Non-anti-MAG DADS neuropathy as a variant of CIDP: clinical, electrophysiological, laboratory features and response to treatment in 10 cases.

BACKGROUND AND PURPOSE: Some patients within the spectrum of chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) have distal acquired demyelinating symmetric (DADS) neuropathy, usually associated with anti-myelin-associated-glycoprotein (MAG) IgM monoclonal gammopathy. The aim of this retrospective study was to investigate patients with DADS neuropathy without anti-MAG antibodies, and study their response to immunotherapy. METHODS: Patients were selected on the basis of (i) 'Definite CIDP' according to the EFNS/PNS Guideline criteria, (ii) The presence of disproportionately prolonged motor latencies resulting in a terminal latency index (TLI) ≤ 0.25 in at least two motor nerves and (iii) The absence of anti-MAG antibodies on ELISA. Response to immunotherapy was defined as persistent improvement by at least one point on the INCAT disability score. RESULTS: Data from 146 CIDP patients were analysed, and 10 patients were included. Six had clinically pure sensory neuropathy, and four had sensorimotor neuropathy. Ataxia was present in nine patients, generalized areflexia in seven and postural tremor in two. Five of the 10 patients had abnormal sensory potentials only in the upper limbs. An associated condition was found in nine patients: two chronic lymphocytic leukaemias, four IgG monoclonal gammopathies (one associated with non-Hodgkin's lymphoma) and two IgM monoclonal gammopathies of unknown significance. Patients were mostly improved with intravenous immunoglobulin (IVIg), corticosteroids, plasma exchanges, or a combination thereof. CONCLUSION: DADS neuropathy without anti-MAG antibodies is more likely to be considered a variant of CIDP. In addition, such patients should be systematically investigated for an associated haematological or immunological condition.

Superfluorinated and NIR-luminescent gold nanoclusters.

A novel class of superfluorinated and NIR-luminescent gold nanoclusters were obtained starting from a branched thiol, bearing 27 equivalent 19F atoms per molecule. These unprecedented clusters combine in a unique nanosystem both NIR photoluminescence and 19F NMR properties, thus representing a promising multimodal platform for bioimaging applications.

Characterization of the bionano interface and mapping extrinsic interactions of the corona of nanomaterials.

Nanoparticles in physiological environments are known to selectively adsorb proteins and other biomolecules forming a tightly bound biomolecular 'corona' on their surface. Where the exchange times of the proteins are sufficiently long, it is believed that the protein corona constitutes the particle identity in biological milieu. Here we show that proteins in the corona retain their functional characteristics and can specifically bind to cognate proteins on arrays of thousands of immobilised human proteins. The biological identity of the nanomaterial is seen to be specific to the blood plasma concentration in which they are exposed. We show that the resulting in situ nanoparticle interactome is dependent on the protein concentration in plasma, with the emergence of a small number of dominant protein-protein interactions. These interactions are those driven by proteins that are adsorbed onto the particle surface and whose binding epitopes are subsequently expressed or presented suitably on the particle surface. We suggest that, since specific tailored protein arrays for target systems and organs can be designed, their use may be an important element in an overall study of the biomolecular corona.

Radiofrequency catheter ablation in drug refractory maternal supraventricular tachycardias in advanced pregnancy.

BACKGROUND: Treatment of maternal tachyarrhythmias in pregnancy is a major clinical issue. Pharmacological treatment raises important concerns regarding partial efficacy and side effects. Radiofrequency ablation of arrhythmogenic substrate has rarely been performed during pregnancy because of the fetal risks related to x-ray exposure and potential fetomaternal procedural complications. CASES: Three women affected by supraventricular tachycardias refractory to pharmacological therapy underwent successful radiofrequency catheter ablation at 29 to 30 weeks' pregnancy. All patients had cesarean delivery of newborns with normal Apgar scores. CONCLUSION: Radiofrequency catheter ablation is an effective treatment of drug refractory maternal supraventricular tachycardias in advanced pregnancy. Further studies are required to establish its long-term fetal safety.

Organ-specific autoimmunity in patients with premature ovarian failure.

The diagnosis of premature ovarian failure (POF) was made in 45 women (aged 14 to 39 yr) with high concentrations of FSH and LH. Sera from these patients and a control group of 28 women were used to detect organ-specific autoantibodies. Eighteen patients with POF (40%) were positive for at least one autoantibody. In the control group only one woman (3.6%) showed autoimmunity. Antithyroid was the most representative autoimmunity (20%) in the POF group. By indirect immunofluorescence, only one patient was positive for antiovarian and antiadrenal antibodies. In summary, the results of this study are consistent with previous immunohistochemical data which indicate that autoimmune disorders are common in patients with POF. Furthermore, the detection of antiovarian antibodies in only one patient confirms that they are probably detectable in a short period of the disease.

