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Background: The non-inferiority of dexamethasone (DEX) on day 1, with or without low-dose DEX on days 2 and 3, combined with oral NEPA (netupitant/palonosetron), compared with the guideline-consistent use of DEX was demonstrated in cisplatin. Here, we complete the analysis by assessing the impact of emesis on daily lives of patients receiving DEX-sparing regimens using the Functional Living Index-Emesis (FLIE). Methods: Chemotherapy-naïve patients undergoing cisplatin (≥70 mg/m2), were given NEPA and DEX (12 mg) on day 1 and randomized to receive either 1) no further DEX (DEX1), 2) oral DEX (4 mg daily) on days 2–3 (DEX3), or 3) DEX (4 mg twice daily) on days 2–4 (DEX4; control). Patients completed the FLIE questionnaire on day 6 of cycle 1. Endpoints included the FLIE nausea domain, vomiting domain, and overall combined domain scores, as well as the proportion of patients with no impact on daily life (NIDL; overall score > 108). This was a protocol-planned analysis. Results: In the DEX1 group, no significant differences were observed in the FLIE nausea score (48.9 [±1.8; SE] vs. 53.7 [±1.5]), vomiting score (56.6 [±1.4] vs. 58.7 [±0.8]) and overall score (105.6 [±2.8] vs.112.4 [±1.9]) versus DEX4 control; similar results were observed in the DEX3 group for nausea score (49.6 [±1.7]), vomiting score (58.2 [±1]) and overall score (107.8 [±2.4]) versus control. There were no significant between-group differences in the proportion of patients reporting NIDL. Conclusion: Reducing DEX, when administered with NEPA, does not seem to adversely impact the daily functioning in patients undergoing cisplatin. Trial registration: ClinicalTrials.govNCT04201769. Registration date: 17/12/2019 - Retrospectively registered.

BACKGROUND: Treatment options for penile squamous cell carcinoma are limited. We sought to investigate clinical outcomes and safety profiles of patients with penile squamous cell carcinoma receiving immune checkpoint inhibitors. METHODS: This retrospective study included patients with locally advanced or metastatic penile squamous cell carcinoma receiving immune checkpoint inhibitors between 2015 and 2022 across 24 centers in the United States, Europe, and Asia. Overall survival and progression-free survival were estimated using the Kaplan-Meier method. Objective response rates were determined per Response Evaluation Criteria in Solid Tumours 1.1 criteria. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events, version 5.0. Two-sided statistical tests were used for comparisons. RESULTS: Among 92 patients, 8 (8.7%) were Asian, 6 (6.5%) were Black, and 24 (29%) were Hispanic and/or Latinx. Median (interquartile range) age was 62 (53-70) years. In all, 83 (90%) had metastatic penile squamous cell carcinoma, and 74 (80%) had received at least second-line treatment. Most patients received pembrolizumab monotherapy (n = 26 [28%]), combination nivolumab-ipilimumab with or without multitargeted tyrosine kinase inhibitors (n = 23 [25%]), or nivolumab (n = 16 [17%]) or cemiplimab (n = 15 [16%]) monotherapies. Median overall and progression-free survival were 9.8 months (95% confidence interval = 7.7 to 12.8 months) and 3.2 months (95% confidence interval = 2.5 to 4.2 months), respectively. The objective response rate was 13% (n = 11/85) in the overall cohort and 35% (n = 7/20) in patients with lymph node-only metastases. Visceral metastases, Eastern Cooperative Oncology Group (ECOG) performance status of 1 or higher, and a higher neutrophil/lymphocyte ratio were associated with worse overall survival. Treatment-related adverse events occurred in 27 (29%) patients, and 9.8% (n = 9) of the events were grade 3 or higher. CONCLUSIONS

