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A Eckstein

Publications and source records attributed to A Eckstein.

At least 19 recordsLinked to original sources

Improved prediction of relapse of Graves' thyrotoxicosis by combined determination of TSH receptor and thyroperoxidase antibodies.

BACKGROUND: Recently, we and others have demonstrated that high levels of auto-antibodies to the human TSH-receptor (TRAb) predict relapse of hyperthyroidism in Graves' disease (GD). Our objective was to extend the outcome of the prediction by combining TRAb with thyroperoxidase antibody (TPO-Ab) measurement. PATIENTS AND METHODS: One hundred and thirty-one GD patients (118 females, 13 males) were analysed, of whom 94 patients (71.8%) had relapse, whereas 37 (28.2%) went into remission. Second generation TRAb and TPO-Ab assays were performed in GD patients with relapse and remission in mean 4.3 months after initial diagnosis. RESULTS: The mean anti-TPO-Ab levels were similar in all patients with relapse and remission. However, there was a steady decline from 4047 U/ml to 530 U/ml in the remission group that correlated positively with TRAb values (>2 to >10 IU/l). The relapse group remained at consistently high levels. The positive predictive value (PPV) for relapse in patients with TRAbs >6 IU/l and anti-TPO-Abs >5000 U/ml was 100, whereas TRAbs >6 IU/l and anti-TPO-Abs >500 U/ml were associated with a PPV of 93.7 up to 96 (p=0.008). These Ab constellations accounted for about one third of all GD patients. For patients with TRAbs between >2 and <6 IU/l the PPV was 66.7-90.0. CONCLUSION: Our follow-up analysis indicates that the prediction of relapse of GD can be improved by a combined measurement of TRAb and TPO-Ab. In patients with moderately increased Abs, determined about 6 months after initial diagnosis, an ablative therapy can be approached without delay.

Adolescent↗

[Is combined surgical correction of horizontal and vertical squint of value in graves' ophthalmopathy?].

BACKGROUND: In Graves' ophthalmopathy squint can be corrected in about two-thirds of the patients with a single recession of an inferior or medialis rectus muscle. The dose-effect correlation is linear over a wide range. Combined vertical and convergent misalignments are rare. The aim of this study was to evaluate the dose-effects in combined recessions of medialis and inferior rectus muscles. MATERIAL AND METHODS: The dose-effect of combined recessions (one side medialis and inferior n = 28, both sides medialis and one side inferior n = 9) was evaluated. The control groups were patients with Graves' ophthalmopathy, who had single inferior recession (n = 187), single medialis recession (n = 37) and bilateral medialis recession (n = 44). RESULTS: Small hypotropias (up to 5 degrees ) at the eye with the poorer abduction disappear after single (17 of 21) or bilateral (11 of 19) medialis recessions. This obvious influence of horizontal recession on the vertical angle leads to a higher dose-effect for the inferior recessions in combined surgery, and was stronger for bilateral cases (from 2.0 degrees to 2.7 degrees /mm recession) than for unilateral cases (from 2.0 degrees to 2.2 degrees /mm recession). The dose-effect for medialis recession in combined surgery increased for the unilateral procedures only from 1.7 degrees to 1.8 degrees /mm recession and not for the bilateral medialis recession. CONCLUSIONS: The dose-effect for combined medialis and inferior recessions is enhanced and varies to a much higher degree in comparison to single muscle recessions. Because of the higher variability, patients who need both medialis and inferior recession should be better operated in separate sessions, beginning with the horizontal muscle(s).

Adult↗

Impact of smoking on the response to treatment of thyroid associated ophthalmopathy.

