelanins were found to be present in small quantities in each sample. Eumelanosomes were round and oval-shaped, displaying transverse striations clearly visible at low magnification. Dark brown samples revealed all four melanosomes stages. Stages I and II melanosomes appeared as large, asymmetrical vacuoles containing numerous microvesicles randomly scattered within an amorphous proteinaceous material (vesiculo-globular bodies). Stage III melanosomes had microgranular melanin deposits in the microvesicles and in the matrix. The fully melanized melanosomes (stage IV) were primarily round-shaped, showing an irregular outline and the electron-dense pigment was arranged to form large clusters. In light brown melanocytes, numerous melanosomes at different maturation stages could be found. Premelanosomes appeared ovoid, containing amorphous proteinaceous material and spotty and microgranular deposits. Mature melanosomes were fully melanized, homogeneously electron-dense, ovoid granules.

A Case of Pulmonary Alveolar Proteinosis Associated With Silicosis

Pulmonary alveolar proteinosis is characterized by the accumulation of PAS positive lipoproteinaceous or amorphous proteinaceous material in the alveolar space with spared delicate septal architecture of the lung interstitium and impaired gas exchange of alveoli. We experienced a case of secondary pulmonary alveolar proteinosis in a 41 year old male patient who have occupational history of engagement as a mason over 4year. He compalined exertional dyspnea and chest discomfort, and presented fine inspiratory crackle at both lower lung field, numerous fine nodular denisties in both lung field with peripheral sparing. Light microscopic finding of lung tissue obtained by transbronchiallung biopsy revealed homogenous eosinophilic colloid-like luminal content in the alveolar space, and electron microscopy of bronchoalveolar lavage fluid concentrate showed electron-dense multilamellated structures. To treat the disease, we tried whole lung lavage of right lung with isotonic saline under general anesthesia. After whole lung lavage of right lung, he showed markid improvement of symptom and partial improvement of chest X-ray findings. The patient has been followed for 12 month until now, with no evidence of aggravation.

Experimental Clogging of Biliary Endoprostheses. Role of Bacteria, Endoprosthesis Material, and Design

The major problem facing patients treated with biliary endoprostheses is their frequent clogging, necessitating their exchange. Clogged endoprostheses contain mainly bacteria embedded in an amorphous proteinaceous material with the occasional presence of food fibres. We studied this problem in an in vitro model, evaluating the role of bacteria, endoprosthesis design, and material in sludge formation. We found endoprostheses perfused with artificially contaminated bile to contain significantly more sludge than those perfused with sterile bile (p less than 0.05). The amount of sludge varied with the bacterial species used. Endoprostheses perfused with bacteria producing beta-glucuronidase were not associated with a particularly large amount of sludge. Endoprostheses with side holes contained significantly more sludge than those without (p less than 0.05). Furthermore, endoprostheses made of material with a low friction coefficient, such as Teflon, contained significantly less sludge than endoprostheses made of materials with a higher friction coefficient, such as polyethylene and polyurethane (p less than 0.05). These results emphasize the role of bacteria in endoprostheses clogging and clearly demonstrate the harmful effect that side holes have on endoprosthesis function.

Crotalaria spectabilis toxicity in swine: Characterization of the renal glomerular lesion

Eighteen feeder pigs each received a cumulative dose of 84 g. of ground Crotalaria spectabilis seeds in 43 days. Varying degrees of lack of thrift were seen in all pigs. During the experiment 2 pigs died from crotalaria poisoning. The survivors were necropsied in 2 groups, 70 and 90 days after the experiment began. Five pigs had multiple petechial renal haemorrhages and subcapsular pitting. Mean numbers of glomerular tuft and parietal cells were significantly lower in both groups of test animals than in controls. Histologically, variable degrees of glomerular dilatation and reduced cellularity and haemorrhage of glomerular tufts occurred. Ultrastructurally, lesions were classified as moderate and severe. Moderate lesions consisted of cytoplasm swelling leading to occulusion of capillary lumina, detachment of endothelial cells, focal dense deposits in the basal lamina and a moderate increase in mesangial matrix. Severe lesions were characterized by the absence of endothelial and mesangial cells, and attenuation and thinning of basal lamina. In such cases, large spaces containing blood cells, amorphous proteinaceous material and occasionally collagen fibrils were seen in place of normal capillary lumina.

Dense bodies of the contractile system of cardiac muscle in Venus mercenaria

AbstractDense bodies in the heart muscle of Venus mercenaria exist in two forms, free and attached. Free dense bodies morphologically consist of fascicles of thin filaments in parallel array and bound together by a dense, amorphous proteinaceous material. The binding of dense bodies to the cell membrane is effected via connecting filaments of the amorphous material of the dense body which join a condensation of morphologically similar material attached to the inner osmiophilic layer of the unit membrane. This composite of dense body, connecting filaments, membrane condensation and unit cell membrane has been termed collectively the attachment plaque. The attachment plaque is part of an extensive network on the cell surface which obligates that surface to a role in the contractile process. Moreover, this set of attachment plaques imposes an organization and an orientation to most thin filaments of the cell and preserves the contractile axis of the cell.