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PubMed1996

[Indications for liver transplantation in severe amanita phalloides mushroom poisoning].

The clinical course of 12 patients with mushroom poisoning was evaluated in order to define the parameters considered to be relevant to the indication for liver transplantation. Eight patients recovered under conservative therapy; one patient died due to pre-existing, concomitant cardiopulmonary disease. In three patients transplantations had to be performed because of severe liver failure. On admission, the transplanted patients had a decreased Quick's test score and factor V value (< 10%). The peak of liver enzymes, serum bilirubin, serum creatinine, partial thromboplastin time and azotemia were not of any prognostic value. Main indications for liver transplantation were a very low initial Quick's test score and factor V value (both < 10%) and their inadequate response under substitution therapy. The development of encephalopathy and renal failure were further parameters indicating poor prognosis.

Adolescent↗
PubMed2000

Massive rhabdomyolysis and multiple organ dysfunction syndrome caused by leptospirosis.

We report a case of leptospiral infection in a 63-year-old man who acquired the infection while swimming in canals and streams in Hawaii. The patient's course was atypical in that he was anicteric and had no evidence of meningitis when he presented with fever, rapidly progressive and severe rhabdomyolysis, thrombocytopenia, acute renal failure, and respiratory distress syndrome. Although he recovered after a protracted illness, he required major life support, including mechanical ventilation and hemodialysis. Initial antimicrobial therapy was designed to cover major bacterial and atypical pathogens, including leptospires. An in-depth work-up for causes of this catastrophic illness confirmed acute leptospirosis. Although rare, leptospirosis is a potentially lethal infection classically associated with hepatitis, azotemia, and meningitis. Most patients experience self-limited illness, with fever, myalgias, and malaise followed by an immune-mediated aseptic meningitis. A small proportion develop shock and multiple organ dysfunction. Whereas myalgias are ubiquitous in leptospiral infection, and most patients show mildly elevated muscle enzymes, life-threatening rhabdomyolysis is rare. This atypical case is reported to urge clinicians to consider leptospirosis in the evaluation of a patient with cryptogenic sepsis who develops multiple organ dysfunction associated with rhabdomyolysis. Appropriate antimicrobial therapy, with penicillin or doxycycline, can be life-saving.

Hawaii↗
PubMed2002

Handport-assisted laparoscopic living-donor nephrectomy; initial experience in Taiwan.

The feasibility of handport-assisted laparoscopic living-donor nephrectomy in Taiwan was assessed by comparison with conventional open nephrectomy. Six serial patients undergoing laparoscopic living-donor nephrectomy (LLDN) were compared with six patients undergoing open donor nephrectomy. Body-mass index (BMI), operating time, hospital stay, and short-term graft function were assessed in both groups of patients. Handport-assisted LLDN was successfully attempted in all six patients. Mean ischemic time was 4.5 min in the laparoscopic group. There was no major complication in either group. Short-term graft function was good in all patients, except for one case of chronic rejection with mild azotemia in the open group. The length of stay was significantly longer in the open group, but the operation time of the laparoscopic group was much longer than that of the open group. There was no difference in the resumption of diet and in the use of narcotic analgesics in addition to patient-controlled analgesia. LLDN is a technically demanding approach. With handport assistance, the surgeons could shorten their learning curve. While initial graft function rates are equal to those of the open method, cosmesis and hospital stay are improved by the laparoscopic approach. Longer follow-up and larger patient numbers are needed to confirm these initial results in Taiwan.

Adult↗
PubMed2003

Response to Shiga toxin 1 and 2 in a baboon model of hemolytic uremic syndrome.

