Raised galactose plus galactose-1-phosphate blood levels in patients under PUVA therapy

Background PUVA has become a preferred method of treatment for vitiligo and psoriasis. However, 8-methoxypsoralen (8-MOP) as well as a high galactose diet may cause liver and kidney function damage and enhance cataract formation. Patients and Methods Twenty one patients aged 32 ± 2.6 yrs with vitiligo (N= 11) or psoriasis (N= 10) underwent eye-examination, liver and kidney function tests and galactose + galactose 1-phosphate (gal + gal P) blood evaluation using Porton-Cambridge Ltd reagents, before and after the 12th PUVA treatment during a 35–40 day study. Results All these functional tests including eye-examination were normal before and after commencing therapy. However, the mean gal + gal P blood levels (1.29 ± 0.41 mg/dl) were statistically elevated (2.95 ± 0.42 mg/dl) at the end of the study. It is suggested that 8-MOP either impairs liver function or acts as an inhibitor of the enzymes responsible for galactose metabolism. We propose gal + gal P blood estimation in all patients before and, at intervals, after the initiation of PUVA therapy in order to prevent as early as possible an additional risk factor for liver or kidney dysfunction as well as cataract formation.     

Leberfunktion von Patienten mit arterieller Hypoxämie bei chronisch obstruktiver Atemwegserkrankung und der Einfluß der nasalen Sauerstoffinsufflation

Summary Patients suffering from chronic obstructive airway disease often have arterial hypoxemia, which is more or less severe. If hypoxemia deteriorates the function of the liver is still unknown. The aim of this study was to determine, if a mostly oxygen dependent process of liver metabolism (galactose elimination capacity) is disturbed and if it can be increased by oxygen insufflation. Under the same conditions an oxygen independent process (indocyaninegreen clearance) should not be influenced. Our results show, that galactose elimination capacity under room air was pathologic and can be increased significantly by oxygen breathing. No change of indocyaninegreen clearance was seen under the same conditions. After termination of oxygen therapy the galactose elimination capacity was as bad as before oxygen breathing. Dysfunctions of the liver, which are caused by hypoxemia, can be positively influenced by oxygen, but only for the duration of oxygen insufflation.

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Abkürzungsverzeichnis Adm Mitteldruck im rechten Vorhof - CHE Cholinesterase - Gamma-GT Gamma-Glutamyl-Transferase - GEK Galaktose-Eliminations-Kapazität - ICG-HWZ Indocyaningrün-Halbwertzeit - KG Körpergewicht - NAD Nicotinsäureamid - NADH₂ reduziertes Nicotinsäureamid - O₂ molekularer Sauerstoff - pCO_2a arterieller Kohlensäurepartialdruck - pO_2a arterieller Sauerstoffpartialdruck - SGOT Serum-Glutamat-Oxalacetat-Transaminase - SGPT Serum-Glutamat-Pyruvat-Transaminase - SO_2a arterielle Sauerstoffsättigung     

Effects of galactosemia in utero

There is direct evidence that in galactosemia, due to galactose-1-phosphate uridyltransferase deficiency, galactose, galactose-1-phosphate and galactitol accumulate in the fetus by week 20 of gestation. The metabolic abnormality may develop earlier than this, however, since the key enzymes in galactose metabolism have been shown to be present in normal fetal liver from the 10th week of gestation. There has been a single report of increased galactitol in amniotic fluid obtained at 10 weeks gestation, the outcome being a baby affected with galactosemia. Cataract formation in the fetus is rare and the only direct evidence that galactosemia may have harmful effects in utero. However, it has been concluded that the liver pathology seen in some patients who died in the neonatal period originated prenatally, and some studies have found that galactosemia is associated with reduced birth weight. Reports of two patients with histologically normal ovaries very soon after birth have been cited as evidence against gonadal dysfunction arising during fetal life, but it should be noted that this is not a constant feature in female galactosemics. Other observations, particularly those made from animal models, would suggest that ovarian dysfunction is most likely to have been caused in utero.     

维生素E对D-半乳糖诱致衰老小鼠脑抗氧化能力、钙稳态和线粒体DNA（mtDNA）损伤的影响

目的：探讨维生素E（Vit-E）对D-半乳糖诱致衰老小鼠脑抗氧化能力、胞浆游离Ca²⁺（［Ca²⁺］i）稳态和线粒体DNA（mtDNA）损伤的影响。方法:小鼠连续皮下注射（sc）D-半乳糖（1 000 mg·k^-1·d^-1）8周制备衰老模型，并于第3周开始给予维生素E（100 mg· kg^-1；250 mg· kg^-1）处理；8周后采用水迷宫测定小鼠学习记忆能力，并取脑组织测定谷胱甘肽过氧化物酶(GSH-Px)和琥珀酸脱氢酶（SDH）活性，测定一氧化氮（NO）含量和一氧化氮合酶（NOS）活性。Fura-2/AM负载法和PCR方法分别测定海马神经细胞［Ca²⁺］i浓度和mtDNA缺失突变。结果:维生素E处理能明显改善D-半乳糖诱致衰老小鼠学习记忆障碍，抑制脑组织NOS活性，降低NO含量，提高GSH-Px和SDH活性，降低［Ca²⁺］i水平（P＜0.01， P＜0.05），并防止mtDNA缺失突变的发生。结论:维生素E具有提高衰老小鼠脑抗氧化能力和调节［Ca²⁺］i稳态的作用，并抑制氧化应激引起的mtDNA损伤，从而改善衰老动物学习记忆障碍。     

