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At least 1,171 records · Page 65Linked to original sources
PubMed1997

Crohn's disease associated with renal amyloidosis successfully treated with an elemental diet.

We report a case of Crohn's disease associated with nephrotic syndrome due to renal amyloidosis in a 21-year-old man in whom remission of both Crohn's disease and the nephrotic syndrome has been maintained with an elemental diet. The patient developed toxic megacolon and nephrotic syndrome due to renal amyloidosis. Intensive intravenous prednisolone therapy with total parenteral nutrition was dramatically effective in treating the toxic megacolon and inducing remission in Crohn's disease and afterward, remission of the nephrotic syndrome. Remission of both conditions has been maintained for more than 2 years with the elemental diet. To our knowledge, this is the first confirmed case of Crohn's disease complicated with renal amyloidosis in which only slight proteinuria (below 0.3 g/day) was shown with an elemental diet used for a long period.

Adult↗
PubMed1990

Amyloidosis associated bleeding diatheses in the surgical patient.

The coagulopathies associated with amyloidosis have not been widely appreciated. We encountered a patient with amyloidosis and mildly abnormal coagulation studies who presented for an emergency laparotomy for acute bowel obstruction. Upon gentle manipulation of the bowel, an unexpected bleeding diathesis was manifested by the formation of several large haematomas resulting in a large amount of blood loss (approximately 800 ml). Numerous defects of platelet function and coagulation have been associated with amyloidosis, including abnormal platelet aggregation, increased vascular fragility, factor IX and X deficiencies, decreased levels of alpha-2-plasmin inhibitor, and increased levels of plasminogen. Intraoperative therapy for our patient included the rapid administration of four units of fresh frozen plasma and ten units of platelet concentrate. A review of the pathology and therapeutic modalities for this potentially life-threatening complication is presented.

Amyloidosis↗
PubMed2004

Laryngo-tracheo-bronchial stenosis in a patient with primary pulmonary amyloidosis: a case report and brief review.

PURPOSE: To report a case of lower respiratory tract obstruction occurring in a patient with primary pulmonary amyloidosis and discuss anesthetic management. CLINICAL FEATURES: A 53-yr-old man was referred to our institution for microlaryngoscopy and laser treatment of the larynx. He presented with a five-year history of primary laryngo-tracheo-bronchial amyloidosis and symptoms consistent with narrowing of the conducting airways. General anesthesia was induced with iv propofol 150 mg and remifentanil 50 microg. Mivacurium 20 mg provided muscle relaxation for endotracheal intubation. Following endotracheal intubation, the airway became obstructed and patient ventilation impossible. The endotracheal tube was removed and a Dedo laryngoscope inserted. Gas exchange was maintained using supraglottic jet ventilation via a distal port of the laryngoscope. Rigid bronchoscopy showed tissue partially obstructing the lumen of the lower trachea. This was removed and the airway appeared patent. At the end of the case, a further episode of lower airway obstruction occurred requiring reinsertion of the laryngoscope and resumption of jet ventilation. Extensive debridement through the bronchoscope was required before adequate ventilation could be restored. Some days later when the patient's condition deteriorated again and he required further debridement of the trachea and insertion of a tracheostomy, guide wires were positioned in the femoral vessels in the event that cardiopulmonary bypass was required for gas exchange. CONCLUSIONS: Primary laryngo-tracheo-bronchial amyloidosis is a recurrent disease, requiring repetitive surgical procedures. Airway compromise can be a persistent problem. Awareness of this uncommon disease process and its presentation may serve to caution the anesthesiologist presented with this type of case.

Amyloidosis↗
PubMed2000

Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis.

Although involvement of the liver is common in systemic amyloidosis, clinical manifestations of hepatic dysfunction and liver biochemical abnormalities are often absent or only mild. Here we report on a patient with primary amyloidosis and rapid development of liver failure, who was successfully treated by liver transplantation. The patient is a 61-year-old Swedish man who was admitted to the local hospital for spontaneous rupture of the spleen. Before admission, he had suffered from diffuse upper abdominal discomfort, diminished appetite, and had lost 15 kg in 6 months. Shortly after splenectomy, he developed cholestatic liver failure with moderate hepatomegaly, jaundice, ascites and hyponatremia. Over a period of 3 weeks his liver failure progressed, renal function deteriorated rapidly, and he developed encephalopathy. Liver transplantation was performed on the 35th day after splenic rupture. Histological examination revealed extensive deposits of amyloid in the spleen and liver. N-terminal amino acid sequence analysis of the amyloid protein, purified from the patient's native liver, revealed an AL protein of kappa I-type origin. The postoperative course was uncomplicated, apart from one episode of sepsis and one course of treatment for acute rejection. He was discharged from hospital with normal liver function and good kidney function. One year after surgery, he was in good condition, with normal liver function. However, a liver biopsy taken at the same time showed de novo amyloid deposits in the grafted liver. We conclude that liver transplantation may be indicated as a life-saving procedure in rapidly progressing hepatic amyloidosis with cholestatic jaundice, although the underlying disease has not changed.

