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Biomedical subjects

C Althaus

Publications and source records attributed to C Althaus.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Uveitis masquerade syndrome in Gaucher disease. Causal treatment by alglucerase substitution therapy].

BACKGROUND: Gaucher's disease, a sphingolipidose transmitted by autosomal-recessive inheritance, is caused by a deficiency of the lysosomal enzyme beta-glucocerebrosidase which is responsible for hydrolysation of glucocerebroside to ceramid and glucose. Thus glucocerebroside is accumulated in the reticuloendothelial cells of spleen, liver and bone marrow and pathognomonic Gaucher's cells are formed. CASE REPORT: In May 1997 a 42-year-old female patient presented with left-sided intermediate uveitis of unknown origin and decreasing visual acuity to perception of hand movements and intact projection of light since September 1996. To exclude a systemic disease a thorough medical examination--showing hepatoslpenomegaly, anemia, thrombocytopenia and bone lesions--was initiated and revealed advanced M. Gaucher (Type I) by bone marrow punction. Intravenous therapy with alglucerase was administered promptly. After five months of treatment vitreous opacities resolved almost completely and visual acuity increased to 0.7. CONCLUSIONS: The dramatic improvement occurred under treatment with alglucerase after no response to steroid treatment. Thus intermediate uveitis with severe vitritis can be interpreted as uveitis masquerade syndrome with M. Gaucher. To the best of our knowledge, this is the first case of M. Gaucher presenting initially as intermediate uveitis and showing successful clinical improvement during administration of alglucerase.

Adult↗
PubMed1998

Recombinant tissue plasminogen activator in cases with fibrin formation after cataract surgery: a prospective randomised multicentre study.

AIMS: This study investigated the effect of tissue plasminogen activator (tPA) in patients with severe intracameral fibrin after extracapsular cataract extraction or phacoemulsification with posterior chamber intraocular lens implantation. METHODS: A randomised prospective multicentre study was carried out in 86 patients with intraocular fibrin formation 2-8 days after cataract surgery. While the first group (n = 41) received only anti-inflammatory drugs, a single anterior chamber injection of tPA (10 micrograms) as an additional treatment to the standard was given in the second group (n = 44). On days 1, 2, 14, and 90 after randomisation, the visual acuities, slit lamp findings, and intraocular pressures were documented in standardised protocols. Efficacy of treatment was judged by the rate of fibrinolysis (primary objective), the frequency of synechiae, and central capsular fibrosis (secondary objectives). RESULTS: The incidence and quantity of intraocular fibrin were significantly lower in the patients treated with tPA than in the control group (p < 0.05). The frequencies of synechiae were reduced by tPA injection. The capsule fibrosis noted after 3 months was significantly lower in the tPA group (p = 0.027). No ocular side effects were noted after the tPA injections. CONCLUSIONS: Lysis of postcataract fibrin formation is accelerated and increased by a single intracameral injection of 10 micrograms tPA in addition to standard anti-inflammatory treatment. The findings suggest that the tPA injection reduces posterior capsule fibrosis, which still has to be addressed in larger study populations and with a long term follow up.

Adolescent↗
PubMed1997

[Surgical management of Marfan-associated and idiopathic lens dislocations].

BACKGROUND: The ideal and safe surgical method for Marfan-associated or idiopathic lens subluxations is still a matter of debate. PATIENTS AND METHODS: Between 1990 and 1995, 23 eyes were operated for lens subluxations, mainly because of decreased visual acuity, but also because of conservatively uncontrolled secondary glaucoma. Marfan patients were 27.0 (5-62) years old at surgery; patients with idiopathic lens subluxations were 38.5 (11-63) years old. Surgical procedure depended on patient age and anatomical conditions. RESULTS: All patients achieved an increase in visual acuity. Amblyopia existed in six patients. All problems due to glaucoma were controlled postoperatively. Our greatest concern was rhegmatogenous retinal detachment. It occurred in only one eye of a non-Marfan patient. CONCLUSION: The prognosis for lens surgery in Martan-associated and idiopathic lens subluxations is good. The implantation of a posterior chamber lens provides a good and secure means of optical rehabilitation. Our preferred primary transscleral suture technique guarantees high security and stability of position. Previously feared surgical risks have been reduced by modern surgical procedures.

