Rheumatic heart disease associated with secondary renal amyloidosis

Introduction: Amyloidosis is a disorder of protein folding in which normally soluble proteins undergo conformational changes and are deposited in the extracellular space in an abnormal fibrillar form. Accumulation of these fibrils causes progressive disruption of the structure and function of tissues and organs, and the systemic forms of amyloidosis are frequently fatal. The conditions that underlie amyloid deposition may be either acquired or hereditary. Amyloid-A (AA) amyloidosis is the most common form of systemic amyloidosis worldwide, AA amyloidosis occurs in the course of chronic inflammatory diseases, hereditary periodic fevers, and with certain neoplasms such as Hodgkin disease and renal cell carcinoma. Amyloidosis due to rheumatic heart disease (RHD) is not common but can be seen. We report here a patient with RHD and AA renal amyloidosis. Case Report: we present a 30 year-old Egyptian male with a history of RHD, accidently discovered to have nephrotic range proteinuria and rising serum creatinine. Serology studies were negative or normal, including antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA). C3 and C4 complement levels were normal. Kidney biopsy revealed AA renal amyloidosis. CT chest and abdomen revealed bilateral hilar and mediastinal lymphadenopathy and para-aortic lymph nodes. Endobronchial biopsy and bronchoalveolar lavage revealed non-specific chronic inflammatory changes. The patient's secondary amyloidosis was presumed to be related to the long standing RHD after exclusion of other causes of secondary amyloidosis. The patient finally died due to heart failure and acute pulmonary edema. Conclusion: Long standing RHD can lead to secondary AA amyloidosis. Keywords: AA Amyloidosis; Kidney Biopsy; Rheumatic Heart Disease; Renal Amyloidosis.     

Biventricular endomyocardiofibrosis associated with renal amyloidosis

Endomyocardiofibrosis is a restrictive cardiomyopathy characterized by fibrotic involvement of the endocardium and adjacent myocardium, and by diastolic dysfunction caused by changes in distensibility making ventricular filling inadequate while preserving the systolic function. Clinically, it appears as heart failure, but etiological symptomatic discernment, suspicion and a clinical examination would be necessary in order to make a correct etiological diagnosis. The case of a patient with biventricular endomyocardial fibrosis associated with renal amyloidosis is presented.     

Mild renal insufficiency is associated with angiographic coronary artery disease in women

BACKGROUND: Mild renal insufficiency is associated with an increased risk for cardiovascular events in women with coronary artery disease (CAD). However, the relationship between mild renal insufficiency and atherosclerotic CAD in women is not known. Methods and Results- Women with chest pain who were referred for coronary angiography in the NHLBI Women's Ischemia Syndrome Evaluation (WISE) study underwent quantitative coronary angiography, blood measurements of creatinine, lipids, and homocysteine, and assessment of CAD risk factors. Fifty-six women had mild renal insufficiency (serum creatinine 1.2 to 1.9 mg/dL), and 728 had normal renal function (creatinine <1.2 mg/dL). Creatinine correlated with angiographic CAD severity score (r=0.11, P<0.004) and maximum coronary artery stenosis (r=0.11, P<0.003). Compared with women with normal renal function, those with mild renal insufficiency were more likely to have significant angiographic CAD (>/=50% diameter stenosis in >/=1 coronary artery) (61% versus 37%; P<0.001) and CAD in multiple vessels (P<0.001 for association) and had greater maximum percent diameter coronary stenosis (59+/-35% versus 38+/-36%; P<0.001). Mild renal insufficiency was associated with significant angiographic CAD independent of age and risk factors (OR=1.9, 95%CI=1.1 to 3.5). After controlling for homocysteine in 509 women, mild renal insufficiency remained predictive of CAD (OR=3.2, 95%CI=1.4 to 7.2). CONCLUSIONS: In women with chest pain, mild renal insufficiency is an independent predictor of significant angiographic CAD. Mildly increased serum creatinine is probably a marker for unmeasured proatherogenic factors.     

Multicentric Castleman's disease associated with renal amyloidosis and pure red cell aplasia

 A 50-year-old man was admitted suffering from severe anemia and renal dysfunction. He had been admitted for the first time at the age of 49, and was diagnosed with multicentric Castleman's disease (MCD) and secondary amyloidosis. At that time, marked erythroid hypoplasia was demonstrated by both aspiration and biopsy of bone marrow. A diagnosis of pure red-cell aplasia (PRCA) was made. Immunosuppressive agents improved his symptoms and laboratory data. We report here a very rare case of PRCA following MCD and amyloidosis, and with reference to the literature, we discuss the relation between MCD and related diseases. Received: February 12, 1998 / Accepted: June 17, 1998     

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.     

Ascites associated with the initial presentation of Crohn''s disease

We report a case of a 23-yr-old patient who was initially admitted with severe Crohn's ileocolitis complicated by a large amount of exudative ascites. There was no evidence of malignancy, portal hypertension, or inflammation in any organ other than the bowel. We present the clinical course and response to treatment and discuss the possible mechanisms by which Crohn's disease might contribute to the development of exudative ascites.     

