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The immotile-cilia syndrome (ICS) is a congenital disorder characterized by dysmotility or even complete immotility of the cilia in the ciliated epithelia. The most frequent consequences include recurrent airway infections from early childhood. Neonatal asphyxia often occurs. Males are usually sterile, whereas females may be fertile or infertile. The disease is inherited as an autosomal recessive trait, but previous attempts to localize the ICS susceptibility gene have so far been unsuccessful. Here, we present the case of two sib pairs affected by ICS from two unrelated families. The electron microscopic investigation of nasal biopsies showed structural anomalies of the cilia, characterized by single microtubules or doublets, arranged randomly in the axoneme. Histocompatibility antigen (HLA)-genotyping of all family members revealed: 1) a significant association of ICS with the HLA-DR7; DQW2 haplotype, which is shared by all the affected sibs (P = 0.0099; RR = 25.94); 2) a possible linkage of the ICS susceptibility gene with HLA, both the affected sibs being HLA-identical, the healthy brother in family B being HLA-different (sib-pair analysis: P less than 0.001).

The epidemiological, clinical and therapeutical findings are described in a case of recurrent pulmonary histoplasmosis due to Histoplasma capsulatum. The patient, a bulldozer-operator, worked in Africa for a long period in extremely dusty conditions without any protection. Three different episodes of H. capsulatum pneumonia recurred during eighteen months after his return from Africa. A full dose treatment by Amphotericin B failed to eliminate disease recurrence on three occasions. The high concentration of airborne H. capsulatum spores inhaled could have been the main cause of the difficulty obtaining a rapid sterilization of the microorganism by Amphotericin B and disease recurrence. The late start of the treatment or the unexplained ability of some persons to develop repeated infections even with normal immunological parameters could be another explanation for the reported phenomenon.

We addressed the problem of the circadian changes in neural control of heart period in ambulant hypertensive subjects. A running spectral analysis of R-R variability from Holter tapes provided markers of sympathetic, i.e. low-frequency component (LF) almost equal to 0.10 Hz, and vagal, i.e. high-frequency component (HF) almost equal to 0.25 Hz, controlling activities for the 24-h period of the recording. Significant circadian differences were observed in LF between the two groups of subjects: during night-time rest (0300-0400 h), LF was greater in hypertensives than in normotensives (56 +/- 2 and 48 +/- 2 nu, respectively; P less than 0.05). Furthermore, the difference between daytime and night-time LF values was progressively reduced with increasing severity of the hypertensive state, as assessed by resting arterial pressure levels. Spectral analysis of R-R variability suggests that essential hypertension may be characterized by a reduced day-night oscillation in sympathetic activity than can be quantified non-invasively using this approach.

We report the follow-up and treatment of HCMV infections in three patients with AIDS. The patients, affected by HCMV retinitis, have been followed 24, 12 and 6 months respectively. The antiviral treatment was based on the DHPG administration which was substituted in one case of resistance to DHPG with Foscarnet. In the follow-up period, virological tests have been performed to detect the presence of the HCMV antigenemia/viremia. The results show that, to avoid the progression of the retinitis, the antiviral treatment must not be stopped or discontinued. DHPG and Foscarnet were able to limit the infection to the eye and were well tolerated. In the HCMV-infected patients, the continuous monitoring of the antigenemia/viremia is of main importance to follow the clinical and therapeutical course of the disease.

This paper describes a case of "Red man's syndrome" in a patient with staphylococcal sepsis. The patient was initially treated with intravenous Vancomycin and afterwards with Teicoplanin. The adverse reaction appeared immediately after the start of pharmacological treatment.

The present study was set up to evaluate the efficacy of hyperimmune anti-CMV immunoglobulins (IVIG) in the prophylaxis of CMV infection in five patients suffering from dilatative myocardiopathy and submitted to hearth transplant. Different commercial IVIG were tested for anti-CMV neutralizing antibodies before administrated them to patients; the lots immunochemically prepared by PEG resulted to have the highest anti-CMV titer. IVIG treatment, when administered at high doses and at closes intervals of time, proved able to prevent and control CMV infections; when treatment was stopped, a primary infection occurred, easily controlled by short courses of DHPG. Even if our survey was limited to only five cases, it should be emphasized that after the suspension of the therapy, clinical symptoms were restricted to simple mononucleosis syndrome without organ disease. Furthermore it should be noted that during IVIG therapy no immediate or delayed adverse effects were observed.

