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Biomedical subjects

J Briner

Publications and source records attributed to J Briner.

At least 91 records · Page 5Linked to original sources
PubMed1985

[Acquired immunodeficiency syndrome in a child of Swiss origin whose mother died from AIDS].

Report of a now 2 8/12-year-old girl, who presented at the age of 8 months with chronic progressive pneumonia, mucocutaneous candidiasis, diarrhea, failure to thrive and a non-progressive paraplegia. The child's mother presented AIDS with pneumocystis carinii pneumonia and progressive general paralysis one year after the beginning of the child's disease and died within a few months. Additional findings in the child include lymphopenia, hyperimmunoglobulinemia, cutaneous anergy and an abnormal T helper/T suppressor cell ratio. HTLV-III antibodies were positive (ELISA and Western blot virus strip RIA). Prophylactic treatment with Co-trimoxazole relieved pulmonary infections but failure to thrive remained unchanged in spite of a continuous nutritional support. A vertical mode of transmission of AIDS from mother to child seems very probable.

Acquired Immunodeficiency Syndrome↗
PubMed1984

[Recurrence of the original disease in the transplanted kidney].

Recurrence of the original disease in the transplanted kidney is observed in 5.6%-9.3% of the patients. However, the clinical significance of recurrence is often minor. Diagnosis is easy in diseases with specific renal lesions, e.g., in dense deposit disease and IgA-nephropathy, but may be difficult if such a marker is missing. Recurrence is of special clinical importance in the following conditions: Membranoproliferative GN type I (in 33%, often severe) and type II (= dense deposit disease, recurrence in 90%, often minor), focal segmental glomerulosclerosis (in 48% of patients with a rapid course (less than 3 years) and in 12% of patients with a longer duration of the original disease; often severe), membranous nephropathy (recurrence rather rare, but often serious), and primary hyperoxaluria (in 100%). Mesangial IgA deposits recur in half of the patients with IgA-nephropathy and anaphylactoid purpura, but clinical findings are often minimal. Recurrence in anti-GBM-nephritis and SLE is rare. The study of recurrence may contribute to a better understanding of many renal diseases.

Anti-Glomerular Basement Membrane Disease↗
PubMed1984

[Glomerular changes in interstitial kidney diseases].

49 kidney specimens of patients with primary interstitial kidney disease (hydronephrosis in 12, tubulo-interstitial nephritis in 13, analgesic nephropathy in 10, adult polycystic renal disease in 9, oxalosis in 4, and renal dysplasia in 1) were examined for glomerular lesions. Light microscopic investigation showed severe focally and segmentally accentuated glomerular changes in 13 cases (27%). Lesions resembling focal sclerosis were found in 11 cases and focal-segmental mesangial proliferation in 2 cases. The results reveal that glomerular lesions occur frequently in primary interstitial kidney disease and can be found in all of the above-mentioned diagnostic entities.

Adolescent↗
PubMed1984

Prenatal ultrasonographic diagnosis of holoprosencephaly. Two cases of cebocephaly and two of cyclopia.

Four instances of ultrasonographic detection of holoprosencephaly, one during the second and three during the third trimester of gestation, are reported. Horizontal scans of the skull showed absence of midline structures, communication between the lateral ventricles and reduction of the brain mass, particularly anteriorly. Sagittal scans revealed absence of the nose and presence of a proboscis in the fetuses with cyclopia and hypoplasia of the nose in one cebocephalic fetus. Hypotelorism or a single midline orbit are further pathognomonic features detectable by ultrasound, but only the latter was identified in one of the cyclopic fetuses. Facultative findings of holoprosencephaly identifiable by ultrasound, but absent in our cases, are premaxillary agenesis and bilateral cleft lip and maxilla. The characteristic pattern of ultrasonographic findings in holoprosencephaly should allow an unequivocal prenatal diagnosis during the second and third trimester.

Adult↗
PubMed1983

[Glomerular changes in reflux nephropathy].

