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<meta name="keywords" content="C0041349, disease or syndrome, interstitial nephritis, nephritides, tubulointerstitial, nephritis, tubulointerstitial, nephropathy, tubulointerstitial, renal tubulo-interstitial disease, tubulo-interstitial nephritis, tubulointerstitial disease, tubulointerstitial nephritides, tubulointerstitial nephritis, tubulointerstitial nephropathy, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
UID=11952
ConceptID=C0041349
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Interstitial nephritis</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>11952</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0041349</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonym:</td>
<td>Tubulointerstitial nephritis</td></tr>
<tr><td><span class="bold">SNOMED CT: </span></td>
<td>Tubulo-interstitial nephritis (428255004); Tubulointerstitial nephritis (428255004); Interstitial nephritis (428255004); Tubulointerstitial nephropathy (428255004); Renal tubulo-interstitial disease (428255004)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0001970">HP:0001970</a></td></tr>
<tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0001085" target="_blank">MONDO:0001085</a></td></tr>
<tr><td>OMIM<span class="superscript">®</span>:</td>
<td><a href="https://omim.org/entry/590070" target="_blank">590070</a></td></tr>
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<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0041349[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=11952">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=11952" target="_blank" href="/omim/590070">O</a></span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=11952" ref="ncbi_uid=11952">V</a></span></span><span class="TLline">Interstitial nephritis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867444" ref="tree=MeSH" title="MedGen record for Abnormality of the urinary system">Abnormality of the urinary system</a></span><ul><li><span class="TLline"><a href="/medgen/869219" ref="tree=MeSH" title="MedGen record for Abnormality of the upper urinary tract">Abnormality of the upper urinary tract</a></span><ul><li><span class="TLline"><a href="/medgen/78593" ref="tree=MeSH" title="MedGen record for Abnormality of the kidney">Abnormality of the kidney</a></span><ul><li><span class="TLline"><a href="/medgen/1633142" ref="tree=MeSH" title="MedGen record for Abnormal renal morphology">Abnormal renal morphology</a></span><ul><li><span class="TLline"><a href="/medgen/1713223" ref="tree=MeSH" title="MedGen record for Abnormal renal insterstitial morphology">Abnormal renal insterstitial morphology</a></span><ul><li><span class="TLline"><a href="/medgen/892966" ref="tree=MeSH" title="MedGen record for Abnormal tubulointerstitial morphology">Abnormal tubulointerstitial morphology</a></span><ul><li><span class="matched_ds">Interstitial nephritis</span><ul><li><span class="TLline"><a href="/medgen/334716" ref="tree=MeSH" title="MedGen record for Acute tubulointerstitial nephritis">Acute tubulointerstitial nephritis</a></span></li><li><span class="TLline"><a href="/medgen/65957" ref="tree=MeSH" title="MedGen record for Chronic tubulointerstitial nephritis">Chronic tubulointerstitial nephritis</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
</div>
<div class="portlet mgSection" id="ID_112">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat">
<div class="divPopper rprt" id="rdis_78675"><div><strong>Alstrom syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>78675</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C0268425</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Alström syndrome is characterized by cone-rod dystrophy, obesity, progressive bilateral sensorineural hearing impairment, acute infantile-onset cardiomyopathy and/or adolescent- or adult-onset restrictive cardiomyopathy, insulin resistance / type 2 diabetes mellitus (T2DM), nonalcoholic fatty liver disease (NAFLD), and chronic progressive kidney disease. Cone-rod dystrophy presents as progressive visual impairment, photophobia, and nystagmus usually starting between birth and age 15 months. Many individuals lose all perception of light by the end of the second decade, but a minority retain the ability to read large print into the third decade. Children usually have normal birth weight but develop truncal obesity during their first year. Sensorineural hearing loss presents in the first decade in as many as 70% of individuals and may progress to the severe or moderately severe range (40-70 db) by the end of the first to second decade. Insulin resistance is typically accompanied by the skin changes of acanthosis nigricans, and proceeds to T2DM in the majority by the third decade. Nearly all demonstrate hypertriglyceridemia. Other findings can include endocrine abnormalities (hypothyroidism, hypogonadotropic hypogonadism in males, and hyperandrogenism in females), urologic dysfunction / detrusor instability, progressive decrease in renal function, and hepatic disease (ranging from elevated transaminases to steatohepatitis/NAFLD). Approximately 20% of affected individuals have delay in early developmental milestones, most commonly in gross and fine motor skills. About 30% have a learning disability. Cognitive impairment (IQ &lt;70) is very rare. Wide clinical variability is observed among affected individuals, even within the same family.