Extrarenal Complications of the Nephrotic Syndrome
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PDI Appendix G Intermediate-Risk Immunocompromised State Ef Ec2
AHRQ QI™ ICD‐10‐CM/PCS Specification Enhanced Version 5.0 1 of 6 Pediatric Quality Indicators Appendices www.qualityindicators.ahrq.gov Pediatric Quality Indicators Appendices APPENDIX G: Intermediate-Risk Immunocompromised State Diagnosis Codes Intermediate-risk immunocompromised state diagnosis codes: (IMMUITD) ICD-9-CM Description ICD-10-CM Description 07022 VIRAL HEPATITIS B W HEPATIC COMA, CHRONIC WO B180 Chronic viral hepatitis B with delta‐agent MENTION OF HEPATITIS DELTA 07023 VIRAL HEPATITIS B W HEPACTIC COMA, CHRONIC W B181 Chronic viral hepatitis B without delta‐agent HEPATITIS DELTA 07044 CHRONIC HEPATITIS C WITH HEPACTIC COMMA B182 Chronic viral hepatitis C 2894 HYPERSPLENISM B520 Plasmodium malariae malaria with nephropathy 28950 DISEASE OF SPLEEN NOS D5702 Hb‐SS disease with splenic sequestration 28951 CHRONIC DIGESTIVE SPLENOMEGALY D57212 Sickle‐cell/Hb‐C disease with splenic sequestration 28952 SPLENIC SEQUESTRATION D57412 Sickle‐cell thalassemia with splenic sequestration 28959 OTHER DISEASE OF SPLEEN, OTHER D57812 Other sickle‐cell disorders with splenic sequestration 4560 ESOPHAGEAL VARICES W BLEEDING D730 Hyposplenism 4561 ESOPHAGEAL VARICES WO MENTION OF BLEEDING D731 Hypersplenism 45620 ESOPHAGEAL VARICES IN DISEASE CLASSIFIED D732 Chronic congestive splenomegaly ELSEWHERE, W BLEEDING 45621 ESOPHAGEAL VARICES IN DISEASE CLASSIFIED D733 Abscess of spleen ELSEWHERE, WO MENTION OF BLEEDING 5723 PORTAL HYPERTENSION D735 Infarction of spleen 5728 OTHER SEQUELAE OF CHRONIC LIVER DISEASE D7389 Other diseases of spleen 5735 -
Glomerulonephritis Management in General Practice
Renal disease • THEME Glomerulonephritis Management in general practice Nicole M Isbel MBBS, FRACP, is Consultant Nephrologist, Princess Alexandra lomerular disease remains an important cause Hospital, Brisbane, BACKGROUND Glomerulonephritis (GN) is an G and Senior Lecturer in important cause of both acute and chronic kidney of renal impairment (and is the commonest cause Medicine, University disease, however the diagnosis can be difficult of end stage kidney disease [ESKD] in Australia).1 of Queensland. nikky_ due to the variability of presenting features. Early diagnosis is essential as intervention can make [email protected] a significant impact on improving patient outcomes. OBJECTIVE This article aims to develop However, presentation can be variable – from indolent a structured approach to the investigation of patients with markers of kidney disease, and and asymptomatic to explosive with rapid loss of kidney promote the recognition of patients who need function. Pathology may be localised to the kidney or further assessment. Consideration is given to the part of a systemic illness. Therefore diagnosis involves importance of general measures required in the a systematic approach using a combination of clinical care of patients with GN. features, directed laboratory and radiological testing, DISCUSSION Glomerulonephritis is not an and in many (but not all) cases, a kidney biopsy to everyday presentation, however recognition establish the histological diagnosis. Management of and appropriate management is important to glomerulonephritis (GN) involves specific therapies prevent loss of kidney function. Disease specific directed at the underlying, often immunological cause treatment of GN may require specialist care, of the disease and more general strategies aimed at however much of the management involves delaying progression of kidney impairment. -
Management of Adult Minimal Change Disease