We demonstrated the non-inferiority of a dexamethasone (DEX)-sparing (single-dose) regimen with NEPA, a netupitant/palonosetron fixed combination, for preventing chemotherapy-induced nausea and vomiting (CINV) caused by cisplatin. This pre-planned exploratory analysis assessed the effect of the DEX-sparing regimen on a patient's food intake. Chemotherapy-naive patients undergoing cisplatin (>= 70 mg/m(2)) were given NEPA and DEX (12 mg) on day 1 and randomized to receive either no further DEX (DEX1), or oral DEX (4 mg BID) on days 2-4 (DEX4). Patient-reported endpoint maintenance of usual daily food intake was assessed during the 5-days post-chemotherapy. The relationship between usual daily food intake and CINV control, pre-chemotherapy self-rated food intake and BMI-adjusted weight loss (WL) were evaluated. One-hundred fifty-two patients (76/group) were assessable. The proportion of patients reporting maintenance of usual daily food intake was similar in both groups: 69.7% (95% CI, 58.6-78.9) for DEX1 vs. 72.4% (95% CI, 61.4-81.2) for DEX4. Only CINV control was significantly associated with maintenance of usual daily food intake (P <= 0.001) during the overall phase. The DEX-sparing regimen does not adversely affect patient-reported daily food intake post-chemotherapy. The current analysis adds further insights into antiemetic efficacy of DEX sparing beyond day 1 in the challenging setting of cisplatin.Trial registration: The parent study was registered on ClinicalTrials.gov (NCT04201769).

Background: To reduce the overall exposure to dexamethasone (DEX) in patients receiving cisplatin-based chemotherapy, we evaluated the noninferiority of DEX on day 1, with or without low-dose DEX on days 2 and 3, combined with an oral fixed-dose combination of netupitant and palonosetron (NEPA), compared with the guideline-consistent use of 4-day DEX. Patients and Methods: In this open-label, multicenter study, chemotherapy-naïve patients undergoing high-dose cisplatin (≥70 mg/m2), were given NEPA and DEX (12 mg) on day 1 and randomized (1:1:1 ratio) to receive either (a) no further DEX (DEX1), (b) oral DEX (4 mg daily) on days 2–3 (DEX3), or (c) DEX (4 mg twice daily) on days 2–4 (DEX4). The primary efficacy endpoint was complete response (CR: no emesis and no rescue medication) during the 5-day overall phase. The noninferiority margin was set at −15% difference (DEX1 or DEX3 minus DEX4). Secondary efficacy endpoints included complete protection (CP: CR and none or mild nausea). Results: Two-hundred twenty-eight patients, 76 in each arm, were assessable. Noninferiority was met for both DEX-sparing regimens and the reference arm, with overall phase CR rates of 76.3% in each of the DEX1 and DEX3 arms and 75.0% in the DEX4 arm (95% confidence interval, −12.3% to 15% for each comparison). During the overall phase, CP rates were similar between groups. Conclusion: A simplified regimen of NEPA plus single-dose DEX offers comparable chemotherapy-induced nausea and vomiting prevention throughout 5 days post-chemotherapy with the advantage of sparing patients additional doses of DEX in the high–emetic-risk setting of cisplatin-based chemotherapy. Implications for Practice: Dexamethasone (DEX) has traditionally played an integral role in the management of chemotherapy-induced emesis. Although generally considered safe, even short-term DEX use is associated with various side effects, and some evidence suggests that concurrent steroids may reduce the efficacy of immunotherapi

Clear cell renal cell carcinoma (ccRCC) is fundamentally a metabolic disease. Given the importance of lipids in many cellular processes, in this study we delineated a lipidomic profile of human ccRCC and integrated it with transcriptomic data to connect the variations in cancer lipid metabolism with gene expression changes. Untargeted lipidomic analysis was performed on 20 ccRCC and 20 paired normal tissues, using LC-MS and GC-MS. Different lipid classes were altered in cancer compared to normal tissue. Among the long chain fatty acids (LCFAs), significant accumulations of polyunsaturated fatty acids (PUFAs) were found. Integrated lipidomic and transcriptomic analysis showed that fatty acid desaturation and elongation pathways were enriched in neoplastic tissue. Consistent with these findings, we observed increased expression of stearoyl-CoA desaturase(SCD1) and FA elongase 2 and 5 in ccRCC. Primary renal cancer cells treated with a small molecule SCD1 inhibitor (A939572) proliferated at a slower rate than untreated cancer cells. In addition, after cisplatin treatment, the death rate of tumor cells treated with A939572 was significantly greater than that of untreated cancer cells. In conclusion, our findings delineate a ccRCC lipidomic signature and showed that SCD1 inhibition significantly reduced cancer cell proliferation and increased cisplatin sensitivity, suggesting that this pathway can be involved in ccRCC chemotherapy resistance.

