All that seems well is not always well--intermittently malfunctioning flexometallic tracheal tubes.
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Adult↗
Biomedical subjects
J Radhakrishnan
Publications and source records attributed to J Radhakrishnan.
Minimal change disease in systemic lupus erythematosus.
We report the clinical and pathologic findings in 7 patients with systemic lupus erythematosus and minimal change disease. All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria (mean 9.6 g/day), and hypoalbuminemia (mean 1.8 g/dl). In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. In all cases, steroid therapy induced a rapid remission of nephrotic syndrome. Minimal change disease is an underrecognized and readily reversible form of nephrotic syndrome in systemic lupus erythematosus. Because it may occur superimposed on mild mesangial proliferative lupus nephritis, this entity may be misinterpreted as an atypical presentation of lupus nephritis class II. Proper recognition of this entity requires careful integration of the renal biopsy immunofluorescence and electron microscopic findings.
Adolescent↗
Prevalence and patterns of presentation of genetic disorders in a pediatric emergency department.
OBJECTIVES: To determine the prevalence and patterns of presentation of previously diagnosed and of suspected genetic disorders among pediatric emergency department (ED) visits to a hospital that serves an inner-city population. PATIENTS AND METHODS: A retrospective review of 15,258 pediatric (<18 years old) ED visits at Lincoln Medical and Mental Health Center was undertaken for visits that occurred between October 1998 and February 1999. Suspected genetic disorders, classified into chromosomal, single gene, multifactorial, and other syndromic categories, were recorded. RESULTS: Of 15,258 visits reviewed, 2839 visits (18.6%) were by patients who had known or suspected genetic disorders. Previously diagnosed genetic disorders were documented in 80 visits (2.8%). Of these, 69 visits (86.2%) were related to single gene disorders, 3 (3.8%) to chromosomal disorders, 6 (7.5%) to multifactorial disorders, and 2 (2.5%) to disorders in the "other" category. Of these 80 visits, 59 (74%) were associated with sickle cell disease. The remaining 2759 visits (97.2%) were associated with complaints or diagnoses that suggested the possibility of an underlying genetic disorder requiring further evaluation and diagnostic work-up. CONCLUSIONS: Pediatric patients with known or suspected genetic disorders are frequently treated in EDs. Awareness of underlying genetic disorders facilitates diagnostic evaluation, treatment planning, and referral to a genetics clinic for counseling.
Adolescent↗
Effects of tezosentan, a dual endothelin receptor antagonist, on the cardiovascular and renal systems of neonatal piglets.
BACKGROUND/PURPOSE: Endothelin is a potent biological vasoactive mediator in the cardiovascular and renal systems. Little is known of the effects of endothelin antagonism on the developing heart and kidney, and we hope to show that endothelin does have an important role in the cardiovascular and renal systems of the developing neonate. In this study the authors have examined the effects of tezosentan, a nonselective endothelin-A and endothelin-B receptor antagonist designed for parenteral use, on the cardiovascular and renal systems of healthy neonatal piglets. METHODS: Eight, 7- to 10-day old domestic piglets weighing 2.5 to 3.0 kg were anesthetized, intubated, and ventilated with catheters placed into the jugular vein, left ventricle, and femoral artery. Urine output was monitored via a suprapubic cystostomy. After baseline data were obtained the piglets received tezosentan (1 mg/kg/h) for 1 hour. A set of data was collected just before discontinuation of the 1-hour infusion of tezosentan and another set was collected 1 hour after the discontinuation of the drug. Mean arterial pressure (MAP), heart rate (HR), and urine output (UV) were monitored continuously and cardiac index (CI), systemic vascular resistance (SVR), renal blood flow (RBF), and renal vascular resistance (RVR) were calculated from gamma counts obtained from injections of radio-labeled microspheres at end of the different time periods. Glomerular filtration rate (GFR) was obtained by the sodium iothalamate method. Data were averaged and plotted versus time and analyzed statistically by a Student's t test. RESULTS: (P <.05 versus baseline*). In our experimental animals the infusion of tezosentan diminished MAP and SVR from baseline values of 94 +/- 7 mm Hg and 0.14 +/- 0.03 mm Hg/mL/min, respectively to TEZO values of 62 +/- 4* mm Hg and 0.07 +/- 0.02* mm Hg/mL/min. CI increased from 278 +/- 58 to 367 +/- 75* mL/min/kg with tezosentan. There also was a statistically significant increase in RBF from 1.16 +/- 0.38 to 1.86 +/- 0.37* mL/min/kg, an increase in UV from 0.57 +/- 0.24 to 0.64 +/- 0.12* mL/min, a decrease in RVR from 4.60 +/- 1.47 to 2.03 +/- 0.36* mm Hg/mL/min, and no change in the GFR. CONCLUSIONS: The inhibition of endothelin receptors with tezosentan produced a statistically significant effect on the piglet cardiovascular system with a drop in MAP and SVR and an increase in CI and HR. It also produced a statistically significant increase in RBF and UV and a decrease in RVR without affecting GFR. J Pediatr Surg 36:1824-1828.
