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H Kellner

Publications and source records attributed to H Kellner.

At least 19 recordsLinked to original sources

The pathogenetic aspects of spondyloarthropathies from the point of view of HLA-B27.

The association of HLA-B27 and seronegative spondyloarthropathies, especially ankylosing spondylitis has been known for almost two decades. The spontaneous development of spondyloarthropathy like joint disease in HLA-B27 transgenic rats verifies the suspicion that the HLA-B27 antigens are directly responsible for disease development. With the recently revealed crystal structure of HLA-B27 and understanding of the class I molecule in general, a new hypothesis can be formulated based on the assumption that the pathogenesis of these diseases is a subversion of the physiological function.

Amino Acid Sequence

Immunogenetics of spondyloarthropathies.

The association of HLA-B27 with ankylosing spondylitis and related spondyloarthropathies has been known for two decades and has provided a great impetus to the epidemiologic studies and also helped broaden the clinical spectrum of these diseases. The etiology of these diseases is likely to be multifactorial and include genetic, immunologic, and environmental mechanisms. The detailed three-dimensional x-ray crystallographic structure of B27 has now been reported. It has revealed electron density compatible with oligopeptides that are nine amino acid-long (nonamers) bound in the antigen-binding cleft of the molecule. Microsequence analysis of 11 peptides eluted from the antigen-binding cleft has confirmed that all are nonamers. The most restricted position in the bound peptide is the second position, where all the 11 peptides contain arginine. The side chain of arginine extends into the B pocket ("45 pocket"), which seems to act as a specificity side pocket in the antigen-binding cleft of the B27 molecule. It is very likely that an understanding of the detailed structure of B27, including the peptide-binding motif and the structural domains recognized by cytotoxic T cells, along with the recent development of the B27 transgenic rat model for spondyloarthropathies, will further enhance our understanding of the immunogenetics of these diseases. It is hoped that this will lead to the source of the arthritogenic triggers and possibly disease prevention by antigen-specific immunomodulation. Because T-cell activation is initiated by the formation of antigen-MHC complexes that are the ligands that are recognized by the antigen-specific T-cell receptor (TCR), it might be possible to inhibit this activation by blocking the antigen-binding cleft of MHC molecules by using high-affinity MHC-binding peptides (MHC blockade) or by a novel, new, and more efficient method of TCR antagonism.

Amino Acid Sequence

Repeated isovolemic large-volume erythrocytapheresis in the treatment of idiopathic hemochromatosis.

In order to assess the effectiveness of cytapheresis as a possible alternative therapy for iron depletion, we performed a prospective study on eight unrelated patients with idiopathic hemochromatosis (HC). Isovolemic large-volume erythrocytapheresis (EA) (1000 ml apherisate) was carried out every four weeks until serum ferritin levels dropped below 300 micrograms/l (initial therapy). In all patients iron depletion was achieved after a mean of 8.5 months (8.9 EA with a total removal of 9.41 RBC). Serum ferritin levels decreased during initial therapy from 2596 +/- 399 to 168 +/- 83 ug/l. Serum iron level (240 +/- 35 to 125 +/- 48 ug/dl) and transferrin saturation (91 +/- 6 to 19 +/- 10%) declined accordingly. Clinical reexamination after initial therapy revealed improvement of clinical symptoms, normalization of hepatic iron, but liver histology remained unchanged. Reaccumulation of iron was prevented by maintenance EA therapy every five to six months (follow-up 18-36 months). Isovolemic large-volume EA is an effective, fast and safe method to remove excessive stored iron in patients with HC. Compared to phlebotomy, EA can selectively remove RBC, while saving plasma proteins, platelets, and clotting factors. Although, the need for special equipment and trained personnel as well as the relatively high costs are limiting factors of EA so far, it can be of crucial advance in some patients with HC.

Adult

Ultrasound findings in Löfgren's syndrome: is ankle swelling caused by arthritis, tenosynovitis or periarthritis?

Arthritis of acute sarcoidosis or Löfgren's syndrome commonly affects the ankles. However, it is often difficult to determine by clinical means whether swelling of the ankles is due to frank arthritis or involvement of periarticular tissues. Therefore, sonographic examinations were performed in 24 consecutive patients with acute sarcoidosis, 16 of them with the typical triad of Löfgren's syndrome. Joint effusions could be demonstrated only by ultrasound in 6 patients. Sonographic findings consistent with tenosynovitis were found in 8 cases. In the majority of cases (20/24) the predominant abnormalities were hypoechogenic structures within the subcutis and periarticular tissues. Thus, frank arthritis is rather a rare cause of ankle swelling in Löfgren's syndrome; more often presumed joint manifestations can be attributed to subcutaneous or periarticular inflammation.

Acute Disease

Polymorphisms in the VK gene patterns of rheumatoid arthritis patients and control individuals.

Restriction nuclease digests of DNAs from rheumatoid arthritis patients and control persons were studied by blot hybridization using immunoglobulin VK gene probes of subgroups I-III. Two restriction fragment length polymorphisms (RFLP) were detected in Bg1II digests with a VKI gene probe. One of the RFLPs is linked to rheumatoid arthritis with a relative risk of 5 (p less than 0.01). The observation may be of pathogenetic interest.

