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OBJECTIVES@#To summarize the clinical characteristics and nutrition therapy for children with lysinuric protein intolerance (LPI).@*METHODS@#The clinical manifestations, laboratory test results and enteral nutrition treatment in a girl with LPI diagnosed in Xiangya Hospital, Central South University were retrospective analyzed. Additionally, the data of the children with LPI reported in China and overseas were reviewed.@*RESULTS@#A case of 4-year-old girl was presented, who exhibited significant gastrointestinal symptoms, such as chronic abdominal distension, prolonged diarrhea, recurrent pneumonia, and limited growth. She had a poor response to anti-infection treatment. After receiving enteral nutrition therapy, she did not experience any gastrointestinal discomfort, and there were improvements in the levels of hemoglobin, albumin, and blood ammonia. Unfortunately, due to serious illness, she declined further treatment and later passed away. A total of 92 cases of pediatric patients with LPI have been reported to date, including one case reported in this study. Most children with LPI experienced disease onset after weaning or introduction of complementary foods, presenting with severe digestive system symptoms, malnutrition, and growth retardation. It is noteworthy that only 50% (46/92) of these cases received nutritional therapy, which effectively improved their nutritional status. Among the 92 children, 8 (9%) died, and long-term follow-up data were lacking in other reports.@*CONCLUSIONS@#LPI often involves the digestive system and may result in growth restriction with a poor prognosis. Nutritional therapy plays a crucial role in the comprehensive treatment of LPI.
Assuntos
Pré-Escolar , Feminino , Humanos , Erros Inatos do Metabolismo dos Aminoácidos/terapia , Nutrição Enteral/métodos , Desnutrição , Estudos RetrospectivosRESUMO
@#AIM:To analyze the efficacy and safety of pterygium resection respectively combined with Ologen collagen matrix implantation and autologous conjunctival flap transplantation.METHODS:A total of 80 patients(87 eyes)with pterygium admitted to the hospital between January 2017 and January 2020 were selected, and randomly divided into observation group(40 patients, 44 eyes)treated with pterygium resection combined with Ologen collagen matrix implantation and control group(40 patients, 43 eyes)treated with pterygium resection combined with autologous conjunctival flap transplantation. The patients were followed up till 12mo after operation. The best corrected visual acuity, corneal astigmatism, surface asymmetry index(SAI), surface regularity index(SRI), corneal epithelial healing, pterygium recurrence and the incidence of complications were compared between the two groups. RESULTS:Visual changes of the two groups were similar at 6 and 12mo after operation(<i>P</i>>0.05). Corneal astigmatism, SAI and SRI were reduced in the two groups at 1 and 3mo after operation. The corneal astigmatismat 3mo after operation and SAI, SRI at 1 and 3mo after operation were significantly lower in the observation group than in the control group(all <i>P</i><0.05). The corneal epithelial healing rate in observation group at 1wk after operation was significantly higher than that in the control group(73% <i>vs</i> 46%, <i>P</i><0.05), and corneal epithelium of the two groups healed at 2wk after operation. No pterygium recurrence was observed in the two groups within 12mo after operation. The situation of pterygium recurrence was similar in the two groups at 6 and 12mo after operation(<i>P</i>>0.05). There was no significant difference in the incidence of complications between the two groups(7% <i>vs </i>14%, <i>P</i>>0.05).CONCLUSION:Both pterygium resection combined with Ologen collagen matrix implantation and pterygium resection combined with autologous conjunctival flap transplantation are effective in the treatment of pterygium, with equivalent safety. However, the former has more advantages in improving corneal astigmatism and regular shape of corneal wound.
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To observe the curative effect of treating small -incision cataract extraction by intraocular lens implantation combined with trabeculectomy for primary angle-closure glaucoma with cataract. ●METHODS: Totally 44 cases (52 eyes) of primary angle-closure glaucoma combined with cataract were selected to undergo the combined surgery, in order to observe the patients' pre - and postoperative eyesights, intraocular pressures and the postoperative complications. ●RESULTS: The postoperative eyesight was improved significantly as compared with the preoperative eyesight. The intraocular pressure was declined dramatically. The result was of statistical significance (P ● CONCLUSlON: Treating the primary angle - closure glaucoma combined with cataract through the combined surgery has high reliability and desirable curative effect. The surgical method is simple to learn and applicable for promotion on the basic level.