Amyloidosis↗
PubMedDate not supplied

Primary isolated urethral amyloidosis.

We report an unusual case of primary amyloidosis isolated to the urethra in a patient with tender, periurethral masses and obstructive voiding symptoms. When not secondary to other phenomena, amyloidosis most commonly affects the skin, respiratory, and urinary tracts. Isolated amyloidosis of the urethra should be in the differential diagnosis of a patient with irregular strictures of the penile urethra, a clinical presentation consistent with urethral carcinoma, and no evidence of systemic disease.

Adult↗
PubMedDate not supplied

Cardiac amyloidosis: typical imaging findings and diffuse myocardial damage demonstrated by delayed contrast-enhanced MRI.

Amyloidosis is a rare systemic disease. However, involvement of the heart is a common finding and is the most frequent cause of death in amyloidosis. We report the sonographic, scintigraphic, and MRI features of a pathologically proven case of cardiac amyloidosis. Delayed contrast-enhanced MR images, using an inversion recovery prepped gradient-echo sequence, revealed diffuse enhancement in the wall of both left and right ventricles. This enhancement suggested expansion of the extracellular space of the myocardium caused by diffuse myocardial necrosis secondary to deposition of amyloid.

Aged↗
PubMed1998

CT findings in extensive tracheobronchial amyloidosis.

Primary pulmonary amyloidosis is a rare disorder that appears in three forms: tracheobronchial, nodular parenchymal, and diffuse parenchymal. We report the case of a 46-year-old women with extensive tracheobronchial amyloidosis which presented with a 2-year history of dyspnea and with signs of severe fixed obstruction in pulmonary function tests. Computed tomography of the thorax demonstrated marked thickening of the trachea and the central bronchial tree with substantial narrowing of the main, lobar, and segmental bronchi. Transbronchial specimen showed typical birefringence under polarizing microscope after staining with Congo Red. We did not find hints for systemic amyloidosis.

Amyloidosis↗
PubMed1999

Amyloidosis presenting as a perforated giant colonic diverticulum.

Colonic amyloidosis is frequent, although rarely radiographically evident. The radiological findings are varied and unspecific. We report a case of perforated giant colonic diverticulum (GCD) diagnosed by helical CT in which amyloid deposition was pathologically demonstrated. The patient was a renal transplant recipient who previously had undergone hemodialysis for 10 years. A possible role of amyloidosis in the pathogenesis of this case is discussed. Giant colonic diverticulum must be included in the spectrum of gastrointestinal manifestations of amyloidosis.

Aged↗
PubMed2001

Pancreatic involvement in primary amyloidosis: radiologic findings.

Amyloidosis is a rare disease that can involve a great variety of organs. As a consequence, clinical and radiological manifestations are very different as well. We report on a patient with primary amyloidosis who presented with diffuse pancreatic involvement detected by ultrasound, helical CT, and MRI. There are very few imaging descriptions about this glandular involvement in amyloidosis in the literature. Despite the apparent uncommon radiological appearance, we believe that this entity should be taken into account in the differential diagnosis of diffuse infiltrative pancreatic lesions in the appropriate clinical and analytical setting.

Adult↗
PubMed2004

The effect of oxidative stress on amyloid precursor protein processing in cells engaged in beta-amyloidosis is related to apolipoprotein E genotype.

