[Extended hemoperfusion in the treatment of acute carbamazepine intoxication]. / Tratamiento con hemoperfusión prolongada en la intoxicación aguda por carbamazepina.

Carbamazepine is used in the treatment of epilepsy, and also prescribed in neuralgic pain syndromes, and certain affective disorders. Carbamazepine intoxication with suicide attempt is a relatively common clinical problem that can result in coma, respiratory depression, arrhythmia, hemodynamic instability and death. The drug's relatively high molecular weight, elevated volume of distribution and intense protein-binding render it difficult to extracorporeal removal, but published experience with hemoperfusion or hemodialysis present variable results. We describe a case report involving carbamazepine intoxication who was successfully treated with charcoal hemoperfusion. With this treatment the half-life of carbamazepine was reduced with rapid lowering of carbamazepine levels and clinical improvement. Based on our experience in this patient and a review of previously reported cases, extended charcoal hemoperfusion should be considered for serious carbamazepine intoxication because free as well as bound drug fractions are eliminated via this technique.

[MALT B cell lymphoma with kidney damage and monoclonal gammopathy: a case study and literature review]. / Linfoma de células B tipo MALT con afectación renal y gammapatía monoclonal: presentación de un caso y revisión de la literatura.

We report a case of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) involving the left kidney and simultaneous onset of a monoclonal gammopathy IgM kappa. No predisposing local inflammatory condition was identified. Following left nephrectomy, the renal specimen showed the centrocyte like cells and lymphoid cells in the lymphoepithelial lesions were positive for CD20 and CD79α. The neoplastic cells expressed monotypic cytoplasmic IgM kappa. The demonstration of bone marrow cells of B-lineage expressing the same monoclonal protein as the tumor suggested bone marrow involvement, even in the absence of identical morphology. Despite chemotherapy and rituximab treatment, clinical follow-up showed right kidney extension with high-grade transformation, and finally systemic dissemination. This case illustrates that the kidney is among the sites that may be involved by MALT B-cell lymphomas in a primary or secondary fashion, and the need for expanded investigation of the possible dissemination. We review the literature on this unusual extranodal lymphoma.

Acute spontaneous tumor lysis syndrome in a patient with Crohn's disease taking immunosuppressants.

Acute tumour lysis syndrome (TLS) is a catastrophic complication of the treatment of certain neoplastic disorders. It most commonly occurs in association with hematologic malignancies and appears a few hours to a few days after initiation of specific chemotherapy, as the result from the release of intracellular components into the bloodstream due to abrupt malignant cell death. Acute spontaneous TLS is rare, and it has been described in leukemia and lymphoma and in some patients with solid tumors prior to institution of therapy. The syndrome is characterized by hyperuricemia, hyperphosphatemia, hypocalcemia, hyperkalemia, and acute oliguric or anuric renal failure due to uric acid precipitation within the tubules (acute uric acid nephropathy) and to calcium phosphate deposition in the renal parenchyma and vessels.We report a case of acute spontaneous TLS in a patient with Crohn s disease treated with immunosuppressive drugs, who developed a plasmocytoma, in which serum uric acid concentration attained exceptionally high levels (44 mg/dL). The patient underwent acute oliguric renal failure, which required treatment with hyperhydration, urine alkalinization, urate oxidase and hemodialysis, with a fatal evolution.In conclusion, the present case report has several peculiarities: that of being one of the rare examples of spontaneous TLS, that of showing an exceptionally severe hyperuricemia, probably the highest ever reported in the literature, and that of the possible increased risk of tumours in patients with Crohn s disease taking inmunosuppressives and/or TNF antagonists.

[Effectiveness of pre-emptive hemodialysis with high-flux membranes for the treatment of life-threatening alcohol poisoning]. / Eficacia de la hemodiálisis precoz con membranas de alto flujo en el tratamiento de las intoxicaciones graves por alcoholes.

