Potential blindness from nutritional xerophthalmia in autistic patients.

BACKGROUND: Vitamin A is vital to retinal rod function and epithelial cell differentiation. Although uncommon in the developed world, vitamin A deficiency (VAD) secondary to poor diets or gastrointestinal disease has been reported and can lead to xerophthalmia, which is characterized by night blindness and a spectrum of ocular surface changes. Patients with autism spectrum disorder have been shown to have restrictive diets secondary to sensory issues leading to rejection of foods except for those of certain color or texture. METHODS: We present a case series of 6 pediatric patients with autism who developed varying degrees of xerophthalmia due to VAD, which resulted from restrictive eating. RESULTS: All patients presented with a history of eye irritation that was not relieved by antibiotic or allergy eye drops. Further questioning revealed they had restrictive diets consisting of only or mostly white and tan foods, and serum vitamin A testing confirmed severe VAD. Most stages of xerophthalmia were completely reversed with vitamin A supplementation, but in 2 patients more advanced xerophthalmia resulted in irreversible blindness and ocular damage. CONCLUSIONS: Both pediatricians and pediatric eye care providers must be vigilant for VAD as an etiology of eye irritation, photophobia, or new-onset visual impairment in autistic children. A review of the child's diet must be implemented as a standard part of routine history taken in this vulnerable population. Early identification and vitamin A supplementation can prevent irreversible ocular compromise and vision loss.

Recognizing vitamin A deficiency: special considerations in low-prevalence areas.

PURPOSE OF REVIEW: Although vitamin A deficiency (VAD) is rare in well resourced countries, there is a growing trend of VAD in at-risk pediatric populations. Early diagnosis is critically important to prevent its associated morbidity and mortality. This review highlights key lessons for evaluation, diagnosis, and management of children with xerophthalmia in the United States. It synthesizes the latest findings from the literature on the pathophysiology, epidemiology, risk factors, evaluation, and management of VAD in low-prevalence areas. RECENT FINDINGS: Vitamin A is crucial for maintaining the functional integrity of the eye, immune system, skin, and mucous membranes. Despite the scarcity of VAD in developed countries, there are increasing reports of VAD in at-risk children, including those with autism spectrum disorder and gastrointestinal conditions. There is a broad range of manifestations of VAD, posing a diagnostic challenge. Familiarity with the variable presentations of VAD and having a high index of suspicion in at-risk populations can aid in its early diagnosis. Systemic vitamin A supplementation and a multidisciplinary approach are important components of the management of VAD. SUMMARY: Even in well resourced countries, VAD should remain on the differential in patients with risk factors who present with relevant signs and symptoms. Early diagnosis and appropriate involvement of a multidisciplinary care team can help prevent morbidity and mortality associated with VAD.

Xerophthalmia due to vitamin A deficiency following Frey's procedure for chronic calcific pancreatitis.

We report a rare presentation of xerophthalmia due to partial pancreatic exocrine insufficiency following Frey's surgery (pancreatic resection) in a 12-year-old girl. The child had undergone this surgery for chronic calcific pancreatitis 3 years before and presented with ocular irritation and decreased vision of 3 months duration. Ocular examination showed severe conjunctival and corneal xerosis. Her serum retinol levels and 25-hydroxyvitamin D were tested and were extremely low. The condition rapidly reversed following high-dose replacement therapy with vitamin A and D. This case highlights the importance of continuous enzyme replacement therapy as well as dietary modification and nutritional supplement therapy and monitoring of ocular symptoms in post-pancreatic surgery.

Aucuba japonica Extract and Aucubin Prevent Desiccating Stress-Induced Corneal Epithelial Cell Injury and Improve Tear Secretion in a Mouse Model of Dry Eye Disease.

Dry eye disease is affected by a broad range of causes such as age, lifestyle, environment, medication and autoimmune diseases. These causes induce tear instability that activates immune cells and promotes expression of inflammatory molecules. In this study, we investigated the therapeutic effects of an ethanolic extract of Aucuba japonica (AJE) and its bioactive compound, aucubin, on dry eye disease. The human corneal cells were exposed to desiccation stress induced by exposing cells to air, so that viability was decreased. On the other hand, pre-treatment of AJE and aucubin restored cell survival rate depending on the dose under the dry condition. This result was confirmed again by terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) staining. The mRNA expression of inflammatory molecules was reduced by the pretreatment of AJE and aucubin under the dry state. The therapeutic effects of AJE and aucubin were examined in the animal model for dry eye induced by unilateral excision of the exorbital lacrimal gland. Declined tear volumes and corneal irregularity in the dry eye group were fully recovered by the administration of AJE and aucubin. The apoptotic cells on the cornea were also decreased by AJE and aucubin. Therefore, this study suggests that administration of AJE can be a novel therapeutic for dry eye disease and that the pharmacological activities of AJE may be in part due to its bioactive compound, aucubin.

