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Biomedical subjects

K Andrassy

Publications and source records attributed to K Andrassy.

At least 127 records · Page 7Linked to original sources
PubMed1989

Acrorenal syndrome in an adult--presentation with proteinuria, hypertension, and glomerular lesions.

Acrorenal syndrome is characterized by central longitudinal axis defects of the limbs, ie, split hand and/or foot. Associated renal lesions described so far comprise agenesis, bilateral hypoplasia (originally diagnosed as oligomeganephronia), and duplication abnormalities. The case of a 29-year-old patient with split hand resulting from bilateral aplasia of the third phalanges associated with dysplasia of the third and fourth metacarpals is reported. In addition, the following lesions were noted: hypoplasia of the middle phalanx of the fifth toe, arched palate, pectus excavatum, hypoplastic mammilae, scoliosis, and congenital hip dislocation. The patient presented with hypertension, modest reduction of glomerular filtration, proteinuria, microhematuria, cylindruria, and moderate harmonic hypoplasia of the right kidney on angiography. Glomeruli had no immune deposits on immunohistology. Light microscopy showed widening of the mesangial axis, focal segmental glomerular sclerosis, and renal interstitial fibrosis with occasional foam cells. This case shows that the spectrum of renal abnormalities in the acrorenal syndrome is wider than previously noted.

Abnormalities, Multiple↗
PubMed1989

Detection and clinical implication of anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis and rapidly progressive glomerulonephritis.

The specificity of anti-neutrophil cytoplasm antibodies (ACPA/ANCA) was investigated in patients suffering from Wegener's granulomatosis (WG), various forms of systemic diseases including vasculitides (non-WG), and different types of biopsy-proven glomerulonephritides. In particular, the diagnostic significance of ACPA/ANCA was assessed in patients affected by rapidly progressive glomerulonephritis with and without systemic manifestations. From 25 patients with active Wegener's granulomatosis 22 showed the classical diffuse finely granular cytoplasmic staining of neutrophils as did 4/31 patients with idiopathic rapidly progressive glomerulonephritis. One patient with biopsy confirmed Wegener's granulomatosis, six patients with microscopic polyarteritis and 3/31 patients with idiopathic rapidly progressive glomerulonephritis showed a focal cytoplasmic staining exhibiting a rosette-like pattern. One further patient with Wegener's granulomatosis displayed a prominent fluorescence of the outer nuclear membrane as well as a punctate-diffuse nuclear staining resembling that of granulocyte specific antibodies. Another patient with active Wegener's granulomatosis did not react in the ACPA/ANCA-test. These findings demonstrate different staining patterns of neutrophils which are related to different clinical entities within the spectrum of small vessel vasculitis. Moreover, they point out that different antigens are involved in the various types of vasculitis. In classical cases of Wegener's granulomatosis, but not in other forms of vasculitis, the titer of ACPA/ANCA showed a close relationship to the number of organs involved.

Adolescent↗
PubMed1989

Treatment of granulomatous disorders of the nose and paranasal sinuses.

The granulomatous disorders discussed in this review are Wegener's granulomatosis (WG), lymphomatoid granulomatosis (polymorphic reticulosis) and "idiopathic midline granuloma". The treatment of choice of WG is combined therapy with corticosteroids and cyclophosphamide. Severely ill patients may be treated with intravenous bolus infusions of cyclophosphamide. Otherwise oral administration is used. Therapy must be adjusted according to leucocyte and thrombocyte counts. After clinical remission cyclophosphamide must be continued for at least a year under hydration sufficient to cause nycturia in order to protect the bladder mucosa. Corticosteroids can be withdrawn 9-10 months after clinical remission. Relapse of WG can be identified by clinical and laboratory (ESR, CRP, HB, urinary sediment) findings, including detection of anti-neutrophil cytoplasm antibodies (ANCA). Alternate treatment with azathioprine or trimethoprim/sulfamethoxazole may be used in patients with localized or smoldering disease. Furthermore trimethoprim/sulfamethoxazole may be used as adjunctive treatment. Lymphomatoid granulomatosis appears to be a T-cell lymphoma and should be treated aggressively with combination cytotoxic therapy and irradiation of localized manifestations. "Idiopathic midline granuloma" does not seem to exist but appears to be either WG or lymphomatoid granulomatosis when repeated biopsies are examined with monoclonal antibodies and/or serum examined for ANCA.

Autoimmune Diseases↗
PubMed1988

Human pharmacological studies of a defined low molecular weight heparin fraction (Fragmin) evidence for a simultaneous inhibition of factor Xa and IIa (thrombin).