Post-diarrheal (D+) hemolytic uremic syndrome (HUS) is caused by Shiga-toxin (Stx)-producing Escherichia coli. There is epidemiological, cell culture, and mouse model evidence that Stx2-producing E. coli are more likely to cause HUS than strains that produce only Stx1, but this hypothesis has not been tested in a primate model of HUS. We have developed a baboon model of Stx-mediated HUS that was employed to compare the clinical, cytokine, and histological response to equal amounts of the two Shiga toxins. Animals given IV Stx2 developed progressive thrombocytopenia, hemolytic anemia, and azotemia, and urinary interleukin-6 levels rose significantly. Glomerular thrombotic microangiopathy was found at necropsy. Animals given Stx1 showed no cytokine response and no clinical, laboratory, or histological signs of HUS. Our findings from the primate model corroborate previous epidemiological, cell culture, and mouse model observations, and suggest that enteric infection with Stx2-producing E. coli is more likely to cause HUS than infection with organisms that produce only Stx1.

Anemia, Hemolytic↗
PubMed2003

Citrate anticoagulation in pediatric continuous venovenous hemofiltration.

Regional citrate anticoagulation has become a common alternative to systemic heparinization in adult continuous venovenous hemofiltration (CVVH) practice. We report our experience with the technique in critically ill children. We carried out a retrospective chart review of a 22-bed pediatric intensive care unit. CVVH with pre-filter citrate and systemic calcium replacement infusions was performed according to a strict protocol in nine consecutive critically ill children. All charts were reviewed for patient characteristics and CVVH circuit parameters, including filter survival. All complications were noted. Nurse specialists were interviewed about the practical management of citrate anticoagulation. All patient measurements of blood urea nitrogen, creatinine, sodium, ionized calcium (iCa), potassium, and bicarbonate were collected over the CVVH period. In seven patients, 12 simultaneous citrate measurements were taken from patient blood, pre-filter blood, and hemofiltrate fluid. Nine patients (mean age 8.8+/-6.8 years) were treated with CVVH and regional citrate anticoagulation for 1-14 days (mean 5.2+/-4.0 days). Of 19 filters used, 15 were replaced non-electively (mean filter survival 55.6+/-22.0 h). Control of azotemia and hyperkalemia was good. Sodium and iCa levels were well maintained. Bicarbonate levels were elevated in four patients without adverse effects. The mean systemic citrate level at equilibrium was 1.6+/-0.23 mmol/l. No systemic bleeding complications were observed. In children, regional citrate anticoagulation provides equivalent filter survival to heparin without bleeding complications. With good staff preparation, it is simple to perform and safe with respect to metabolic side effects.

Adolescent↗
PubMed2004

Snakebite-induced acute renal failure. A case report and review of the literature.

Snakebite is not an uncommon cause of acute renal failure (ARF) in developing countries. We report a12-year-old boy who presented with oliguric ARF following snakebite. He had pallor, icterus, generalized edema, hypertension, and was oliguric. Investigations revealed severe azotemia, microangiopathic hemolytic anemia, thrombocytopenia, prolonged coagulation parameters, and raised fibrin degradation products, suggesting disseminated intravascular coagulation as the cause of ARF. The patient improved with antisnake venom, dialysis, and other supportive treatment.

Acute Kidney Injury↗
PubMed2005

A case of atypical hemolytic uremic syndrome with a transient decrease in complement factor H.

We report a case of sporadic atypical hemolytic uremic syndrome (HUS) with a transient decrease in complement factor H. Referred for hemolysis and azotemia without diarrhea prodrome, this 31-month-old boy showed a decreased complement 3 (C3) and complement factor H (FH) level. However, the factor H gene (HF1) mutation was missing. After the hemolysis was controlled with plasma infusion, the C3 and FH levels recovered. The patient's renal function fully recovered and remained normal, and there was no recurrence of the HUS.

Child, Preschool↗
PubMed2001

Renal dysfunction but not cystic change is ameliorated by neonatal epidermal growth factor in bpk mice.