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/78675">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_96586"><div><strong>Cranioectodermal dysplasia 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>96586</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0432235</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Cranioectodermal dysplasia (CED) is a ciliopathy with skeletal involvement (narrow thorax, shortened proximal limbs, syndactyly, polydactyly, brachydactyly), ectodermal features (widely spaced hypoplastic teeth, hypodontia, sparse hair, skin laxity, abnormal nails), joint laxity, growth deficiency, and characteristic facial features (frontal bossing, low-set simple ears, high forehead, telecanthus, epicanthal folds, full cheeks, everted lower lip). Most affected children develop nephronophthisis that often leads to end-stage kidney disease in infancy or childhood, a major cause of morbidity and mortality. Hepatic fibrosis and retinal dystrophy are also observed. Dolichocephaly, often secondary to sagittal craniosynostosis, is a primary manifestation that distinguishes CED from most other ciliopathies. Brain malformations and developmental delay may also occur.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/96586">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_282890"><div><strong>Sjogren syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>282890</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1527336</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006).&#13; See 200400 for association of Sjogren syndrome with achalasia in sisters.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/282890">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_344424"><div><strong>Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>344424</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1855114</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">For this GeneReview, the term "isolated methylmalonic acidemia" refers to a group of inborn errors of metabolism associated with elevated methylmalonic acid (MMA) concentration in the blood and urine that result from the failure to isomerize (convert) methylmalonyl-coenzyme A (CoA) into succinyl-CoA during propionyl-CoA metabolism in the mitochondrial matrix, without hyperhomocysteinemia or homocystinuria, hypomethioninemia, or variations in other metabolites, such as malonic acid. Isolated MMA is caused by complete or partial deficiency of the enzyme methylmalonyl-CoA mutase (mut0 enzymatic subtype or mut enzymatic subtype, respectively), a defect in the transport or synthesis of its cofactor, 5-deoxy-adenosyl-cobalamin (cblA, cblB, or cblD-MMA), or deficiency of the enzyme methylmalonyl-CoA epimerase. Prior to the advent of newborn screening, common phenotypes included: Infantile/non-B12-responsive form (mut0 enzymatic subtype, cblB), the most common phenotype, associated with infantile-onset lethargy, tachypnea, hypothermia, vomiting, and dehydration on initiation of protein-containing feeds. Without appropriate treatment, the infantile/non-B12-responsive phenotype could rapidly progress to coma due to hyperammonemic encephalopathy. Partially deficient or B12-responsive phenotypes (mut enzymatic subtype, cblA, cblB [rare], cblD-MMA), in which symptoms occur in the first few months or years of life and are characterized by feeding problems, failure to thrive, hypotonia, and developmental delay marked by episodes of metabolic decompensation. Methylmalonyl-CoA epimerase deficiency, in which findings range from complete absence of symptoms to severe metabolic acidosis. Affected individuals can also develop ataxia, dysarthria, hypotonia, mild spastic paraparesis, and seizures. In those individuals diagnosed by newborn screening and treated from an early age, there appears to be decreased early mortality, less severe symptoms at diagnosis, favorable short-term neurodevelopmental outcome, and lower incidence of movement disorders and irreversible cerebral damage. However, secondary complications may still occur and can include intellectual disability, tubulointerstitial nephritis with progressive impairment of renal function, "metabolic stroke" (bilateral lacunar infarction of the basal ganglia during acute metabolic decompensation), pancreatitis, growth failure, functional immune impairment, bone marrow failure, optic nerve atrophy, arrhythmias and/or cardiomyopathy (dilated or hypertrophic), liver steatosis/fibrosis/cancer, and renal cancer.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/344424">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_358137"><div><strong>Tubulointerstitial kidney disease, autosomal dominant, 2</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>358137</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1868139</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Autosomal dominant tubulointerstitial kidney disease MUC1 (ADTKD-MUC1) is characterized by slowly progressive tubulointerstitial disease that leads to end-stage renal disease (ESRD) and the need for dialysis or kidney transplantation. The rate of loss of kidney function for individuals is variable within and between families, with a median age of onset of end-stage renal disease (ESRD) of 46 years (range: ages 20-70 years). There are no other systemic manifestations.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/358137">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_762097"><div><strong>Mitochondrial complex III deficiency nuclear type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>762097</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C3541471</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Autosomal recessive mitochondrial complex III deficiency is a severe multisystem disorder with onset at birth of lactic acidosis, hypotonia, hypoglycemia, failure to thrive, encephalopathy, and delayed psychomotor development. Visceral involvement, including hepatopathy and renal tubulopathy, may also occur. Many patients die in early childhood, but some may show longer survival (de Lonlay et al., 2001; De Meirleir et al., 2003).