Adolescent↗
PubMed1997

[Homologous central limbo-keratoplasty in limbus stem cell damage. Retrospective study of 3 years' experience].

BACKGROUND: Problems with epithelial healing are the main cause of corneal graft failure in patients with limbal stem cell deficiency. This post-operative complication cannot be eliminated by conventional penetrating keratoplasty alone, but only by additional limbal grafting, which is, however, highly immunogenic. We report here our 3 years' experience with a new surgical procedure which we developed called homologous central limbokeratoplasty. PATIENTS AND METHODS: We have performed 52 homologous central limbo-keratoplasties in patients with limbal stem cell deficiency since February 1993. We report here the results of the first 18 transplantations with the longest follow-up periods (mean 22 months). The unmatched donor cornea was trephined in a way such that 40% of its circumference contained limbus. These grafts were then sutured centrally into the host cornea. Systemic cyclosporin A (CSA) was administered for at least 1 year after all transplantations. RESULTS: Fourteen of the 18 grafts failed during the follow-up period. The reasons for graft failure were severe surface disorders (7), severe surface disorders in combination with endothelial immune reactions (5) and endothelial immune reactions alone (2). The four patients with centrally clear grafts no longer receive systemic CSA with follow-up periods between 20 and 30 months. CONCLUSIONS: Central limbo-keratoplasty is a very promising procedure. However, the average results are not yet satisfying, because the majority of limbal stem cells undergo rejection within 2 years. Improved results will be achievable in the future by using well-matched instead of non-matched transplants and by further improving immune modulation beyond the effectiveness of current CSA treatment.

Adolescent↗
PubMed1997

[Bilateral candida endophthalmitis in 2 i. v. drug-dependent patients with oral L-methadone substitution].

BACKGROUND: Controlled therapy programs for former i.v. heroin abusers supervised by physicians offer levomethadone (L-Polamidon) as an oral substitute for heroin to allow social stabilisation of the addict. As well they reduce the risk of bloodborne infections (e.g. HIV and hepatitis) by giving up "needle sharing". Assuming that i.v. drug abuse is one of the major risk factors for Candida endophthalmitis in young, otherwise healthy patients, in case of strict oral substitution no onset of Candida endophthalmitis should be expected. CASE REPORT: The clinical courses of two HIV-negative and primarily i.v. heroin addicted young male white patients are presented. While participating in an exclusively controlled, oral program of methadone substitution for several months, both patients developed bilateral Candida endophthalmitis. CONCLUSION: A persisting i.v. abuse, either of heroin or of L-methadone, parallel to the supposingly strict orally applied substituting drug L-methadone has definitely to be suspected leading to the Candida endophthalmitis.

Administration, Oral↗
PubMed1997

[Mooren ulcer. 4 severe bilateral disease courses with systemic cyclosporin A therapy].

BACKGROUND: Mooren's ulcer is a rare autoimmunologic disease of the cornea. Many forms of medical and surgical treatments have been proposed in the past, but none of them was regularly successful. Severe progressive cases of Mooren's ulcer are therefore still a blinding disease. Only the use of systemic cyclosporin A (CSA) treatment appears for the first time to have a significant positive effect on the outcome. PATIENTS: One male (30 years old) and three female (52 y, 68 y, 84 y) patients with severe progressive bilateral Mooren's ulcer were treated with cyclosporin A systemically. RESULTS: On the male patient constant blood levels of CSA were not achievable and he became blind suffering basically from a severe proliferative diabetic retinopathy. The 52-year-old female patient got a relapse of Mooren's ulcer in the graft after penetrating keratoplasty à chaud. With increased blood levels of CSA the progression of the relapsing of Mooren's ulcer could be stopped. Also only with high-dose CSA further progression of the rapidly progressive colliquation of both corneas of a 68-year-old female could be stopped. A definitive improvement with some residual activity could be achieved in a 84-year-old female after only three months of follow-up. CONCLUSIONS: High-dose systemic CSA treatment with plasma trough levels of 150-200 ng/ml is recommended as initial treatment of choice and should be started immediately. For what period of time after clinical healing this high-dose therapy must be contained without the risk of a relapse of Mooren's ulcer remains to be seen.

