scholarly journals Prognostic Biomarkers of Sarcoidosis: A Comparative Study of Serum Chitotriosidase, ACE, Lysozyme, and KL-6

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Laura Bergantini ◽  
Francesco Bianchi ◽  
Paolo Cameli ◽  
Maria Antonietta Mazzei ◽  
Annalisa Fui ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Lung Fibrosis ◽  
Clinical Utility ◽  
Clinical Course ◽  
Unknown Etiology ◽  
Serum Concentrations ◽  
Referral Centre ◽  
Clinical Presentations ◽  
Serum Detection

Purpose. Sarcoidosis is a systemic granulomatous disease with unknown etiology. Many clinical presentations have been reported, and acute disease needs to be distinguished from subacute and chronic disease. The unpredictable clinical course of the disease prompted us to evaluate the clinical utility of biomarker serum detection in sarcoidosis follow-up. Methods. Serum concentrations of chitotriosidase, ACE, KL-6, and lysozyme were analyzed by different methods in a population of 74 sarcoidosis patients (46 on steroid therapy at sampling) regularly monitored at Siena Sarcoidosis Regional Referral Centre and in a group of controls with the aim of comparing their contribution to clinical management of sarcoidosis patients. Results. KL-6 concentrations were significantly elevated in sarcoidosis patients with lung fibrosis and were significantly correlated with DLco and CPI score, while chitotriosidase was significantly higher in patients with extrapulmonary localizations. With a cut-off value of 303.5 IU/ml, KL-6 showed the best sensitivity (78%), while chitotriosidase reported the best specificity (85%) among the biomarkers. Conclusions. KL-6 is a reliable biomarker of fibrotic lung involvement in sarcoidosis patients. Among biomarkers, KL-6 showed the best sensitivity and serum chitotriosidase the best specificity, even in patients on chronic steroid therapy, and seemed to correlate with extrapulmonary localizations.

scholarly journals Clinical Presentations, Pathogenesis, and Therapy of Sarcoidosis: State of the Art

2020 ◽  
Vol 9 (8) ◽  
pp. 2363
Author(s):  
Francesca Polverino ◽  
Elisabetta Balestro ◽  
Paolo Spagnolo
Keyword(s):  
Clinical Course ◽  
State Of The Art ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
Therapeutic Approaches ◽  
Epidemiologic Factors ◽  
Adaptive Immune ◽  
Clinical Presentations ◽  
Specific Organ ◽  
Noncaseating Granulomas

Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas that can occur in any organ, most commonly the lungs. Early and accurate diagnosis of sarcoidosis remains challenging because initial presentations may vary, many patients are asymptomatic, and there is no single reliable diagnostic test. Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement. From a pathobiological standpoint, sarcoidosis represents an immune paradox, where an excessive spread of both the innate and the adaptive immune arms of the immune system is accompanied by a state of partial immune anergy. For all these reasons, the optimal treatment for sarcoidosis remains unclear, with corticosteroid therapy being the current gold standard for those patients with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease. This review is a state of the art of clinical presentations and immunological features of sarcoidosis, and the current therapeutic approaches used to treat the disease.

scholarly journals Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases

2016 ◽  
Vol 125 (1) ◽  
pp. 160-166 ◽  
Author(s):  
Tao Yu ◽  
Xingwen Sun ◽  
Yan You ◽  
Jie Chen ◽  
Jun-mei Wang ◽  
...  
Keyword(s):  
Vision Loss ◽  
Clinical Course ◽  
Complete Removal ◽  
Neurological Deficits ◽  
Radiological Findings ◽  
Brain Capillary ◽  
Generalized Seizures ◽  
Clinical Presentations ◽  
Uncontrolled Bleeding

Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left untreated. Therefore, large BCTs should be managed with special caution. Because of the lack of reports, diagnosis of large BCTs has been difficult. Strategies of management are undefined for large or giant BCTs. The current study presents 5 cases of giant and large BCTs. To the authors’ knowledge, this is the largest series of this disease ever reported. Radiological findings, histopathological characteristics, clinical presentations, and surgical management were analyzed in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8–8 cm). Four patients presented with focal or generalized seizures, and 1 patient presented with transient vision loss attributed to the lesions. Gross-total resection of the lesion was achieved in all patients. After surgery, the 4 patients with seizures were symptom free for follow-up periods varying from more than 1 to 5 years with no additional neurological deficits. The unique location, radiological characteristics, and clinical course suggest that giant BCTs could be a different entity from small BCTs. Surgery might be a good option for treatment of patients with intractable neurological symptoms, especially in those with surgically accessible locations. Complete removal would be anticipated to provide relief of the symptoms without causing new neurological deficits.

scholarly journals Myocarditis: Which Role for Genetics?

2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Chiara Baggio ◽  
Giulia Gagno ◽  
Aldostefano Porcari ◽  
Alessia Paldino ◽  
Jessica Artico ◽  
...  
Keyword(s):  
Genetic Background ◽  
Clinical Course ◽  
Risk Profile ◽  
Myocardial Inflammation ◽  
Genetic Defects ◽  
Prognostic Role ◽  
Clinical Presentations

Abstract Purpose of Review Myocarditis is a polymorphic disease, both in its presentation and clinical course. Recent data suggests that the genetic background, interacting with environmental factors, could be diriment both in the susceptibility and evolution of myocarditis in different clinical presentations. The aim of this paper is to expose the current available evidences and the evolving concepts on this topic, in order to provide insight for improving the clinical management of those patients. In this regard, the main goal is an optimal characterization of each patient’s risk, with the purpose of individualizing the treatment and the follow-up. Recent Findings The latest research highlights the possible prognostic role of some pathogenic mutations that could create a vulnerable myocardium prone to myocardial inflammation and also to the development of a long-lasting cardiomyopathy. Summary The identification of these genetic defects and of myocarditis patients requiring genetic testing is emerging as a challenge for the future. In fact, identifying a possible genetic background responsible for a particularly high-risk profile could be of extreme importance in improving management of myocarditis. This and many other aspects in the genetics of myocarditis remain uncovered, and further studies are expected based to refine our daily clinical practice.

scholarly journals Recurrent unilateral restrictive elevation deficit in childhood: a tailored approach

2021 ◽  
Vol 14 (6) ◽  
pp. e240561
Author(s):  
Deepesh Unni ◽  
Abdul Shameer ◽  
Pradeep Sharma
Keyword(s):  
Clinical Course ◽  
Rectus Muscle ◽  
Complete Relief ◽  
Single Muscle ◽  
Inferior Rectus ◽  
Clinical Presentations ◽  
Tailored Approach ◽  
Multiple Recurrences ◽  
Inferior Rectus Muscle

Monocular elevation deficiency poses a challenge to strabismus surgeons on account of its varied clinical presentations as well as management which often needs a tailored approach. We report on a young child who presented to us at 6 months of age with a clinical course marked by primary involvement of the inferior rectus muscle in one eye causing restricted elevation in all gazes and complete relief of hypotropia following disinsertion of the affected muscle but followed by recurrence and additional procedures (antimitotic application and superior rectus plication) for the same. She followed a recalcitrant clinical course which was marked by multiple recurrences requiring a tailored approach and finally managed successfully with a follow-up of 3 years, by now. This case demonstrates the almost intractable nature of restrictive pathology involving a single muscle warranting multiple surgeries and a close follow-up with good surgical outcome.

scholarly journals The Prognostic Role of Biomarkers in Patients With Chronic Heart Failure