BACKGROUND: In patients with Graves' disease, smoking considerably increases the incidence and severity of thyroid associated ophthalmopathy (TAO). The authors sought to determine if smoking also influences the course of TAO during treatment, and the efficacy of therapy. METHODS: 41 smokers and 19 non-smokers with moderate untreated TAO were included in this prospective study. All patients were treated with steroids and, 6 weeks after the beginning of drug therapy, with orbital irradiation. Follow up was performed 1.5, 4.5, 7.5, and 12 months after the beginning of the study. Proptosis, clinical activity score (CAS), and motility were evaluated. The extent of smoking was derived from the concentration of the haemoglobin adduct N-2-hydroxyethylvaline (HEV), a parameter of long term smoking. RESULTS: There was no difference in the clinical manifestations of TAO between smokers and non-smokers at the beginning of treatment. However, CAS decreased (p<0.05) and motility improved (p<0.02) significantly faster and to a greater extent in non-smokers than smokers. Inverse correlations between the CAS decrease and the HEV levels observed 4.5 and 7.5 months after the beginning of treatment and between the improvement of motility and the HEV levels after 1.5, 4.5, and 7.5 months indicated a dose dependence. Mean HEV levels did not vary much during the follow up period and were significantly different in smokers (mean 5.4 (SD 2.7) micro g/l) and non-smokers (mean 1.8 (1.3) micro g/l; p<0.01). CONCLUSION: Smoking influences the course of TAO during treatment in a dose dependent manner. The response to treatment is delayed and considerably poorer in smokers.

Adolescent↗

Compensatory visual field training for patients with hemianopia after stroke.

Twenty-one patients with hemianopia received 4 weeks of compensatory visual field training. Detection of and reaction time to visual stimuli were measured with eyes fixating (condition A) and with use of exploratory eye movements (condition B) before and after training. Twenty-three healthy individuals served as control subjects for measurements of parameters during both conditions. Patients with hemianopia to either side showed a marked improvement of detection and reaction time during condition B, but minimum or no change during condition A. Improvements were maintained 8 months after training. Activity of daily living skills also improved in all patients. The size of scotoma on computerized perimetry, in contrast, remained unchanged. Training improved detection of and reaction to visual stimuli without restitution of the visual field defect.

Activities of Daily Living↗

Characterization of aldose reductase from the thick ascending limb of Henle's loop of rabbit kidney.

BACKGROUND: The organic osmolyte sorbitol plays an important role in the osmoregulation of immortalized epithelial cells of the thick ascending limb of Henle's loop (TALH) of rabbit. The intracellular sorbitol content seems to depend strongly on the extracellular osmolarity. To investigate the nature of the osmotic regulation we characterized the aldose reductase. METHODS: We determined aldose reductase activity enzymatically and the content of organic osmolytes by HPLC. RESULTS: The aldose reductase activity correlates with the extracellular tonicity. Elevating the osmolarity of the medium from 300 to 600 mosm/l by addition of NaCl or sucrose resulted in a significant increase of maximal velocity (V(max)) of the adapted cells from 8 +/- 1 micromol/g x min (300 mosm/l) to 322 +/- 28 micromol/g x min (600 mosm/l, NaCl) or 54 +/- 9 micromol/g x min (600 mosm/l, sucrose), respectively, while affinity (K(m)) remained unchanged. But we found no rise of aldose reductase activity when extracellular urea concentration was elevated. Similar alterations in V(max) were observed when the activity of the highly enriched enzyme was determined with glucose as substrate. Elevation of the extracellular osmolarity by NaCl and sucrose strongly induced the expression of aldose reductase protein with an apparent molecular weight of 39 kD. The affinity of glucose is characteristically low with a K(m) above 300 mmol/l. Aldose reductase utilizes both NADPH and with lower affinity NADH as coenzymes. In vitro sulfate ions (0.4 mol/l) results in a two-fold activation of the aldose reductase activity whereas sodium (200-400 mmol/l) decreased the activity significantly (22-33%). Potassium and chloride up to 400 mmol/l did not alter the aldose reductase activity in vitro. CONCLUSIONS: These results indicate that the aldose reductase of TALH cells of the outer medulla is osmotically regulated and has many similarities with aldose reductase in renal inner medulla. Therefore, intracellular sorbitol synthesis seems to be of similar importance in the osmoregulation of TALH cells as in the inner medulla.

Adaptation, Physiological↗

Assessment of keratoconjunctivitis sicca in patients with fibromyalgia: results of a prospective study.