Alcohol intoxication (methanol, ethanol and ethylene glycol) may result in metabolic acidosis with increased anion gap, increased serum osmolal gap, and neurologic abnormalities ranging from drunkenness to coma, and death. The mortality and morbidity rates remain very high despite intensive care therapy. The toxicity of methanol and ethylene glycol is clearly correlated to the degree of metabolic acidosis. The established treatment of severe methanol and ethylene glycol intoxication is ethanol administration and hemodialysis (HD). By inhibiting the main metabolic pathway of methanol and ethylene glycol (alcohol dehydrogenase), ethanol prevents the formation of major toxic metabolites (formic acid, glycolic acid and oxalic acid). Conventional HD can reduce serum methanol, ethanol and ethylene glycol and its metabolites rapidly, but high-flux membranes should be capable of removing more toxic per hour of HD. In this report, we describe 14 cases of life-threatening alcohol intoxication (11 methanol, 1 ethanol, and 2 ethylene glycol) who were treated successfully with supportive care, ethanol infusion (methanol and ethylene glycol), and early HD with a high-flux dialyser. The median pH was 7.04 +/- 0.06 (range 6.60-7.33), median bicarbonate 9.9 +/- 1.9 mmol/l (range 1.4-25), and median base deficit 18.4 +/- 2.6 mmol/l (range 2-33). The median anion gap was 29.1 +/- 2.3 mmol/l (range 16-45) and the median osmolal gap was 119 +/- 47 mOsm/l (range 16-402). On admission there was an excellent linear correlation between the serum toxic alcohol concentrations and the osmolal gaps (R2 = 0.98, p = 0.0006). In all cases early HD corrected metabolic acidosis and osmolal abnormalities. The mortality was 7 % (1 from 14). We conclude that pre-emptive HD should be performed in severe intoxications to remove both the parent compound and its metabolites. The HD prescription should include a large surface area dialyser with high-flux membrane, a blood flow rate in excess of 250 ml/min, a modified bicarbonate bath enriched with phosphorus and potassium, and a long time session. The phosphorus and potassium-enriched bicarbonate-based dialysis solution used in patients with normal phosphorus and potassium serum levels avoided HD-induced hypophosphatemia and hypopotassemia. HD as implemented in these cases is a safe and very effective approach to the management of alcohol poisoning.

[Calcification in nonfunctioning transplanted kidneys]. / Calcificación del injerto renal no funcionante.

Failed renal allografts often are left in situ in patients who revert to chronic dialysis therapy or who undergo retransplantation. These organs may be the site of massive calcification despite their lack of physiological function. Calcification of an endstage renal allograft is sometimes found incidentally. We report here two patients who developed extensive calcification of the renal graft, one was on chronic hemodialysis and the other had a second renal transplantation with normal renal function. The precise pathogenesis of calcification and the factors which determine its tissue localization are unclear. Factors postulated to promote the development of metastatic calcification include an elevated calcium phosphate product, severe secondary hyperparathyroidism, aluminium toxicity and duration of dialytic therapy. In some cases local factors related with the chronic inflammatory rejection process are probably involved as well. However, the exact relative contribution of these factors remains unresolved. Unless specific clinical indications are present, transplant nephrectomy is not necessary for calcified end-stage renal allografts.

[Hemoperfusion in the treatment of acute valproic acid intoxication]. / Tratamiento con hemoperfusión de la intoxicación aguda por ácido valproico.

Valproic acid is increasingly used in the treatment of epilepsy, and also prescribed for bipolar affective disorders, schizoaffective disorders, schizophrenia and migraine prophylaxis. Valproic acid intoxication with suicide attempt is a relatively common clinical problem that can result in coma, respiratory depression, pancytopenia, hemodynamic instability and death. The drug's relatively low molecular weight, small volume of distribution and saturable protein-binding render it potentially amenable to exracorporeal removal (hemodialysis, hemoperfusion or hemofiltration ), but published experience is scarce. We describe a case report involving valproic acid intoxication with ingestion of ethanol, who was successfully treated with charcoal hemoperfusion. With this treatment the half-life of valproic acid was reduced with rapid lowering of valproic acid levels and clinical improvement. Based on our experience in this patient and a review of previously reported cases, charcoal hemoperfusion should be considered for serious valproic acid intoxication because free as well as bound drug fractions are eliminated via this technique.

[Treatment of acute lithium intoxication with high-flux haemodialysis membranes]. / Tratamiento de la intoxicación aguda por litio mediante hemodiálisis con dializadores de alta eficiencia.

