Abstract:
BACKGROUND:Several enzyme defects of the galactose pathway may lead to cataract formation. We report on a family with familiar cataract. PATIENTS:A 2-year-old Turkish girl (daughter of first cousins) presented with dense cortical and subcapsular opacifications and mature cataract respectively. Bilateral phacectomy, planned posterior capsulotomy, transpapillary vitrectomy and implantation of a posterior chamber lens were performed. The child was otherwise healthy and the pregnancy had been unremarkable. The 25-year-old mother showed circumscribed drop-like opacities of the lens cortex bilaterally, the 5-year-old sister a diffuse opacification of the lens cortex in both eyes, the 27-year-old father and the 13-year-old uncle clear lenses. RESULTS:The girl's level of galactitol was elevated to 2.8 nmol/ml in the plasma (normal values 0.25-1.13 nmol/ml) and to 3.1 nmol/mg protein in the lens (normal values 0.5-1.7 nmol/mg protein). The levels of galactose-1-phosphate in RBC and sorbitol in plasma were in the normal range. The enzyme activities of galactokinase, galactose-1-phosphate uridyl transferase, UDP-galactose epimerase and sorbitol dehydrogenase in RBC, as well as the sorbitol dehydrogenase activity in the lens were in the normal range. The sister and the uncle both had slightly elevated plasma galactitol levels. CONCLUSIONS:Cataract-formation in this family is most likely due to a defect in the galactitol pathway, e.g. cataract in galactosemia without known enzyme defect (Shin-Jakobs disease). In patients with unexplained congenital or infantile cataracts, disorders of the polyol pathway should be thoroughly checked for to ensure a therapeutic diet if necessary.
journal_name
Klin Monbl Augenheilkdjournal_title
Klinische Monatsblatter fur Augenheilkundeauthors
Budde M,Gusek-Schneider GC,Jünemann A,Jansen F,Shin YSdoi
10.1055/s-2008-1034710subject
Has Abstractpub_date
1999-10-01 00:00:00pages
255-7issue
4eissn
0023-2165issn
1439-3999journal_volume
215pub_type
杂志文章abstract::Non-penetrating glaucoma surgery was primarily developed as an alternative to the widely applied trabeculectomy. Since the anterior chamber in non-penetrating surgery is not directly opened, common postoperative complications such as hypotony are rare. The most frequently applied technique in this group is the deep sc...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,随机对照试验
doi:10.1055/s-0033-1351060
更新日期:2014-06-01 00:00:00
abstract::Using the CANON CG 6 60 Z wide-angle fundus camera, fluorescein angiographic examinations of the fundus periphery were performed on 90 diabetic patients presenting with exudative diabetic maculopathy. The peripheral angiogram demonstrates that perimacular hyperpermeability - the main cause of diabetic maculopathy - is...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1050901
更新日期:1985-03-01 00:00:00
abstract:BACKGROUND:The aim of this study was to evaluate retrospectively the clinical outcome of retinal photocoagulation of age-related juxtapapillary choroidal neovascularisation (CNV) with macular oedema. Juxtapapillary CNV represents a rare form of extrafoveal CNV in age-related macular degeneration (AMD). PATIENTS AND ME...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0031-1273430
更新日期:2011-11-01 00:00:00
abstract:BACKGROUND:Long-term follow-up and angiographic findings of serpiginous choroiditis, a chronic disease with recurrences of unknown origin, are reported. PATIENTS AND METHODS:A retrospective analysis of the data of 4 patients with serpiginous choroidopathy, who were treated in the eye clinic of Mainz in the past 10-18 ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2006-926575
更新日期:2006-05-01 00:00:00
abstract::During the last four years 232 macrodacryocystographies have been performed. The results are compared with the clinical evaluation of these patients. There are two groups of findings, simple stenosis on one side and anatomical abnormalities on the other side. The stenosis is mostly located before the lacrimal sac and ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1055193
更新日期:1982-09-01 00:00:00
abstract::The panel of therapeutic options available for the treatment of retroviral disorders has expanded explosively in recent years with the result that it has now become possible to reconstitute the immune function of individuals with advanced HIV-disease to a remarkable degree. The ophthalmologist is thus confronted with ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-2000-10520
更新日期:2000-02-01 00:00:00
abstract::Forty eyes were studied in order to establish the relative function of the upper and lower lacrimal ducts. Following artificial occlusion first of the upper and then of the lower lacrimal ducts. Schirmer's No. 1 test was performed. The wetting of the test strips was used as an indirect measure of tear discharge. Highe...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054425
