Unusual case of malignant pleomorphic fibrohistiocytoma of the abdominal wall

The authors report their experience with a case of a voluminous malignant pleomorphic histiocytoma of the anterior abdominal wall occurring in an 81-year-old female patient. She presented with a one-year history of a non-painful, poorly mobile tumefaction which had grown in size over the 2 months prior to admission. A preoperative CT scan confirmed the presence of a 10-cm mass located in the anterior abdominal wall. The patient underwent resection of the tumour and was discharged on postoperative day 15. A 12-month follow-up has so far revealed no evidence of recurrence. The authors go on to examine the diagnostic and therapeutic treatments of these rare neoplasms with reference to the international scientific literature. They confirm the validity of radiotherapy but stress the elective role of surgical treatment.     

Circadian organization of serum electrolytes in physiological aging

Age-related structural and neurochemical changes occurring in the central nervous system have been related to changes in some rhythmometric parameters. In spite of their clinical importance, only a few studies have investigated the modifications over time of serum electrolytes in senescence. The aim of our study was to evaluate the circadian pattern of serum potassium, chloride, sodium, calcium and phosphorus in 30 clinically healthy elderly subjects, with no cognitive impairment, and to compare the findings with those given by 24 healthy young controls. The subjects were synchronized as regards their daily activities, sleeping/waking habits, time/quality of meals and dietary electrolyte intake. After an overnight fast, samples were taken beginning at 08.00 and every 4 h thereafter until 20.00, and every 2 h from 20.00 to 04.00. Both the young and the elderly subjects exhibited statistically significant circadian rhythms for all serum electrolytes considered. Our findings suggest that circadian organization of serum electrolytes is maintained in physiological aging, even though it should be noted that sodium and phosphorus acrophases differed significantly in the two experimental groups.     

Ectopic thyroid: report of a case

The development of thyroid tissue can occur in any moment of the migration of the thyroid along the thyroglossal duct from the tongue, resulting in lingual (at tongue base), sublingual (below the tongue), prelaryngeal (in front of the larynx), and substernal (in the mediastinum) ectopy. Thyroglossal duct cyst is the most common type of clinical abnormality related to thyroid ectopy. Surgical removal of such ectopic tissue is justified since some Authors describe thyroid cancer arising from aberrant thyroid tissue. The Authors report a case of thyroid ectopy in a patient who underwent 20 years before a left thyroid resection with isthmectomy; during the operation the surgeon described a hypertrophic pyramidal lobe which was left in situ and the patients did not receive any hormone suppressing therapy.     

Anti-endotoxin therapy in primate bacteremia with HA-1A and BPI.

OBJECTIVE: The in vivo neutralizing activities of an anti-lipopolysaccharide (LPS) antibody HA-1A (Centoxin [Centocor, Malvern, PA]), a human immunoglobulin M monoclonal antibody, and of bactericidal/permeability-increasing protein (BPI), an endogenously produced human LPS-neutralizing protein, were studied in a primate model of lethal Escherichia coli bacteremia. SUMMARY BACKGROUND DATA: HA-1A has been used with variable success against LPS activity in some animal models and in a recently reported clinical trial. However, no data assessing the efficacy of this agent in subhuman primates is available. Bactericidal/permeability-increasing protein is a product of polymorphomononuclear cells (PMNs) that is stored in azurophilic granules and exhibits LPS-neutralizing activity in vitro and in some in vivo models. METHODS: Immediately after E. coli infusion and in a blinded fashion, three baboons were treated with BPI (5 mg/kg bolus infusion and 95 micrograms/kg/min infusion over 4 hr). Three animals received 3 mg/kg BW of HA-1A, whereas another three baboons received a placebo treatment. RESULTS: The BPI-treated animals demonstrated significantly (p < 0.03) lower circulating LPS-limulus amoebocyte lysate (LAL) activity compared with the control animals, but this reduction in LPS-LAL activity was not associated with improved survival. HA-1A treatment did not reduce LPS-LAL activity. However, both BPI and HA-1A treatment did attenuate the pro-inflammatory cytokine response. CONCLUSION: The current data suggests that incomplete neutralization of endotoxin activity does not alter mortality from severe bacteremia. Given the diversity of mediator production under such circumstances, a strategy of combination therapy in the form of anti-lipopolysaccharide and anticytokine treatment may be necessary to achieve optimal survival.     