Clear cell renal cell carcinoma (ccRCC) is fundamentally a metabolic disease. Given the importance of lipids in many cellular processes, in this study we delineated a lipidomic profile of human ccRCC and integrated it with transcriptomic data to connect the variations in cancer lipid metabolism with gene expression changes. Untargeted lipidomic analysis was performed on 20 ccRCC and 20 paired normal tissues, using LC-MS and GC-MS. Different lipid classes were altered in cancer compared to normal tissue. Among the long chain fatty acids (LCFAs), significant accumulations of polyunsaturated fatty acids (PUFAs) were found. Integrated lipidomic and transcriptomic analysis showed that fatty acid desaturation and elongation pathways were enriched in neoplastic tissue. Consistent with these findings, we observed increased expression of stearoyl-CoA desaturase(SCD1) and FA elongase 2 and 5 in ccRCC. Primary renal cancer cells treated with a small molecule SCD1 inhibitor (A939572) proliferated at a slower rate than untreated cancer cells. In addition, after cisplatin treatment, the death rate of tumor cells treated with A939572 was significantly greater than that of untreated cancer cells. In conclusion, our findings delineate a ccRCC lipidomic signature and showed that SCD1 inhibition significantly reduced cancer cell proliferation and increased cisplatin sensitivity, suggesting that this pathway can be involved in ccRCC chemotherapy resistance.

Background and aim: Over 80% (365/454) of the nation’s centers participated in the Italian Society of Nephrology COVID-19 Survey. Out of 60,441 surveyed patients, 1368 were infected as of April 23rd, 2020. However, center-specific proportions showed substantial heterogeneity. We therefore undertook new analyses to identify explanatory factors, contextual effects, and decision rules for infection containment. Methods: We investigated fixed factors and contextual effects by multilevel modeling. Classification and Regression Tree (CART) analysis was used to develop decision rules. Results: Increased positivity among hemodialysis patients was predicted by center location [incidence rate ratio (IRR) 1.34, 95% confidence interval (CI) 1.20–1.51], positive healthcare workers (IRR 1.09, 95% CI 1.02–1.17), test-all policy (IRR 5.94, 95% CI 3.36–10.45), and infected proportion in the general population (IRR 1.002, 95% CI 1.001–1.003) (all p < 0.01). Conversely, lockdown duration exerted a protective effect (IRR 0.95, 95% CI 0.94–0.98) (p < 0.01). The province-contextual effects accounted for 10% of the total variability. Predictive factors for peritoneal dialysis and transplant cases were center location and infected proportion in the general population. Using recursive partitioning, we identified decision thresholds at general population incidence ≥ 229 per 100,000 and at ≥ 3 positive healthcare workers. Conclusions: Beyond fixed risk factors, shared with the general population, the increased and heterogeneous proportion of positive patients is related to the center’s testing policy, the number of positive patients and healthcare workers, and to contextual effects at the province level. Nephrology centers may adopt simple decision rules to strengthen containment measures timely.

Although Metformin (MT) is a first-line therapy for the treatment of type 2 diabetes mellitus, some patients may not receive it owing to the risk of MALA (metformin-associated lactic acidosis), an exceptionally rare but fatal adverse event. Then, the drug is contraindicated in many individuals with impaired kidney function because of concerns of MALA. MT, along with other drugs in the biguanide class, increases plasma lactate levels in a plasma concentration-dependent manner by inhibiting mitochondrial respiration predominantly in the liver. Elevated plasma MT concentrations (as occur in individuals with renal impairment) and a secondary event or condition that further disrupt lactate production or clearance (e.g., acute illness in diabetic patients where cardiac, hepatic, pulmonary, or renal function are compromised), are typically necessary to cause MALA. As these secondary events may be unpredictable and the mortality rate for MALA approaches 50%, MT has been contraindicated in moderate and severe renal impairment since its FDA approval in patients with normal renal function or mild renal insufficiency to minimize the potential for toxic MT levels and MALA. However, the reported incidence of MALA in clinical practice has proved to be very low. Several groups have suggested that current renal function cutoffs for MT are too conservative, thus depriving a substantial number of type 2 diabetes patients from the potential benefit of MT therapy. In keeping with these data the FDA and the EMA recently revised the warning regarding MT. Considering these new recommendations the authors propose a territorial diagnostic and therapeutic path on the use of MT in the Chronic Kidney Disease., non disponibile

