A case of congenital glaucoma in type I neurofibromatosis

BACKGROUND: Recklinghausens generalized neurofibromatosis (NF) is a hereditary disease characterized by the formation of benign tumors from the nervous tissue that provokes skin and bone changes of various types. Often, involvement in the pathological process and the organ of vision. The variability of eye lesions in NF-I type is known according to the clinical course, severity, and clinical forms. Gliomas and atrophy of the optic nerves, neurofibromas in the iris, sclera, conjunctiva, eyelids, less often glaucoma, buphthalmos, orbital osteodystrophy are described. RESULTS: The article describes a rare case of type I neurofibromatosis, the first clinical manifestation of congenital glaucoma. Modern complex ophthalmological examination made it possible to identify the cause and clarify the nature of the development of glaucoma. CONCLUSION: The need for an interdisciplinary approach to the diagnosis, dispensary observation, and treatment of NF-I is emphasized.

Late-onset panuveitis in a Chinese girl with sporadic Blau syndrome: a case report

The onset of ocular symptoms at age 18 is relatively rare in BS and the eye lesions are usually serious.We reported a case of a 18 year-old Chinese girl with late-onset mild panuveitis with sporadic Blau syndrome .

CRISPR-Mediated Transcriptional Repression in Toxoplasma gondii

Toxoplasma gondii is a ubiquitous intracellular parasite of humans and animals that causes life-threatening disease in immunocompromised patients, fetal abnormalities when contracted during gestation, and recurrent eye lesions in some patients. Despite its health implications, about half of the Toxoplasma genome still lacks functional annotation.

About eye lesions due to typhoid processes

Typhoid epidemic in 1919 - 20 in Kazan and its environs made it possible to observe, as its consequences, numerous cases of damage to the organs of vision, which almost exclusively resulted in the form of suffering of the vascular tract, with a predominant defeat of the choroid itself.

Chemical Weapons in the Iran-Iraq War (1980–1988) 5. Accumulated Experience in the Treatment of Lesions Caused by Sulfur Mustard

ulfur mustard was widely used during the Iran-Iraq war (1980–1988) and in other conflicts in the Middle East. Due to its availability, it can be used by terrorist organizations on the territory of the Russian Federation. Iran is the only country, against which mustard gas was used in modern warfare. The aim of this work is to analyze and summarize the experience of treating of sulfur mustard exposure, accumulated by Iranian specialists during the Iran-Iraq war (1980–1988). The UN official documents and materials, declassified CIA documents, articles of Iranian authors as well as other open sources have been used during its preparation. The Iraqi army used mustard gas in two aggregate states: liquid and dry. Dry mustard gas was a novelty of that war. It was a powdered silica steeped in mustard gas, with a particle size of less than 5 μm. The first signs and symptoms of poisoning could appear after 15 minutes, while acute poisoning effects of exposure to mustard gas vapour or liquid were typically delayed for several hours. The reasons for the severe mustard lesions of the servicemen were the lack of personal protective equipment, untimely evacuation from the mustard gas contamination zone, and the absence of mobile reserves of clean water and degassing installations in battle formations. Iranian experts consider 0.5% sodium hypochlorite solution to be the most effective decontaminant. The only safe antidote is sodium thiosulfate, administered within 60 minutes after exposure to mustard gas. During the evacuation of victims and their treatment in hospitals it is necessary, in severe cases, to insert an intravenous catheter, carry out a tracheotomy with the installation of a tracheotomy tube, and use bronchoscopy as early as possible to flush the bronchi. The treatment of skin and eye lesions should be conservative. In case of a corneal perforation, if its diameter is more than 2 mm – keratoplasty, if it is less – cyanoacrylate glue. Treatment of respiratory lesions aims to relieve symptoms and reduce the severity of complications. For this, bronchodilators, antitussives, mucolytics and, if necessary, antibiotics are used. The use of corticosteroids should be limited to the treatment of those patients with bronchospasm, who can not be treated by bronchodilators. The Iranians also consider gene therapy and treatment with mesenchymal stem cells to be the promising method used in the treatment of lung lesions

Epizootological features and clinical manifestation of cat chlamydiosis in Poltava

