Cardiac atrophy in women following bed rest

Both chronic microgravity exposure and long‐duration bed rest induce cardiac atrophy, which leads to reduced standing stroke volume and orthostatic intolerance. However, despite the fact that women appear to be more susceptible to post‐spaceflight presyncope and orthostatic hypotension than male astronauts, most previous high‐resolution studies of cardiac morphology following microgravity have been performed only in men. Because female athletes have less physiological hypertrophy than male athletes, we reasoned that they also might have altered physiological cardiac atrophy after bed rest. Magnetic resonance imaging was performed in 24 healthy young women (32.1±4 years) to measure left ventricular (LV) and right ventricular (RV) mass, volumes, and morphology accurately before and after 60 days of 6° head‐down tilt (HDT) bed rest. Subjects were matched and then randomly assigned to sedentary bed rest (controls, n=8) or two treatment groups consisting of (1) exercise training using supine treadmill running within lower body negative pressure plus resistive training (n=8), or (2) protein (0.45 g/kg/day increase) plus branched‐chain amino acid (BCAA) (7.2 g/day) supplementation (n=8). After sedentary bed rest without nutritional supplementation, there were significant reductions in LV (96±26–77±25 mL; P=0.03) and RV volumes (104±33–86±25 mL; P=0.02), LV (2.2±0.2–2.0±0.2 g/kg; P=0.003) and RV masses (0.8±0.1–0.6±0.1 g/kg; P<0.001), and the length of the major axis of the LV (90±6–84±7 mm. P<0.001), similar to what has been observed previously in men (8.0%; Perhonen M.A., Franco F., Lane L.D., Buckey J.C., Blomqvist Zerwekh J.E., Peshock R.M., Weatherall P.T., Levine B.D. J Appl Physiol 2001: 91: 645–653). In contrast, there were no significant reductions in LV or RV volumes in the exercise‐trained group, and the length of the major axis was preserved. Moreover, there were significant increases in LV (1.9±0.4–2.3±0.3 g/kg; P<0.001) and RV masses (0.7±0.1–0.8±0.2 g/kg; P=0.002), as well as mean wall thickness (9±2–11±1 mm; P=0.02). The interaction between sedentary and exercise LV and RV masses was highly significant (P<0.0001). Protein and BCAA supplementation led to an intermediate phenotype with no change in LV or RV mass after bed rest, but there remained a significant reduction in LV volume (103±14–80±16 mL; P=0.02) and major‐axis length (91±5–88±7 mm; P=0.003). All subjects lost an equivalent amount of body mass (3.4±0.2 kg control; 3.1±0.04 kg exercise; 2.8±0.1 kg protein). Cardiac atrophy occurs in women similar to men following sedentary 60 days HDT bed rest. However, exercise training and, to a lesser extent, protein supplementation may be potential countermeasures to the cardiac atrophy associated with chronic unloading conditions such as in spaceflight and prolonged bed rest.     

The Peutz-Jeghers syndrome. Case reports.

Two additional cases of Peutz-Jeghers syndrome are described. One of them, a 19 year-old female, is a sporadic case, whereas in the other case, also a 19 year-old female, there are two members of the family with the Peutz-Jeghers syndrome. A review of some salient features of this entity is made. These include clinical presentation, histopathological features, malignant potential and treatment.     

Percutaneous drainage of 335 consecutive abscesses: results of primary drainage with 1-year follow-up.

Retrospective review of percutaneous abscess drainage (PAD) of 335 abscesses in 323 consecutive patients was undertaken. Particular attention was directed to body location, associated organ system, communications and fistulae, and to the underlying immunologic status of the patient. One-year follow-up was available in all patients. Overall, the cure rate was 62.4% (209 of 335 abscesses), with a failure rate of 8.95% (30 of 335 abscesses). There were 14.2% (46 of 323 patients) deaths in the follow-up period, of which 4.6% (15 of 323 patients) were believed attributable to sepsis or septic complications. The overall complication rate was 9.8% (33 of 335 abscesses), most of which were minor in nature. For the patient exhibiting immunocompromise, representing 53.1% (172 of 323 patients) of the patient population, the cure rate was 53.4% (95 of 178 abscesses), which was significantly lower than the cure rate of 72.6% (114 of 157 abscesses) for the immunocompetent patient population (n = 151) (P less than .001). The recurrence rate was 2.1% (seven of 335 abscesses), with all recurrences within 3 months of initial drainage. PAD is effective and permanent treatment for both immunocompromised and immunocompetent patients.     

Arteriosclerosis obliterans in a rabbit model.