更新日期:1984-02-01 00:00:00
abstract:BACKGROUND:Rapid advances in digital imaging technology that characterised the early part of the 21 (st) century have caused modern hospital information systems (HIS) to face a big challenge. One of the key goals of their reorganisation is integration based on open standards in order to achieve optimal interoperability...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1027722
更新日期:2008-12-01 00:00:00
abstract::In an attempt to determine the accuracy of ultrasound biometry, the spherical equivalent of the refraction obtained after ECCE and implantation of a posterior chamber IOL in 1663 patients was compared to the desired refraction determined prior to surgery. Follow up time was 21 months on the average. In only 7.4% of th...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1046055
更新日期:1991-08-01 00:00:00
abstract:BACKGROUND:Morbus Morbihan is a rare complication of rosacea, consisting of a persistent lymphoedema of the upper part of the face. It has typically a chronic course, unspecific histopathological findings and an extreme refractoriness to therapy. PATIENTS AND METHODS:Between February 2008 and January 2010, 5 patients ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0029-1245960
更新日期:2011-01-01 00:00:00
abstract:PURPOSE:Sturge-Weber syndrome is a rare congenital disorder consisting of cutaneous, leptomeningeal and ocular vascular malformations. Glaucoma occurs in 30-70 % of patients. Treatment is challenging due to a high risk of severe complications such as expulsive haemorrhage, massive choroidal effusion and serous retinal ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章,评审
doi:10.1055/s-0032-1315217
更新日期:2012-10-01 00:00:00
abstract::In a prospective clinical study covering 41 eyes (28 patients) with narrow-angle glaucoma the central and peripheral anterior chamber depths were determined biomicroscopically before and after Nd:YAG laser iridectomy. In 29 eyes the central anterior chamber depth was also measured by ultrasonography. In contrast to th...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1046142
更新日期:1990-03-01 00:00:00
abstract::Using the laser flare-cell meter (LFMC), we have previously determined the intensity and pattern of post-ALT ocular inflammation. Inflammation peak occurs 48 hours after ALT and clinically relevant inflammation is seen in 100% of pigmentary glaucomas (PIG), 75% of pseudoexfoliative glaucomas (PEXG) but only in 25% of ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2008-1045765
更新日期:1992-05-01 00:00:00
abstract:BACKGROUND:Earlier studies in monkeys have shown that latanoprost 0.005% lowers the IOP by improving the uveoscleral Outflow. We wanted to know if this is also the case in the human eye. PATIENTS AND METHODS:We used our new aqueous humor outflow test with 2-nitrophenyl-acetate in 9 healthy human volunteers, mean age 3...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1034877
更新日期:1998-05-01 00:00:00
abstract::In 21 patients with macular hole and retinal detachment a macular buckling procedure was performed including diathermic or cryogenic coagulation. An absorbable fibrin sponge (Fibrospum) was used as a new buckling material, which needs not to be sutured to the sclera at the posterior pole. In comparison to other proced...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-08-01 00:00:00
abstract::Fifty patients with open-angle glaucoma were treated twice daily for one year with topical 0.5% levobunolol, 1% levobunolol, or 0.5% timolol. Both concentrations of levobunolol were as effective as timolol in reducing intraocular pressure over the one-year period. At the concentrations tested, levobunolol and timolol ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2008-1054395
更新日期:1985-12-01 00:00:00
abstract::A rare form of malignant large-cell non-Hodgkin's lymphoma or reticulum cell sarcoma is confined to the eye and central nervous system (CNS). We report the case of a 62-year-old man, who presented with recalcitrant progressive uveitis in his right eye. Although lymphoma was suspected, the patient refused to undergo vi...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1045892
更新日期:1992-09-01 00:00:00
abstract:BACKGROUND:The number of medical screenings is increasing. This fact is specially pronounced in the field of occupational health. Due to restricted financial resources and limitations in time there is a need for rationalisation in medical screenings. MATERIALS AND METHODS:Possible uses of personal computers (PC) enabl...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1035476
更新日期:1995-05-01 00:00:00