Administration, Oral↗
PubMed1997

[Endogenous Aspergillus endophthalmitis in AIDS].

BACKGROUND: We present the course of an endogenous Aspergillus endophthalmitis in a 38-year-old HIV-positive man. HISTORY AND CLINICAL SIGNS: The first examination because of a sudden visual loss in the right eye showed a central subretinal infiltration, peripheral vascular sheeting and retinal hemorrhages. THERAPY AND OUTCOME: In spite of high-dose systemic antifungal therapy we could not prevent the penetration of the posterior wall of the eye and infiltration of adjacent orbital structures. Enucleation had to be performed. CONCLUSIONS: In case of scleral involvement in Aspergillus endophthalmitis the necessity of enucleation has to be considered.

AIDS-Related Opportunistic Infections↗
PubMed1997

Elevation of serum IGF-1 rather than sex steroids precedes proliferative diabetic retinopathy in Mauriac's syndrome.

We report the case of a 21-year-old man with Mauriac's syndrome, in whom diabetic retinopathy progressed to the proliferative state despite slightly improved glycemic control and completely suppressed sex steroids (by a GnRH agonist). However, a gradual increase in serum IGF-1 from 162 to 482 ng/ml over 9 months clearly preceded retinal deterioration, which responded to panretinal laser coagulation. We conclude that substantial elevation of serum IGF-1 may be another risk factor for the progression of mild diabetic retinopathy to the proliferative state.

Adult↗
PubMed1996

CNS complications in a girl with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD).

A 13-year-old girl presented with doughy swelling of both hands, a pruritic maculopapular rash, fever, malaise, lymphadenopathy and splenomegaly. Examination of an enlarged cervical lymph node revealed typical histopathological features of AILD. Partial remission was achieved by treatment with prednisolone. During the next 4 years 3 flare-ups of the disease could be controlled by corticosteroids, one going along with an enlargement of the right tonsil, histologically proven as an AILD manifestation, the other two with generalized lymphadenopathy. At the age of 17 years, an acute reduction of visual acuity occurred in both eyes in the absence of lymphadenopathy or cutaneous manifestations. Plasma viscosity was elevated in connection with high levels of IgM and IgG. Fundoscopy revealed papilledema compatible with hyperviscosity syndrome. Plasmapheresis resulted in a slow recovery of vision when suddenly a bilateral loss of vision occurred. MRI at this time revealed a lesion within the optic chiasm and additional high-signal lesions in parietal white matter of brain. All of these regions showed enhancement after i.v. application of Gd-DTPA. Again, high-dose corticosteroids were introduced and a partial restoration of vision could be achieved. This was paralleled by an improvement of the changes on followup MRI examinations. The last examination ten months after onset of CNS complications revealed a single small hyperintense residual area positioned in left parietal white matter. Enhancement of contrast medium was absent. These cerebral and retinal complications are so far undescribed complications of AILD which occurred in a childhood case.

Adolescent↗
PubMed1996

[Combination therapy of circumscribed choroid hemangioma in contralateral Sturge-Weber syndrome with acetazolamide and laser coagulation].

