Primary pulmonary hypertension: an Italian multicenter study. A retrospective epidemiological survey in the period 1975-1985

An epidemiological retrospective survey on primary pulmonary hypertension was undertaken in Italy in 1985-6. The aim of the study was to obtain clinical and laboratory data on patients observed in the major cardiological Institutions, both Universities and Hospital Clinical Centers, in the period 1975-85. Forms were sent to 67 centers and twenty-three of them communicated their data on 124 cases of primary pulmonary hypertension. The diagnosis was considered certain or probable in 91 patients by the referring center. The mean age of patients was 38.5 years, with an overall female-to-male ratio of 2:1. Data were subjected to statistical analysis which confirmed the poor prognosis of the disease particularly for patients with high pulmonary resistances, low cardiac output and in class 3-4 of the NYHA. Half of the 124 cases were treated with calcium antagonists after 1980, but the results of therapy in modifying the clinical course of the disease could not be assessed by the study. Like all retrospective studies even the Italian Multicenter Study is biased but it can be considered as an useful preliminary investigation which can form the basis for a prospective registry of primary pulmonary hypertension cases.     

Spontaneous circadian fluctuations of prostate specific antigen and prostatic acid phosphatase serum activities in patients with prostatic cancer

Summary Spontaneous circadian variations of prostate specific antigen (PSA) and prostatic acid phosphatase (PAP), determined simultaneously by radioimmunoassay (RIA), were investigated by multiple sampling, over a 24-hour period, in 32 patients with prostatic cancer. In 29/32 patients (91%), the coefficient of variation of 24-hour values, for either marker, was greater than that of the RIA method at the same range of values; stage D patients showed the greatest spontaneous variability. Fluctuations around the mean of 24-hour values ranged from-65% to +85% for PAP, from-72% to +190% for PSA, occurring random and independently for each marker. Variability was about 20% greater for PSA than for PAP. The existence of spontaneous fluctuations should be considered in multiple marker evaluation of prostatic cancer patients.Preliminary results of this study have been presented at the International Symposium on Hormonal Therapy of Prostatic Diseases —Basic and Clinical Aspects, April 6–8, 1987, Milan, Italy     

Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions. Part II: ventricular arrhythmias, channelopathies and implantable defibrillators.

This consensus paper on behalf of the Study Group on Sports Cardiology of the European Society of Cardiology follows a previous one on guidelines for sports participation in competitive and recreational athletes with supraventricular arrhythmias and pacemakers. The question of imminent life-threatening arrhythmias is especially relevant when some form of ventricular rhythm disorder is documented, or when the patient is diagnosed to have inherited a pro-arrhythmogenic disorder. Frequent ventricular premature beats or nonsustained ventricular tachycardia may be a hallmark of underlying pathology and increased risk. Their finding should prompt a thorough cardiac evaluation, including both imaging modalities and electrophysiological techniques. This should allow distinguishing idiopathic rhythm disorders from underlying disease that carries a more ominous prognosis. Recommendations on sports participation in inherited arrhythmogenic conditions and asymptomatic gene carriers are also discussed: congenital and acquired long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, arrhythmogenic right ventricular cardiomyopathy and other familial electrical disease of unknown origin. If an implantable cardioverter defibrillator is indicated, it is no substitute for the guidelines relating to the underlying pathology. Moreover, some particular recommendations for patients/athletes with an implantable cardioverter defibrillator are to be observed.     

Surgical treatment of Aspergillus mycetomas of the maxillary sinus: review of the literature.

The present study reviews the literature concerning the surgical treatment of Aspergillus mycetoma (AM) in the last 20 years to identify a gold standard surgical technique. Aspergillus mycetoma of the maxillary sinus, or mycetoma (fungus ball), is a noninvasive or extramucosal mycotic infection. Surgical removal of the sinus fungal masses to ensure drainage and aeration is performed using the traditional Caldwell-Luc (CL) procedure or endoscopic sinus surgery (ESS). Results of this review suggest that the gold standard surgical technique for AM is ESS with middle meatal antrostomy. General or local antifungal drugs are not indicated. Combined approach with an intraoral surgical access from the anterolateral wall of the maxillary sinus has to be reserved for selected cases in which ESS doesn't permit complete extraction of all fungal concretions or foreign bodies. The CL procedure should be avoided, because it has detrimental consequences for sinus physiology.     

Lateral unicompartimental knee arthroplasty: indications,technique and short-medium term results

Lateral unicompartmental knee arthroplasty (UKA) is a valid alternative treatment in the event of arthritis confined to the lateral compartment. This paper examines its indications, technique and short to medium-term results. A total of 159 Miller–Galante cemented UKA prostheses (Zimmer, Warsaw, Indiana) were implanted consecutively (131 medial and 28 lateral) by the same surgeon. This study investigates 28 lateral UKAs in 27 patients. Twenty-five implants in 24 patients (including a subject operated bilaterally) were followed up for 12–60 months. Three patients were discarded on account of to short a follow-up period. The Hospital for Special Surgery (HSS) knee score was used to compare the pre- and post-operative results of the lateral UKA patients. The HSS score improved from a pre-op mean of 59.92 (range 48–68) to 88.04 (range 71–95) at the last follow-up. There was a positive increase in the pain, function and ROM components of the score. The lateral UKA prosthesis can be regarded as a sound alternative to total knee replacement. Correct patient selection on the basis of optimum surgical indications, however, is essential. No benefits of funds were received in support of the study.     

Altered dimerization of metabotropic glutamate receptor 3 in schizophrenia.

BACKGROUND: Metabotropic glutamate receptors (mGlus) may be involved in the pathophysiology of schizophrenia. Group II mGlus (mGlu2 and mGlu3) have attracted considerable interest since the development of potent specific agonists that exhibit atypical antipsychotic-like activity and reports of a genetic association between the mGlu3 gene and schizophrenia. METHODS: In this postmortem study, mGlu3 protein levels in Brodmann area 10 of prefrontal cortex from schizophrenic (n = 20) and control (n = 35) subjects were analyzed by western immunoblotting using a novel specific mGlu3 antibody and an antibody for the vesicular glutamate transporter 1 (VGluT1). RESULTS: We report a significant decrease in the dimeric/oligomeric forms of mGlu3 in schizophrenic patients compared with control subjects, whereas total mGlu3 and VGluT1 levels were not altered significantly. CONCLUSIONS: This is the first experimental evidence that mGlu3 receptor levels are altered in schizophrenia and supports the hypothesis that neurotransmission involving this particular excitatory amino acid receptor is impaired in schizophrenia.     

Testicular regression syndrome. A case report

Testicular regression syndrome occurred in a 20-year-old, white, phenotypic female with a 46,XY karyotype. The basal levels of serum gonadotropins were elevated, while the testosterone was in the normal range. Estrogens were undetectable. At laparotomy no gonadal rudiments or müllerian or wolffian derivatives were found. The logical diagnosis was late embryonic testicular regression with a specific testicular insult 62-63 days after fertilization.     

Assessment of enamel hypoplasia in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)

The features of enamel hypoplasia in a small group of patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) are described. Using a recently developed method, the authors evaluated quantitatively the amount of defect in each tooth by measuring the width of the hypoplastic lesions and dividing the value by the crown height. They then assessed the degree of damage in each tooth type (from central incisors to second premolars) and patient. Canines were the most severely affected among maxillary and mandibular teeth, but all tooth types were involved. Analysing both the differences between patients and their age at the beginning of the defect, the authors observe that hypoparathyroidism is not responsible for the onset of enamel hypoplasia in APECED, although it may contribute to the damage.