Although Metformin (MT) is a first-line therapy for the treatment of type 2 diabetes mellitus, some patients may not receive it owing to the risk of MALA (metformin-associated lactic acidosis), an exceptionally rare but fatal adverse event. Then, the drug is contraindicated in many individuals with impaired kidney function because of concerns of MALA. MT, along with other drugs in the biguanide class, increases plasma lactate levels in a plasma concentration-dependent manner by inhibiting mitochondrial respiration predominantly in the liver. Elevated plasma MT concentrations (as occur in individuals with renal impairment) and a secondary event or condition that further disrupt lactate production or clearance (e.g., acute illness in diabetic patients where cardiac, hepatic, pulmonary, or renal function are compromised), are typically necessary to cause MALA. As these secondary events may be unpredictable and the mortality rate for MALA approaches 50%, MT has been contraindicated in moderate and severe renal impairment since its FDA approval in patients with normal renal function or mild renal insufficiency to minimize the potential for toxic MT levels and MALA. However, the reported incidence of MALA in clinical practice has proved to be very low. Several groups have suggested that current renal function cutoffs for MT are too conservative, thus depriving a substantial number of type 2 diabetes patients from the potential benefit of MT therapy. In keeping with these data the FDA and the EMA recently revised the warning regarding MT. Considering these new recommendations the authors propose a territorial diagnostic and therapeutic path on the use of MT in the Chronic Kidney Disease., non disponibile

Although Metformin (MT) is a first-line therapy for the treatment of type 2 diabetes mellitus, some patients may not receive it owing to the risk of MALA (metformin-associated lactic acidosis), an exceptionally rare but fatal adverse event. Then, the drug is contraindicated in many individuals with impaired kidney function because of concerns of MALA. MT, along with other drugs in the biguanide class, increases plasma lactate levels in a plasma concentration-dependent manner by inhibiting mitochondrial respiration predominantly in the liver. Elevated plasma MT concentrations (as occur in individuals with renal impairment) and a secondary event or condition that further disrupt lactate production or clearance (e.g., acute illness in diabetic patients where cardiac, hepatic, pulmonary, or renal function are compromised), are typically necessary to cause MALA. As these secondary events may be unpredictable and the mortality rate for MALA approaches 50%, MT has been contraindicated in moderate and severe renal impairment since its FDA approval in patients with normal renal function or mild renal insufficiency to minimize the potential for toxic MT levels and MALA. However, the reported incidence of MALA in clinical practice has proved to be very low. Several groups have suggested that current renal function cutoffs for MT are too conservative, thus depriving a substantial number of type 2 diabetes patients from the potential benefit of MT therapy. In keeping with these data the FDA and the EMA recently revised the warning regarding MT. Considering these new recommendations the authors propose a territorial diagnostic and therapeutic path on the use of MT in the Chronic Kidney Disease., non disponibile

Although Metformin (MT) is a first-line therapy for the treatment of type 2 diabetes mellitus, some patients may not receive it owing to the risk of MALA (metformin-associated lactic acidosis), an exceptionally rare but fatal adverse event. Then, the drug is contraindicated in many individuals with impaired kidney function because of concerns of MALA. MT, along with other drugs in the biguanide class, increases plasma lactate levels in a plasma concentration-dependent manner by inhibiting mitochondrial respiration predominantly in the liver. Elevated plasma MT concentrations (as occur in individuals with renal impairment) and a secondary event or condition that further disrupt lactate production or clearance (e.g., acute illness in diabetic patients where cardiac, hepatic, pulmonary, or renal function are compromised), are typically necessary to cause MALA. As these secondary events may be unpredictable and the mortality rate for MALA approaches 50%, MT has been contraindicated in moderate and severe renal impairment since its FDA approval in patients with normal renal function or mild renal insufficiency to minimize the potential for toxic MT levels and MALA. However, the reported incidence of MALA in clinical practice has proved to be very low. Several groups have suggested that current renal function cutoffs for MT are too conservative, thus depriving a substantial number of type 2 diabetes patients from the potential benefit of MT therapy. In keeping with these data the FDA and the EMA recently revised the warning regarding MT. Considering these new recommendations the authors propose a territorial diagnostic and therapeutic path on the use of MT in the Chronic Kidney Disease., non disponibile