BALB/c mice homozygous for the bpk gene exhibit a form of autosomal recessive (AR) polycystic kidney disease (PKD) with massive collecting duct cysts, common bile duct dilation and chaotic intrahepatic bile ducts/portal triads. The combined renal and biliary pathology mimics much of the pathology seen in human ARPKD. Murine models of ARPKD generally have a reduced renal expression of epidermal growth factor (EGF) and an increased expression of EGF receptors (EGF-R). However, the role that EGF and EGF-R play in the progression of PKD has been unclear. Evidence from various model systems/ages of treatment produces conflicting results. Treating neonatal C57BL/6J-cpk mice with EGF ameliorates the renal pathology and dysfunction while treating 2- and 3-week-old bpk mice with an EGF-receptor tyrosine kinase inhibitor also ameliorates ARPKD. Therefore, to determine whether neonatal EGF treatment would accelerate or inhibit the progression of the PKD in bpk mice, we administered exogenous EGF (1 microgram/g body weight subcutaneously) daily from postnatal days 3-9 (a critical period for tubule maturation). Neonatal EGF treatment but not sham treatment retarded the development of azotemia and common bile duct dilation and the chaotic hepatic triad changes in cystic mice. However, EGF treatment neither reduced the severity of the renal cystic pathology nor reduced the degree of cystic enlargement of the kidneys. Cystic mice treated past 9 days of age died prior to their scheduled termination at 21 days of age. The role of EGF in the progression of polycystic kidney disease in bpk mice is relatively complicated, with neonatal treatment being associated with some amelioration of the renal dysfunction and extrarenal pathology without an effect on the renal pathology. Continuation of treatment beyond 9 days increased morbidity. Therefore, in discussing the role of EGF or EGF receptor in mediating the pathophysiology of PKD, the stage of development may be an important consideration.

Agammaglobulinaemia Tyrosine Kinase↗
PubMed1998

A bridge over troubled water...mixed water and electrolyte disorders.

Plasma volume, nitrogenous wastes in the plasma, and plasma osmolality profoundly affect the function of various organs in the body, particularly the central nervous system and the heart. Some parameters are more critical for these organs than others, so that, when homeostatic mechanisms come into play, the body often defends the parameter of greatest importance at the expense of others. The hyponatremia and azotemia that develop in dehydrated infants exemplify this prioritization of the body. The primary goal of therapeutic intervention in such circumstances is, therefore, geared toward a correction of dehydration. While alterations in plasma and urinary electrolyte patterns commonly reflect adjustments made by the body's machinery, it is essential at the bedside to identify factitiously abnormal laboratory results produced by laboratory analytical machines. They measure not only biologically active but also insignificant components of plasma electrolytes and osmolality.

Child↗
PubMed1999

Prominent medial hypertrophy of renal arterioles in an infant with hyporeninemic hypoaldosteronism.

We describe an 11-month-old boy who presented clinically with hyperkalemic renal tubular acidosis due to hyporeninemic hypoaldosteronism. Persistent hyperchloremic acidosis and mild azotemia were present. All abnormal laboratory values were corrected by the administration of fludrocortisone. Renal biopsy showed prominent medial hypertrophy of renal arterioles and interstitial fibrosis, which closely resemble those of the gene-targeted mice with disruption of the renin angiotensin system. This is the first case report raising the possibility that a defective renin angiotensin system in infancy may lead to tubulointerstitial damage with medial hypertrophy of intrarenal arterioles.

Arterioles↗
PubMed1999

Possible association of retinoic acid with bone marrow transplant nephropathy.

Bone marrow transplant (BMT) nephropathy is characterized by the acute onset of nephritis more than 100 days after BMT. The renal lesion in BMT nephropathy is similar to radiation nephritis, but BMT nephropathy occurs earlier and with lower radiation doses than radiation nephritis. The combined effects of chemotherapeutic agents and nephrotoxic drugs given before and after BMT appear to sensitize or unmask radiation nephritis. Reporting of drugs that may contribute to BMT nephropathy is critical for the development of optimal treatment regimens. Herein, we report two cases of BMT nephropathy that developed coincident with retinoic acid therapy. Both patients received autologous BMT for neuroblastoma after preparative therapy with total body irradiation/melphalan/carboplatin/etoposide. They were randomized to receive cis-retinoic acid as part of a clinical trial. Both patients developed acute nephritis during their second 2-week course of retinoic acid on post-BMT days 105 and day 139. The nephritis was associated with hypertension, anemia, thrombocytopenia, azotemia, hematuria, and proteinuria. Clinical features, laboratory evaluation, and renal biopsy indicated that these two patients developed radiation-induced BMT nephropathy. The fact that both patients developed nephritis concurrent with retinoic acid therapy raises a concern that retinoic acid may have unmasked radiation injury and triggered BMT nephropathy.