Lithium carbonate is commonly prescribed for the treatment of bipolar (manic-depressive) disorders. However, because of its narrow therapeutic index an excessive elevation of serum lithium concentration, either during chronic maintenance therapy or after an acute overdose, can result in serious toxicity. In addition to supportive care, the established treatment of severe lithium toxicity is haemodialysis. Conventional haemodialysis can reduce serum lithium rapidly, but post-dialysis rebound elevations with recurrent toxicity have been documented in old publications. High-flux membranes should be capable of removing more lithium per hour of haemodialysis, but published values are not available. We report here three patients with acute lithium intoxication who were treated successfully with bicarbonate and high-flux haemodialysis membranes. Our patients presented with a severe degree of intoxication, based on the amount of drug ingested, the initial serum lithium level, the severity of neurologic symptoms and systemic manifestations. Two patients developed acute renal failure probably as a result of volume depletion since it was rapidly reversible by haemodialysis and infusion therapy. In addition, consecutive haemodialysis sessions and improvement of renal function allowed a rapid decrease in serum lithium levels without haemodynamic instability or rebound elevations in lithium concentration. The effectiveness of the procedure in these cases can be attributed to the use of bicarbonate dialysate and high-efficiency dialysers. This is the first report describing the effect of high-efficiency dialysers on lithium pharmacokinetic. Using this technique the elimination rate of lithium was found to be greater than previously reported with haemodialysis.

[Seminal vesicle cysts and infertility in autosomal dominant polycystic kidney disease]. / Quistes de las vesículas seminales e infertilidad en la poliquistosis renal autosómica dominante.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic hereditary disorder characterized by bilateral diffuse renal cysts. Extrarenal involvement is a well known manifestation of ADPKD. Cysts in the liver, pancreas, lung, spleen, oesophagus, ovary, testis, epididymis, prostate, thyroid, bladder, uterus, brain, paraespinal, and seminal vesicle have also been described. The occurrence of seminal vesicle cysts is often unrecognised. We report here a man with seminal vesicle cysts and azoospermia associated with ADPKD. Seminal vesicle cysts are not uncommon in ADPKD and in some cases it is associated with infertility. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively. Studies evaluating fertility in patients with seminal vesicle cysts and ADPKD are needed.

[Chronic renal failure secondary to uterine prolapse]. / Insuficiencia renal crónica secundaria a prolapso uterino.

Acute and chronic renal failure secondary to bilateral severe hydroureteronephrosis is a rare sequela of uterine prolapse. We report a case of neglected complete uterine prolapse in a 72-year-old patient resulting in bilateral hydroureter, hydronephrosis, and chronic renal failure. In an attempt to diminish the ureteral obstruction a vaginal pessary was used to reduce the uterine prolapse. Finally, surgical repair of prolapse by means of a vaginal hysterectomy was performed. In conclusion, all patients presenting with complete uterine prolapse should be screened to exclude urinary tract obstruction. If present, obstructive uropathy should be relieved by the reduction or repair of the prolapse before irreversible renal damage occurs.

Pathogenesis of hyperprolactinemia in uremic rats.

Experiments have been carried out in order to clarify to what extent the absence of PRL renal catabolism in experimental renal insufficiency is responsible for the high PRL circulating levels. Furthermore, the relative contribution of the glomerular filtration rate and peritubular degradation to PRL renal clearance have been assessed. Circulating PRL basal levels were measured by RIA in sham-operated and intact control rats and in three uremic rat models: urine autoinfusion, bilateral ureteral ligation, and bilateral nephrectomy. Plasma PRL basal levels (nanograms per ml; mean +/- SEM) were increased in sham-operated rats (30.3 +/- 5.1) with respect to control animals (18.5 +/- 2.7; P less than 0.05). Bilaterally nephrectomized animals (66.4 +/- 16.4) and those with bilateral ureteral ligation (69.3 +/- 15.9) developed similar hyperprolactinemia, in contrast to urine-autoinfused rats (20.2 +/- 2.1; P less than 0.005) whose hormone levels were similar to those of control animals. Creatinine levels were markedly elevated and comparable in the three uremic rat groups. The results suggest that: 1) hyperprolactinemia in rats in acute renal insufficiency is due primarily to reduced renal function; 2) PRL renal catabolism in the rat requires a certain rate of glomerular filtration; and 3) PRL peritubular degradation does not seem to be relevant in PRL catabolism by rat kidney.

Sequential development of pulmonary hemorrhage with MPO-ANCA complicating anti-glomerular basement membrane antibody-mediated glomerulonephritis.