Arthritis, Rheumatoid

[Echinococcus cysticus--rare differential diagnosis of a splenic cyst].

Splenic involvement in hydatid disease is uncommon, representing less than 2% of all human organ manifestations by echinococcus cysticus. We report on the rare case of an isolate splenic infection. The case history and the clinical findings were compatible with a traumatic cyst. Ultrasound, CT and MR findings are discussed.

Cysts

[Cryptococcus neoformans meningoencephalitis and multiple infections in AIDS].

A 40-year old homosexual AIDS patient recovering from a Pneumocystis carinii pneumonia developed a Cryptococcus neoformans infection with involvement of the central nervous system (CNS) which could be treated successfully with amphotericin B and flucytosine. After a symptom-free interval of 4 1/2 months, a new acute fatal disease of the CNS did not reveal a cryptococcosis relapse but a necrotizing Toxoplasma encephalitis, a cytomegalovirus infection and striking cultural findings of Staphylococcus aureus in all organs examined. Neither by culture nor by histology Cr. neoformans could be detected in the CNS or in the other organs examined. The temporal course of the Cr. neoformans infection and its specific diagnosis are commented. It is demonstrated that (during or after successful therapy of Pneumocystis carinii pneumonia) a specific cultural examination of specimens from the respiratory tract for Cr. neoformans is needed, in order to recognize a Cr. neoformans infection in its primary stage, i.e. before hematogenous dissemination of Cr. neoformans leading to the secondary stage of the infection.

Acquired Immunodeficiency Syndrome

[Renal and enteral elimination of coproporphyrin isomers in Rotor's syndrome. A family study].

Urinary and fecal total, isomer I, and isomer III coproporphyrin excretion of a Rotor's syndrome patient and his family were determined. The propositus showed increased urinary total coproporphyrin excretion (248 micrograms/24 h) and a shift of the coproporphyrin isomer I/III relation (70%/30%). The propositus's father and two siblings also had elevated renal excretion of coproporphyrin I. Total coproporphyrin excretion was enhanced only in the propositus's father and one sibling, while being normal in another sibling. All family members that could be investigated showed considerably decreased fecal porphyrin excretion. In Rotor's syndrome porphyrin excretion is mainly renal. The coproporphyrin isomer I/III relation is shifted towards isomer I. Phenotypically normal relatives with normal bilirubin plasma levels may have alterations in both their renal and enteral coproporphyrin excretion.

Adult

Erythrocytapheresis. A method for rapid extracorporeal elimination of erythrocytes. Results in 65 patients.

We report on the progress of a new modified method of phlebotomy, erythrocytapheresis, as a means of fast therapeutic removal of erythrocytes from the circulation. We performed erythrocytapheresis in 65 patients. In 18 patients with central venous thrombosis of the eye, the beneficial effect of this procedure proved superior to the traditional approach. In 29 patients with primary or secondary polycythemia, hemoglobin, hematocrit, and blood viscosity could be lowered drastically for up to 11 months by a single erythrocytapheresis. We performed erythrocytapheresis in an effort to deplete the iron stores in patients with hemochromatosis (14 cases) and in patients with porphyria cutanea tarda (4 cases). Several consecutive erythrocytaphereses were necessary, however, to even slightly lower the amounts of stored iron as measured by serum iron, iron-binding capacity, and serum ferritin in these patients. The intervals between treatment were 2 to 11 months, thus much longer than the intervals between blood-lettings. We did not observe any adverse side effects. There was no significant influence on the clotting system, and no reactive thrombocytosis as described after phlebotomies.

Adult

[Diverticulosis of the duodenum].

A 62-year-old woman was admitted to hospital because of extremely severe colicky pain in the mesogastric region and vomiting. Six years earlier two duodenal diverticles had been found to be responsible for similar symptoms. Endoscopy and X-ray examination now revealed four extensive duodenal diverticles, the largest of which had an extension of 8 X 7 cm. There were no diverticular complications. The patient became free from complaints by conservative treatment, at first with "zero" diet, then with a regimen rich in dietary fibres and frequent small meals. She remained symptom-free during a follow-up period of six months so far.

Diverticulum

[Treatment of chronic arterial circulatory disorders. Double blind trial with Trental 400 (author's transl)].

For the objective determination of its efficacy, Trental 400 was examined against placebo in a double blind trial in 40 patients with chronic peripheral circulatory disorders and concomitant symptoms of cerebro-vascular insufficiency typical of age. Treatment lasted 7 to 8 weeks with a dosage of 1 tablet 3 times a day. Trental 400 was significantly more effective than placebo in the treatment of peripheral and cerebral disorders. The preparation was well tolerated, subjectively and objectively.

Administration, Oral

[Determination of skull volume (author's transl)].

Various means for indirect skull volume determination were investigated. For this purpose--the procedure of direct measurement of skull volume was improved first, using a latex method. The examinations were carried out on 20 macerated skulls, which were x-rayed in the usual manner and stereoscopically also. Various distances and planes were then determined from the radiographs and varied calculations carried out. From these calculations it appears that skull volume can be simply determined most accurately from the product of transverse diameter (greatest width) and the area of median plane.

Adult