1/102. Bilateral nephrectomy, peritoneal dialysis and subsequent cadaveric renal transplantation for treatment of renal failure due to polycystic kidney disease requiring continuous ventilation.We report here on a newborn with end-stage renal failure due to autosomal recessive polycystic kidney disease, also causing ventilation-requiring respiratory distress. peritoneal dialysis was able to keep the newborn alive but not wean it from the respirator. After removal of both huge kidneys, dialysis became more effective and allowed the neonate to be extubated only 5 days later. It was decided to register the baby for a pediatric cadaveric kidney transplant when it reached 6 kg/body wt or to perform a living related transplant if no such kidney became available and the baby grew to 7 kg/body wt. At the age of 9 months and a weight of 6 kg a cadaveric kidney from a 20-month-old donor became available and was transplanted extraperitoneally. Prophylactic immunosuppression included cyclosporin, mycophenolate mofetil and steroids. pneumonia on post-operative day 10 required respiratory care for several days and acute rejection requiring peritoneal dialysis. Both complications were controlled with antibiotics and conversion from cyclosporin to tacrolimus and a temporary increase in steroids. Thirteen months later the child is alive and well with a serum creatinine of 0.6 mg%. From this experience we would recommend early removal of both polycystic kidneys causing end-stage renal failure and respiratory insufficiency, starting peritoneal dialysis and performing a renal transplant as soon as possible. This therapeutic strategy seems appropriate for this complex situation.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/102. Anaphylactoid reaction to muromonab-cd3 in a pediatric renal transplant recipient.muromonab-cd3 (OKT3), a murine IgG2a antibody directed against the T3 (CD3) complex on mature lymphocytes, triggers adverse immune reactions. Anaphylactic reactions have occurred in patients exposed to OKT3 and are mediated by anti-OKT3 IgE antibodies. The reactions are not antibody mediated and can occur within seconds of administration of a mast cell secretogogue. A renal transplant recipient became hypotensive and hypoxic immediately after receiving her first dose of OKT3 and required advanced life support. serum antibody tests were negative for anti-OKT3 IgG, IgE, and antimouse protein antibodies. To our knowledge, this is the first published report of a patient with an anaphylactoid reaction to the initial infusion of OKT3.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/102. subclavian artery injury following percutaneous insertion of dialysis catheter.Iatrogenic subclavian artery injury is a rare but recognized complication of central venous catheterization. The lesion is more severe and complex to treat when produced by large catheters such as dialysis catheters. A case is presented below with a discussion of vascular access for renal replacement therapy and the principles of treating penetrating arterial trauma at this location.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/102. heparin-induced thrombocytopenia type II on hemodialysis: switch to danaparoid.We report two pediatric patients with end-stage renal failure who developed heparin-induced thrombocytopenia type II (HIT II) on hemodialysis (HD). Both developed acute respiratory distress and chest pain within 30 min of initiating the 5th HD session. The platelets dropped during HD from 168 to 38x10(9)/l and from 248 to 109x10(9)/l, respectively. Marked clots were observed in the dialyzers. Substitution of heparin with the low molecular weight heparin dalteparin had no effect. Switching from anticoagulation to the heparinoid danaparoid resulted in immediate disappearance of all adverse effects, and further long-term HD was uneventful. HIT II was diagnosed clinically; heparin-induced platelet activation test (HIPA) and serum IgG, IgA, and IgM to heparin-platelet factor 4 complexes (HPF4) were both negative. We conclude that HIT II may occur in children on HD. HIT II is essentially a clinical diagnosis, as HIPA and antibodies to HPF4 are not always positive. Once HIT II is suspected, heparin (and low-molecular-weight heparins) should be stopped immediately. Long-term anticoagulation with danaparoid is a valuable option for patients on HD.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/102. C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis.C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. Kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in earlier reports, are an early age of onset, severe crescentic glomerulonephritis, and rapid progression to ESRD. C1q nephropathy should be added to the differential diagnosis of glomerulonephritis in young children and in the patient with crescentic glomerulonephritis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/102. Renal cystic disease associated with tuberous sclerosis complex: renal failure treated by cadaveric kidney transplantation.Chronic renal failure in patients with tuberous sclerosis may be secondary to diffuse cystic disease, a lesion less common than the better known hamartomatous angiomyolipomas. uremia, in the case of a nineteen-year old female with end-stage renal disease, was associated with severely atrophic kidneys that contained numerous collapsed and scarred cysts. No hamartomas were present. The patient survived for more than three years following cadaveric renal transplantation.- - - - - - - - - - ranking = 4keywords = complex (Clic here for more details about this article) |