Animals↗
Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation.
Castleman's disease is an infrequent and usually benign lymphoproliferative disorder. Resection of the tumor usually is curative. The immunostimulatory nature of the tumor can, in rare instances, result in paraneoplastic manifestations. The authors present a case of a 14 year old with mucocutaneous ulcerations and progressive dyspnea that was found to have a large mediastinal mass and circulating autoantibodies that were responsible for his paraneoplastic pemphigus and bronchiolitis obliterans. In spite of aggressive immunotherapy to control the autoimmune mucocutaneous lesions, the pulmonary fibrosis was irreversible and progressed to pulmonary failure necessitating lung transplantation. J Pediatr Surg 36:E22.
Adolescent↗
Racial and socioeconomic factors in glomerular disease.
The influence of racial and socioeconomic factors on the incidence, prognosis, and response to therapy of many diseases has long been noted. Although glomerular diseases comprise 10% to over 16% of the dialysis and renal transplant populations, respectively, only recently have racial and socioeconomic factors been evaluated. Several glomerular diseases are associated with a striking African-American predominance. These include idiopathic focal sclerosis, and especially its collapsing variant, human immunodeficiency virus (HIV)-associated nephropathy, and severe lupus nephritis. In many of these entities the renal disease is also more aggressive and rapidly progressive than in other populations. Recent data points to genetic-biologic as well as social and economic factors that may be responsible for these findings. Studies dealing with the therapy of glomerular lesions must be stratified for racial and social background differences to avoid bias in outcome. Whether racial and socioeconomic data should be used in treating individual patients, and how, remains an area of controversy.
AIDS-Associated Nephropathy↗
Antiphospholipid antibody syndrome and renal disease.
The antiphospholipid syndrome is a disorder of hypercoagulability in association with circulating antiphospholipid antibodies directed against epitopes on oxidized phospholipids complexed with a glycoprotein, beta 2-glycoprotein I, or against the glycoprotein itself. Disorders associated with antiphospholipid antibodies but not the antiphospholipid syndrome, such as HIV and hepatitis C infection, appear to lack antibodies to beta 2-glycoprotein I. Patients with systemic lupus erythematosus have a high incidence of antiphospholipid antibodies with a high risk of thrombosis, often associated with anticardiolipin antibodies, beta 2-glyocoprotein I antibodies, and the presence of the lupus anticoagulant. Antiphospholipid antibodies are a significant cause of morbidity and mortality in renal patients with and without systemic lupus erythematosus. Renal manifestations include thrombotic microangiopathy and large vessel thrombosis. In patients with end-stage renal disease, antiphospholipid antibodies are prevalent and may increase in frequency with time on dialysis, possibly as a result of oxidative stress incurred during dialysis. The presence of anticardiolipin antibodies have been associated with a high incidence of hemodialysis access clotting. In renal transplant recipients, the incidence of antiphospholipid antibodies is also elevated and may be associated with a higher incidence of primary graft non-function. Although patients with systemic lupus erythematosus have similar renal allograft survival rates to the general population, survival is worse for those patients who are also antiphospholipid antibody positive. Additionally, in hepatitis C positive renal transplant recipients, the presence of anticardiolipin antibodies confers an increased risk of thrombotic complications and the development of thrombotic microangiopathy. Treatment of antiphospholipid antibody syndrome remains centered around anticoagulation with warfarin. The use of immunosuppressive agents has had no dramatic effect on antiphospholipid antibody titers and little clinical effect on thrombotic events.