Regulation of neutrophil migration and superoxide production by recombinant tumor necrosis factors-alpha and -beta: comparison to recombinant interferon-gamma and interleukin-1 alpha

We compared the ability of recombinant human tumor necrosis factor- alpha (rHuTNF-alpha) and tumor necrosis factor-beta (rHuTNF-beta) to stimulate polymorphonuclear neutrophil (PMN) migration and superoxide production. Significant PMN migration occurred across polycarbonate filters after stimulation with rHuTNF-alpha at concentrations ranging from 10(-7) to 10(-10) mol/L and at 10(-7) to 10(-8) mol/L for rHuTNF- beta and N-formylmethionyl-leucyl phenylalanine (FMLP), whereas recombinant human interferon-gamma was only minimally active at 10(-7) mol/L and recombinant human interleukin-1 alpha was inactive at the doses tested. In addition, antibodies to rHuTNF-alpha completely inhibited rHuTNF-alpha but not rHuTNF-beta or FMLP-induced PMN migration. Combinations of rHuTNF-alpha and rHuTNF-beta (at similar molar concentrations) stimulated PMN migration levels comparable to that obtained with rHuTNF-alpha alone. Checkerboard analyses performed by placing different concentrations of rHuTNF-alpha and rHuTNF-beta above and below polycarbonate filters of microchemotaxis chambers demonstrated that rHuTNF-alpha and rHuTNF-beta stimulated both chemotactic and chemokinetic responses by PMN. Additional studies demonstrated that 1 X 10(-8) mol/L rHuTNF-alpha and 3 X 10(-9) mol/L rHuTNF-beta (which represents 10(4) U/mL of each cytokine) were similar in their ability to induce superoxide production by PMNs; however, at ten- to 100-fold lower molar concentrations (10(3) and 10(2) units), rHuTNF-alpha was significantly more active than rHuTNF-beta. At the doses tested, both cytokines were less active than phorbol myristate acetate at stimulating O2- release. The results demonstrate that rHuTNF- alpha and rHuTNF-beta differ quantitatively but not qualitatively in their effects on PMN functions in vitro and suggest that rHuTNF-beta may be less toxic than rHuTNF-alpha in vivo.     

Comparative neuropsychological profile of pathological gambling,hypersexuality, and compulsive eating in Parkinson's disease

Background: Impulse control disorders (ICDs), in particular pathological gambling, hypersexuality, and compulsive eating, are being increasingly identified in Parkinson's disease (PD) patients. Pathological gambling has been associated with frontal/executive dysfunctions, whereas hypersexuality and compulsive eating, and their relation with cognitive dysfunctions, have not been investigated in PD. Methods: We investigated cognitive correlates underpinning pathological gambling, hypersexuality, and compulsive eating in PD. PD outpatients were screened for pathological gambling, hypersexuality, and compulsive eating. Based on clinical criteria, we identified 13 patients with hypersexuality, 12 with compulsive eating, 14 with pathological gambling, and 10 with multiple ICDs. Fourteen PD patients matched for age and education without ICDs served as controls. Clinical features and neuropsychiatric and neuropsychological functioning were assessed in the 5 groups. Results: Demographic, clinical, neuropsychiatric, and neurological aspects did not differ among groups. All 4 groups of ICD patients were impaired on tasks exploring spatial‐planning and set‐shifting tasks compared with the controls. The main difference among patients with pathological gambling, hypersexuality, and compulsive eating was that patients with hypersexuality were more impaired on the Stroop test than patients with pathological gambling. Individuals with hypersexuality, compulsive eating, and multiple ICDs performed worse on verbal learning and memory tests than did patients with pathological gambling. Discussion: ICDs are associated with impaired cognitive functions; the severity of impairment decreased in the order multiple ICDs and hypersexuality > compulsive eating > pathological gambling. Our findings support the idea that hypersexuality is associated with prefrontal and memory dysfunctions, whereas pathological gambling seems to be related only to frontal dysfunction. © 2011 Movement Disorder Society     

The 9-bp deletion in region V of mtDNA: a risk factor of hearing loss and encephalomyopathy in Caucasian populations?

A deletion of one of the two copies of the 9-bp tandem repeat sequence (CCCCCTCTA), in the small non-coding/untranslated segment located between the cytochrome oxidase II and lysine tRNA genes of mitochondrial DNA (mtDNA), has previously been used as a polymorphic anthropological marker (MIC9D) for people of Africa and Asia, but it has been rarely reported in Europe. 32 Sicilian patients with syndromic hearing loss, negative for mutations in GJB2 and GJB6 genes, were tested for mtDNA known point mutations associated with syndromic or non-syndromic hearing loss by RFLP and/or direct sequencing. We identified the presence of the MIC9D in homoplasmy in lymphocytes and muscle of three subjects with sensorineural hearing loss and encephalomyopathy, two of these also presented moderate mental retardation. This deletion was absent in 300 Caucasian controls. Although further studies are warranted, our results suggest that the MIC9D polymorphism could have a susceptibility role in Caucasus, such as Sicily population.