The effect of a low molecular weight heparin (Fragmin) (20-80 U/kg) was studied in 10 healthy male volunteers after intravenous administration. Clotting abnormalities are extensive shortly after injection, lasting for 1-2 hours depending on heparin dosage. Dilute thrombin time, showing a close correlation to anti-F-Xa activity, is as sensitive as anti-F-Xa. The parallel time course of dilute thrombin time and anti-F-Xa activity clearly demonstrates that LMWH has an effect on F-IIa lasting as long as that of F-Xa. After subcutaneous administration clotting abnormalities are only slight with 40-60 U/kg, in contrast to the intravenous study. The platelet system remains unchanged.

Adult↗
PubMed1988

Mechanism of cephalosporin-induced hypoprothrombinemia: relation to cephalosporin side chain, vitamin K metabolism, and vitamin K status.

The mechanism of cephalosporin-induced hypoprothrombinemia has been investigated in hospitalized patients, with respect to cephalosporin structure, vitamin K metabolism, and vitamin K status. Cephalosporins containing side chains of N-methylthiotetrazole (latamoxef, cefmenoxime, cefoperazone, cefotetan, cefamandole) or methyl-thiadiazole (cefazolin) all caused the transient plasma appearance of vitamin K1 2,3-epoxide in response to a 10-mg intravenous dose of vitamin K1, whereas two cephalosporins without a heterocyclic side chain (cefotaxime and cefoxitin) did not. The plasma accumulation of vitamin K1 2,3-epoxide was qualitatively similar to, but quantitatively less than, that produced by the oral anticoagulant phenprocoumon. Patients eating normally had plasma vitamin K1 concentrations (176 to 1184 pg/mL) that were within the normal range (150 to 1550 pg/mL) and their clotting tests remained consistently normal for all antibiotics tested. Patients on total parenteral nutrition had lower plasma vitamin K1 concentrations (50 to 790 pg/mL) but normal clotting before starting antibiotic therapy. Of 19 parenterally fed patients, all seven treated with latamoxef developed hypoprothrombinemia, PIVKA-II and a decrease of protein C within four days whereas 12 patients treated with cefotaxime or cefoxitin showed no clotting changes. Latamoxef-associated hypoprothrombinemia was readily reversible by 1 mg of vitamin K1 given intravenously, but hypoprothrombinemia and sub-normal plasma vitamin K1 could recur within two to three days. The data suggest that NMTT-cephalosporins are inhibitors of hepatic vitamin K epoxide reductase and that a lower nutritional-vitamin K status predisposes to hypoprothrombinemia.

Adult↗
PubMed1988

The spectrum of renal involvement in epidermolysis bullosa dystrophica hereditaria: report of two cases.

Epidermolysis bullosa dystrophica Hallopeau-Siemens (EBDH) is one of the most severe inherited epidermolyses, a group of mechanobullous dermatological disorders. We observed two patients presenting with a severely multilating type of EBDH who developed biopsy-proven renal disease, which substantially altered the evolution and pathogenesis of their disease. In a boy, chronic postinfectious glomerulonephritis developed, most probably due to recurring superinfections of bullous skin lesions. He also experienced acute oliguric renal failure due to severe diarrhea during exacerbation of EBDH. A female patient developed a nephrotic syndrome due to secondary amyloidosis. Hypoalbuminemia caused further fluid losses through bullous skin lesions, aggravating intravascular hypovolemia and leading to rapid renal failure secondary to bilateral renal vein thrombosis. The study shows that, although rare, renal complications may alter the natural course of EBDH.

Adolescent↗
PubMed1987

[Acute granulomatous interstitial nephritis with iritis. Possible induction by non-steroidal antiphlogistics].

A 49-year old woman developed non-oliguric acute renal failure accompanied by bilateral acute anterior uveitis, following a three weeks' period of lethargy, anorexia and temporary fever. Kidney biopsy revealed acute interstitial nephritis with interstitial infiltrations of lymphocytes and monocytes, as well as multiple perivascular epithelioid granulomas. A substantial improvement of renal function was achieved under treatment with systemic corticosteroids. The uveitis resolved completely under additional topical treatment. During a follow-up period of 9 months, there has been no relapse of nephritis or uveitis. The disease of this patient resembles the so-called TINU syndrome of unknown aetiology. Remarkable features of the present case are the histological diagnosis of granulomatous acute interstitial nephritis in the absence of systemic granulomatous disease, as well as a possible association with the administration of non-steroidal antiinflammatory drugs.

Acute Disease↗
PubMed1987

Familial glomerulonephritis.