Two patients with bilateral vesicoureteral reflux are presented. In both cases renal insufficiency progressed despite surgical correction of the vesicoureteral reflux and proteinuria persisted. Biopsy specimens from both patients revealed interstitial damage and segmental and focal glomerulosclerosis. These glomerular lesions in association with reflux nephropathy may be an important cause of renal function deterioration.

Adult↗
PubMed1983

Antley-Bixler syndrome in sisters: a term newborn and a prenatally diagnosed fetus.

Sisters with the Antley-Bixler syndrome are reported herein. The first infant died at 14 days of respiratory failure and the following findings characteristic of the syndrome: craniosynostosis of coronal and lambdoid sutures, brachycephaly, frontal bossing, severe midface hypoplasia with proptosis and choanal stenosis/atresia, humero-radial synostosis, medial bowing of ulnae, long slender fingers with camptodactyly, narrow iliac wings, anterior bowing of femora, cardiac and renal malformations. Unlike two previously published cases, she did not have connatal fractures, but she had vaginal atresia. Ultrasound examination in a subsequent pregnancy showed immobility at the elbows of the fetus, humero-radial synostosis, medial bowing of ulnae, and long hands and fingers. The fetus also had mild bowing of femora. The pregnancy was terminated in the 21st week and the findings were confirmed by clinical and radiologic examinations. Additional autopsy findings included cardiac and renal malformations. The Antley-Bixler syndrome is a recognizable malformation syndrome with a probable autosomal recessive mode of inheritance, which can be diagnosed in the fetus by midtrimester ultrasound examination.

Abnormalities, Multiple↗
PubMed1982

[Plasma exchange in progressive lupus nephritis].

The clinical course in 8 patients with systemic lupus erythematosus (SLE) complicated by sever lupus nephritis (LN) with progressive renal insufficiency was investigated to evaluate the therapeutic role of plasma exchange in this disease. In 6 patients treated with corticosteroids and immunosuppressive drugs, only a creatinine level exceeding 4.0 mg% resulted in hemodialysis or death. In 2 patients under additional plasma exchange the creatinine value fell below 4.0 mg% and was stabilized at that level over a now lengthy period of time. It cannot yet be concluded that additional plasma exchange definitely prevents hemodialysis. However, stabilization of renal function could optimize conventional therapy, thereby limiting side effects as well as risk of infection.

Adolescent↗
PubMed1982

[Malignant tumors in bearers of kidney grafts in immunosuppressive therapy].

Out of 420 kidney transplant recipients at the University Hospital, Zurich, operated on between 1964 and 1978, 23 developed one or more malignant tumors. This corresponds to 5.8% of all patients. They included 8 cases of malignant lymphoma (non-Hodgkin), one of subacute myeloic leukemia, one of acute lymphatic leukemia, 6 skin cancers and 9 cancers of internal organs. Thirteen patients died, a figure corresponding to 8% of all deaths after kidney transplantation. Nine of the 10 patients with lymphoma and leukemia died, in 5 cases despite therapy. The response to therapy (radiotherapy and/or chemotherapy) was much poorer than in other patients with comparable tumors and in some patients completely absent. The only surviving patient (malignant lymphoma of the small bowel and the retroperitoneum) was treated by a combination of surgery, radiotherapy and chemotherapy and has had a symptom-free follow-up time of 3 1/2 years. The 6 skin cancers (4 of the spinocellular type) were excised. Recurrences were not noted. The visceral carcinomas (2 breast cancers, 1 carcinoma respectively of the pancreas, the rectum, the liver, the kidneys, the renal pelvis, and the urinary bladder, and one seminoma) were treated by generally accepted surgical principles as far as treatment of the patients was possible. The breast cancer and seminoma patients have survived thus far without recurrences or metastases.

Adult↗
PubMed1982

[Clinical aspects of neuronal intestinal dysplasia (author's transl)].

