scholarly journals Relentless Placoid Chorioretinitis: A Case Series of Successful Tapering of Systemic Immunosuppressants Achieved with Adalimumab

2019 ◽  
Vol 10 (1) ◽  
pp. 145-152 ◽  
Author(s):  
Shotaro Asano ◽  
Rie Tanaka ◽  
Hidetoshi Kawashima ◽  
Toshikatsu Kaburaki
Keyword(s):  
Immunosuppressive Agents ◽  
Case Series ◽  
Clinical Findings ◽  
Posterior Pole ◽  
Peripheral Retina ◽  
Conjunctival Hyperemia ◽  
Systemic Corticosteroids ◽  
First Case ◽  
Adalimumab Therapy ◽  
Relentless Placoid Chorioretinitis

Background: Adalimumab, a human anti-tumor necrosis factor-ɑ monoclonal antibody, was recently reported to be effective in lowering the risk of recurrence of noninfectious uveitis. This is the first case series of adalimumab administrations for relentless placoid chorioretinitis (RPC) patients. Case Presentation: We report 2 cases of RPC where successful treatments were achieved with adalimumab. A 34-year-old woman developed conjunctival hyperemia, mild iridocyclitis, and multiple atrophic retinal lesions, along with exudative changes that were widespread from the posterior pole to peripheral retina in both eyes. The diagnosis of RPC was made based on the characteristic recurrences of choroiditis despite systemic corticosteroid and cyclosporine. Adalimumab therapy was introduced to the patient, and thereafter no recurrence was observed while tapering the immunosuppressive agents. The second case was a 22-year-old man with visual deterioration in both eyes who exhibited widespread multiple chorioretinal atrophic lesions. We diagnosed the case as RPC based on characteristic clinical findings and recurring chorioretinitis during tapering of systemic corticosteroids. Adalimumab therapy was administrated, and immunosuppressant dosage was successfully reduced without any recurrences. Conclusions: In the current two RPC cases, adalimumab was quite effective and useful to reduce the dosages of systemic immunosuppressants. Further study is necessary to confirm the effectiveness of adalimumab in RPC patients.

scholarly journals Case Series : Operative vs Non-Operative Management in Grade III Pancreatic Injury

Medicinus ◽  
2020 ◽  
Vol 7 (6) ◽  
pp. 199
Author(s):  
Andry Irawan ◽  
Clinton Clinton ◽  
Sutanto D ◽  
Agustina F
Keyword(s):  
Case Series ◽  
Operative Management ◽  
Pancreatic Injury ◽  
Clinical Findings ◽  
First Case ◽  
Non Operative Management ◽  
Left Abdomen ◽  
Grade Iii ◽  
Selection Of

<p><strong>Introduction</strong><strong>:</strong> Pancreatic injury is a rare case, caused by blunt or sharp trauma. Difficulty in making diagnose on pancreatic trauma cases are associated with high mortality, and the treatment can be either operative or conservatively. However, It is still unclear which treatment is more favorable.</p><p><strong>Case:</strong> We present 2 cases of Grade III pancreatic injury with stable hemodynamic who suffered bicycle accident. First case, 12-year-old boy complaining severe pain on the upper left abdomen (VAS 9-10) and get worsening by time, with vomiting. The patient underwent distal Pancreatectomy-Splenectomy. Second case, 8-year-old boy complaining of pain on the upper left abdomen (VAS 6-7) without extension on whole abdominal region with vomiting and fever. The patient was treated conservatively. In both cases, patient was discharged with improvement. However, about 3 months later patients who were treated conservatively developed into a pseudocyst.</p><p><strong>Conclusion:</strong> The selection of management in grade III pancreatic injury can be operative or conservative depending on clinical findings such as hemodynamic condition and the quality of abdominal pain. But the occurrence of pseudocysts pancreas is another surgical challenge.</p>

Clinical findings in five Turkish patients with citrin deficiency and identification of a novel mutation on SLC25A13

2020 ◽  
Vol 33 (1) ◽  
pp. 157-163 ◽  
Author(s):  
Melis Demir Köse ◽  
Mehtap Kagnici ◽  
Taha Reşit Özdemir ◽  
Cahit Barış Erdur ◽  
Gülin Erdemir ◽  
...  
Keyword(s):  
Novel Mutation ◽  
Genetic Disorder ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Case Series ◽  
Clinical Findings ◽  
First Case ◽  
Age Related ◽  
Citrin Deficiency ◽  
Turkish Patients