Anti-Inflammatory and Anti-Apoptotic Effects of Acer Palmatum Thumb. Extract, KIOM-2015EW, in a Hyperosmolar-Stress-Induced In Vitro Dry Eye Model.

The aim of this study was to assess the anti-inflammatory and anti-apoptotic effects of KIOM-2015EW, the hot-water extract of maple leaves in hyperosmolar stress (HOS)-induced human corneal epithelial cells (HCECs). HCECs were exposed to hyperosmolar medium and exposed to KIOM-2015EW with or without the hyperosmolar media. Tumor necrosis factor (TNF)-α, interleukin (IL)-1ß, and IL-6 production and apoptosis were observed, and the activation of mitogen-activated protein kinases (MAPKs) including extracellular signal regulated kinase (ERK), p38 and c-JUN N-terminal kinase (JNK) signaling and nuclear factor (NF)-κB was confirmed. Compared to isomolar medium, the induction of cell cytotoxicity significantly increased in HCECs exposed to hyperosmolar medium in a time-dependent manner. KIOM-2015EW-treatment significantly reduced the mRNA and protein expression of pro-inflammatory mediators and apoptosis. KIOM-2015EW-treatment inhibited HOS-induced MAPK signaling activation. Additionally, the HOS-induced increase in NF-κB phosphorylation was attenuated by KIOM-2015EW. The results demonstrated that KIOM-2015EW protects the ocular surface by suppressing inflammation in dry eye disease, and suggest that KIOM-2015EW may be used to treat several ocular surface diseases where inflammation plays a key role.

Combined effect of traditional Chinese and Western medicine on inflammatory factors in patients with diabetes-induced xerophthalmia.

Diabetes-induced xerophthalmia is a general metabolic disorder with high incidence and increased treatment difficulty. Our study aimed to explore the combined effect of traditional Chinese and Western medicines on diabetes-associated xerophthalmia. We recruited 60 diabetic xerophthalmia patients, and randomly assigned them to either the control (Western medicine treatment) or the experimental (combined treatment of traditional Chinese medicine and Western medicine) groups. Pre-treatment and post-treatment analyses were performed to assess the combined therapeutic effect of traditional Chinese and Western medicine on xerophthalmia-associated indicators. We found that the experimental group expressed reduced levels of IL-1, IL-8, and TNF-α (P < 0.05) as compared to the control group. Furthermore, the experimental group showed higher treatment efficacy as compared to the control group (85.00 vs 51.67% Z = 22.244, P < 0.05). In addition, break-up time (t = 20.582, P < 0.05) and tear section (t = 23.082, P < 0.05) was increased in the experimental group as compared to the controls. Lastly, it was found that the combined treatment of traditional Chinese and Western medicine effectively reduced corneal injuries, as indicated by reduced fluorescein staining. This study suggested that a combination treatment consisting of both traditional Chinese and Western medicines may be effective against xerophthalmia in diabetes, and that inflammatory factors are potential biomarkers to examine the treatment efficacy.

Keratomalacia in a Patient With Psychogenic Vitamin A Deficiency.

PURPOSE: To report the clinical and histopathological findings of a patient with bilateral keratomalacia arising from severe vitamin A deficiency from panic disorder-related malnutrition. METHODS: Case report. RESULTS: A 47-year-old male with panic disorder presented with 1 month of painful vision loss sequentially affecting the right and left eyes. He exhibited bilateral conjunctival xerosis with complete corneal melt in the right eye and a large corneal epithelial defect with underlying anterior chamber inflammation in the left eye. Laboratory investigation revealed undetectable serum vitamin A levels attributed to self-induced vomiting and starvation. He was treated with high-dose vitamin A, but the right eye required enucleation. The histological findings are reported. CONCLUSIONS: Vitamin A deficiency in the absence of organic gastrointestinal abnormalities is exceedingly rare in the developed world. A strong index of suspicion and thorough review of systems are invaluable in evaluating patients with unexplained corneal melt.

Herpetic keratouveitis mixed with bilateral Pseudomonas corneal ulcers in vitamin A deficiency.

