Recurrent migratory angioedema as cutaneous manifestation in a familiar case of TRAPS: dramatic response to Anakinra.

BACKGROUND: Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a hereditary autoinflammatory syndrome characterized by recurrent episodes of fever and localized inflammation. Clinical presentation can be very variable in terms of duration of fever attacks, periodicity, and accompanying manifestations. One of the most characteristic symptoms is the occurrence of migrating skin rash with myalgia that is sustained by monocytic inflammation. OBSERVATIONS: We herein present the case of a family suffering from TRAPS who had been misdiagnosed for a long period of time and whose main symptom was migrating angioedema. Skin biopsy from one of the patients documented a monocytic panniculitis. All the living patients responded dramatically to anakinra treatment. CONCLUSIONS: The classic symptom of migratory angioedema with myalgia in TRAPS can be produced by monocytic panniculitis.This manifestation is so characteristic of TRAPS that its occurrence, even in the absence of other manifestations, should prompt genetic analysis. Our patient's condition responded promptly to anakinra treatment.

Shock induction by arterial hypoperfusion of the gut involves synergistic interactions between the peripheral enkephalin and nitric oxide systems.

To determine whether critical splanchnic artery hypoperfusion can provoke systemic shock and to identify the roles of the peripheral opioid and nitric oxide (NO) systems in this process, various degrees of superior mesenteric artery hypoperfusion (SMA-H) were produced in anesthetized adult rabbits (n=40), and hemodynamic and metabolic indices were measured. Metabolic acidosis and irreversible hypodynamic shock occurred with SMA-H at levels representing 25-20% of mean baseline SMA blood flow. In 112 other rabbits subjected to SMA-H at 20% (SMA-H20%), we studied plasma NO and enkephalin (ENK) levels, cardiovascular reactivity to selected physiological agonists, effects of ENKs on plasma NO levels, and effects of peripheral opioid receptor blockade and inducible NO synthase (iNOS) inhibition. SMA-H20% progressively increased systemic blood levels of NO and ENKs. Exogenous ENK administration accentuated SMA-H20%-induced increases in plasma NO levels, and their cardiovascular depressing effects were significantly greater when they were administered during SMA-H20% (vs. administration under baseline conditions). Selective blockade of cardiovascular delta-opioid receptors improved hemodynamics, prevented shock irreversibility and reduced plasma NO levels; similar effects were obtained by selective iNOS inhibition. These findings demonstrate that critical arterial hypoperfusion of the gut can induce hypodynamic systemic shock through ENK-induced hyperactivation of cardiovascular delta-opioid receptors, which leads to increased plasma levels of NO related in part to increased iNOS activity. Since pronounced splanchnic artery hypoperfusion occurs in all advanced systemic shock states, selective delta-opioid receptor antagonists and/or iNOS inhibitors may prove to be useful in improving shock hemodynamics and metabolic derangements and/or preventing progression toward irreversibility.

[Noise-related occupational risk aboard fishing vessels: considerations on prevention and the protection of exposed workers]. / Il rischio rumore a bordo dei pescherecci: alcune considerazioni sulla prevenzione e protezione degli esposti.

Recent legislation regarding the safety of workers aboard fishing vessels requires the appointment by ship owners of a Reference Physician in charge of health surveillance, preventive inspections and related tasks. As maritime workers, especially fishermen, have always been excluded from legal protection of occupational health, there are no exhaustive data on the incidence of their occupational disease. Several epidemiological studies of fishermen have evidenced a high prevalence and incidence of occupational conditions, among which noise-related hypoacousia. We report data of a phonometric survey conducted aboard six fishing vessels carrying a crew of less than six fishing in the mid-Adriatic. Measurements were performed during fishing and navigation aboard five vessels fitted with a fixed-pitch propeller and during fishing only aboard one vessel fitted with an controllable pitch propeller. Measurements were conducted: 1) in the engine rooms; 2) in the work area on deck; 3) at the winch; 4) in the wheelhouse; 5) in the mess-room and kitchen; 6) in the sleeping quarters. Results show that the equivalent sound pressure level in the engine rooms consistently exceeded 90 dBA on all vessels. The speed of the vessels fitted with the fixed-pitch propeller is 3-4 knots in the fishing phase and around 10 knots during navigation to and from the fishing grounds; noise emission is lower with the former regimen because of the smaller number of engine revolutions per minute. Our survey demonstrated considerably different noise levels in the various areas of vessels. One key element in workers' exposure, the tasks assigned and the environmental working conditions is of course the type of fishing in which the vessel is engaged. Further phonometric studies are required to assess the daily level of exposure per crew member, which represents the reference for the noise-related risk of each subject. Knowledge of the sound pressure levels in the work environment and the length of daily exposure of each crew member will allow to assess the level of occupational exposure and consequently enact the proper prevention and protection measures by the Reference Physician.