Patients with fibromyalgia (FM) often describe the presence of dry eyes and other ocular symptoms. It has been claimed that a subgroup of patients with FM might have features suggestive of primary Sjögren syndrome. In others, such a relationship could not be found. The purpose of the present study was to investigate the association and prevalence of keratoconjunctivitis sicca (KCS) in patients with FM. Among 285 patients with FM, 40 patients reporting sicca symptoms were screened with Schirmer's I test, break-up time and Rose-Bengal score. KCS was diagnosed when two of the selected three tests gave pathological results. A detailed ophthalmological examination was also performed. In 15 patients the diagnosis of KCS could be confirmed. Eighteen of 40 patients had been taking low-dose antidepressants and 7 of them had objective signs of KCS. Eight of 40 patients had signs of chronic blepharitis and 4 of them had KCS. Fourteen patients showed unremarkable test results. Chronic blepharitis and the use of tricyclic antidepressants may play a role in developing KCS. It seems that the rate of KCS does not increase in patients with FM and they probably have objective ocular findings comparable with the normal population.

Adult↗

Ocular muscle and eyelid surgery in thyroid-associated orbitopathy.

The aim of ocular muscle surgery in Graves' disease is to determine how an optimal normalization of ocular motility disorders - diplopia, compensatory head posture, eyelid dispositions - can be achieved. The results of ocular muscle surgery of fibrotic ocular muscles allow the following conclusions to be drawn. Correcting the motility disorder can be precisely dosed by recessing only one fibrotic ocular muscle with a strabismic angle of up to 15 degrees. This leads to reproducible and dependable results, with a dose-effect coefficient independent of the initial strabismus angle. Indications as to the amount of surgery and which side should be varied according to horizontal an vertical deviations, and are also dependent on the compensatory head posture. Improvement of the binocular visual field is possible in nearly all cases. Over corrections occur more often when the muscle is not directly fixed at the sclera but adjusted on the following day. The time factor is important both before and after the operation. Before the operation, the motility status should have been stable for at least six months. Postoperatively, within the first few days, an insufficient correction should be expected, however this should not lead to premature revision of the amount of surgery. The surgery of the vertical rectus muscles influences the eyelid position. The upper lid retraction is improved with surgery on the vertical extraocular muscle and depends on prior upper lid motility. In contrast, an increase in lower lid retraction is not dependent on an inferior rectus recession. The results permit a precise series of steps to be drawn up in Graves' disease regarding surgical indications, proportionally correcting each side, and the dosage of ocular muscle operations.

Eyelids↗

Prognostic value of the pattern electroretinogram in cases of tumors affecting the optic pathway.

BACKGROUND: Tumors compressing the optic pathway may lead to irreversible loss of vision which may be detected by the pattern electroretinogram (PERG) because of its relation to ganglion cell function. METHODS: Eyes of 19 patients were tested shortly before and 5-10 days after tumor surgery. Visual acuity, the 30-deg visual field and the transient and steady-state pattern reversal ERG were measured. RESULTS: Using patterns of 1.5 x 1.2 deg there was a good correlation between the change of pre- and post-surgical visual performance and most of the pattern ERG amplitudes. For all variables tested--P50, N95- and steady-state amplitude--there was a critical value beyond which the visual outcome could be bad or favorable, whereas patients showing higher amplitudes always remained stable or improved after surgery. CONCLUSION: The positive correlation between pattern ERG amplitudes and the post-surgical outcome in the case of tumors affecting the optic pathway may be helpful in predicting the outcome for these patients.

Adolescent↗

Ocular findings in patients with autosomal dominant retinitis pigmentosa and Cys110Phe, Arg135Gly, and Gln344stop mutations of rhodopsin.

This report describes ocular findings obtained in four patients from three families with autosomal dominant retinitis pigmentosa (adRP) due to missense mutations in the rhodopsin gene. Phenotypes were characterized by standard ophthalmologic examinations, visual fields, electroretinography (ERG), dark adaptation, and two-color dark-adapted threshold perimetry. Two patients aged 38 and 45 years, respectively, from a family with the Cys110Phe mutation showed mild fundus changes without bone spicules as well as small arcuate scotomas in the inferior quadrants of their visual fields but displayed severe functional loss of rods and cones in the ERG. Two-color dark-adapted threshold perimetry revealed a regional type of degeneration. A 48-year-old patient with an Arg135Gly mutation had typical RP with concentrically narrowed visual fields and nondetectable ERG responses. Central visual functions were well preserved for a long time. Two-color dark-adapted threshold perimetry indicated a diffuse type of retinal degeneration. An 18-year-old patient with a Gln344stop mutation has been followed for 13 years. His ERG was clearly reduced at the age of 5 years; since that time, disease progression has been very slow. Currently, there are relatively mild alterations in visual acuity, rod sensitivity, and visual fields. Our findings confirm that there is a large phenotypic variety among patients with adRP and different rhodopsin mutations.

