Please cite this article in press as: Bekdas M, et al. Congenital nephrotic syndrome secondary to pertussis. Nefrologia. 2019. https://doi.org/10.1016/j.nefro.2019.03.009
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w w w . r e v i s t a n e f r o l o g i a . c o m
RevistadelaSociedadEspañoladeNefrología
Letter
to
the
Editor
Congenital
nephrotic
syndrome
secondary
to
pertussis
Síndrome
nefrótico
congénito
secundario
a
la
tos
ferina
DearEditor,
Nephrotic syndrome shows itself as massive proteinuria, hypoalbuminemia,edemaandhypercholesterolemia.Inthe first year of life,nephrotic syndrome is arare but serious disease.Thephysio-pathology ofcongenitalnephrotic syn-drome(CNS),especiallyinthefirst3monthsoflife,isdifferent from other nephrotic syndromes of childhood. CNS may developas aresultofprimaryor secondarycauses.NPHS1 or NPHS2 genes are detected in primary CNS, while sec-ondaryCNSoftenpresentswithintrauterineinfections,drug reactions,and infantilesystemic lupuserythematosus. The diagnosis ofCNSshould be basedon various criteria such asclinical presentation, family history, laboratory findings, andgenetictesting.AlthoughprimaryCNSprogressesto end-stagerenalfailuredespitevarioustreatments,thetreatment ofsecondaryCNSismorepleasing.1
Pertussis is an acute and contagious infectious diseasecharacterizedbyoverlappingandspasmodic cough-ing attacks. Pertussis can be seen in all age groups.2 CNS
secondarytopertussisisveryrareintheliterature.3,4Inthis
study, we present a case with CNS that secondary to the pertussisininfantandimprovedwithantibiotherapy.
A45-day-oldmaleappliedtohospitalwithcomplaintsof feverandrecurrentcoughingattacks.Hewasbornat3120g withcesarean section after39 weeksofpregnancy. Itwas learnedfromhistory thathismother had persistentcough attacksbeforeonemonthago.Physicalexaminationrevealed arterialtensionwas92/51mmHg,body weight4.8kg(25–50 percentile),height53cm(25thpercentile),bodytemperature 36.2◦C,oxygensaturation80%,pulse160/minandrespiration 66/min.Therewereintercostalretractionsandrales,liverand spleenwerepalpable.
Thepatient’swhitebloodcellwas56,000/mm3(64%
poly-morphonuclearleukocyte,28%lymphocytes,8%monocytes). Bloodgaseswerenormal.ChestX-rayexaminationrevealed pneumonicinfiltration.Thepatientwasgiven100mg/kg cef-triaxoneIVand15mg/kgclarithromycinIV.Bordetellapertussis PCRwasdetectedaspositiveinnasopharynxswabs.
Onthe5thdayoffollow-up,edemawasdetectedonthe eyelids.Inlaboratorytests,albuminwasreportedas2.8g/dl (N:2.5–4.4),totalprotein4.3g/dl(N:4.6–7.4)andtriglyceride 150mg/dl (0–150). In complete urinalysis pH was reported as 8, protein 2+. Protein/creatinine in spot urine was 3.6 (N<0.7).Urineculturewasnegative.Renal ultrasonography revealed nopathology.AntiHCV(−), AntiHIV(−),HBsAg(−), ToxoIgM(−),CMVIgM(−),RubellaIgM(−),andVDRL(−)were detected.GeneticanalysisrevealednomutationinNPHS1and NPHS2genes.Coughattackssignificantlyreduced,andedema and rales improved, and the antibiotics were discontinued onthe10thday.Ondischargetotalproteinwasnormal, pro-tein/creatinineinspoturinewas1.6.Attheageof6months developmentofthepatientwasnormalandprotein/creatinine was0.5inspoturine.
Massive proteinuria is caused from mutations in genes encodingstructuralorregulatoryproteinsoftherenal filtra-tion barrierin the glomerularcapillarywall. Proteinuria is caused bythe lossofsizeand load selectivityprovidedby thisbarrier.5CNSisoftencausedbyprimarycauses,themost
common cause being mutations inthe NPHS1 and NPHS2 genes,respectively.Renalbiopsycannotexplaintheetiology ofthedisease,andpatientsshouldbeexaminedforthesetwo mutationsespeciallyfordefinitediagnosis.6Inourstudy,no
mutationwasdetectedinNPHS1andNPHS2genes.