Impact of laparoscopy on the management of an unusual case of nonpalpable testis in an adult patient.

BACKGROUND: In the past decade, cryptorchidism has generally been treated in the first 36 months of life using an inguinal approach, in the case of palpable testis, or using laparoscopy, in the case of nonpalpable testis. Nevertheless, before this period some children were managed incorrectly. This case report shows how laparoscopy and the collaboration between pediatric surgeons and urologists may lead to optimal results. CASE REPORT: A 19-year-old male was referred to our unit following routine medical examination for enrollment in the military service with a diagnosis of right cryptorchidism. The clinical history showed the anamnesis of right cryptorchidism diagnosed at birth as a right nonpalpable testis, confirmed at 2 years of age with clinical examination and computer tomography. The patient came to our attention to again undergo a clinical examination, ultrasonography, computed tomography, and magnetic resonance imaging. We found a left, well-positioned testis of 20 ml in volume and an empty right scrotum; all the instrumental examinations were negative. The patient was thus scheduled for a diagnostic laparoscopy. A pediatric surgeon with extensive experience in this pathology performed the procedure. The 10-mm 30 degrees optics introduced through the umbilicus showed a closed right inguinal ring, with no evidence of either vas deferens or inner spermatic vessels at the level of the inguinal region or pelvis. Two more trocars were introduced and a testis of 15 ml in volume was found under the ascending colon 10 cm away from the cecum. An orchiectomy was performed via laparoscopy. CONCLUSION: This case clearly shows that some males with nonpalpable testis may have been treated incorrectly in the prelaparoscopic era and may now have an intraabdominal testis. In addition, our experience shows that intraabdominal testis may sometime be in an unusual location, and a laparoscopic surgeon with experience in this pathology is fundamental to finding the testis. In the case of adults with nonpalpable testis, when echography, computed tomography, and magnetic resonance imaging are not useful, the only effective diagnostic procedure is laparoscopy.

Colorectal cancer: obstruction is an independent negative prognostic factor after radical resection.

The rate of colorectal tumors causing large bowel obstruction is still high (about 16%). In our experience, 93 out of 985 patients (9.4%) affected by colorectal cancer required surgery because of large bowel obstruction. The comparative analysis between 64 (68.8%) obstructed and 738 (82.7%) non-obstructed radically resected patients showed that sex and age of patients, tumor site, tumor diameter, parietal infiltration, grading, lymphnode involvement, distant metastases, and staging were not significantly different. Post-operative mortality rates (1.6% vs. 0.5%) and morbidity rates (15.6% vs. 15.6%) were not significantly different as well. On the contrary, survival rate was different. Patients submitted to radical resections had a far poorer prognosis as compared with non-obstructed radically resected ones: the five-year survival was 41.2% and 78.9% respectively. Radically resected obstructed patients showed an higher and earlier ráte of local and distant recurrence with a disease-related death rate of 47.6% vs. 16.3% as compared with non-obstructed ones. The occlusive phenomenon by itself resulted to represent an independent unfavorable factor negatively affecting long term prognosis after radical resections.

Empathy and the unconscious.

The author examines the complex relationship between the concepts of empathy and unconscious, including exploration of topical, structural, and dynamic aspects; and the risk of oversimplification of empathy is discussed. Two clinical examples are then presented to demonstrate some of the complex factors that may contribute to or hinder the development and utilization of empathy by the analyst, many of which lie outside the analyst's conscious awareness.

[Neurinoma of the vagus nerve. Description of 2 cases and review of the literature]. / Neurinomi del nervo vago. Descrizione di due casi e revisione della letteratura.

The Authors report two rare cases of schwannoma of the cervical and thoracic portion of the vagus nerve. Schwannomas of the vagus nerve are particularly uncommon; patients suffering from these neurogenic tumors typically exhibit a paucity of symptoms and in the majority of cases they present with an asymptomatic mass noted on incidental chest X-ray. Chest pain and cough may occur with tumors arising next to and compressing the trachea or major bronchi. In the cases observed, schwannomas appeared like a mass whose size had increased during the last months without producing any clinical symptom. Clinical features of the mass, laboratory tests, ultrasound, CT scanning, magnetic resonance imaging were useful only to define its extension and relationships with the adjacent structures. Needle aspiration of these lesions is not indicated because of the paucity of the material obtained for the exact diagnosis. Surgery, with preservation of the vagus nerve when possible, is the treatment of choice also for a correct diagnosis of nature and to prevent further growth and compression on adjacent structures. When the individual fibers of the vagus nerve are displayed over the surface of the tumor within a discrete capsule nerve preservation is possible. On the contrary, when it is technically difficult to preserve the nerve trunk microsurgical procedures allow to reanastomose the cut ends. Injury of homolateral recurrent nerve often is the complication of a radical removal. Recurrence of benign lesions is not usual; malignant tumors carry a poor prognosis with patients rarely surviving beyond 1 year.