Adolescent↗

RDS/peripherin gene mutations are frequent causes of central retinal dystrophies.

Patients from 76 independent families with various forms of mostly central retinal dystrophies were screened for mutations in the RDS/peripherin gene by means of SSCP analysis and direct DNA sequencing. Two nonsense mutations (Gln239ter, Tyr285ter), five missense mutations (Arg172Trp, Lys197Glu, Gly208Asp, Trp246Arg, Ser289Leu), and one single base insertion (Gly208insG), heterozygous in all cases, were detected. Only one of these mutations, Arg172Trp, has been reported previously. Cosegregation of the mutation with the disease phenotype could be established in selected families. Other missense mutations were excluded from a panel of 55-75 control subjects. The patients showed remarkable variation in phenotype and disease expression not only between cases with different mutations but also between affected members of the same family. This study indicates that RDS/peripherin mutations are a frequent cause of various types of central retinal dystrophies and that the RDS/peripherin gene exhibits a broad spectrum of allelic mutations. Comparative analysis of known mutations allowed us to hypothesise that the deleterious effect of RDS/peripherin gene mutations is the result of different molecular mechanisms.

Exons↗

[Behçet's disease].

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Adrenal Cortex Hormones↗

Analysis of 21 Stargardt's disease families confirms a major locus on chromosome 1p with evidence for non-allelic heterogeneity in a minority of cases.

BACKGROUND: Autosomal recessive Stargardt's disease is a macular degeneration characterised by a juvenile onset and a rapidly progressive course resulting in an atrophic macular area typically surrounded by yellowish retinal flecks. METHOD: The disease locus has previously been assigned to markers from chromosome 1p21-p13 by genetic linkage analysis in eight multiplex Stargardt's disease families. RESULTS: In an extended analysis, the assignment to chromosome 1p was confirmed in the majority of the 21 families with Stargardt's disease who were studied. In addition, a series of recombinant chromosomes further narrowed the Stargardt's disease region to an approximately 3 cM interval between markers at D1S424 and D1S497. CONCLUSION: Multipoint linkage analysis most probably excludes this locus in three of these families suggesting non-allelic heterogeneity with at least one additional minor Stargardt's disease locus.

Chromosomes, Human, Pair 1↗

Osmotic regulation of sorbitol in the thick ascending limb of Henle's loop.

Organic osmolytes, such as sorbitol, inositol, glycerophosphorylcholine, and betaine, play an important role in the osmoregulation of inner medullary cells of the kidney. The cells of the outer medulla are also exposed to elevated NaCl and urea concentrations during antidiuresis. Therefore, we investigated the mechanisms involved in the regulation of outer medullary organic osmolytes, especially cell sorbitol content of immortalized epithelial cells of the thick ascending limb of Henle's loop (TALH). In the cultured cell model, a 600 mosmol/l medium (osmolarity adjusted by addition of 150 mM NaCl or 300 mM sucrose) increased the intracellular sorbitol content significantly compared with a 300 mosmol/l medium. The accumulation of sorbitol appeared to be due to an increase of aldose reductase activity, which catalyzed sorbitol synthesis. Sorbitol degradation by sorbitol dehydrogenase was not detectable under our experimental conditions. After a sudden decrease of the extracellular osmolarity, the sorbitol permeability of the cellular membrane increased sevenfold within 10 min compared with isosmolar conditions. These results indicate that sorbitol, like inositol, plays an important role in the osmoregulation of TALH cells. Although the short-term regulation involves rapid changes in sorbitol membrane permeability, the longterm adaptation to low osmolarities is regulated by intracellular sorbitol synthesis.

Aldehyde Reductase↗

Osmotic regulation of the betaine metabolism in immortalized renal cells.