Kidney CaseCJASN Conference: ePress. Published on April 5, 2019 as doi: 10.2215/CJN.01920219 How I Treat Management of Adult Minimal Change Disease Stephen M. Korbet and William L. Whittier Clin J Am Soc Nephrol 14: ccc–ccc, 2019. doi: https://doi.org/10.2215/CJN.01920219 Introduction Initial Treatment and Course in Adult Minimal Minimal change disease is responsible for idiopathic Change Disease Division of nephrotic syndrome in .75% of children and up to Minimal change disease in adults is highly steroid Nephrology, 30% of adults (1–5). Although secondary causes of sensitive, but steroid resistance is seen in 5%–20% of Department of Medicine, Rush minimal change disease (i.e., nonsteroidal anti- adult patients (1–6). When steroid resistance is ob- fl University Medical in ammatory drugs, lithium, and lymphoproliferative served, the patient often has FSGS on re-examination Center, Chicago, disorders) are uncommon in children, they account of the initial biopsy or on rebiopsy (1,3,6). Although Illinois for up to 15% of minimal change disease in adults 95% of children attain a remission with steroid (1,3). Thus, it is important to assess adults with therapy by 8 weeks, only 50%–75% of adults do so Correspondence: minimal change disease for secondary causes as the (1–6). It is not until after 16 weeks of treatment that Dr. Stephen M. Korbet, – Division of prognosis, and therapeutic approach is determined most adults (75% 95%) enter a remission, with the Nephrology, by the underlying etiology. majority attaining a complete remission (proteinuria Department of of #300 mg/d) and a minority attaining a partial Medicine, Rush remission (proteinuria of .300 mg but ,3.5 g/d). -
T Cells and Minimal Change Disease
EDITORIALS J Am Soc Nephrol 13: 1409–1411, 2002 T Cells and Minimal Change Disease ROBYN CUNARD AND CAROLYN J. KELLY Research Service, VA San Diego Healthcare System; and Department of Medicine, University of California, San Diego, California. It is one of the ironies of medical practice that as physicians we the lymphokine may exert a direct effect on the GBM or can competently and confidently treat diseases of whose patho- activate mesangial cells to produce a factor that altered glo- genesis we remain woefully ignorant. merular permeability (2). Such is the case with minimal change disease, the most This publication preceded by over a decade the molecular common diagnosis associated with the nephrotic syndrome in definition of the T cell antigen receptor as a heterodimeric children (MCNS). The disease manifestations of nephrotic- structure derived from gene rearrangements (3) and the cloning range proteinuria, hypoalbuminemia, and hyperlipidemia in of the first cytokine or lymphokine (4). It preceded by several such patients are typically reversible with corticosteroid ther- years the earliest attempts to characterize subsets of T lympho- apy. MCNS has, by definition, no abnormalities apparent on cytes by their functional and phenotypic heterogeneity (5,6). analysis of kidney biopsy specimens by light microscopy. Shalhoub’s hypothesis has resurfaced multiple times since Humoral components of the immune system, such as Ig and 1974, as clinical investigators take advantage of increased complement, are absent on immunofluorescent analysis of cor- knowledge about immunology and technical breakthroughs to tical kidney sections. The sole abnormalities seen in the kidney reexamine the role of T cells in MCNS. -
Portal Vein Thrombosis in Minimal Change Disease
Ewha Med J 2014;37(2):131-135 Case http://dx.doi.org/10.12771/emj.2014.37.2.131 Report pISSN 2234-3180 • eISSN 2234-2591 Portal Vein Thrombosis in Minimal Change Disease Gyuri Kim, Jung Yeon Lee, Su Jin Heo, Yoen Kyung Kee, Seung Hyeok Han Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Among the possible venous thromboembolic events in nephrotic syndrome, renal Received October 22, 2013, Accepted January 6, 2014 vein thrombosis and pulmonary embolism are common, while portal vein thrombosis (PVT) is rare. This report describes a 26-year-old man with histologically proven mini- Corresponding author mal change disease (MCD) complicated by PVT. The patient presented with epigastric Seung Hyeok Han pain and edema. He had been diagnosed with MCD five months earlier and achieved Department of Internal Medicine, Yonsei complete remission with corticosteroids, which were discontinued one month before University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea the visit. Full-blown relapsing nephrotic syndrome was evident on laboratory and clini- Tel: 82-2-2228-1975, Fax: 82-2-393-6884 cal findings, and an abdominal computed tomography revealed PVT. He immediately E-mail: [email protected] received immunosuppressants and anticoagulation therapy. An eight-week treatment resulted in complete remission, and a follow-up abdominal ultrasonography showed disappearance of PVT. In conclusion, PVT is rare and may not be easily diagnosed in patients with nephrotic syndrome suffering from abdominal pain. Early recognition of Key Words this rare complication and prompt immunosuppression and anticoagulation therapy Minimal change disease; Portal vein are encouraged to avoid a fatal outcome. -