A 56-year-old woman complained of blurred vision and pain in her right eye for several days. Slit lamp examination revealed a large epithelial defect and disciform stromal edema with ring infiltration in her right cornea. Unfortunately, hypopyon and purulent discharge subsequently developed in both eyes. Herpetic keratouveitis and a superimposed pseudomonas infection were diagnosed. A systemic review on the patient showed malnutrition due to her dietary preference and vegetarianism. After the infection was controlled, bilateral epithelial defects persisted for a long time. We performed amniotic membrane transplantation on both eyes and the clinical status improved with administration of vitamin and protein supplements. Although rare in Taiwan, vitamin A deficiency should be kept in mind when conjunctival and corneal xerosis occurred. Vitamin A supplements are suggested because of the increased susceptibility to infection in patients with this clinical status.

Xerophthalmia and keratomalacia secondary to diet-induced vitamin A deficiency in Scottish adults.

OBJECTIVE: To report the ocular features, underlying systemic conditions, and management of diet-induced vitamin A deficiency in Scottish adults. DESIGN: Retrospective case series. METHODS: Three middle-aged white adults presented with ocular signs of xerophthalmia and keratomalacia that led to the diagnosis of previously undiagnosed vitamin A deficiency. Case 1 presented with rapidly progressing bilateral keratomalacia with corneal perforation, whereas Cases 2 and 3 presented with conjunctival and corneal xerosis. RESULTS: Corneal gluing was performed in Case 1. Cases 2 and 3 were treated topically with preservative-free tears, retinoic acid, and punctual plugs to optimize the ocular surface. This, combined with dietary supplementation, resulted in normalizing the ocular surface. CONCLUSIONS: Diet-induced vitamin A deficiency can occur in middle-aged white adults. Ocular surface changes may be the presenting sign of hypovitaminosis A in the Western world. A high degree of suspicion with prompt recognition and management of the underlying dietary deficiency can lead to complete reversal of the ocular surface changes.

Xerophthalmia--a potential epidemic on our doorstep?

PURPOSE: Xerophthalmia refers to the ocular manifestations associated with vitamin A deficiency, including xerosis, keratomalacia, nyctalopia and Bitot's spot. Hypovitaminosis A is well-recognised in developing countries, but is rare in the developed world. Most cases in the latter relate to fat malabsorption. Conditions in which vitamin A metabolism or storage is deranged (chronic liver disease, including alcoholism) are also aetiologies. We wanted to see whether this was common in our department. METHODS: Oral vitamin A supplements were given to patients who presented with hypovitaminosis A. RESULTS: All patients were found to have hypovitaminosis A on biochemical testing and responded dramatically to oral vitamin A supplementation, resulting in an improved final visual outcome. DISCUSSION: This series demonstrates that prompt recognition and treatment of xerophthalmia can lead to rapid recovery and avert significant visual morbidity. The prevalence of xerophthalmia is likely to increase in the developed world largely owing to alcoholic liver disease. It is thought by some that we are on the verge of a potential epidemic. We hope that by increasing the profile of this important public health issue, we may be able to influence future prevalence of hypovitaminosis.

Xerophthalmia and acquired night blindness in a patient with a history of gastrointestinal neoplasia and normal serum vitamin A levels.

A 69-year-old male patient presented to our department with a 3-month history of nyctalopia. Reviewing of his general health revealed a history of gastrointestinal tumor treated with a modified WHIPPLE operation. Ocular findings at presentation included mild xerophthalmic features and nonspecific pigmentary retinal changes. A standard full-field electroretinogram (ERG) was obtained that showed normal photopic function and extinguished scotopic function. The ocular symptoms, the history and the ERG findings suggested vitamin A deficiency as a possible cause for his complaints. Serum vitamin A levels were subsequently requested, but the results were within normal limits. Despite the normal serum vitamin A levels, the patient was instructed to commence treatment with high doses of oral vitamin A supplements. One month after the onset of the treatment, the patient reported that his visual function has significantly improved, while repeat ERG testing revealed that scotopic function has improved to normal levels. This case highlights that in patients with acquired night blindness due to vitamin A deficiency, the ERG responses possibly represent a more sensitive marker compared to the serum levels of vitamin A.

The use and interpretation of serum retinol distributions in evaluating the public health impact of vitamin A programmes.

