scholarly journals Diagnostic features of pemphigus vulgaris in patients with pemphigus foliaceus: detection of both autoantibodies, long-term follow-up and treatment responses

2001 ◽  
Vol 125 (3) ◽  
pp. 492-498 ◽  
Author(s):  
N. Sami ◽  
K. C. Bhol ◽  
A. R. Ahmed
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Diagnostic Features ◽  
Long Term Follow Up ◽  
Treatment Responses

scholarly journals IgM Deficiency in Autoimmune Blistering Mucocutaneous Diseases Following Various Treatments: Long Term Follow-Up and Relevant Observations

2021 ◽  
Vol 12 ◽  
Author(s):  
A. Razzaque Ahmed ◽  
Merve Aksoy
Keyword(s):  
Autoimmune Diseases ◽  
Pemphigus Vulgaris ◽  
Mucous Membrane Pemphigoid ◽  
Pemphigus Foliaceus ◽  
Ocular Cicatricial Pemphigoid ◽  
Long Term Follow Up ◽  
Health And Disease ◽  
Igm Deficiency

IgM deficiency has been reported in patients with many autoimmune diseases treated with Rituximab (RTX). It has not been studied, in detail, in autoimmune mucocutaneous blistering diseases (AIMBD). Our objectives were: (i) Examine the dynamics of IgM levels in patients with and without RTX. (ii) Influence of reduced serum IgM levels on clinical and laboratory parameters. (iii) Explore the possible molecular and cellular basis for reduced serum IgM levels. This retrospective study that was conducted in a single-center from 2000 to 2020. Serial IgM levels were studied in 348 patients with five AIMBD (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, and ocular cicatricial pemphigoid) and found decreased in 55 patients treated with RTX, IVIG, and conventional immunosuppressive therapy (CIST). Hence the incidence of decreased serum IgM is low. The incidence of decreased IgM in patients treated with RTX was 19.6%, in patients treated with IVIG and CIST, it was 52.8% amongst the 55 patients. IgM levels in the post-RTX group were statistically significantly different from the IVIG group (p<0.018) and CIST group (p<0.001). There were no statistically significant differences between the groups in other clinical and laboratory measures. Decreased serum IgM did not affect depletion or repopulation of CD19+ B cells. Patients in the three groups achieved clinical and serological remission, in spite of decreased IgM levels. Decrease in IgM was isolated, since IgG and IgA were normal throughout the study period. Decreased IgM persisted at the same level, while the patients were in clinical remission, for several years. In spite of persistent decreased IgM levels, the patients did not develop infections, tumors, other autoimmune diseases, or warrant hospitalization. Studies on IgM deficiency in knockout mice provided valuable insights. There is no universally accepted mechanism that defines decreased IgM levels in AIMBD. The data is complex, multifactorial, sometimes contradictory, and not well understood. Nonetheless, data in this study provides novel information that enhances our understanding of the biology of IgM in health and disease.

Two cases of juvenile pemphigus vulgaris: long-term follow-up

2001 ◽  
Vol 18 (6) ◽  
pp. 541-541 ◽  
Author(s):  
Elisa Pianigiani ◽  
Francesca Ierardi ◽  
Andrea Andreassi ◽  
Michele Fimiani
Keyword(s):  
Pemphigus Vulgaris ◽  
Long Term Follow Up

Long-Term Follow-Up of Dental Rehabilitation of a Patient with Clouston Syndrome and Congenital Edentulism (Issues of Theory and Clinical Practice)

2020 ◽  
Vol 3 (4) ◽  
pp. 128-146
Author(s):  
V.G. Galonsky ◽  
N.V. Tarasova ◽  
V.N. Chernov ◽  
M.Yu. Makarchuk ◽  
A.V. Gradoboev
Keyword(s):  
Clinical Practice ◽  
Dental Treatment ◽  
Long Term Results ◽  
Treatment Results ◽  
Diagnostic Features ◽  
Study Results ◽  
Long Term Follow Up ◽  
Dental Rehabilitation