The present study describes a case of AIDS with asymptomatic intracranial focal lesions. The DDD-enhancement CT technique detected the lesions as inflammatory or neoplastic. A subsequent MR demonstrated the vascular nature of the intracranial findings. MR in the neuropathology of AIDS can be of primary importance in the detection of the intracranial focal lesions, thus avoiding a cerebral stereotaxic needle biopsy.

In this study, we tested the hypothesis that the neural control of circulation in humans undergoes continuous but in part predictable changes throughout the day and night. Dynamic 24-hour recordings were obtained in two groups of ambulant subjects. In 18 hospitalized patients free to move, direct high-fidelity arterial pressures and electrocardiograms were recorded, and in an additional 28 nonhospitalized subjects, only electrocardiograms were obtained. Spectral analysis of systolic arterial pressure and of RR interval variabilities provided quantitative markers of sympathetic and vagal control of the sinus node and of sympathetic modulation of vasomotor tone. With this approach, the low-frequency (approximately 0.1 Hz) component of RR interval and systolic arterial pressure variabilities is considered a marker primarily of sympathetic activity, whereas the high-frequency (approximately 0.25 Hz) component of RR interval variability, related to respiration, seems to be a marker primarily of vagal activity. We observed a pronounced and consistent reduction in the markers of sympathetic activity and an increase in those of vagal activity during the night. In the invasive studies, while the subjects were still lying in bed after waking up, the markers of sympathetic activity rose rapidly and concomitantly with a simultaneous vagal withdrawal. Noninvasive studies confirmed the early morning rise of the markers of sympathetic activity and the circadian pattern of sympathovagal balance. These data indicate that the ominously increased rate of cardiovascular events in the morning hours may reflect the sudden rise of sympathetic activity and the reduction of vagal tone.

Morphological, cytochemical and ultrastructural studies of mast cells were carried out in a patient affected with systemic mastocytosis. Neoplastic mast cells showed morphological features between classic tissue mast cells and circulating basophils. They showed strong granule metachromasia after toluidine blue, faint positivity to Hotchkiss reaction, strong positivity to chloroacetate esterase, while they were negative to alkaline phosphatase. Ultrastructural observations showed heterogeneity of granules, most of which had homogenous fine dotted contents. The origin and linkage between mast cells and circulating basophils are discussed.

A case of recurrent pulmonary aspergillosis in a young girl with common variable hypogammaglobulinaemia and the treatment adopted is described. We employed the plasma-exchange, at the beginning of immunosubstitutive treatment, to provide a high serum concentration of all classes of immunoglobulins and to better control the patient's state of shock. Intravenous immunoglobulins and Amphotericin B were also given in combination with plasma-exchange, having a synergic therapeutic effect. The good condition of the patient, despite the recurrent episodes of fungal pneumonia, suggests that such an approach can lead to long-term, high quality survival in humoral immunodeficient patients.

The relationship between IgE and IgG antibodies against the metabolic exoantigen of Toxocara canis (TEX antigen) in sera of selected patients with the ocular and visceral form of the disease have been investigated. The specificity and sensitivity of the TEX antigen employed in immunoenzymatic and radioimmunologic assays have been verified. Six hundred and forty-one sera from healthy donors were tested as controls. Our data emphasize the importance of IgE antibodies to ocular larva migrans syndrome and suggest that they play a significant role as a marker of infection. In the visceral form, IgG antibodies seem to mask the presence of IgE by a competitive mechanism toward the same epitopes of the parasite. In fact, IgE antibody titers increase after depletion of IgG from serum.

Three cases of aspergillosis observed in Pavia Infectious Disease Clinic in 1983-85 are described. The cases differed both in the site of the infection (lungs, bones and liver) and in the patients' basic immunological situation. The importance of mycological investigations during diagnosis is emphasised, though they should of course be flanked by instrumental examinations and blood chemical assays. The efficacy of specific treatment with amphotericin B combined with surgery and plasma exchange is also emphasised.

