Coexisting Behçet's syndrome and spondyloarthritis.
A patient suffering from Behçet's syndrome and undifferentiated spondyloarthritis developed dactylitis and psoriasis over the next three years.
Biomedical subjects
Publications and source records attributed to E Scarano.
A patient suffering from Behçet's syndrome and undifferentiated spondyloarthritis developed dactylitis and psoriasis over the next three years.
We evaluated growth hormone (GH) secretion in 81 patients with Turner's syndrome (TS) (mean age 10.7+/-3.6 y) with respect to karyotype, auxological characteristics and growth response to GH treatment (1 IU/kg/wk). None of the patients had spontaneous puberty or had started replacement therapy with estrogens. Thirty-nine patients (48%) had monosomia 45X, 29 (36%) structural abnormalities of the X chromosome and 13 (16%) X mosaicism. Before the start of GH therapy, each patient underwent an evaluation of mean nocturnal GH concentration (MGHC) and 75 patients also underwent 2 pharmacological tests. MGHC of the TS patients did not differ from that of 29 prepubertal GH-deficient girls (GH peaks < 8 microg/l after pharmacological tests) and both groups were lower (p < 0.0001 and p < 0.0005, respectively) than MGHCs of 27 short normal girls (GH peak > 8 microg/l). MGHC of the patients with TS was negatively correlated (p < 0.001) with bodyweight excess (BWE) at multiple regression analysis. MGHC of the TS patients with BWE < 20% was significantly higher (p < 0.02) than that of the TS patients with BWE > 20%, but again did not differ from that of the GH-deficient patients and was lower (p < 0.001) than that of the short normal girls. MGHC did not significantly differ between the 3 groups subdivided according to karyotype. Forty-four percent of the TS patients showed GH responses to pharmacological tests < 8 microg/l. Height velocity SDS at first and second year of therapy was not influenced by MGHC levels, chronological or bone age, target height or BWE. In conclusion, spontaneous secretion in our patients with TS was lower than that of the short normal prepubertal girls and did not differ from that of GH-deficient subjects, even if we excluded overweight patients. The level of GH secretion was unable to predict GH response to treatment.
The aim of this paper was to verify if the growth of the nasomaxillary complex can be influenced by a purely functional alteration such as nasal obstruction, which was induced experimentally in a genetically controlled animal model. Sixty albino rats were employed. Twenty of them had the right nostril occluded by a synthetic resin; another twenty had both nostrils occluded; the other 20 were taken as control group. When the growth was completed, the rats were sacrificed and cephalometric analysis was carried out. Both treated groups showed a statistically significant reduction in overall weight and height, in the vertical development of the nasomaxillary complex and in the skullbase longitudinal axis. After discussing the literature on the subject, the authors conclude that normal craniofacial growth in the rat must somehow depend on physiological nasal breathing, which should therefore be considered of crucial importance.
Some authors have shown that lysine acetylsalicylate (LAS) may help prevent nasal polyp relapses. As some anti-inflammatory drugs have been found to regulate cell growth, we investigated the antiproliferative effect of LAS on fibroblasts derived from nasal polyps. Moreover, we studied the effect of LAS on the growth of fibroblasts derived from normal skin to determine whether the response was similar to that obtained in the above-mentioned cells. Fibroblasts were obtained from tissue samples of nasal polyps from two aspirin-tolerant and two aspirin-intolerant patients, and from the normal skin of a healthy donor. The cells were treated with LAS (20-2000 microg/ml of culture medium). Cell growth and viability were evaluated after 3 and 6 days of culture. LAS had a growth-inhibitory effect on cells independently of their derivation. A reduction in cell growth was seen at the concentrations of LAS tested, which correspond to those used in the local treatment of nasal polyposis.
BACKGROUND: Twenty-six patients underwent application of a new posterior plating system (CIDEF SYSTEM) for the alignment and the stabilization of C3-C7 cervical unstable fractures from January 1992 to December 1993. METHODS: The osteosynthesis apparatus employs posterior articular screw fixation and plate application. Indications for surgery were the presence of neurological damage and cervical column instability. Operative techniques included decompressive and exploratory laminectomy in 20 cases and an articular resection in six cases of posterior superior articular fractures. A one-stage operation with an anterior approach, designed to remove anterior compression from herniated discs, or bone fragments, was also performed in four cases. Three cervical motion segments were immobilized in 15 of the treated cases; four in nine; and five in two. All patients were studied pre- and postoperatively with plain radiographs and computed tomography. Pre- and postoperative examinations demonstrated no alteration in neurological status. RESULTS: At a mean follow-up period of 47 months, no evidence was found of infection, rejection of the materials used, or mechanical failure of the plates and screws; all patients with incomplete lesion showed improvement in neurological function. Four patients tetraplegic at admission expired from systemic complications (one from pulmonary embolism; one from Disseminated Intravascular Coagulopathy; one for sepsis; and one from myocardial infarction). All patients were immobilized for at least 4 months with a Philadelphia collar. No injury to the neural or vascular structures contiguous to the cervical spine has occurred. CONCLUSIONS: The authors' experience suggests that the new system offers ease and versatility of application, restoration of vertebral alignment, enhanced spinal stability and is useful in the treatment of urgent and rather complex traumatic lesions of the lower cervical spine.
