Hemophilia is a hereditary disorder in which the major clinical manifestation is bleeding into the joints, muscles, internal organs and the CNS, often without any obvious trauma. Bleeding can be fatal as in the case of CNS hemorrhage, or severely debilitating following repeated bleeding into joints that results in crippling arthritis. Treatment for hemophilia includes the intravenous administration of clotting factor concentrates to replace the missing or defective protein. Venous access is therefore critical to the treatment of hemophilia and the prevention of complications due to bleeding. According to the US Centers for Disease Control and Prevention, approximately half of patients less than 16 years of age and one-third of all patients with hemophilia receive regular prophylactic injections of clotting factor concentrates. Prophylaxis, or the regular scheduled administration of antihemophilic factor concentrate, is effective in preventing bleeding. Among those patients with severe disease, in the absence of prophylaxis, approximately 13 bleeds, including nine joint hemorrhages, occur annually. In contrast, when prophylaxis is administered, the annual number of total and joint bleeds decreases to five and three, respectively. One of the major barriers to the more wide-spread use of prophylaxis is venous access. While peripheral venipuncture remains the first choice for venous access, central venous access devices are frequently used to facilitate repeated and/or urgent administration of clotting factor concentrates. The advantages of central venous access devices are well recognized in certain treatment regimens such as prophylaxis and immune tolerance therapy, as well as certain patient groups such as young children in whom venipuncture is often difficult and traumatic, and adults with scarred veins. Central venous access devices also allow earlier commencement of both home treatment and prophylaxis. The goal of this review is to discuss the different types of central venous access devices and their role in the management of hemophilia to provide practitioners that care for patients with hemophilia with the necessary information to make sound therapeutic recommendations to their patients.

Obstructive sleep apnea (OSA) is a fairly common condition that, if left untreated, can lead to complications such as high blood pressure and heart disease. Polysomnography (PSG) is the most accurate method for diagnosing OSA, but it is a cumbersome and expensive test. A well-validated, easier to perform and less expensive alternative is the home sleep test (HST). The purpose of this review is to educate the primary care provider about the important differences between PSG and HSTs, the advantages and limitations of both modalities, identifying patients who are appropriate candidates for the HST, identifying patients in whom the HST should not be performed, and further evaluation of patients who have a negative HST. (J Am Board Fam Med 2015;28:504 -509.)

School-based scoliosis screening programs, although controversial, remain well established. Our hypothesis was that with the addition of several inexpensive and simple measurements (height/weight, questionnaire), the overall impact of our screening program could be dramatically increased. Of the 1,058 children screened, 30 (2.8%) were positive for scoliosis. We found that 284 children (27%) were overweight and 468 (44%) children were overweight or at risk for overweight. Additionally, 61% of children did not have an identified primary health provider. These additional measurements did not change the efficacy of scoliosis screening but dramatically increased the number of children identified at risk for significant health problems.

We report the case of a 52-year-old female with spinocerebellar ataxia (SCA) 13. She presented with complaints of insomnia and had a history of restless legs syndrome. Her polysomnogram revealed that she had a signifi cantly elevated periodic limb movement index, mild obstructive sleep apnea, and the absence of REM sleep. Sleep disorders have previously been described in patients with SCA 1, SCA 2, SCA 3, and SCA 6. To our knowledge, this is the fi rst description of sleep disorders in a patient with SCA 13.

Comment on Ayache and Strohl. High interrater reliability of overnight pulse oximetry interpretation among inexperienced physicians using a structured template.