Membranoproliferative glomerulonephritis with relapsing episodes of acute kidney injury in the Schnitzler syndrome

Abstract

The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years the SS has evolved from an elusive, little-known disorder to the paradigm of a late-onset auto-inflammatory acquired syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin 1 (IL-1) receptor antagonist anakinra. Here we describe the case of a 56-year-old male Caucasian patient affected by SS and hospitalized several times in our unit because of relapsing episodes of acute kidney injury. He underwent an ultrasound-guided percutaneous kidney biopsy in September 2012, which showed the histologic picture of type I membranoproliferative glomerulonephritis. He has undergone conventional therapies, including nonsteroidal anti-inflammatory drugs, steroids and immunosuppressive drugs; more recently, the IL-1 receptor antagonist anakinra has been prescribed, with striking clinical improvement. Although the literature regarding kidney involvement in the SS is lacking, it can however be so severe, as in the case reported here, to lead us to recommend the systematic search of nephropathy markers in the SS.

 

Keywords: Acute kidney injury, auto-inflammatory diseases, chronic urticarial rash, membranoproliferative glomerulonephritis, monoclonal IgM gammopathy, Schnitzler syndrome

Sorry, this entry is only available in Italian.

Acute kidney injury in elderly patients

Abstract

In the last few years, more and more studies have been focusing on Acute Kidney Injury (AKI) because of its incidence, its effects on patients, and the costs associated to its treatment. Elderly patients are especially in danger of developing AKI given the para-physiological deterioration of their renal functions and the presence of several co-morbidities. Together with sepsis, AKI is in fact one of the most common complications occurring during hospitalization. However, some strategies have proved to help in preventing renal damage in the elderly.

Fluid overload, malnutrition and sepsis, which are far more common and momentous in older patients, can conceal or minimize rising creatinine levels. As such, it is not always possible to rely on the creatinine behaviour to diagnose AKI as proposed by KDIGO, AKIN e RIFLE guidelines. On the one hand, these and other peculiarities make AKI very difficult to diagnose and treat in elderly patients. On the other, the dialysis treatment, with its intrinsic “un-physiology”, further complicates matters and (if it is very aggressive in terms of efficiency and removal of fluids) can destabilize these fragile patients and their poor haemodynamic compensation.

 

Keywords: acute kidney injury, comorbidity, elderly patients, renal functional decline, dialysis

Sorry, this entry is only available in Italian.

Carnitin-Palmitoyl Transferase type 2 deficiency: a rare cause of acute renal failure due to rhabdomyolysis

Abstract

Fatty acid oxidation disorders are inborn errors of metabolism. One of the possible alterations involves the failure of the carnitin-based transport of long-chain fatty acids into the mitochondria, necessary for muscle metabolism in case of prolonged physical exertion. Three kinds of Carnitin-Palmitoyl Transferase type 2 (CPT2) deficiency have been described: a myopathic form, a severe infantile form and a neonatal form. The clinical picture is characterized by recurrent attacks of rhabdomyolysis, muscular pains and fatigue, secondary to a prolonged physical exercise and sometimes aggravated by intercurrent events. Rhabdomyolysis episodes are associated with a significant increase in creatine phosphokinase and myoglobinuria and may result in acute renal failure. Patients are usually asymptomatic during intercurrent periods. When acute renal failure from rhabdomyolysis arises after intense physical activity, it is therefore necessary to also investigate the presence of metabolic myopathies due to enzymatic deficiency.

Keywords: deficit CPT2, rhabdomyolysis, acute renal failure

Sorry, this entry is only available in Italian.

Protected: Carnitin-Palmitoyl Transferase type 2 deficiency: a rare cause of acute renal failure due to rhabdomyolysis

Abstract

Fatty acid oxidation disorders are inborn errors of metabolism. One of the possible alterations involves the failure of the carnitin-based transport of long-chain fatty acids into the mitochondria, necessary for muscle metabolism in case of prolonged physical exertion. Three kinds of Carnitin-Palmitoyl Transferase type 2 (CPT2) deficiency have been described: a myopathic form, a severe infantile form and a neonatal form. The clinical picture is characterized by recurrent attacks of rhabdomyolysis, muscular pains and fatigue, secondary to a prolonged physical exercise and sometimes aggravated by intercurrent events. Rhabdomyolysis episodes are associated with a significant increase in creatine phosphokinase and myoglobinuria and may result in acute renal failure. Patients are usually asymptomatic during intercurrent periods. When acute renal failure from rhabdomyolysis arises after intense physical activity, it is therefore necessary to also investigate the presence of metabolic myopathies due to enzymatic deficiency.

