Calcific uremic panniculitis is a rare cutaneous calcinosis on the spectrum of calciphylaxis. It mainly affects patients with end-stage renal disease (ESRD). The mortality rate is high, approaching 50% of cases. We report the case of a patient on chronic hemodialysis with secondary hyperparathyroidism who developed calcific panniculitis. The diagnosis was clinical: bluish subcutaneous nodules in the abdomen with rapid extension to the lower limbs and even the back, which quickly became necrotic. X-rays of the abdomen and lower limbs showed rounded calcifications in the fatty tissue. The evolution was rapidly fatal and the patient died of septic shock following superimposed infection of the lesions. Calcific uremic panniculitis is an increasingly rare fatal complication associated with ESRD and secondary hyperparathyroidism. The treatment remains poorly understood and difficult to apply, hence the importance of preventing this complication.
Long-term immunosuppression can lead to malignant tumors after kidney transplant. In this case study, we report a rare case of breast cancer occurring in a man after 33 years of kidney transplant. This 55-year-old kidney transplant patient presented with a right breast mass. The biopsy showed an infiltrating carcinoma. The immunohistochemical study was positive for estrogen. The patient underwent a right mastectomy with axillary dissection. Chemotherapy based on docetaxel and radiotherapy was started. Adjuvant treatment with tamoxifen and a switch from azathioprine to sirolimus were conducted. Follow-up showed that the patient died 24 months after presentation. Through this case, we emphasize the importance of vigilance about this rare cancer, often diagnosed at a late stage, which can negatively affect prognosis. Screening for risk factors is crucial.
Introduction: Toll-like- receptors (TLR) control important aspects of innate and adaptive immune responses. Renal cells are among the non-immune cells that express (TLR). Therefore, their activation might be implicated in renal tubulo-interstitial injury. Aim: The study aimed to compare TLR9 expression in patients with primary membranous nephropathy (MN) to patients with lupus membranous nephropathy. Methods: Kidney sections from 10 Lupus nephritis (LN) patients and ten patients with primary MN were analyzed by immunohistochemistry using anti-human TLR9 antibody. Results: Results showed that TLR9 expression was weak and exclusively tubular in primary MN patients’ biopsies. There was a significant difference between LN patients’ biopsies and primary MN patients’ biopsies. TLR9 expression was more diffused in LN patients’ specimen than in those with primary MN. Conclusion: This study focuses on molecular level pathogenesis of MN. The data suggest that the receptors TLR9 may play role in tubulointerstitial injury in the pathogenesis of LN but not primary membranous nephropathy.
Objective: Arterial hypertension is common in hemodialysis patients of which physiopathology is multifactorial. Moreover, it is associated with an increased risk of cardiovascular disease and a higher mortality. Fluid overload is the main contributing factor. Few studies have assessed the interpretation of parameters obtained through bioelectrical impedance (BIA) for the management of these patients. Design and method: we conducted a cross-sectional study at the Hemodialysis Center of Hedi Chaker in Sfax, Tunisia. Multifrequency bioimpedance analysis (BIA) was used to assess the hydration status which is performed at the end of the dialysis session. Results: Our study included 29 patients, with a mean age of 46.8 years (21-78 years) and an average dialysis vintage of 92.4 months. Male predominance was noted with a sex ratio of 1.06. Forty-eight percent of patients were hypertensive. Twenty-three percent of patients were on triple antihypertensive therapy, 13.3% were on dual therapy, and 16.7% were on monotherapy. Hypertension was essential in 64.2% of cases, renin-dependent in 14.2%, and volume-dependent in 21.6% of cases. Twenty-point seven percent (20.7%) of patients were in fluid overload, with an average overhydration of 0.9 liters. The impedance measurements were consistent with the data from cardiac ultrasound. The diameter of the inferior vena cava was significantly correlated with overload (p=0.01). However, there is no correlation between pulmonary arterial hypertension (HTAP) and the hyperhydration (p=0.4). Conclusions: Bio-impedancemetry is a valuable and simple tool at the patient's bedside for the dry weight estimation and the proper management of volume status in chronic hemodialysis patients, and consequently, blood pressure control.