RATIONALE AND OBJECTIVES. The authors induced atherosclerotic occlusions in a rabbit model, using and comparing different experimental methods. METHODS. Lesions were induced in 40 femoral arteries in 20 rabbits. Four combinations of lesion induction methods were used: 1) drying of the endothelium with carbon dioxide gas; 2) gas-drying of the artery plus mechanical injury; 3) gas-drying plus induced thrombosis of the treated segment using thrombin; and 4) gas-drying, mechanical injury, and induced thrombosis. All rabbits were fed a high-fat, high-cholesterol diet for 1 to 2 months after lesion induction. RESULTS. Seventeen rabbits were available for follow-up. Sixty-eight percent (13 of 19) of femoral arteries treated with thrombin-induced thrombosis demonstrated atherosclerotic occlusions, compared with 27% of those that did not receive this treatment (4 of 15; P < .01). CONCLUSIONS. Thrombin-induced thrombotic occlusion of a segment of artery which has been de-endothelialized, followed by a high-fat, high cholesterol diet, results in a higher yield of experimental occlusive atherosclerosis in rabbits than is achievable by other methods.     

Tumor-induced osteomalacia and rickets

Tumor-induced osteomalacia is a clinicopathological entity in which vitamin D-resistant osteomalacia or rickets occurs in association with a tumor. A total of 72 cases (three current, 69 from review of literature) has been reported to date. Men and women are equally affected. The majority are adults over 30 years old who exhibit progressive lower leg and back pain. Forty bone and 31 soft-tissue tumors were responsible for this syndrome; two-thirds occurred in the extremities. Chemical findings are typical: low serum phosphorus, normal serum calcium, and elevated alkaline phosphatase. Serum levels of 1,25-dihydroxyvitamin D were low or undetectable. Histologically, more than a third were classified as vascular tumors, and half of these cases were hemangiopericytomas that were distributed equally between bone and soft tissues. Other common diagnoses included nonossifying fibromas, "mesenchymal" and giant-cell tumor variants. Features common to all tumors were prominent vascularity, and giant and primitive stromal cells. Only 10 were histologically malignant. Ultrastructural studies have not shown any secretory granules suggestive of a hormone-secreting tumor. It is clear, however, that the tumor is responsible for the osteomalacia because the complete removal generally results in a dramatic reversal of all symptoms and signs.     

The Turner syndrome research registry: Creating equipoise between investigators and participants

To address knowledge gaps about Turner syndrome (TS) associated disease mechanisms, the Turner Syndrome Society of the United States created the Turner Syndrome Research Registry (TSRR), a patient‐powered registry for girls and women with TS. More than 600 participants, parents or guardians completed a 33‐item foundational survey that included questions about demographics, medical conditions, psychological conditions, sexuality, hormonal therapy, patient and provider knowledge about TS, and patient satisfaction. The TSRR platform is engineered to allow individuals living with rare conditions and investigators to work side‐by‐side. The purpose of this article is to introduce the concept, architecture, and currently available content of the TSRR, in anticipation of inviting proposals to utilize registry resources.     

Inflammatory pseudotumor of lymph node and spleen: An entity biologically distinct from inflammatory myofibroblastic tumor

Inflammatory pseudotumors (IPTs) of the lymph node and spleen are an uncommon, benign cause of lymphadenopathy and/or splenomegaly that often bear striking clinicopathologic similarities to the inflammatory myofibroblastic tumors (IMTs) found in soft tissues. These tumors have classically been grouped together under the umbrella category of "inflammatory pseudotumor." Recent evidence shows that IMTs are in fact neoplastic processes that often harbor balanced chromosomal translocations involving the ALK kinase gene. These translocations result in expression of ALK kinase in IMTs as assessed by immunohistochemical studies. However, the relationship between IMT and IPT of the lymph node and spleen is uncertain. To determine if ALK tyrosine kinase expression is also present in IPT, 13 cases of IPT (9 involving lymph nodes, 4 splenic lesions) were examined for the presence of ALK tyrosine kinase by immunohistochemical staining on paraffin-embedded tissue. In addition, in situ hybridization studies for Epstein-Barr virus--encoded RNAs (EBER) and immunoperoxidase studies for human herpesvirus-8 (HHV8)--specific proteins were performed. All cases had clinical, morphologic, and immunophenotypic findings typical of IPT and had varying proportions of fibroblastic and inflammatory components. Age ranged from 11 to 75 (median, 40) years; 8 subjects were male, and 5 were female. None of the cases (0 of 13) had positive staining for ALK kinase or HHV8, and in 1 a lymph node (1 of 13) was focally positive for EBV (EBER) by in situ hybridization. The absence of ALK kinase as detected by immunohistochemical studies in IPT of the lymph node and spleen suggests that this entity is biologically distinct from the histologically similar IMT.