Background: Pregnancy during dialysis is increasingly being reported and represents a debated point in Nephrology. The small number of cases available in the literature makes evidence-based counselling difficult, also given the cultural sensitivity of this issue. Hence, the need for position statements to highlight the state of the art and propose the unresolved issues for general discussion. Methods: A systematic analysis of the literature (MESH, Emtree and free terms on pregnancy and dialysis) was conducted and expert opinions examined (Study Group on Kidney and Pregnancy; experts involved in the management of pregnancy in dialysis in Italy 2000–2013). Questions regarded: timing of dialysis start in pregnancy; mode of treatment, i.e. peritoneal dialysis (PD) versus haemodialysis (HD); treatment schedules (for both modes); obstetric surveillance; main support therapies (anaemia, calcium-phosphate parathormone; acidosis); counselling tips. Main results: Timing of dialysis start is not clear, considering also the different support therapies; successful pregnancy is possible in both PD and HD; high efficiency and strict integration with residual kidney function are pivotal in both treatments, the blood urea nitrogen test being perhaps a useful marker in this context. To date, long-hour HD has provided the best results. Strict, personalized obstetric surveillance is warranted; therapies should be aimed at avoiding vitamin B12, folate and iron deficits, and at correcting anaemia; vitamin D and calcium administration is safe and recommended. Women on dialysis should be advised that pregnancy is possible, albeit rare, with both types of dialysis treatment, and that a success rate of over 75 % may be achieved. High dialysis efficiency and frequent controls are needed to optimize outcomes.

Background Pregnancy on dialysis is increasingly being reported. This study evaluates the behavioural profile of the children of mothers on dialysis and the parental stress their mothers undergo when compared with a group of mothers affected by a different chronic disease (microcythaemia) and a group of healthy control mothers. Methods Between 2000 and 2012, 23 on-dialysis mothers gave birth to 24 live-born children in Italy (23 pregnancies, 1 twin pregnancy, one of the twins deceased soon after delivery); of these, 16 mothers and 1 father (whose wife died before the inquiry) were included in the study (1 mother had died and the father was unavailable; 2 were not asked to participate because their children had died and 3 were unavailable; children: median age: 8.5, min-max: 2-13 years). Twenty-three mothers affected by transfusion-dependent microcythaemia or drepanocitosis (31 pregnancies, 32 children) and 35 healthy mothers (35 pregnancies, 35 children; median age of the children: 7, min-max: 1-13 years) were recruited as controls. All filled in the validated questionnaires: 'Child Behaviour Checklist' (CBCL) and the 'Parental Stress Index-Short Form' (PSI-SF). Results The results of the CBCL questionnaire were similar for mothers on dialysis and healthy controls except for pervasive developmental problems, which were significantly higher in the dialysis group, while microcythaemia mothers reported higher emotional and behavioural problems in their children in 8 CBCL sub-scales. Two/16 children in the dialysis and 3/32 in the microcythaemia group had pathological profiles, as assessed by T-scores (p: ns). PSI-SF indicated a normal degree of parental stress in microcythaemia subjects and healthy controls, while mothers on dialysis declared significantly lower stress, suggesting a defensive response in order to minimize problems, stress or negativity in their relationship with their child. Conclusions According to the present analysis, the emotional and behavioural o

Un numero sempre maggiore di pazienti anziani inizia la dialisi. Tuttavia, malgrado i potenziali vantaggi della DP rispetto all'HD, tale tecnica è sottoutilizzata, specialmente nell'anziano. È, di recente, comparso un dibattito sulla rivista Seminars in Dialysis, relativamente al fatto di considerare o meno la DP come terapia sostitutiva di prima scelta per il paziente anziano., An increasing number of elderly patients start dialysis. Despite peritoneal dialysis' potential benefits this technique is underutilized, particularly in the elderly. Recently, a debate entitled “Should peritoneal dialysis be the first option offered to the elderly who require renal re-placement therapy for continued longevity?” was published on Seminars in Dialysis.

