Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-two cases of multiple myeloma were seen in the Department of Internal Medicine, Tikur Anbessa (Black Lion) Hospital, a teaching and referral hospital in Addis Abeba, Ethiopia, from January 1983 to December 1990. The age range was 38 to 76 (mean +/- SD = 51.5 +/- 12.2) years; a third were in the fifth decade. The male:female ratio was 1.75:1. The common clinical findings were bone pain in 20 (91%), bone tenderness in 15 (68%), anaemia in 14 (64%) and spinal cord compression in 8 (36%). The erythrocyte sedimentation rate (ESR) was raised in 21. Serum protein was raised in 17 (77%) and hyperglobulinaemia was seen in 20 (91%). Serum uric acid, blood urea nitrogen (BUN) and calcium were elevated in 10, 8 and 5 patients respectively, Bence-Jones proteinuria and albuminuria were each found in 9 patients. All patients had radiological abnormalities; 9 had a combination of lytic lesions, osteoporosis and pathological fractures (41%). Ten patients presented in clinical stage III. Four patients are being followed after 3-84 (median 40.5) months; eight were lost to follow-up 1-8 (median 2.0) months after diagnosis. Ten patients have died after 1-55 (median 11) months. Multiple myeloma is not uncommon in Ethiopians. Except for a lower age at presentation, the clinical, haematological, biochemical, and radiological findings, and the response to therapy, are similar to those reported elsewhere.
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PMID:Multiple myeloma in Ethiopians: analysis of 22 cases. 139 16

This report deals with an unusual case of primary macroglobulinemia with hypercalcemia, chronic renal failure and systemic amyloidosis. In May 1990, a 63-year-old male was transferred to our hospital because of hypercalcemia (13.5 mg/dl) and renal failure. Clinical examinations showed anemia, macroglossia, lymph node swellings and hepatomegaly. Laboratory findings included Bence-Jones (kappa type) proteinuria (0.8 g/day), a monoclonal gammopathy of the IgM-kappa type (2.8 g/dl), a proliferation of lymphoid cells in the peripheral blood (5%) and the bone marrow (59.6%), and lymphomatous involvement of an inguinal lymph node. Serum creatinine concentration was 8.5 mg/dl. The serum levels of parathormone and vitamin D3 metabolites were normal. The roentgenogram of bones showed a compression fracture of the lumbar spine and systemic osteoporosis. The treatment included eel calcitonin, prednisolone and the CHOP regimen, followed by hemodialysis and plasmapheresis. The serum level of IgM increased to 4.6 g/dl. The patient died three months later and postmortem examination demonstrated marked systemic amyloidosis.
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PMID:[Primary macroglobulinemia with hypercalcemia, renal failure and systemic amyloidosis]. 146 88

Multiple myeloma associated primary biliary cirrhosis (PBC) is very rare and only two cases have been reported. In this paper, we reported the first case of male patient with asymptomatic PBC and multiple myeloma. A 66 year-old Japanese male was referred to our hospital for the further examination of a monoclonal gammopathy. He was diagnosed of multiple myeloma (IgG-lambda type) because of 2.3 g/day of Bence Jones proteinuria (lambda type), 3,401 mg/dl of monoclonal IgG (lambda type), 12.8% of bone marrow plasmocytosis and generalized osteoporosis. The alkaline phosphatase was 314 mU/ml and serum IgM level (polyclonal) 937 mg/dl. The patient was started on intermittent courses of melphalan and prednisolone, achieving transient improvement. After two years, hepatosplenomegaly developed gradually and the levels of serum ALP elevated increasingly. At that time, relevant investigation results were: serum ALP 663 mU/ml, serum IgG 4,144 mg/dl, serum IgM 823 mg/dl, positive anti-mitochondrial antibody test x 320. The liver biopsy showed chronic nonsuppurative destructive cholangitis. PBC (stage 1-2 according to Sheuer's criteria) associated with multiple myeloma was diagnosed. A pathogenetic relationship such as loss of immunoregulatory function could be speculated although the simultaneous occurrence of PBC and multiple myeloma could be coincidental.
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PMID:[Multiple myeloma of IgG-lambda type associated with asymptomatic primary biliary cirrhosis]. 251 98

A 44-year-old Chinese male with a 7-year history of diabetes developed spontaneous fractures affecting the femur and distal tibia and fibula within a period of 4 months. Spontaneous rib fractures were also present. There was additional evidence of extensive tissue damage with retinopathy, proteinuria, necrobiosis lipoidica diabeticorum, peripheral neuropathy and autonomic neuropathy. Investigation confirmed the presence of generalised osteoporosis and showed no evidence of other metabolic bone disease or abnormal vitamin D metabolism. Mild hypogonadism was also present and investigation suggested a disturbance of hypothalamic-pituitary control of gonadal function. It is suggested that the severe generalised osteoporosis resulted from poorly controlled diabetes with a possible additional contribution from androgen deficiency secondary to the diabetes.
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PMID:Fractures due to severe generalised osteoporosis in a 44-year-old male with diabetes mellitus. 277 56