Toxocariasis, Hydatidosis and Fascioliasis are common autochthonous parasitic infections in Italy. RAST and ELISA have been evaluated for serological diagnosis. Eight sera from Toxocariasis, fifty from Hydatidosis and two from Fascioliasis have been examined. Excretory/secretory antigen has been employed for the serology of Toxocariasis, hydatic fluid for Hydatidosis and embryonated-eggs antigen for Fascioliasis. The RAST resulted positive in 4/5 cases of the visceral form of Toxocariasis and 3/3 in the ocular form, in 2/2 cases of Fascioliasis and 27/50 of Hydatidosis. The ELISA resulted positive respectively in 5/5, 3/3, 2/2, and 19/50 cases. The sensitivity and specificity of the tests employed have been proved.

We report three cases of cryptococcal meningitis in patients with AIDS observed in our Institution. In addition, we discuss antifungal treatment during and after the acute phase of the disease and the use of fluconazole as a prophylactic treatment of disease relapse.

This paper describes a case of cavernous sinus septic thrombosis with cerebellar complication. The CNS was involved after a primitive piodermitis of the face. The diagnostic tools employed in the management of the case and the therapeutic procedures in the acute phase and in the convalescence are discussed. The patient completely recovered after chemotherapeutic treatment.

We measured the chemiluminescence (CL) of human leukocytes (PMNs) exposed to different concentrations of Clindamycin (1; 5; 1 mcg/ml) using a standardized luminolamplified reagent-instrument methodology (Auto Picolite 6500 Luminometer and ZAP/Picolite Kits, Packard Instruments Co. Drowners Grove, IL. USA). Cells obtained from healthy donors were also tested for chemotaxis with Boyden chambers, for phagocytosis and for killing activity against Staphylococcus aureus by the agar-medium culture plate technique. Clindamycin does not induce significant variations of the CL response in whole blood, or changes in phagocytosis and killing activity. On the contrary, concentrations of drug corresponding to therapeutically obtainable levels significantly increase light emission by isolated cells. A concentration effect was seen on leukotaxis, that was increased, but not significantly, at 1 and 5 mcg/ml and decreased (P less than 0.01) at 10 mcg/ml. CL assay is a simple, sensitive, reproducible technique to assess the PMNs functions during antibiotic therapy.

This paper describes the epidemiological, clinical and laboratory features of 76 cases of Mediterranean spotted fever occurring during the years 1977-1986. The patients observed all lived in the Lombardy region of Italy, which is not located on the Mediterranean Basin. Our data show the highest prevalence of the disease, as reported by other authors, in southern regions of Italy, in the same years: 1979-1983. No differences were noted between age groups or sexes, and the late spring-summer seasonality was confirmed. The clinical signs and symptoms in our cases were those common to the disease. The microimmunofluorescence method was found to be more sensitive and specific than the Weil-Felix test for serological diagnosis. Finally, we showed that doxycycline and oxytetracycline both induce rapid remission of fever.

In 10 ambulant subjects we studied the circadian changes in sympathetic vasomotor control as assessed by the spectral power of the 0.1-Hz low-frequency component of systolic arterial pressure variability measured with a Millar phi 3F tip transducer. The low-frequency component was higher during the daytime, while the subjects were performing light physical activity, and lower during the night, thus paralleling the circadian systolic blood pressure pattern. However, the morning low-frequency rise preceded the blood pressure increase by about 3 h, suggesting that vasometer control and blood pressure control are at least partly related to different mechanisms.

The Authors have evaluated the use of anti-human immunoglobulins conjugated with peroxidase, alkaline-phosphatase, glucose-oxidase as a diagnostic tool for assaying autoantibodies. A comparison study was done using the classical indirect immunofluorescence technique. Immunoenzymatic techniques, of minor cost compared to immunofluorescence, have revealed a better appreciation of global and particular structure of the tissue in examination. From the three enzymes examined peroxidase revealed itself as first choice, because, other than having on overlapping sensitivity to immunofluorescence, it can allow a further ultrastructural study.