deoxycholic-acid and Brain-Diseases

Patients with diabetes are known as an important high-risk group for cerebral mucormycosis (CM).. We conducted a structured search using PubMed/MEDLINE to collect both case reports and case series case (ie including at least two patients) onto CM in diabetic patient published between 2000 and March 2020.. Forty-five reports of individual cases and eighteen case series articles were included. India accounted for the largest share of reports with 37.7% and 38.8% of individual cases and case series, respectively. Mortality ranged from 0% to 100% in the case series. The overall mortality in the individual cases was 46.3%, and 64.2% of deaths were reported in patients with ketoacidosis diabetes. Facial swelling (53.3%), headache (44.4%), loss of vision (35.5%) and ophthalmoplegia (35.5%) were the most frequently reported clinical symptoms. In all patients except 4 (91.1%), CM was treated surgically; however, in many cases (42%), despite the use of surgery, death occurred. Amphotericin B deoxycholate (AMB) and lipid-based AMB (LAMB) were used as the first lines of treatment for all patients; however, posaconazole, echinocandins, hyperbaric oxygen therapy (HBOT) and deferasirox were used in combination for a number of patients. Posaconazole has been shown to have positive therapeutic effect; however, posaconazole, LAMB and HBOT are not commonly used in low-income and health-challenged countries.. Cerebral mucormycosis is a rapidly progressive infection in diabetic patients and carries immense morbidity despite early diagnosis and treatment. Low-income countries have had the highest number of reports of the disease in recent years, indicating the need to control diabetes in these countries.

Topics: Amphotericin B; Antifungal Agents; Brain Diseases; Deoxycholic Acid; Diabetes Complications; Diabetes Mellitus; Drug Combinations; Humans; Mucormycosis; Risk Factors; Triazoles

To report the frequency and factors affecting patients', globe and vision survivals in rhino-orbito-cerebral mucormycosis (ROCM).. This is a retrospective study of 63 patients (79 eyes) with biopsy-proven ROCM at a university hospital 2008-2016. Systemic and ophthalmic manifestations, imaging, management and final outcomes were recorded. Globe survival was defined as no exenteration and vision survival as final visual acuity of light perception and more.. Mean age was 55.5 (SD 12.9) years with no gender preference. Diabetes was the most common underlying disease (68.3%). Patient survival was observed in 57.1 % (36/63). Presence of frozen eye (OR 4.6), nasal mucosal involvement (OR 7.3) and shorter duration of antifungal therapy (OR 1.03) were significantly associated with lower patient survival. Exenteration did not significantly change the survival. Globe survival was detected in 43% (34/79). Higher white blood cell (WBC) count was associated with a lower globe survival (p=0.02). Vision survival was observed in 25.3% (20/79) in whom younger age was significantly associated with a worse vision survival.. Patient, globe and vision survivals were 57%, 43% and 25%, respectively. Exenteration did not affect the patients' survival. While frozen eye and nasal mucosal involvement were significantly associated with a lower survival, higher WBC count significantly increased the risk of exenteration.

Topics: Adolescent; Adult; Aged; Amphotericin B; Antifungal Agents; Brain Diseases; Combined Modality Therapy; Debridement; Deoxycholic Acid; Eye Infections, Fungal; Female; Humans; Male; Middle Aged; Mucormycosis; Natural Orifice Endoscopic Surgery; Orbital Diseases; Paranasal Sinus Diseases; Retrospective Studies; Treatment Outcome; Triazoles; Young Adult

Topics: Amphotericin B; Antifungal Agents; Biopsy; Brain Diseases; Deoxycholic Acid; Diabetes Mellitus, Type 2; Drug Combinations; Eye; Eye Diseases; Fatal Outcome; Female; Humans; Kidney Failure, Chronic; Magnetic Resonance Imaging; Middle Aged; Mucormycosis; Multiple Organ Failure; Paranasal Sinuses; Temporal Lobe

Urine samples and serum samples of a patient with cerebrotendinous xanthomatosis (CTX) were investigated by means of capillary gas chromatography, both before and during oral treatment with ursodeoxycholic acid (UDCA), and the results compared with those obtained during chenodeoxycholic acid (CDCA) therapy. The predominantly excreted bile alcohol, 5 beta-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol and two abnormal bile acids, i.e. 23-norcholic acid and 23-hydroxycholic acid were determined. In addition, the serum cholestanol/cholesterol ratio was determined. Whereas previous experiments demonstrated that the urinary excretion of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol and the abnormal bile acids decreased within a few weeks during CDCA therapy, the present study shows that their urinary excretions remain essentially the same during UDCA treatment. In contrast to the decrease in the serum cholestanol/cholesterol ratio during CDCA therapy, this ratio remains essentially the same during UDCA therapy. It is therefore concluded that, in contrast to CDCA therapy, UDCA treatment is not effective in the treatment of CTX.

Topics: Bile Acids and Salts; Brain Diseases; Cholestanols; Chromatography, Gas; Deoxycholic Acid; Female; Humans; Middle Aged; Models, Biological; Ursodeoxycholic Acid; Xanthomatosis

Cerebrotendinous xanthomatosis (CTX) is a rare familiar disease characterized by tendon xanthomas, cataracts, cerebellar ataxia, dementia and an elevated serum cholestanol level. In this paper, a 50-year-old man with typical signs and symptoms of CTX is described. Serum cholestanol and chelesterol concentrations were 17.9-28.6 micrograms/ml and 109-153 mg/dl, respectively. The determination of non-sulfated bile acid concentration in the serum assayed by mass fragmentography disclosed an abnormal profile. The concentration of cholic acid (0.30-0.52 microgram/ml) was higher than normal, while those of chenodeoxycholic acid, ursodeoxycholic acid, deoxycholic acid and lithocholic acid were extremely low or undetectable. Administration of ursodeoxycholic acid (300 mg per day, orally) for 2 weeks resulted in a marked reduction of serum cholic acid concentration. However, serum cholestanol levels remained unchanged.

Topics: Bile Acids and Salts; Brain Diseases; Cerebellar Ataxia; Cholestanols; Cholesterol; Cholic Acid; Cholic Acids; Dementia; Deoxycholic Acid; Humans; Male; Middle Aged; Tendons; Ursodeoxycholic Acid; Xanthomatosis