scholarly journals Mystery called sarcoidosis: Forty-four years follow-up of chronic systemic disease

2012 ◽  
Vol 140 (11-12) ◽  
pp. 768-771
Author(s):  
Vesna Skodric-Trifunovic ◽  
Violeta Vucinic ◽  
Sanja Simic-Ogrizovic ◽  
Ruza Stevic ◽  
Mihailo Stjepanovic ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Inflammation ◽  
Kidney Biopsy ◽  
Clinical Course ◽  
Systemic Disease ◽  
Body Weight Loss ◽  
Final Diagnosis ◽  
Pulmonary Sarcoidosis ◽  
Laboratory Findings

Introduction. This is a presentation of a 61-year-old female patient. Since 44 years have passed from the onset of her first symptoms until the final diagnosis of sarcoidosis, this was the reason of our decision to publish the case. Case Outline. During the follow-up period of 44 years the patient had ocassional polymorphic complains, such as adynamia, nausea, abdominal pains, myalgia, arthralgia, body weight loss (8-10 kg) etc. The clinical course was predominated by splenomegaly, hepatitis and arthralgia, and later chronic renal failure also developed. Laboratory findings showed elevated markers of acute inflammation and autoantibodies. The patient was hospitalized in different university internal hospitals (gastroenterology, allergology, rheumatology, nephrology and pulmology). Liver biopsy was performed three times, rectum and kidney biopsy once each and finally bronchoscopy and pulmonary biopsy was done. At last, about 40 years from the onset of the first symptoms, in 2006 the diagnosis of lung sarcoidosis was established. Conclusion. The final diagnosis of spleen sarcoidosis was confirmed by pathologically verified sarcoidosis of the lungs. This case is particularly interesting because of the presence of familial sarcoidosis (the patient?s son also had recurrent pulmonary sarcoidosis).

scholarly journals Effect of Genetic and Laboratory Findings on Clinical Course of Antisynthetase Syndrome in a Hungarian Cohort

2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Katalin Szabó ◽  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Anett Vincze ◽  
Erika Zilahi ◽  
...  
Keyword(s):  
Prognostic Markers ◽  
Clinical Course ◽  
Disease Onset ◽  
Skin Lesions ◽  
Clinical Database ◽  
Laboratory Findings ◽  
Disease Phenotypes ◽  
Genetic Features ◽  
Hla Dqa1

The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud’s phenomenon, 43% fever, 33% mechanic’s hand, and 12% dysphagia. We could detect significant correlation between anti-Jo-1 titer and the CK and CRP levels at disease onset and during disease course. HLA DRB1⁎03 positivity was present in 68.96% of patients, where the CK level at diagnosis was significantly lower compared to the HLA DRB1⁎03 negative patients. HLA DQA1⁎0501-DQB1⁎0201 haplotype was found in 58.62% of patients, but no significant correlation was found regarding any clinical or laboratory features. Higher CRP, ESR level, RF positivity, and the presence of fever or vasculitic skin lesions at the time of diagnosis indicated a higher steroid demand and the administration of higher number of immunosuppressants during the follow-up within anti-Jo-1 positive patients. The organ involvement of the disease was not different in HLA-DRB1⁎0301 positive or negative patients who were positive to the anti-Jo-1 antibody; however, initial CK level was lower in HLA-DRB1⁎0301 positive patients. Distinct laboratory and clinical parameters at diagnosis could be considered as prognostic markers.

Clinical course in children and adolescents with ventricular septal defect

2019 ◽  
Vol 27 (7) ◽  
pp. 529-534
Author(s):  
Noor Mohammad Noori ◽  
Alireza Teimouri
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Clinical Course ◽  
Defect Size ◽  
Eisenmenger Syndrome ◽  
Ventricular Septal Defects ◽  
Laboratory Findings ◽  
Septal Defects

