Extracapillary Proliferative Glomerulonephritis in Children

PEDIATRICS ◽  
1975 ◽  
Vol 56 (3) ◽  
pp. 434-442
Author(s):  
Sudhir K. Anand ◽  
Carl W. Trygstad ◽  
Hari M. Sharma ◽  
James D. Northway
Keyword(s):  
Streptococcal Infection ◽  
Clinical Course ◽  
Renal Involvement ◽  
Therapeutic Agents ◽  
Proliferative Glomerulonephritis ◽  
The Mean ◽  
Histopathologic Changes ◽  
Better Than

The clinical course and renal pathology of 17 children with acute extracapillary proliferative glomerulonephritis is reported. Patients with systemic ses associated with renal involvement were excluded. The onset followed streptococcal infection in ten; of these, four have died, one has been transplanted, and the remaining five have completely healed. The mean follow-up of the latter five patients was 32 months (range, 18 to 57 months). Of the other seven patients without evidence of preceding streptococcal infection, two have died, two have been transplanted, and the remaining three all have hypertension, proteinuria, and reduced creatinine clearance. The mean follow-up of the latter three patients was 29 months (range, 14 to 38 months). The initial renal histopathologic changes and their progress in later renal biopsies is described. The role of various therapeutic agents is discussed. The prognosis in acute extracapillary proliferative glomerulonephritis following streptococcal infection appears to be better than in ones without preceding streptococcal infection.

scholarly journals POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  
Keyword(s):  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Age At Diagnosis ◽  
Systemic Involvement ◽  
Risk Of Death ◽  
Anca Associated Vasculitis ◽  
The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

scholarly journals SAT0267 ROLE OF AGGRESSIVE IMMUNOSUPPRESSION ON SUBGLOTTIC STENOSIS IN GRANULOMATOSIS WITH POLYANGIITIS: RETROSPECTIVE ANALYSIS OF A MONOCENTRIC COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1076.1-1077
Author(s):  
L. Moroni ◽  
L. Giudice ◽  
G. A. Ramirez ◽  
S. Sartorelli ◽  
A. Cariddi ◽  
...  
Keyword(s):  
Medical Treatment ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Balloon Catheter ◽  
Subglottic Stenosis ◽  
Damage Accrual ◽  
The Mean ◽  
The One

Background:Subglottic stenosis (SGS) is defined as airway narrowing below the vocal cords and is a common and potentially life-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with an estimated prevalence of 16-23% (1). Balloon catheter dilation is effective in GPA-related SGS, but relapses are frequent. Little is known about the role of immunosuppression in this setting.Objectives:to analyse the clinical characteristics of a monocentric GPA cohort, describe phenotype differences among patients with and without SGS and investigate the role of surgical and medical treatments on relapse risk and general outcome.Methods:Biopsy-proven patients with SGS were identified by review of medical charts among a cohort of patients with GPA, classified according to the algorithm of the European Medicine Agency (2). The clinical characteristics of patients with SGS were retrospectively collected over a median follow-up time of 15.9 years and compared to those of patients without SGS.Results:Fourteen patients with SGS-GPA were identified, with a female to male ratio of 1:1 and a prevalence of 29.2% among the cohort. The mean ± SD age at GPA onset was 30.8 ± 14.4 years, with a mean time from GPA diagnosis to SGS onset of 4.7 ± 4.2 years. ANCA were positive in 78.6% (54.0% anti-PR3, 18.1% anti-MPO and 27.9% IFI only). The mean Birmingham Vasculitis Activity Score (BVAS) at onset was 10.0 ± 5.6. The main clinical manifestations associated with SGS were crusty rhinitis (100%), sinusitis (78%), pulmonary disease (72.7%), otitis/mastoiditis (50%), glomerulonephritis (42.9%), orbital pseudotumor (28.6%). Six patients (42.9%) received medical treatment only, other six (42.9%) had one to three balloon dilations and two (14.2%) underwent four or more procedures. Eight patients had no SGS relapse (maximum one dilation) and they all received immunosuppression with rituximab (RTX), cyclophosphamide (CYC) or azathioprine (AZA). All patients who received no immunosuppression, methotrexate (MTX) or mycophenolate (MMF) had at least one relapse. Patients treated with MTX or MMF had a mean relapse-free survival of 13.1 months, which was comparable to the one of patients not receiving medical treatment (40.2 months; p=NS) and shorter than the one of patients receiving CYC or RTX (153.2 months; p=0.032). CYC use also inversely correlated with the number of surgical procedures (r=-0.691, p=0.006). Compared to patients without SGS (31 consecutive patients with at least 4 years of follow-up), patients with SGS-GPA had an earlier disease onset (mean age 30.8 vs 50.4 years; p<0.001), but with lower BVAS (mean 10.0 vs 15.3; p=0.013) and showed a higher prevalence of crusty rhinitis (100% vs 67.7%; p=0.019). No difference was observed in damage accrual over time between the two groups.Conclusion:Subglottic stenosis is highly prevalent in patients with GPA and may define a milder disease subset occurring more frequently in younger patients. MTX and MMF might be insufficient to prevent SGS relapses requiring balloon dilation. Aggressive immunosuppression (CYC or RTX) might have a non-redundant role in this setting and reduce the risk of relapses.References:[1]Quinn KA, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019; 58 (12), 2203-2211.[2]Watts R, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-7.Disclosure of Interests:Luca Moroni: None declared, Laura Giudice: None declared, Giuseppe Alvise Ramirez: None declared, Silvia Sartorelli: None declared, adriana cariddi: None declared, Angelo Carretta: None declared, Enrica Bozzolo: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

