Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum.

Patients with xeroderma pigmentosum (XP) have defective DNA repair and a high predisposition to developing abnormalities and neoplasia in the sun-exposed areas of the skin and mucous membranes. The most common tumors reported in patients with XP are squamous cell carcinomas, basal cell carcinomas, and melanomas. Atypical fibroxanthoma (AFX) is a pleomorphic tumor that arises predominantly in the sun-damaged skin of the head and neck regions of the elderly. We describe a unique case of a 6-year-old African American boy with XP who developed an atypical fibroxanthoma and 2 squamous cell carcinomas in the conjunctiva. The clinical and histopathologic findings of AFX are discussed.     

Zur Acylierung von Hydroxy- und Mercapto-carbonsäureestern nach dem Carbodiimid/Acylierungskatalysator-Verfahren

On the Acylation of Hydroxy- and Mercaptocarboxylic Acid Esters Using the Carbodiimide/Acylation Catalyst Method Acylation of esters of hydroxycarboxylic acids can be carried out easily and in good yields by using the carbodiimide/acylation catalyst method. The reaction has also been applied to dihydroxy- and mercaptocarboxylic acid esters. The compounds formed from certain esters of hydroxycarboxylic acids are unstable to heat and decompose easily to the free carboxylic acid. No racemization is observed when using enantiomeric hydroxycarboxylic acids. Use of racemic carboxylic acids leads to diastereomers, whose ¹H-NMR shifts for characteristic protons are different as expected. Five drugs are among the carboxylic acids investigated in this context.     

Patient-specific determinants of delay in goal-oriented patient behavior in acute myocardial infarct. Results of the post-infarct late potential study]

369 (63%) of 586 consecutive patients with confirmed myocardial infarction were admitted to hospital within 6 h of symptom onset. Patients' arrival to hospital followed a characteristic circadian distribution with a marked morning increase between 0600 and 1200 hours, and a corresponding decrease in the late night hours. There were no differences in the occurrence of late potentials and in ECG data between early and late arrival group. The early group (less than 5.9 h) was, however, characterized by significantly higher cardiac enzyme levels than the late group (greater than 6.0 h); they also experienced more arrhythmic events in the acute phase (16.0% vs. 9.7%; p less than 0.032). Catecholamines were significantly more often necessary (26.4% vs. 10.3%; p less than 0.0001). Six-month prognosis after AMI was markedly worse. The time between symptom onset and hospital arrival was not affected by age and risk factors. In univariate analysis, pain history and recurrent infarction also had no influence on delay of admission to hospital. The time interval was, however, significantly shorter when the acute event occurred during the night (18.4% vs. 8.3%; p less than 0.0001). Absence of prodromi in the prehospital phase (18.5% vs. 12.0%; p less than 0.04) and a higher socio-economic level (68.3% vs. 61.0%; p less than 0.077) also shortened the admission time, whereas a hyperactive behavioral pattern prolonged the delay time (21.1% vs. 28.9%; p less than 0.05).     

Computer-generated bone models in the planning of osteotomy of multidirectional distal radius malunions.

Computer-assisted design and manufacturing technology has been used to create solid models of five unusually complex, multidirectional malunions of distal radius fractures. Preoperative planning was dramatically enhanced by the ability to perform the surgical procedure on these models, with a model of the uninjured limb used for comparison. All five patients had significant malunions, with malrotation in the horizontal plane in five and an impacted articular fragment in two. A satisfactory outcome was achieved in each case.     

Acute peripheral arterial and graft occlusion: treatment with selective infusion of urokinase and lysyl plasminogen

Thirty-five patients hospitalized for recent angiographically documented arterial occlusion in the legs (27 femoropopliteal arteries and eight grafts) benefited from local fibrinolytic therapy delivered at the site of the occlusion with a 4- or 5-F catheter. This therapy combined a continuous urokinase (UK) infusion of 1,000 U/kg/hour and a lysyl plasminogen (LYS-PLG) infusion of 15 microkatals every 30 minutes. Angiographically confirmed lysis was obtained in 85% of the cases. Only 3% of the patients had major and 6% had minor groin hematomas. Only two patients had concentrations of fibrinogen as low as 100 mg/dl. Intravascular infusion of UK-LYS-PLG is as effective as streptokinase. Its excellent tolerance makes it a good alternative in the treatment of acute ischemia in the lower limbs.     

