Pseudoxanthoma Elasticum-Like Disorder with Multiple Coagulation Factor Deficiency in a Patient with Crohn's Disease / 대한내과학회지

Pseudoxanthoma elasticum (PXE)-like disorder with multiple coagulation factor deficiency is a genetic disease characterized by dystrophic mineralization of the connective tissue in multiple organs such as the skin, arteries, and retina. Patients with this disorder have excessive folding and sagging of the skin with loss of recoil, as well as coagulopathy. Crohn's disease is a chronic inflammatory condition of the gastrointestinal tract, which primary affects the small intestine, often leading to malnutrition. Here, we describe the case of a 25-year-old woman who presented with these diseases. We found that nutrient deficiency due to long-term Crohn's disease aggravated the coagulopathy associated with PXE-like disorder.

Perioperative blood transfusion of coagulation factor deficiency in children / 中华实用儿科临床杂志

Perioperative blood transfusion therapy for coagulation factor deficiency in children is very important to save patient's life or conducive to the postoperative recovery.With the advances in transfusion medicine,the cooperation through pediatric hematologists and surgeons in the perioperative period,more and more operation of children accompany coagulation factor deficiency can be safety finished.Different types of coagulation factor deficiency in children are treated by high-purity clotting factor,in order to correct abnormal coagulation factors levels.Clinical data showed that coagulation factor deficiency in children after proper treatment can be safely performed surgical procedure.

Nursing experience on the patients of non neonatal acquired deficiency of vitamin K dependent coagulation factor deficiency due to rodenticide poisoning / 中国实用护理杂志

Objective To analyze the effect of health education and caring experience on the patients of non neonatal acquired deficiency of vitamin K dependent coagulation factor deficiency (VKDCF) due to rodenticide poisoning.Methods A total of 55 patients diagnosed with acquired deficiency of VKDCF through determining anticoagulant rodenticide concentration in blood were provided with health education and rational medication for 6 months.The patients with unclear poisoning reasons and definite diagnosis were analyzed and given corresponding nursing measures.Results All patients received treatment and corresponding nursing measures.Hemorrhage improved quickly in 2 days and was under control in 1 week.No recurrence happened in half year follow-up visit.Conclusions Systematic treatment and standardized health education are clinically effective on the patients of non neonatal acquired deficiency of VKDCF,and can gain active cooperation and favorable comment of the patients.

Pseudoxanthoma Elasticum Accompanied with Vitamin K-dependent Coagulation Factor Deficiency / 대한피부과학회지

Pseudoxanthoma elasticum is a multiorgan disorder, characterized by ectopic mineralization of elastic fibers of skin, eyes, and cardiovascular system. The dermatologic manifestations include laxity of skin, as well as cutis laxa-like wrinkling especially on both axillae. The classic forms of pseudoxanthoma elasticum are due to mutations in the ATP-binding cassette subfamily C member 6 (ABCC6) gene, a presumed transmembrane transporter expressed primarily in the liver and the kidneys. Recent case reports of mutations of gamma-glutamyl carboxylase (GGCX) gene describe associations with vitamin K-dependent coagulation factor deficiency and pseudoxanthoma elasticum as well. A 23-year old woman presented with laxity on both axillae and trunk and a history of vitamin K-dependent coagulation factor deficiency, diagnosed 2 years ago. Fragmented and markedly degenerated elastic fibers with calcium deposition were identified on biopsy specimens. Herein, we report a case of autosomal recessive pseudoxanthoma elasticum combined with vitamin K-dependent coagulation factor deficiency.

Tardive hemorrhoea cause of osteofascial compartment syndrome after trauma / 中国矫形外科杂志

[Objective]To analyze the reason for osteofascial compartment syndrome caused by tardive massive bleeding and to discuss the first-aid measure to treat continuing bleeding in operation.[Method]There were 5 cases in this study.Two osteofascial compartment syndrome with tardive haematoma were caused by secondary disturbances of blood coagulation.One iliopsoas muscle haematoma was caused by over-dose of warfarin(WARF).Vitamine K was applied to anti-warfarin and depressed haematoma by ultrasonic guiding puncturation aider a pause of warfarin.The other one suffered from continuing bleeding after a depression operation on right femoral bone haematoma.The bleeding could not be stopped until carbasus obturation and a supplementary of lood coagulation factor.The carbasus were taken out 36 hours later.Three traumatic pseudoaneurysm rupture happened to superior gluteal artec,arteriae tibialis posterior and deep femoral artery separately.The bleeding artery was ligated and removed the body of the blood tumor in these three cases.In addition,arterial embolism was applied in two of them before operation.[Result]No recurrence happened to the case with iliopsoas muscle haematoma after 16 weeks as a result of absorption and degeneration.As the patient with right femoral bone haematoma referred,the bleeding stopped after treated by carbasus obturation.The wound was closed 36 hours after the operation.A satisfactory sensory recovery,pulsation of dorsal pedal artery and arteria tibialis posterior were found.Rebleeding was found in 3 weeks after the wound closed.The pathobiology inspection of bone and muscle tissue around the fracture site showed osteoma sarcomatosum in right femoral bone.The author gave the patient to the operation of high amputation.No rebleeding,nervous symptoms caused by compression,limbs necrosis and ischemic contracture was found after 3 patients suffering from traumatic pseudoaneurysm rapture with followed-up from 8 month to 3 years.[Conclusion]Hemorrhea caused by secondary disturbances of blood coagulation or pseudoaneurysm can lead to osteofascial compartment syndrome.Embolism of the bleeding artery before removal of the aneurysm and haematoma can reduce bleeding in operation.It is the best choice for the doctor to stop operation immediately and stop bleeding by effective carbasus obturation.