AbstractBackgroundCitrin deficiency (CD) is an autosomal recessive genetic disorder caused by a defect in the mitochondrial aspartate/glutamate antiporter, citrin. Three clinical manifestations have been described until today.Case presentationWe reported 5 CD patients from two families. Four patients were male and one patient was female. Two of them have NICCD (neonatal intrahepatic cholestasis caused by citrin deficiency); three of them have CTLN2 (adult-onset type II citrullinemia). Both NICCD patients showed typical clinical and biochemical changes with a diagnosis confirmed by mutations in the SLC25A13 gene. We detected a previously unreported homozygous novel mutation c.478delC (L160Wfs*36 ) on the SLC25A13 gene. All of the CTLN2 patients were siblings. Proband was a 15-year-old mentally retarded and autistic male who had admitted to our emergency with disorientation. Laboratory data showed hyperammonemia and citrullinemia.ConclusionsTwo different profiles of age-related CD have been depicted with this article. It has been aimed to underline that the CD can be observed in different forms not only in neonatals or little infants but also in adolescents. This article is the first case series that covers both NICCD and CTLN2 cases together and that has been published in Turkey. Considering the fact that especially the majority of CTLN2 cases have been identified in Asian countries, our article has vital importance in terms of defining phenotypic features of the disease.

Therapeutic options for cutaneous polyarteritis nodosa: a systematic review

Rheumatology ◽  
2021 ◽  
Author(s):  
Eleni Papachristodoulou ◽  
Loukas Kakoullis ◽  
Eleni Tiniakou ◽  
Konstantinos Parperis
Keyword(s):  
Systematic Review ◽  
Polyarteritis Nodosa ◽  
Immunosuppressive Agents ◽  
Case Series ◽  
Treatment Modalities ◽  
Induction Treatment ◽  
Cutaneous Polyarteritis Nodosa ◽  
Systemic Corticosteroids ◽  
Increased Risk ◽  
Effect Of Treatment

Abstract Objective Cutaneous polyarteritis nodosa (CPAN) is a necrotizing vasculitis of the middle-size vessels, confined to the skin. We conducted a systematic review in order to identify studies evaluating the different treatment modalities used in CPAN. Methods This systematic review was conducted according to PRISMA guidelines, registered in PROSPERO: CRD42020222195. PubMed/Medline databases were searched from inception to December of 2020 using the terms: “Polyarteritis nodosa[Title/Abstract]) AND ((therapy[Title/Abstract]) OR (management[Title/Abstract]) OR (treatment[Title/Abstract]))” and “Cutaneous arteritis [Title/Abstract]”. Articles evaluating pertaining to the management of CPAN in adults were eligible for inclusion. Results A total of 7 eligible case series with 325 unique patients were included. No study included a control population. In general, systemic corticosteroids were widely used as induction treatment. Immunosuppressive agents combined with corticosteroids were azathioprine, hydroxychloroquine, sulfasalazine, sulphapyridine, cyclophosphamide, methotrexate, mycophenolate, tacrolimus, rituximab, and thalidomide. Other agents utilized in the studies were dapsone, colchicine, non-steroid anti-inflammatory drugs, salicylates, warfarin and clopidogrel. In some studies, the presence of ulcerations was associated with an increased risk of relapse. Conclusion The evidence available regarding the management of patients with CPAN is limited at best. Further studies are needed in order to evaluate the effect of treatment on disease remission, relapses, and mortality.

scholarly journals Sodium-Glucose Cotransporter-2 Inhibitors in Patients with Hereditary Podocytopathies, Alport Syndrome, and FSGS: A Case Series to Better Plan a Large-Scale Study

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1815
Author(s):  
Jan Boeckhaus ◽  
Oliver Gross
Keyword(s):  
Glomerular Filtration ◽  
Alport Syndrome ◽  
Large Scale ◽  
Case Series ◽  
Hereditary Diseases ◽  
Glomerular Filtration Barrier ◽  
Early Effect ◽  
Filtration Barrier ◽  
First Case ◽  
Sodium Glucose Cotransporter