AbstractThe article presents results of a concise analysis of domestic and foreign literature addressing the problem of determining of distinctive diagnostic features in patients with Clouston syndrome as an orphan disease rarely encountered in clinical practice of dentistry. A clinical case of effective orthopaedic rehabilitation of a 10-years-old patient with Clouston syndrome and congenital edentulism using minimally invasive orthopaedic dentistry measures: removable dentures application using shape memory materials is presented. Technological peculiarities and advantages of the chosen approach to treatment are described. Photographic documents of orthopaedic dental treatment results including long-term results within the follow-up period of 10 years are presented providing strong evidence of effective aesthetic, functional and social rehabilitation of the patient. Detailed analytical conclusion is drawn according to the study results.

scholarly journals Clinical, biochemical and molecular phenotype of congenital disorders of glycosylation: long-term follow-up

2020 ◽  
Author(s):  
Anna Bogdańska ◽  
Patryk Lipiński ◽  
Paulina Szymańska-Rożek ◽  
Aleksandra Jezela-Stanek ◽  
Dariusz Rokicki ◽  
...  
Keyword(s):  
Failure To Thrive ◽  
Congenital Disorders ◽  
Type I ◽  
Congenital Disorders Of Glycosylation ◽  
Diagnostic Features ◽  
Strong Negative Correlation ◽  
Single Centre Study ◽  
Long Term Follow Up

Abstract Background: Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and the attachment of glycans to proteins and lipids. Our study aimed to describe the clinical, biochemical and molecular findings of CDG patients, and to present the long-term follow-up. Material and methods: A single-centre study (1995-2019 years) of patients with congenital disorders of N-glycosylation and combined N- and O-hypoglycosylation, diagnosed based on the serum transferrin (Tf) and apolipoprotein C-III (apoC-III) isoforms analysis, and confirmed molecularly, was performed. Results: Among 32 patients included into the study, 24 had type I Tf isoform profile, in 12 of them deficient PMM2 activity was detected. Three patients were diagnosed with ALG13-CDG; serum Tf isoform profile was normal in one of them, in one other was indicative for type I. Four patients had type II Tf isoform profile. The phenotypic and genotypic spectrum of 32 patients with CDG during long-term (in some cases over 20 years) observation was characterised and several measurements of serum Tf isoforms taken. Statistical analysis revealed strong negative correlation between Asialo-Tf and Tetrasialo-Tf, as well as between Disialo-Tf and Tetrasialo-Tf. Positive correlation was shown between Tetrasialo-Tf and Penasialo-Tf. Within type I CDG, no difference in % Tf isoforms was revealed between PMM2-CDG and non-PMM2-CDG patients. However, these two groups differed significantly in the such diagnostic features as: cerebellar ataxia, failure to thrive, hypothyroidism, pericardial effusion, cardiomyopathy, inverted nipples, prolonged INR. The effect of treatment with mannose in 2 patients with MPI-CDG were assesed and we found that % of Asialo-Tf, Monosialo-Tf, and Disialo-Tf was significanty lowered, whereas Tetrasialo- Tf and Pentasialo-Tf rose, coming closer or falling into the reference range. Conclusions: The novel finding was an abnormal Tf IEF pattern in two ALG13-CDG patients and normal in one ALG1-CDG patient. Clinical manifestation of presented CDG patients was similar to that reported in the literature. Mannose supplementation in MPI-CDG patients, as well as galactose supplementation in PGM-CDG patient improved patients’ clinical picture and Tf isoform profiles.

Rituximab in Childhood Pemphigus Vulgaris: A Long-Term Follow-Up Case and Review of the Literature

Dermatology ◽  
2010 ◽  
Vol 221 (1) ◽  
pp. 13-16 ◽  
Author(s):  
Irene Fuertes ◽  
Antonio Guilabert ◽  
Jose Manuel Mascaró, Jr. ◽  
Pilar Iranzo
Keyword(s):  
Pemphigus Vulgaris ◽  
Review Of The Literature ◽  
Long Term Follow Up

scholarly journals Lack of long-term follow-up after paediatric-adult transition in coeliac disease is not associated with complications, ongoing symptoms or dietary adherence