The reduced antioxidative defense in allele epsilon4 carriers is suggested to contribute to beta-amyloidosis in Alzheimer's disease and Down's syndrome. We studied the effect of oxidative stress on accumulation of amyloid-beta peptide (Abeta) in vascular smooth muscle cells (SMCs) that are engaged in production of amyloid-beta in vivo. Previously, we found that oxidative stress caused by ferrous ions induced accumulation of Abeta-apolipoprotein E deposits in lysosomes and was associated with a greater oxidative protein damage in epsilon4 carriers. Here, we demonstrate that ferrous ions induce formation of Abeta deposits also in vascular tunica media in organotypic cultures of whole brain vessels, suggesting the role of oxidative stress in development of vascular beta-amyloidosis. Cellular accumulation of Abeta in SMCs treated with ferrous ions was associated with a greater accumulation of C-terminal amyloid precursor protein (APP) fragments in epsilon4/epsilon4 than in epsilon3/epsilon3 myocytes and reduced the amount of soluble APPalpha in epsilon3/epsilon3, but not epsilon4/epsilon4, cultures. Antioxidant vitamin E prevented these effects, and, when applied alone, diminished the amount of APP C-terminal fragments and increased the amount of secreted APP in epsilon3/epsilon3, but not epsilon4/epsilon4, cells. C-terminal APP-immunoreactive material was accumulated in lysosomes partly with Abeta- and N-terminal APP immunoreactivities. These results suggest that the increased accumulation of APP and its fragments in lysosomes may yield additional amounts of cellular Abeta, particularly in epsilon4 carriers. We hypothesize that the altered processing of APP in SMCs locally exposed to oxidative stress facilitates cellular deposition of Abeta and contribute to the increased risk of development of beta-amyloidosis in epsilon4/epsilon4 carriers.

Aged↗
PubMed2004

Nonsecretory multiple myeloma with amyloidosis. A case report and review of the literature.

We report a case concerning a 49-year-old female patient with thoracic pain. X-rays showed a single osteolytic lesion on the right seventh rib. The excision of the rib disclosed a plasmocytic plasmocytoma with extensive amyloidosis. Serum and urine protein electrophoresis were both negative for monoclonal gammopathy. Bone marrow biopsy showed that 80% of the marrow had been replaced by plasma cells. A diagnosis of nonsecretory multiple myeloma was made. Immunohistochemistry revealed amyloid light (AL) amyloidosis of kappa-light chain origin. The relationship between nonsecretory multiple myeloma and amyloidosis is discussed.

Amyloid↗
PubMed2006

Renal amyloidosis in a child with sickle cell anemia.

The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.

Adolescent↗
PubMed1997

Renal, gastric and thyroidal amyloidosis due to familial Mediterranean fever.

Chronic renal failure developed in a 10-year-old girl due to renal amyloidosis secondary to familial Mediterranean fever (FMF). During management of the chronic renal failure by hemodialysis and of FMF with colchicine, goiter and hypothroidism were observed. Thyroid fine-needle aspiration and gastric endoscopical biopsies, performed when recurrent abdominal pain could not be controlled, revealed amyloid deposits in both thyroid and gastric tissues. After 6 months' therapy with colchicine and levothyroxine, there was no significant change in the thyroid volume. This is the first case in which gastric amyloidosis secondary to FMF in childhood has been demonstrated. Patients with amyloidosis secondary to FMF who have thyroid enlargement and unexplained gastrointestinal symptoms despite adequate therapy should be evaluated with imaging studies and biopsy examinations.

Amyloidosis↗
PubMed2006

Autologous stem-cell transplantation in progressing amyloidosis is associated with severe transplant-related toxicity.

BACKGROUND: Amyloid light-chain (AL) amyloidosis is a disorder of plasma cells in which depositions of immunoglobulin light-chain fragments cause progressive organ failure and death with a median survival of one year, but autologous stem-cell transplantation can induce high response rates, especially in isolated renal involvement. METHODS: Six patients aged between 43 and 59 years were diagnosed with AL-amyloidosis and had stem cells mobilized with either recombinant human granulocyte colony-stimulating factor (rhG-CSF) alone (n = 2) or cyclophosphamide (2-4 g/m(2)) and rhG-CSF (n = 4). All six patients had kidney involvement and nephrotic syndrome, four had cardiac involvement and two involvement of the vascular, nervous and gastrointestinal systems. Five of the patients received high-dose melphalan (200 mg/m(2)) and autologous blood stem-cell support. RESULTS: One patient died as a result of sepsis after stem-cell mobilization. The other five patients received high-dose melphalan but experienced severe toxicity. One patient died as the result of gastrointestinal perforation on day 6, one presented with hyperfibrinolysis and spontaneous rupture of the spleen, and another experienced severe bleeding of the gastrointestine, tachyarrhythmia and hemolytic anemia. Four patients had acute renal failure: three required hemodialysis and one underwent renal transplant 21 months later. Restaging after a follow-up of 31-52 months revealed reversal of nephrotic syndrome in all three patients who regained adequate renal function. With respect to cardiac involvement (n = 2), one patient showed a decrease in NYHA class from II to I but baseline wall thickness remained stable. CONCLUSION: Treatment of selected patients with AL-amyloidosis by high-dose melphalan and stem-cell support results in reversal of amyloid-related disease in a substantial proportion of patients and improved survival.