Background Ventricular septal defect, the most common congenital heart defect, is characterized by an opening between the ventricles. This study aimed to evaluate the clinical course and associations between the characteristics of ventricular septal defect. Methods This cross-sectional study was conducted on 1498 children with ventricular septal defects, aged <19 years, who were referred to our center between 2003 and 2018. The diagnosis was suspected from a combination of clinical and laboratory findings, and confirmed by transthoracic echocardiography. Results Of the 1498 children, 54.9% were boys, 78.4% of defects were perimembranous, 30.4% of patients had pulmonary hypertension, 67.5% had regular follow-up, and 76 (5.1%) had complications including 28 (1.9%) with Eisenmenger syndrome; 10 died due to Eisenmenger syndrome during follow-up. The defects closed spontaneously in 38.9% and after surgery in 20.9%. Boys tended to have more perimembranous, inlet, and outlet forms (56.40%, 50.60%, 51.90%, respectively), whereas girls had more muscular types (51.80%). Most patients with pulmonary hypertension had perimembranous defects (83.10%). The majority of patients with pulmonary hypertension had large (63.40%) or moderate (36.60%) defects. Pulmonary hypertension had a significant association with defect size ( p < 0.001). After surgery, residual defects were found mostly in cases of large defects (84.60%). Most patients with spontaneous closure were younger than 4 years. Conclusion Almost four-fifths of children with ventricular septal defects had perimembranous types, and almost one-third had pulmonary hypertension which was associated with defect size. Two-fifths of the defects closed spontaneously. There was a low incidence of complications after surgery.

scholarly journals Management of Nephrotic Syndrome: A Case Report from Lao PDR

2017 ◽  
Vol 44 (Suppl. 1) ◽  
pp. 31-34
Author(s):  
Phanekham Souvannamethy
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
Lao Pdr ◽  
Deficiency Anemia ◽  
Laboratory Findings ◽  
Poor Diet ◽  
History Of ◽  
Laboratory Investigations ◽  
Pedal Edema

We report the case of a 23-year-old woman with a 2-week history of swelling around the eyes and both legs, and generalized body swelling. She had a history of chronic constipation and poor diet but no fever, recent illnesses, or hematuria. Examination revealed bilateral pedal edema and mild ascites. Laboratory investigations showed low hemoglobin 79 g/L, low mean corpuscular volume 53 fL, thrombocytosis 973 × 109/L, and marked hypochromia and microcytosis, with low iron and ferritin. She had hypoalbuminemia and reduced serum protein (albumin 1.9 g/dL, globulin 2.8 g/dL) with elevated triglycerides (454 mg/dL). Although kidney biopsy could not be performed due to a lack of facilities in the country, we made a diagnosis of idiopathic nephrotic syndrome (NS) with iron deficiency anemia secondary to poor diet based on clinical and laboratory findings. The patient was admitted and treated with intravenous methylprednisolone and iron supplements. Antiplatelet therapy was instituted with dipyridamole to prevent thromboembolism from the combination of nephrotic syndrome and thrombocytosis. She was later treated with albumin and furosemide due to elevated blood pressure and worsened edema. The edema resolved and her general condition improved. She was discharged to follow up and has remained stable, requiring no further treatment as of 18 months after admission. Kidney biopsy is important for diagnosis of NS. Diagnosis may be made from clinical and laboratory findings alone in some cases; however, biopsy is required to determine the type of NS and improve further management and treatment outcomes for patients.

Rhabdomyolysis in a case of free-base cocaine ("crack") overdose.

1989 ◽  
Vol 35 (7) ◽  
pp. 1547-1549 ◽  
Author(s):  
M A Jandreski ◽  
E W Bermes ◽  
R Leischner ◽  
S E Kahn
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Crack Cocaine ◽  
Clinical Course ◽  
Medical Center ◽  
Clinical Manifestations ◽  
Blood Chemistry ◽  
Free Base ◽  
Laboratory Findings ◽  
Remarkable Recovery

Abstract This is the case of a 27-year-old black man who was admitted to Loyola University Medical Center after a one-time experience of smoking free-base ("crack") cocaine. Clinical manifestations of the resulting cocaine intoxication were rhabdomyolysis, acute renal failure, and transient liver failure. This patient came to our attention because of the striking alterations in his blood-chemistry values, which indicated acute tissue damage, and his remarkable recovery within 96 h. We discuss the dramatic changes in the laboratory findings and the clinical course of this patient.

scholarly journals Familial occurrence of nodular regenerative hyperplasia of the liver: a report on three families

Gut ◽  
1999 ◽  
Vol 45 (2) ◽  
pp. 289-294 ◽  
Author(s):  
J Dumortier ◽  
O Boillot ◽  
M Chevallier ◽  
F Berger ◽  
P Potier ◽  
...  
Keyword(s):  
Renal Failure ◽  
Portal Hypertension ◽  
Clinical Course ◽  
Systemic Disease ◽  
Nodular Regenerative Hyperplasia ◽  
Histological Lesion ◽  
Renal Lesions ◽  
Familial Cases ◽  
Biopsy Specimens ◽  
Male Patients