2020 ◽  
Vol 8 (2) ◽  
pp. 157-166
Author(s):  
Laura Kivelä ◽  
Sointu Hekkala ◽  
Heini Huhtala ◽  
Katri Kaukinen ◽  
Kalle Kurppa
Keyword(s):  
Coeliac Disease ◽  
Term Treatment ◽  
Dietary Adherence ◽  
Diagnostic Features ◽  
Adult Transition ◽  
Unsuccessful Treatment ◽  
Long Term Treatment ◽  
Long Term Follow Up

Background Follow-up of coeliac disease is recommended to prevent complications associated with unsuccessful treatment. Objective The objective of this article is to evaluate the implementation and significance of long-term follow-up. Methods Medical data were collected from 585 and follow-up questionnaires sent to 559 current adult coeliac disease patients diagnosed in childhood. Diagnostic features and adulthood health outcomes were compared between those with and without adulthood follow-up. Results Of paediatric patients, 92% were followed up 6–24 months after diagnosis. A total of 235 adults responded to the questionnaires a median of 18 years after diagnosis, and 25% of them reported regular follow-up. They were diagnosed more recently than those without follow-up (median year 2001 vs 1995, p = 0.001), being otherwise comparable at diagnosis. Those with follow-up were less often smokers (5% vs 16%, p = 0.042) and relatives of coeliac patients (48% vs 66%, p = 0.018), and more often students (48% vs 28%, p = 0.005) and type 1 diabetics (19% vs 4%, p = 0.001). Lack of follow-up was not associated with complications, ongoing symptoms, poorer general health or dietary adherence. All completely non-adherent patients were without follow-up. Conclusions Most coeliac disease patients diagnosed in childhood were not followed up according to recommendations in adulthood. The individual effect of this on long-term treatment outcomes varied markedly.

scholarly journals Clinical, biochemical and molecular phenotype of congenital disorders of glycosylation: long-term follow-up

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Anna Bogdańska ◽  
Patryk Lipiński ◽  
Paulina Szymańska-Rożek ◽  
Aleksandra Jezela-Stanek ◽  
Dariusz Rokicki ◽  
...  
Keyword(s):  
Failure To Thrive ◽  
Congenital Disorders ◽  
Type I ◽  
Congenital Disorders Of Glycosylation ◽  
Diagnostic Features ◽  
Strong Negative Correlation ◽  
Single Center Study ◽  
Long Term Follow Up

Abstract Background Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and the attachment of glycans to proteins and lipids. Our study aimed to describe the clinical, biochemical, and molecular findings of CDG patients, and to present the long-term follow-up. Material and methods A single-center study (1995–2019 years) of patients with congenital disorders of N-glycosylation and combined N- and O-hypoglycosylation was performed. Results Among 32 patients included into the study, there were 12 PMM2-CDG, 3 ALG13-CDG, 3 ALG1-CDG, 1 ALG3-CDG, 3 MPI-CDG, 1 PGM1-CDG, 4 SRD5A3-CDG, 1 DPAGT1-CDG, 3 ATP6AP1-CDG, 1 ATP6V0A2-CDG. The phenotypic and genotypic spectrum during long-term (in some cases over 20 years) observation was characterised and several measurements of serum Tf isoforms taken. Statistical analysis revealed strong negative correlation between asialo-Tf and tetrasialo-Tf, as well as between disialo-Tf and tetrasialo-Tf. Within CDG type I, no difference in % Tf isoforms was revealed between PMM2-CDG and non-PMM2-CDG patients. However, these two groups differed significantly in such diagnostic features as: cerebellar ataxia, failure to thrive, hypothyroidism, pericardial effusion, cardiomyopathy, inverted nipples, prolonged INR. The effect of treatment with mannose in 2 patients with MPI-CDG was assessed and we found that % of asialo-Tf, monosialo-Tf, and disialo-Tf was significantly lowered, whereas tetrasialo-Tf and pentasialo-Tf rose, coming closer or falling into the reference range. Conclusions The novel finding was an abnormal Tf IEF pattern in two ALG13-CDG patients and normal in one ALG1-CDG patient. Clinical manifestation of presented CDG patients was similar to that reported in the literature. Mannose supplementation in MPI-CDG patients, as well as galactose supplementation in PGM1-CDG patient, improved patients’ clinical picture and Tf isoform profiles.

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