Hereditary haemochromatosis presenting as diabetes and complicated with abnormal liver function, hypogonadism, osteoporosis: a case report / 中华内分泌代谢杂志

Hereditary hemochromatosis(HH) is relatively rare in the Chinese population, and the disease can involve multiple systems. It is easy to be missed and misdiagnosed due to nonspecific clinical manifestations. We report on a case with diabetes as the first diagnosis and being confirmed HH later. In addition to abnormal liver function, this patient also developed a variety of endocrine and metabolic diseases such as hypogonadism and osteoporosis. Included with this case report is a literature based discussion of clinical features, management of HH along with its relationship with endocrine dysfunction to improve disease understanding.

Application of ultrasound in extracorporeal membrane oxygenation for the liver donation after brain-cardiac death / 器官移植

Objective To evaluate the application value of ultrasound in extracorporeal membrane oxygenation (ECMO)for protecting the liver donation after brain-cardiac death (DBCD).Methods Forty patients with brain death or irreversible brain injury,admitted to Guangzhou General Hospital of Guangzhou Military Command from April 2006 to November 201 4,were eligible for liver donation.The hepatic artery blood flow (QHA),portal vein blood flow (QPV)and ECMO-induced ECMO flow of hepatic artery (VE)of the donor liver were monitored by ultrasound before,5 min after the initiation of ECMO and immediately after ECMO.The changes of total bilirubin (TB),alanine transaminase (ALT)and lactic acid were observed at corresponding time points.Hepatic recovery was subsequently evaluated within 3 months after liver transplantation.Results The mean time of ECMO was (1 .0 ±0.2)h.There was no significant difference in QHA and QPV before and after ECMO (both in P >0.05).And there was no significant difference in liver function parameters before and after ECMO (all in P >0.05).At different time points within postoperative 3 months,the results of ultrasound evaluation and liver function test revealed that the transplant liver function was well recovered in 40 recipients.Conclusions Through monitoring QHA by ultrasound,the best ECMO flow should be chosen,which protects DBCD liver and averts perfusion injury and hypoperfusion.