abstract:BACKGROUND:The laser flare meter FM-500 (Kowa, diode laser, 670 nm) is a simplified version of the laser flare-cell meter FC-1000 (Kowa, He-Ne laser, 632.8 nm). the aim of this study was to compare flare measurements of the laser flare meter FC-500 with the established laser flare-cell meter FC-1000 and to determine th...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1055/s-2008-1035284
更新日期:1996-08-01 00:00:00
abstract::The authors describe some modifications of the surgical technique of external dacryocystorhinostomy. The preliminary exploration methods and their evaluation are considered. An arched skin incision is used for a more anatomical dissection to approach the lacrimal sac. The following bone trepanation is then made with b...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1975-01-01 00:00:00
abstract::A brother and a sister with DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy, deafness, etc.) are described. The 15-year-old girl was suffering from severe optic atrophy, severe sensorineural hearing loss but only slight diabetic retinopathy. The 16-year-old boy presented with symptoms which were...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054578
更新日期:1984-08-01 00:00:00
abstract::Report on a leiomyoma of the ciliary body in a 12-year-old girl. The clinical findings were misinterpreted as a traumatic ciliary body cyst because of the associated iridodialysis, the fact that the tumor could be transilluminated, because it appeared cystic on ultrasonography, and because there was a history of sever...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1979-12-01 00:00:00
abstract::Description of a new holder for experimental intraocular surgery on human autopsy eyes. Stability of shape and position of the experimental eyes is achieved by a vacuum surrounding the posterior segment. ...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1976-03-01 00:00:00
abstract::The authors report on 120 patients who underwent iridencleisis (155 eyes). Of these, 36 had open-angle glaucoma, 26 congestive glaucoma and 54 were suffering from acute glaucoma of long standing. Two patients had exfoliation syndrome and two, secondary glaucoma. The kind of filtration bleb, postoperative complications...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1054765
更新日期:1983-04-01 00:00:00
abstract::The small degree of morbidity and a better postoperative visual acuity are the advantages of pneumatic retinopexy in comparison to encircling procedures in the treatment of retinal detachment. The injection of air or gas into the vitreous cavity can theoretically induce a cataract postoperatively. Two months after pne...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2008-1046024
更新日期:1991-06-01 00:00:00
abstract::The author describes the genuine indications for the preventive treatment of retinal detachment. He was above all guided by the minute observations of the eye fundus of two eyes with the Goldmann-glass. The author's long experience and his auto-observation of the retinal detachment operation and of preventive treatmen...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:
更新日期:1977-06-01 00:00:00
abstract:PURPOSE:We report the clinical, morphological, and ultrastructural findings of 13 consecutively explanted opacified Hydroview(R) (hydrogel) intraocular lenses (IOLs). Our purpose was to provide a comprehensive account on the possible factors involved in late postoperative opacification of these IOLs. PATIENTS AND METH...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 临床试验,杂志文章
doi:10.1055/s-0028-1109319
更新日期:2009-04-01 00:00:00
abstract:BACKGROUND:The present study investigates how often a simultaneous fluorescein and indocyanine green (ICG) angiography had therapeutic consequences and if it is useful as a clinical routine diagnostic tool. PATIENTS AND METHODS:502 consecutive simultaneous angiographies in eyes with exudative macular degeneration were...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2005-858011
更新日期:2005-03-01 00:00:00
abstract:PURPOSE:The purpose of this study was to assess the vasoconstrictor effect of the prostaglandin F2 alpha analog latanoprost in isolated porcine ciliary arteries. MATERIAL AND METHODS:With a myograph system to measure isometric forces, porcine ciliary arteries were exposed, in a cumulative manner, to increasing concent...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-2001-15904
更新日期:2001-05-01 00:00:00
abstract:BACKGROUND:The evaluation of the anterior ocular chamber sections along the optical axis/axis of vision has been carried out using single-line partial coherence interferometry for many years. Now, new technologies such as the Scheimpflug-technique of the Pentacam (Oculus Optikgeräte GmbH, Wetzlar) and the optical coher...
journal_title:Klinische Monatsblatter fur Augenheilkunde
pub_type: 杂志文章
doi:10.1055/s-0028-1109367
更新日期:2009-06-01 00:00:00