Adrenal Gland Neoplasms↗
PubMed2001

Glomerular involvement in myelodysplastic syndromes.

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. Prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.

Child↗
PubMed2001

Endovascular revascularization of renal artery stenosis in the solitary functioning kidney.

The treatment of renal artery stenosis by angioplasty and stenting is an effective and accepted alternative to surgery for the treatment of renovascular hypertension and preservation of renal function. We report the technical and clinical outcomes of renal artery stenting in patients with a solitary functioning kidney and renal artery stenosis. From October 1993 to November 1999, 30 stents were placed in the renal arteries of 27 patients (mean age 72+/-8 years) with a solitary functioning kidney and azotemia. The mean diameter renal artery stenosis was 86+/-14%. The mean preprocedure serum creatinine (Cr) level was 3.0+/-1.5 mg/dL (range 1.5-7.5 mg/dL), arterial blood pressure was 171+/-29/85+/-13 mmHg, and the number of antihypertensive drugs was 2.9+/-1.1. Indications for stenting were suboptimal balloon dilation (n = 16), intimal dissection (n = 6), and restenosis following angioplasty (n = 5). Atherosclerotic ostial lesions were present in 25 (93%) of 27 renal arteries. This represents the largest series of renal artery stenting in patients with a solitary functioning kidney, and demonstrates this treatment modality to be a relatively safe alternative to conventional surgery in this high-risk patient group. Most (74%) of the patients in this series had improved or stabilized renal function. Further efforts to define preprocedural indicators of success are necessary to identify the patients who may benefit from revascularization of their solitary kidney.

Aged↗
PubMed2003

The nephrotoxicity of Aristolochia manshuriensis in rats is attributable to its aristolochic acids.

BACKGROUND: Although Aristolochia manshuriensis (AM), which was incriminated in the Japanese variety of Chinese herbs nephropathy, has been recently shown to be nephrotoxic in rats, less is known about whether this toxicity is attributable to its aristolochic acids (AA). We compared the renal effect of AM with that of Akebia quinata (AQ), which has similar components to AM but is free of AA; we also compared this effect of AM with that of pure AA, and studied its possible mechanism. METHODS: In study 1, rats were divided into four groups. Each group was orally given either 0.4 g of AM, 4 g of AM, or 4 g of AQ per day, or vehicle, for 5 days. In study 2, rats were given 4 g of AM (which contained 4 mg of AA), or 4 mg of pure AA per day, or vehicle, for 5 days. Renal function, and renal histology were evaluated on day 5 in study 1 and on days 1, 3, 5, and 14 in study 2. In study 2, terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) and proliferating cell nuclear antigen (PCNA) staining were also performed. In study 3, rats were treated in the same way as in study 2, but were all killed on day 5. Concentrations of AA (I + II) were measured in serum and urine in study 2, and in the kidney, lung, heart, liver, and spleen in study 3. RESULTS: In study 1, only the rats that received 4 g/day of AM developed tubular necrosis, azotemia, proteinuria, and glucosuria. In study 2, both AM- and AA-treated rats showed progressive tubular damage, decreased renal function, and increased urinary protein excretion. The degree of these alterations was comparable in the AM and AA groups. Moreover, the concentrations of AA (I + II), in serum, urine, and kidney were also comparable in the AM- and AA-treated rats. High levels of AA were detected in the lung and kidney. Apoptotic tubular cells increased on day 5 and had decreased by day 14 after the withdrawal of AM or AA. Meanwhile, proliferating tubular cells progressively increased from day 3 to day 14. CONCLUSIONS: Our study indicates that the renal toxicity of AM can be attributed to its AA component. Tubular cell apoptosis might be one of the mechanisms involved in this renal injury.