Neuronal intestinal dysplasia is histologically and histochemically well defined. The resection of the neuronal dysplastic segment is usually recommended in the literature as being curative. Whether a resection is necessary in every case remains to be debated. The case history of a male patient with Hirschsprung's disease and proximal intestinal neuronal dysplasia of the entire colon is presented. After manometrical, radiological and functional examination the aganglionic portion of the gut only has been resected and the neuronal dysplastic part has been left in situ. Several months later the patient is well. It seems possible to avoid in some selected cases a subtotal or total colectomy.

Colectomy↗
PubMed1981

[Incidence and clinical significance of thromboses and thrombo-embolic complications in nephrotic syndrome patients].

Chest X-ray, pulmonary isotopic photoscanning. Doppler sonography of iliac and femoral veins, inferior venacavagram and phlebography of the renal veins have been performed in 26 patients with nephrotic syndrome in order to determine the frequency and localization of thromboses and thromboembolic complications in these patients. 7 of 26 patients (26.9%) exhibited thromboses or thromboembolic complications (2 left sided renal vein thromboses, 1 right sided ileofemoral thrombosis and 1 bilateral ileofemoral thrombosis with occlusion of the vena cava inferior). In one patient renal vein thrombosis caused pulmonary embolism. In 3 other cases with life-threatening severe episodes of pulmonary embolism, the origin of the emboli could not be detected. Serum albumin level was found to be an appropriate parameter to assess the risk of thrombosis development in these patients. In 7 patients with thromboses or thromboembolic complications the serum albumin level was below 2 g/dl, whereas in 19 patients without these complications the serum albumin level was, with one exception, higher than 2 g/dl (1.5 +/- 0.3 g/dl vs. 2.6 +/- 0.5 g/dl; p less than 0.001). The possible pathophysiologic mechanisms for this observation are discussed. Our results help to identify the population of nephrotic patients who are most likely to experience thromboembolic disease. It therefore would be justifiable to carry out a prospective controlled study examining the question, whether this group of patients with benefit from prophylactic anticoagulation.

Adult↗
PubMed1981

Platelet aggregation and beta-thromboglobulin levels in nephrotic patients with and without thrombosis.

Platelet aggregation and beta-thromboglobulin levels were studied in 17 patients with the nephrotic syndrome. Thrombosis or thromboembolic complications occurred in 4 of these patients with serum albumin levels below 2 g/100 ml. Pathologic platelet aggregation assessed by estimating alpha 2-angle values derived from platelet aggregation curves was seen in the 4 patients with thromboembolic complications (alpha 2-angle 69.5 degrees +/- 10.1 degrees), whereas patients without thrombosis showed normal alpha 2-angle values (less than 30 degrees) with only one exception. In addition, patients with thromboembolic complications demonstrated significantly elevated beta-thromboglobulin levels, when compared with those not having thrombosis or thromboembolic complications (76.8 +/- 14.3 ng/ml vs 44.8 +/- 8.6 ng/ml, P less than 0.001). The decrease in serum albumin concentration showed an inverse relationship with both, alpha 2-angle values (r = -0.82, P less than 0.001) and beta-thromboglobulin levels (r = -0.83, P less than 0.001) indicating a regulatory role of serum albumin in platelet aggregation. We conclude, that altered platelet aggregation as well as hypercoagulability may be involved in the pathogenesis of thrombosis and thromboembolic complications in the course of the nephrotic syndrome.

Adult↗
PubMed1980

[Value of renal biopsy in deteriorating graft function following renal allotransplantation].

The value of renal transplant biopsy in determining the cause of deteriorating graft function was evaluated by comparing morphologic diagnoses with clinical findings and ultimate course in a series of 104 biopsies. Transplant biopsy was a valuable tool in diagnosing acute and chronic transplant rejection. It was superior to clinical parameters, mainly in differentiating vascular transplant rejection (which is usually associated with poor prognosis) from cellular rejection (associated with a much better prognosis). Biopsy was less effective in detecting deteriorating function due to ureteral stenosis. Transplant biopsy was essential in determining the case of proteinuria, thanks to its ability to differentiate the various types of glomerular transplant lesions.

