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Introduction

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) is a syndrome that combines dermatological, articular and osseous inflammatory manifestations. Bilateral laryngeal immobility relative to cricoarytenoid joint origin is very uncommon. This article presents a case of bilateral cricoarytenoid joint ankylosis in a SAPHO syndrome context.

Case report

A 53-year-old patient presenting with a two year history of intermittent bouts of dyspnea. A SAPHO syndrome was discussed on repeated thoracic CT-scan. The link between dyspnea and SAPHO syndrome had not been made immediately given the absence of any known anteriority. However, having ruled out other etiologies and after having had to perform a tracheotomy due a worsening of the respiratory condition, this diagnosis was considered. Treatment by corticosteroids and infliximab permitted a clinical improvement of the patient.

Conclusion

This clinical case report should increase awareness of possible cricoarytenoid joint involvement in SAPHO.  相似文献   

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The authors report the case of a 81 years-old-woman who had simultaneously atypical Cogan's syndrome, cutaneous vasculitis and diverticular sigmoiditis. The relations being between these three diseases are discussed.  相似文献   

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Severe hypokalemia with alkalosis, increased plasma renin activity and aldosterone levels were detected in 5 patients without hypertension or peripheral oedema. Hyperuricemia was observed in 3 cases and hypomagnesemia in 2 cases.Although angiotensin II infusions did not elicit a vaso-pressor response in 2 patients the symptomatology was not related to Bartter's syndrome as urinary chloride levels were very low. Four patients admitted self-induced vomiting.Prostaglandin levels, isotopic dilution tests, acidification with arginine chlorhydrate and fractional distal tubular chlorid clearance were assessed in some of these patients. The results support the pathogenic hypothesis of chloride depletion in Bartter's syndrome and pseudo Bartter's syndromes.  相似文献   

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ObjectiveTo evaluate the effect of 25 (OH) vitamin D supplementation on blood pressure (BP) variability in hypertensive women in the pre-menopausal and post-menopausal periods.Materials and methods881 hypertensive women prospectively followed for an interventional study between January 2016 and September 2017, in specialized consultation at the department of internal medicine at the Blida University Hospital (Algeria). Four hundred and thiry nine premenopausal women (group I) and 442 menopausal women (group II). The initial serum 25 (OH) D level for each group was determined by the enzyme immunoassay. In groups I and II, we identified 2 subgroups, A: insufficiency (vit D between 29 and 20 ng/ml) and B: deficiency (vit D less than 20 ng/L). Antihypertensive therapy was supplemented with an additional 200000 IU/month cholecalciferol for the two B subgroups. The variability in BP was calculated as the ratio of mean systolic and diastolic BP during daytime and nighttime, with performing ambulatory BP measurement at baseline, 3, 6, and 12 months of follow-up.ResultsAt inclusion, the level of 25 (OH) D was lower (P < 0.05) in subgroups IB (19.3 ± 8.5 ng/ml) and IIB (18.2 ± 9, 5 ng/ml) compared to subgroups IA (28.1 ± 10.7 ng/ml) and IIA (25.2 ± 10.1 ng/ml). After supplementation, the level of 25 (OH) D increased in subgroup IB (38.3 ± 11.9 ng/ml) and in subgroup IIB (37.3 ± 10, 5 ng/ml) and became higher (P < 0.001) than in subgroups IA and IIA. Between subgroups IA and IB, at inclusion, there is no difference (P > 0.05) in the SBP and DBP variability during the day and at night. After treatment, the variability of the SBP at night became lower (P < 0.02) in group IB compared to group IA. In subgroup IIB, daytime variability indices were higher (P = 0.04) at inclusion than in group IIA. After treatment, the variability of SBP during the day decreased but remained the highest (P < 0.05) in subgroup IIB (14.8 ± 10.8 mmHg) compared to subgroup IB (12.0 ± 8.1 mmHg), as well as to subgroups IIA (10.9 ± 9.8 mmHg) and IA (10 ± 8.1 mmHg). We found a significant correlation of cholecalciferol with the variability of SBP during the day.ConclusionsVitamin D deficiency appears to be a factor of BP variability. Although the variability of the postmenopausal group remains higher than that of the other groups, the correction of the level of 25 (OH) D by the supply of cholecalciferol 200000 IU per month leads to a reduction in the variability of BP in the studied hypertensive women could help to prevent morbimortal complications.  相似文献   