Animals↗
PubMed2006

Acute renal failure in the elderly: particular characteristics.

Elderly individuals comprise the faster growing patient population group and acute renal failure (ARF) is quite common among them, although exact numbers are not known. We reviewed the literature with regards to the characteristics of ARF in elderly patients and describe some useful guidelines. The ageing kidney is characterized by many structural and functional changes, which are mainly due to various chronic disorders, such as hypertension, diabetes and atherosclerosis, which are highly prevalent in these patients. A number of structural and functional changes characteristic of the ageing kidney make elderly people especially prone to renal damage. ARF in the elderly is frequently of multifactorial origin and often with an atypical presentation, like the "intermediate syndrome", which combines characteristics of pre-renal azotemia and acute tubular necrosis. Physical examination and laboratory blood and urine indices may sometimes be misleading occasionally leading to misdiagnosis. Prophylaxis remains the preferred approach to therapy: one should avoid nephrotoxic drugs and poly-pharmacy, adjust drug doses and achieve adequate hydration of the patient as cautiously as possible. Dialysis therapies can be used for treatment of ARF irrespective of age and carry a good prognosis.

Acute Kidney Injury↗
PubMed2001

Bone disease in myeloma.

The major clinical manifestation of multiple myeloma results from osteolytic bone destruction. The only currently Food and Drug Administration-approved drug for the treatment of the bony complications of multiple myeloma is monthly intravenous pamidronate at a dose of 90 mg infused over 4 hours. Recent studies have shown the safety of 2-hour infusions. A randomized trial comparing pamidronate to placebo continued to show benefits throughout the 21-month trial. Although the duration of therapy has not been firmly determined, it is likely that discontinuation of this drug will be met by enhanced bone loss and an increased risk of bony complications for these patients. Thus, it is recommended that the drug be continued indefinitely. Support for this recommendation also comes from the reduced bone density observed in women with postmenopausal osteoporosis following the withdrawal of bisphosphonate treatment. Recent attempts to give higher doses, more frequent infusions (every 2 weeks or less), or more rapid infusions (1 hour or less) of pamidronate have occasionally been associated with albuminuria and azotemia. These modifications should therefore be avoided. Importantly, the drug can be safely administered at 90 mg monthly to patients with poor renal function. The use of pamidronate for myeloma patients without lytic bone involvement or with Durie-Salmon stages I or II disease has not been evaluated. However, it is recognized that most patients with earlier stages of disease or without lytic bone involvement also develop bony complications. There is no reason to believe that these patients would not benefit from monthly intravenous infusions of pamidronate. The potential antimyeloma effect of this agent is another reason to administer this drug in these types of patients. Thus, it is our practice to administer monthly pamidronate to myeloma patients regardless of stage or bone involvement. However, trials evaluating oral bisphosphonates have produced inconsistent clinical results, probably as a result of the erratic and scanty poor absorption as well as poor oral tolerability of these drugs. Although these oral agents may be useful in some patients, it is impossible to identify which myeloma patients will benefit from orally administered bisphosphonates. The more potent nitrogen-containing bisphosphonate zoledronic acid more effectively reverses hypercalcemia of malignancy than pamidronate, and it appears promising in reducing bone loss in cancer patients. However, its efficacy in preventing skeletal complications is still being evaluated. Many other types of new agents are in early clinical trials, but their efficacy remains unproven at the present time.

Antineoplastic Agents↗
PubMed2002

Spontaneous Bacterial Peritonitis.