We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 67-year-old woman with diabetes. Intensive combined immunosuppressive therapy with methylprednisolone bolus, oral prednisone, and cyclophosphamide led to negativity of anti-GBM antibodies but was not able to restore renal function. After 28 months of hemodialysis, the patient suddenly presented with pulmonary hemorrhage. In this setting, high levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and negative anti-GBM antibodies were found. Therapy with oral prednisone and cyclophosphamide led to resolution of pulmonary hemorrhage and negativity of MPO-ANCA.

Left atrial calcification in a hemodialysis patient with cor triatriatum.

Myocardial calcification is a rare manifestation of abnormal calcium metabolism seen in some patients with chronic renal failure. This report describes the transesophageal echocardiographic and spiral computed tomography (CT) findings in a young hemodialysis female with severe secondary hyperparathyroidism. These findings included calcification of the multiperforated membrane of a cor triatriatum and the wall of the left atrium.

[Partial lipodystrophy in two HLA identical sisters with hypocomplementemia and nephropathy]. / Lipodistrofia parcial en dos hermanas HLA idénticas con hipocomplementemia y nefropatía.

Partial lipodystrophy is a rare disorder with both autosomal recessive and familial forms. The cutaneous findings, which are often subtle, consist of gradual loss of subcutaneous fat from the face and upper body. Low levels of C3 and the presence of C3NeF help to identify these patients. Associated systemic abnormalities include the development of membranoproliferative glomerulonephritis, insulin resistance and an increased incidence of autoimmune diseases. We report here two HLA identical sisters with the typical features of partial lipodystrophy associated with recurrent infections, low levels of C3, and nephropathy. Our data suggest an autosomal recessive transmission. We discuss the genetic and molecular basis of this rare association.

[The spectrum of cystic kidney disease in adulthood: differential diagnosis and complications]. / El espectro de los quistes renales en el adulto: diagnóstico diferencial y complicaciones.

Simple renal cysts are the most common renal masses, accounting for roughly 65 to 70% of cases. They most often occur in patients over the age of 50 as determined from post-mortem examination or renal ultrasonography. The major concern with simple renal cysts is differentiating them from more serious disorders, such as polycystic kidney disease and solid masses such as a renal carcinoma or abscess. Renal arteriovenous malformations may present with ultrasound picture mimicking simple parapelvic cyst. Ultrasound, doppler ultrasound, computed tomography and magnetic resonance imaging are effective in documenting the underlying lesions non-invasively. Arteriography may be useful to characterise vascular lesion. We report here the spectrum of cystic kidney disease in adulthood in a group of patient with different disorders. The differential diagnosis, complications and associated process are discussed.

[Fabry disease: clinic and enzymatic diagnosis of homozygous and heterozygous. New therapeutic prospects]. / Enfermedad de Fabry: diagnóstico clínico y enzimático de homocigotos y heterocigotos. Nuevas perspectivas terapéuticas.

Fabry disease is an X-linked recessive lysosomal storage disorder caused by a partial or complete deficiency of alpha-galactosidase A. Intracellular accumulation of globotriaosylceramide, the glycolipid substrate of this enzyme, leads to severe painful neuropathy with progressive renal, cardiovascular, and cerebrovascular dysfunction and early death. Men are predominantly affected but many female carriers have similar clinical involvement, including increased risk of stroke. Physical stigmata, such as angiokeratomas in skin and mucous membranes and characteristic benign corneal abnormalities, facilitate identification of Fabry disease. The finding of a marked decreased activity of (alpha-galactosidase A in plasma, white blood cells or cultured skin fibroblasts confirms the diagnosis. Treatment thus far has been symptomatic only. Etiology-based therapies are being developed that include enzyme replacement therapy, gene therapy, and substrate deprivation. The recently completed double-blind, placebo-controlled trials of intravenous infusions of (alpha-galactosidase A in patients with Fabry disease demonstrated the safety and efficacy of this treatment. We report a family with Fabry disease composed of hemicygous and heterocygous. The propositus developed chronic renal failure and received a cadaver renal transplant, which remained with adequate renal function during 15 years.

[Association of hypercalcemia, elevated levels of calcitriol and tuberculosis in patients on hemodialysis]. / Asociación de hipercalcemia, niveles elevados de calcitriol y tuberculosis en hemodiálisis.