scholarly journals THU0517 THE ROLE OF ULTRASOUND-DEFINED SYNOVITIS GRADE I IN PATIENTS PRESENTING WITH INFLAMMATORY ARTHRALGIA, A RETROSPECTIVE STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 497.2-497
Author(s):  
J. Arroyo Palomo ◽  
M. Arce Benavente ◽  
C. Pijoan Moratalla ◽  
B. A. Blanco Cáceres ◽  
A. Rodriguez
Keyword(s):  
Rheumatoid Arthritis ◽  
Inflammatory Arthritis ◽  
Statistical Tests ◽  
Median Number ◽  
Painful Joint ◽  
Significant Difference ◽  
The Mean ◽  
Specialized Unit

Background:Musculoeskeletal ultrasound (MSUS) is frequently used in several rheumatology units to detect subclinical inflammation in patients with joint symptoms suspected for progression to inflammatory arthritis (IA). Synovitis grade I (EULAR-OMERACT combined score) is known to be a casual finding in healthy individuals, but studies headed to unravel its possible role on rheumatic diseases are sparse.Objectives:To investigate the correlation between synovitis grade I, and the diagnosis of IA made after a year follow-up period since MSUS findings, in patients of an MSUS-specialized unit of a Rheumatology Department.Methods:We conducted a descriptive, retrospective and unicentric study. 30 patients were selected from the MSUS-specialized unit of our Rheumatology Department from July-18 to January-19. Patients presenting synovitis grade 0 (exclusively), 2 and/or 3 on combined score were excluded. Data collection at baseline included age, sex, immunological profile and previous physical examination to the MSUS findings, as well as the diagnosis made by the rheumatologist in 1-year visit follow-up: dividing the patient sample into two groups: those who were diagnosed with IA and those not. Non-parametric statistical tests for comparing means were used.Results:The mean age was 51,6 years and 70% were females. 6 (20%) patients were diagnosed with inflammatory arthritis after a year follow-up: 2 (4,8%) psoriatic arthritis, 1 (3,3%) undifferentiated arthritis, 1 (3,3%) rheumatoid arthritis, 1 (3,3%) Sjögren’s syndrome. Non-inflammatory arthropathies were also found 24 (80%), of which, 12 (40%) were non-specific arthralgias and 8 (19%) osteoarthritis.In the group of patients who did not developed an IA the mean C-reactive protein (CPR) value was 3,12 mg/L and erythrocyte sedimentation rate (ESR) was 8,2 mm; all of them were rheumatoid factor (RF) positive and ACPA-negative except one patient. 5 (31,3%) patients presented low antinuclear antibodies (ANAs) levels. In those who HLA B-27 and Cw6 were tested (4,25%); both were negative except for one that was HLA B-27 positive. The median number of swollen and painful joint count was 0, and the mean of joints with MSUS involvement was 3,5; the mean involved metacarpophalangeal (MCP) joints was 1,83; proximal interphalangeal (PIP) joints was 1,48 and distal interphalangeal (DIP) joints 0,21.Among the group of patients that developed an IA the mean of CPR and ESR was 9,27 mg/L and 14,17 mm respectively; 2 (33%) patients were RF- positive, and 1 ACPA-positive. ANAs were positive in 3 cases (50%). The median of swollen joint count was 2 and for painful joint count was 0, the median of joints with MSUS involvement was 4,5. The mean of MSUS involvement was for MCP, PIP and DIP joints: 1,67, 2 and 0. Comparing the means of CPR values in the two groups with Student’s t-test we obtained a statistically significant difference (p=0,023). No other significant differences were found.Conclusion:Despite the limitations and possible statistical bias, the presence of MSUS-defined synovitis grade I and elevated CRP levels could be related to further diagnoses of inflammatory arthropathy. Besides, the absence of synovitis in DIP joints might have a diagnostic role. Normal physical exploration and normal levels of CRP might suggest low MSUS value. However, further research is needed to clarify the role of MSUS-defined synovitis grade I.References:[1]D’Agostino MA et al. Scoring ultrasound synovitis in rheumatoid arthritis: a EULAR-OMERACT ultrasound taskforce-Part 1: definition and development of a standardized, consensus-based scoring system. RMD Open. 2017;3(1):e000428.[2]Van den Berg R et al. What is the value of musculoskeletal ultrasound in patients presenting with arthralgia to predict inflammatory arthritis development? A systematic literature review. Arthritis Research & Therapy (2018) 20:228.Disclosure of Interests:None declared