&#13; Genetic Heterogeneity of Mitochondrial Complex III Deficiency&#13; Mitochondrial complex III deficiency can be caused by mutation in several different nuclear-encoded genes. See MC3DN2 (615157), caused by mutation in the TTC19 gene (613814) on chromosome 17p12; MC3DN3 (615158), caused by mutation in the UQCRB gene (191330) on chromosome 8q; MC3DN4 (615159), caused by mutation in the UQCRQ gene (612080) on chromosome 5q31; MC3DN5 (615160), caused by mutation in the UQCRC2 gene (191329) on chromosome 16p12; MC3DN6 (615453), caused by mutation in the CYC1 gene (123980) on chromosome 8q24; MC3DN7 (615824), caused by mutation in the UQCC2 gene (614461) on chromosome 6p21; MC3DN8 (615838), caused by mutation in the LYRM7 gene (615831) on chromosome 5q23; MC3DN9 (616111), caused by mutation in the UQCC3 gene (616097) on chromosome 11q12; and MC3DN10 (618775), caused by mutation in the UQCRFS1 gene (191327) on chromosome 19q12.&#13; See also MTYCB (516020) for a discussion of a milder phenotype associated with isolated mitochondrial complex III deficiency and mutations in a mitochondrial-encoded gene.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/762097">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_766688"><div><strong>Karyomegalic interstitial nephritis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>766688</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C3553774</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Karyomegalic tubulointerstitial nephritis (KMIN) is a rare kidney disease characterized clinically by onset in the third decade of progressive renal failure. Renal biopsy shows chronic tubulointerstitial nephritis and interstitial fibrosis associated with enlarged and atypical tubular epithelial cell nuclei (summary by Baba et al., 2006).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/766688">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_855697"><div><strong>Nephronophthisis 18</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>855697</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C3890591</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Nephronophthisis-18 is an autosomal recessive disorder characterized by chronic tubulointerstitial nephritis resulting in end-stage renal disease in early childhood. Extrarenal manifestations, including intellectual disability or liver changes, may occur in some patients (summary by Failler et al., 2014).&#13; For a general phenotypic description and a discussion of genetic heterogeneity of nephronophthisis, see NPHP1 (256100).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/855697">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_899086"><div><strong>Senior-Loken syndrome 9</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>899086</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4225263</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Senior-Loken syndrome-9 is an autosomal recessive disorder characterized by early-onset nephronophthisis and pigmentary retinopathy. Additional more variable features can include liver defects, skeletal anomalies, and obesity (summary by Bizet et al., 2015).&#13; For a phenotypic description and a discussion of genetic heterogeneity of Senior-Loken syndrome, see 266900.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/899086">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_934768"><div><strong>Developmental delay with short stature, dysmorphic facial features, and sparse hair</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>934768</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4310801</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">A rare genetic multiple congenital anomalies/dysmorphic syndrome with characteristics of craniofacial dysmorphism (including an abnormal skull shape, hypertelorism, downslanting palpebral fissures, epicanthal folds, low-set ears, depressed nasal bridge, micrognathia), short stature, ectodermal anomalies (such as sparse eyebrows, eyelashes, and scalp hair, hypoplastic toenails), developmental delay, and intellectual disability. Additional features may include cerebral/cerebellar malformations and mild renal involvement.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/934768">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_1621949"><div><strong>Psychomotor regression-oculomotor apraxia-movement disorder-nephropathy syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1621949</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4539828</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Birk-Landau-Perez syndrome (BILAPES) is an autosomal recessive syndromic developmental disorder characterized by global developmental delay apparent from infancy or early childhood. Some patients have developmental regression with loss of speech and motor skills, whereas other patients never achieve these milestones. More variable features may include hypotonia, poor overall growth, ataxia, dystonia, abnormal eye movements, and renal insufficiency (Perez et al., 2017; Kleyner et al., 2022).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1621949">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_1645893"><div><strong>Familial juvenile hyperuricemic nephropathy type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1645893</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4551496</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Autosomal dominant tubulointerstitial kidney disease UMOD (ADTKD-UMOD) is characterized by normal urinalysis and slowly progressive chronic kidney disease (CKD), usually first noted in the teen years and progressing to end-stage renal disease (ESRD) between the third and seventh decades. Hyperuricemia is often present from an early age, and gout (resulting from reduced kidney excretion of uric acid) occurs in the teenage years in about 8% of affected individuals and develops in 55% of affected individuals over time.