The article presents epizootological data and clinical signs of feline chlamydia in Poltava. According to the results of studies in 2018–2020, a wide spread of chlamydia infection among cats in the city of Poltava was established. The largest number of the studied cats, were pedigree or crossbred. There was no explicit clear seasonality, but a greater number of cases of respiratory diseases were recorded in the autumn-winter period of the year. In the structure of infectious diseases during the research period, chlamydia mono-infection was registered in 68 % of cats, association of chlamydia with infectious rhinotracheitis in 18.5 %, kalitsivirus infection in 8.6 %, with panleukopenia in 4.9 %. The greatest number of the registered cats had acute and subacute course of chlamydia. In the acute course, there was an increase in temperature, oppression and refusal to eat. In the majority of cases of acute, subacute and associated chlamydia, the structures of the eye were damaged. Conjunctivitis, keratitis, chemosis, uveitis, third eyelid inflammation, changes in the shape of the pupil developed. A Sphynx breed cat showed signs of acute chlamydial arthritis with pronounced inflammatory edema. The latent course was accompanied by abortions, which were mainly registered in young cats. Animals older than four years more often showed stillborn and weak kittens. The main clinical signs of chlamydia in kittens born from sick cats were eye lesions: swelling of the conjunctiva, its reddening and secretion of a significant amount of brown purulent exudate with the viscosity of the catarrh and as a consequence of eyelid adhesion and complications as ankyloblepharon or symblepharon. Submandibular and other lymph nodes in the head area were significantly enlarged. Often pus dried up near the mouth or nostrils. Pus crusts accumulated in the nasal cavity and prevented breathing. Gastrointestinal tract disorders were observed in some cases. Part of the affected litter died within the first three days after birth. Neonatal chlamydia was more often manifested by conjunctivitis, keratitis, panophthalmitis and growth retardation. On the second or third day after birth, the kittens showed weakness and lack of appetite. In two cases of neonatal chlamydia, bronchopneumonia developed. When chlamydia and infectious rhinotracheitis were associated, the structures of the eye were affected in all cases. These lesions could be different and depended on the stage of the infectious process.

Clinical and Forensic Aspects of the Different Subtypes of Argyria

Argyria encompasses the different cosmetic alterations that can develop if enough silver particles deposit in a specific tissue, typically in the skin, ranging from localized dark-blue macules to a generalized slate-gray/bluish tinge following systemic absorption. This work aims to fully review the state of the art regarding pathophysiology, diagnosis, treatment, and relevant clinical and forensic features of argyria. Argyria has been diagnosed in a wide range of ages, both sexes and varied ethnicities, with no known individual predisposing factors. Ultraviolet radiation with subsequence increases of melanin production aggravates the discoloration due to a reduction in the silver deposits. Physical examination and silver exposure in the anamnesis can be highly suggestive of the diagnosis, but a histopathological analysis with Energy-Dispersive X-ray Spectroscopy is required to unequivocally determine the discoloration etiology. Safe and effective treatment has only been accomplished with laser techniques, though only a few cases have been reported and with limited follow-up time. In conclusion, argyria typically has an occupational or iatrogenic etiology. It should be suspected when a patient presents with typical skin or eye lesions. A seemingly viable treatment modality, with laser technology, is finally within the horizon.

Successful Pregnancy in a Patient with Infertility Due to Congenital Plasminogen Deficiency Treated with Intravenous Plasminogen (Human) Replacement Therapy