Real-time ultrasonography was performed in 142 patients with Turner syndrome, aged 0.57 to 21 years, with different karyotypes (45,X [4896], X mosaicism [17%], and X structural abnormalities [35%]). Ovarian and uterine volumes were calculated and the data collected in a mixed longitudinal and cross-sectional mode. Thirty-eight patients were followed longitudinally during pubertal age (10 to 18 years bone age) for ovarian data. Patients with Turner syndrome were divided into two groups according to the presence or absence of detectable ovaries. Patients with Turner syndrome with detectable ovaries showed the first increase in ovarian volume at about 9 years of bone age; this increase was continuous and more evident only after 14 years of age and appeared later than in control subjects. When followed longitudinally during puberty, the ovaries showed a hormonal function in some cases. Girls with X mosaicism had the highest percentage of bilateral detectable ovaries and the greatest total ovarian volume; about 50% of them had spontaneous breast appearance and 38.5% had spontaneous menarche. They showed also the lowest gonadotropin levels, when bilateral ovaries were present during puberty. On the contrary, patients with the 45,X karyotype had the lowest percentage of detectable ovaries, ovarian volume, and spontaneous breast appearance. In our patients with Turner syndrome, uterine measures increased significantly with age and this was more evident in subjects with detectable ovaries after 13 years of bone age. Compared with control subjects, they showed significantly lower uterine measures, and patients with X mosaicism had greater and more progressive increments. In conclusion, pelvic ultrasonography in Turner syndrome is particularly useful in detecting ovaries and their possible increase in volume. These data, linked with karyotype pattern and gonadotropin levels, have prognostic value in predicting the future sexual development of these patients.
Wegener's granulomatosis is a systemic vasculitis that may present with a variety of findings and be difficult to diagnose. We report a case of a patient who presented with serous otitis media and subsequently developed a suspected primary lung tumour. Thoracotomy and pulmonary mass excision were required to establish the diagnosis. Otological manifestations of Wegener's granulomatosis, differential diagnosis, pathological findings and c-ANCA test role are discussed.
PURPOSE: Magnetic resonance angiography (MRA) can be used to measure flow velocity in the portal vein noninvasively. Our study was aimed at measuring mean flow velocity in the portal vein and section area and overall portal flow with bolus tracking MRA versus color Doppler US. MATERIAL AND METHODS: Twenty healthy volunteers were submitted to presaturation bolus tracking MRA and color Doppler US before and after a 1500 Kcal meal. The images were acquired during breath-holding and analyzed prospectively for the following parameters: mean flow velocity, portal vein caliber and flow, before and after a meal. MRA measurements were made on both baseline images and MIP reconstructions. RESULTS: Before the meal, mean portal flow velocity was 17.07 +/- 3.01 cm/s with MRA versus 17.46 +/- 3.12 cm/s with color Doppler US (r = .85). After the meal, mean velocity was 24.52 +/- 3.8 cm/s with MRA and 24.8 +/- 4.0 cm/s with color Doppler US (r = .85). After the meal, portal velocity increased by 44% with MRA and by 42% with color Doppler US. Before the meal, the portal vein section area was 1.27 +/- .32 cm2 with MRA and 1.17 +/- .29 cm2 with color Doppler US (r = .86), versus 1.52 +/- .30 cm2 with MRA and 1.44 +/- .27 cm2 with color Doppler US (r = .85) after the meal. Portal vein flow was 1248.4 +/- 302.46 mL/min with MRA and 1202.85 +/- 316.12 mL/min with color Doppler US before the meal, versus 2252.45 +/- 523.90 mL/min with MRA and 2202 +/- 576.74 mL/min with color Doppler US (r = .91) after the meal. Portal vein flow increased by 78% with MRA versus 83% with color Doppler US after the meal. DISCUSSION AND CONCLUSIONS: Bolus tracking MRA is an accurate method to quantitate mean velocity, section area and blood flow in the portal vein.
