Sickle cell disease: imaging of cerebrovascular complications

This case is reported because of the unusual blood findings, no duplicate of which I have ever seen described. Whether the blood picture represents merely a freakish poikilocytosis or is dependent on some peculiar physical or chemical condition of the blood, or is characteristic of some particular disease, I cannot at present answer. I report some details that may seem non-essential, thinking that if a similar blood condition is found in some other case a comparison of clinical conditions may help in solving the problem.     

Cervical screening adjuncts: recent advances

In an effort to reduce the false-negative rate of cervical cytologic findings, several new technologies have recently evolved. Automated cytologic testing (PapNet, AutoPap 300 QC) proposes to rescreen negative conventional cytologic findings to identify smears likely to be false negative. Fluid-based monolayers (ThinPrep, CytoRich) propose to reduce the false-negative rates by optimizing the collection and preparation of cells. Human papillomavirus deoxyribonucleic acid testing by Hybrid Capture has been proposed for a variety of screening and triage roles. Visual screening after application of acetic acid is done by cervicography by use of a photographic technique, whereas in speculoscopy the screening is done by direct visualization of the cervix by the primary care provider. Polarprobe uses biophysical parameters and a computer algorithm to give an instantaneous prediction of the likelihood of cervical disease. Each of these techniques, as well as the clinical experience with them, is reviewed. Current and possible future uses are discussed with regard to both clinical usefulness and cost-effectiveness.     

Sickle cell disease pain: Relation of coping strategies to adjustment.

This study examines pain coping strategies in a relatively neglected pain population, sickle cell disease (SCD) patients. Seventy-nine patients diagnosed with SCD were given a structured interview to assess pain, activity level, and health care use during painful episodes. Patients also completed the SCL-90—R as an index of psychological distress and the Coping Strategies Questionnaire. Regression analyses controlled for age, sex, and disease severity measures. Results indicated that the coping strategies factors were important predictors of pain and adjustment. Individuals high on Negative Thinking and Passive Adherence had more severe pain, were less active and more distressed, and used more health care services. Individuals high on Coping Attempts were more active during painful episodes. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Sickle cell anemia

Sickle cell disease is a hereditary chronic hemolytic anaemia mostly found in negroes. The blood of these patients contains hemoglobin S. Absence of oxygen determines the sickling to the red blood cells. In the heterozygous state clinical manifestations usually are absent. The homozygous form shows different symptoms. The progressive course with hemolytic, aplastic or thrombotic crises mostly ends in an early death in infancy or childhood. Treatment is largely palliative. Transfusions, treatment of the crises and general measures play the most essential role.     

Sickle cell hepatopathy

Hepatic dysfunction is a common finding in patients with sickle cell disease but viral hepatitis appears to be an unusual complication in the adult SS patient. Only five cases of viral hepatitis were recorded in 378 admissions for SS crisis. In contrast, hepatic crisis occurred as a distinct event in 9% of 88 patients with sickle cell anemia. This entity must be differentiated from acute cholecystitis or viral hepatitis. Transiently abnormal results of hepatitic function tests were observed in another 26 patients with extrahepatic crisis. Cirrhosis is relatively common and often the terminal event in SS disease. Choledocholithiasis and cholecystitis are infrequent complications despite the prevalence of gallstones in SS anemia.     

Molecular biology of opioid receptors: recent advances

Endogenous opioid peptides and opiates like morphine produce their pharmacological effects through the membrane bound opioid receptors. These receptors belong to a superfamily of G-protein-coupled receptors, all of which possess seven membrane-spanning regions. Structure-activity relationship studies of opioids opened up new avenues for the pharmacological characterization of the opioid receptors. As a further advancement in this direction, molecular cloning has led to the identification of three different types of opioid receptors -- OP1 (delta), OP2 (kappa) and OP3 (mu) -- thereby supporting the results of earlier pharmacological studies which postulated their existence. The three opioid receptors are highly homologous. Consequent to the development of highly specific and selective agonists and antagonists, it was proposed that the three types of opioid receptors could be further categorized into different subtypes. However, the molecular biology data generated so far do not support the presence of the various subtypes of the three well-characterized opioid receptors. Recent strides towards the advancement of our knowledge relating to the molecular biology of these receptors have been reviewed in this article.     

Dental hypersensitivity: recent advances in diagnosis and treatment

Perirenal extravasation of urine with an intact renal parenchyma and with dye visualized in the ureter was seen on an excretory urogram (IVP) and computed tomographic (CT) scan in two patients (bilateral in one) with multiple injuries following blunt trauma. Expectant management with no invasive procedures resulted in disappearance of the extravasation within 3 to 5 days. Traumatic rupture of a calyceal fornix is thought to be the cause of the extravasation similar to the perirenal extravasation seen with renal colic from a ureteral calculus.     

Sickle cell disease pain in children and adolescents: change in pain frequency and coping strategies over time

Examined 9-month follow-up data obtained from children and adolescents with sickle cell disease (SCD) and their parents participating in a longitudinal study of pain coping strategies. Of 87 subjects completing the baseline assessment of pain coping strategies, 70 (80%) of their parents completed a structured pain interview assessing their child's health care use and activity reduction during painful episodes over the follow-up period. Regression analyses controlling for age and pain frequency revealed that baseline Coping Attempts were associated with higher levels of school, household, and social activity during painful episodes. Baseline Passive Adherence was associated with more frequent health care contacts during the subsequent 9 months. Increases in Negative Thinking over time were associated with further increases in health care contacts during the follow-up period. Comparing pain coping strategies assessed at baseline to pain coping strategies measured at follow-up revealed that pain coping strategies were relatively stable over time for younger children but changed more for adolescents.     

Receptor-mediated endocytosis in kidney proximal tubules: recent advances and hypothesis

Preparation of kidney proximal tubules in suspension allows the study of receptor-mediated endocytosis, protein reabsorption, and traffic of endosomal vesicles. The study of tubular protein transport in vitro coupled with that of the function of endosomal preparation offers a unique opportunity to investigate a receptor-mediated endocytosis pathway under physiological and pathological conditions. We assume that receptor-mediated endocytosis of albumin in kidney proximal tubules in situ and in vitro can be regulated, on the one hand, by the components of the acidification machinery (V-type H+-ATPase, Cl(-)-channel and Na+/H+-exchanger), giving rise to formation and dissipation of a proton gradient in endosomal vesicles, and, on the other hand, by small GTPases of the ADP-ribosylation factor (Arf)-family. In this paper we thus analyze the recent advances of the studies of cellular and molecular mechanisms underlying the identification, localization, and function of the acidification machinery (V-type H+-ATPase, Cl(-)-channel) as well as Arf-family small GTPases and phospholipase D in the endocytotic pathway of kidney proximal tubules. Also, we explore the possible functional interaction between the acidification machinery and Arf-family small GTPases. Finally, we propose the hypothesis of the regulation of translocation of Arf-family small GTPases by an endosomal acidification process and its role during receptor-mediated endocytosis in kidney proximal tubules. The results of this study will not only enhance our understanding of the receptor-mediated endocytosis pathway in kidney proximal tubules under physiological conditions but will also have important implications with respect to the functional consequences under some pathological circumstances. Furthermore, it may suggest novel targets and approaches in the prevention and treatment of various diseases (cystic fibrosis, Dent's disease, diabetes and autosomal dominant polycystic kidney disease).