scholarly journals Presenting IgG4-related disease: an emerging entity in Pakistan

2021 ◽  
Author(s):  
Lena Jafri ◽  
Sadori Khawaja ◽  
Bilal Hashmi ◽  
Saira Fatima ◽  
Om Parkash ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Tertiary Care ◽  
The Body ◽  
Clinical Feature ◽  
Care Hospital ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Group I ◽  
Serum Igg4 ◽  
Group Ii

Abstract Background IgG4 related disease (IgG4-RD) is a spectrum of immune mediated disorder involving various organs of the body. In this study the clinical spectrum of possible IgG4-RD was explored. Methods Subjects tested for serum IgG4 and all biopsies of subjects with suspected IgG4-RD received at the clinical laboratory of a tertiary care hospital from April 2015 to December 2019 were included. Medical charts of subjects registered were reviewed and telephonic interviews were conducted. Subjects were divided into two groups: group I had biochemical evidence of IgG4-RD while group II had histopathological evidence of IgG4-RD. “Comprehensive diagnostic criteria for IgG4-RD, 2011” was used for labeling patients as possible, probable and definitive IgG4-RD. Results A total of 177 study subjects were recruited in the current study. Group I included 10 children and 105 adults whereas group II had 5 children and 57 adults. Out of the total 177 subjects definitive, probable and possible IgG4-RD were seen in (n = 2, 1.1%), (n = 61, 34.4%) and (n = 114, 64.4) subjects respectively. The commonest organs involved in all the study subjects were pancreas (57.6%), submandibular gland (12.4%) and liver (6.2%). Conclusion The clinical feature of IgG4-RD include single or multiple organ involvement with pancreas being the most frequently affected organ in the current population. Amalgamation of clinical, biochemical and histopathological findings are essential for the IgG4-RD, although none is pathognomonic by itself.

Thoracic Involvement in IgG4-Related Disease

2020 ◽  
Vol 41 (02) ◽  
pp. 202-213 ◽  
Author(s):  
Marta Casal Moura ◽  
Ria Gripaldo ◽  
Misbah Baqir ◽  
Jay H. Ryu
Keyword(s):  
Clinical Laboratory ◽  
Wide Spectrum ◽  
The Body ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
First Line Therapy ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Histopathologic Features

AbstractImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, but genetic factors, microorganisms, and autoimmunity are thought to play important roles. Serum IgG4 concentration is elevated in the majority of patients with IgG4-RD but is a nonspecific finding. Characteristic histopathologic features include dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis. Lung involvement in IgG4-RD was first reported in 2004 in two patients with AIP and coexisting interstitial lung disease. Since then, a wide spectrum of intrathoracic involvement has been reported and includes not only parenchymal lung diseases but also pleural, airway, vascular, and mediastinal lesions. Thoracic involvement in IgG4-RD is often found incidentally during the workup of extrathoracic lesions but can sometimes be the presenting abnormality. The diagnosis of IgG4-RD requires correlation of clinical, laboratory, imaging, and histopathologic features. Glucocorticoids are the first-line therapy but other options including B cell depletion are being investigated. IgG4-RD is generally associated with an indolent clinical course and most patients improve with glucocorticoid therapy.

scholarly journals Transport Factors Affecting the Outcome of Referred Neonates Admitted in A Tertiary Care Hospital

2018 ◽  
Vol 41 (3) ◽  
pp. 159-164 ◽  
Author(s):  
Madhabi Baidya ◽  
Mahfuza Shirin ◽  
Liton Chandra Saha
Keyword(s):  
Perinatal Asphyxia ◽  
Care Center ◽  
Tertiary Care ◽  
Preterm Neonates ◽  
P Value ◽  
Care Hospital ◽  
Cross Sectional ◽  
Neonatal Transport ◽  
Group I ◽  
Group Ii