半乳糖基抗CD3单抗的制备及体内趋肝性研究

目的　了解半乳糖基抗 CD3 单抗 - TIL 复合物 (Gal- Anti- CD3 - Mc Ab- TIL)的体内趋肝细胞性。方法　制备半乳糖基抗 CD3 单抗 (Gal- Anti- CD3 - Mc Ab) ,采用苯酚 -硫酸法测其糖密度 ;经动物外周静脉分别注入用 1 2 5 I标记的抗 CD3 单抗 (Anti- CD3 - Mc Ab)和用 1 3 1 I标记的 Gal- Anti- CD3 - Mc Ab,分别测定两者在动物体内各脏器的放射活性。结果　 Gal- Anti- CD3 - Mc Ab的糖密度值为 5 8.12 ;外周静脉注射给药 ,Anti- CD3 - Mc Ab趋于肺内浓聚 ,而 Gal- Anti- CD3 - Mc Ab有显著的趋肝性 ,且于靶器官有较长时间停留。结论　经外周静脉途径注射 ,Gal- Anti-CD3 - Mc Ab有显著的体内趋肝细胞性 ,其中半乳糖基与肝结合蛋白所介导的受体 -配体反应在 Gal- Anti- CD3 - Mc Ab的趋肝细胞过程中发挥主导作用     

Galectin-3和VEGF表达水平与宫颈癌的相关性研究

目的：探讨宫颈癌组织表达半乳糖凝集素-3(Galectin-3)以及血管内皮生长因子(VEGF)水平与患者临床病理特征的相关性。方法选取2013年1月-2014年10月我院收治的139例宫颈癌患者作为研究对象,所有患者均采用免疫组化S-P法对宫颈癌组织行Galectin-3、VEGF表达水平检测,同时收集患者的年龄、肿瘤直径、淋巴结转移、病理学类型、宫颈间质浸润深度等临床病理特征指标。结果年龄＜45岁组与≥45岁组之间、鳞癌组与非鳞癌组之间、淋巴结转移阳性组与阴性组之间,宫颈癌组织表达Galectin-3、VEGF相比差异无统计学意义(P＞0.05);宫颈间质浸润≤1/2肌层组与＞1/2肌层组之间、肿瘤直径≤2cm组与＞2cm组之间,宫颈癌组织表达Galectin-3、VEGF相比差异有统计学意义(P＜0.05)。结论宫颈癌组织表达Galectin-3以及VEGF与肿瘤直径、宫颈间质浸润深度等临床病理特征指标具有一定的相关性。     

应用半乳糖氧化酶检测大肠癌及癌前病变的临床价值

决定半乳糖氧化酶－雪夫氏（ＧＯ－Ｓ）试验对检测大肠癌及癌前病变的临床价值。方法对１２７例具有消化道症状、体征的患者和１０例正常对照者的直肠粘液进行涂片染色。另对７３例大肠癌和癌前病变患者的石蜡切片进行ＧＯ－Ｓ染色。结果ＧＯ－Ｓ试验在各种大肠癌和癌前病变中的阳性率超过８０％。其对大肠癌和癌前病变的特异性和敏感性分别为９８％和８６％,阳性预示值为９０％。结论ＧＯ－Ｓ试验是一种对大肠癌及癌前病变理想、敏感的标志物,可用于大肠癌及癌前病变的筛查和普查     

Hidden sources of galactose in the environment

A galactose-restricted diet free of lactose is lifesaving in patients with galactose-1-phosphate uridyl transferase (GALT) deficiency, but does not prevent long-term complications such as developmental delay, abnormal speech, poor growth and, in females, ovarian failure. Lactose, found in dairy products and as an extender in drugs, has been considered the primary source of galactose in the diet. Two recent publications reported that small amounts of galactose are present in many fruits and vegetables. We report the presence of considerable amounts of free galactose in some legumes (dried beans and peas) and the presence of bound galactose in many food plants. Galactose, in various glycosidic linkages, such as -1,6, –1,3 and –1,4, and as a component of lipids, is ubiquitous in animals and plants. The bioavailability of –1,6 and –1,3 linked galactose in foods is unknown. However, -galactosidases found in plant and animal tissues may release galactose in –1,6 linkage, and from digalactosyldiacylglycerol. Galactose in –1,4 linkage and as monogalactosyldiacylglycerol may be released by -galactosidases in animal and plant tissues. Foods fermented by micro-organisms for preparation or preservation purposes may contain free galactose. The role of free and bound galactose in cereals, fruits, legumes, nuts, organ meats, seeds, and vegetables in the poor outcome seen in some patients with GALT deficiency is unknown. It is certain that no patients with GALT deficiency have ever ingested a galactose-free diet.     

Biochemical monitoring of pregnancy and breast feeding in five patients with classical galactosaemia – and review of the literature

Pregnancy, delivery, and postpartal metabolic control was monitored biochemically in five patients (22–38 years of age) with clinically, enzymatically, and genotypically established classical galactosaemia and good dietary compliance. Three of the patients performed breast feeding of their newborns. Monitoring parameters were galactose-1-phosphate and galactitol concentrations in erythrocytes and urinary excretion of galactose, galactitol, galactonate, and lactose. During pregnancy, a small but steady increase of renal metabolite excretion rates was observed. After delivery, a moderate transient increase of metabolite concentrations with peak values within the first week post partum occurred, irrespective of breast feeding. Altogether, there was no evidence for clinically or subclinically significant changes of metabolic control during pregnancy, delivery, or lactation. In conclusion, a specific metabolic monitoring is apparently not required in pregnant galactosemic women, and breast feeding of the nongalactosemic offspring can be recommended.