Introduction: Lupus nephritis (LN) is an immune complex glomerulonephritis, caused by systemic lupus erythematosus. It is associated with an increase of morbidity and mortality. In LN, the immune responses dysregulation is one of the crucial pathogenic pathways. Annexin A1 (AnxA1), as an anti-inflammatory mediator, plays a critical role in immune responses, in addition to a variety of pathological processes. Aim: This study aimed to evaluate the AnxA1 expression in renal tissues, in order to explore its potential role in LN pathogenesis. Methods: AnxA1 expression was performed by immunohistochemistry staining in renal biopsies of 24 LN patients compared to 8 controls. Results: LN patient’s biopsies showed an increased distribution of AnxA1 in glomeruli compared to controls (p=0.00019). When comparing AnxA1 expression in different LN classes, a high AnxA1 intensity score was positively correlated with glomerular proliferation. Conclusion: Our data suggest AnxA1 as a useful marker to differentiate between severe proliferative and non severe proliferative classes of LN.
Background: This study aimed to investigate the effects of intradialytic concurrent (resistance-endurance) training combined with melatonin (MEL) supplementation on oxidative stress, inflammation, and cellular damage in hemodialysis (HD) patients. Methods: Thirty-two HD patients were randomly assigned to three groups: Exercise (EX)-MEL, EX-Placebo (PLA), and Control (C)-PLA. Participants in the EX-MEL and EX-PLA groups underwent 12 weeks of concurrent training. Before nocturnal sleep, they ingested either 3 mg of MEL (EX-MEL) or a placebo (EX-PLA and C-PLA). Blood samples were collected at baseline and after 12 weeks of intervention to assess lipid peroxidation [malondialdehyde (MDA)], antioxidant biomarkers [ferric-reducing antioxidant power (FRAP), reduced glutathione (GSH), total thiol (THIOL)], total bilirubin (TBIL), uric acid (UA), biomarkers of muscle and liver damage [aspartate aminotransferase (ASAT), alanine aminotransferase (ALAT), creatine kinase (CK), lactate dehydrogenase (LDH), and Gamma-glutamyltransferase (Gamma-GT)], and inflammation [C-reactive protein (CRP)]. Results: EX-MEL demonstrated a decrease in MDA (p < 0.05) and CRP (p < 0.05), and an increase in FRAP (p < 0.05) pre- and post-training. Both EX-MEL and EX-PLA showed an increase in GSH (p < 0.001, and p < 0.05, respectively) and THIOL (p < 0.01, and p < 0.05, respectively) pre- and post-training. No significant changes were observed in TBIL, UA, ASAT, ALAT, CK, LDH, or Gamma-GT pre- and post-training across all groups. Conclusion: Concurrent training combined with MEL supplementation enhances oxidant-antioxidant balance and reduces inflammation in HD patients more effectively than intradialytic concurrent training alone.
Objective: High blood pressure is widespread among individuals undergoing hemodialysis and is considered a significant contributor to the elevated cardiovascular mortality observed in this population. The aim of our study was to assess the prevalence of HTN and investigate its contributing factors. Design and method: This is a retrospective descriptive and analytic study conducted within our hemodialysis unit, including 70 patients. For each participant, the following clinical data were collected: age, gender, personal medical history, initial nephropathy, dialysis vintage, and evolution Results: Our study revealed a hypertension (HTN) prevalence of 51,4%. Among them, 63,9% were male, resulting in a sex ratio of 1,76. The mean age was 49.1 years (range: 21-86 years). undetermined nephropathy was the predominant initial nephropathy (39%). The average blood pressure of hypertensive patients was 159 mmHg for systolic (142-189 mmHg). Diabetic patients were more hypertensive with no statistically significant difference. Hypertensive patients exhibited a larger waist circumference, lower hemoglobin levels, and a lower left ventricular ejection fraction (LVEF) with no statistically significant difference. hypertensive patients were more prone to cardio vascular events such Cerebrovascular Accidents (2% of patients), transient ischemic attacks (2% of patients), and coronary artery disease (11% of patients). Conclusions: In summary, our study emphasizes the importance of investigating hypertension and its related factors, aiming to contribute insights for better cardiovascular health management.
