1/53. Through a shade darkly.A 43-year-old man complained of difficulty seeing in dim light (nyctalopia). A prolonged photostress test and abnormal electroretinogram confirmed retinal rather than optic nerve dysfunction. vitamin a deficiency secondary to remote ileal-jejunal bypass was diagnosed, and his visual symptoms and signs reversed with oral vitamin A supplementation.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/53. vitamin a deficiency phrynoderma: due to malabsorption and inadequate diet.We describe a patient with vitamin a deficiency phrynoderma caused by a combination of inadequate dietary intake of vitamin A and beta-carotene and malabsorption secondary to primary visceral myopathy and total colectomy.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
3/53. A case of human vitamin a deficiency caused by an inherited defect in retinol-binding protein without clinical symptoms except night blindness.Two German siblings were found to suffer from night blindness and mild retinal dystrophy but no other clinical symptoms of vitamin a deficiency. Even though they had no detectable plasma retinal-binding protein (RBP) and their plasma retinol was exceedingly low, they showed normal physiologic functions and growth. Their RBP gene was found to harbor two point mutations. Their post-prandial plasma levels of retinyl esters were normal, and it is likely that they derived their tissue retinol from retinyl esters.- - - - - - - - - - ranking = 5.0023794965626keywords = deficiency, protein (Clic here for more details about this article) |
4/53. The vitamin A spectrum: from deficiency to toxicity.dark adaptation has been used as a tool for identifying patients with subclinical vitamin a deficiency. With this functional test it was shown that tissue vitamin A deficiency occurs over a wide range of serum vitamin A concentrations. However, serum vitamin A concentrations >1.4 micromol/L predict normal dark adaptation 95% of the time. Other causes of abnormal dark adaptation include zinc and protein deficiencies. Stable isotopes of vitamin A and isotope-dilution techniques were used recently to evaluate body stores of vitamin A and the efficacy of vitamin A intervention programs in field settings and are being used to determine the vitamin A equivalences of dietary carotenoids. Vitamin A toxicity was described in patients taking large doses of vitamin A and in patients with type I hyperlipidemias and alcoholic liver disease. Conversely, tissue retinoic acid deficiency was described in alcoholic rats as a result of hepatic vitamin A mobilization, impaired oxidation of retinaldehyde, and increased destruction of retinoic acid by P450 enzymes. Abnormal oxidation products of carotenoids can cause toxicity in animal models and may have caused the increased incidence of lung cancer seen in 2 epidemiologic studies of the effects of high-dose beta-carotene supplementation. Major issues that remain to be studied include the efficiency of conversion of carotenoids in whole foods to vitamin A by using a variety of foods in various field settings and whether intraluminal factors (eg, parasitism) and vitamin A status affect this conversion. In addition, the biological activity of carotenoid metabolites should be better understood, particularly their effects on retinoid signaling.- - - - - - - - - - ranking = 7.0004758993125keywords = deficiency, protein (Clic here for more details about this article) |
5/53. 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 = 0.00095179862504827keywords = protein (Clic here for more details about this article) |
6/53. fluorescein angiography and vitamin A and oxalate levels in fundus albipunctatus.Two patients had fundus albipunctatus, one of the variants of congenital stationary night blindness. Neither the white dots in the retinas of these patients nor the retarded course of dark adaptation characteristically associated with the disease could be attributed to vitamin a deficiency or raised oxalate levels since both substances were present in normal amounts. Fluorescin angiography showed multiple discrete lesions in the pigment epithelium not coincident with the ophthalmoscopically visible changes. Since the pigment epithelium is the storage site for bleached visual pigment, these findings suggest abnormalities that underlie the major functional deficit in this disease, that is, the slow recovery of retinal sensitivity.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