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1645893">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_1634481"><div><strong>Combined oxidative phosphorylation defect type 9</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1634481</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4706315</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">A rare mitochondrial disease due to a defect in mitochondrial protein synthesis characterized by initially normal growth and development followed by the infantile-onset of failure to thrive, psychomotor delay, poor feeding, dyspnea, severe hypertrophic cardiomyopathy and hepatomegaly. Laboratory studies report increased plasma lactate and alanine, abnormal liver enzymes and decreased activity of mitochondrial respiratory chain complexes I, III, IV, and V. Caused by compound heterozygous mutation in the MRPL3 gene on chromosome 3q22.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1634481">Condition Record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_78675" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Alstrom syndrome</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1634481" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Combined oxidative phosphorylation defect type 9</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_96586" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cranioectodermal dysplasia 1</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_934768" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Developmental delay with short stature, dysmorphic facial features, and sparse hair</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1645893" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial juvenile hyperuricemic nephropathy type 1</a></div><div class="jig-moreless" data-jigconfig="class: 'moveDown', moreText: 'See full list (13)', lessText: 'Show less', nodeBefore: 0"><span id="clinMore">
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_766688" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Karyomegalic interstitial nephritis</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_344424" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_762097" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Mitochondrial complex III deficiency nuclear type 1</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_855697" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Nephronophthisis 18</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1621949" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Psychomotor regression-oculomotor apraxia-movement disorder-nephropathy syndrome</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_899086" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Senior-Loken syndrome 9</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_282890" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Sjogren syndrome</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_358137" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Tubulointerstitial kidney disease, autosomal dominant, 2</a></div></span></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/39138117">Immune checkpoint inhibitor-associated nephritis-treatment standard.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Barbir EB,
Kitchlu A,
Herrmann SM</span><br />
<span class="medgenPMjournal">Nephrol Dial Transplant</span>
2024 Oct 30;39(11):1785-1798.
doi: 10.1093/ndt/gfae184.
<span class="bold">PMID: </span><a href="/pubmed/39138117" target="_blank">39138117</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36168055">Diagnosis and management of immune checkpoint inhibitor-associated acute kidney injury.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sprangers B,
Leaf DE,
Porta C,
Soler MJ,
Perazella MA</span><br />
<span class="medgenPMjournal">Nat Rev Nephrol</span>
2022 Dec;18(12):794-805.
Epub 2022 Sep 27
doi: 10.1038/s41581-022-00630-8.
<span class="bold">PMID: </span><a href="/pubmed/36168055" target="_blank">36168055</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30249419">Urine Sediment Examination in the Diagnosis and Management of Kidney Disease: Core Curriculum 2019.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Cavanaugh C,
Perazella MA</span><br />
<span class="medgenPMjournal">Am J Kidney Dis</span>
2019 Feb;73(2):258-272.
Epub 2018 Sep 21
doi: 10.1053/j.ajkd.2018.07.012.
<span class="bold">PMID: </span><a href="/pubmed/30249419" target="_blank">30249419</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22interstitial%20nephritis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (77)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/35534373">Acute kidney injury associated with non-steroidal anti-inflammatory drugs.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Klomjit N,
Ungprasert P</span><br />
<span class="medgenPMjournal">Eur J Intern Med</span>
2022 Jul;101:21-28.
Epub 2022 May 6
doi: 10.1016/j.ejim.2022.05.003.
<span class="bold">PMID: </span><a href="/pubmed/35534373" target="_blank">35534373</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35273009">Drug-Induced Acute Kidney Injury.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Perazella MA,
Rosner MH</span><br />
<span class="medgenPMjournal">Clin J Am Soc Nephrol</span>
2022 Aug;17(8):1220-1233.
Epub 2022 Mar 10
doi: 10.2215/CJN.11290821.