Empathy and 'empathism'.

The author begins by tracing the history of the concept of empathy in psychoanalysis, noting that, largely through the influence of Kohut, it began to feature prominently in the literature from the late 1950s on and has since tended, wrongly in his opinion, to be regarded as an all-purpose instrument to be deployed at will. What is often described in theoretical contributions as empathy should in the author's view more properly be called concordance. On the clinical level, the idea that the analyst must deliberately seek to empathise with the patient is stated to have gained currency, but the author argues that such an attempt to achieve empathy by force can lead only to 'empathism', which is a dogmatic, hyperconcordant attitude whereby the inexperienced analyst in particular thinks he can control the process better. Clinical material is presented to show how some patients set out to induce 'empathism' in the analyst for defensive reasons and how the analyst's concordance, until analysed, may lead to an impasse. The author stresses that genuine empathy is a state of complementary conscious-preconscious contact based on separateness and sharing; covering not only the patient's ego-syntonic subjectivity but also his defensive ego and split-off parts, its achievement requires prolonged hard work on the countertransference and a capacity for contact with the analyst's own primitive aspects. The paper ends with a consideration of the possible obstacles to empathic contact.

[Inflammatory carcinoma of the breast: the immunological findings]. / Carcinoma infiammatorio della mammella: rilievi immunologici.

The authors discuss a case of inflammatory breast cancer, treated by interdisciplinary therapy (surgical, radiating immuno and chemo therapy). The immune status was evaluated by the determination of the lymphocytes population, monocytes and granulocytes. This evaluation was made before and after immunotherapy (i.e. subcutaneous administration of IL 2). IL 2 receptors were evaluated too. A daily dose of recombinant IL 2 (18 millions U.I.) was administered for six consecutive days. The follow-up studies showed a clear increase of total T lymphocytes, a normalization of T helper/inducers ratio, an improvement of T cytotoxic/suppressors ratio, an increased level of IL 2 receptors. The induction of mRNA for CM-CMF, G-CFS, IL 3, IL 5, IL 6, was also demonstrated, as well as a plasmatic increase of all the growth factors. A rise in neutrophils and eosinophils was documented. These results allowed the continuation of therapeutic approach with the complex radiation-chemotherapy program.

Transference: erotised, erotic, loving, affectionate.

Within a set of phenomena traditionally problematic for psychoanalysis, four types of erotic transference are outlined with a description of their dynamic genesis and related case histories (transference and countertransference developments, fundamental treatment procedures): erotised, erotic, loving, and affectionate transference. The first type is based mostly on psychotic modalities, the second on neurotic modalities. Both the loving and the affectionate transference, on the other hand, turn out to be clinical forms corresponding to a normal substantially same development, different from each other by virtue of a diverse level of maturation in the Oedipus complex: they often provoke--in analysts--a defensive stiffening which is harmful and--from the theoretical point of view--not above reproach. The work is particularly focused on the analytic evolution of a female patient who develops an amorous transference in which the defensive aspects prove to be less important than the potentially evolutionary ones. The treatment has been based--in particular--on the analytic utilisation of this transference. The last part of the contribution is devoted to the analysts' 'guarantee factors', which permit them to work in psychoanalysis with the 'highly explosive forces' of these transference configurations, maintaining a useful, rigorous, sensitive and sufficiently creative framework.

[Regional hemodynamic evaluation of heterotopic liver transplantation by the determination of the hepatic artery resistance index]. / Valutazione emodinamica distrettuale nel trapianto eterotopico de fegato attraverso la determinazione dell'indice di resistenza dell'arteria epatica.

Hepatic artery resistance index (I.R.) was evaluated in six pigs through Doppler scan after progressive gauge reduction of the portal vein up to complete clamping. The I.R. values were (mean +/- S.D.): 0.48 +/- 0.01 in basal conditions; 0.37 +/- 0.03, and 0.33 +/- 0.01 with a reduction of 1/3 and 2/3 respectively; 0.27 +/- 0.02 after complete portal clamping. These data confirm the close relationship existing in the hepatic circulation between changes of the arterious resistence and those of the portal flow. The same animals, subsequently, underwent heterotopic liver transplantation in the right paracolic gutter. Immediately after surgery the I.R. values of the transplanted hepatic artery were recorded without clamping (basal portal flow 0.52 +/- 0.02) and after clamping of the transplanted portal vein (0.33 +/- 0.03), while maintaining clamped the recipient portal vein at the hilus. These values were comparable to those obtained in the recipient hepatic artery. These data suggest that, in spite of parenchymal damages secondary to surgical procedure and of the haemodynamically unfavorable site in which the organ is transplanted, heterotopic liver transplantation does not produce significant changes in hepatic arterious resistance.