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Vol 18, No 2 (2023)

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Original study article

The role of plasminogen and urokinase activator of plasminogen in the treatment of endogenous uveitis in children

Katargina L.А., Chesnokova N.B., Denisova E.V., Khrabrova M.A., Beznos O.V.


AIM: To determine the content of plasminogen and urokinase activator of plasminogen in tears and blood serum and to identify correlations between the studied parameters and the clinical picture of uveitis.

MATERIAL AND METHODS: One hundred thirty-three eyes with uveitis were examined in 74 patients aged 3 to 17 yr (average 10.45±3.35 yr). The content of the urokinase activator of plasminogen (UPA) was studied in 188 tear samples and 22 blood serum samples. The dynamics of UPA in tears were studied in 28 patients (51 eyes). The plasminogen content of 86 tear samples and 34 blood serum samples was studied. The dynamics of plasminogen in tears were studied in five patients (nine eyes). The concentrations of UPA and plasminogen were measured using the ELISA method and the kits “ELISA kit for plasminogen activator, urokinase (UPA)/ELISA Kit for Plasminogen”, Cloud-Clone Corp., USA).

RESULTS: An increase in the content of UPA in the tears of children with uveitis was associated with higher inflammatory activity (p=0.04). An increase in the content of UPA in tears was associated with an increase in the degree of proliferative changes (p=0.04). An increase in the content of UPA and plasminogen in tears was found 1–2 months after surgery. There was an increase in the content of UPA (p=0.0001) and plasminogen in tears (p=0.009) and blood serum (p=0.09) with age.

CONCLUSION: The content of UPA in tears increased significantly when severe uveitis was compared with inactive uveitis. An increase in the content of UPA in tears was associated with an increase in the degree of proliferative changes, which reflects the severity of the uveitis course. The content of UPA and plasminogen in tears and blood serum increased with age. An increase in UPA and plasminogen was observed within 1–2 months after surgery, with both returning to preoperative values by the third month of the postoperative period, which reflects the normal course of the wound healing process.

Russian Pediatric Ophthalmology. 2023;18(2):57-66
pages 57-66 views

Clinical and genetic aspects of glaucoma associated with congenital aniridia

Sukhanova N.V., Katargina L.A., Panova A.Y., Zinchenko R.A.


Congenital aniridia is a hereditary congenital malformation of the visual organ with an autosomal dominant type of inheritance. The prognosis for vision largely depends on the development and progression of multiple complications (glaucoma, keratopathy, and aniridia fibrotic syndrome) at different ages.

AIM: To identify the most significant risk factors for glaucoma development and a poor prognosis associated with congenital aniridia.

MATERIAL AND METHODS: Seventy-three children (146 eyes) with PAX6-associated aniridia aged 0–16 yr were examined, with 41 males (56.2%) and 32 females (43.8%). The follow-up period of patients ranged from 2 to 6 yr. Thirty-five (47.9%) patients had complete aniridia, and 38 (52.1%) patients had partial aniridia. All patients underwent a comprehensive ophthalmological and molecular genetic examination.

RESULTS: Glaucoma developed in 28.8% of children. Anomalies in the anterior chamber angle (ACA) structure were detected in most patients with congenital aniridia, both with and without glaucoma. However, the relationship between the ACA and the timing of the manifestation of glaucoma was revealed. Furthermore, according to our study, the presence of glaucoma increases the risk of keratopathy progression.

CONCLUSION: In a molecular genetic study, the presence of deletions in the 3'-cis-regulatory region of the PAX6 gene was a predictor of glaucoma development.

Russian Pediatric Ophthalmology. 2023;18(2):67-74
pages 67-74 views

Case reports

Girate atrophy: clinical and functional features

Kogoleva L.V., Zolnikova I.V., Kokoeva N.S., Bobrovskaya J.A., Milash S.V.


Gyrate atrophy is a rare genetic metabolic disease with an autosomal recessive inheritance that causes progressive chorioretinal atrophy, fundus manifestations, and decreased visual function. The prognosis of the disease largely depends on the development and progression of complications (macular changes and cataracts) as well as concomitant neurological and somatic pathology.

AIM: To describe three clinical cases of hypertension.

MATERIAL AND METHODS: We examined three children with gyrate atrophy at 4, 10, and 15 years old. All patients underwent a comprehensive ophthalmological examination, including modern diagnostics, visualization, and electrophysiological studies.

RESULTS: Although older patients have more pronounced changes in the fundus with involvement of the macular zone in the pathological process, a 4-year-old child has pronounced functional retinal disorders detected during electroretinogram registration, indicating an earlier manifestation of the pathological process. Gyrate atrophy was combined with foveoschisis and ornithinemia in older patients (10 and 15 years old).

The differential diagnosis of gyrate atrophy should be carried out with high myopia with areas of dystrophy of the “cobblestone pavement” type on the periphery of the fundus, resembling foci of chorioretinal changes in hypertension.

CONCLUSION: Patients with gyrate atrophy require an interdisciplinary approach that includes not only ophthalmologists but also pediatricians, medical geneticists, and other specialists with comorbidities.

Russian Pediatric Ophthalmology. 2023;18(2):75-82
pages 75-82 views

Nonstandard cases of blepharoptosis in patients with subcutaneous dirofilariasis, chronic progressive external ophthalmoplegia, and blepharochalyasis of allergic origin (clinical observations)

Filatova I.A., Kondratieva J.P., Shemetov S.A., Trefilova M.S.


The medico-social significance of upper eyelid omission is associated, on the one hand, with a relatively high frequency of occurrence (up to 32% among ophthalmic patients) and, on the other hand, with a significant decrease in the quality of life of patients with blepharoptosis due to the appearance of various consequences—a deficit of the field of vision from above, functional blindness, contracture of the neck muscles, and a reduction in the psycho-emotional background of appearance changes.

