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The authors conducted a study in a family pedigree comprising 33 patients (men 16, women 17). In this pedigree there coexisted patients with progressive external ophthalmoplegia and corneal lattice dystrophy. Two patients with progressive external ophthalmoplegia and ten with lattice corneal dystrophy were found. One of our patients (propositus) suffered from both diseases. Our study proves that, in this pedigree, progressive external ophthalmoplegia and corneal lattice dystrophy have an autosomal dominant mode of inheritance.

Over a period of 6 years, 23 patients (4 males and 19 females: mean age 56 years) who presented dry eyes developed 33 sterile corneal ulcers. Treatment included occlusion of the eyes or bandage soft contact lenses, prophylactic topical administration of antibiotics, punctal occlusions and currently available tear substitutes. Seventeen eyes healed completely without any corneal opacity and 10 eyes healed with opacity. Nine of the 33 eyes developed microbial keratitis. The causes of microbial keratitis were Staphylococcus aureus in 7 cases, beta-hemolytic Streptococcus in one and Pseudomonas aeruginosa in one case. The microbial keratitis was treated with intensive topical antibiotics. In 6 eyes, corneal perforation occurred. Rheumatoid arthritis coexisted in four cases.

Sterile corneal ulceration is a serious complication in patients with keratoconjunctivitis sicca. The records of 134 patients, 19 males and 115 females, who presented with dry eyes in the Ophthalmologic Clinic were reviewed. Over a period of 6 years, 33 eyes of 23 (17%) patients developed a sterile corneal ulcer. The etiologies of dry eyes in these patients were: Primary Sjogren's syndrome: 10 cases, rheumatoid arthritis: 5 cases, ocular pemphigoid 6 cases, atopy: 1 case, local irradiation: 1 case. Patient's age and sex were not significantly associated with ulcer development (p greater than 0.05). The presence of a major underlying disease was the major contributing factor. Appropriate local treatment and patient compliance were also contributing factors. Blepharitis was found in 90% of patients.

The authors undertook a clinical and genetic study in a large family with the aim of identifying the mode of inheritance of Fabry syndrome and congenital ptosis. These two types of pathology were present to varying extents. The family pedigree consisted of 95 individuals, spanning 5 generations. Three individuals (males) were found to have Fabry syndrome and 14 (males and females) congenital ptosis. The patients with Fabry syndrome also had congenital ptosis. According to these results, Fabry syndrome is inherited by an X-linked recessive mode and congenital ptosis by an autosomal dominant mode.

The authors present a clinical, epidemiologic and genetic study of juvenile macular dystrophy (Stargardt's disease) in a large kindred from Epirus in Greece. The family tree consists of 372 individuals spanning six generations over more than a century. 257 are direct descendants of the founding couple. Nineteen individuals were found to suffer from Stargardt's disease, thirteen of whom are still alive today. Segregation analysis of the data showed that in this pedigree, the disease is transmitted with the autosomal dominant mode of inheritance.

The authors have accomplished an epidemiological clinical and genetic study on primary open-angle glaucoma among 411 persons in the North Western district of Greece (Epirus), belonging to 4 genealogical trees. 112 of the 411 persons were offsprings aged 30 years or more, alive or dead, and they had direct blood-relation to the propositus. 35 of these offsprings were suffering from primary open-angle glaucoma. The classification and the number of the affected subjects suggested that one major gene, expressed by the autosomal dominant character, is the main factor for the heredity of primary open-angle glaucoma in Epirus-Greece.