Un numero sempre maggiore di pazienti anziani inizia la dialisi. Tuttavia, malgrado i potenziali vantaggi della DP rispetto all'HD, tale tecnica è sottoutilizzata, specialmente nell'anziano. È, di recente, comparso un dibattito sulla rivista Seminars in Dialysis, relativamente al fatto di considerare o meno la DP come terapia sostitutiva di prima scelta per il paziente anziano., An increasing number of elderly patients start dialysis. Despite peritoneal dialysis' potential benefits this technique is underutilized, particularly in the elderly. Recently, a debate entitled “Should peritoneal dialysis be the first option offered to the elderly who require renal re-placement therapy for continued longevity?” was published on Seminars in Dialysis.

An increasing number of elderly patients start dialysis. Despite peritoneal dialysis' potential benefits this technique is underutilized, particularly in the elderly. Recently, a debate entitled “Should peritoneal dialysis be the first option offered to the elderly who require renal re-placement therapy for continued longevity?” was published on Seminars in Dialysis., Un numero sempre maggiore di pazienti anziani inizia la dialisi. Tuttavia, malgrado i potenziali vantaggi della DP rispetto all'HD, tale tecnica è sottoutilizzata, specialmente nell'anziano. È, di recente, comparso un dibattito sulla rivista Seminars in Dialysis, relativamente al fatto di considerare o meno la DP come terapia sostitutiva di prima scelta per il paziente anziano.

An increasing number of elderly patients start dialysis. Despite peritoneal dialysis' potential benefits this technique is underutilized, particularly in the elderly. Recently, a debate entitled “Should peritoneal dialysis be the first option offered to the elderly who require renal re-placement therapy for continued longevity?” was published on Seminars in Dialysis., Un numero sempre maggiore di pazienti anziani inizia la dialisi. Tuttavia, malgrado i potenziali vantaggi della DP rispetto all'HD, tale tecnica è sottoutilizzata, specialmente nell'anziano. È, di recente, comparso un dibattito sulla rivista Seminars in Dialysis, relativamente al fatto di considerare o meno la DP come terapia sostitutiva di prima scelta per il paziente anziano.

An increasing number of elderly patients start dialysis. Despite peritoneal dialysis' potential benefits this technique is underutilized, particularly in the elderly. Recently, a debate entitled “Should peritoneal dialysis be the first option offered to the elderly who require renal re-placement therapy for continued longevity?” was published on Seminars in Dialysis., Un numero sempre maggiore di pazienti anziani inizia la dialisi. Tuttavia, malgrado i potenziali vantaggi della DP rispetto all'HD, tale tecnica è sottoutilizzata, specialmente nell'anziano. È, di recente, comparso un dibattito sulla rivista Seminars in Dialysis, relativamente al fatto di considerare o meno la DP come terapia sostitutiva di prima scelta per il paziente anziano.

La diagnosi differenziale nei casi d'insufficienza renale acuta del postpartum associata ad anemia emolitica microangiopatica e trombocitopenia, include, tra le altre: pre-clampsia grave/eclampsia, grave eclampsia, la sindrome HELLP (Hemolysis, Elevated Liver enzyme, Low Platelet), la acute fatty liver of pregnancy (AFLP), la porpora trombotica trombocitopenica/sindrome emolitico-uremica associata alla gravidanza (TTP/aHUS), esordio acuto o flare di LES in gravidanza e la sindrome catastrofica da anticorpi antifosfolipidi (CAPS). Si tratta di condizioni potenzialmente pericolose per la vita data la presenza di disfunzione multiorgano. Il verificarsi di uno stato di ipercoagulabilità e la concentrazione decrescente di ADAMTS 13 in gravidanza e nel post-parto aumentano il rischio di sviluppare porpora trombotica trombocitopenica (TTP). Vi è però una notevole sovrapposizione riguardo la clinica ed i test di laboratorio tra queste condizioni, e quindi la diagnosi può essere un problema anche per clinici esperti. Tuttavia è importante stabilire un'accurata diagnosi poiché la gestione e le complicanze di tali sindromi possono essere differenti. Il caso presentato sottolinea la complessità connessa alla diagnosi differenziale dei quadri clinici che includono anemia emolitica microangiopatica e trombocitopenia connessi alla gravidanza ed il ruolo del plasma exchange nella loro gestione., The differential diagnosis of postpartum acute renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia includes, among others: severe preeclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzyme, low platelet), acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura/hemolytic uremic syndrome associated with pregnancy (TTP/aHUS), acute onset or flare of SLE in pregnancy and catastrophic antiphospholipid syndrome (CAPS). These conditions are potentially life threatening due to the presence of multi-organ dysfunction. The occurrence of an hypercoagulable state with decreasing concentration of ADAMTS 13 in pregnancy and in postpartum increases the risk of developing thrombotic thrombocytopenic purpura (TTP). Yet there is a considerable overlap of the clinical and laboratory tools detecting these conditions, therefore the diagnosis may be problematic even for experienced clinicians. However, it is important to establish an accurate diagnosis as the management and complications of these syndromes may be different. This case highlights the complexity of the differential diagnosis in case of microangiopathic hemolytic anemia and thrombocytopenia associated with pregnancy and the role of plasma exchange in their management.

