1/21. Keratomalacia.xerophthalmia and keratomalacia are public health problems of great magnitude which are usually associated with multiple vitamin and protein deficiencies. The authors report the case of a 27-year-old commune member who subjected herself to a bizarre protein and vitamin deficient diet for many months. This ultimately produced nyctalopia, xerophthalmia and keratomalacia with bilateral corneal perforation. Despite therapy, she remained comatose and expired shortly after admission. Ocular pathological changes included bilateral corneal melting with prolapse of intraocular contents, conjunctival epidermidalization, goblet cell atrophy and thinning of the outer nuclear layer of the retina. It is noted that ocular findings in pure avitaminosis A produced experimentalyy include epithelial atrophy followed by keratinization.- - - - - - - - - - ranking = 1keywords = protein (Clic here for more details about this article) |
2/21. association of chronic symptomatic neutropenia with the triple A syndrome.Chronic neutropenia syndromes include distinct hereditary disorders with varying degrees of neutropenia. Among the more common inherited disorders associated with symptomatic neutropenia are cyclic neutropenia, severe congenital neutropenia (Kostmann disease), and Schwachman-diamond syndrome. The authors describe a 17-year-old girl with triple A syndrome who developed a progressive decrease in the granulocyte count, finally resulting in long-standing neutropenia. Its probable pathogenesis may be related to dysfunction of ALADIN (the protein known to be mutated in triple A syndrome), resulting in abnormal nucleocytoplasmic transport of essential proteins, in myeloid precursor cells. Chronic neutropenia should therefore be considered among the clinical manifestations of triple A syndrome.- - - - - - - - - - ranking = 1keywords = protein (Clic here for more details about this article) |
3/21. adult blindness secondary to vitamin a deficiency associated with an eating disorder.OBJECTIVE: We examined an adult patient who lost one eye due to severe keratomalacia secondary to self-induced vitamin a deficiency. methods: This case report provides a clinical, ophthalmologic, and laboratory description in addition to a review of the medical literature. RESULTS: A 33-y-old woman with a 17-y history of an eating disorder presented with bilateral conjunctival xerosis, an infected corneal ulcer in the right eye and a large descemetocele in the left eye. Laboratory and clinical findings were consistent with vitamin a deficiency. Despite a tectonic penetrating keratoplasty, her left eye perforated and had to be eviscerated. In parallel, vitamin A replacement improved her clinical status and the ocular findings in her right eye. CONCLUSIONS: The present report indicates that vitamin a deficiency secondary to eating disorders should be considered in the differential diagnosis of patients with severe dry eye and corneal ulceration.- - - - - - - - - - ranking = 132.04834697917keywords = deficiency (Clic here for more details about this article) |
4/21. Ocular complications of hypovitaminosis a after bariatric surgery.PURPOSE: To report the ocular complications of xerophthalmia, nyctalopia, and visual deterioration to legal blindness as a result of inadequate vitamin A supplementation after malabsorptive bariatric surgery. DESIGN: Observational case report and literature review. PARTICIPANT: A 39-year-old woman with xerophthalmia and nyctalopia occurring 3 years after gastric bypass surgery. RESULTS: We report a patient with a rare finding of xerophthalmia and visual deterioration after gastric bypass surgery as a result of vitamin a deficiency. The patient was referred for decreased vision associated with chronic dry eyes, bilateral diffuse punctate keratitis, and corneal scarring of unknown cause after several ophthalmologic examinations. The medical history, ophthalmic findings, and clinical course are discussed. CONCLUSIONS: gastric bypass procedures can cause vitamin a deficiency leading to serious ocular complications, including xerophthalmia, nyctalopia, and ultimate blindness. The increasing incidence of obesity and gastric bypass procedures warrants patient and physician education regarding strict adherence to vitamin supplementation. education is imperative to avoid detrimental ophthalmic complications resulting from hypovitaminosis A and to prevent a potential epidemic of iatrogenic xerophthalmia and blindness.- - - - - - - - - - ranking = 37.728099136905keywords = deficiency (Clic here for more details about this article) |