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Incidence of malignant melanoma has been increasing since the 1980s. For loco-regional stages, surgery is still the best treatment. Melanoma has a high distant metastatic potential and prognosis of advanced stages was until recently very poor. Since 2011 however, a real revolution has taken place in the treatment of metastatic melanoma. This is based upon considerably improved knowledge of the molecular mechanisms of melanoma and cancer immunology. Thus, two new classes of systemic therapeutic agents are now available: immunotherapies (immunological checkpoint inhibitors), which increase the antitumor immune response, and targeted therapies (BRAF and MEK inhibitors) for patients with BRAF V600-mutant melanoma. Overall survival is now 2 years or above, with hope for a cure in some cases. Unfortunately, the efficacy of these treatments is incomplete and many studies are underway to try to identify predictive biomarkers, and multiple combinations are being evaluated to increase response rates. The efficacy of these treatments has also been shown in the adjuvant setting in high-risk melanoma, they should be available shortly.  相似文献   

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It is currently well established that more than half of heart failure patients have preserved ejection fraction. The diagnosis of heart failure with preserved ejection fraction is complex in clinical practice despite ESC recommendations issued in 2019. In this context, the demonstration of increased left ventricular filling pressures at rest or during exercise allows a definite diagnosis of heart failure with preserved ejection fraction in patients with signs and/or symptoms compatible with the diagnosis and a preserved ejection fraction. The spectral tissue Doppler-derived E/e’ ratio by transthoracic Doppler echocardiography has been validated in the noninvasive assessment of left ventricular diastolic pressures at rest and during exercise. Several studies report the validity of E/e’ in the diagnosis of heart failure with preserved ejection fraction in patients with both isolated exertional and acute dyspnea, as well as in risk stratification. In light of the current literature, E/e’ deserves to be included on every transthoracic Doppler echocardiography report in patients with suspected heart failure with preserved ejection fraction. This updated review provides an overview of the diagnostic relevance of E/e’ in patients in its two modes of clinical presentation, isolated exertional dyspnea and the decompensated congestive form.  相似文献   

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IntroductionThe adult emergency department at Rouen University hospital (CHU) welcomes over 100.000 patients per year. In order to streamline unscheduled hospital admissions from the emergency room (ER), a 20-bed pre-hospitalization unit and a centralized bed management system (bed manager, bed manager software, dedicated beds) have been put into place.Patients and methodsEmergency admissions have increased by (+ 3.5% between 2017 and 2018) with 20% direct hospitalization from the ER to other conventional units (2/3 in medicine, 1/3 in surgery). In 2018, 3450 patients, of which 54% aged over 75 years have been admitted in the pre-hospitalization unit with an average length of stay of 1.3 ± 1.4 days: 35.4% stayed less than 24 hours and 34.8% more than 48 hours of which 5.2% stated more than 4 days, 132 patients (3.8%) died, 805 patients (23.3%) were discharged at home, 220 (6.4%) transferred to another facility, and 2287 (66.3%) were secondarily hospitalized in another hospital unit: more than 9 times out of 10 in a medicine unit (internal medicine 30%, geriatrics 27.9%, respiratory medicine 12.2%). This unscheduled emergency hospitalization allowed a daily hospitalization of 50 short stay inpatients beds. It has to be noted that the number of available inpatient beds clearly decreases during the week-ends. The main pathologies were respiratory infections (14.2%), heart diseases (9.7%), metabolic disorders (3.9%), and urinary tract infections (13.6%).ConclusionThis pre-hospitalization unit associated with a centralized bed management system has clearly improved the unscheduled hospital admissions, in particular concerning the emergency medical sector. The lack of inpatient beds at the week-end and the management of epidemic periods still remain a challenge that has to be taken up.  相似文献   

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《Annales d'endocrinologie》2020,81(6):567-571
BackgroundInsulinomas are usually benign, small-sized, well-encapsulated and often solitary pancreatic tumors. Currently, enucleation is the treatment of choice for sporadic solitary insulinoma if diameter is less than 2 cm and the structural integrity of the pancreatic duct can be maintained. However, the procedure has a risk of postoperative complications, and especially of pancreatic fistula. There is growing interest in endoscopic ultrasound-guided radiofrequency ablation (EUS-RFA) as an effective and less invasive alternative treatment for benign sporadic insulinoma.MethodWe retrospectively analyzed the efficacy and safety of EUS-RFA in four patients with benign localized insulinoma treated in our tertiary care center between June 2018 and November 2019. EUS-RFA was performed with a EUS-guided RFA 19-gauge needle electrode (Starmed; Taewoong Medical, Seoul, South Korea) that released energy at 50W up to 100 Ohms impedance.ResultsThe series comprised three women and one man, with a median age of 58 years (range 52–82 years). Mean tumor size was 12 mm. Although three of the four patients would have been eligible for surgery, EUS-RFA was proposed to them. Symptomatic and biological improvement occurred immediately, generally straight after the procedure, in all patients, and no serious complications were observed. During the mean follow-up period of 22 months, no symptom recurrence was observed.ConclusionsThis preliminary report in 4 patients showed that EUS-RFA was an effective and relatively safe alternative treatment, devoid of major complications, for benign sporadic insulinoma. Larger-scale prospective multicenter studies are, however, needed to confirm the long-term effectiveness and safety of this novel technique.  相似文献   

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Introduction

MRI should be performed in the presence of an acute febrile urinary retention, when septic and obstructive causes are eliminated. We report a case of post-infectious probable acute disseminated encephalomyelitis (ADEM) with a mostly spinal cord tropism of involving Campylobacter.