Hypercalcemia is associated with numerous chronic granulomatous processes and chronic infections. Increased production of calcitriol by activated macrophages has been shown to be the cause in most cases. In this article, we describe three cases of hypercalcemia associated with inappropriately elevated calcitriol levels and suppressed PTH in hemodialysis. In addition to conventional techniques for tuberculosis diagnosis we used Ligase Chain Reaction (LCR) to detect mycobacterial DNA in pleural effusion with acid-fast stain and culture negativity. Antituberculous therapy was associated with a decrease in the levels of calcium, as well as in serum calcitriol concentrations, and a substantial increase in the levels of iPTH. The serum levels of 25(OH)D3 remained unchanged. These findings suggested ectopic production of calcitriol. The discussion reviews the previously reported cases of hypercalcemia and tuberculosis that occurred during hemodialysis, and concludes that ectopic production of calcitriol by tuberculous granulomas is extremely unusual and its demonstration requires a high index of suspicion. Molecular techniques are a potentially useful approach for early and rapid diagnosis of tuberculous infection in dialysis patients.

[Amyloidosis associated with chronic granulomatous disease in a patient with a renal transplant and recurrent urinary tract infections]. / Amiloidosis asociada a enfermedad granulomatosa crónica en una paciente con un trasplante renal e infecciones urinarias de repetición.

Chronic granulomatous disease is a group of syndromes which share a defect in a component of the phagocyte NADPH-oxidase complex. Without this enzyme activity, phagocytic cells cannot produce superoxide, peroxide, and other potent microbicidal radicals, and are less able to kill ingested pathogens. The clinical picture is characterised by recurrent life-threatening bacterial and fungal infections and abnormal tissue granuloma formation. On the other hand, amyloidosis is a systemic disease with renal involvement occurring in the majority of cases. Recurrent amyloidosis is a rare but well documented event in renal transplant recipients. However, graft loss secondary to amyloidosis has been noted infrequently. In addition, de novo amyloidosis has not been previously associated with graft loss. We report here a renal transplant recipient with chronic granulomatous disease and history of recurrent urinary tract infections, who developed nephrotic syndrome and progressive renal insufficiency secondary to de novo AA amyloidosis leading to graft loss 66 months after transplantation.

[Polycystic liver disease without autosomal dominant polycystic kidney disease]. / Enfermedad poliquística hepática no asociada a poliquistosis renal autosómica dominante.

Polycystic liver disease is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. The natural history and clinical manifestations of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The occurrence of polycystic liver disease independently from polycystic kidney disease has been known for a long time. More recently, a gene for autosomal dominant polycystic liver disease has been identified on chromosome 19p 13.2-13.1. Isolated polycystic liver disease is underdiagnosed and genetically distinct from polycystic liver disease associated with ADPKD but with similar pathogenesis and clinical manifestations. We report here two men with polycystic liver disease no associated with ADPKD. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively.

[Hemolytic anemia caused by graft-versus-host reaction in ABO-nonidentical renal transplants from blood group O donors]. / Anemia hemolítica por reacción injerto contra huésped en trasplantados renales ABO no idénticos con donantes del grupo sanguíneo O.

Acute hemolytic anemia is one of the side effects associated with cyclosporin and tacrolimus therapy, and three mechanisms have been described to account for hemolytic anemia in patients receiving these drugs: drug induced hemolysis, autoimmune hemolysis and alloimmune hemolysis resulting from donor lymphocytes derived from the allograft (passenger lymphocyte syndrome). We report four cases of renal transplant recipients who developed alloimmune hemolytic anemia due to minor ABO incompatibility while under treatment with cyclosporin (two) and tacrolimus (two). The anti-erythrocyte antibodies responsible for hemolysis were of the IgG isotype and showed anti-A or anti-B specificity. These findings suggest that the hemolysis could be related to alloantibodies derived from the clonal development of donor B lymphocytes in the recipients (microchimerism). In summary, hemolytic anemia due to ABO-minor incompatibility occurs infrequently after renal transplantation. Risks are higher for patients A, B or AB blood group receiving an O blood group graft under treatment with cyclosporin or tacrolimus. Follow-up of these patients is warranted for the early detection and optimal management may be achieved by reduction of immunosuppression and change to mycophenolate mofetil.