<span class="bold">PMID: </span><a href="/pubmed/35273009" target="_blank">35273009</a><a href="/pmc/articles/PMC9435983" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34972976">Renal Toxicity.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Abdelrahim M,
Abudayyeh A</span><br />
<span class="medgenPMjournal">Adv Exp Med Biol</span>
2021;1342:389-397.
doi: 10.1007/978-3-030-79308-1_16.
<span class="bold">PMID: </span><a href="/pubmed/34972976" target="_blank">34972976</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26568188">Renal involvement in primary Sjögren syndrome.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">François H,
Mariette X</span><br />
<span class="medgenPMjournal">Nat Rev Nephrol</span>
2016 Feb;12(2):82-93.
Epub 2015 Nov 16
doi: 10.1038/nrneph.2015.174.
<span class="bold">PMID: </span><a href="/pubmed/26568188" target="_blank">26568188</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20336051">Acute interstitial nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Praga M,
González E</span><br />
<span class="medgenPMjournal">Kidney Int</span>
2010 Jun;77(11):956-61.
Epub 2010 Mar 24
doi: 10.1038/ki.2010.89.
<span class="bold">PMID: </span><a href="/pubmed/20336051" target="_blank">20336051</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (1258)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/35761015">Renal sarcoidosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Calatroni M,
Moroni G,
Reggiani F,
Ponticelli C</span><br />
<span class="medgenPMjournal">J Nephrol</span>
2023 Jan;36(1):5-15.
Epub 2022 Jun 27
doi: 10.1007/s40620-022-01369-y.
<span class="bold">PMID: </span><a href="/pubmed/35761015" target="_blank">35761015</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30454737">Tubulointerstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ruebner RL,
Fadrowski JJ</span><br />
<span class="medgenPMjournal">Pediatr Clin North Am</span>
2019 Feb;66(1):111-119.
doi: 10.1016/j.pcl.2018.08.009.
<span class="bold">PMID: </span><a href="/pubmed/30454737" target="_blank">30454737</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28893923">Drug-Induced Acute Interstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Moledina DG,
Perazella MA</span><br />
<span class="medgenPMjournal">Clin J Am Soc Nephrol</span>
2017 Dec 7;12(12):2046-2049.
Epub 2017 Sep 11
doi: 10.2215/CJN.07630717.
<span class="bold">PMID: </span><a href="/pubmed/28893923" target="_blank">28893923</a><a href="/pmc/articles/PMC5718279" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28284385">IgG4-Related Tubulointerstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Zhang P,
Cornell LD</span><br />
<span class="medgenPMjournal">Adv Chronic Kidney Dis</span>
2017 Mar;24(2):94-100.
doi: 10.1053/j.ackd.2016.12.001.
<span class="bold">PMID: </span><a href="/pubmed/28284385" target="_blank">28284385</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20336051">Acute interstitial nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Praga M,
González E</span><br />
<span class="medgenPMjournal">Kidney Int</span>
2010 Jun;77(11):956-61.
Epub 2010 Mar 24
doi: 10.1038/ki.2010.89.
<span class="bold">PMID: </span><a href="/pubmed/20336051" target="_blank">20336051</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (2060)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/35830831">Facing the Challenge of Drug-Induced Acute Interstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sanchez-Alamo B,
Cases-Corona C,
Fernandez-Juarez G</span><br />
<span class="medgenPMjournal">Nephron</span>
2023;147(2):78-90.
Epub 2022 Jul 13
doi: 10.1159/000525561.
<span class="bold">PMID: </span><a href="/pubmed/35830831" target="_blank">35830831</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35534373">Acute kidney injury associated with non-steroidal anti-inflammatory drugs.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Klomjit N,
Ungprasert P</span><br />
<span class="medgenPMjournal">Eur J Intern Med</span>
2022 Jul;101:21-28.
Epub 2022 May 6
doi: 10.1016/j.ejim.2022.05.003.
<span class="bold">PMID: </span><a href="/pubmed/35534373" target="_blank">35534373</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30454737">Tubulointerstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ruebner RL,
Fadrowski JJ</span><br />
<span class="medgenPMjournal">Pediatr Clin North Am</span>
2019 Feb;66(1):111-119.
doi: 10.1016/j.pcl.2018.08.009.
<span class="bold">PMID: </span><a href="/pubmed/30454737" target="_blank">30454737</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28893923">Drug-Induced Acute Interstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Moledina DG,
Perazella MA</span><br />
<span class="medgenPMjournal">Clin J Am Soc Nephrol</span>
2017 Dec 7;12(12):2046-2049.
Epub 2017 Sep 11
doi: 10.2215/CJN.07630717.