This study aimed to determine the impact of genetic variation in FKBP prolyl isomerase 5 (FKBP5) on mRNA and protein expression levels, on glucocorticoids (GC) responsiveness and its clinical association in systemic lupus erythematosus (SLE). The genotyping of the FKBP5-rs1360780 was performed using TaqMan SNP genotyping technology. The mRNA expression level in peripheral blood mononuclear cells was evaluated using realtimePCR. The immunohistochemistry staining was used to analyze FKBP5 protein expression level in renal biopsies. A risk association was revealed between rs1360780 > C allele and SLE pathogenesis. We found an upregulation in the FKBP5 mRNA expression level in SLE patients with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score <= 6 compared to patients with SLEDAI score > 6. Our results revealed an altered renal cell expression of FKBP5 protein in patients with lupus nephritis compared to control samples. However, no association was observed between the FKBP5 and the response to GC treatment. Our study is the first to demonstrate a link between SLE and FKBP5 gene, in terms of mRNA and protein expression levels, and the variant of the SNP rs1360780 in SLE, suggesting a susceptibility role of rs1360780 > C.
Purpose: The present study aimed to investigate for the first time the effects of melatonin (MEL) intake on oxidative stress and cellular damage during intradialytic exercise (IEX).Methods: Thirteen hemodialysis (HD) patients volunteered to participate in the current randomized crossover trial. Participants performed four HD sessions in four different conditions: (Exercise (EX)-MEL), (EX-Placebo (PLA)), (Control (C)-MEL), and (C-PLA). 3 mg of MEL or PLA were taken 60 min before starting exercise, or at the equivalent time in the C conditions. Blood samples were taken before HD (T0), immediately after the end of IEX (T1), 60 min after IEX (T2), or at the corresponding times in the C conditions to measure free radicals damage, antioxidant biomarkers, as well as biomarkers of muscle and liver damage.Results: Malondialdehyde and Advanced Oxidation Protein Products decreased in (C-MEL) (p < 0.05, d = 2.19; p < 0.01, d = 0.99, respectively) at T2 compared to T0. Catalase and total thiol levels increased in (C-MEL) (p < 0.01, d = 1.51; p < 0.01, d = 1.56, respectively) and in (EX-MEL) (p = 0.01, d = 1.28; p < 0.01, d = 1.52, respectively) at T1 compared to T0. Total bilirubin levels increased in (EX-MEL) and (C-MEL) at T2 compared to T0 (p < 0.001, d = 2.77; p < 0.001, d = 1.36, respectively), but only at T2 compared to T1 in (EX-MEL) (p < 0.001, d = 1.67). In all conditions, uric acid levels decreased at T1 compared to T0 and at T2 compared to T1, while biomarkers of muscle and liver damage remained unchanged.Conclusion: This pilot study is the first to show that MEL ingestion, alone or combined with IEX, could improve oxidant-antioxidant balance during HD.
Introduction: We evaluated the effect of intradialytic concurrent (resistance-endurance) training combined with melatonin (MEL) supplementation on functional capacity, muscle strength, postural balance, and quality of life (QoL) in hemodialysis (HD) patients.Methods: Thirty-three HD patients were randomized into three groups: Exercise (EX)-MEL (n = 11); EX-Placebo (PLA) (n = 11) and Control (C)-PLA (n = 11). Participants included in the EX-MEL and EX-PLA groups were submitted to concurrent training for 12 weeks.Results: EX-MEL and EX-PLA improved functional capacity, muscle strength, QoL, and postural balance parameters in eyes open and eyes closed conditions. Parameters of postural balance in the dual task condition were improved only in EX-MEL. EX-MEL induced better results in some domains of QoL compared with EX-PLA.Conclusion: Intradialytic concurrent training induced beneficial effects on physical function, muscle strength, postural balance, and QoL in HD patients. MEL supplementation combined with intradialytic exercise lead to better improvements in postural balance and QoL.
Dorfman-Chanarin syndrome (DCS) is a rare autosomal recessive disease. It is a multisystemic disease in which renal involvement is uncommon. We report the case of a woman with nephrotic syndrome associated with DCS. A 36-year-old woman was referred to the nephrology department for edema with known history for DCS. On physical examination, she had ichthyosiform erythroderma with generalized scaly skinand ascites. The ophthalmologic examination revealed a cataract in the right eye. Abdominal ultrasound examination showed hepatomegaly and splenomegaly. Laboratory tests showed normal renal and liver function. The blood cell count showed pancytopenia. Immunologic exams showed the presence of anti-mitochondrial antibodies. Kidney biopsy showed mesangial proliferative glomerulonephritis with extensive lipid vacuoles in the tubular epithelial cells. Immunofluorescence study showed mesangial deposits of IgG, C3, kappa, and lambda. To the best of our knowledge, this is the first case of DCS with renal involvement reported in an adult.