A 7-year-old boy was referred to the children's hospital because of gross oedema and tiredness. Massive proteinuria was found and the condition was diagnosed as a childhood nephrotic syndrome. Concomitantly, pathologically low levels of serum alkaline phosphatase were recorded, and this, together with generalized osteoporosis and premature synostosis of cranial sutures, led to a second diagnosis: hypophosphatasia. The patient's family history further confirmed this condition of a heritable defect of metabolism. Dental inspection revealed very carious teeth with characteristically enlarged pulp chambers in molars. Histological examination of an extracted tooth revealed an unusually wide zone of predentine with some other dentinal irregularities. No cement layer was found. The skeletal age and exfoliation of primary teeth, however, were normal, unlike most reported cases of hypophosphatasia. The patient's renal disease was treated mainly with corticosteroids. There is no treatment for hypophosphatasia.
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PMID:Cranial manifestations of hypophosphatasia in childhood nephrotic syndrome. 643 Aug 33

The authors present a case of a 15-year-old girl with Leber's congenital amaurosis with associated nephronophthisis. The main findings in this case are: congenital blindness; enophthalmos; photophobia; nystagmus; keratoconus; cataracts; pigmentary degeneration in the fundus of both eyes; progressive uremia with absence of hematuria, proteinuria, pyuria, and glycosuria; low urinary density, normal lipidic profile; osteoporosis; absence of edema; polydipsia; polyuria; and a history of consanguinity between her parents. Tranmission of this entity allows an autosomal recessive pattern.
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PMID:Leber's congenital amaurosis with associated nephronophthisis. 739 8

A patient with primary amyloidosis had infiltration of liver, bone and bone marrow and a nephrotic syndrome with massive proteinuria. In addition, there was evidence for a plasma cell dyscrasia with an increased number of plasma cells and other lymphoreticular cell elements in the bone marrow and an impairment of immunoglobulin synthesis without M-gradient. The course of disease was observed over a period of five years. The patient was treated intermittently with a combination of penicillamine, melphalan, fluocortolone and fluoxymesterone. Under treatment the nephrotic syndrome disappeared and liver size decreased. Hemopoiesis remained sufficient but bone marrow infiltration by amyloid did not respond to therapy and massive osteoporosis developed. Comparison of our with other reported cases treated with the same regime suggests that response to this treatment may be influenced by factors such as sex and clinical manifestation of the disease.
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PMID:[Primary amyloidosis with nephrotic syndrome: 5 year follow-up under polychemotherapy. A case report (author's transl)]. 745 5

Analysis of 2,547 cases of multiple myeloma (MM) reported in China in 1980s showed that the clinical manifestations are characterized by multipliey. High misdiagnosis rate (69%) and multiple complications. While the monoclonal protein had more than 25 immunological type, IgG myeloma was the commonest (43.1%). Light chain subgroup trended to have higher incidence of renal damage (76.9%). Plasma cell leukemia eventually developed in 30 cases. In order to improve diagnosis and avoid misdiagnosis, the key points are 1, to better the recognition of clinical features of MM. 2. patient should receive urine Bence-Jones protein, immunoglobulins, immunoeletrophresis, bone X-ray and multiple site bone marrow puncture whenever one of such manifestations as unexplained anemia, skeletal pain, proteinuria, elevation of ESR, hyperviscosity syndrome, hypercalcemia, hyperuricemia, elevation of alkaline phosphatase, pathological fractures and diffuse osteoporosis. 3. immuno-binding electrophoresis and immunofluorence antibody detection should be done for suspected cases with normal immunoglobulin level.
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PMID:[On the diagnosis of multiple myeloma an analysis of 2,547 domestic cases]. 765 87

A 19-year old caucasian patient suffered from ulceration and scaring of his fingers since age two. During childhood, fibrosing contractures on the phalanges developed. Since puberty, his eunuch-like stature, bird-like face and exophthalmos had become under obvious. His hair was extraordinarily fine; his pubic hair rare. Radiological examination revealed ankylosis and osteoporosis of the phalanges and carpals. Angiography showed occlusion of multiple digital and interdigital arteries. Furthermore, a bilateral posterior cataract, restrictive respiratory disease, impaired glucose tolerance, hyperuricemia, proteinuria and primary hypogonadism were diagnosed. These findings are characteristic for the premature aging syndromes. The cardial symptoms of tall stature, bird-like face, pseudoexophthalmos, skleroderma-like and poikiloderma-like cutaneous lesions, scarce hair growth, early diabetes mellitus and arteriosclerosis led to the diagnosis of metageria.
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PMID:[Metageria--clinical manifestations of a premature aging syndrome]. 941 Aug 52

Aggressive immunosuppressive therapy should be considered for patients with proliferative lupus nephritis as the risk for progression to end stage renal disease is high. Intermittent intravenous cyclophosphamide therapy improves renal survival; longer duration of therapy is associated with fewer relapse of nephritis and decreased risk of diminished renal function. While azathioprine therapy does not differ statistically from steroids alone in prolonging renal survival, this therapy may be considered in patients with few risk factors for progression to renal insufficiency. Methylprednisolone as a single therapy does not prolong renal survival compared with regimens including cyclophosphamide. Plasmapheresis remains under study but has not shown additional benefit in treatment of severe lupus nephritis. The potential roles for cyclosporin A and mycophenylate mofetil in the therapy of proliferative lupus nephritis remain to be defined. Supportive care including rigorous control of hypertension, consideration of angiotensin receptor inhibition or blockade to reduce proteinuria and prolong renal function, control of hyperlipidemia, prevention of osteoporosis, and prevention of pregnancy remain important clinical goals. Current research efforts focus on genetic and socioeconomic factors involved in racial differences in expression of lupus nephritis, hormonal manipulation to preserve gonadal function during cyclophosphamide therapy, and the potential impact on lupus activity of estrogen-containing oral contraceptives or postmenopausal hormone replacement therapy.
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PMID:Immunosuppressive therapy of lupus nephritis. 988 1


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