Congenital plasminogen deficiency (C-PLGD) is a rare autosomal recessive multisystem disorder of the fibrinolytic system that is often underdiagnosed. It is caused by mutations in the PLG gene and is characterized by extravascular fibrinous deposits on mucus membranes resulting in tissue injury or organ dysfunction. The abnormal accumulation or growth of fibrin-rich pseudomembranous lesions on mucous membranes, such as the conjunctiva, gingiva, linings of airways and genitourinary tract have been termed ligneous for their "woody" appearance. Ligneous conjunctivitis is the most common manifestation of C-PLGD occurring in greater than 80% of affected patients. Ligneous lesions affecting the female reproductive system have been reported in one large case series in 9% of patients (Shuster et. al. JTH 2007). An extensive review of the literature (Baithun et. al. Haemophilia 2018) identified 30 cases of C-PLGD patients with gynecologic involvement. A diagnosis of infertility, when performed, was reported in 8 (26.7%) of these females of child-bearing age. Due to the rarity of the disease, infertility may be under diagnosed and under reported in the literature as no natural history studies of this disease have been conducted. We report a case of a patient with C-PLGD with a documented diagnosis of infertility who received intravenous plasminogen therapy. Although the protocol stated that adequate contraceptive measures be taken to avoid becoming pregnant during the study, she became pregnant, went to term and had a successful delivery of a healthy child. She was a 33-year-old woman with a diagnosis of C-PLGD in childhood, after developing ligneous conjunctivitis before the age of 1, developed uterine ligneous lesions and dysmenorrhea as a teenager. At age 27, a hysteroscopy was attempted and laparoscopy was performed which revealed fibrosis and web-like fibrin-rich adhesions in the uterus and fallopian tubes. She was referred to the Oslo University Hospital, Norway, for evaluation and management of her infertility and eye lesions at the age of 28. She was treated with fresh frozen plasma (FFP) infusions with improvement in eye symptoms and dysmenorrhea. At the age of 29, a vaginoscopy and hysteroscopy were performed which again demonstrated fibrin membranes in the uterus. A repeat evaluation at the age of 30 revealed fibrin membranes in the cervix, fibrin accumulation in the uterus, and tube ostia obstructed. At age 33 she was enrolled in a clinical trial, an open-label phase 2/3 study of human Glu-plasminogen administered intravenously at 6.6 mg/kg every 2-4 days in 15 patients with plasminogen deficiency, registered at www.clinicaltrials.gov as #NCT02690714. She received intravenous plasminogen replacement therapy, starting in October 2016. At baseline, she had ligneous conjunctivitis and gynecologic lesions with regular, but atypical menstrual bleeding, discharging very small amounts of thick, mucus-like material over 1-2 days. Her eye lesions had resolved by week 12 of treatment. At the end of the first menstrual cycle on plasminogen treatment, she discharged a large mucus-like plug from the vagina, and had regular and normal menstrual bleeds after that. Approximately 20 weeks into treatment, she reported being pregnant to the investigator. After discussion with the clinical study sponsor, regulatory agencies and the regional ethical committee, the patient and her physician, it was agreed to continue plasminogen replacement therapy through the pregnancy, delivery and recovery period. After approximately 55 weeks into the study treatment, the patient had an uncomplicated pregnancy and delivered a healthy child with APGAR score of 9. The patient remained on PLG treatment post-partum, and her PLG activity trough levels were maintained until she came off the study when her levels went back to baseline. (Figure 1) This is the first reported case of intravenous plasminogen used in a patient with C-PLGD and documented infertility who subsequently became pregnant and delivered a healthy child. Figure Disclosures Parker: Liminal BioSciences: Current Employment, Current equity holder in publicly-traded company. Trybul:Liminal BioSciences: Current Employment, Current equity holder in publicly-traded company. Sandset:Liminal BioSciences: Other: Investigator Clinical Trial.

Bacterial canker of tomato: revisiting a global and economically damaging seedborne pathogen

The Gram-positive actinobacterium Clavibacter michiganensis is the causal agent of bacterial canker of tomato, an economically impactful disease with a worldwide distribution. This seedborne pathogen systemically colonizes tomato xylem leading to unilateral leaflet wilt, marginal leaf necrosis, stem and petiole cankers, and plant death. Additionally, splash dispersal of the bacterium onto fruit exteriors causes bird’s-eye lesions, which are characterized as necrotic centers surrounded by white halos. The pathogen can colonize developing seeds systemically through xylem and through penetration of fruit tissues from the exterior. There are currently no commercially available resistant cultivars, and bactericidal sprays have limited efficacy for managing the disease once the pathogen is in the vascular system. In this review we summarize research on epidemiology, host colonization, the bacterial genetics underlying virulence, and management of bacterial canker. Finally, we highlight important areas of research into this pathosystem that have the potential to generate new strategies for prevention and mitigation of bacterial canker.