BACKGROUND: In Sturge-Weber syndrome choroidal hemangiomas occur in up to 55%. With a few exceptions it is a diffuse hemangioma and it always affects the eye ipsilateral to the naevus flammeus. To the best of our knowledge, a contralateral circumscribed choroidal hemangioma has not yet been described. HISTORY AND CLINICAL FINDINGS: We present a 34-year-old male caucasian with a left-sided naevus flammeus and a right-sided circumscribed choroidal hemangioma. Ipsilateral to the naevus, the left eye showed typical vascular anomalies of the conjunctiva and episclera as well as in the anterior chamber angle with anterior synechia formation and circumscribed iris atrophy without secondary glaucoma. Retinal and choroidal vasculature was normal. In the contralateral eye the anterior segment was normal, but there was a juxtafoveolar circumscribed choroidal hemangioma of about 7.5 x 4.5 mm along the superior temporal vascular arcade. The tumor was detected when vision dropped to 0.4 due to an exsudative retinal detachment over the tumor involving the fovea. THERAPY AND CLINICAL COURSE: Complete regression of the exsudative detachment occurred under oral Acetazolamide (1000 mg/d), but after dose reduction to 500 mg/d the exsudation recurred. Thereafter, the surface of the tumor was treated with argongreen grid lasercoagulation in 3 sessions under Acetazolamide cover. Following scar formation the retina has stayed completely attached even after discontinuation of oral Acetazolamide therapy with a follow-up of now 7 months. CONCLUSIONS: Visual prognosis after exsudative macula detachment is depended on rapid retinal reattachment. Therefore, early and effective treatment is mandatory. Systemic application of Acetazolamid (1000 mg/d orally) led to a rapid reattachment of the retina with an excellent visual result. However, dose-reduction was followed by an immediate recurrency. We therefore suggest to perform an Argongreen grid lasercoagulation of the tumor surface while the patient is still under systemic Aceazolamid. This strategy has proven successful in this case.

Acetazolamide↗
PubMed1996

[Treatment of toxoplasmosis retinochoroiditis with atovaquone in an immunocompetent patient].

BACKGROUND: In Central Europe ocular toxoplasmosis is the leading cause of posterior uveitis. It is a major cause of severe visual loss and blindness in young people. Drugs for treatment of active lesions (tachyzoites) have been available for decades but are seen controversial especially because of sometimes serious side effects. These drugs don't seem to shorten the active inflammation nor the recurrence rate, in particular because of the poor effect on the cystic form (bradyzoites). Atovaquone (hydroxynaphthoquinone) is well tolerated systemically and is effective against tachyzoites and bradyzoites of Toxoplasma gondii so that we hope to reduce the recurrence rate. PATIENT HISTORY AND CLINICAL FINDINGS: Two immunocompetent patients with the first and respective second symptomatic recurrence of unilateral active toxoplasmic retinochorioiditis located within the major temporal vascular arcades were treated with Atovaquone and Fluorocortolone because of an impending loss of central visual function. THERAPY AND CLINICAL COURSE: Under the treatment with Atovaquone (3 x 750 mg/day) for three weeks and tapering of the Fluorocortolone the active lesions healed quickly. After a few weeks, atrophic and remarkably little pigmented scars remained. No side effects were observed. After a period of 7 and respective 11 months no recurrence occurred. CONCLUSIONS: Atovaquone is an effective and well tolerated drug for the treatment of active ocular toxoplasmosis in immunocompetent patients. Its efficacy against tachyzoites and cysts of Toxoplasma gondii relative to other drugs remains to be determined by further clinical trials.

Adult↗
PubMed1996

[Acute band-shaped keratopathy after intraocular fibrinolysis with recombinant tissue plasminogen activator (rt-PA)].