Minimal Change Disease and Focal Segmental Glomerulosclerosis
4 Minimal Change Disease and Focal Segmental Glomerulosclerosis Agnes B. Fogo Introduction/Clinical Setting Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are both common causes of the nephrotic syndrome. Minimal change disease accounts for greater than 90% of cases of nephrotic syn- drome in children, vs. 10% to 15% of adults with nephrotic syndrome (1). Focal segmental glomerulosclerosis has been increasing in incidence in the United States in both African Americans and in Hispanics, in both adult and pediatric populations (2–4). It is now the most common cause of nephrotic syndrome in adults in the U.S. Patients with FSGS may have hypertension and hematuria. Serologic studies, including complement levels, are typically within normal limits in both MCD and FSGS. Pathologic Features Light Microscopy Minimal change disease shows normal glomeruli by light microscopy (Fig. 4.1). In FSGS, sclerosis involves some, but not all, glomeruli (focal), and the sclerosis affects a portion of, but not the entire, glomerular tuft (seg- mental) (Fig. 4.2) (1,5–7). Sclerosis is defined as increased matrix with obliteration of the capillary lumen. Uninvolved glomeruli in FSGS show no apparent lesions by light microscopy; FSGS may also entail hyalinosis, caused by insudation of plasma proteins, producing a smooth, glassy (hyaline) appearance (Fig. 4.3). Adhesions (synechiae) of the capillary tuft to Bowman’s space are a very early sclerosing lesion. Focal segmental glomerulosclerosis is diagnosed when even a single glomerulus shows segmental sclerosis. Therefore, samples must be ade- quate to detect these focal and segmental lesions. A biopsy with only 10 glomeruli has a 35% probability of missing a focal (10% involved) lesion, decreasing to 12% if 20 glomeruli are sampled (8). -
Acute Renal Failure Introduction • Incidence Can Range from 7% to 50
Acute Renal Failure Introduction Incidence can range from 7% to 50% of ICU patients Abrupt decline in kidney function (hours to weeks) Acute Kidney Injury Network Criteria (Bellomo R et al. Crit Care. 2004;8(4):R204-R212) Stage Serum Creatinine Criteria Urine Output 1 Cr inc by 1.5-2x or Cr inc by 0.3 mg/dL <0.5 mL/kg/hr for 6 h 2 Cr inc by 2-3x <0.5 mL/kg/hr for 12 h 3 Cr inc by more than 3x or Cr inc of 0.5 <0.3 mL/kg/hr for 24 hr (or anuria with baseline Cr >4 mg/dL for 12 h) Use criteria after fluid challenge in most cases Causes Pre-renal o Volume depletion o Cardiac o Redistribution o Hepatorenal syndrome . Cirrhosis with ascites . Serum creatinine > 1.5 mg/dL . Not improved by holding diuretics and giving albumin challenge (1g/kg for 2 days) . Absence of shock and nephrotoxins . No intrinsic renal disease i.e. no proteinuria (<500 mg/day) or microhematuria (< 50 RBCs) o NSAIDS o ACE-inhibitor Post-renal o Consider obstruction in every patient with ARF o Sites of obstruction . Bladder neck obstruction . Bilateral ureters o Urine volume is variable. Patients can be asymptomatic and with no change in urine output. o Diagnose with renal USG, straight catherization, or bladder scan Intrinsic o Vascular . Vascular occlusion . Atheroembolic disease Eosinophilia, low complement Can see multi-organ dysfunction, livedo reticularis, blue toes Generally irreversible . Thrombotic microangiopathy Fibrin deposition in the microvasculature, intravascular hemolysis, thrombocytopenia, organ dysfunction Associated disorders: Malignant HTN, HUS/TTP, Scleroderma renal crises, HELLP, drugs (tacrolimus, cyclosporine, mitomycin, Plavix) o Glomerular: RPGN . -
An Unusual Occurrence of Erythrocytosis in a Child with Nephrotic Syndrome and Advanced Chronic Kidney Disease