METHODS: Twenty-four cases of unstable thoraco-lumbar fractures with significant canalar stenosis were operated in the Emergency Neurosurgical Unit of the Cardarelli Hospital from November 1989 to October 1991. The surgical technique was the combined transpedicular (TPA) and transarticular (TAA) postero-lateral approach, posterior osteosynthesis and arthrodesis. The average follow-up was 20 months. RESULTS: No evidence of implant breakage or pseudoarthrosis was detected. CONCLUSIONS: The authors affirm recalibration of the posterior wall by direct impaction, epidural hemostasis, removal of extruded disc fragments and dural repair are the advantages offered by this technique without compromise the contsrist stability. This approach represents an alternative to single posterior and combined anterior-posterior approaches in the management of the thoraco-lumbar fractures with severe canal stenosis.
Although human papillomaviruses (HPVs) have been found in many, but not all, tumours of the oral cavity, nose, pharynx and larynx, the true role of HPV in malignant tumours of the head and neck is still unclear. The presence of HPV DNA was investigated in 45 fresh squamous cell carcinoma (SCC) specimens and in 29 normal mucosa specimens collected from 45 primary laryngeal SCC patients. HPV DNA was detected using the polymerase chain reaction (PCR) with consensus primers that detect HPV types 6, 11, 16 and 18.9 of the 45 patients (20%) were HPV positive; the presence of HPV was also detected in the corresponding normal laryngeal mucosa of four of the 29 specimens (14%). No statistically significant differences were found between the presence of HPV DNA in normal specimens and in neoplastic mucosa specimens. No correlation was found between HPV DNA positive tumours and size, T classification, lymph node involvement and histological grading. This study adds further evidence suggesting a possible role of HPV DNA infection in laryngeal carcinogenesis.
This study was aimed at investigating the current knowledge on the similarities between pulmonary and portal circulation to try to improve the diagnostic capabilities of functional radiology in these two districts. These two organs are similar both from an anatomical and a functional viewpoints, sharing the same microarchitecture and a double vascular system with similar hemodynamic characteristics. In the past, the parameters to evaluate pulmonary flow and pressure consisted in the analysis of the distribution, diameter and number of vessels with conventional radiology, but today, HRCT permits the regional assessment of perfusion and air volume, using density values. Dynamic density changes (expiratory and prone scanning), together with the morphological features of peripheral bronchial and vascular structures, play a fundamental diagnostic role in differentiating small airway conditions from normal and hemodynamic changes. When HRCT shows a "mosaic" pattern--i.e., regions with different density values--reduced perfusion can be distinguished, because in this case hypodense areas are vascularized by fewer, and smaller, vessels. Expiratory scanning can exclude abnormal ventilation. Hyperperfusion is characterized by higher density areas vascularized by more, and bigger, vessels. Doppler US and MRA show, once again, their limitations in calculating the absolute values of flow velocity and flow volume in the splanchnic district; in clinical studies, only the semiquantitative data yielded by Doppler US are considered reliable. Therefore, also in this district, relative data must be preferred to absolute ones; for instance, it is interesting to analyze the hemodynamic changes occurring in patients under different physiologic or experimental conditions. We believe that, in the near future, technological progress and growing operators' skills will make functional radiology a major tool helping the clinician approach and treat these patients correctly.
Between 1990 and 1992 six cases of subdural empyema were surgically treated at the Neurosurgical Division of Emergency Department of Cardarelli Hospital in Naples. Three cases were associated with paranasal sinusitis and three cases with otitis media. Headache and fever were the presenting symptoms in all cases; in only two cases they were associated with seizures and altered mental status. CT scans showed convexity low density collections in five cases and multilocalized pus collection in one; concurrent paranasal or mastoid infections were visualized as well. The organisms responsible for the subdural empyema were Peptococcus in four cases, Streptococcus and anaerobius in the other two cases. In five cases surgical treatment consists in pus drainage by selective burr hole and placement of a subdural small silicon tube for local antibiotic therapy. In one case with a loculated diffuse empyema, craniotomy was performed in order to provide a better access to all the localizations. In all cases drainage of the wound and intravenous antibiotic therapy were used. Paranasal sinus drainage or mastoidectomy performed by the otolaryngologist when a localized collection of pus was present, grave a quicker regression of symptoms. A full recover of the original neurological status was achieved in all cases; a 20 months mean followup confirms the results.