Hereditary diseases of the glomerular filtration barrier are characterized by a more vulnerable glomerular basement membrane and dysfunctional podocytes. Recent clinical trials have demonstrated the nephroprotective effect of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in chronic kidney disease (CKD). SGLT2-mediated afferent arteriole vasoconstriction is hypothesized to correct the hemodynamic overload of the glomerular filtration barrier in hereditary podocytopathies. To test this hypothesis, we report data in a case series of patients with Alport syndrome and focal segmental glomerulosclerosis (FSGS) with respect of the early effect of SGLT2i on the kidney function. Mean duration of treatment was 4.5 (±2.9) months. Mean serum creatinine before and after SGLT-2i initiation was 1.46 (±0.42) and 1.58 (±0.55) mg/dL, respectively, with a median estimated glomerular filtration rate of 64 (±27) before and 64 (±32) mL/min/1.73 m2 after initiation of SGLT2i. Mean urinary albumin-creatinine ratio in mg/g creatinine before SGLT-2i initiation was 1827 (±1560) and decreased by almost 40% to 1127 (±854) after SGLT2i initiation. To our knowledge, this is the first case series on the effect and safety of SGLT2i in patients with hereditary podocytopathies. Specific large-scale trials in podocytopathies are needed to confirm our findings in this population with a tremendous unmet medical need for more effective, early on, and safe nephroprotective therapies.

scholarly journals Ocular adverse events associated with immune checkpoint inhibitors: a novel multidisciplinary management algorithm

2021 ◽  
Vol 13 ◽  
pp. 175883592199298
Author(s):  
Orthi Shahzad ◽  
Nicola Thompson ◽  
Gerry Clare ◽  
Sarah Welsh ◽  
Erika Damato ◽  
...  
Keyword(s):  
Adverse Events ◽  
Checkpoint Inhibitors ◽  
Case Reports ◽  
Case Series ◽  
Simple Algorithm ◽  
Cancer Therapeutics ◽  
Management Algorithm ◽  
Ocular Symptoms ◽  
Systemic Corticosteroids ◽  
Permanent Loss

Ocular immune-related adverse events (IrAEs) associated with use of checkpoint inhibitors (CPIs) in cancer therapeutics are relatively rare, occurring in approximately 1% of treated patients. Recognition and early intervention are essential because the degree of tissue damage may be disproportionate to the symptoms, and lack of appropriate treatment risks permanent loss of vision. International guidelines on managing ocular IrAEs provide limited advice only. Importantly, local interventions can be effective and may avoid the need for systemic corticosteroids, thereby permitting the continuation of CPIs. We present a single institution case series of eight affected patients managed by our multidisciplinary team. Consistent with previously published series and case reports, we identified anterior uveitis as the most common ocular IrAE associated with CPIs requiring intervention. Based on our experience, as well as published guidance, we generated a simple algorithm to assist clinicians efficiently manage patients developing ocular symptoms during treatment with CPIs. In addition, we make recommendations for optimising treatment of uveitis and address implications for ongoing CPI therapy.

scholarly journals Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid

2021 ◽  
Vol 10 (9) ◽  
pp. 1916
Author(s):  
Ágnes Kinyó ◽  
Anita Hanyecz ◽  
Zsuzsanna Lengyel ◽  
Dalma Várszegi ◽  
Péter Oláh ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Clinical Laboratory ◽  
Case Series ◽  
Dipeptidyl Peptidase ◽  
Area Index ◽  
Histological Features ◽  
Dipeptidyl Peptidase 4 ◽  
First Case ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
The Mean

Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our aim was to assess DPP4i-induced bullous pemphigoid among our BP patients and characterize the clinical, laboratory and histological features of this drug-induced disease form. In our patient cohort, out of 127 BP patients (79 females (62.2%), 48 males (37.7%)), 14 (9 females and 5 males) were treated with DPP4i at the time of BP diagnosis. The Bullous Pemphigoid Disease Area Index (BPDAI) urticaria/erythema score was significantly lower, and the BPDAI damage score was significantly higher in DPP4i-BP patients compared to the nonDPP4i group. Both the mean absolute eosinophil number and the mean periblister eosinophil number was significantly lower in DPP4i-BP patients than in nonDPP4i cases (317.7 ± 0.204 vs. 894.0 ± 1.171 cells/μL, p < 0.0001; 6.75 ± 1.72 vs. 19.09 ± 3.1, p = 0.0012, respectively). Our results provide further evidence that DPP4i-associated BP differs significantly from classical BP, and presents with less distributed skin symptoms, mild erythema, normal or slightly elevated peripheral eosinophil count, and lower titers of BP180 autoantibodies. To our knowledge, this is the first case series of DPP4i-related BP with a non-inflammatory phenotype in European patients.