Case Report An Unusual Occurrence of Erythrocytosis in a Child with Nephrotic Syndrome and Advanced Chronic Kidney Disease Ratna Acharya 1 and Kiran Upadhyay 2,* 1 Division of General Pediatrics, Department of Pediatrics, University of Florida, Gainesville, FL 32610, USA; racharya@ufl.edu 2 Division of Pediatric Nephrology, Department of Pediatrics, University of Florida, Gainesville, FL 32610, USA * Correspondence: kupadhyay@ufl.edu; Tel.: +1-352-273-9180 Abstract: Background: Anemia is common in patients with nephrotic syndrome (NS) for various reasons. Furthermore, anemia can occur in patients with chronic kidney disease (CKD) predominantly owing to inappropriately low erythropoietin (EPO) production relative to the degree of anemia. However, erythrocytosis is uncommon in patients with NS and advanced CKD who are not treated with exogenous erythropoietin stimulating agents, and when present, will necessitate exploration of the other etiologies. Case summary: Here, we describe an 8-year-old girl with erythrocytosis in association with NS and advanced CKD. The patient was found to have erythrocytosis during the evaluation for hypertensive urgency. She also had nephrotic range proteinuria without edema. Serum hemoglobin and hematocrit were 17 gm/dL and 51%, respectively, despite hydration. Renal function test showed an estimated glomerular filtration rate of 30 mL/min/1.73 m2. There was mild iron deficiency anemia with serum iron saturation of 18%. Serum EPO level was normal. Urine EPO was not measured. Renal biopsy showed evidence of focal segmental glomerulosclerosis. Genetic testing for NS showed mutations in podocyte genes: NUP93, INF2, KANK1, and ACTN4. Gene Citation: Acharya, R.; Upadhyay, K. sequence analysis of genes associated with erythrocytosis showed no variants in any of these genes. -
Nephritis Fact Sheet
Last Reviewed March 2017 Page 1 Prevent, Detect, Support. Fact sheet Nephritis How do the kidneys work? The kidneys are two large bean-shaped organs located in your lower back. Each kidney contains up to one million nephrons, the filtering units of your kidneys. Inside a nephron, there is a tiny set of blood vessels called the glomerulus. The glomerulus filters your blood allowing excess fluid and waste to be removed in your urine. What is nephritis? What causes nephritis? Nephritis (also called Most types of nephritis are caused by glomerulonephritis) is a group of your body’s immune system reacting diseases that cause inflammation to an ‘insult’ of some sort. This might (swelling) of the nephrons. This can be a medication, poison, infection reduce your kidney’s ability to filter or a change in the way your immune waste from your blood. system behaves. Your immune system makes antibodies to attack bacteria or poisons. These antibodies can damage your kidneys and nephrons, causing swelling and scarring. Connect with us www.kidney.org.au Freecall 1800 454 363 Kidney Health Australia Nephritis Last Reviewed March 2017 Prevent, Detect, Support. Page 2 What are the different types of nephritis? There are many different types of Different types of nephritis include: Nephrotic syndrome: Damage to the nephritis. It can vary from a mild, nephrons causes them to leak large Focal nephritis: Less than a half of non-damaging condition to a serious amounts of protein into your urine your nephrons have scarring, and problem causing kidney failure. Some but little blood. Losing this protein blood and a small amount of protein types of nephritis appear mild at means your body does not have are found in your urine. -
Nephrotic Syndrome What You Should Know
Nephrotic Syndrome what you should know WHAT IS NEPHROTIC SYNDROME? • Tiny filtering units (glomeruli) in the kidney are damaged or not working. • Protein normally kept in your body, leaks into the urine. Signs and symptoms include: Many diseases can cause it: • High urine protein (proteinuria) • Minimal change disease (MCD) • Swelling (edema) around the eyes, • Membranous glomerulonephritis face, feet, ankles, and/or belly • Focal segmental glomerulosclerosis (FSGS) • Weight gain (from fluid retention) • IGA nephropathy • Foamy urine • Lupus • Poor appetite • Diabetes • High blood cholesterol • Certain infections such as Hepatitis B and C, HIV, others HOW IS IT TESTED? HOW IS IT TREATED? • Physical exam: • Depending on the disease and person’s Visible signs and symptoms overall health, dietary changes and medicines are used to: • Blood and urine tests: - Lower excess salt and fluids in the body Signs of kidney damage and - Lower loss of protein in the urine other diseases - Lower cholesterol in the blood • Imaging tests and/or • Certain medicines that suppress or “calm” kidney biopsy: the immune system can be used. Signs of kidney disease • Sometimes, the dose might need to be • Genetic tests: changed, or a different medicine might Inherited diseases that are be used. linked with kidney disease • In some cases, nephrotic syndrome can lead to kidney failure, which is treated with dialysis or a kidney transplant. Nephrotic Syndrome what you should know HOW CAN I REDUCE MY RISK? Diet, Exercise, and Lifestyle Changes Medicines • Follow a healthy diet that is low in salt • Before taking any over-the-counter and cholesterol. medicine or supplement, ask your healthcare provider which is safe. -
Minimal Change Disease: a Case Report of an Unusual Relationship Fahad Edrees Washington University School of Medicine in St
Washington University School of Medicine Digital Commons@Becker Open Access Publications 2016 Minimal change disease: A case report of an unusual relationship Fahad Edrees Washington University School of Medicine in St. Louis Robert M. Black Saint Vincent Hospital Laszlo Leb Saint Vincent Hospital Helmut Rennke Harvard Medical School Follow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubs Recommended Citation Edrees, Fahad; Black, Robert M.; Leb, Laszlo; and Rennke, Helmut, ,"Minimal change disease: A case report of an unusual relationship." Saudi Journal of Kidney Diseases and Transplantation.27,4. (2016). https://digitalcommons.wustl.edu/open_access_pubs/5287 This Open Access Publication is brought to you for free and open access by Digital Commons@Becker. It has been accepted for inclusion in Open Access Publications by an authorized administrator of Digital Commons@Becker. For more information, please contact [email protected]. Saudi J Kidney Dis Transpl 2016;27(4):816-820 © 2016 Saudi Center for Organ Transplantation Saudi Journal of Kidney Diseases and Transplantation Case Report Minimal Change Disease: A Case Report of an Unusual Relationship Fahad Edrees1, Robert M. Black2,4,Laszlo Leb3,4, Helmut Rennke5 1Department of Medicine, Division of Nephrology, Washington University School of Medicine, Barnes Jewish Hospital, Saint Louis, MO, 2Division of Renal Medicine and 3Department of Hematology Oncology, Saint Vincent Hospital, 4Reliant Medical Group, Worcester, 5Department of Renal Pathology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA, USA ABSTRACT. Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presen- ting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis. -
Unique Proximal Tubular Cell Injury and the Development of Acute
Fujigaki et al. BMC Nephrology (2017) 18:339 DOI 10.1186/s12882-017-0756-6 RESEARCH ARTICLE Open Access Unique proximal tubular cell injury and the development of acute kidney injury in adult patients with minimal change nephrotic syndrome Yoshihide Fujigaki1*, Yoshifuru Tamura2, Michito Nagura2, Shigeyuki Arai2, Tatsuru Ota2, Shigeru Shibata2, Fukuo Kondo3, Yutaka Yamaguchi3 and Shunya Uchida2 Abstract Background: Adult patients with minimal change nephrotic syndrome (MCNS) are often associated with acute kidney injury (AKI). To assess the mechanisms of AKI, we examined whether tubular cell injuries unique to MCNS patients exist. Methods: We performed a retrospective analysis of clinical data and tubular cell changes using the immunohistochemical expression of vimentin as a marker of tubular injury and dedifferentiation at kidney biopsy in 37 adult MCNS patients. AKI was defined by the criteria of the Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guidelines for AKI. Results: Thirteen patients (35.1%) were designated with AKI at kidney biopsy. No significant differences in age, history of hypertension, chronic kidney disease, diuretics use, proteinuria, and serum albumin were noted between the AKI and non- AKI groups. Urinary N-acetyl-β-D-glucosaminidase (uNAG) and urinary alpha1-microglobulin (uA1MG) as markers of tubular injury were increased in both groups, but the levels were significantly increased in the AKI group compared with the non- AKI group. The incidence of vimentin-positive tubules was comparable between AKI (84.6%) and non-AKI (58.3%) groups, but vimentin-positive tubular area per interstitial area was significantly increased in the AKI group (19.8%) compared with the non-AKI group (6.8%) (p = 0.011).