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Biomedical subjects

J R Harle

Publications and source records attributed to J R Harle.

At least 55 records · Page 3Linked to original sources
PubMed1992

Behçet's syndrome and factor XII deficiency.

Several mechanisms have been proposed to explain thrombotic tendency in Behçet's syndrome. We report the case of a 43-year old woman presenting retinal-vein thrombosis, factor XII deficiency and Behçet's syndrome. This kind of association has thus far never been reported. Factor XII deficiency is known to possibly induce various types of thrombosis and might explain the prevalence of ocular symptoms in our patient.

Adult↗
PubMed1992

Polymyalgia rheumatica and mitochondrial myopathy: clinicopathologic and biochemical studies in five cases.

PURPOSE: The coexistence of mitochondrial myopathy and polymyalgia rheumatica without giant cell arteritis is an interesting association. The frequency of this association was assessed in a prospective study. PATIENTS AND METHODS: Muscle biopsy specimens were obtained from 15 patients with polymyalgia rheumatica. When ragged red fibers (RRF) were observed, histochemical, ultrastructural, and biochemical studies were performed. RESULTS: In five cases, we found the typical appearance of mitochondrial myopathy, with the presence of numerous RRF. Histochemical and biochemical results confirmed these mitochondrial myopathies, showing miscellaneous deficiencies of mitochondrial respiratory chain enzymes. CONCLUSION: Persistence of histologic and biochemical abnormalities after steroid treatment in two patients seems to indicate that a subclinical mitochondrial myopathy preceded polymyalgia rheumatica. How a mitochondrial myopathy could induce or facilitate the emergence of a polymyalgia rheumatica remains unknown.

Aged↗
PubMed1992

[Isolated memory disorders disclosing antiphospholipid antibody syndrome].

Most of the neurological complications of the antiphospholipid syndrome are consequences of arterial or venous cerebral thromboses. We report the case of a 46-year-old woman presenting with the main signs of antiphospholipid syndrome: Raynaud's phenomenon, livedo, leg ulcers, repeated miscarriages, presence of a circulating anticoagulant and of antiphospholipid antibodies, who developed an isolated amnestic syndrome with a peculiar pattern; 1) almost complete sparing of the ability to learn new skills and of short-term recall; 2) deterioration, followed by disappearance of recent memories after a sufficient delay; 3) progressive alteration of increasingly old memories including knowledge memory suggested a gradual alteration of mnestic traces. The lack of neuroradiological signs of multiple infarcts suggests a direct intervention of antibodies in this patient's memorisation mechanisms.

Antibodies, Antiphospholipid↗
PubMed1992

[Importance of hypergastrinemia in diagnosis of Biermer's disease in adults].

Achlorhydria that is due to antrum sparing atrophic gastritis is a characteristic finding in pernicious anemia. As a consequence of this achlorhydria serum gastrin level rise. Usually several signs are required for assessing a pernicious anemia: megaloblastic anemia, low serum cobalamin level, positivity of a Schilling test, low level of intrinsic factor in the gastric juice, existence of anti-intrinsic factor antibodies in plasma and/or in gastric juice. In this study the usefulness of the serum gastrin level is assessed. We report here the results in investigating sixteen patients with a pernicious anemia. All had: megaloblastic changes on bone marrow examination, serum cobalamin level lower than 150 pg/ml. For 14 patients the performed Schilling test was positive. For 2, no Schilling test was done, but anti-intrinsic factor antibodies were elevated. Serum gastrin levels were higher than 250 pg/ml (254 to 1770 pg/ml). In our patients with pernicious anemia, as a result of hypo or achlorhydria, serum gastrin increase was higher than in any other cause of hypochlohydria: anti-acid treatment, vagotomy... Measurement of serum gastrin is easy. High levels are simple to explain if found together with a sparing antrum atrophic gastritis and/or a low level of serum cobalamin. Serum gastrin measurement may be a cornerstone to rapid and reliable diagnosis of pernicious anemia.

Adult↗
PubMed1992

[Value and limits of the tilt-test in the etiological diagnosis of transient malaise].

This study was undertaken to assess the usefulness of the head-up tilt test in the diagnosis of vasovagal syncope and to evaluate the indications of this investigation. The test consists in a 30 minute period in the horizontal decubitus position followed by a 60 degrees head-up tilt position for 30 minutes. In the 26 subjects in whom the diagnosis of repeated vasovagal syncope had been made on the basis of typical prodromic symptoms after exclusion of all other causes, the test induced a vasodepressive or vagal reaction in 10 cases (38%) 20 +/- 4 minutes after tilting. In 8 patients in whom the test was performed within 48 hours of their syncopal episode, a similar malaise was induced in 7 cases. Twenty patients with repeated malaise without loss of consciousness had no symptoms during the test which was also negative in 10 normal control subjects. In a group of 27 subjects with unexplained syncope after clinical and paraclinical investigations, the test was accompanied by a malaise on five occasions (19%). Using this protocol, the tilt test had good specificity but moderate sensitivity in the diagnosis of vasovagal syncope. Its sensitivity increased when performed within 48 hours of syncope. It is of no value in the diagnosis of recurrent malaise without loss of consciousness.

Blood Pressure↗
PubMed1991

Severe fibromyalgia after hypophysectomy for Cushing's disease.

We report a case of fibromyalgia occurring after hypophysectomy for Cushing's disease. Clinical examination revealed tender points at 12 of the 18 tender point sites described in the American College of Rheumatology 1990 criteria for the classification of fibromyalgia. The cause of fibromyalgia remains unknown. In our patient, hypophysectomy may have played a role by disturbing endorphin secretion and pain modulation.

Cushing Syndrome↗