Biopsy↗
PubMed1980

[Loss of 2 kidney grafts due to recurrence of type II membrano-proliferative glomerulonephritis].

Recurrent glomerulonephritis after renal transplantation is seen in a very high percentage of patients with dense "deposit" disease (= membrano-proliferative glomerulonephritis type II). However, loss of graft due to recurrence is rare; only 4 cases have been reported. Two additional pediatric patients are described. Both had recurrent dense "deposit" disease with extracapillary proliferation and crescent formation. In one of them, a quiet initial phase of 20 months was followed by an extremely rapid progression of the disease leading to loss of graft function within 3 weeks.

Acute Kidney Injury↗
PubMed1980

[Transplant pyelonephritis (author's transl)].

Examinations of the urine in 216 kidney allograft recipients resulted in significant bacteriuria in 274 samples of 1,802 urines tested. Bacteruria was found in 30 patients with recurrent or chronic persistent infections of the urinary tract; this patient group was studied by examination of 399 urine samples (mean 13.3 samples per patient). Four patients suffered from urologic complications after kidney grafting and were excluded from the study; 15 patients were diagnosed clinically and/or histologically with transplant pyelonephritis, 11 patients with cystitis. Of main importance for the diagnosis of transplant pyelonephritis were findings of persistent leucocyturia and the presence of antibody-coated bacteria. Both of these findings were repeatedly seen in all patients with transplant pyelonephritis. Clinical symptoms included fever and dysuria. In contrast to patients suffering from cystitis, transplant function detoriated in 13 of 15 patients with transplant pyelonephritis; two patients had to be treated by hemodialysis. Septicemia occurred in eight of the 15 patients studied. The data illustrate the frequency of transplant pyelonephritis as observed in 15 of 26 patients accompaining chronic urinary tract infection after kidney allograft transplantation. As a predisposing factor, obstruction of the urinary tract was diagnosed in eight of the 15 transplant recipients with pyelonephritis. The prednisone dose was higher than 10 mg in eight of 15 patients at the time transplant infection was diagnosed. Successful antibiotic treatment resulted in stable transplant function in three patients; four patients exhibited even lower serum creatinine levels after therapy.

Adult↗
PubMed1980

Familial glomerulopathy with giant fibrillar deposits.

Proteinuria and microhaematuria were observed in three siblings and one first-degree cousin. Histological examination of three kidney biopsies and one autopsy specimen shows the same diffuse glomerular lesions in all patients, characterized by mainly subendothelial but frequently transmembranous and mesangial deposits of a unique fibrillar structure, visible by electron microscopy. Examination by immunfluorescence gave inconstant findings. No serological abnormalities could be established. To our knowledge, such a pecular form of familial glomerulopathy has not been described so far.

Adult↗
PubMed1980

[Acute leukemia after kidney allotransplantation (author's transl)].

Four cases of acute myelogenous leukemia and six cases of chronic myelogenous leukemia after treatment with azathioprine and prednisone for renal allotransplantation have been described in the literature. We report another two cases of acute leukemia 10 and 5 years after successful renal allotransplantation. Patient 1, a 29-year-old farmer, exhibited the signs of acute lymphatic leukemia resistent to treatment with cytostatic agens. Death was due to pneumonia. Patient 2, a 47-year-old salesman, developed pancytopenia together with splenomegaly. After splenectomy an atypical subacute myeloid leukemia became apparent which was not treated due to withdrawal of the patient. He died 2 months after diagnosis. Both patients received long-term immunosuppressive therapy with azathiopine and prednisone until the leukemia was diagnosed. A relationship between long-term immunosuppression and the occurrence of leukemia is postulated.

Adult↗