Adult↗
PubMed2006

Abeta-2M-amyloidosis and related bone diseases.

Abeta-2M-amyloidosis is a type of systemic amyloidosis that is specifically seen in patients with chronic kidney diseases. The precursor protein of Abeta-2M-amyloid fibril is beta2-microglobulin, and its elevated serum level is the main cause of Abeta-2M-amyloidosis in patients with kidney failure. However, the precise mechanism of Abeta-2M-amyloidogenesis remains unclear. In vitro analyses of Abeta-2M amyloidogenesis are still being actively conducted. Osteolytic lesions are often found around synovial membrane with Abeta-2M-amyloid deposition. Both evident osteoclastogenesis and active osteoclastic bone resorption are found, while osteoblastic bone formation is absent in the lesion most likely associated with the inflammation caused by infiltrating macrophages/monocytes into Abeta-2M-amyloid deposition. The precise cell biological mechanism of this inflammatory change is unknown. Further studies are needed to establish specific treatments against this as yet unsolved problem with long-term dialysis therapy.

Amyloid↗
PubMed2005

An elderly female who survived more than 30 years following a diagnosis of Takayasu's arteritis, complicated by fatal intestinal amyloidosis.

Compared to young patients with Takayasu's arteritis (TA), little information about elderly patients with TA has been reported. Additionally, no reports were found regarding TA cases with complications of intestinal amyloidosis. This is a case report of an elderly female, who developed intestinal amyloidosis, during late-stage TA. After years of outpatient management, she developed sudden severe dyspnea with pulmonary effusion, requiring hospitalization. After this event, betamethasone was replaced by methotrexate (MTX) for the next 34 months, but it seemed ineffective. After 1.5 years, she developed intractable diarrhea, followed by increases in BUN and serum creatinine (Cr), requiring several courses of hemodialysis. Colonoscopy revealed the presence of amyloid in her intestine, although she died of complicated sepsis caused by MRSA infection. This may be the first paper describing intestinal amyloidosis in a TA patient. Additionally, her case is rare in that she lived more than 30 years after the onset and diagnosis of TA.

Aged↗
PubMed2002

Primary systemic amyloidosis presenting as a colonic stricture: successful treatment with left hemicolectomy followed by autologous hematopoietic stem-cell transplantation: report of a case.

Intestinal tract involvement by primary systemic amyloidosis is frequent but usually asymptomatic. Ischemic colitis caused by amyloid infiltration of wall blood vessels can occasionally be observed. We report a 62-year-old female with primary systemic amyloidosis who presented with intestinal obstruction caused by ischemic stricture of the sigmoid colon, secondary to submucosal amyloid deposition. The patient was successfully treated with surgical resection followed by high-dose chemotherapy and hematopoietic stem-cell transplantation. The clinical manifestations and differential diagnosis of gastrointestinal involvement of primary systemic amyloidosis, as well as its current treatment, are discussed.

Amyloidosis↗
PubMed2007

Schistosoma-induced amyloidosis in hamsters is gender-dependent.

BACKGROUND: A high serum level of female protein (FP), found to be a constituent of Syrian hamster amyloid was associated with enhanced amyloidosis. In this work, we studied the sex-limited factors in the induction of amyloidosis in Syrian hamsters infected with either Schistosoma mansoni or S. hematobium cercariae. METHODS: Hamsters were infected with different species of schistosome cercariae and sacrificed after different time periods of infection. Kidney and liver specimens were processed in paraffin, stained with Congo-red and examined by ordinary light and polarized light microscopy. RESULTS: Statistical analysis showed a significant difference in intensity of kidney and liver amyloid deposits (P<0.002 & <0.007 respectively) between females and male hamsters with extensive deposits in the former. Amyloid deposits were correlated significantly to the duration of infection (P<0.001) than the load of worm recovered. CONCLUSION: From this study, we conclude that, in hamster model, Schistosoma-induced amyloidosis is enhanced in females than male hamsters. This may be due to the high serum level of FP that is normally detected in females. As an experimental model for schistosomal nephropathy, we recommend to use male hamsters instead of females to minimize the effect of amyloid deposits, which may mask other pathological changes associated with schistosomal infection.

Amyloidosis↗