<span class="bold">PMID: </span><a href="/pubmed/28893923" target="_blank">28893923</a><a href="/pmc/articles/PMC5718279" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20336051">Acute interstitial nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Praga M,
González E</span><br />
<span class="medgenPMjournal">Kidney Int</span>
2010 Jun;77(11):956-61.
Epub 2010 Mar 24
doi: 10.1038/ki.2010.89.
<span class="bold">PMID: </span><a href="/pubmed/20336051" target="_blank">20336051</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (2152)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/35830831">Facing the Challenge of Drug-Induced Acute Interstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sanchez-Alamo B,
Cases-Corona C,
Fernandez-Juarez G</span><br />
<span class="medgenPMjournal">Nephron</span>
2023;147(2):78-90.
Epub 2022 Jul 13
doi: 10.1159/000525561.
<span class="bold">PMID: </span><a href="/pubmed/35830831" target="_blank">35830831</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35761015">Renal sarcoidosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Calatroni M,
Moroni G,
Reggiani F,
Ponticelli C</span><br />
<span class="medgenPMjournal">J Nephrol</span>
2023 Jan;36(1):5-15.
Epub 2022 Jun 27
doi: 10.1007/s40620-022-01369-y.
<span class="bold">PMID: </span><a href="/pubmed/35761015" target="_blank">35761015</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35534373">Acute kidney injury associated with non-steroidal anti-inflammatory drugs.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Klomjit N,
Ungprasert P</span><br />
<span class="medgenPMjournal">Eur J Intern Med</span>
2022 Jul;101:21-28.
Epub 2022 May 6
doi: 10.1016/j.ejim.2022.05.003.
<span class="bold">PMID: </span><a href="/pubmed/35534373" target="_blank">35534373</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30454737">Tubulointerstitial Nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ruebner RL,
Fadrowski JJ</span><br />
<span class="medgenPMjournal">Pediatr Clin North Am</span>
2019 Feb;66(1):111-119.
doi: 10.1016/j.pcl.2018.08.009.
<span class="bold">PMID: </span><a href="/pubmed/30454737" target="_blank">30454737</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20336051">Acute interstitial nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Praga M,
González E</span><br />
<span class="medgenPMjournal">Kidney Int</span>
2010 Jun;77(11):956-61.
Epub 2010 Mar 24
doi: 10.1038/ki.2010.89.
<span class="bold">PMID: </span><a href="/pubmed/20336051" target="_blank">20336051</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (998)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/35761015">Renal sarcoidosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Calatroni M,
Moroni G,
Reggiani F,
Ponticelli C</span><br />
<span class="medgenPMjournal">J Nephrol</span>
2023 Jan;36(1):5-15.
Epub 2022 Jun 27
doi: 10.1007/s40620-022-01369-y.
<span class="bold">PMID: </span><a href="/pubmed/35761015" target="_blank">35761015</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32599218">Balancing Cancer Immunotherapy Efficacy and Toxicity.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Johnson DB,
Jakubovic BD,
Sibaud V,
Sise ME</span><br />
<span class="medgenPMjournal">J Allergy Clin Immunol Pract</span>
2020 Oct;8(9):2898-2906.
Epub 2020 Jun 26
doi: 10.1016/j.jaip.2020.06.028.
<span class="bold">PMID: </span><a href="/pubmed/32599218" target="_blank">32599218</a><a href="/pmc/articles/PMC7318967" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26972314">IgG4-related nephropathy.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Quattrocchio G,
Roccatello D</span><br />
<span class="medgenPMjournal">J Nephrol</span>
2016 Aug;29(4):487-93.
Epub 2016 Mar 14
doi: 10.1007/s40620-016-0279-4.
<span class="bold">PMID: </span><a href="/pubmed/26972314" target="_blank">26972314</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26259069">IgG4-related disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kubo K,
Yamamoto K</span><br />
<span class="medgenPMjournal">Int J Rheum Dis</span>
2016 Aug;19(8):747-62.
Epub 2015 Aug 10
doi: 10.1111/1756-185X.12586.
<span class="bold">PMID: </span><a href="/pubmed/26259069" target="_blank">26259069</a></div>
<div class="nl"><a target="_blank" href="/pubmed/20336051">Acute interstitial nephritis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Praga M,
González E</span><br />
<span class="medgenPMjournal">Kidney Int</span>
2010 Jun;77(11):956-61.
Epub 2010 Mar 24
doi: 10.1038/ki.2010.89.