7/53. PAGOD syndrome: eighth case and comparison to animal models of congenital vitamin a deficiency.We observed a 46, XY infant with atrophy of the optic nerve, complex congenital heart disease including a double outlet right ventricle, hypoplasia of the right pulmonary artery and lung, eventration of the diaphragm, and ambiguous genitalia. The baby died of cardiac arrhythmias at 204 days. The pattern of malformations was compatible with pulmonary tract and pulmonary artery, agonadism, omphalocele, diaphragmatic defect, and dextrocardia (PAGOD) syndrome. The condition may resemble the malformation complex associated with developmental deficiency of vitamin A or retinoic acid, as described in animal models.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
8/53. vitamin a deficiency in a newborn resulting from maternal hypovitaminosis A after biliopancreatic diversion for the treatment of morbid obesity.BACKGROUND: biliopancreatic diversion (BPD) has been advocated for the treatment of morbid obesity. This procedure has the theoretical advantage that patients retain normal eating capacity and lose weight irrespective of their eating habits. However, vitamin deficiencies may develop because BPD causes malabsorption. OBJECTIVE: This report describes a 40-y-old mother and her newborn infant, who developed vitamin a deficiency as a result of iatrogenic maternal malabsorption after BPD. Our primary objective is to show that BPD patients need close follow-up and lifelong micronutrient supplementation to prevent nutrient deficiencies in themselves and their offspring. DESIGN: The medical records of the mother and infant were reviewed, and their clinical course was followed until 10 mo postpartum. The mother was also interviewed on several occasions about her medical care, follow-up, and supplemental vitamin use. RESULTS: The mother developed night blindness with undetectable serum vitamin A concentrations in the third trimester of her pregnancy. Her vitamin a deficiency was untreated until she delivered her infant. At delivery, the infant also had vitamin a deficiency. He may have permanent retinal damage, but this is still unclear because the ophthalmologic examination performed at 2 mo of age was inconclusive. CONCLUSIONS: Complications of BPD may take many years to develop, and the signs and symptoms may be subtle. Because of the malabsorption that results from BPD, patients need lifelong follow-up and appropriate vitamin supplementation to prevent deficiencies. These nutrient deficiencies can also affect the offspring of female BPD patients.- - - - - - - - - - ranking = 7keywords = deficiency (Clic here for more details about this article) |
9/53. Amniotic membrane transplantation for corneal perforation related to vitamin a deficiency.corneal perforation is one of the most vision-threatening consequences of vitamin a deficiency. Amniotic membrane transplantation was performed in a 36-year-old man who presented with bilateral corneal ulcers. There was a small, sharply demarcated superficial ulcer with crevices of surrounding keratinized epithelium converging on the ulcer and minimal infiltration at the inferior periphery of the right cornea. A 3-mm perforation on a 9 x 7-mm area of stromal melting was noticed in the left cornea. The serum vitamin A level was 14.0 microg/dL (normal, 24.57 to 105.71 microg/dL) at presentation. A biopsy of the right corneal epithelium was performed to confirm the diagnosis. Multilayered amniotic membrane transplantation with debridement of the necrotic corneal stroma was performed on the left cornea. In addition to systemic vitamin A replacement, topical antibiotics and lubricants were also administered. The cornea ulcers healed in 10 days along with the restoration of the serum vitamin A level. The visual acuity improved from 20/250 to 20/20 in the right eye and from light perception to hand movement in the left eye at 3 months. Amniotic membrane transplantation could save eyes with corneal perforation related to vitamin a deficiency before reconstructive surgeries, such as penetrating keratoplasty, are necessary.- - - - - - - - - - ranking = 6keywords = deficiency (Clic here for more details about this article) |
10/53. vitamin a deficiency presenting as night blindness during pregnancy.We describe a patient with a 6-year history of pancreatic malabsorption following surgical subtotal pancreatectomy. She presented at 33 weeks of pregnancy with night blindness as a result of vitamin a deficiency. She had had two successful pregnancies 9 and 8 years previously, giving birth to a healthy baby boy on each occasion. We suggest that patients with long-term malabsorption due to intestinal or pancreatic disease should have vitamin A status checked prior to and during pregnancy so that prompt supplementation can be commenced if necessary. The possibility that vitamin a deficiency may be contributing to anaemia present in pregnancy should also be considered.- - - - - - - - - - ranking = 6keywords = deficiency (Clic here for more details about this article) |
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