Recurrent intrahepatic pigmented stones after liver transplantation in a patient with hemoglobin SC disease: case report and review of the literature

Patients with hemoglobinopathies may have hepatic involvement, which if severe, can lead to chronic liver disease and a need for liver transplant. Here, we present a case of a 16-yr-old female adolescent who presented to our center with hemoglobin SC disease, obstructive jaundice because of pigmented intrahepatic biliary stones, and progressive liver disease. She underwent a successful liver transplant but a few years later, she developed recurrent cholangitis and graft dysfunction because of recurrent intrahepatic biliary stones. Recurrent formation of intrahepatic stones after liver transplant is a rare and severe complication in patients with hemoglobinopathies. We recommend hypertransfusion therapy and surveillance imaging studies after liver transplant for early detection and prevention of this complication.     

Beliefs and attitudes related to narghile (waterpipe) smoking among university students in Syria

PURPOSE: To assess the beliefs and attitudes related to narghile (waterpipe) smoking that are likely to contribute to its increased popularity among young people in Syria. METHODS: In 2003, a cross-sectional survey was administered to university students in Aleppo, using an interviewer-administered questionnaire. Overall, 587 students participated in the study (278 males, 309 females; mean age, 21.8 +/- 2.1; response rate 98.8%). RESULTS: The most common positive perceptions of narghile were related to its smell and taste. Negative perceptions of narghile included the smoke produced, the pollution, and perceived adverse health effects. Students believed the popularity of narghile to be part of a rising regional trend in its use, due to its being an appealing way to spend leisure time socializing with friends. More students (49.7%) believed narghile to be more harmful to health than cigarettes, compared with 30% who believed the opposite. Respiratory disease was the most commonly cited health effect of narghile smoking. Family attitudes regarding tobacco use by younger members were more permissive about narghile compared with cigarettes, and, surprisingly, were more permissive about females smoking narghile than males doing so. CONCLUSIONS: The rise in narghile smoking as a trendy social habit appears to be occurring despite considerable appreciation of its potential health risks. Permissiveness of adult family members towards narghile use by young female members, especially in the presence of a strong taboo against female cigarette smoking may contribute to the continuous spread of narghile smoking among women in Syria.     

Gamma-secretase as a pharmacological target in Alzheimer disease research: when, why and how?

Alzheimer disease (AD) is characterized by excessive deposition of amyloid beta-peptides (Abeta peptides) in the form of senile plaques as well as neurofibrillary tangles (NFTs) in the brain. In the amyloidogenic pathway, the amyloid-beta precursor protein (APP) is cleaved by beta-secretase first, followed by gamma-secretase cleavage producing therefore Abeta. This review summarizes the recent findings in the AD field and focuses on the different gamma-secretase inhibitors that have been developed as a therapeutic approach toward AD.     

Breast cancer: adjuvant modalities

The mortality rate due to breast cancer has declined over the preceding decades to a great extent, secondary to the development and use of effective adjuvant therapy. Tamoxifen remains the standard of care in premenopausal women, whereas aromatase inhibitors have become standard therapy after menopause for women with hormone-sensitive disease. Tumor gene profiling assays are being increasingly used to identify women with hormone-sensitive disease, who would benefit from adjuvant chemotherapy. For those women with hormone negative cancer, systemic chemotherapy provides substantial reduction in the risk of disease recurrence and death.     

Delayed hemolytic transfusion reaction in adult sickle‐cell disease: presentations,outcomes, and treatments of 99 referral center episodes

Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle‐cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3–22] days after the triggering transfusion (TT). The most frequent DHTR‐related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso‐occlusive crisis and 50% developed a secondary acute chest syndrome (ACS). The median [IQR] hemoglobin‐concentration nadir was 5.5 [4.5–6.3] g/dL and LDH peak was 1335 [798–2086] IU/L. Overall mortality was 6%. None of the patients had been receiving chronic transfusions. Among these DHTRs, 61% were developed in previously immunized patients, 28% in patients with prior DHTR. Among Abs detected after the TT in 62% of the episodes, half are classically considered potentially harmful. No association could be established between clinical severity and immunohematological profile and/or the type and specificity of Abs detected after the TT. Management consisted of supportive care alone (53%) or with adjunctive measures (47%), including recombinant erythropoietin and sometimes rituximab and/or immunosuppressants. Additional transfusions were either ineffective or worsened hemolysis. In some cases, severe intravascular hemolysis can be likely responsible for the vascular reaction and high rates of ACS, pulmonary hypertension and (multi)organ failure. In conclusion, clinicians and patients must recognize early DHTR signs to avoid additional transfusions. For patients with a history of RBC immunization or DHTR, transfusion indications should be restricted. Am. J. Hematol. 91:989–994, 2016. © 2016 Wiley Periodicals, Inc.     

Tunnelled internal jugular vein catheters with taurolidine lock: an acceptable challenge to arterio-venous fistula in 70 years old haemodialyzed patients: a prospective pilot study

Arteriovenous fistula (AVF) is still in 2010 the gold standard of vascular(2) access in haemodialysis (HD) patients. Nevertheless it may be difficult to obtain and/or to use AVF in elderly. With this prospective randomised pilot study, we compare two strategies of vascular access in 70 years old or more new HD patients. AVF were compared to tunnelled jugular vein catheters (TIJC) with taurolidine as bacterial lock solution. Results were as follow: [table: see text] The responses with the visual analogic scale of comfort was 8/10 for TIJC and 5/10 with AVF * P<0.05. In five TIJC patients, heparin was added with success to taurolidine because of partial clotting of catheters. Albuminemia was significantly lower in AVF failure patients compared to AVF success patients (24.8g/L vs 31.1g/L). This pilot study allows to conclude that TIJC is an acceptable challenge to AVF in haemodialysed patients of 70 years or more in a two years long use.