The role of exudation in chronic subdural hematomas

2007 ◽  
Vol 107 (2) ◽  
pp. 290-295 ◽  
Author(s):  
Mehmet Tokmak ◽  
A. Celal Iplikcioglu ◽  
Sirzat Bek ◽  
Cem Atilla Gökduman ◽  
Mustafa Erdal
Keyword(s):  
Outer Membrane ◽  
Clinical Status ◽  
Ct Scans ◽  
Subdural Hematomas ◽  
The Mean ◽  
Grade 3 ◽  
Age Range ◽  
Exudation Rate

Object Chronic subdural hematomas (SDHs) are a local inflammatory process that causes the formation of a granulation tissue often referred to as the external or outer membrane. This membrane has abnormally permeable macrocapillaries. Therefore, exudation from the macrocapillaries in the outer membrane of chronic SDH may play an important role in the enlargement of chronic SDH. In this study the authors investigated the role of exudation in chronic SDH. Methods The authors examined 24 patients (16 men and eight women; age range 38–86 years [mean age 61.4 years]) with 27 chronic SDHs. The clinical status of the patients was evaluated according to the classification described by Markwalder. The diagnosis was established on computed tomography (CT) scans in all cases. The authors also used the Nomura Classification for judging the lesion's appearance on CT scans. Immediately after the diagnosis, all patients were administered 20 mCi (740 mBq) technetium-99m human serum albumin. Four hours later, blood and SDH samples were taken and radioactivity levels were measured in each. The ratio of activity of the samples taken from chronic SDH to the radioactivity of blood was determined as a percentage and defined as the exudation rate. On the follow-up CT scan obtained on postoperative Day 20, subdural collections thicker than 5 mm were determined to be a reaccumulation. Results The correlations between the exudation rate and age of the patients, clinical grades, CT appearances, and amount of reaccumulation were investigated. In this series the average exudation rate was 13.24% (range 2.05–28.88%). The mean exudation rates according to the clinical grades assigned to patients were as follows: Grade 0, 8.67 ± 5.64% (three patients); Grade 1, 5.07 ± 1.43% (eight patients); Grade 2, 17.87 ± 3.73% (seven patients); and Grade 3, 19.65 ± 7.67% (six patients). Exudation rates in patients with Grades 2 and 3 were significantly higher than those in Grades 0 and 1 (p < 0.05). The mean exudation rates according to the lesion's appearance on CT scans were found as follows: hypodense appearance, 6.55 ± 4.52% (eight patients); isodense appearance, 11.07 ± 6.32% (five patients); hyperdense appearance, 19.47 ± 13.61% (three patients); and mixed-density appearance, 17.40 ± 5.80% (nine patients). The differences among the groups were significant (p < 0.05). The average exudation rate was statistically higher in the patients with reaccumulation (16.30 ± 8.16%) than that in the patients without reaccumulation (9.96 ± 6.84%) (p < 0.05). Conclusions The exudation rate in chronic SDH is correlated with a higher clinical grade (Markwalder Grade 2 or 3), mixed-density CT appearance, and reaccumulation. Therefore, exudation from macrocapillaries in the outer membrane of chronic SDH probably plays an important role in the pathophysiology and the growth of chronic SDH.