T2-weighted spin echo MR images are widely used in the detection and characterization of focal liver lesions. The main pitfall of this technique is its relatively long acquisition time. Fast spin echo sequences can provide the same contrast as conventional T2-weighted SE images in a shorter scanning time. Our study prospectively compared the effectiveness of T2-weighted conventional spin echo (CSE) versus fast spin echo (FSE) sequences in MRI of focal liver lesions. We examined 24 patients with this condition. All lesions were submitted to percutaneous biopsy and to the diagnostic gold standard technique, which was intraoperative US (13 patients), Lipiodol CT (10 patients) or 6-month follow-up (1 patient). MR images were analyzed from both a quantitative (signal-to-noise and contrast-to-noise ratios) and a qualitative viewpoints (overall image quality, lesion detection rate, number of lesions as compared with the gold standard, lesion conspicuity, internal features and the absence of artifacts). There was no statistically significant difference in contrast-to-noise ratio between the two sequences (p = 0.713). In the qualitative analysis, CSE sequences were superior to FSE for overall image quality in 50% of cases, for lesion conspicuity in 41.5% of cases and for internal features and the absence of artifacts in 46% of cases, FSE sequences had a higher detection rate in 17% of cases, even though both types of sequences underestimated the number of lesions in 29% of cases, as compared with the gold standard. To conclude, FSE sequences were inferior to CSE for image quality, lesion conspicuity, internal features and the absence of artifacts. FSE sequences were superior in the detection and characterization of fluid and nearly fluid lesions.
BACKGROUND: Congenital arteriovenous malformations represent a rare cause of heart failure in neonates. MATERIALS: Four neonates with arteriovenous malformations (cerebral in one, hepatic in two and pulmonary in one) are reported. RESULTS: In the first three cases Color Doppler echography was able to image the arteriovenous malformations, to identify the afferent and efferent vessels and to quantify the arteriovenous shunt. In the neonate with pulmonary malformation, a cardiac cause of heart failure was excluded by echocardiography, and the diagnosis was stated by selective angiography. The neonate with cerebral great arteriovenous malformation died at 4th day of life. The two neonates with hepatic malformations improved both with medical therapy; in the last neonate, with multiple arteriovenous pulmonary fistulae, selective embolization was performed, but the child died a few months later. CONCLUSION: Congenital arteriovenous malformations are rare, and an early diagnosis is the base for a correct therapy; Color Doppler echography represents the gold standard for cerebral and hepatic malformations, while selective angiography should be reserved to patients who might profit of embolization or surgical resection.
The influence of nasal obstruction on the development of the anterior skull base, of the tympanic bulla, and of the Eustachian tube was investigated in three groups of 20 albino Wistar rats in which one (group B) or both nostrils (group C) had been experimentally obstructed by means of synthetic resin mixed with radiopaque material. After two months, a cephalometric investigation demonstrated that the angle formed by the median line passing through the superior interincisive point and the most posterior median point of the occipital bone and by the line connecting the tympanic bulla and the superior interincisive point was significantly increased when the homolateral nostril had been obstructed if compared to controls (group A). On the basis of these preliminary cephalometric observations we suggest that nasal obstruction is able to determine anatomical changes of the superior maxilla, the skull base, and the jaw, with abnormal skeletal growth and consequent possible Eustachian tube dysfunction.
CO2 cerebrovascular reactivity has been recorded in 12 healthy volunteers and 10 patients with unilateral > 70% extracranial internal carotid artery (ICA) stenosis, using non invasive techniques. The relative changes of middle cerebral artery blood flow velocity (VMCA) and velocity waveform pulsatility (PIMCA) after that hypocapnia was induced by spontaneous hyperventilation were recorded. 35.5% average VMCA reduction and 63% PIMCA increment of basal values was produced in healthy subjects after hyperventilation. The percentage variation of CO2 Reactivity Index (RI), expressed in terms of VMCA (V-RI) and PIMCA (PI-RI), per mmHg change in pCO2, presents a good right-left side correlation (r = 0.82 and r = 0.83 respectively) in healthy subjects, while a dissociation between V-RI and PI-RI was found in our patients. A significant reduction of PI-RI was also recorded in the group of patients on the side of ICA stenosis. From our data CO2 reactivity index recorded in terms of PI seems to allow a better separation between pathology and normality, without the need to assume a close relationship between velocity and blood flow under the condition considered. Furthermore, PI-RI seems to be a valid index in the evaluation of some attribute pertaining to the distal vascular bed.
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An innovative instrumentation and stabilization system was used for the treatment of unstable thoracolumbar fractures. The new osteosynthesis apparatus employs pedicle screw fixation, plate application, and a mechanical device for dynamic reduction in stable posterior fixation. Sixteen patients were treated with this operative technique that included a decompressive laminectomy, an arthropedunculectomy, and the removal of vertebral canal bone fragments. In most patients, three vertebral bodies, rather than four or five, were immobilized. All patients were studied pre- and postoperatively with plain radiographs and computed tomography. Pre- and postoperative examinations demonstrated no alteration in neurological status. All patients, however, with incomplete lesions showed improvement in neurological function at a mean follow-up period of 12 months. The authors' experience suggests that the new system offers ease and versatility of application, restoration of vertebral alignment through mechanical reduction, and enhanced spinal stability. The new system, moreover, enables the reduction of lateral dislocation.