AIM： To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome, METHODS： Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome （confir...AIM： To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome, METHODS： Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome （confirmed by contrast echocardiography） at the dosage of 1 g/1.73 m^2 per day. Patients were evaluated clinically and by arterial blood gas every four weeks. RESULTS： The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 ± 3.9 years. The underlying problems were biliary tract atresia （4 patients）, autoimmune hepatitis （4 patients）, cryptogenic cirrhosis （4 patients） and presinusoidal portal hypertension （3 patients）. Eight patients （53.3%） showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 ± 12.1 mmHg in the responder group and 47.1 ± 11.2 mmHg in nonresponder group. At the end of treatment the mean PaO2 in responders and non-responders was 92.2 ± 7.75 mmHg and 47.5 ± 11.87 mmHg, respectively （P 〈 0.01）. CONCLUSION： Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome.展开更多
Introduction: Microvillous inclusion disease (MVD) or microvillous atrophy disorder is a congenital disorder of the small intestinal epithelial cells that presents with persistent and severe diarrhea and it is charact...Introduction: Microvillous inclusion disease (MVD) or microvillous atrophy disorder is a congenital disorder of the small intestinal epithelial cells that presents with persistent and severe diarrhea and it is characterized by enterocytes abnormalities [1] 08D0C9EA79F9BACE118C8200AA004BA90B02000000080000000E0000005F005200650066003300380035003800380033003700360030000000 . For these children, prognosis is generally poor due to metabolic acidosis with poor compensation. To our experiment, this disease is very rare in Iran and it is yet unreported, so we decided to report two consecutive siblings with the same disease from Iran. Report of Cases: Two siblings were born to healthy parents. Parents were cousins. Both siblings were hospitalized due to severe diarrhea starting shortly after breast feeding. The frequency of diarrhea in both cases was 10 to 17 times per day and their stools were loose and green. Histological studies of both siblings revealed duodenal mucosa with complete flattening of villi (total villous atrophy). Superficial lining cells showed atrophy. Crypts showed no hyperplasia, however it showed distortion and difference in size. By PAS staining and CD10 staining, a poorly developed brush border and typical inclusions were seen in apical boarder of enterocytes. Electron microscopy was performed for the second case and showed microvillous involution and inclusions in the apical part of the epithelial cells. Discussion: Microvillous congenital atrophy is a rare congenital disorder. Due to rareness of congenital microvillous atrophy (CMA), it is crucial to distinguish it from other diseases with persistent and severe diarrhea as soon as possible.展开更多
文摘AIM： To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome, METHODS： Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome （confirmed by contrast echocardiography） at the dosage of 1 g/1.73 m^2 per day. Patients were evaluated clinically and by arterial blood gas every four weeks. RESULTS： The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 ± 3.9 years. The underlying problems were biliary tract atresia （4 patients）, autoimmune hepatitis （4 patients）, cryptogenic cirrhosis （4 patients） and presinusoidal portal hypertension （3 patients）. Eight patients （53.3%） showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 ± 12.1 mmHg in the responder group and 47.1 ± 11.2 mmHg in nonresponder group. At the end of treatment the mean PaO2 in responders and non-responders was 92.2 ± 7.75 mmHg and 47.5 ± 11.87 mmHg, respectively （P 〈 0.01）. CONCLUSION： Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome.
文摘Introduction: Microvillous inclusion disease (MVD) or microvillous atrophy disorder is a congenital disorder of the small intestinal epithelial cells that presents with persistent and severe diarrhea and it is characterized by enterocytes abnormalities [1] 08D0C9EA79F9BACE118C8200AA004BA90B02000000080000000E0000005F005200650066003300380035003800380033003700360030000000 . For these children, prognosis is generally poor due to metabolic acidosis with poor compensation. To our experiment, this disease is very rare in Iran and it is yet unreported, so we decided to report two consecutive siblings with the same disease from Iran. Report of Cases: Two siblings were born to healthy parents. Parents were cousins. Both siblings were hospitalized due to severe diarrhea starting shortly after breast feeding. The frequency of diarrhea in both cases was 10 to 17 times per day and their stools were loose and green. Histological studies of both siblings revealed duodenal mucosa with complete flattening of villi (total villous atrophy). Superficial lining cells showed atrophy. Crypts showed no hyperplasia, however it showed distortion and difference in size. By PAS staining and CD10 staining, a poorly developed brush border and typical inclusions were seen in apical boarder of enterocytes. Electron microscopy was performed for the second case and showed microvillous involution and inclusions in the apical part of the epithelial cells. Discussion: Microvillous congenital atrophy is a rare congenital disorder. Due to rareness of congenital microvillous atrophy (CMA), it is crucial to distinguish it from other diseases with persistent and severe diarrhea as soon as possible.