Kardiologiia ◽  
2020 ◽  
Vol 60 (1) ◽  
pp. 16-22
Author(s):  
E. K. Kurlianskaya ◽  
A. G. Mrochek ◽  
T. L. Denisevich ◽  
M. G. Kaliadka ◽  
I. I. Russkich
Keyword(s):  
Poor Prognosis ◽  
Clinical Course ◽  
Cardiac Complications ◽  
Serum Concentrations ◽  
Laboratory Measurements ◽  
Blood Concentrations ◽  
Galectin 3

Objective Investigate the role of biomarkers in the prognosis of the clinical course of the disease in patients with chronic heart failure (CHF) of different NYHA functional classes (FC).Material and Methods The study included 132 patients with CHF: Group 1 was composed of 70 patients with NYHA FC II CHF, and Group 2 included 62 patients with FC III-IV CHF. The patients underwent clinical, instrumental, functional, and laboratory measurements, which included serum concentrations of NT-proBNP, ST-2, galectin-3, and C-reactive protein. Patients were examined at baseline and at 3, 6, and 12 mos of follow-up. The following cardiac complications were used as endpoints: urgent hospitalization due to decompensated CHF, heart transplantation, cardiovascular death. Endpoints were registered during the 12-mo follow-up period.Results Endpoints were recorded for 58 patients (44%) of the total sample of patients with CHF: 38 patients were urgently hospitalized, 10 patients underwent heart transplantation, 10 patients died. Cardiac complications were recorded at a higher rate in patients with FC III-IV CHF (63% vs. 27% of patients with FC II; p<0.001). In FC II CHF patients, the incidence of cardiac complications was significantly correlated with NT-proBNP blood concentrations (Rpb=0.53; p=0.023), left ventricular end-diastolic volume (LVEDV) (Rpb=0.50; p=0.044), and mitral regurgitation (Rpb=0.53; p=0.038). Cardiac complications in patients with FC III-IV CHF were associated with ST-2 (Rpb=0.52; p=0.004) and galectin-3 (Rpb=0.46; p=0.009) blood concentrations, and with systolic pulmonary artery pressure (PAP) (Rpb=0.41; p=0.014). Unlike other laboratory measurements, galectin-3 concentrations were significantly correlated with type 2 diabetes mellitus (DM2) (Rpb=0.40; p=0.003). In this study, correlation analysis and evidence of significant differences in the concentrations of biomarkers provided a rationale for identifying potential predictors of severe cardiac complications during medium- and long-term follow-up periods in patients with CHF of different severity: NT-proBNP concentrations in FC II patients; ST-2 and galectin-3 serum concentrations in FC III-IV patients; galectin-3 concentrations in patients with CHF and DM2.Conclusion NT-proBNP blood concentrations are associated with CHF severity and serious cardiac complications in patients with FC II CHF within the following 12 mos. The poor prognosis of FC III-IV CHF is associated with the concentration of the ST-2 biomarker. The blood concentration of galectin-3 is a significant predictor of poor prognosis in patients with CHF and DM2. Predictors of the adverse course of CHF of varying severity were differentiated. For FC II CHF, NT-proBNP > 1723 pg/ml or, if NT-proBNP < 1723 pg/mL, then EDV > 311 ml. For FC III-IV CHF, ST-2 > 67 ng/mL or, if ST-2 < 67 ng/mL, then PAP > 61 mm Hg. Galectin-3 has a prognostic value for the clinical course of the disease at different follow-up periods in patients with CHF and DM2: galectin-3 concentrations > 16 ng/mL and 13-16 ng/mL are risk factors for mid- and long-term cardiac complications, respectively.

scholarly journals Serial KL-6 measurements in COVID-19 patients

2021 ◽  
Author(s):  
Miriana d’Alessandro ◽  
◽  
Laura Bergantini ◽  
Paolo Cameli ◽  
Giuseppe Curatola ◽  
...  
Keyword(s):  
Potential Role ◽  
Good Accuracy ◽  
Clinical Course ◽  
Lung Damage ◽  
University Hospital ◽  
Receiver Operating Curve ◽  
Serum Concentrations ◽  
Cytopathic Effects ◽  
Type Ii Pneumocytes