Spontaneous bacterial peritonitis (SBP) is the prototypical ascitic fluid infection occurring in patients with advanced liver disease and ascites. The key to successful treatment of SBP is a knowledge of appropriate antibiotic regimens and an understanding of the setting in which infection develops, particularly those individuals at high risk for infection. A high index of suspicion should lead to early diagnostic paracentesis and ascitic fluid analysis. Treatment of SBP involves the use of non-nephrotoxic broad-spectrum antibiotics expected to cover the typical bacterial flora associated with SBP. SBP typically involves infection with a single organism, with Escherichia coli, Klebsiella spp, and Streptococcus spp responsible for nearly three fourths of cases. The treatment of choice is cefotaxime 2 g given intravenously every 8 hours for a total of 5 days. The antibiotic regimen is adjusted based on the results of ascitic fluid cultures. Other antibiotic regimens for SBP are less well studied. Given the significant morbidity and mortality rates associated with SBP, efforts to prevent its development and recurrence with antibiotic prophylaxis are warranted. The most extensively studied form of prophylaxis involves selective intestinal decontamination (SID) with the oral fluoroquinolone norfloxacin. Individuals with low-protein ascites (ascitic fluid total protein < 1g/dL) benefit from SID with norfloxacin 400 mg daily during times of hospitalization. Long-term primary prophylaxis during outpatient management of individuals awaiting liver transplantation with severe ascites and advanced liver failure should also be considered. Patients with cirrhosis and upper gastrointestinal bleeding should receive norfloxacin 400 mg twice daily for 1 week following their bleed. Those individuals surviving an episode of SBP should be treated with norfloxacin 400 mg daily until the risk of SBP is removed by definitive resolution of the ascites or liver transplantation surgery. Although the infection-related mortality associated with SBP has decreased to less than 10%, hospitalization-related mortality remains as high as 30% as a result of the severe underlying liver disease in which the infection arises and the marked generation of cytokines and nitric oxide resulting from the infection. Recently, the simultaneous administration of intravenous albumin and antibiotics for SBP has been shown to result in the decreased development of azotemia and hospitalization-related mortality. Further improvement in the outcomes of SBP will require treatments targeting this cytokine cascade rather than the development of more potent antibiotics.

Journal Article↗
PubMed2003

Treatment of Ascites.

Ascites is the most common complication of cirrhosis and occurs in more than half of all patients with cirrhosis. The development of ascites indicates progression of the underlying cirrhosis and is associated with a 50% 2-year survival rate. Conventional therapies used for the treatment of ascites include sodium restriction (<88 mmol/d), diuretics, and large volume paracentesis (LVP) (>5 L). The most effective diuretic combination is that of a potassium-sparing, distal-acting diuretic (eg, spironolactone) with a loop diuretic (eg, furosemide). LVP provides rapid resolution of symptoms with minimal complications and is well tolerated by most patients. Post-paracentesis circulatory dysfunction (PPCD) may occur after LVP and is characterized by hyponatremia, azotemia, and an increase in plasma renin activity. PPCD is associated with an increased mortality and may be prevented by administration of albumin intravenously (6 to 8 g/L of ascites removed) along with LVP. The development of either diuretic-resistant or diuretic-intractable ascites occurs in approximately 5% to 10 % of all cases of ascites. This is a poor prognostic sign, as 50% of such patients die within 6 months of its development. The only definitive therapy for refractory ascites with cirrhosis is orthotopic liver transplantation. The other options that are available include LVP, peritoneovenous shunts, and transjugular intrahepatic portosystemic shunts (TIPS). The TIPS procedure has not been shown to have any influence on survival in patients with cirrhosis and refractory ascites, and TIPS is contraindicated in patients who have advanced liver failure because it can hasten death in such individuals. Peritoneovenous shunts are associated with a high incidence of complications and frequent occlusion. They are, therefore, rarely used for refractory ascites. Spontaneous bacterial peritonitis (SBP) is a common complication of cirrhotic ascites. It may precipitate hepatorenal syndrome. The overall mortality rate from an episode of SBP is approximately 20%. Following an episode of SBP, the 1-year mortality rate approaches 70%. Hospitalized patients should be treated with intravenous third-generation cephalosporins (eg, cefotaxime), and patients at risk should receive prophylaxis with either orally administered quinolones (eg, norfloxacin) or cotrimoxazole.

Journal Article↗