[Large colpohematometra with bilateral hematosalpinx resulting from progressive obstruction caused by incomplete vaginal septum]. / Voluminoso colpoematometra con ematosalpinge bilaterale da ostruzione progressiva di setto vaginale incompleto.

Complete or incomplete transverse vaginal septum is a rare malformation of the female genital tract. Usually the complete congenital type occurs at puberty because of the collection of menstrual blood above the septum with amenorrhea and cyclic lower abdominal pain as presenting symptoms. On the contrary, in the case shown by the authors, the subacute epilogue occurred in the perimenopausal phase: a very large colpohematometra is reported in a 49 years old woman, with an incomplete vaginal septum resulting in progressive obstruction. The association between this malformation and the presence of endometriotic localizations in the genital tract, as reported by other authors, is interesting. In this case, endometriosis can be secondary to the presence of the septum or could have determined the impairment of the obstruction in consequence of the associated status of chronic flogosis.

Complete testicular feminization with normal gonadotropin and high androgen secretion: a case report.

A patient with a complete form of testicular feminization with normal gonadotropin and high testosterone levels is described here. These findings are in contrast to previous reports that have shown high circulating luteinizing hormone (LH) levels in adults affected by this syndrome. Testicular histology revealed tubular atrophy and hyperplastic Leydig cells which are in accordance with the high testosterone levels and the difficulty in visualization of the testes. Because of the increased risk of gonadal malignancy, a laparoscopic gonadectomy was performed.

Superoxide dismutase, catalase and glutathione peroxidase activities in maternal and cord blood erythrocytes.

The "antioxidant" enzymes superoxide dismutase, catalase and glutathione peroxidase were assayed in a biological model with low oxygen tension (red cells from cord blood of newborn infants). Catalase and glutathione peroxidase activities in red cells of newborns were significantly lower when compared with their mothers and with normal controls. In contrast, superoxide dismutase activity was unchanged. Thus, normal activities of superoxide dismutase seem to be necessary in order to protect red blood cells from superoxide radicals during foetal life, while even low activities of catalase and glutathione peroxidase are sufficient to protect red blood cells from hydrogen peroxide. No correlation was found between the "antioxidant" enzyme activities present in cord blood erythrocytes and the bilirubin concentrations during the first days of life.

[Sensitivity of the respiratory center to various hormonal stimulations]. / Sensibilità del centro respiratorio a stimoli ormonali di diversa natura.

PIP: It is well known that estrogen and progesterone can cause different male and female respiratory answers to stimulation with CO2, and that different concentrations of these hormones during pregnancy modify the sensibility of the respiratory center. The authors investigated this hypothesis by the rebreathing technique on 20 young females, and on 9 young males at basal conditions and after treatment with estroprogestational agents for the women, and with D-Norgestrel for the men. There were no great changes on the respiratory center sensibility, although a possible effect was not excluded, especially as it regarded estroprogestins. The article also discusses briefly the literature on the subject.^ieng

Effects of clomiphene citrate on cervical mucus: analysis of some influencing factors.

A multicenter study was performed to investigate the effects of a standard dose of clomiphene citrate (100 mg/day from day 5 of the cycle for five days) on the quality of cervical mucus. A total of 82 cervical mucus samples from 60 infertile patients (15 with secondary amenorrhea, 16 with delayed ovulation, 15 with anovulatory cycles, 14 with oligomenorrhea) was examined on the day of maximum follicular diameter. As a control we studied 54 cervical mucus samples from 47 patients with documented tubal sterility or infertile partners, who had spontaneous cycles with normal rhythm. Cervical mucus was unfavorable in 59% of the treatment cycles compared with 11% of the control cycles. Clomiphene citrate's antiestrogenic effect on cervical mucus was found to be related to the length of time between the last drug administration and the day of maximum follicular diameter (delta days), since cervical mucus was favorable in 64% of the cycles with a delta days more than 6 and in only 16% of the cycles with a delta days of 6 or less. Clomiphene citrate thus seems able to decrease cervical mucus quality. This could be one of the causes of the discrepancy between the rates of ovulation and pregnancy reported in patients treated with this drug.