OBJECTIVE: Developing countries have adopted universal, high-potency vitamin A (VA) supplementation and food fortification as major strategies to control deficiency, prevent nutritional blindness and reduce child mortality. Yet questions persist regarding how best to measure impact and when to phase out supplementation. The present paper provides guidance on the use and interpretation of serum retinol (SROL) distributions as indicators of both programme impact and adequate VA intake in a population. DESIGN: We reviewed extant data on SROL's response to high-potency VA supplementation and VA-fortified foods in children. RESULTS: Supplementation virtually eliminates xerophthalmia and reduces child mortality; however, it shifts the SROL distribution only transiently (<2 months). Regular consumption of VA-fortified foods prevents xerophthalmia, lowers mortality and sustainably improves SROL distributions, from which both compliance and public health impact can be inferred. CONCLUSIONS: Given SROL's limited responsiveness to high-potency VA supplementation, target population coverage remains the preferred performance indicator. However, periodic SROL surveys do reflect underlying dietary risk and can guide programming: low or marginal SROL distributions in areas with high supplementation coverage do not signify programme failure, but rather suggest the need to continue supplementation while working to effectively raise dietary VA intakes. We propose that a sustained rise in the SROL distribution, defined as ≤5 % prevalence of SROL < 0·70 µmol/l among vulnerable population groups in at least two consecutive surveys (≥1 year apart), be used as an indicator of stable and adequate dietary VA intake and status in a population, at which point programmes may re-evaluate the need for continued universal supplementation.

Fried-potato diet causes vitamin A deficiency in an autistic child.

A 5-year-old boy with autism developed dry eye and xerophthalmia. Serum vitamin A was undetectable. Dietary history revealed a markedly altered intake consisting of only fried potatoes and rice balls for 2 years. Fried potatoes contain no vitamin A. Autism is a multifaceted developmental disorder infrequently accompanied by abnormal eating practices. To the authors' knowledge, most children with autism who develop dietary vitamin A deficiency have consumed an excess of fried potatoes. Attention to possible vitamin A deficiency is essential when fried potatoes are consumed exclusively.

Epidemiology of vitamin A deficiency and xerophthalmia in at-risk populations.

Vitamin A deficiency (VAD) is an important public health problem worldwide that contributes significantly to the global burden of disease. Vitamin A deficiency disorders include xerophthalmia and increased risk of infectious diseases, both of which increase risk of mortality. Xerophthalmia is also a leading cause of preventable blindness. Areas with highly prevalent VAD often share common dietary and other environmental exposures, including poverty, infectious diseases, limited development and poor availability of vitamin A containing food. Globally, the prevalence of VAD has been declining, which may be due to widespread vitamin A supplementation in conjunction with measles immunisation in at-risk populations. Recent meta-analyses confirm that provision of vitamin A to children aged between 6 months and 5 years confers a significant mortality benefit. Further preventative measures for VAD comprise improving availability of vitamin A containing food, including foods biofortified with vitamin A. Ensuring vitamin A is available in any form in adequate quantities remains problematic, especially in areas affected by environmental catastrophes and conflict, and other areas where access to vitamin A containing foods and healthcare interventions is limited. Hence, it remains essential that maternal and child health workers remain vigilant for VAD in nutritionally vulnerable populations.

Phrynoderma: a rare dermatologic complication of bariatric surgery.

Biliopancreatic diversion is a predominantly malabsorptive bariatric procedure that can lead to the development of several nutrition complications, including fat-soluble vitamin deficiencies. Routine supplementation with vitamins and trace elements and a strict medical follow-up are essential to prevent these nutrition risks. Vitamin A deficiency is common after bariatric surgery but rarely causes clinical symptoms. Case reports have described ophthalmological and fetal complications associated with vitamin A deficiency after malabsorptive bariatric surgery. Phrynoderma is a type of follicular hyperkeratosis located on the extensor surfaces of the extremities whose main cause is vitamin A deficiency. The simultaneous occurrence of phrynoderma and ocular symptoms secondary to hypovitaminosis A after bariatric surgery is exceptional. The authors describe a man who presented follicular hyperkeratosis with nyctalopia and xerophthalmia that had appeared 1 year after biliopancreatic diversion. He admitted poor compliance with diet and daily supplementation of vitamins and oligoelements. Serum vitamin A levels were decreased. Treatment with high doses of vitamin A was associated with a clear improvement of cutaneous and ocular lesions with complete resolution after 2 months. The patient was readmitted 2 years later because of the reappearance of cutaneous lesions and micronutrient deficiency. Revisional bariatric surgery was performed. The authors review and discuss the relationship between phrynoderma, malnutrition, and vitamin A deficiency.

[Phrynoderma secondary to vitamin A deficiency in a patient with biliopancreatic diversion]. / Frinoderma secundario a déficit de vitamina A en un paciente con derivación biliopancreática.