<span class="bold">PMID: </span><a href="/pubmed/20336051" target="_blank">20336051</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (823)</a></div></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">
<div class="nl"><a target="_blank" href="/pubmed/35488310">5-ASA induced interstitial nephritis in patients with inflammatory bowel disease: a systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Moss JG,
Parry CM,
Holt RCL,
McWilliam SJ</span><br />
<span class="medgenPMjournal">Eur J Med Res</span>
2022 Apr 29;27(1):61.
doi: 10.1186/s40001-022-00687-y.
<span class="bold">PMID: </span><a href="/pubmed/35488310" target="_blank">35488310</a><a href="/pmc/articles/PMC9052675" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33561271">Tubulointerstitial nephritis and uveitis syndrome: a systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Regusci A,
Lava SAG,
Milani GP,
Bianchetti MG,
Simonetti GD,
Vanoni F</span><br />
<span class="medgenPMjournal">Nephrol Dial Transplant</span>
2022 Apr 25;37(5):876-886.
doi: 10.1093/ndt/gfab030.
<span class="bold">PMID: </span><a href="/pubmed/33561271" target="_blank">33561271</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33652638">Acute Tubulointerstitial Nephritis in Clinical Oncology: A Comprehensive Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Martínez-Valenzuela L,
Draibe J,
Fulladosa X,
Gomà M,
Gómez F,
Antón P,
Cruzado JM,
Torras J</span><br />
<span class="medgenPMjournal">Int J Mol Sci</span>
2021 Feb 26;22(5)
doi: 10.3390/ijms22052326.
<span class="bold">PMID: </span><a href="/pubmed/33652638" target="_blank">33652638</a><a href="/pmc/articles/PMC7956739" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26231345">Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ramos-Casals M,
Brito-Zerón P,
Seror R,
Bootsma H,
Bowman SJ,
Dörner T,
Gottenberg JE,
Mariette X,
Theander E,
Bombardieri S,
De Vita S,
Mandl T,
Ng WF,
Kruize A,
Tzioufas A,
Vitali C;
EULAR Sjögren Syndrome Task Force</span><br />
<span class="medgenPMjournal">Rheumatology (Oxford)</span>
2015 Dec;54(12):2230-8.
Epub 2015 Jul 31
doi: 10.1093/rheumatology/kev200.
<span class="bold">PMID: </span><a href="/pubmed/26231345" target="_blank">26231345</a><a href="/pmc/articles/PMC6281074" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25738476">Histopathological diagnostic value of the IgG4+/IgG+ ratio of plasmacytic infiltration for IgG4-related diseases: a PRISMA-compliant systematic review and meta-analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Deng C,
Li W,
Chen S,
Zhang W,
Li J,
Hu C,
Wen X,
Zhang F,
Li Y</span><br />
<span class="medgenPMjournal">Medicine (Baltimore)</span>
2015 Mar;94(9):e579.
doi: 10.1097/MD.0000000000000579.
<span class="bold">PMID: </span><a href="/pubmed/25738476" target="_blank">25738476</a><a href="/pmc/articles/PMC4553963" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20nephritis%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (24)</a></div></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C0041349%5bDISCUI%5d&amp;filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (6)</a></li>
<li><a href="/gtr/tests?term=C0041349%5bDISCUI%5d&amp;filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (6)</a></li>
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C0041349%5bDISCUI%5d" target="_blank">See all (6)</a></total></li>
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<div class="portlet_content ln"><ul><li><a href="https://clinicaltrials.gov/search?cond=Interstitial%20nephritis" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Practice_guidelines">Practice guidelines</h1><a sid="121" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22interstitial%20nephritis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li><li><a target="_blank" href="/books/?term=((%22clinical%20guidelines%22%5BResource%20Type%5D)%20OR%20%22practice%20guideline%22%5BPublication%20Type%5D)%20AND%20(%22Interstitial%20nephritis%22)">Bookshelf</a><div class="help-popup">See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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<div class="portlet_content ln"><ul><li><a href="https://vsearch.nlm.nih.gov/vivisimo/cgi-bin/query-meta?v:project=medlineplus&amp;query=Interstitial%20nephritis" target="_blank">MedlinePlus</a></li></ul></div>
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