scholarly journals Bleeding during laparoscopic partial nephrectomy: Can a hemostatic matrix help to improve hemostasis?

2016 ◽  
Vol 88 (3) ◽  
pp. 228 ◽  
Author(s):  
Faruk Ozgor ◽  
Abdulmuttalip Simsek ◽  
Ozgu Aydogdu ◽  
Onur Kucuktopcu ◽  
Omer Sarilar ◽  
...  
Keyword(s):  
Partial Nephrectomy ◽  
Tumor Size ◽  
Operation Time ◽  
Demographic Characteristics ◽  
Ischemia Time ◽  
Nephrometry Score ◽  
The Mean ◽  
Group 1

Objectives: To evaluate the possible role of an hemostatic matrix on hemostasis, perioperative outcomes and complications in patients who underwent laparoscopic partial nephrectomy (LPN). Materials and methods: Patients charts were analyzed retrospectively and their demographic characteristics, operative parameters and follow-up results were recorded. Patients were divided into two groups, according to those who used an hemostatic matrix as Group 1 (n = 41) and those who did not used as Group 2 (n = 44). Demographic characteristics of patients, tumor features, operation time, clamping of the renal vessels, ischemia time, suturing of the collecting system, perioperative hemorrhage and complications were evaluated. Histopathological results, surgical margin status, creatinine level and recurrence at the 3rd month of follow up were analyzed. Statistical analyses were performed with SPSS 17.0 and significance was set at p value of &lt; 0.05. Results: The mean RENAL nephrometry score was 5.9 ± 2.0 and the mean tumor size was 35 ± 12 mm. All patients had a single tumor and 44 of them had a tumor in the right kidney. The renal artery was clamped in 79 cases and the mean ischemia time was 20.1 ± 7 minutes. The mean tumor size and the mean RENAL nephrometry score was statistically higher in Group 1 (p: 0.016 and p &lt; 0.001, respectively). Pelvicaliceal repair was more common in Group 1 due to deeper extension of tumors in this group (p: 0.038). In Group 1, less hemorrhage and blood transfusion requirement, with shorter ischemia and operation time was detected. Conclusion: The outcomes of the recent study showed that adjunctive use of an hemostatic matrix improves hemostasis and decreases hemorrhagic complications during LPN. Further prospective studies are required to assess the potential role of an hemostatic matrix in LPN.

Effects of Orthokeratology Lens On Axial Length Elongation in Myopia With Anisometropia Children

2021 ◽  
Author(s):  
Ziyang Chen ◽  
Kai-Ming Chen ◽  
Ying Shi ◽  
Zhao-Da Ye ◽  
Sheng Chen ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Axial Length ◽  
Statistical Significance ◽  
Spherical Equivalent ◽  
Significant Difference ◽  
The Mean ◽  
The Difference ◽  
Better Than ◽  
Group 1

