RESUMO
Serum sickness is an immune-complex-mediated systemic illness that can occur after treatment with monoclonal or polyclonal antibodies such as Rituxan (Rituximab) or antithymocyte globulin (Thymoglobulin), respectively. Since Rituximab is now being used as an adjuvant treatment for acute humoral rejection and its prevalence to cause serum sickness is comparable to Thymoglobulin-associated serum sickness (20% versus 27%), it should be considered a potential cause of serum sickness after rejection treatment. In kidney transplant patients, there are no case reports where patient received both Thymoglobulin and Rituximab before developing serum sickness. We are reporting a patient who developed serum sickness after receiving Thymoglobulin and Rituximab that led us to consider Rituximab as one of the potential causes in this patient's serum sickness. Since diagnosis of serum sickness is clinical, and Rituximab use has expanded into treatment of glomerulonephritis and acute humoral rejection, it should be considered as a potential offender of serum sickness in these patient populations. There are not any evidence-based guidelines or published clinical trials to help guide therapy for antibody-induced serum sickness; however, we successfully treated our case with three doses of Methylprednisone 500 mg intravenously. Further studies are needed to evaluate Rituximab-associated serum sickness in nephrology population to find effective treatment options.
RESUMO
AL amyloidosis complicating monoclonal gammopathy of undetermined significance (MGUS) has usually a predominant glomerular deposition of lambda light chain. Heavy proteinuria is one of its cardinal manifestations. A 78-year-old man with a 9-year history of IgG kappa light-chain-MGUS and normal urine protein excretion developed severe renal failure. Serum levels of kappa light chain and serum IgG had been stable while proteinuria was absent throughout the nine-year period. For the first eight years, he had stable stage III chronic kidney disease attributed to bladder outlet obstruction secondary to prostatic malignancy. In the last year, he developed progressive serum creatinine elevation, without any increase in the serum or urine levels of paraproteins or any sign of malignancy. Renal ultrasound and furosemide renogram showed no evidence of urinary obstruction. Renal biopsy revealed AL amyloidosis, with reactivity exclusive for kappa light chains, affecting predominantly the vessels and the interstitium. Glomerular involvement was minimal. Melphalan and prednisone were initiated. However, renal function continues deteriorating. Deposition of AL kappa amyloidosis developing during the course of MGUS predominantly in the wall of the renal vessels and the renal interstitium, while the involvement of the glomeruli is minimal, leads to progressive renal failure and absence of proteinuria. Renal biopsy is required to detect both the presence and the sites of deposition of renal AL kappa light chain amyloidosis.
RESUMO
Retroperitoneal fibrosis or Ormand's disease is rare in incidence and clinically elusive to diagnosis until obstructive uropathy clinically manifests by the mechanism of ureteral fibrotic strangulation and acute renal failure. We encountered a 50-year-old woman with months of nonspecific abdominal pain and presented with signs and symptoms of acute renal failure. Laboratory data was significant for blood urea nitrogen 47 mg/dL and creatinine of 8.47 mg/dL. Renal ultrasound revealed bilateral hydronephrosis and an abdominal computed tomogram confirmed an abnormal soft tissue retroperitoneal confluence that encased the pelvic vessels. Urologic consultation was requested and bilateral ureteral stents were placed with relief of her obstructive uropathy. Five days after ureteral stenting her creatinine dropped to 1.64 mg/dL. One month later patient underwent ureterolysis with biopsy showing fibroblast proliferation consistent with acute and chronic inflammation. By ruling out infections and malignancy, the final diagnosis was made to be idiopathic retroperitoneal fibrosis.
RESUMO
BACKGROUND: To date, our knowledge of morbidity and mortality in neonatal short bowel syndrome (SBS) is based on individual case series. Shortcomings of the published literature include long patient recruitment time, selection bias, variable SBS definitions, failure to account for gestational age, and incomplete follow-up. By applying more rigorous methodology, our aim was to determine outcomes of SBS neonates compared with a control group of neonates without SBS. METHODS: A cohort study of all neonates with abdominal pathology requiring laparotomy between January 1, 1997, and December 31, 1998, with observation through July 1, 2001. Short bowel syndrome was defined as patients requiring parenteral nutrition for more than 42 days or residual small bowel length of less than 25% predicted by gestational age. Student's t test, Mann-Whitney U test, and chi2 were used where appropriate. Kaplan-Meier curves were used to determine cumulative survival. Covariates important in the development of SBS were examined using forward step-wise logistic regression. RESULTS: There were 175 patients (with SBS = 40, without SBS = 135) with a mean gestational age of 30.7 +/- 4.6 weeks vs 35.9 +/- 4.8 weeks, respectively (P < .0005). The patients with SBS suffered significantly more morbidity than the group without SBS in all categories of investigation (surgical complications, septic events, central venous line complications, duration to adaptation and parenteral nutrition independence, cholestasis and liver failure, and duration of hospitalization). The case fatality rate was 37.5% in patients with SBS vs 13.3% in patients without SBS (P = .001). Most of the deaths were caused by liver failure or sepsis and occurred within 1 year from the date of surgery. Presence of an ileostomy (exp(B) = 12.29; P < .0005) and a residual small bowel length less than 50% of the original length (exp(B) = 26.84; P < .0005) were the only 2 variables in a logistic regression analysis found to be independently associated with the development of SBS. CONCLUSION: This cohort study clearly illustrates the tremendous morbidity experienced by infants with SBS relative to other surgical neonates. Accurate estimates of the morbidity associated with SBS enables clinicians to appropriately counsel parents, allocate resources and initiate therapeutic trials.