Background: Adequate neonatal transport is a key component of care of the sick newborns who require referral to tertiary care center. Poor transportation is one of the iatrogenic factors associated with greater neonatal mortality. Neonatal transport is the greatest challenge faced today in our country. The purpose of this study was to find out characteristics of transport of referred neonates and to idention the factors that contribute to mortality.Methodology: This cross sectional study was conducted in Dhaka Shishu (Children) Hospital from June 2013 to November 2013. Both term and preterm neonates who were referred within first seven days of life were included and those with gross congenital abnormalities and left against medical advice were excluded from the study. After enrollment, data were collected using a structured questionnaire including birth details, interventions before transportation, reasons for referral, and details of transportation. Outcome & duration of hospital stay were also recorded. Neonates who were expired considered as group I and who were survived considered as group II. The study variables were analyzed for their association with immediate outcome by applying chi square test and t test. P value <0.05 was considered significant.Results: This study found that out of 332 neonates 181 were expired with 54.5% mortality rate. One eighty one neonates who were expired, considered as group I and one fifty one neonate were survived, considered as group II. The mortality was significantly high in male neonates [RR 0.80 (0.66-0.97)] and neonates those delivered at home [RR 1.34(1.10-1.64)] (p<0.05). Perinatal asphyxia, pre-term low birth weight, neonatal sepsis were the main causes of referral. It was found that transportation without any referral note [RR 1.40 (1.14- 1.71)], no advice regarding maintenance of airway[RR 1.50(1.17- 1.92)]and keeping warm [RR 1.51(1.17-1.950], resuscitation on admission [RR 1.63(1.23-2.17)] and transportation required > 3hours [RR 1.36(1.09-1.69)] were associated with significantly higher mortality among referred transported neonates(p<0.05).Conclusions: This study found that male neonates, home delivery, transportation without any referral note, no advice regarding maintenance of airway and keeping warm, resuscitation needed on admission and prolonged transportation time were significantly associated with mortality of referred transported neonates.Bangladesh J Child Health 2017; VOL 41 (3) :159-164

scholarly journals Prevalence of anaemia among children under five years in tertiary care hospital of Nepal.

2015 ◽  
Vol 13 (1) ◽  
pp. 33-36 ◽  
Author(s):  
Sundari Joshi ◽  
Megha Pradhan Pradhan ◽  
Ujjwal Joshi
Keyword(s):  
Tertiary Care Hospital ◽  
Tertiary Care ◽  
Severe Anaemia ◽  
Care Hospital ◽  
Cross Sectional ◽  
Children Under Five ◽  
Group Iii ◽  
Under Five ◽  
Group I ◽  
Group Ii

Introduction: Anaemia is a pathologic deficiency in oxygen-carrying haemoglobin in red blood cells which may be the result of genetic diseases, infections and deficiency of several nutrients. Nepal is one of the developing countries where anaemia is one of the most serious public health problems. So, a study was planned to evaluate the prevalence of anaemia among children under five years in tertiary care hospital of Nepal. Methods: A cross-sectional study was carried out from August 2011 to January 2012 using a structured questionnaire interview and observation. The children were divided into three groups: group I (0-1 years), group II (>1-3 years), group III (>3-5 years). Anaemia was assessed using  haemoglobin measurement in gram in decilitre on Sysmex KX -21 (automated haematology analyzer). The severity of anaemia were grouped as follows: severe anaemia, < 7.0 g/dL; moderate anaemia, 7.0 to 8.9 g/dL; and mild anaemia, 9.0 to 10.9 g/dL as per WHO classification. Stool test was also carried out to assess worm infestation. Data were analysed using SPSS 11.5. Results: There were 208 children among which 52.9 % were male while 47.10 % were female.  The overall prevalence of anaemia was found to be 49.5% of which 43.3% had mild, 15.8% had moderate and 0.5% had severe anaemia. Out of 20 children in group I, 70 % were anaemic. Among the 94 each in group II and group III, children suffering from anaemia were 51% and 43% respectively. The stool investigations showed that 5.3 % children suffered from worm infestations.   Conclusions: prevalence of anaemia was seen in 49.5% of children below five years age group and the diet and worm infestations didn’t affect it.doi:  http://dx.doi.org/10.3126/mjsbh.v13i1.12998

scholarly journals A randomised trial of 5% minoxidil versus combination of 5% minoxidil and oral spironolactone in treatment of female pattern hair loss

