Onychopathy in a Patient with Fetal Hydantoin Syndrome

Abstract: Hydantoin is an anticonvulsant drug with several side effects. A teralogenic potential has been suggested. The fetal hydantoin syndrome is an entity that consists of a broad range of morphologic and developmental disorders in children born of epileptic mothers exposed to hydantoin during pregnancy. We treated a girl in whom onychopathy was a monosymptomatic or mild form of this syndrome.     

Expression profiling of metalloproteinases and inhibitors in cartilage

Objective   To profile the expression of all known members of the matrix metalloproteinase ( MMP ), a disintegrin and metalloproteinase with thrombospondin motifs ( ADAMTS ), and tissue inhibitor of metalloproteinases ( TIMP s) gene families in normal cartilage and that from patients with osteoarthritis (OA).
Methods   Human cartilage was obtained from femoral heads at joint replacement for either osteoarthritis or following fracture to the neck of femur. Total RNA was purified and expression of genes assayed using quantitative real-time PCR.
Results   Several members of the above gene families were regulated in OA. Genes increasing in expression in OA were: at P  < 0.001, MMP-13 , MMP-28 , ADAMTS-16 ; at P  < 0.01, MMP-9 , MMP-16 , ADAMTS-2 , ADAMTS-14 and at P  < 0.05, MMP-2 , TIMP-3 , ADAMTS-12 . Genes decreasing in expression in OA were: at P  < 0.001, MMP-1 , MMP-3 , ADAMTS-1 ; at P  < 0.01, MMP-10 , TIMP-1 , ADAMTS-9 and at P  < 0.05, TIMP-4 , ADAMTS-5 , ADAMTS-15 . Correlation analysis revealed that groups of genes across the gene families are co-expressed in cartilage.
Conclusion   This is the first comprehensive expression profile of all known MMP , ADAMTS and TIMP genes in cartilage. Patterns of expression provide a foundation on which to understand mechanisms of gene regulation in OA and potentially for refining the specificity of anti-proteolytic therapies.     

Quantifying the blood oxygenation level dependent effect in cerebral blood volume-weighted functional MRI at 9.4T.

In cerebral blood volume (CBV)-weighted functional MRI (fMRI) employing superparamagnetic contrast agent, iron dose and blood oxygenation level dependent (BOLD) contamination are two important issues for experimental design and CBV quantification. Both BOLD and CBV-weighted fMRI are based upon the susceptibility effect, to which spin-echo and gradient-echo sequences have different sensitivities. In the present study, CBV-weighted fMRI was conducted using spin-echo and gradient-echo sequences at 9.4T by systematically changing the doses of contrast agent. Results suggest that BOLD contamination is a significant component in CBV-weighted fMRI at high field, particularly when relatively low dose of contrast agent is administered. A mathematical model was developed to quantify the extravascular (EV) BOLD effect. With a TE of 35 ms, the EV BOLD effect was estimated to account for 76+/-12% of the observed spin-echo fMRI signal at 9.4T. These data suggest that correcting BOLD effect may be necessary for accurately quantifying activation-induced CBV changes at high field.     

Body composition and physical activity in end-stage renal disease.

OBJECTIVE: The study objective was to examine the relationship between visceral and somatic protein stores and physical activity in individuals with end-stage renal disease. DESIGN: This was a prospective single-center study. SETTING: The study took place at the Vanderbilt University Outpatient Dialysis Unit and General Clinical Research Center. PATIENTS: Fifty-five patients with prevalent chronic hemodialysis (CHD) were included: 33 males, 22 females, 45 African Americans, 9 Caucasians, and 1 Asian. The mean age was 47.0 +/- 1.6 years, height was 166.4 +/- 13.9 cm, and weight was 83.1 +/- 2.6 kg. METHODS: Body composition was measured by dual-energy x-ray absorptiometry. Minute-by-minute physical activity was assessed over a 7-day period with a triaxial accelerometer. Participants were interviewed by a trained registered dietitian for two 24-hour diet recalls (one from a hemodialysis day; one from a nonhemodialysis day). Laboratory values for serum concentrations of albumin, prealbumin, C-reactive protein, and creatinine were also collected. MAIN OUTCOME MEASURE: Predictors of somatic protein stores were the main outcome measure. RESULTS: Serum albumin was negatively and significantly correlated with the percentage of fat mass (P = .016) and kg of fat mass (P = .044). C-reactive protein was positively and significantly correlated with body weight (P = .006), percentage of fat mass (P = .017), kg of fat mass (P = .006), and body mass index (P = .004). Physical activity and total daily protein intake were the strongest predictors of the amount of lean body mass (P = .01 and .003, respectively). CONCLUSION: The association between somatic protein and visceral protein stores is weak in patients with CHD. Whereas increased levels of physical activity and total daily protein intake are associated with higher lean body mass in patients with CHD, higher adiposity is associated with higher C-reactive protein and lower albumin values.     

Obesity and litigation predict workers' compensation costs associated with interbody cage lumbar fusion.