Biliopancreatic diversion (BPD) is a malabsorptivebariatric procedure can lead to the development of several nutritional complications, including fat-soluble vitamins deficiencies. Routine supplementation with vitamins and trace elements and a close follow-up long-term can prevent these nutritional risks. Vitamin A participates in ocular metabolism, epithelial differentiation, growth, and embryogenesis. Have been described several cases of ophthalmological and fetal complications associated with vitamin A deficiency in patients who have undergone BPD. Few information exists in literature about dermatologic manifestations that may occur in these patients. Phrynoderma is a type of follicular hyperkeratosis located on the extensor surfaces of the extremities whose main cause is vitamin A deficiency. We report an exceptional case of severe cutaneous and ocular complications in a patient who had undergone BPD with poor adherence to treatment and postoperative follow-up. Our patient presented simultaneously the characteristic skin lesions of phrynoderma with nytalopia and xerophthalmia in a setting of low serum levels of vitamin A. Treatment with high doses vitamin A obtained the resolution of both processes. We review and discuss the relationship between phrynoderma, malnutrition and vitamin A deficiency.

[Vision jeopardizing xerophthalmia in Denmark]. / Synstruende xeroftalmi i Danmark.

A 54-year-old alcoholic was admitted with keratomalacia in 2001. The right cornea had an ulceration and the left cornea had stromal necrosis and a perforation. Serum retinol and serum betacaroten were extremely low. Treatment consisted of high-dose retinol and left sided cornea transplantation. The right eye healed with little scarring, vision: 0.5. The left eye was stabilized after a second cornea transplantation and covering with conjunctiva, vision: hand movement at a distance of 1 m. This proves the existence of symptomatic vitamin A deficiency among socially exposed subjects in Denmark.

Stomatological approach to Sjögren's syndrome: diagnosis, management and therapeutical timing.

Sjögren's syndrome is a chronic multisystem autoimmune disease characterized by the exocrine glands inflammation, with subsequent hypofunction. More frequently lachrymal and salivary glands are interested with subsequent xerophthalmia and xerostomia. Sjögren's syndrome can be present in an idiopathic type or in association with other autoimmune diseases as rheumatoid arthritis, systemic lupus erythematosus, schlerodermia, etc. It interests mainly the women (with a ratio F:M=9:1) with an age between 40 and 60 years old. The disease prevalence varies from 0.4% to 4.8%. The glandular lesions determine in the time a volume reduction and a secretum quality alteration. The most frequent oral manifestations are xerostomia, that allows the establishment of caries, gingivitises, periodontal disease and oral candidiasis. The aim of this work was to perform a thorough review of the literature on Sjögren's syndrome, illustrating the most internationally accredited diagnostic criteria, the patient's management and therapeutical approach in the odontostomatological discipline. The Authors conclude that it doesn't exist a resolutive treatment of the disease. The therapy is only palliative, and is turned to the treatment of xerostomia and xerophthalmia, through systemic and aspecific sialogogues drugs. From the odontostomatological point of view, particularly useful results the domiciliary and professional oral hygiene to contrast the xerostomia effect on the oral structures.

Effects of topical application of ibudilast for seasonal allergic conjunctivitis in patients wearing soft contact lenses.

PURPOSE: To assess the influence of topical application of ibudilast for seasonal allergic conjunctivitis in patients wearing soft contact lenses (SCLs). MATERIALS AND METHODS: There were 16 SCL wearers (32 eyes) with allergic conjunctivitis due to cedar pollen, who were studied from February to April 2007. Before enrollment, informed consent to participation in this study was obtained from all subjects. A frequent replacement SCL (2 Week Pure) was worn for 2 weeks, and ibudilast was applied topically four times daily during this period. The severity of allergic symptoms and the severity of SCL-related symptoms were assessed by scoring using two questionnaires, and before and after topical application of ibudilast results were compared. The severity of objective ocular findings was also scored and compared in the same way. After the final examination, the SCLs were collected and immersed in physiologic saline. Then morphologic changes and drug adsorption were investigated. RESULTS: Among the allergic symptoms, itching and a dry sensation improved after topical application of ibudilast ophthalmic solution (both P<0.05). Phlyctenular conjunctivitis was noted in one eye after topical application, but there were no significant differences of SCL-related symptoms and objective ocular findings between before and after application. There were also no morphologic changes of the contact lenses, and the ibudilast concentration in the lenses was below the detection limit. CONCLUSIONS: These results suggest that topical application of ibudilast while using 2 Week Pure lenses can improve subjective symptoms without influencing drug adsorption or lens morphology.