2019 ◽  
Vol 5 (4) ◽  
pp. 668
Author(s):  
Nachiket Madhukarrao Palaskar ◽  
Nitin Dinkar Chaudhari ◽  
Garima Laxminarayan Balpande ◽  
Swapna Subhash Khatu
Keyword(s):  
Combination Therapy ◽  
Hair Loss ◽  
Tertiary Care ◽  
Randomised Trial ◽  
Life Questionnaire ◽  
Care Hospital ◽  
Female Pattern Hair Loss ◽  
Group I ◽  
Topical Minoxidil ◽  
Group Ii

<p class="abstract"><strong>Background:</strong> Female pattern hair loss (FPHL) is a common form of nonscarring hair loss. We compared the usefulness and safety of topical minoxidil alone with combination of oral spironolactone and topical minoxidil in the treatment of FPHL.</p><p class="abstract"><strong>Methods:</strong> This prospective, single-centre, randomised open label study over 100 patients attending tertiary care hospital in Mumbai during period December 2011 to June 2012. The data were entered into SPSS version 21 for analysis. Data collected were coded and described as frequency and percentage for qualitative data and means and standard deviation for quantitative data. Statistical analysis was done using chi-square and student t test. Statistical significance was considered if p value was less than 0.05.<strong></strong></p><p class="abstract"><strong>Results:</strong> There were 48 patients in Group I and 46 patients in Group II. At 6 months, significantly higher mean Sinclair grade was observed among Group I patients as compared to Group II patients (2.85±0.68 vs 2.56±0.50, p=0.02). We observed a significant improvement in women’s androgenetic alopecia quality of life questionnaire in Group I patients at 12 months after treatment (26.93±2.25 vs 23.47±2.95, p&lt;0.001). Minoxidil and spironolactone were tolerated well by the patients.</p><p class="abstract"><strong>Conclusions:</strong> Combination therapy of topical minoxidil and oral spironolactone has an additive effect. However, plateau of effectiveness of the combination therapy in normoandrogenic patients at 6 months of therapy was observed. We recommend the combination for 6 months and continuation of therapy with minoxidil only.</p>

scholarly journals Clinical Manifestations of Orbital Involvement in IgG4-Related Disease: A Retrospective Study of 573 Patients

2021 ◽  
Author(s):  
Linyang Gan ◽  
Xuan Luo ◽  
Yunyun Fei ◽  
Linyi Peng ◽  
Jiaxin Zhou ◽  
...  
Keyword(s):  
Lacrimal Gland ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Chinese Patients ◽  
Laboratory Findings ◽  
Male Predominance ◽  
Orbital Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

Abstract Purpose: To investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD). Methods: A total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.Results: Male predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.Conclusions: Lacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.

scholarly journals A comparative study of tapentadol versus tramadol in the treatment of low back pain

2020 ◽  
Vol 9 (7) ◽  
pp. 1064
Author(s):  
Laxman Verma ◽  
Pankaj Kumar Chaudhary ◽  
Chandresh Gupta ◽  
Umesh Saroj
Keyword(s):  
Pain Intensity ◽  
Tertiary Care ◽  
Care Hospital ◽  
Medical Assessment ◽  
Group I ◽  
Parallel Group ◽  
Reduction Of Pain ◽  
The Mean ◽  
Group Ii ◽  
The Difference

Background: Nontraumatic musculoskeletal disorders are the main reason for presentation to the emergency department with Rachialgia being the most common reason to request medical assessment among them. Methods: This was a prospective, randomized, parallel group open labelled study conducted in a district level tertiary care hospital attached to a medical teaching institute. Patients were randomized into two groups. Group I (63 patients) received tablet tramadol 50 mg twice daily orally and group II (63 patients) received tablet tapentadol 50 mg twice daily orally.Results: The mean age of the patients of group I was 40.6±9.6 years and in the group II was 42.7±10.6 years. A total of 61 males participated in the study of which 31 males were enrolled in group I and 30 in group II while 65 females participated in the study of which 32 females were enrolled in group I and 33 in group II. The mean reduction of pain intensity VAS score at the end of 4 weeks from baseline in group I and group II were 34.57 and 37.55 respectively. The difference in the mean reduction of pain intensity VAS between the two groups was not statistically significant.Conclusions: We conclude that both the drugs show significant reduction in the pain intensity in moderate to severe CLBP patients. Tapentadol is as efficacious as tramadol in moderate to severe CLBP. However, tapentadol is better tolerated than tramadol.