BACKGROUND/AIMSNodular regenerative hyperplasia of the liver is a histological lesion usually associated with systemic diseases, haematological malignancies, or drugs. Its prognosis depends on portal hypertension, which usually is well tolerated and requires medical management only.PATIENTSThree unrelated families, in which two sibling adult male patients presented with nodular regenerative hyperplasia of the liver, were studied.METHODSComplete clinical charts and liver biopsy specimens were available for all patients. In addition, explanted livers were available for examination for the two transplanted patients.RESULTSThere was no evidence of any of the various clinical situations known to be associated with nodular regenerative hyperplasia of the liver. Portal hypertension was severe, requiring surgical treatment in two cases. Renal lesions were present in three patients. In two patients, progressive evolution to liver atrophy and hepatic failure, associated with renal failure, led to combined liver and renal transplantation.CONCLUSIONSThis report describes the existence of familial cases of nodular regenerative hyperplasia of the liver, occurring without underlying or associated systemic disease, characterised by a poor clinical course and often associated with progressive renal failure.

scholarly journals Acute tubulointerstitial nephritis and polyclonal hypergammaglobulinaemia: Which is the culprit?

2018 ◽  
Vol 8 (4) ◽  
Author(s):  
Ana E. Sirvent ◽  
Ricardo Enríquez ◽  
Tania Muci ◽  
Francisco Javier Ardoy-Ibañez ◽  
Isabel Millán ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Serum Creatinine ◽  
Renal Biopsy ◽  
Proton Pump ◽  
Tubulointerstitial Nephritis ◽  
Systemic Disease ◽  
Acute Tubulointerstitial Nephritis ◽  
Long Period

Proton pump inhibitors (PPIs) are among the most frequent implicated drugs in acute tubulointerstitial nephritis (ATIN), nevertheless it is important to report cases with atypical profiles. A 80-year-old female, exposed during 34 months to omeprazole, presented with polyclonal hypergammaglobulinaemia and renal failure. After stopping omeprazole there was a partial improvement in serum creatinine and IgG. Renal biopsy revealed ATIN; immunohistochemistry for IgG4 was negative. Treatment with steroids and mycophenolate sodium improved renal function and normalized immunoglobulins. The lack of data of other entities and the patient’s evolution strongly point omeprazole as the culprit. After 27 months of follow-up, she remains clinical and analytically stable. ATIN caused by PPIs may appear after a long period of exposure and may be accompanied by analytical anomalies that simulate a systemic disease.

scholarly journals Fibrillary glomerulonephritis with a favourable prognosis of 26 years

2020 ◽  
Vol 10 (4) ◽  
pp. e44-e44
Author(s):  
David Micarelli ◽  
Valentina Pistolesi ◽  
Emanuela Cristi ◽  
Anna Rita Taddei ◽  
Ilaria Serriello ◽  
...  
Keyword(s):  
Renal Function ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Kidney Biopsy ◽  
Clinical Course ◽  
Glomerular Sclerosis ◽  
Fibrillary Glomerulonephritis ◽  
Stage Renal Disease ◽  
End Stage

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. The prognosis is usually unfavorable with nearly half of patients progressing to end-stage renal disease within 4 years. We report a case of biopsy-proven FGN characterized by an unusual benign clinical course in which a kidney biopsy, repeated after an extended follow-up of 26 years, confirmed the presence of fibrils deposition. In 1993, a 32-year-old Caucasian man was admitted to our nephrology ward because of macroscopic hematuria. Renal function was normal. Kidney biopsy displayed an FGN with mesangial pattern. The patient was treated with lisinopril, titrated for blood pressure; the therapy was maintained during 26 years of follow-up. The yearly slope of estimated glomerular filtration rate was -3.17 mL/ min). Starting from March 2018, a rapid worsening of renal function was observed and proteinuria increased up to a nephrotic range. We planned a second renal biopsy to assess the cause of the rapid change of clinical course. The diagnosis of FGN on advanced sclerosis was made, and the severity of glomerular sclerosis. We report a case of FGN with an unusually benign clinical course, characterized by a slow progression to end-stage renal disease over a very extended follow-up time; thus, to better clarify the reason for renal function worsening, a second renal biopsy was performed. The persistence of fibrils deposition confirmed the initial diagnosis of FGN, and a histological pattern characterized by global glomerular sclerosis and interstitial fibrosis has been observed.

scholarly journals The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy—A National Study in Children

2021 ◽  
Vol 10 (19) ◽  
pp. 4405
Author(s):  
Małgorzata Mizerska-Wasiak ◽  
Agnieszka Such-Gruchot ◽  
Karolina Cichoń-Kawa ◽  
Agnieszka Turczyn ◽  
Jadwiga Małdyk ◽  
...  
Keyword(s):  
Kidney Biopsy ◽  
Clinical Presentation ◽  
Immunofluorescence Microscopy ◽  
Clinical Course ◽  
National Study ◽  
Oxford Classification ◽  
Complement Component C3 ◽  
The Oxford Classification