BACKGROUND CONTEXT: Results of lumbar fusion surgery have been mixed and procedures are costly. Interbody cage lumbar fusion (ICLF) has been advanced to improve arthrodesis and clinical outcomes; however, little attention has been given to ICLF costs or potential predictors of these expenses. PURPOSE: To depict medical and compensation costs associated with ICLF in a Utah cohort of patients receiving workers' compensation as well as to investigate predictors of costs. STUDY DESIGN/SETTING: A retrospective-cohort research design was used involving completion of presurgical and postsurgical medical record reviews and accrual of medical and compensation costs. Presurgical variables included in a regression model were presurgical spinal pathophysiology rating, obesity, and litigation status. PATIENT SAMPLE: Forty-three consecutive patients who were compensated by the Workers' Compensation Fund of Utah and underwent ICLF. OUTCOME MEASURES: Total accrued compensation and medical costs. METHODS: A retrospective review of presurgical variables and total accrued compensation and medical costs was conducted. RESULTS: Multiple regression analysis indicated that nonpathophysiological factors predicted compensation costs (lawyer involvement [beta=0.40]; obesity [beta=0.34]). Specifically, compensation for those with versus without lawyers was $41,657 versus $24,837, and for those who were obese versus nonobese was $46,152 versus $28,168. Arthrodesis was correlated with medical costs (r=-0.47, p=.002), with incurred costs for patients achieving solid fusion versus pseudarthrosis equaling $38,881 versus $71,655, respectively. CONCLUSIONS: Considerable costs were associated with ICLF, particularly for those who were obese, involved in litigation, or failed to achieve solid fusion. With regard to compensation costs, the findings support the importance of assessing nonpathophysiological factors in spinal fusion patients.     

Antiparasitic drugs and lactation: focus on anthelmintics, scabicides, and pediculicides.

Infections involving helminths or parasitic worms affect more than 25% of the population worldwide. Because lactating women and their infants are often affected by the complications of these infections such as iron deficiency anemia and malnutrition, they benefit the most from drug therapy. However, scientific literature offers little information regarding transfer of anthelmintics in breast milk and its effects in lactating infants. Scabies and pediculosis, or lice, are common skin infections affecting school-aged children. Close contacts of infected children, which often include lactating mothers, need treatment with one of the several topical products available in the market. This article will review the physicochemical properties and use of anthelmintics, pediculicides, and scabicides while breastfeeding.     

Genetic services for men: the preferences of men with a family history of prostate cancer.

PURPOSE: Men have a lower uptake of genetic services than women; however, the specific needs and preferences of men at risk of genetic conditions other than hereditary breast ovarian cancer are not known. We ascertain the information preferences of men with a family history of prostate cancer. METHODS: Unaffected men and their partners were administered a written questionnaire. RESULTS: Responses were received from 280 men (response rate: 59.2%) and 174 partners (response rate: 74%). Most men (59.6%) reported having insufficient information about their risk and wanted further information about personal risk (93.2%) and risk management (93.6%). Strikingly, 56.3% preferred to receive information related only to positive outcomes. Urologists were the preferred source of information, but there was considerable interest in a multidisciplinary service approach significantly associated with the number of affected relatives (odds ratio = 1.94, P < .002). Partners' level of concern was not associated with interest in multidisciplinary services, satisfaction with information, or support received. CONCLUSIONS: Delivering services to men at risk will require a multifaceted approach by primary care providers and specialists. Challenges include meeting men's expectations in the face of uncertain medical knowledge, engaging those at high risk in multidisciplinary services, and delivering tailored information to those at lower risk.     

Continuous epidural infusion of racemic methadone results in effective postoperative analgesia and low plasma concentrations

PURPOSE: To compare two protocols of epidural administration of racemic methadone for postoperative analgesia (continuous infusion and intermittent bolus), focussing on plasma concentration, analgesic efficacy and side effects. METHODS: Ninety patients undergoing abdominal or lower-limb surgery were randomly assigned to two groups in a prospective double-blind design. The continuous infusion patients (n=60) received initial doses of 3 to 6 mg followed by 6 to 12 mg by continuous infusion over 24 hr. The bolus administration patients (n=30) received repeated boluses of 3 to 6 mg of racemic methadone every eight hours. Pain intensity was assessed on a visual analog scale. Amount of supplementary analgesia was recorded, as was the incidence of side effects. Plasma methadone concentrations were determined by high performance liquid chromatography. Treatment was continued for 72 hr. RESULTS: Pain relief was good and comparable in both groups throughout the three days of treatment. No accumulation of plasma racemic methadone was observed in either group, although the concentrations were significantly higher in the bolus group. Miosis was significantly more frequent in the bolus group. CONCLUSION: Plasma methadone concentrations were significantly lower with continuous infusion. Plasma methadone accumulation, which is considered the main disadvantage for its purported influence on the incidence of side effects, did not occur at the doses used over the three days of treatment that we report.