Betaine plays an important role in the osmoregulation of various renal cells. In the kidney betaine synthesis seems to be highest in the cortex, whereas osmotically regulated accumulation seems to play a crucial role in the inner medulla. Therefore, the influence of betaine synthesis on the long-term osmotic regulation of betaine content was investigated in epithelial SV40 transfected cell culture, derived from the outer medullary thick ascending limb of the loop of Henle (TALH) of rabbit kidney. Under hyperosmotic conditions the betaine content of TALH was significantly increased from 218 +/- 35 mumol/g protein (300 mOsm/liter; control) to 334 +/- 27 mumol/g (600 mOsm/liter; P < 0.0005). In addition the intracellular accumulation of 14C-betaine from 14C-choline was significantly elevated from 4.3 +/- 1.0 mumol/g protein x hr) to 8.2 +/- 1.0 mumol/g protein x hr; P < 0.001) under hyperosmotic conditions. Synthesis of betaine was also influenced by the extracellular betaine content. In a betaine free medium the synthesis of betaine was increased by 7% (300 mOsm/liter; NS) or 40% (600 mOsm/liter; P < 0.0001) when compared to betaine containing medium. The alteration of betaine synthesis is presumably caused by osmotic regulation of the betaine aldehyde dehydrogenase. Activity of this enzyme was significantly higher under hyperosmotic conditions compared to isoosmotic control conditions (Vmax 4.1 +/- 0.8 U/g protein; 600 mOsm/liter) versus 1.4 +/- 0.1 U/g (300 mOsm/liter; P < 0.0001), while the affinity to betaine aldehyde remained unaltered. These results demonstrate that during long-term adaptation, betaine synthesis in TALH cells of the outer medulla of rabbit kidney can be regulated by extracellular osmolarity.

Alcohol Oxidoreductases↗

[Varicose vein of the iris].

A 56-year-old man presented with a pigmented iris nodule which showed prominent vessels and enlargement within 6 weeks. The tumour caused recurrent hyphemata. Differential diagnosis included a pigmented iris hemangioma and a malignant melanoma. A sectoriridectomy was performed. Histology revealed a stromal cavity filled with fibrin and abundant erythrocytes. A diagnosis of an iris varix was established. However, it has to be pointed out that the distinction between a varix of the iris and an iris hemangioma may be difficult not only clinically but also histologically, and that iris melanomas may produce hemangioma-like patterns.

Diagnosis, Differential↗

[Atypical optic neuritis in systemic lupus erythematosus (SLE)].

HISTORY AND CLINICAL DATA: A 67-year-old woman experienced acute unilateral visual loss accompanied by pain with eye movements. There was a marked relative afferent pupillary defect and a nerve fiber bundle defect in the upper half of the visual field. Optic discs were normal. After 4 days vision worsened to motion detection and only a temporal island was left in the visual field. The optic disc margin was blurred. Since thirty years she had been suffering from renal insufficiency. Immunoserologic examination revealed elevated ANA and DS-DNA antibody titers. An optic neuritis in systemic lupus erythematosus was diagnosed, which is called atopic, because of its association to a systemic disease and the old age of the patient. TREATMENT AND FOLLOW UP: The patient was treated with 100 mg prednisolone/day, slowly tapered. Within 6 weeks visual acuity improved to 0.6 and visual field normalized except for a small nerve fiber bundle defect. CONCLUSION: Autoimmune optic neuritis often responds to treatment with corticosteroids. Early onset of treatment is important. Immunopathologic examinations are an important diagnostic tool in atopic optic neuritis. Their results may even have consequences for the treatment of the underlying disease.

Aged↗

[Tarantule hairs as corneal foreign bodies].

A 22 year-old-man and owner of several tarantulas visited his ophthalmologist because of red itchy eyes. Additionally to kerato-conjunctivitis epidemica, uncommon hairs were found especially in his left cornea. Corrected visual acuity was 0.8. According to literature these spider hairs can induce granulomas in the meaning of ophthalmia nodosa, and even migrate inside the eye. Therefore we removed as many hairs as possible, but we left two, not to cause iatrogen perforation. The penetration depth was measured by laser tomography. Local steroids successfully prevented further granuloma formation. Regular ophthalmological controls are important to early detect possible changes, and if necessary to remove these hairs even from deeper corneal layers, in case by keratectomy.

Adult↗