Abstract AimTo investigate the effect of orthokeratology (OK) lens on axial length (AL) elongation in myopia with anisometropia children.MethodsThirty-seven unilateral myopia (group 1) and fifty-nine bilateral myopia with anisometropia children were involved in this 1-year retrospective study. And bilateral myopia with anisometropia children were divided into group 2A (diopter of the lower SER eye under − 2.00D) and group 2B(diopter of the lower SER eye is equal or greater than − 2.00D). The change in AL were observed.The datas were analysed using SPSS 21.0.Results(1) In group 1, the mean baseline AL of the H eyes and L eye were 24.70 ± 0.89 mm and 23.55 ± 0.69 mm, respectively. In group 2A, the mean baseline AL of the H eyes and L eyes were 24.61 ± 0.84 mm and 24.00 ± 0.70 mm respectively. In group 2B, the mean baseline AL of the H eyes and L eyes were 25.28 ± 0.72 mm and 24.70 ± 0.74 mm. After 1 year, the change in AL of the L eyes was faster than the H eyes in group 1 and group 2A (all P<0.001).While the AL of the H eyes and L eyes had the same increased rate in group 2B. (2) The effect of controlling AL elongation of H eyes is consistent in three groups (P = 0.559).The effect of controlling AL elongation of L eyes in group 2B was better than that in group 1 and group 2A (P < 0.001). And the difference between group 1 and group 2A has no statistical significance. (3) The AL difference in H eyes and L eyes decreased from baseline 1.16 ± 0.55mm to 0.88 ± 0.68mm after 1 year in group 1.And in group 2A, the AL difference in H eyes and L eyes decreased from baseline 0.61 ± 0.34mm to 0.48 ± 0.28mm. There was statistically significant difference (all P<0.001). In group 2B, the baseline AL difference in H eyes and L eyes has no significant difference from that after 1 year (P = 0.069).ConclusionsMonocular OK lens is effective on suppression AL growth of the myopic eyes and reduce anisometropia value in unilateral myopic children. Binocular OK lenses only reduce anisometropia with the diopter of the low eye under − 2.00D. Binocular OK lenses cannot reduce anisometropia with the diopter of the low eye equal or greater than − 2.00D. Whether OK lens can reduce refractive anisometropia value is related to the spherical equivalent refractive of low refractive eye in bilateral myopia with anisometropia children after 1-year follow-up.

A Successful Application of the Intraoperative Parathyroid Hormone Assay in Tertiary Hyperparathyroidism

2007 ◽  
Vol 73 (3) ◽  
pp. 281-283 ◽  
Author(s):  
Leila Thanasoulis ◽  
Juliane Bingener ◽  
Kenneth Sirinek ◽  
Melanie Richards
Keyword(s):  
Parathyroid Hormone ◽  
Success Rates ◽  
Tertiary Hyperparathyroidism ◽  
Equivalent Rate ◽  
Parathyroid Hormone Assay ◽  
The Mean ◽  
Intraoperative Parathyroid Hormone ◽  
Intraoperative Parathyroid Hormone Assay

The role of the intraoperative parathyroid hormone (IOPTH) assay in patients with tertiary hyperparathyroidism (3HPT) is not well defined. To evaluate the utility of the IOPTH in 3HPT, we compared its use in 72 patients with primary hyperparathyroidism (1HPT) and 3HPT undergoing parathyroidectomy. Sixty-three patients with 1HPT and nine patients with 3HPT were identified. There were 30 men and 42 women (mean age, 58 years). The mean serum calcium and preoperative intact PTH levels in 1HPT were 11.1 mg/dL and 214 pg/mL compared with 11.2 mg/dL and 849 pg/mL in 3HPT (Ca, non significant; PTH, P < 0.05). Intraoperatively, a solitary abnormal gland was found in 62 of 72 (86%) patients. Seven patients with 3HPT had three- or four-gland hyperplasia. The two groups were compared to determine if a 10-minute postexcision IOPTH decline >50 per cent would have similar success rates. Seventy-one of 72 (98.6%) patients had a >50 per cent decline from the baseline IOPTH at the end of the operation. The average reduction from baseline was 85.3 per cent in 1HPT and 88.6 per cent in 3HPT (not significant). Average follow-up was 9.8 months for 1HPT and 11.1 months in 3HPT. Three of 63 patients (4.8%) with 1HPT and five of nine patients (55.6%) with 3HPT had inappropriate elevations in PTH ( P < 0.05). All patients with 3HPT were normocalcemic compared with 62 of 64 (97%) patients with 1HPT (not significant). The IOPTH assay can be used in 3HPT in an identical fashion with an equivalent rate of normocalcemia compared with its applications in 1HPT.