Antibodies, Antiphospholipid↗
Advances in the treatment of lupus nephritis.
Systemic lupus erythematosus (SLE) is an autoimmune disease that leads to the formation and deposition of immune complexes throughout the body, which are pathogenic for the disease. Different forms of glomerulonephritis can occur in patients with SLE and can contribute significantly to the associated morbidity and, ultimately, mortality from the disease. Over the past two decades, there have been significant strides in our understanding of the disease and in treatments that attempt to control the formation and deposition of anti-DNA auto-antibodies and immune complexes, as well as the subsequent inflammatory cascade mediated through various cellular and humoral pathways leading to progressive renal damage and end-stage renal disease. In this chapter, we review the current understanding of the pathogenesis and treatment of lupus nephritis in its various stages and discuss the experimental and human data regarding some of the potential newer forms of therapy. We discuss data regarding the use of steroids, azathioprine, cyclophosphamide, cyclosporine A, mycophenolate mofetil, gammaglobulin, plasmapheresis, LJP 394, flaxseed oil, bindarit, anti-CD40 ligand, and CTLA4Ig.
Abatacept↗
An analysis of cardiac mortality in patients with new-onset end-stage renal disease in New York State.
End-stage renal disease (ESRD) is associated with an overall one-year mortality of 23.5% in the US, of which cardiac causes constitute 50% of all deaths. Data on incident ESRD patients were obtained from the Health Care Financing Administration's 2728 and 2746 forms by special request from the ESRD Network of New York. 4,948 ESRD patients, who started dialysis in New York State from April 1, 1995, through April 1, 1996, were assessed to identify risk factors present at the initiation of dialysis that predict cardiac death. 899 deaths were registered during the 19-month-follow-up period, 50% of which were from cardiac causes. Using the Cox-proportional hazards model, the increasing age category, white race, the presence of one or more vascular co-morbid conditions, and the presence of diabetes and one or more cardiac co-morbid conditions significantly predicted cardiac death (p < 0.05). Diabetes increased the risk for cardiac death by 48% for those patients without any cardiac co-morbidities (RR = 1.48, p < 0.0082). In contrast with results observed in the general population, gender, serum albumin and body mass index were not significant predictors of cardiac death. In identifying risk factors present at the initiation ofdialysis that predict cardiac death, this study highlights factors that may be modified prior to dialysis initiation in order to improve life expectancy and mortality rates and decrease health care costs for the ESRD population.
Adolescent↗
Use of mycophenolate mofetil in resistant membranous nephropathy.
Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Optimal therapy for this disease is still debated. We report our experience using mycophenolate mofetil (MMF), an immunosuppressive agent widely used in transplant recipients, to treat 16 nephrotic patients with MN. All patients had biopsy-documented MN; secondary forms were ruled out. Fifteen patients had steroid-resistant disease; cytotoxic agents had failed in 6 patients and cyclosporine therapy had failed in 5 patients. Patients were treated with MMF (dose range, 500 to 2,000 mg) for a mean of 8 months. Six patients experienced a halving of proteinuria, which occurred after a mean duration of 6 months of therapy. Partial remissions occurred in 2 patients. There were no significant changes in mean values for serum creatinine, serum albumin, or proteinuria. Mean cholesterol levels were significantly less. Side effects of MMF were infrequent and generally mild. In summary, MMF appears to reduce proteinuria in some patients with idiopathic MN previously resistant to steroids, cytotoxic agents, or cyclosporine. Further trials with this agent are warranted.