The differential diagnosis of postpartum acute renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia includes, among others: severe preeclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzyme, low platelet), acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura/hemolytic uremic syndrome associated with pregnancy (TTP/aHUS), acute onset or flare of SLE in pregnancy and catastrophic antiphospholipid syndrome (CAPS). These conditions are potentially life threatening due to the presence of multi-organ dysfunction. The occurrence of an hypercoagulable state with decreasing concentration of ADAMTS 13 in pregnancy and in postpartum increases the risk of developing thrombotic thrombocytopenic purpura (TTP). Yet there is a considerable overlap of the clinical and laboratory tools detecting these conditions, therefore the diagnosis may be problematic even for experienced clinicians. However, it is important to establish an accurate diagnosis as the management and complications of these syndromes may be different. This case highlights the complexity of the differential diagnosis in case of microangiopathic hemolytic anemia and thrombocytopenia associated with pregnancy and the role of plasma exchange in their management., La diagnosi differenziale nei casi d'insufficienza renale acuta del postpartum associata ad anemia emolitica microangiopatica e trombocitopenia, include, tra le altre: pre-clampsia grave/eclampsia, grave eclampsia, la sindrome HELLP (Hemolysis, Elevated Liver enzyme, Low Platelet), la acute fatty liver of pregnancy (AFLP), la porpora trombotica trombocitopenica/sindrome emolitico-uremica associata alla gravidanza (TTP/aHUS), esordio acuto o flare di LES in gravidanza e la sindrome catastrofica da anticorpi antifosfolipidi (CAPS). Si tratta di condizioni potenzialmente pericolose per la vita data la presenza di disfunzione multiorgano. Il verificarsi di uno stato di ipercoagulabilità e la concentrazione decrescente di ADAMTS 13 in gravidanza e nel post-parto aumentano il rischio di sviluppare porpora trombotica trombocitopenica (TTP). Vi è però una notevole sovrapposizione riguardo la clinica ed i test di laboratorio tra queste condizioni, e quindi la diagnosi può essere un problema anche per clinici esperti. Tuttavia è importante stabilire un'accurata diagnosi poiché la gestione e le complicanze di tali sindromi possono essere differenti. Il caso presentato sottolinea la complessità connessa alla diagnosi differenziale dei quadri clinici che includono anemia emolitica microangiopatica e trombocitopenia connessi alla gravidanza ed il ruolo del plasma exchange nella loro gestione.