5/21. Subacute aseptic meningitis as neurological manifestation of primary sjogren's syndrome.sjogren's syndrome (SS) is an autoimmune disease characterized by inflammatory infiltration and secondary chronic dysfunction of exocrine glands. Systemic (extraglandular) manifestations of the disease occur in one-third of the patients, including a wide spectrum of peripheral and central neurological disorders. We report a case of subacute afebrile aseptic meningitis (AM) as neurological manifestation of primary SS. The neurological symptomatology presented gradual onset and progression, including diplopia, mild headache, pain and stiffness of the neck. The clinical examination pointed out xerostomia and xerophthalmia. diagnosis of SS was confirmed by Schirmer's tear test and histopathology of the labial salivary glands. The neurological involvement was highlighted by gadolinium-enhanced magnetic resonance imaging of the brain which displayed an increased diffuse leptomeningeal enhancement. cerebrospinal fluid (CSF) analysis showed moderate pleocytosis with prevalence of polymorphonuclear leukocytes and increased protein level but no evidence of Ig intrathecal synthesis. A cycle of intravenous steroid therapy led to a complete disappearance of the neurological symptomatology and to normalization of the CSF inflammatory pattern. Given the unusual presentation of this case of AM, which resembled the characteristics of a chronic meningitis rather than those of an acute form, in patients affected by SS we must stress the importance of cephalic symptoms such as headaches and cervical stiffness (even if mild and without fever) as possible signs of central neurological involvement of the disease.- - - - - - - - - - ranking = 0.5keywords = protein (Clic here for more details about this article) |
6/21. xerophthalmia secondary to alcohol-induced malnutrition.BACKGROUND: xerophthalmia refers to the ocular manifestations associated with vitamin a deficiency. vitamin a deficiency can be caused by numerous disorders, including alcohol-induced malnutrition. The ocular manifestations of xerophthalmia include conjunctival and corneal xerosis (drying), keratomalacia (corneal necrosis/ulceration), nyctalopia (night blindness), and Bitot's spots (conjunctival lesions). CASE REPORT: A 47-year-old white male with complaints of dryness and difficulty seeing at night presented to our clinic for consultation from general medicine to rule out xerophthalmia. Laboratory testing and general medicine, psychiatry, and nutrition evaluations confirmed the systemic diagnosis of alcohol-induced malnutrition. He admits that his alcoholism was induced by depression. Confirmed associated disorders compounding the malnutrition include alcoholic cirrhosis, protein deficiency, and megaloblastic anemia. The patient had xerophthalmia diagnosed in the optometry clinic as a result of symptoms, slit lamp examination signs, and the associated disorders. The associated disorders were treated with systemic medications and vitamins. Ophthalmic treatment consisted of carboxymethylcellulose-based artificial tears. CONCLUSIONS: Although xerophthalmia and vitamin a deficiency are more common in underdeveloped countries, their presentation in the united states may be induced by conditions such as liver cirrhosis, malnutrition, and alcoholism. This report summarizes ocular manifestations of alcoholism and presents a case of xerophthalmia secondary to alcohol-induced malnutrition and the role of optometry in its treatment and management.- - - - - - - - - - ranking = 43816.690353041keywords = protein deficiency, deficiency, protein (Clic here for more details about this article) |
7/21. night blindness, yellow vision, and yellow skin: symptoms and signs of malabsorption.BACKGROUND: Rapidly progressing bilateral night blindness in an elderly patient suggests primarily a diagnosis of paraneoplastic retinopathy. Occasionally diffuse rod dysfunction can result from vitamin a deficiency. history AND SIGNS: A 70-year-old man complained of progressive night blindness and xanthopsia for the past 6 months. visual acuity was 0.8 in both eyes with severe dyschromatopsia. Slit-lamp and fundus examination were normal. Visual field disclosed bilateral depression. Scotopic full-field ERG was severely reduced. The patient's medical history revealed an acute pancreatitis one year ago, followed by chronic jaundice and an increased blood bilirubin. serum vitamin A level was decreased to 0.1 micromol/L (normal range 1.5 to 4.0). THERAPY AND OUTCOME: Intramuscular injections of vitamin A were provided. Subjective visual improvement was reported already one day after initiation of therapy. Scotopic full-field ERG was markedly improved 3 days after the injection and was only slightly subnormal 3 months later. CONCLUSIONS: In developed countries, vitamin a deficiency usually results from malabsorption syndromes and manifests initially by rod more than cone dysfunction. This diagnosis should be entertained early as vitamin A supplementation induces a rapid restoration of vision.- - - - - - - - - - ranking = 37.728099136905keywords = deficiency (Clic here for more details about this article) |