Case report

A 32-year-old man with no medical history was admitted for an acute febrile urinary retention. He reported severe diarrhea 3 days before. Clinical course was then complicated by a progressive tetraparesis predominating in the lower limbs. Medullar MRI showed thoracic myelitis. A five-day course of intravenous corticosteroids allowed a full recovery of both the motor and urinary symptoms. Fecal culture isolated Campylobacter sp. Final diagnosis was post-bacterial ADEM.

Conclusion

Clinical findings and MRI allow clinicians to suspect acute disseminated encephalomyelitis. This hypothesis implies to actively look for recent infections or vaccinations preceding the clinical presentation.  相似文献   

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Among the antibodies described in Systemic Sclerosis (SSc), anti-Th/To antibodies (anti-Th/To) are rare and have been poorly studied. Thus, little is known about the profile of anti-Th/To positive patients. From our local Biobank (Marseille, France), we retrospectively selected data for 6 patients positive for anti-Th/To with an Immunodot assay. All of them suffered from SSc, sharing clinical and biological common features such as a limited cutaneous form of SSc, a decreased lung diffusing capacity and a speckled nuclear nucleolar immunofluorescence pattern of antinuclear antibodies screening on HEp-2 cells. In order to further characterize patients positive for anti-Th/To, we performed a thorough literature review. From 402 studied patients positive for anti-Th/To, we confirmed that these antibodies are associated with the limited cutaneous form of the disease (88% of the patients), and with an SSc related-pulmonary involvement (50%). The review analysis pointed out the rarity of the anti-Th/To with an estimated mean frequency of 3.4% of all SSc patients worldwide, their usual exclusivity with respect to the specific antibodies of scleroderma, and their high specificity (around 98%) for the diagnosis of SSc.  相似文献   

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Elevation of serum angiotensin-converting enzyme (ACE) has been reported to occur in 25 p. 100 of diabetic patients. This finding would decrease the specificity of increased ACE level in sarcoidosis. We measured serum ACE in 90 diabetic patients with simultaneous analysis of various parameters including those appreciating the degree of metabolic control. Serum ACE values, observed in diabetics are not different from values measured in controls, respectively 22,3 +/- 6,4 and 21,1 +/- 6,7 U/ml (means +/- SD). Only 5,5 p. 100 of diabetic patients had (slightly) increased serum ACE levels, i.e.: levels above mean +/- 2 SD of values observed in controls. Serum ACE levels were higher in insulin-dependent diabetics: 24,7 +/- 6,7 (n = 34) than in non insulin-dependent diabetics: 20,8 +/- 5,9 (n = 56) (p = 0,01). In the diabetic group, there is a positive linear correlation between serum ACE level and 24h-glycosuria or glycosylated A1c hemoglobin. These are parameters appreciating middle or long-term metabolic control. In the other hand, no correlation was observed between serum ACE and glycemia, triglyceridemia, serum creatinin, blood pressure or the presence of diabetic retinopathy. These findings do not show an elevation of serum ACE in diabetic patients. However, they suggest that long-term metabolic control of diabetes influence serum ACE level.  相似文献   

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Introduction

Nocardia is an opportunist bacteria involved in patients with cellular immunodepression or chronic lung disease. The most frequent portals of entry are the respiratory tract by inhalation or direct inoculation through a cutaneous effraction. Nocardiosis may be localised or disseminated.

Case report

We report a rare case of disseminated nocardiosis to Nocardia nova with pulmonary, cutaneous, cerebral attacks and femoral osteomyelitis. The diagnosis was confirmed by prolonged cultures of the bronchoalveolar fluid and the pus extracted from a cutaneous lesion. The outcome was favorable under adapted and prolonged antibiotherapy with imipenem and amikacine and then cotrimoxazole.

Conclusion

This observation is original because it involves a immunocompetent patient with an association of two exceptional locations for N. nova: brain abscesses and femoral osteomyelitis. Screening for cerebral involvement should be systematic, even in the lack of neurological signs. An adapted and prolonged antibiotherapy must be conducted.  相似文献   

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