Between 1970 and 1984, the diagnosis of glomerulonephritis was made in 860 patients on the basis of a nephritic sediment and/or renal biopsy; of these patients, 86 (10%) had at least one first-degree relative with glomerulonephritis. These patients originated from 45 families and 1674 family members were screened; 172 had glomerulonephritis, of whom 101 could be classified. The diagnostic breakdown of the 101 patients showed that 50.5% had classical Alport's syndrome; 21.8% had atypical forms; 17.8% had familial IgA glomerulonephritis; 1.9% had focal segmental glomerulosclerosis with Wolff-Parkinson-White syndrome; and 7.9% had benign familial haematuria. The proportion of patients with glomerulonephritis who had familial disease was higher than expected. The family history is an important point to consider in the examination of patients with glomerulonephritis.

Adolescent↗
PubMed1987

Hemostasis in patients with normal and impaired renal function under treatment with cefodizime.

Ten patients (two with normal, eight with impaired renal function) on their usual diet were treated with cefodizime (HR 221) for seven days. The dosage was 4 g/day, adapted to renal function as appropriate. Platelet function, plasma coagulation and vitamin K metabolism were investigated before and on day 7 of therapy. Platelet function and plasma coagulation remained unchanged, regardless of the size of the serum antibiotic trough levels, in both normal and impaired renal function. Vitamin K1 metabolism remained unaffected, since no increase in vitamin K1 2,3 epoxide in the circulation was observed during the therapy. Cefodizime (HR 221), a parenteral aminothiazole cephalosporin, does not affect hemostasis.

Adult↗
PubMed1987

Hemostasis and thromboembolism in children with nephrotic syndrome: differences from adults.

Eleven of 204 children with nephrotic syndrome had thrombotic complications: arterial thrombosis in five, venous thrombosis in four, and pulmonary embolism in two. Fifty-one episodes of thromboembolism were recognized in 116 adult patients with nephrotic syndrome. Despite the lower incidence, thromboembolic complications tended to be more severe in children. In vitro indices of hemostasis and clinical evidence of thromboembolic complications were compared in children and adults. Antithrombin III concentrations and activities were abnormal in seven of 10 children, but in only two of 32 adults. In both groups, alpha 2-macroglobulin was elevated, but more markedly so in children. No evidence for circulating granulocyte-derived proteases (elastase/antielastase complexes) was noted in either group. Protein C was significantly elevated in children with nephrotic syndrome, but was normal in adults. Children also differed from adults with nephrotic syndrome in laboratory evidence of subthreshold disseminated intravascular coagulation (i.e., elevated soluble fibrinogen monomeric complexes and fibrin degradation products) and indicators of in vivo platelet activation (elevated beta-thromboglobulin). The more severe coagulation abnormalities in children may be linked to the more pronounced hypoalbuminemia.

Adolescent↗
PubMed1987

Mesangial IgA nephropathy and idiopathic nephrotic syndrome.

A 17-year-old male presented with nephrotic syndrome associated with microscopic hematuria. Renal biopsy showed only minor glomerular abnormalities (light microscopy). Immunohistology demonstrated strong mesangial deposition of IgA. Electronmicroscopy disclosed widespread effacement of foot processes in combination with isolated osmiophilic mesangial deposits. The patient responded to standard corticosteroid therapy with complete disappearance of proteinuria. Microscopic hematuria, however, persisted. Five months after steroid therapy was stopped, the nephrotic syndrome relapsed. It was again steroid-responsive with persisting microhematuria. From clinical and morphological data we conclude that the patient has concomitant idiopathic nephrotic syndrome (minimal change glomerulonephritis) and mesangial IgA glomerulonephritis. The simultaneous presence of these two diseases may give some hint as to their pathogenesis. In both, abnormalities in T cell regulation have been found. If these were indeed involved in the pathogenesis of the two glomerular diseases, a higher than expected probability for the two entities to coexist in the same patient is to be expected.

Adolescent↗
PubMed1986

[Acute kidney failure caused by Hantaan virus. Case report from the West Germany].

A man aged 41 years who spent his leisure time in a marshy region infested with brown rats developed an infectious disease with high fever associated with diarrhoea, arthralgia and lumbago. In the course of the disease an acute renal failure occurred not requiring dialysis which spontaneously receded. Histology revealed signs of an acute renal failure with sparse interstitial round-cell infiltrations. Serological investigations showed a rising titre against Hantaan virus, Strain CG 1820 (1:1024). According to experience in neighbouring countries, it may be assumed that quite a number of cases of spontaneous acute renal failure occurring in the Federal Republic of Germany is caused by Hantaan virus.

Acute Kidney Injury↗