 

Keywords: deficit CPT2, rhabdomyolysis, acute renal failure

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Efficacy of SUPRA HFR in the treatment of acute renal damage during multiple myeloma

Abstract

Acute Kidney Injury (AKI) is a frequent complication of multiple myeloma (MM) with unfavorable prognostic significance.

Light chains removal, combined with hematological therapy (CT) seems to offer significant benefits to renal function recovery (RFR).

The SUPRA HFR, through the combination of high cut-off membrane without albumin loss and adsorbent cartridge, represents one of the “emerging” light chain removal methods. We report our multicentric retrospective experience with SUPRA HFR in 7 MM patients.

At the end of the treatment with SUPRA HFR a significant reduction in serum free light chains compared to baseline was observed (min 24%; max 90%; median 74%). Despite a not always early start of the treatment, all patients recovered renal function with withdrawal from dialysis in 6/7 cases.

Our preliminary experience of a combination of SUPRA HFR treatment with CT in 7 MM patients with AKI showed a significative renale functional recovery, with favourable cost/benefit ratio and a simple treatment schedule. These encouraging data suggest to further extend such treatment option, waiting for larger studies in this field.

 

Keywords:

Acute Kidney Injury, haemodiafiltration, multiple myeloma

Sorry, this entry is only available in Italian.

Atypical Hemolytic Uremic Syndrome: experience of a pediatric center

Abstract

In the last two years we admitted in our Hospital  38 children with acute renal failure (ARF). Six of them were affected by hemolytic uremic syndrome (HUS) atypical. The aHUS is diagnosed in the presence of thrombotic microangiopathy (MAT), renal insufficiency (GFR 5%).

The clinical presentation of our children has been varied and so also its evolution. Patients observed were all male, aged 2 to 12 years, and no one had a family history of kidney disease. In four patients we documented alterations of complement factors (MCP deficiency and factor H and presence of anti factor H). Repeated blood transfusions were required in 4 patients and in 3 patients the platelet count was slightly reduced. In 5 patients we did plasmapheresis and in 3 patients dialysis (hemodialysis and peritoneal dialysis). In three patients in whom the diagnosis was not clear, renal biopsy was performed to confirm the diagnosis. Eculizumab was administered in 3 patients resistant to plasma exchange. We obtain a rapid response on MAT with normalization of platelet count. The effect on renal function was variable (complete remission in a patient, partial improvement in another, and unresponsiveness in the last). The last had on Kidney biopsy signs of severe impairment and we documented the presence of antibodies to eculizumab. HUS is a rare condition, but probably much more common than reported. In children with ARF and microangiopathic anemia is necessary evaluated  complement factors as early to obtain an improved clinical response to treatment with eculizumab.

Keywords: atypical hemolytic uremic syndrome, acute renal failure, pediatric, eculizumab.

Sorry, this entry is only available in Italian.

Extra-hepatic manifestations associated with Hepatitis and virus infection

Abstract

Hepatitis E virus (HEV) is a significant public health problem that affects almost 20 million individuals annually and cause acute liver injury in 3,5 million. Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestation. The spectrum of these manifestation is still emerging. Acute pancreatitis and neurological, renal, hematologic, and muscoloskeletal manifestations have been described. Renal injury include membranoproliferative glomerulonephritis with or without cryoglobulinemia, membranous glomerulonephritis and tubular necrosis. The etiopathogenesis of extrahepatic manifestation is only supposed. It could be caused by a direct tossic effect of HEV or by an autoimmune process. We report a case of a 46 years old man who presented with acute hepatitis E. He was diagnosed to have acute severe renal failure and severe pancreatitis due to hepatitis E. Few cases have been reported in the literature concerning patients suffering from hepatitis E and severe extraepatic manifestations with a benign course and complete recovery.

Key words: hepatitis E, extrahepatic manifestation, renal injury

Sorry, this entry is only available in Italian.

Acute kidney injury and rhabdomyolysis after cocaine overdose: case report and literature review

Abstract

Cocaine, a natural alkaloid derived from the coca plant, is one of the most commonly used illicit drugs.
Cocaine abuse causes systemic adverse effects like stroke, myocardic infarction, arterial dissection, vascular thrombosis and rhabdomyolysis.
Cocaine use is, also, associated with renal complications such as acute kidney injury, vasculitis, acute interstitial nephritis, chronic kidney disease, malignant hypertension with thrombotic microangiopathy.
Acute kidney injury may or may be not associated to rhabdomyolysis.
Rhabdomyolysis caused by cocaine abuse is multifactorial, involving tissue ischemia secondary to vasoconstriction and cellular damage caused by the drug.
We report a 50-year-old man with history of chronic hepatitis C and substance abuse admitted to our unit with severe rhabdomyolysis and acute kidney failure after nasal insufflation of cocaine overdose. Renal function recovered after several treatments of dialysis.
We conclude that cocaine adversely impacts kidney function; in addition cocaine and rhabdomyolysis are the double danger for acute kidney injury. Medical management of cocaine toxicity requires a multisystem approach, with close monitoring cardiac, neurological and renal function.