Ptosis of the upper eyelid can develop from a variety of rare and even casuistic causes, including slowly progressing external ophthalmoplegia, blepharochalasis of allergic origin, and extremely rare as one of the manifestations/consequences of subcutaneous dirofilariasis. Because these diseases are rare (less than 10 cases per 100,000 population), even descriptions of individual clinical cases are important for detailing and generalizing knowledge about etiopathogenesis, the possibilities of clinical diagnostic measures, and the effectiveness of treatment for the pathologies under consideration.

AIM: Using clinical cases of blepharoptosis in patients with chronic progressive external ophthalmoplegia, subcutaneous dirofilariasis, and blepharochalasis of allergic origin to show the relationship between etiopathogenesis, upper eyelid lifting dynamometric muscle parameters, and surgical treatment methods.

MATERIAL AND METHODS: This study presents three clinical cases with different causes of the development of upper eyelid ptosis, which were performed in addition to the standard ophthalmological examination, diagnosis of the underlying disease, and dynamometric measurements of contractility and fatigue of the upper eyelid lifting muscle, followed surgical treatment methods.

CONCLUSION: Data were used in determining the contractility and fatigue of the muscle that raises the upper eyelid while deciding on a surgical treatment method for blepharoptosis. Considering the causes for the development of blepharoptosis, it is possible to obtain good results from operative correction of the upper eyelid omission, which improves their quality of life.

Russian Pediatric Ophthalmology. 2023;18(2):83-94
pages 83-94 views


Clinical features, diagnosis, and treatment of dry eye syndrome in children

Kovaleva L.A., Kuznetsova T.V., Baisangurova A.A., Zaitseva A.A.


AIM: To investigate the etiopathogenesis and clinical features of dry eye syndrome in children, methods for diagnosing the disease, and the treatment algorithm.

MATERIAL AND METHODS: One hundred eighty-seven children with dry eye syndrome aged 3 to 17 yr were examined. To diagnose dry eye syndrome in children, the following research methods were used: biomicroscopy of the anterior part of the eye under diffuse illumination and with a cobalt filter after instillation of vital dyes; examination of the height of the lacrimal meniscus; identification of a characteristic discharge in the conjunctival sac, xerosis of the ocular surface; and evaluation of tear production (Schirmer I test) and stability of the tear film.

RESULTS: We found that the links in the pathogenesis of dry eye syndrome in children were as follows: diseases of the ocular surface and appendage of the eye, contact correction of ametropia, and surgical operations on the conjunctiva and oculomotor muscles; otogenic neuritis of the facial nerve; rheumatoid arthritis; endocrine ophthalmopathy; lagophthalmos; and thermal and chemical burns. It is now always possible to evaluate tear production and tear film stability in preschool and primary school-aged children. In this case, the main diagnostic method is biomicroscopy. We identified objective clinical symptoms of dry eye syndrome in children using biomicroscopy. Dry eye syndrome was classified into four degrees of severity: mild, moderate, severe, and extremely severe. The subjective and objective clinical signs of each disease severity are described.

An individual approach and personalized therapy are required to treat dry eye syndrome in children, focusing on the individual tolerability and effectiveness of the drug. Maximum medical alertness and early and accurate clinical differential diagnosis between dry eye syndrome and infectious inflammatory pathology of the anterior part of the eye are required.

CONCLUSION: The features of the etiopathogenesis of dry eye syndrome in children with various nosologies are examined, and the characteristic clinical symptoms and severity of dry eye syndrome, diagnostic methods, and algorithm for treating dry eye syndrome in children are described. The clinical and diagnostic features of dry eye syndrome in children described by us contribute to its early diagnosis, allowing for the initiation of personalized tear replacement and reparative therapy on time, preventing the development of a chronic course of the disease, the occurrence of complications, and the preservation and/or restoration of visual acuity.

Russian Pediatric Ophthalmology. 2023;18(2):95-104
pages 95-104 views

Blepharoptosis: diagnostics and significance of dynamometry for optimizing the treatment of upper lid drooping

Filatova I.A., Kondratieva J.P., Shemetov S.A., Trefilova M.S.


Ptosis of the upper eyelid remains the most common pathology of the auxiliary apparatus of the eye in children and adults alike. Presently, there are no methods of pharmacological correction for the omission of the upper eyelid; hence, only surgical treatment is available. However, the recommended surgery has been associated with unsatisfactory outcomes in 8–26% of all patients. There are several directions of surgical treatment of blepharoptosis, depending on the main cause of its development and degree, such as operations on the muscle that raises the upper eyelid (levator resection, recession, levatoroplasty with the formation of a duplicate of the levator) and its tendons (aponeurosis); operations on the tarsal plate; and “suspension type” operations. Despite the large number of approaches to surgical treatment available for blepharoptosis they are associated with a high risk of hypo and hyper side effects. Therefore, it is not always possible to eliminate the existing changes or damage in different types of ptosis, which may raise the need for a reoperation, which is quite complicated. The standard linear methods for determining the biometric parameters of the mobility of the upper eyelid and degree of ptosis conducted in the preoperative period do not always result in good outcomes. In fact, no reliable criteria allow the prediction of the outcome of surgical treatment with a high degree of probability and planning the volume of surgery. Therefore, it is extremely likely that the addition of dynamometric analysis of the contractile activity of the upper eyelid lifting and tarsal muscles to the scheme of preoperative diagnosis of blepharoptosis, as well as the continuation and intensification of research aimed at creating the doctrine of the pathomorphology of upper eyelid prolapse in the future, will serve as the key factors contributing to the improvement of the results of surgical treatment of blepharoptosis.

Russian Pediatric Ophthalmology. 2023;18(2):105-114
pages 105-114 views

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