Primary Dermal Melanoma (PDM): Histological and Clinical Interdependence to Guide Therapy

2020 ◽  
pp. 120347542095763
Author(s):  
Hadas Zamir ◽  
Meora Feinmesser ◽  
Haim Gutman
Keyword(s):  
Mitotic Rate ◽  
Primary Treatment ◽  
Stage Iii ◽  
Sentinel Nodes ◽  
Time Period ◽  
Nodal Dissection ◽  
The Mean ◽  
Tight Correlation ◽  
Better Than

Background/purpose This study examined clinical and histological parameters of primary dermal melanoma (PDM) to aid in its distinction from dermal metastasis. Methods Retrospective analysis of a prospective cohort of PDM patients. Includes patients fulfilling the strict histologic criteria for PDM ( N = 9) and patients who did not, but clinically, unequivocally had an intradermal melanoma—clinical PDM (cPDM; N = 17). Histopathology slides were re-examined. Prognosticators and outcome measures were compared between groups. Sentinel nodes’ retrieval and wide local excision (WLE) were offered to all patients as primary treatment. Results 26 patients identified, 15 females with a median age of 69 years (range 3.5-85). Mean Breslow was 7.9 ± 5.7 mm (median 5.8, range 1.8-25.0), and the mean mitotic rate was 4.9 ± 3.8/mm2 (median 4.0, range 0-17). Initial treatment and follow-up were as for cutaneous melanoma. One patient in each group with a palpable stage III underwent primary radical dissection. Sentinel nodes were retrieved in all 20 lymphatic mappings performed and found to be metastatic in 5 (25%) patients. Treatment consisted of completion lymph-node dissection. At a median postoperative follow-up of 62 months (range 8-132), 20 patients were disease-free, including 6 of 7 patients with stage III disease at presentation. Six patients died all of cPDM; 5 of 6 patients had primary ulcerated or epidermal-abutting melanomas. Conclusions This is the first study to highlight cPDM. Diagnosis requires expert pathology review and a tight correlation to the clinical parameters. Patients seem to benefit from WLE with sentinel node retrieval and complete dissection when appropriate. However, clinical guidelines for dissection have changed since the time period of this retrospective review. Based on this series, complete nodal dissection in these melanomas is associated with better than expected outcome, for stage III disease.

scholarly journals Development of Algorithm for Clinical Management of Sickle Cell Bone Disease: Evidence for a Role of Vertebral Fractures in Patient Follow-up

2020 ◽  
Vol 9 (5) ◽  
pp. 1601 ◽  
Author(s):  
Lucia De Franceschi ◽  
Daniele Gabbiani ◽  
Andrea Giusti ◽  
Gianluca Forni ◽  
Filippo Stefanoni ◽  
...  
Keyword(s):  
Bone Density ◽  
Sickle Cell ◽  
Bone Disease ◽  
Vertebral Fractures ◽  
Cell Disease ◽  
High Incidence ◽  
The Mean

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T-score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.

scholarly journals The role of radiosurgery for hemangiopericytomas

2003 ◽  
Vol 14 (5) ◽  
pp. 1-5 ◽  
Author(s):  
Steven D. Chang ◽  
Gordon T. Sakamoto
Keyword(s):  
Stereotactic Radiosurgery ◽  
Distant Metastases ◽  
Residual Tumor ◽  
High Rate ◽  
Tumor Control ◽  
Small Subset ◽  
Patient Database ◽  
The Mean

Object Hemangiopericytomas represent a small subset of meningeal tumors. Despite their relatively uncommon nature, they are aggressive tumors known for recurrence. Resection is the standard treatment in most, although regrowth and metastases are common even after resection. The authors evaluate the role of stereotactic radiosurgery in the treatment of recurrent hemangiopericytomas. Methods In a review of the Stanford radiosurgery patient database between 1989 and 2002, the authors found eight patients with recurrent hemangiopericytoma who underwent stereotactic radiosurgery. The mean age of this population was 45.1 years (range 24–67 years). All patients had been previously treated with resection, and five patients (63%) had undergone conventional radiotherapy. The mean radiosurgery dose to the tumor margin was 20.5 Gy (range 16–24 Gy). The mean clinical and radiographic follow-up period was 44 months (range 8–77 months). Of the eight tumors treated with radiosurgery, six decreased in size and two ultimately progressed. There were no radiosurgery-related complications. Conclusions Stereotactic radiosurgery of hemangiopericytomas can result in increased tumor control and should be considered as a treatment option for patients in whom the diagnosis has been established and in whom residual tumor is demonstrated postoperatively. Close clinical and radiographic follow-up evaluation is necessary in this patient population because of the high rate of local recurrence and distant metastases.

Sign in / Sign up

Export Citation Format

Share Document