The effect of immobilization on goat knees following reconstruction of the anterior cruciate ligament

Significant patellofemoral chondromalacia was observed three months after operations on goat knees that had an excised anterior cruciate ligament (ACL) replaced and then were immobilized for six weeks with a rigid external pin and frame fixator. To determine if the patellofemoral morbidity rate could be reduced, another group of goats was treated with the same operation but without immobilization. The legs of the animals in the immobilized group had muscle weakness and the animals only occasionally used their legs for the next three to four weeks. The animals in the mobilized group protected the leg for only one to two days after surgery, and by ten to 12 days were actively moving the knee. Severe patellofemoral articular cartilage erosions occurred in the immobilized goats but not in the mobilized group. Postoperative immobilization was detrimental to the joint function and to a successful ACL reconstruction.     

Corticosterone controls the developmental emergence of fear and amygdala function to predator odors in infant rat pups

In many altricial species, fear responses such as freezing do not emerge until sometime later in development. In infant rats, fear to natural predator odors emerges around postnatal day (PN) 10 when infant rats begin walking. The behavioral emergence of fear is correlated with two physiological events: functional emergence of the amygdala and increasing corticosterone (CORT) levels. Here, we hypothesize that increasing corticosterone levels influence amygdala activity to permit the emergence of fear expression. We assessed the relationship between fear expression (immobility similar to freezing), amygdala function (c-fos) and the level of corticosterone in pups in response to presentation of novel male odor (predator), littermate odor and no odor. CORT levels were increased in PN8 pups (no fear, normally low CORT) by exogenous CORT (3 mg/kg) and decreased in PN12 pups (express fear, CORT levels higher) through adrenalectomy and CORT replacement. Results showed that PN8 expression of fear to a predator odor and basolateral/lateral amygdala activity could be prematurely evoked with exogenous CORT, while adrenalectomy in PN12 pups prevented both fear expression and amygdala activation. These results suggest that low neonatal CORT level serves to protect pups from responding to fear inducing stimuli and attenuate amygdala activation. This suggests that alteration of the neonatal CORT system by environmental insults such as alcohol, stress and illegal drugs, may also alter the neonatal fear system and its underlying neural control.     

Heart rate changes in chronic insomnia

This study assessed physiological activity in patients with chronic insomnia before sleep, during sleep and in response to acute stress. Twenty-four subjects with chronic insomnia and 25 normal sleepers slept in the laboratory overnight and were given a stressful performance task in the morning. Heart rate was significantly higher in the insomniac group at night. The next morning, heart rate was not different at baseline, but was significantly higher during the performance task in the insomniac group. These results are discussed as supporting the notion that insomniacs have greater physiological responsivity to stress. Further research is needed to determine if altered physiological activity is a cause or consequence of insomnia.     

Stage II carcinoma of the ovary: an analysis of survival after comprehensive surgical staging and adjuvant therapy.

Ninety-three women with FIGO stage II epithelial ovarian carcinoma underwent comprehensive surgical staging and were randomized prospectively to therapy consisting of either intraperitoneal radioactive phosphorus or oral melphalan. No patient had gross residual disease at the time of randomization. Ten of the forty-five women treated with melphalan experienced severe bone marrow depression at some time during therapy and two women expired from leukemia. Four of the forty-eight women treated with intraperitoneal phosphorus required surgical reexploration for intestinal obstruction or bowel injury. Twenty-one women died of their disease. Survival was not statistically different between the two treatment arms. The 5-year actuarial survival was 78%.     

Quantitative whole-body autoradiographic analysis of the tissue distribution of orally-administered [14C]cholesterol in hypercholesterolemic rats

The cholesterol-fed rat model has been used to examine the distribution of radiolabeled cholesterol by whole-body autoradiographic and quantitative videodensitometric methods. Animals were fed a hypercholesterolemic diet for 7 days, and were subsequently killed at 3, 6, 12, 24, or 72 h following a single oral dose of [14C]cholesterol. Maximum blood and tissue levels were observed at 12 h, while liver and adrenals were the most intensely labeled tissues. Liver maintained consistently high levels over the course of the study, while activity in other tissues declined moderately by 72 h, indicating the long half-life of cholesterol radioequivalents in tissue. The results of these experiments suggest that autoradiographic examination of cholesterol distribution in animals treated with pharmaceutical agents designed to modify cholesterol absorption or clearance will be useful in providing supplemental or confirmatory information on the drugs' mode of action.