8/21. A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (hiv) infection: a host immune response associated with HLA-DR5.STUDY OBJECTIVE: To describe the clinical, immunologic, and immunogenetic features of a diffuse infiltrative lymphocytic disorder resembling Sjogren syndrome in persons infected with human immunodeficiency virus (hiv). DESIGN: Clinical case study. SETTING: University-affiliated hospitals and outpatient clinics. patients: Consecutive sample of 17 patients. MEASUREMENTS AND MAIN RESULTS: All of the 17 patients had bilateral parotid gland enlargement; 14 had xerostomia and 6 had xerophthalmia. Of the 17 patients, 14 had generalized lymphadenopathy, 10 had histologically proved lymphocytic interstitial pneumonitis, 4 had neurologic involvement, and 3 had lymphocytic infiltration of the gastrointestinal tract. gallium scanning in all of 11 tested patients showed abnormal salivary gland uptake. Minor salivary gland biopsies showed more than 2 lymphocytic foci per 4 mm2 tissue in all of 11 tested patients, the infiltrate consisting predominantly of CD8 cells. Fifteen patients had circulating CD8 lymphocytosis; the principal phenotype of these cells was CD8 CD29 . Rheumatoid factor and antinuclear antibodies were infrequent, and none of the patients had anti-Ro/SS-A or anti-La/SS-B antibodies. HLA-DR5 was significantly more frequent in the black patients (10 of 12) compared with controls (13 of 45). Only one patient developed an opportunistic infection during 544 patient-months of study, and none has died of AIDS. CONCLUSIONS: A distinct syndrome primarily characterized by parotid gland enlargement, sicca symptoms, and pulmonary involvement occurs in hiv infection. This disorder is associated with CD8 lymphocytosis and the presence of HLA-DR5, and appears to be a genetically determined host immune response to hiv.- - - - - - - - - - ranking = 94.320247842262keywords = deficiency (Clic here for more details about this article) |
9/21. corneal perforation in patients with vitamin a deficiency in the united states.An emaciated alcoholic 52-year-old white woman presented with an old left corneal perforation and bilateral conjunctival and corneal xerosis. The serum vitamin A level was 0 mumol/L. Laboratory and radiologic findings were consistent with the diagnosis of a fat malabsorptive syndrome secondary to chronic alcoholic pancreatitis. Histopathologic evaluation of her enucleated globe revealed conjunctival epidermidalization, corneal perforation with prolapse and loss of intraocular contents, retinal detachment, and massive choroidal hemorrhage. A second patient presented with bilateral conjunctival xerosis, corneal ulcers, and a low serum vitamin A level. corneal perforation of one ulcer ensued during her hospitalization. Medical investigation revealed hepatic metastases of an unknown primary source. Causes of vitamin a deficiency and its ocular complications as well as medical and surgical management options are discussed.- - - - - - - - - - ranking = 94.320247842262keywords = deficiency (Clic here for more details about this article) |
10/21. xerophthalmia and cystic fibrosis.We treated two infants with failure to thrive who presented with clinical evidence of conjunctival and corneal xerosis. One patient was referred with possible infectious corneal ulcer thought to exist because there were deep peripheral ulcerations of the cornea and associated hypopyon. The other patient was initially thought to have a nasolacrimal duct obstruction because of excessive tearing. xerophthalmia secondary to vitamin a deficiency was suspected and led to the diagnosis and treatment of cystic fibrosis in each case. Therapy with vitamin A promptly resolved the xerosis, but it also caused a transient rise in intracerebral pressure. xerophthalmia can still be a problem in developed countries when underlying disorders, such as cystic fibrosis, lead to vitamin A malabsorption.- - - - - - - - - - ranking = 18.864049568452keywords = deficiency (Clic here for more details about this article) |
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