The differential diagnosis of postpartum acute renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia includes, among others: severe preeclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzyme, low platelet), acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura/hemolytic uremic syndrome associated with pregnancy (TTP/aHUS), acute onset or flare of SLE in pregnancy and catastrophic antiphospholipid syndrome (CAPS). These conditions are potentially life threatening due to the presence of multi-organ dysfunction. The occurrence of an hypercoagulable state with decreasing concentration of ADAMTS 13 in pregnancy and in postpartum increases the risk of developing thrombotic thrombocytopenic purpura (TTP). Yet there is a considerable overlap of the clinical and laboratory tools detecting these conditions, therefore the diagnosis may be problematic even for experienced clinicians. However, it is important to establish an accurate diagnosis as the management and complications of these syndromes may be different. This case highlights the complexity of the differential diagnosis in case of microangiopathic hemolytic anemia and thrombocytopenia associated with pregnancy and the role of plasma exchange in their management., La diagnosi differenziale nei casi d'insufficienza renale acuta del postpartum associata ad anemia emolitica microangiopatica e trombocitopenia, include, tra le altre: pre-clampsia grave/eclampsia, grave eclampsia, la sindrome HELLP (Hemolysis, Elevated Liver enzyme, Low Platelet), la acute fatty liver of pregnancy (AFLP), la porpora trombotica trombocitopenica/sindrome emolitico-uremica associata alla gravidanza (TTP/aHUS), esordio acuto o flare di LES in gravidanza e la sindrome catastrofica da anticorpi antifosfolipidi (CAPS). Si tratta di condizioni potenzialmente pericolose per la vita data la presenza di disfunzione multiorgano. Il verificarsi di uno stato di ipercoagulabilità e la concentrazione decrescente di ADAMTS 13 in gravidanza e nel post-parto aumentano il rischio di sviluppare porpora trombotica trombocitopenica (TTP). Vi è però una notevole sovrapposizione riguardo la clinica ed i test di laboratorio tra queste condizioni, e quindi la diagnosi può essere un problema anche per clinici esperti. Tuttavia è importante stabilire un'accurata diagnosi poiché la gestione e le complicanze di tali sindromi possono essere differenti. Il caso presentato sottolinea la complessità connessa alla diagnosi differenziale dei quadri clinici che includono anemia emolitica microangiopatica e trombocitopenia connessi alla gravidanza ed il ruolo del plasma exchange nella loro gestione.

The differential diagnosis of postpartum acute renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia includes, among others: severe preeclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzyme, low platelet), acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura/hemolytic uremic syndrome associated with pregnancy (TTP/aHUS), acute onset or flare of SLE in pregnancy and catastrophic antiphospholipid syndrome (CAPS). These conditions are potentially life threatening due to the presence of multi-organ dysfunction. The occurrence of an hypercoagulable state with decreasing concentration of ADAMTS 13 in pregnancy and in postpartum increases the risk of developing thrombotic thrombocytopenic purpura (TTP). Yet there is a considerable overlap of the clinical and laboratory tools detecting these conditions, therefore the diagnosis may be problematic even for experienced clinicians. However, it is important to establish an accurate diagnosis as the management and complications of these syndromes may be different. This case highlights the complexity of the differential diagnosis in case of microangiopathic hemolytic anemia and thrombocytopenia associated with pregnancy and the role of plasma exchange in their management., La diagnosi differenziale nei casi d'insufficienza renale acuta del postpartum associata ad anemia emolitica microangiopatica e trombocitopenia, include, tra le altre: pre-clampsia grave/eclampsia, grave eclampsia, la sindrome HELLP (Hemolysis, Elevated Liver enzyme, Low Platelet), la acute fatty liver of pregnancy (AFLP), la porpora trombotica trombocitopenica/sindrome emolitico-uremica associata alla gravidanza (TTP/aHUS), esordio acuto o flare di LES in gravidanza e la sindrome catastrofica da anticorpi antifosfolipidi (CAPS). Si tratta di condizioni potenzialmente pericolose per la vita data la presenza di disfunzione multiorgano. Il verificarsi di uno stato di ipercoagulabilità e la concentrazione decrescente di ADAMTS 13 in gravidanza e nel post-parto aumentano il rischio di sviluppare porpora trombotica trombocitopenica (TTP). Vi è però una notevole sovrapposizione riguardo la clinica ed i test di laboratorio tra queste condizioni, e quindi la diagnosi può essere un problema anche per clinici esperti. Tuttavia è importante stabilire un'accurata diagnosi poiché la gestione e le complicanze di tali sindromi possono essere differenti. Il caso presentato sottolinea la complessità connessa alla diagnosi differenziale dei quadri clinici che includono anemia emolitica microangiopatica e trombocitopenia connessi alla gravidanza ed il ruolo del plasma exchange nella loro gestione.