Adverse antibiotic effects associated with renal insufficiency

We review the English-language literature on antibiotic-associated adverse reactions in patients with renal insufficiency in order to highlight this important but often overlooked clinical problem. Because many adverse reactions to antibiotics are not dependent on renal function, we have attempted to review only those reactions that are believed to be associated with renal insufficiency or that have been reported in patients with impaired renal function. Adverse effects of antibiotics in this setting can be divided into six major categories: neurologic toxicity, coagulopathy, nephrotoxicity, hypoglycemia, hematologic toxicity, and aminoglycoside inactivation by penicillins. Neurologic toxicity can be further divided into central nervous system toxicity consisting primarily of encephalopathy and seizures, ototoxicity, peripheral neuropathy, and neuromuscular blockade/respiratory depression. We explore the factors in uremia that may contribute to the susceptibility of patients with renal insufficiency to the adverse effects of antibiotics. Moreover, we make general recommendations regarding the use of the discussed antibiotics in patients with compromised renal function.     

Pregnancy in women with renal disease and moderate renal insufficiency

Data were gathered on 25 pregnancies in 23 women with moderate renal insufficiency as defined by a serum creatinine level of 1.4 mg/dl or greater prior to or at the onset of pregnancy. Twelve of the women had primary glomerular diseases, five (accounting for seven pregnancies) had interstitial diseases, and six had other renal diseases. In seven women with baseline serum creatinine levels ranging from 1.7 to 2.7 mg/dl, pregnancy was accompanied by a decline in renal function that was believed to be greater than expected from the natural history of the disease. The change ranged from a rise in serum creatinine level of 1.2 mg/dl to a functional decline that required dialysis. In 14 women (16 pregnancies), renal function either remained stable or declined to a degree consistent with the natural history of the disease. In two, the follow-up period was not long enough to judge the effect on the natural history of the disease. In 14 pregnancies, development or worsening of hypertension occurred. In nine, the diastolic blood pressure rose to 110 mm Hg or greater, and delivery was required because of hypertension. Twenty-three of 25 pregnancies ended in live births (92 percent) and 21 babies survived (84 percent). Fourteen of the 23 live births were premature. It is concluded that, in a substantial fraction of women with moderate renal insufficiency, pregnancy is accompanied by a decline in renal function; however, the fetal survival is much better than previously reported.     

Beh?et's disease associated with amyloidosis

A case of Beh?et's disease associated with amyloidosis is reported. A 34 year-old woman was referred to our Department after suffering diarrhea for one month. She had the complete type of Beh?et's disease which began development when she was 12 years old. Diarrhea disappeared after she was admitted to the hospital but stool occult blood was strongly positive. A barium enema revealed the disappearance of haustration in the left colon. Urinalysis was normal. She suffered from cholecystolithiasis, and a cholecystectomy was performed. A histopathological examination revealed amyloid deposits in the stomach, duodenum, large bowel, gallbladder, and kidney. Amyloid was indicated as AA type after treating kidney and colon sections with potassium permanganate. Upon discharge, she was free of symptoms. Up to the present time, 19 cases, including our case of Beh?et's disease with amyloidosis, have been reported. Four cases in Japan have been reported in Japanese. Reviewing the 19 cases, however, all of them developed initially as Beh?et's disease, followed by amyloidosis and there have only been three cases including ours where the type of amyloid was studied. Our finding was identical to the previous two reports; namely identification of the AA type which is common in secondary amyloidosis. These findings lead to a conclusion that amyloidosis associated with Beh?et's disease is secondary.     

Mu-heavy chain disease associated with systemic amyloidosis

mu-heavy chain disease (HCD) is very rare, with only 30 cases reported in the literature. We report a patient with mu-HCD associated with systemic amyloidosis. The diagnosis of mu-HCD was based on findings of mu-heavy chain fragments in the serum, Bence Jones proteinuria and vacuolated plasma cells in the bone marrow. To our knowledge, this is the third case in which systemic amyloidosis led to the patient's death.     

Bleeding diathesis due to vitamin K deficiency in an infant with cystic fibrosis

With the wide implementation of newborn screening for Cystic Fibrosis, infants are being diagnosed in the presymptomatic phase of the disease. Nutritional deficiencies (hypoalbuminemia) and fat soluble vitamins A, D and E deficiencies, due to pancreatic insufficiency and malabsorption, have been reported in the past at the time of diagnosis.1, 2 Rarely, infants with CF present with severe bleeding disorder, secondary to vitamin K deficiency, in the first months of life.³ To our knowledge, this is the first case report illustrating bleeding diathesis in a one month old infant with CF. He was diagnosed by newborn screening and presented with a gastrointestinal bleeding due to vitamin K deficient coagulopathy.     

Pancreatitis associated with Crohn''s disease: a premalignant state for cystadenocarcinoma of pancreas?

We report a 74-yr-old woman with Crohn's disease and acute pancreatitis who, 3 yr after resolution of the latter, developed cystadenocarcinoma of the pancreas. No drug, toxin, or other etiologies including contiguous duodenal involvement were identified as responsible for the pancreatitis, suggesting that pancreatitis was an extraintestinal manifestation of her Crohn's disease. Could Crohn's-associated pancreatitis be a premalignant state for cystadenocarcinoma of the pancreas?