Liver Transplantation for Monogenic Metabolic Diseases Involvingthe Kidney

Several metabolic monogenic diseases may be cured by liver transplantation alone (LTA) or by combined liver–kidney transplantation (CLKT) when the metabolic disease has caused end-stage renal disease. Liver transplantation may be regarded as a substitute for an injured liver or as supplying a tissue that may replace a mutant protein. Two groups of diseases should be distinguished. In the first group, the kidney tissue may be severely damaged while the liver tissue is almost normal. In this group, renal transplantation is recommended according to the degree of renal damage and liver transplantation is essential as a genetic therapy for correcting the metabolic disorder. In the second group, the liver parenchymal damage is severe. In this group, liver transplantation is essential to avoid liver failure. LTA may also avoid the progression of the renal disease; otherwise a CLKT is needed. In this review, we describe monogenic metabolic diseases involving the kidney that may have beneficial effects from LTA or CLKT. We also highlight the limitations of such procedures and the choice of alternative medical conservative treatments

Plasmapheresis in the Management of Acute Pancreatitis due to Severe Hypertriglyceridemia—Reporting New Cases

Acute pancreatitis is a potentially life-threatening disease. If the diagnosis and the treatment are not prompt, it can rapidly evolve to a medical emergency. Severe hypertriglyceridemia, defined as above 1000 mg/dl, is the third most common cause of acute pancreatitis. Conventional management includes fat dietary restriction and pharmacological treatment; however, these measures take time to be effective. Plasmapheresis seems to be an alternative and safe adjunctive therapy because it allows the rapid reduction of the trigger agent in circulation. Its use, especially in severe cases, has been increasingly reported. The authors report three cases of severe hypertriglyceridemia-induced pancreatitis in which early plasmapheresis was successfully used with other supportive clinical management

Hepatic Venous Outflow Obstruction: Suggestion of a New Classification

Hepatic venous outflow obstruction (HVOO) is common in developing countries. It is a serious condition that clinically manifests with ascites from sinusoidal hypertension, and carries the risk of high mortality or development of liver cirrhosis. In the past, the eponym Budd–Chiari Syndrome (BCS) was used synonymously for HVOO. In the West, hepatic vein (HV) thrombosis caused by prothrombotic disorders is the main cause of HVOO. In the East, obliterative disease of hepatic portion of inferior vena cava induced by bacterial infection, now renamed hepatic vena cava syndrome, is the common cause of HVOO. These two diseases with different etiology, epidemiology, and natural history are at present grouped together under BCS causing much confusion. Sinusoidal obstruction syndrome, another important cause of HVOO at the level of the sinusoid and terminal HV, was left out in the classification of HVOO. In this article, the pathophysiology of sinusoidal hypertension is described, the term BCS is redefined, and a new classification of HVOO is suggested

An Update on Hepatorenal Syndrome

Hepatorenal syndrome (HRS) is one of the many potential causes of acute kidney injury (AKI) in patients with decompensated liver disease. HRS is associated with poor prognosis and represents the end-stage of a sequence of reductions in renal perfusion induced by progressively severe hepatic injury. The pathophysiology of HRS is complex with multiple mechanisms interacting simultaneously, although HRS is primarily characterised by renal vasoconstriction. A recently revised diagnostic criteria and management algorithm for AKI has been developed for patients with cirrhosis, allowing physicians to commence treatment promptly. Vasopressor therapy and other general management, such as antibiotic prophylaxis, need to be initiated whilst patients are assessed for eligibility for transplantation. Liver transplantation remains the treatment of choice for HRS but is limited by organ shortage. Other management options, such as transjugular intrahepatic portosystemic shunt, renal replacement therapy and molecular absorbent recirculating system, may provide short-term benefit for patients not responding to medical therapy whilst awaiting transplantation. Clinicians need to be aware of the pathophysiology and management principles of HRS to provide quality care for patients with multi-organ failure

Novel Therapeutic Strategies Targeting Molecular Pathways of Cystogenesis in Autosomal Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that results from mutations in PKD1 or PKD2. The disease is characterized by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that destroy the architecture of the renal parenchyma and lead to kidney failure. Until recently, the causes and the molecular pathways that lead to cystogenesis remained obscure. In the last decade, enormous progress has been made in understanding the pathogenesis of ADPKD and the development of new therapies. The purpose of this review is to update on the promising therapies that are being developed and tested based on knowledge of recent advances in molecular and cellular targets involved in cystogenesis

Alcoholic Hepatitis and Liver Transplantation

Some authors affirm that early liver transplant (LT) provides excellent short-term survival in patients with severe alcoholic hepatitis (SAH) (1–4) and similar rates of alcohol relapse compared to patients with 6 months of abstinence. We agree with the choice of not excluding patients who manifest their decompensation with bleeding and infections (common complications of SAH) and patients with psychiatric comorbidities. Data from the literature have stated for a long time that the approach to patients with alcoholic liver disease (ALD) should be changed with no ethical or technical preconceptions. The reasons in favor of this change are as follows

Xanthogranulomatous Pyelonephritis: A Rare Presentation

Xanthogranulomatous pyelonephritis is an uncommon chronic destructive disease process of renal parenchyma, associated with recurrent urinary tract infection. It is seen predominantly in females with no age specificity. The most common symptoms are flank or abdominal pain, fever, palpable mass, and gross hematuria. The common laboratory findings are leukocytosis and anemia. Urine cultures most often reveal Escherichia coli and Proteus mirabilis. Computed tomography is the mainstay of diagnostic imaging for xanthogranulomatous pyelonephritis. Histologically, xanthogranulomatous pyelonephritis presents a granulomatous inflammatory infiltrate mainly composed of lymphocytes, plasma cells, foamy histiocytes, and multinucleated giant cells. The differential diagnosis includes clear cell renal cell carcinoma, sarcomatoid renal cell carcinoma, leiomyosarcoma, malakoplakia, tuberculosis, and interstitial nephritis. Treatment includes antibiotics and surgery. In this article, we report a case of xanthogranulomatous pyelonephritis in a 38-year-old male patient with recurrent urinary tract infection