Adolescent↗
Congenital cysts of the third and fourth pharyngeal pouches or pyriform sinus cysts.
Cysts arising from the III and IV pharyngeal pouches, although uncommon, are typical in their presentation. They occur in neonates, invariably in the left anterior neck and cause respiratory distress. Excision of the cyst with ligation of the tract at the level of the pyriform sinus is curative.
Cysts↗
Cyclosporin treatment of glomerular diseases.
Cyclosporin is a potent immunosuppressive agent that has become the first line therapy in organ transplantation. Its efficacy has led to its use in a variety of immune-mediated glomerular diseases. A selection of controlled and uncontrolled trials has studied the effects of cyclosporin in patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), IgA nephropathy, membranoproliferative glomerulonephritis (MPGN) and lupus nephritis. We review the recent literature and suggest recommendations for using cyclosporin in these diseases, based on this evidence and our experience.
Animals↗
Effect of unilateral testicular torsion on blood flow and histology of contralateral testes.
BACKGROUND/PURPOSE: Infertility occurs in 25% of patients after unilateral testicular torsion; hence, the authors examined hemodynamic and histological changes in both testes after acute testicular torsion in neonatal piglets. METHODS: The animals were anesthetized, intubated, ventilated, catheterized, and assigned randomly to a sham group or one of three experimental groups undergoing 720 degrees torsion of the left testis for 8 hours after which it was untwisted in group I and removed in group II. In group III, both testes were removed. Data were collected at baseline (T = 0), 4 hours (T = 4), and 8 hours of torsion (T = 8) and at the ninth hour of the experiment (T = 9). Testicular blood flow was determined by using radiolabeled microspheres. The testes also were examined blindly with routine and electron microscopy. RESULTS: In group I, testicular blood flow decreased in the affected testis during torsion and increased significantly after detorsion, whereas blood flow to the contralateral testis increased significantly after detorsion. Sham-operated animals showed no histological abnormality in either testis. In all torsion groups, the affected testis showed extensive changes caused by hemorrhagic necrosis. The contralateral testis only showed changes in group I. CONCLUSION: Unilateral testicular torsion resulted in ipsilateral damage caused by a decrease and subsequent increase in blood flow while in the contralateral testis; damage was the result of a significant increase in blood flow after detorsion.
Animals↗
Renal effects of low to moderate doses of dopamine in newborn piglets.
PURPOSE: Administration of dopamine to adult animal and human subjects results in increased renal blood flow, and it may also enhance the glomerular filtration rate. However, renal hemodynamic effects of exogenous dopamine in the neonate are unclear. In this study, we examined the renal actions of low to moderate doses of exogenous dopamine in newborn piglets. METHODS: The animals were anesthetized, catheterized for vascular access and urine collection, and assigned randomly to a control group or treatment groups receiving dopamine infusion at 2, 5, or 10 microg/kg/min. Data were collected at baseline, during dopamine infusion, and 1 hour after cessation of infusion. Mean arterial blood pressure (MAP) and heart rate (HR) were monitored. Glomerular filtration rate (GFR), cardiac index (CI), and renal blood flow (RBF) were determined. Fractional excretion of sodium (FENa) was calculated. RESULTS: Dopamine did not alter renal blood flow nor did it significantly alter CI in spite of a modest increase in heart rate and mean arterial blood pressure. There was a statistically significant increase in GFR at 10 microg/kg/min and in FENa at all doses. CONCLUSIONS: Low doses of dopamine produce significant natriuresis probably by direct action on renal tubules and at moderate doses via, both, increase in GFR and a direct tubular effect. Low and moderate doses of dopamine do not increase RBF as seen in adult animals, possibly because of immaturity of dopaminergic receptors in newborn piglets.