SecondaryCNSisassociatedwithmanydiseasessuchas cytomegalovirusinfection,toxoplasmosis,congenitalrubella, hepatitis B and human immunodeficiency virus infection. ThistypeofCNSdevelopsduetothetriggeringof nephropa-thyeitherdirectlyorthroughimmunomimeticmechanisms.3
Thesediseases,whichcanberecycledandtreatable,should beinvestigatedinall casesthought.7 Oneofthe factorsof
secondaryCNSispertussisinfection,whichisrarelyreported intheliterature.3,4
Coughingadolescentsandadultsarethemostimportant sourceofpertussisinchildhoodpertussis.Ininfants,the con-centrations oftransplacental pertussis antibodies decrease withahalf-lifeofabout6weeksandin2–6monthsofagethe antibodyagainstB.pertussisbecomesundetectable.8Basedon
Please cite this article in press as: Bekdas M, et al. Congenital nephrotic syndrome secondary to pertussis. Nefrologia. 2019. https://doi.org/10.1016/j.nefro.2019.03.009
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nefrologia2019;xxx(xx):xxx–xxxtheanamnesis,themotherofourcasewasacceptedasthe sourceofpertussis.
Pertussisisdiagnosedbycultureorpolymerasechain reac-tion (PCR). Culture is gold standard in diagnosis, but this methodmaygivefalsenegativeresultsinthelateperiodof infection,invaccinatedindividualsandinantibiotictherapy areas.InrecentyearsPCRhasbecomemorepopular.2
The treatment of secondary CNS is directed at the underlying cause, and nephropathy is improved with the treatment directed against the agent. Macrolides are used in the treatment of pertussis. Macrolides have been shown to reduce the symptoms, espe-cially when they are given in the early stages of the disease, to prevent contamination by increas-ing the elimination of nasopharynx9 and to correct
nephropathy.4
Asa result,pertussis isa rare cause ofsecondary CNS, therefore edema or proteinuria, which can be detected in infantswhoarefollowedduetopertussis,shouldbe stimu-latingforCNS.
Funding
Theauthorshavenofinancialorcompetinginterestsin rela-tiontothiswork.
Conflict
of
interest
Theauthorsdeclarethatthereisnoconflictofinterest.
r
e
f
e
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n
c
e
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2.LongS.Pertussis(BordetellapertussisandBordetella
parapertussis).In:KliegmanRM,StantonB,StGemeJW,editors. Nelsontextbookofpediatrics.20thed.Philadelphia:Elsevier Company;2016.p.1377–82.
3.KimJJ,ClothierJ,SebireNJ,MilfordDV,MoghalN,Trompeter RS.Nephroticsyndromeininfancycanspontaneouslyresolve. PediatrNephrol(Berlin,Germany).2011;26:1897–901,
http://dx.doi.org/10.1007/s00467-011-1911-0.PMID:21611885 [Epubaheadofprint].
4.Kaynak-TurkmenM,Cengiz-ErdemF,SonmezF,GirisgenI, TelliM,BerdeliA.Anewbornwithpertussisaccompanying nephroticsyndrome.TurkJPediatr.2014;56:665–8.PMID: 26388601[Epubaheadofprint].
5.TryggvasonK,PatrakkaJ,WartiovaaraJ.Hereditaryproteinuria syndromesandmechanismsofproteinuria.NEnglJMed. 2006;354:1387–401.
6.KoziellA,GrechV,HussainS,LeeG,LenkkeriU,TryggvasonK, etal.Genotype/phenotypecorrelationsofNPHS1andNPHS2 mutationsinnephroticsyndromeadvocateafunctional inter-relationshipinglomerularfiltration.HumMolGenet. 2002;11:379–88.
7.HolmbergC,JalankoH.Nephroticsyndromeinthefirstyearof life.In:KherKK,GreenbaumLA,SchnaperHW,editors.Clinical pediatricnephrology.3rded.Florida:Taylor&Francis;2017.p. 353–65.
8.WendelboeAM,VanRieA,SalmasoS,EnglundJA.Durationof immunityagainstpertussisafternaturalinfectionor vaccination.PediatrInfectDisJ.2005;24:S58–61. 9.MunozFM,editor.Pertussisininfants,children,and
adolescents:diagnosis,treatment,andprevention.Seminars inpediatricinfectiousdiseases.Elsevier;2006.
MervanBekdasa,∗,RecepErozb, BusraCihana
aDepartmentofPediatrics,AbantIzzetBaysalUniversityMedical
Faculty,Bolu,Turkey
bDepartmentofMedicalGenetics,DuzceUniversityMedical
Faculty,Duzce,Turkey
∗Correspondingauthor.
E-mailaddress:merbek14@yahoo.com(M.Bekdas).
0211-6995/©2019SociedadEspa ˜noladeNefrolog´ıa.Published by Elsevier Espa ˜na, S.L.U. This is an open access article undertheCCBY-NC-NDlicense(http://creativecommons.org/ licenses/by-nc-nd/4.0/).
