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)

A mutant strain of mice manifesting high proteinuria, wasting syndrome, and kidney glomerular defect was established from the F5 offspring of an interstrain cross of CBA/Nga and RFM/Nga mice. Affected mice had high levels of proteinuria after 40 days of age. The body weight of about 22.6% of affected mice decreased rapidly and they died between 3 and 5 months of age. We learned that this abnormality is controlled by two pairs of autosomal recessive genes; the mutant strain of mice is designated FGS/Nga. The mutant strain has been characterized by high proteinuria and renal lesions with focal sclerosis of glomeruli and tubular atrophy with interstitial nephritis in the kidney resembling the human disease. The FGS/Nga mouse strain is a potential animal model for studying kidney glomerular defect in humans.
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PMID:A new mouse strain manifesting high proteinuria and kidney glomerular defect. 166 45

There is no consensus regarding the specific management of HIV-associated nephrotic syndrome. We report a child whose first manifestation of human immunodeficiency virus type 1 (HIV-1) infection was nephropathy and wasting syndrome associated with profound immunodeficiency. The patient had a dramatic clinical and immunologic response to triple antiretroviral therapy delivered through a gastrostomy tube, with complete resolution of nephrotic syndrome. A 51/2-year-old African-American girl presented with a 2-week history of cough, chest pain, vomiting, loose stools, abdominal distention, anorexia, and fever. In addition, she had recurrent oral thrush. Her weight and height were below the 5th percentile. She was chronically ill, appearing with oropharyngeal thrush and pitting edema in lower extremities. She had scattered rhonchi and decreased breath sounds on both lung bases. Her abdomen was distended and diffusely tender. A chest radiograph showed consolidation of the right upper and left lower lobes with bilateral pleural effusion. Admission laboratories were consistent with nephrotic syndrome. Streptococcus pneumoniae grew from the blood culture and the child responded well to treatment with intravenous ceftriaxone. She was found to be HIV-infected, her CD4(+) cell count was 3 cells/mcL and her plasma HIV-1 RNA was >750 000 copies/mL. A percutaneous gastrostomy tube was placed for supplemental nutrition. She was treated with stavudine, lamivudine, and nelfinavir via gastrostomy tube with good clinical response. Twenty-one months after instituting antiretroviral therapy, her weight and height had increased to the 50th and 10th percentile respectively, and she had complete resolution of her nephrotic syndrome. Her CD4(+) cell count increased to 1116 cells/mcL and her viral load has remained undetectable. HIV-1 associated nephrotic syndrome has been described in children with profound immunodeficiency. The course of untreated HIV-associated nephrotic syndrome is rapid progression to renal failure in up to 40% of the children. Regardless of the presence of renal insufficiency, if untreated, it is uniformly fatal. A modest improvement of HIV-1 associated nephrotic syndrome has been observed in patients treated with zidovudine. Steroid and cyclosporine treatment have resulted in improved renal function but long-term use of immunosuppressive therapy has raised concerns about safety. We have described, to our knowledge, the first child with HIV-associated nephrotic syndrome who had a remarkable clinical, immunologic, and virologic response to triple-drug combination therapy given by gastrostomy tube, with complete resolution of proteinuria and normalization of the serum albumin. She also had a striking improvement in weight, height, and quality-of-life. Whether the presence of a gastrostomy tube contributed to the excellent response because of improved compliance is unknown, but warrants systematic evaluation.
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PMID:Resolution of HIV-associated nephrotic syndrome with highly active antiretroviral therapy delivered by gastrostomy tube. 1058 95

This paper documents the salient clinical and pathological features of porcine dermatitis and nephropathy syndrome (PDNS) in 96 pigs submitted from 55 units in the UK from 1993 to 1998. This series of cases pre-dated the emergence of post-weaning multisystemic wasting syndrome (PMWS) in the UK. The morbidity during outbreaks was 1% or less. Affected pigs ranged from 14 to 70 kg in weight and most died after a short clinical illness. Fifty-five pigs had multifocal or coalescing erythematous skin lesions, some progressing to dermal necrosis. Biochemistry showed raised serum urea, creatinine and gamma globulin levels accompanied by proteinuria. All cases showed bilateral renal enlargement with petechiae throughout the cortices. Microscopically these renal lesions ranged in chronology from acute necrotizing glomerulitis and vasculitis with multiple hyaline casts in renal tubules to chronic glomerular sclerosis with interstitial inflammation and fibrosis. Haemorrhagic dermatitis when present was associated with necrotizing vasculitis in the dermal vessels. Vasculitis was sometimes detected in other tissues including subcutis, lymph nodes, spleen, liver, joint synovial membrane, gastric and intestinal submucosa or serosa and meninges but its frequency and distribution varied considerably in individual pigs. Immunostaining showed deposits of IgG and IgM in damaged glomeruli, renal casts and skin lesions. The aetiology and pathogenesis of the condition remain unknown but the histopathological and immunological findings suggest a systemic immune-complex disorder resulting in vasculitis with particular predilection for kidney and skin.
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PMID:Porcine dermatitis and nephropathy syndrome. clinical and pathological features of cases in the United Kingdom (1993-1998). 1245 Jan 93