Animals↗
New animal model to evaluate testicular blood flow during testicular torsion.
BACKGROUND/PURPOSE: Unilateral testicular torsion is known to cause infertility because of damage to the contralateral testis. Testicular damage has been attributed to many different mechanisms, one of which is altered contralateral blood flow. In our experiment, in an effort to identify the reason for contralateral testicular injury, the authors developed an accurate method of measuring blood flow in both testes before, during, and after unilateral torsion. METHODS: Four- to 6-week-old piglets weighing 4 to 6 kg were studied. The animals were anesthetized, intubated, ventilated, and catheterized for vascular access. Piglets were assigned randomly to a sham group or a group undergoing 360 degrees or 720 degrees torsion of the left testis (n = 5 per group) for 8 hours, after which it was untwisted. Data were collected at baseline (T = 0), 8 hours of torsion (T = 8), and 1 hour after detorsion (T = 9). Mean arterial blood pressure and heart rate were monitored continuously. Testicular blood flow was determined using radiolabeled microspheres. Blood flow data were evaluated by analysis of variance. RESULTS: In the 360 degrees torsion group, blood flow changes were insignificant during torsion and after detorsion. In the 720 degrees torsion group, blood flow to the twisted testis was reduced significantly, whereas the contralateral testis was unaffected. One hour after detorsion, blood flow to both testes was increased significantly. CONCLUSIONS: The authors describe a new animal model to evaluate testicular blood flow during and after testicular torsion. Increased blood flow after detorsion may be the cause of testicular damage in patients with unilateral testicular torsion.
Animals↗
Cyclosporine treatment of glomerular diseases.
Cyclosporine-A is primary therapy for organ transplantation. Its immunosuppressive effect might suggest a therapeutic role in autoimmune diseases, including several idiopathic and secondary glomerular conditions. Various forms of idiopathic nephrotic syndrome, including focal segmental glomerulosclerosis (FSGS), minimal change disease (MCD), and membranous nephropathy (MN), may respond well to cyclosporine in selected patients. However, frequent relapse limits its use to those who have failed to respond to, or were intolerant of, steroids or cytotoxics. Cyclosporine's efficacy in other glomerulopathies, such as IgA nephropathy (IgAN) and membranoproliferative glomerulonephritis (MPGN) remains poorly studied and, given the risk of nephrotoxicity, cannot be recommended for treatment of these entities until further data are available. Cyclosporine demonstrates some efficacy in treating proliferative lupus nephritis and, based on pilot study data, membranous lupus as well. Again, given relapse rates and potential nephrotoxicity, it should be used only in combination with azathioprine and steroids, assuming cytotoxic therapy has failed. Finally, cyclosporine toxicity is briefly reviewed.
Autoimmune Diseases↗
A large prospective survey of anti-cardiolipin antibodies in chronic hemodialysis patients.
We prospectively measured anti-cardiolipin antibody (ACLA) levels in 230 chronic hemodialysis (HD) patients over a 2-year period. Twenty-nine percent of HD-patients were found to have elevated IgG-ACLA titers. Males were more likely to have elevated IgG-ACLA titers. Elevated IgG-ACLA titers correlated with shortened AVG survival in HD-patients (mean of 156 vs. 238 days, p<0.05). There was no statistically significant correlation with access survival in AVF-patients. There seemed to be a higher mean IgG-ACLA titer in diabetics but they did not have statistically significant shorter angioaccess survival times. By logistic regression analysis, only IgG-ACLA positivity was predictive of premature angioaccess failure (p<0.05). In a selected subset of 16 patients with frequent angioaccess (AVG) failure and elevated IgG-ACLA levels, coumadin, titrated to an INR of 2 - 3, was found to produce a small (though statistically significant) prolongation of AVG survival.
Adolescent↗
Double-barrelled colovaginoplasty in a patient with cloacal exstrophy variant.
Description of a new technique for combining colovaginoplasty with rectal pull-through.
Abnormalities, Multiple↗