Keywords: Acute kidney injury, rhabdomyolysis, cocaine

Sorry, this entry is only available in Italian.

THE TREATMENT OF AKI IN NEPHROLOGY HOSPITALIZATION: THE SLE-HDF 15 LITRES IN 10 HOURS

Abstract

The AKI in intensive care has been widely treated by international and national guidelines. The treatment of AKI in patients not requiring admission in Intensive Care Unit, but often hospitalized in Nephrology Unit, it is showed of less relevance. For over 5 years we have used for the treatment of AKI of patients admitted in Nephrology Unit an intermittent slow technique, implemented in approximately 600 patients with AKI for a total of about 3000 treatments. In this study we report the clinical results obtained in 100 consecutive patients referred to our Nephrology Unit from 1st January 2014. We excluded the patients with AKI and lactic acidosis by metformin, which were treated with CVVHDF. The Dialysis Protocol provides a slow low efficiency intermittent treatment called SLE-HDF (Sustained Low Efficiency Hemo-Dia-Filtration), with 10-hour duration, 1.5 L/h dialysate for a patient up to 75 kg, 2 L/h up to 85 kg, 2.5 L/h over 85 kg. Half of the dialysate was used in convention in post and half in diffusion. Endpoints were the recovery of renal function and the survival of the patient. On each patient was calculated on at least one seat, the Kt/V urea (UKt/V).
Were studied 100 patients, 45 females and 55 males, with mean age 79.4 + 11 years. The weight was 74 kg + 18 kg at the start of treatment. The 65% of patients had diuresis < to 500 ml/24 hours. The causes of AKI were: 41% heart failure, 31 % AKI on MRC, 7% rhabdomyolysis, 6% Hepato-renal Syndrome, 4% sepsis, 11 % other causes. Major comorbidities were heart disease (63%), diabetes (50%), COPD (38%), age over 85 years, cancers 23, liver disease 16, hypotension requiring amine 15, sepsis 10. In total in the 100 patients, 512 treatments were performed, average 5.12 + 3.7. The mean UKt/V was 0.4 + 0.05 per session. The deaths were 43. Patients discharged were 57. Of these, 43 had a recovery of renal function. Fourteen patients have not recovered renal function and were admitted for chronic dialysis treatment. In conclusion, our protocol of SLE-HDF, which uses volumes of dialysate sharply lower than used in literature, has been shown to be effective in correcting the biochemical profile of the patient with AKI. The clinical results are considered satisfactory, having obtained the improvement in 57% of patients, considering that the 43 deaths, 10 were suffering from Hepato-cirrhosis and 13 from malignant neoplasm. Further studies are needed to confirm our findings. KEYWORDS: AKI, SLE-HDF, RRT

Sorry, this entry is only available in Italian.

Complement factor B mutation in atypical hemolytic uremic syndrome. Rare cause of rare disease

Abstract

Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolysis, platelet consumption and multiple organ failure with predominant renal involvement. In the most of cases (85-90%), it is associated with enteric infection due to Shiga-toxin or verocytotoxin (STEC-VTEC)-producer Escherichia coli. Rarely, in about 10-15% of cases, HUS develops in the presence of a disorder of alternative complement pathway regulation and it is defined atypical (aHUS).

We describe the case of a 65-year-old man who came to our attention with a clinical presentation of aHUS and a clinical course characterized by rapidly progressive acute renal failure (ARF), which required renal replacement treatments, and by a stable clinical picture of hematological impairment as a marker of a non-severe and self-limiting form. The clinical and laboratory course allowed us not to perform specific therapies such as plasma exchange and/or block of the complement with eculizumab. Less than two weeks after hospital admission, there was a gradual recovery of renal function with spontaneous diuresis and hematological remission.

Genetic screening has revealed a heterozygous mutation in the complement factor B (CFB) that is not described in the literature and therefore not yet characterized in the genotype/phenotype correlation, also for the extreme rarity of the forms associated with CFB alteration. In conclusion, the presence of a new mutation in the CFB, such as the one described in our case, is probably associated with the development of aHUS but has not led to a poor prognosis, as generally reported in the literature for known variants of the CFB.

Key words: Acute kidney injury., Atypical hemolytic uremic syndrome, Complement factor B mutations, Thrombotic microangiopathy

Sorry, this entry is only available in Italian.