PGNMID and anti-CD38 monoclonal antibody: a therapeutic challenge.

Monoclonal gammopathy of renal significance (MGRS) designates disorders induced by a monoclonal protein secreted by plasma cells or B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma or other B-cell malignancies. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a form MGRS. Until now, no guidelines to decide the best therapeutic approach to manage PGNMID exist, and most patients progress to End Stage Renal Disease (ESRD) without therapy. Recently, daratumumab has showed an acceptable improvement in proteinuria and renal function in patients with PGNMID. We report the clinical outcome and the histological renal evolution and treatment complication of our patient, who was initially treated with a combination regimen including bortezomib, dexamethasone, and cyclophosphamide and then with anti-CD38 monoclonal antibody-based regimen.

[Efficacy of eculizumab in a case of pregnancy-associated aHUS]. / Efficacia di eculizumab in un caso di SEU atipica associata alla gravidanza.

Pregnancy-associated thrombotic microangiopathy (TMA) is a rare condition, but it is burdened by a significant perinatal and maternal morbidity as well as mortality. We describe the case of a 33-year-old woman, who developed a TMA at the 36th week of gestation characterized by increased LDH, haptoglobin consumption, schistocytes, thrombocytopenia and acute renal failure requiring dialysis. There were not gestational hypertension nor proteinuria until the day of hospitalization. ADAMTS 13 deficiency was ruled out and the patient did not have diarrhea. She was initially treated with caesarean section, plasma infusion and plasmapheresis with no benefit. Five days after the onset of TMA, a temptative diagnosis of atypical uremic syndrome (aHUS) was made and the patient was switched to eculizumab. Antibiotic prophylaxis and anti-meningococcal A,B, C, W135 and Y vaccination was performed. TMA rapidly resolved and renal function completely recovered. The newborn had a normal perinatal course. A complement dysregulation was ruled out by testing for mutations on CFH, CFHR3-R1, CFI, MCP, CFB, C3 and for anti CFH antibodies. In conclusion the differential diagnosis of aHUS with HELLP syndrome is often not straightforward. The severity and persistence of TMA, the high mortality associated to peripartum TMA and the risk for irreversible kidney failure require an early therapeutic decision as to the use of eculizumab.

Intraperitoneal hydrostatic pressure and flow characteristics of peritoneal catheters in automated peritoneal dialysis.

BACKGROUND: In automated peritoneal dialysis (APD) one of the most important factors that influence the efficiency of the treatment is the total volume of dialysate infused per session and the dwell time. This study is aimed at examining the relationships between i.p. pressure (IPP), dialysate flow characteristics, and different dialysate fill volumes in order to optimize APD. METHODS: We studied 20 patients who received APD, with the standard fill volume (2 l, A), or individualized fill volumes based on the patient's body surface area (2.5 l/BSA/1.73 m, B) or on body weight (40 ml/kg body weight, C). The patient's tolerance to a given fill volume was evaluated by measuring IPP, and catheter flow characteristics were evaluated by an automated machine. RESULTS: IPP increased with the increase of the infused volume of dialysate (P < 0.05) and tended towards a positive relationship with the patient's body mass index (BMI: A vs IPP: R = 0.39, P = 0.0019; B vs IPP: R = 0.66, P = 0.0012; C vs IPP R = 0.55, P = 0.009). We also found a relationship between fill volume, BMI and IPP: IPP = 1.0839 + 0.53 (beta) x BMI + 0.211 (beta) x fill volume (R = 0.65; r(2) = 0.40 P < 0.01). The mean IPP with different dialysate fill volumes tended to be related to the volume of dialysate drained at the transition point (R = 0.37; P < 0.05). The pre-transition flow rate/mean IPP ratio tended towards a positive relationship with the volume of dialysate drained at the transition point (R = 0.35, P < 0.05), the transition time (R = 0.34; P < 0.05) and a negative one with the transition volume (R = -0.35, P = 0.05). CONCLUSION: It is possible to customize APD, where the tidal percentage coincides with the transition point for a given catheter and a specific initial dialysate fill volume, the tolerance of which can be measured by assessing IPP.