Samra Nashwa M, Emad El Abrak Shaimaa, El Dash Hanaa H, El Said El Raziky Mona, El Sheikh Manal A
Department of Pediatrics, Fayoum University, Fayoum city, Egypt.
Department of Pediatrics, National Hepatology and Tropical Medicine Research Institute, Cairo University, Cairo, Egypt.
Clin Res Hepatol Gastroenterol. 2018 Sep;42(4):368-377. doi: 10.1016/j.clinre.2017.11.010. Epub 2018 Mar 16.
Hepatic osteodystrophy caused by vitamin D and calcium malabsorption is thought to develop in children with cholestatic liver disease leading to secondary hyperparathyroidism and rickets or osteomalacia. The aim of this study was to evaluate the dental and bone mineral densities and the serum level of vitamin D in cholestatic infants and children and to correlate this process with clinical and laboratory parameters.
This is a cross-sectional study that include 50 patients presenting with cholestasis. Thirty age and sex matched controls recruited not complaining of liver disease. All cases were subjected to full history taking, clinical and dental examination, 25(OH)D level, ALT, AST, bilirubin, albumin, GGT, alkaline phosphatase, PT, INR, calcium, corrected calcium, phosphorus and DXA scan to those above 5 years old. Controls were subjected to measuring the serum levels of 25(OH)D, total bilirubin, direct bilirubin, ALT, GGT, AST, PT, INR, alkaline phosphatase, albumin, calcium and phosphorus.
Out of the 50 cases; 23 were females (46%), with a mean age of 6.17±3.9 years ranging from 1.1 to 17 years. Twenty-eight of the cases had signs of rickets (56%), 6 of them had bone fracture (12%) and 42.8% had milky teeth caries. The level of 25(OH) vitamin D was below normal range in around half of the patients. There was significant difference between cases and controls in calcium and phosphorus levels, ALT and alkaline phosphatase. Low bone mineral density (BMD) was present in 50% and 5 cases (17.9%) were diagnosed as having osteoporosis. There was a negative correlation between the Z-score, BMD of total body, BMD and bone mineral content (BMC) of spine and total and direct bilirubin. There was a positive correlation between (BMD of total body, spine and BMC of spine) and serum phosphorus, alkaline phosphatase and albumin. There was a positive correlation between the Z-score of total body and serum calcium.
Decreased level of 25-OH vitamin D is present in more than half of cholestatic patients, and is correlated positively to serum calcium. Decreased BMD was present in more than half of studied cholestatic patients correlated to the low serum calcium rather than the vitamin D level. The decreased BMD and the dental affection in cholestatic children is related to the level of hyperbilirubinemia.
维生素D和钙吸收不良所致的肝性骨营养不良被认为发生于胆汁淤积性肝病患儿,可导致继发性甲状旁腺功能亢进及佝偻病或骨软化症。本研究旨在评估胆汁淤积性婴幼儿及儿童的牙骨密度和血清维生素D水平,并将此过程与临床及实验室参数相关联。
这是一项横断面研究,纳入50例胆汁淤积患者。招募30例年龄和性别匹配、无肝病主诉的对照者。所有病例均进行全面病史采集、临床及牙科检查、检测25(OH)D水平、谷丙转氨酶(ALT)、谷草转氨酶(AST)、胆红素、白蛋白、γ-谷氨酰转肽酶(GGT)、碱性磷酸酶、凝血酶原时间(PT)、国际标准化比值(INR)、钙、校正钙、磷,并对5岁以上者行双能X线吸收法(DXA)扫描。对照者检测血清25(OH)D、总胆红素、直接胆红素、ALT、GGT、AST、PT、INR、碱性磷酸酶、白蛋白、钙和磷水平。
50例病例中,女性23例(46%),平均年龄6.17±3.9岁,年龄范围为1.1至17岁。28例病例有佝偻病体征(56%),6例有骨折(12%),42.8%有乳牙龋齿。约半数患者的25(OH)维生素D水平低于正常范围。病例组与对照组在钙、磷水平、ALT及碱性磷酸酶方面存在显著差异。50%存在低骨密度(BMD),5例(17.9%)被诊断为骨质疏松症。全身Z评分、全身BMD、脊柱BMD及骨矿物质含量(BMC)与总胆红素和直接胆红素呈负相关。全身、脊柱BMD及脊柱BMC与血清磷、碱性磷酸酶及白蛋白呈正相关。全身Z评分与血清钙呈正相关。
超过半数的胆汁淤积患者25-OH维生素D水平降低,且与血清钙呈正相关。超过半数的研究胆汁淤积患者存在BMD降低,这与血清钙水平低有关,而非维生素D水平。胆汁淤积患儿BMD降低及牙齿病变与高胆红素血症水平有关。