scholarly journals Atypical Clinical Presentation of Laryngopharyngeal Reflux: A 5-Year Case Series

2021 ◽  
Vol 10 (11) ◽  
pp. 2439
Author(s):  
Jerome R. Lechien ◽  
Stéphane Hans ◽  
Francois Bobin ◽  
Christian Calvo-Henriquez ◽  
Sven Saussez ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Laryngopharyngeal Reflux ◽  
Case Series ◽  
Clinical Findings ◽  
Aerodigestive Tract ◽  
Common Disease ◽  
Upper Aerodigestive Tract ◽  
Suppurative Otitis Media ◽  
Adequate Treatment ◽  
Atypical Clinical Presentation

Background: Laryngopharyngeal reflux (LPR) is a common disease in otolaryngology characterized by an inflammatory reaction of the mucosa of the upper aerodigestive tract caused by digestive refluxate enzymes. LPR has been identified as the etiological or favoring factor of laryngeal, oral, sinonasal, or otological diseases. In this case series, we reported the atypical clinical presentation of LPR in patients presenting in our clinic with reflux. Methods: A retrospective medical chart review of 351 patients with LPR treated in the European Reflux Clinic in Brussels, Poitiers and Paris was performed. In order to be included, patients had to report an atypical clinical presentation of LPR, consisting of symptoms or findings that are not described in the reflux symptom score and reflux sign assessment. The LPR diagnosis was confirmed with a 24 h hypopharyngeal-esophageal impedance pH study, and patients were treated with a combination of diet, proton pump inhibitors, and alginates. The atypical symptoms or findings had to be resolved from pre- to posttreatment. Results: From 2017 to 2021, 21 patients with atypical LPR were treated in our center. The clinical presentation consisted of recurrent aphthosis or burning mouth (N = 9), recurrent burps and abdominal disorders (N = 2), posterior nasal obstruction (N = 2), recurrent acute suppurative otitis media (N = 2), severe vocal fold dysplasia (N = 2), and recurrent acute rhinopharyngitis (N = 1), tearing (N = 1), aspirations (N = 1), or tracheobronchitis (N = 1). Abnormal upper aerodigestive tract reflux events were identified in all of these patients. Atypical clinical findings resolved and did not recur after an adequate antireflux treatment. Conclusion: LPR may present with various clinical presentations, including mouth, eye, tracheobronchial, nasal, or laryngeal findings, which may all regress with adequate treatment. Future studies are needed to better specify the relationship between LPR and these atypical findings through analyses identifying gastroduodenal enzymes in the inflamed tissue.

Diagnosis and management of hook of hamate fractures

2017 ◽  
Vol 43 (5) ◽  
pp. 539-545 ◽  
Author(s):  
Assaf Kadar ◽  
Allen T. Bishop ◽  
Marissa A. Suchyta ◽  
Steven L. Moran
Keyword(s):  
Case Series ◽  
Clinical Results ◽  
Clinical Findings ◽  
Advanced Imaging ◽  
Level Of Evidence ◽  
Historical Case ◽  
Treatment Groups ◽  
Diagnosis And Management ◽  
Time To Diagnosis ◽  
Hook Of Hamate

The purpose of this study was to evaluate the time to diagnosis and management of hook of hamate fractures in an era of advanced imaging. We performed a retrospective study of 51 patients treated for hook of hamate fractures. Patients were sent a quickDASH questionnaire regarding the outcomes of their treatment. Hook of hamate fractures were diagnosed with advanced imaging at a median of 27 days. Clinical findings of hook of hamate tenderness had better sensitivity than carpal tunnel-view radiographs. Nonunion occurred in 24% of patients with non-operative treatment and did not occur in the operative group. Both treatment groups achieved good clinical results, with a grip strength of 80% compared with the non-injured hand and a median quickDASH score of 2. Advanced imaging improved the time to diagnosis and treatment compared to historical case series. Nonunion is common in patients treated non-operatively. Level of evidence: IV

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