The 2019 coronavirus (COVID-19) is a viral infection caused by a new coronavirus that is affecting the entire world. Patients in dialysis are particularly vulnerable because of their multiple co-morbidities and immunosuppression. We report in our study the incidence and the course of COVID-19 in peritoneal dialysis (PD) unit at the height of the pandemic.
Calcific uremic arteriolopathy (CUA) is a rare but critical complication in the life course of any dialysis patient. It is associated with high mortality in this population. In this work we report our experience with CUA. This is a retrospective descriptive study of 6 chronic haemodialysis patients who were diagnosed with CUA, after a specialist dermatological examination. These patients were selected among those dialysed in our unit or in another centre but their diagnostic of CUA was made in the dermatology department of our hospital. The average age was 53.33 years. The duration on haemodialysis was 13.33 years (10-20 years).Only one patient had a preserved diuresis. Five patients had hypertension and one had diabetes. Half of the patients were on anti-vitamin K. Only one patient had a parathyroidectomy. The mean values for serum calcium (mmol/L), serum phosphorus (mmol/L) and calcium phosphate product(mmol2/L2) were 2.36, 1.9 and 4.45 respectively. CUA is characterised by painful infiltrated livedoid lesions progressing to necrosis. They were located in 100% of cases in the lower limbs, 16.66% in theabdominal area and 16.66% in the upper limbs. Only 2 patients had a skin biopsy, one of which was inconclusive. In this case, the evolution was marked by local superinfection. In 2 patients, CUA was associated with pseudoporphyria treated with n-acetylcysteine with clinical improvement. The mortality rate was 66.66%. CUA remains a serious and poorly understood complication.The particularity of this work is the combination of CUA and pseudoporphyria. The diagnosis is mainly clinical, which makes us think over the usefulness of skin biopsy in this context. The use of n-acetyl-cysteine appeared to be effective.
Cardiovascular complications are the leading cause of death in end stage renal disease.The neutrophils / lymphocytes ratio (RNL) has been presented as a novel and a potential marker to assess the inflammatory status of HD patients.Our study aims to define the relationship between the occurrence of cardiovascular complications in hemodialysis(HD)patients with theratio of neutrophils to lymphocytes (RNL) and the ratio of platelets to lymphocytes (RPL).
Chronic fatigue and sleep disorders remains the most common problems in HD patients which deeply affect their quality of life. This study was conducted to assess the level of post dialysis fatigue among our HD patients.
Vascular access dysfunction is one of the most important causes of morbidity and mortality in hemodialysis patients, contributing to up to one third of hospitalizations and accounting for a significant amount of the health care costs of these patients.
We propose to investigate the correlation between immunohistochemistry renal expression of glucocorticoid receptor (GCR) in patients with idiopathic nephrotic syndrome due to minimal changes diseases (MCD) or focal segmental glomerulosclerosis (FSG) and the evolution on steroids.
Objective: Plasma renin and aldosterone assays are useful in clinical practice for the aetiological assessment of arterial hypertension (hypertension) and / or a disturbance of the electrolyte balance. The objective of our work is to describe the main indications exploration of the renin aldosterone system, its results, its diagnostic and etiological impact. Design and method: We conducted a retrospective study of 40 patients who received a renin / aldosterone dosage, collected in the Nephrology department at the Hédi Chaker CHU in Sfax for a period of 6 years from January 2014 until December 2020. Results: These are 21 women and 19 men with a sex ratio of 1.1. Hypertension was present in 82.5% of cases. The dosage indication was: hypertension with hypokalaemia in 52.5%, hypertension in young subjects in 20%, resistant hypertension and hypertension with incidentaloma in each 7, 5% of cases, and incidentaloma without HTA in each 5% of cases and in one case hypokalaemia without HTA. Aldosterone was elevated in 52.5% of cases. Renin was normal in 67.5% of cases, low in 25% and high in 7.5% of cases. The aldosterone / renin ratio was normal in 60% of cases, elevated, decreased and invalid in respectively 27.5%, 2.5% and 10%. The etiological investigation revealed: primary hyperaldosteronism in 42.5% of cases secondary to a Conn adenoma in 20% of cases, to adrenal hyperplasia in 17.5% of cases and with normal imaging in 5% of cases. Secondary hyperaldosteronism was confirmed in association with Liddle syndrome and renal artery stenosis in 2.5% of cases each. The other etiologies were pheochromocytoma in 12.5% of cases, Takayusu’s disease, tubulopathy in 2.5% of cases each. The dosage was non-contributory in 7.5% of cases and normal in 25% of cases. Conclusions: The exploration of the renin angiotensin aldosterone system has several indications, mainly in hypertension with hypokalaemia. It can help make the etiological diagnosis and guide therapeutic management.