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children.

scholarly journals P0387PATHOLOGICAL FEATURES AND CLINICAL COURSE OF LATE-ONSET LUPUS NEPHRITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Amir Shabaka ◽  
Beatriz Sanchez Alamo ◽  
Serena Gatius ◽  
Clara Maria Cases Corona ◽  
Elena Valdes ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Early Onset ◽  
Kidney Biopsy ◽  
Clinical Course ◽  
Late Onset ◽  
Infectious Complications ◽  
Class V ◽  
Treatment Regimens ◽  
Histological Characteristics

Abstract Background and Aims It has been described that the early onset of lupus nephritis (LN) at a young age can determine the clinical course and outcome of the disease, being more aggressive with a higher mortality rate. However, no studies have explored the influence of age at onset of the disease in European cohorts of LN patients, neither have they analyzed treatment outcomes depending on age at presentation. Therefore, the aim of our study was to compare the clinical and histological characteristics of patients with late-onset LN compared to those with an onset at a younger age. Method We performed a retrospective observational study of biopsy-proven LN diagnosed between 1981 and 2018. Patients were stratified according to their age at presentation of LN, considering an onset with &gt;50 years old as late-onset. Demographic, clinical and serological data at presentation of systemic lupus and LN, during and at the end of follow-up, as well as extrarenal manifestations, histological characteristics of the kidney biopsy, clinical course, received treatment regimens and related adverse effects were registered. Results 85 LN patients were included in the study, 10 of whom had late-onset LN (11.7%). Late-onset LN patients presented significantly higher serum C3 and C4 levels at the time of kidney biopsy (92.8 ± 22.4 mg/dl vs 62.5 ± 25.7 mg/dl, p=0.002, and 18.6 ± 5.0 vs. 9.3±5.8 mg/dl, p&lt;0.001 respectively), and had lower eGFR measured by CKD-EPI (58.50±19.79 vs 90.41±30.53,p=0.003), a higher frequency of serositis manifestations (53.45% vs 17.46%, p=0.034), predominantly pleuritis (79.37% vs 51.75% p=0.013). Early-onset LN had predominantly Class IV LN (50% vs 10%, p=0.017), whereas in late-onset LN the most frequent type was Class V LN (50% vs 21.3%, p=0.048). Tubular atrophy was more common in late-onset LN (75% vs 34.9%, p=0.035). The group of patients with late-onset LN achieved complete clinical remission more frequently (87.5% vs 57.1%), but there were no differences in relapses between the groups. No differences were found in treatment regimens among the groups. Late-onset LN patients were more seronegative (100% vs 61.3%, p=0.015) at the end of follow-up. Severe infectious complications related to immunosuppression were significantly higher in late-onset LN patients (62.5% vs 22.2%, p=0.027) Conclusion Late-onset LN has a milder complement consumption at presentation compared to early-onset, and Class V LN is its most frequent form of presentation. Complete renal remission is more frequently achieved in late-onset LN compared to those with an early-onset, but present more adverse events associated with immunosuppression, particularly infectious complications.

scholarly journals On Two Cases with Autosomal Dominant Hyper IgE Syndrome: Importance of Immunological Parameters for Clinical Course and Follow-Up

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Snezhina Mihailova Kandilarova ◽  
Spaska Stoyneva Lesichkova ◽  
Nevena Todorova Gesheva ◽  
Petya Stefanova Yankova ◽  
Nedelcho Hristov Ivanov ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Clinical Symptoms ◽  
Clinical Course ◽  
Dna Binding Domain ◽  
Disease Course ◽  
Immunological Parameters ◽  
Laboratory Findings ◽  
Hyper Ige Syndrome ◽  
Stat3 Mutation

Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare disease described in 1966. It is characterized by severe dermatitis, a peculiar face, frequent infections, extremely high levels of serum IgE and eosinophilia, all resulting from a defect in the STAT3 gene. A variety of mutations in the SH2 and DNA-binding domain have been described, and several studies have searched for associations between the severity of the clinical symptoms, laboratory findings, and the type of genetic alteration. We present two children with AD-HIES–a girl with the most common STAT3 mutation (R382W) and a boy with a rare variant (G617E) in the same gene, previously reported in only one other patient. Herein, we discuss the clinical and immunological findings in our patients, focusing on their importance on disease course and management.

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