scholarly journals Effect of Povidone Iodine Versus Chlorhexidine Mouthwash on Oral Mucositis among Cancer Patients

2021 ◽  
Author(s):  
. Jyothi ◽  
S Latha ◽  
K Pavithra ◽  
M Nalini ◽  
Sowmya J Rao ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Cancer Patients ◽  
Oral Mucositis ◽  
Povidone Iodine ◽  
Tertiary Care ◽  
World Health ◽  
Care Hospital ◽  
Group I ◽  
Grading Scale ◽  
Group Ii

Introduction: Radiation therapy and chemotherapy are the standard treatment given for cancer, which leads to the variety of adverse effects of which Oral Mucositis (OM) is one of the common side-effects. It is responsible for patient discomfort and decreases their level of functioning. Both Chlorhexidine and Povidone Iodine have got antimicrobial and antifungal activity which decreases the severity of mucositis. Aim: To compare the effectiveness of Povidone Iodine and Chlorhexidine mouthwash on OM among cancer patients undergoing radiation therapy or chemotherapy. Materials and Methods: A prospective observational study was conducted in the tertiary care hospital of Mangaluru, Karnataka, India for the duration of one year and three months from December 2015-March 2017. Fifty cancer subjects aged between 25 to 65 years and who developed OM after radiation therapy or chemotherapy were selected by purposive sampling technique. Data were collected using the demographic profile, clinical proforma and World Health Organisation (WHO) OM grading scale (2004) from 19.09.2016 to 17.12.2016. Experimental group I received 10 mL of diluted Povidone Iodine mouthwash and group II received 10 mL of diluted chlorhexidine mouthwash. Level of OM in the group I and group II were assessed on the 1st, 3rd, 5th and 7th day using WHO OM grading scale (2004). The data were analysed by descriptive and inferential statistics (Wilcoxon signed- rank test, Mann-Whitney U test, Repeated measures ANOVA) using SPSS version 16.0. Results: Among the 50 cancer subjects, majority 30 (60%) were in the age group between 55-64 years and majority 35 (70%) were receiving radiation therapy. On day seven, in the group I (Povidone Iodine) majority 14 (56%) subjects had mild level of mucositis whereas in the group II (Chlorhexidine) majority 14 (56%) subjects had moderate mucositis. Comparison of the effect of Povidone Iodine and Chlorhexidine mouthwash using Friedman’s ANOVA showed that there was a difference in the level of mucositis (p<0.05) at 5% level of significance among two groups. The study findings also revealed a difference in the level of mucositis between day 1 to day 3, 5 and 7 (Mann-Whitney U test) (p<0.05) in both the groups. Conclusion: Povidone Iodine mouth wash was more effective than Chlorhexidine mouthwashes in reducing OM, and the patients were more comfortable after the use of the mouthwash.

Clinical case of membranous nephropathy in a patient with IgG4-related disease

2020 ◽  
Vol 24 (4) ◽  
pp. 110-121
Author(s):  
M. O. Pyatchenkov ◽  
O. A. Vorobyeva ◽  
A. N. Belskykh ◽  
M. V. Zakharov ◽  
M. Y. Dendrikova
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
The Body ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pathologic Features ◽  
Wide Range ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

Livedo Racemosa: Clinical, Laboratory, and Histopathological Findings in 33 Patients

2020 ◽  
pp. 153473461989693
Author(s):  
Marcella Soares Pincelli ◽  
Alejandra Maria Jimenez Echavarria ◽  
Paulo Ricardo Criado ◽  
Gabriela Franco Marques ◽  
Thâmara Cristiane Alves Batista Morita ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Clinical Laboratory ◽  
Tertiary Care ◽  
Clinical Findings ◽  
Lower Limbs ◽  
Clinical Feature ◽  
Care Hospital ◽  
Livedo Racemosa ◽  
Dermatology Department ◽  
Cutaneous Polyarteritis Nodosa