AbstractSARS-CoV2-induced direct cytopathic effects against type II pneumocytes are suspected to play a role in mediating and perpetuating lung damage. The aim of this study was to evaluate serum KL-6 behavior in COVID-19 patients to investigate its potential role in predicting clinical course. Sixty patients (median age IQR, 65 (52–69), 43 males), hospitalized for COVID-19 at Siena COVID Unit University Hospital, were prospectively enrolled. Twenty-six patients were selected (median age IQR, 63 (55–71), 16 males); all of them underwent follow-up evaluations, including clinical, radiological, functional, and serum KL-6 assessments, after 6 (t1) and 9 (t2) months from hospital discharge. At t0, KL-6 concentrations were significantly higher than those at t1 (760 (311–1218) vs. 309 (210–408) p = 0.0208) and t2 (760 (311–1218) vs 324 (279–458), p = 0.0365). At t0, KL-6 concentrations were increased in patients with fibrotic lung alterations than in non-fibrotic group (755 (370–1023) vs. 305 (225–608), p = 0.0225). Area under the receiver operating curve (AUROC) analysis showed that basal KL-6 levels showed good accuracy in discriminating patients with fibrotic sequelae radiologically documented (AUC 85%, p = 0.0404). KL-6 concentrations in patients with fibrotic involvement were significantly reduced at t1 (755 (370–1023) vs. 290 (197–521), p = 0.0366) and t2 (755 (370–1023) vs. 318 (173–435), p = 0.0490). Serum concentrations of KL-6 in hospitalized COVID-19 patients may contribute to identify severe patients requiring mechanical ventilation and to predict those who will develop pulmonary fibrotic sequelae in the follow-up.

scholarly journals Langerhans Cell Histiocytosis: A Single Institution Retrospective Analysis of Eleven Patients

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5458-5458
Author(s):  
Vittorio Stefoni ◽  
Alessandro Broccoli ◽  
Beatrice Casadei ◽  
Enrico Derenzini ◽  
Letizia Gandolfi ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Retrospective Analysis ◽  
Steroid Therapy ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy

Abstract Langerhans cell histiocytosis (LCH), is a rare disorder which has a substantially unknown etiology, pathophysiology, and may manifest through a variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. LCH is more common in children, although it can affect any age; the most common sites of involvement are bone, skin, and lung. From a histological point of view LCH derives from accumulation of proliferating cells with surface markers and ultrastructural features similar to cutaneous Langerhans cells, intermixed with inflammatory cells, particularly eosinophils. Below, a retrospective analysis of LCH patients treated at our institution. Between 1997 and 2013 we have treated 11 LCH patients, including 6 females and 5 males with a median age at time of diagnosis of 42.9 years (range 22.2-62.3). All diagnoses were reviewed by our pathologist. With regard to the site at onset, 9 patients had bone involvment, among these, four patients had only bone involvment, the other five patients also lung, oral cavity and lymph nodes. At time of onset 4 patients showed no symptoms, while the remaining 7 showed a variety of symptoms ranging from B symptoms to tinnitus, dizziness, and other neurological symptoms such as diplopia. Among the study group 6 patients had multisystemic involvement. All patients except one had CT scan performed before, during, and at follow-up, the remaining patient was studied and followed through follow-up with PET scan. As first-line therapy 8 patients underwent chemotherapy, 2 patients radiation therapy, 1 patient required only steroid therapy. The most frequently used chemotherapy regimen for these 8 patients was MACOP-B, a third generation, CHOP-like regimen. Responses to first-line therapy were as follows: 7 complete remissions (CR), resulting with chemotherapy (5), radiation therapy and steroid therapy, two partial remissions (both obtained with chemotherapy) and two stable diseases (1 with chemotherapy and 1 with radiation therapy). Two patients relapsed, of whom one has ran several lines of chemotherapy, including autologous stem cell transplantation. Both are alive at the time of the last follow-up. To date all patients are alive but one, who died of pulmonary embolism while he was in stable disease. Six patients are in CR (60%), two in SD (20%) and two in PD (20%). In conclusion, our monocentric experience of 11 LCH patients confirms what reported in the literature in terms of heterogeneity of presentation, age, sites of involvement, symptomatology and treatment demanded. Coming to the the results our retrospective analisys shows that ten of the eleven study population patients (90.9%) are to date still alive after a significant median time of follow-up; six out of these ten patients (60%) are in CR. Disclosures No relevant conflicts of interest to declare.