Background: Patients (pts) with newly diagnosed Multiple Myeloma (NDMM) face a high risk of developing a renal impairment (RI). It is one of the most feared complications in this population due to its severe immediate consequences and its poor outcome on the prognosis and the overall survival. We report our retrospective single-center study of NDMM in young pts with RI. Aims: This study aims to identify the incidence of renal failure (RF) in NDMM and analyze the renal response (RR), hematological response (HR), the overall survival (OS) and the prognostic impact after induction therapy. Methods: This retrospective single-center review over 4 years (between June 2016 and December 2020) includes young pts under the age of 65 years old with NDMM with RF eligible for autologous stem cells transplantation (ASCT). RF was defined by a serum creatinine higher than 177 μmol/L and the serum Creatinine Clearance (CC) was calculated according to the MDRD formula. All pts with RF in this study were treated with Bortezomib, Thalidomide and Dexamethasone (DT) as an induction regimen. RR and HR were defined according to the International Myeloma Working Group (IMWG14) criteria. In addition to antimyeloma treatment, pts with RI received symptomatic treatment such as intravenous hydration, corticosteroids, correction of hypercalcemia and in some cases dialysis. In this study, we identify the incidence of RF in NDMM and analyze the RR, HR, OS and the prognostic impact after induction therapy. Results: We included in our study 58 pts with NDMM. The incidence of NDMM with RF at diagnosis was 48,3% (n=28), the serum creatinine level median was 213μmol/L and the serum creatinine clearance (CC) median was 23.5 ml/min. According to the CC staging, the renal function was severely decreased in 11 cases (39%) and in the kidney failure stage in 5 cases (17%). The MM with RF immunoglobulin type was Light chain in 11 cases (39%), IgG in 12 cases (43%) and IgA in 5 cases (18%). After receiving symptomatic treatments of RF, 35% (n=10) of NDMM with RF pts had their renal function recovered whereas 64%(n=18) did not and 10% (n=3) needed dialysis. After induction therapy, the serum creatinine level median was 95μmol/L and the serum CC median was 73.5ml/min Thus, the RR was: complete in 64% (n=18), partial in 7%(n=2), minor in 18% (n=5), no response in 2 cases (7%) and one patient died before assessment. HR after induction regimen was 74% (n=20): complete response (CR) in 4 cases (14%), very good partial response (VGPR) in 8 cases (29%) and partial response (PR) in 8 cases (29%). Among the 18 pts with complete RR, 6 pts (33%) underwent ASCT. Pts with RF at diagnosis had a poorer OS median (18.5 months) comparing to those with normal renal function (OS median=21.5 months). The OS was significantly influenced by the degree of RF and the recovery of normal renal function (p<0.05). Summary/Conclusion: Our single center study showed that RI occurs in 48% of the cases in NDMM at diagnosis, which is significantly higher to what is found in the literature (5-30%). It also revealed that NDMM with RI has poorer HR and OS than pts with normal renal function when both treated by a Brotezomib induction regimen followed by ASCT. The RR after supportive care measurements and antimyeloma therapy was 74%, which is slightly lower to what is found in the literature (77%). Thus, early diagnosis and rapid initiation of a triplet Brotezomib based regimen for this high-risk group is essential and improves renal outcomes in RI pts.
Dermatological manifestations in patients with end-stage renal disease (ESRD) are very common, such as pruritus, xerosis and pigmentation disorders, other skin disorders are quite rare such as pseudoporphyria cutanea tarda (PCT). It is a photodistributed vesicobullous disorder with clinical and histologic features similar to those of porphyria cutanea tarda but without accompanying biochemical porphyrin abnormalities. Although PCT is seen in 13% of hemodialysis patients, it is rare in peritoneal dialysis (PD) patients.