Livedo racemosa is a cutaneous finding characterized by a persistent, erythematous, or violaceous discoloration of the skin, in a broken, branched, discontinuous, and irregular pattern. A retrospective review of 33 cases with clinical diagnosis of livedo racemosa over the past 6 years was evaluated in the dermatology department of a tertiary care hospital. We found predominance in Caucasian women (78.8%); age ranged from 8 to 81 years, with a mean age of 36 years. Livedo racemosa was described as generalized in 12 patients (36.4%), although the main localization was on lower limbs (42%). After laboratory testing and histopathological examinations, 12 patients (36.4%) were classified with idiopathic livedo racemosa; secondary diseases were diagnosis in 21 patients (63.6%), including Sneddon’s syndrome, cutaneous polyarteritis nodosa, systemic lupus erythematosus, and others. Medical history of thrombotic events was described in 8 (24.2%) patients, and also 8 (24.2%) patients had abnormal results for 2 or more thrombophilia laboratory tests. Skin biopsy showed no histological abnormalities in 11 cases (33.3%), thrombosis of dermal blood vessels in 10 (30.3%), intimal/subintimal thickening in 7 (21.2%), and vasculitis in 5 (15.2%). In conclusion, livedo racemosa is a clinical feature that might be correlated to vascular disorders, such as thrombotic and/or hypercoagulable states, autoimmune diseases, and neoplastic diseases, or it can be secondary to specific medications. It is essential to establish a correct approach in cases of livedo racemosa, which includes anamnesis, physical examination, laboratory test, histological examination, and complementary examination according to clinical findings, in order to diagnosis underlying causes.

scholarly journals A Study to Evaluate Use of Platelet Indices in Hyperdestructive Thrombocytopenia: A Two-year Experience from Tertiary Care Rural Hospital

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Roniya Francis ◽  
Shruthi N Shetageri ◽  
Roopa A N ◽  
S R Raja Parthiban
Keyword(s):  
Tertiary Care ◽  
Study Groups ◽  
The Body ◽  
Parasitic Infections ◽  
Control Group ◽  
Platelet Destruction ◽  
Distribution Width ◽  
Platelet Indices ◽  
Group I ◽  
Group Ii

Background: Thrombocytopenia is one of the most common causes of abnormal bleeding and is defined as platelet counts < 1.5 lakhs/cumm. Three processes can cause thrombocytopenia, namely: Deficient platelet production, accelerated platelet destruction, and abnormal pooling of the platelets within the body. Of these, accelerated platelet destruction is the most common cause for thrombocytopenia and has variety of etiologies. The usefulness of bone marrow analysis in assessing accelerated platelet destruction is still debated. Therefore, a new simple and non-invasive diagnostic approach for thrombocytopenia is needed. Aims and Objectives: The present study was done with an aim to evaluate the use of platelet indices, namely, mean platelet volume (MPV), Platelet Distribution Width (PDW), and Platelet Large Cell Ratio (P-LCR) in differentiating the various causes of hyperdestructive thrombocytopenia. Materials and Methods: This was a prospective study conducted over a period of 2 years and consisted of 206 cases of hyperdestructive thrombocytopenia. After recording relevant clinical details, platelet count along with platelet indices – MPV, PDW, and P-LCR was recorded. Based on the etiopathology identified, cases were categorized into three groups: Group I: Immunologic – cases of Immune thrombocytopenic purpura (ITP), Group II: Non-immune: Cases of sepsis and other non-immune causes of platelet destruction, and Group III: Viral and parasitic infections. Platelet indices were compared between the study groups and the control group which included 100 healthy individuals. Comparison was done among the three study groups as well. Results & Conclusions: Dengue accounted for the highest number of 131 (89.72%) cases in the study. MPV, PDW, and P-LCR were significantly higher (P < 0.0001) when compared to the healthy controls except P-LCR in Group II. A statistically significant increase in MPV was noted among ITP cases when compared to other causes of thrombocytopenia. There wa

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