scholarly journals Bullous prurigo pigmentosa

2019 ◽  
Vol 14 (1) ◽  
pp. 214-216
Author(s):  
Xinjun Wang ◽  
Chenchen Xu
Keyword(s):  
Unknown Etiology ◽  
Direct Immunofluorescence ◽  
Entire Area ◽  
Reticular Pattern ◽  
Inflammatory Dermatosis ◽  
Clinical Presentations ◽  
History Of ◽  
Minocycline Hydrochloride ◽  
Erythematous Plaques

AbstractPrurigo pigmentosa (PP) is an inflammatory dermatosis with unknown etiology. The clinical presentations of PP varies according to the stages of the disease. Rarely, the formation of numerous vesicles and bullae upon erythematous infiltrative plaques can be found during the entire clinical course. In the present case, a 29-year-old Chinese woman presented with a 6-year history of relapsing pruritic erythematous plaques and bulla on her neck, chest and back. Physical examination revealed multiple erythematous plaques and vesicles in combination with mottled pigmentation in a symmetrical distribution and reticular pattern on the nape of her neck, chest and back. Histological examination of the biopsy specimen collected from the bullous area of her chest indicated a lichenoid reaction with intraepidermal bulla. This inflammatory region is characterized by recruitment of lymphocytes, spongiosis, and a perivascular lymphohistiocytic infiltrate in the upper dermis. Direct immunofluorescence analysis for IgG, IgA, IgM and C3 was negative. The diagnosis of bullous prurigo pigmentosa was verified based on the clinical manifestation and pathological findings. Minocycline hydrochloride therapy (100mg/d) was initiated, and 3 weeks later the rash had completely disappeared, which resulted in pigmentation of the entire area. No recurrence was observed during the 4 years follow-up.

scholarly journals A Case of Possible Neurosarcoidosis Presenting as Intractable Headache and Panhypopituitarism

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Jin Kyung Hwang ◽  
Joo Hee Cho ◽  
So Young Park ◽  
Jung Il Son ◽  
Uk Jo ◽  
...  
Keyword(s):  
Nervous System ◽  
High Risk ◽  
Steroid Therapy ◽  
Surgical Complication ◽  
Symptom Relief ◽  
Granulomatous Inflammation ◽  
Unknown Etiology ◽  
Systemic Involvement ◽  
Pituitary Lesion

Sarcoidosis is a chronic multisystemic inflammatory disease of unknown etiology, which is characterized by noncaseating granulomatous inflammation of the involved organs. It is known that neurosarcoidosis involving the nervous system occurs in about 5% of patients with sarcoidosis. However, neurosarcoidosis without systemic involvement is extremely rare. We present a case of suspicious neurosarcoidosis affecting the pituitary gland, which was manifested as chronic uncontrolled headache, panhypopituitarism, central diabetes insipidus, and hypercalcemia. Though the biopsy at the pituitary lesion was not performed due to the high risk of surgical complication, treatment was needed urgently and we started steroid therapy. After steroid therapy, we observed the immediate symptom relief with improved hypercalcemia. According to the follow-up examination, no recurrent symptom was seen, and resolution of the pituitary lesion with improving panhypopituitarism was noted.

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