7/102. Electrocardiographic manifestations of hyperkalemia.hyperkalemia is one of the more common acute life-threatening metabolic emergencies seen in the emergency department. early diagnosis and empiric treatment of hyperkalemia is dependent in many cases on the emergency physician's ability to recognize the electrocardiographic manifestations of hyperkalemia. The electrocardiographic manifestations commonly include peaked T-waves, widening of the QRS-complex, and other abnormalities of altered cardiac conduction. Peaked T-waves in the precordial leads are among the most common and the most frequently recognized findings on the electrocardiogram. Other "classic" electrocardiographic findings in patients with hyperkalemia include prolongation of the PR interval, flattening or absence of the P-wave, widening of the QRS complex, and a "sine-wave" appearance at severely elevated levels. A thorough knowledge of these findings is imperative for rapid diagnosis and treatment of hyperkalemia.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
8/102. Is nephrogenic ascites related to secondary hyperparathyroidism?Nephrogenic ascites is a complex problem with a poorly understood pathophysiology. We report the case of a patient with nephrogenic ascites and concomitant secondary hyperparathyroidism who was resistant to the usual treatment but had a complete resolution after subtotal parathyroidectomy. We believe that secondary hyperparathyroidism might be implicated in the pathogenesis of nephrogenic ascites.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/102. Renal scars masquerading as complex masses in a patient with vesicoureteral reflux nephropathy.Vesicoureteral reflux can lead to chronic pyelonephritis, renal scarring, and renal failure. We present a case of renal scarring masquerading as bilateral, complex renal masses. A 35-year old woman who was diagnosed with vesicoureteral reflux as a child presented for evaluation of recently developed hypertension and an abnormal renal ultrasound. Her serum creatinine level was 2.5 mg/dL and she had subnephrotic-range proteinuria. A renal sonogram showed small, echogenic kidneys and bilateral complex renal masses of 3.8 (right) and 4.4 (left) cm in greatest dimensions. CT scan of the kidneys revealed slightly contrast-enhancing masses with irregular walls. Renal angiogram showed decreased blood supply to the areas coinciding with the masses consistent with renal scarring. There was no increased vascularity. This case demonstrates that renal scarring may masquerade as renal masses. A step-wise, comprehensive approach is necessary to rule out potentially malignant lesions in these patients.- - - - - - - - - - ranking = 6keywords = complex (Clic here for more details about this article) |
10/102. HBV-related cutaneous periarteritis nodosa in a patient 16 years after renal transplantation: efficacy of lamivudine.Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after kidney transplantation. A 44-year-old man underwent successful renal transplantation in June 1980. In December 1996 he presented multiple painful erythematous subcutaneous nodules on both legs. skin lesion biopsy showed the presence of cutaneous PAN. Six months later laboratory data demonstrated the presence of HbsAg. HBeAg, HBcAb and detectable HBV-dna serum by polymerase-chain-reaction (PCR) assay. Anti-HBs and anti-HBe proved negative. In July 1998 the laboratory tests showed an important increase of HBV-dna (5.1 billion by Branched dna), and so lamivudine (100 mg/day) was introduced. HBV-dna became undetectable by PCR after 3 months of therapy. Seven months later a new skin biopsy was performed. The typical signs of PAN were no longer evident. As HBV infecion was demonstrated six months after the clinical appearance of the PAN, in a patient who was believed to be immune to the virus, it is possible that, in the early stages, the hepatitis B antigen title was methodologically indeterminable, but sufficient to form circulating immune complexes responsible for vasculitis primer. lamivudine therapy resulted efficacious in favouring the regression of cutaneous PAN, but its long-term efficacy requires further evaluation as regards potential selection of drug resistant hepatitis b virus (HBV) mutants during treatment.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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