BACKGROUND: Intraocular fibrinolysis with recombinant tissue-plasminogen activator (rt-PA) in patients with severe fibrin reactions following anterior segment surgery is widely accepted because of the low complication rate. CASES: We present two eyes of two patients developing acute bandkeratopathy within the first week after intraocular rt-PA fibrinolysis (10 mg/100 ml). In the first patient, calcium deposition occurred inferiorly of the optic axis without visual impairment. In the second patient, a dense bandkeratopathy reaching from limbus to limbus developed, with a severe decrease of vision. A corneal abrasion together with the chelating agent disodium ethylene diamine tetra-acetate (EDTA) 0.5% in neutral solution led only to an incomplete resolution of the depositions. CONCLUSIONS: Acute development of a bandkeratopathy is very rare. Several risk factors are identified so far, e.g. the use of phosphate containing eye drops. Their interactions and the possible pathomechanism are discussed in detail. The close coincidence of intraocular rt-PA fibrinolysis and acute band-keratopathy in our two patients is in favor of an at least additive causative role of rt-PA fibrinolysis. Therefore, the indication should be limited to severe fibrin reactions and local application of phosphate containing drugs should be avoided.

Aged↗
PubMed1996

Keratoplasty in newborns with Peters' anomaly.

Severe Peters' anomaly with dense corneal opacities leads to blindness of the affected eye unless perforating keratoplasty is attempted. The optimal timing of this procedure has yet to be established. We performed keratoplasty early after birth in an attempt to optimally treat amblyopia. In eight eyes of five newborns with severe Peters' anomaly a first keratoplasty was performed at an average age of 54 days. A first control was done under general anesthesia 3 weeks thereafter, with subsequent controls being carried out according to the clinical course. Immunosuppressive therapy mostly consisted of topical steroid eye drops only. In two rekeratoplasty cases, systemic cyclosporin A was given in addition. Apart from the eight primary keratoplasties, three repeat keratoplasties, two lentectomies, and numerous glaucoma operations had to be performed. The average follow-up period was 46 months. As compared with the excellent results reported for penetrating keratoplasty in adults, the results obtained in this special group of newborns remain very poor. The observation of four eyes with a clear or partially clear graft and useful ambulatory vision might suggest a success rate of 50%. However, especially secondary glaucoma seems to be the limiting prognostic factor in the long run. At present, two of the four eyes continue to show uncontrolled intraocular pressure despite multiple surgical interventions, and their prognosis is poor. The performance of perforating keratoplasty in patients with Peters' anomaly early after birth is associated with a multitude of problems, especially glaucoma, and currently grafts can rarely be kept clear for an extended period. We would therefore conclude that it might be wise to postpone surgery until the patient is about 1 year old, in the hope that the overall chance for graft survival might be better at that point, even though persistent amblyopia might be quite severe and limit the functional success.

Corneal Opacity↗
PubMed1996

Cyclosporin-A and its metabolites in the anterior chamber after topical and systemic application as determined with high-performance liquid chromatography-electrospray mass spectrometry.

Penetration of cyclosporin A (CSA) into the anterior chamber through the intact cornea after topical application is difficult due to its hydrophobic structure. Following systemic application the anterior-chamber levels of CSA are reported to be higher. CSA metabolites are more hydrophilic than CSA. Only high-performance liquid chromatography-electrospray mass spectrometry allows exact quantification of the CSA level and the identification of all CSA metabolites. We studied the anterior-chamber levels of CSA and different CSA metabolites after topical and systemic application. CSA and CSA-metabolite anterior-chamber levels were measured in 49 patients after topical application of CSA 2% eye drops preceding routine cataract surgery with 2 different application schemes and in 7 patients receiving systemic CSA after high-risk penetrating keratoplasty. After topical application the average CSA level measured in the anterior chamber was 81 ng/ml. The CSA-metabolite levels were much higher, reaching an average of 378 ng/ml. After systemic therapy the anterior-chamber levels of CSA and of the metabolites were much more balanced at 256 and 317 ng/ml, respectively. CSA penetrates into the anterior chamber after topical eye-drop application, but these levels are much lower than those measured after systemic CSA therapy. After topical application the CSA metabolites might play an important role; they are found in the anterior chamber in much higher concentrations than is CSA, and the metabolite pattern differs from that seen after systemic therapy. The relevance of these findings to the immunosuppressive activity of the CSA metabolites, however, remains unclear.

Administration, Topical↗