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Pediatric Nephrology

, Volume 34, Issue 11, pp 2339–2342 | Cite as

Renal failure, respiratory distress, and an atypical purpuric rash in a full-term infant with omphalocele and hypospadias: Answers

  • Aaron J. WeissEmail author
  • Kenny KronforstEmail author
Clinical Quiz

Answers

What is the differential diagnosis for this pathology?

Given the unique presenting symptoms and noted congenital anomalies, an expansive differential was generated. The presence of an intracardiac lesion with concurrent lower extremity purpuric rashes prompted providers to consider endocarditis as an etiology. Initially, concern for infectious endocarditis complicated by embolic cascade and acute kidney injury was high. A non-cardiac source of infection complicated by disseminated intravascular coagulation (DIC) was also postulated. Hereditary thrombophilia with subsequent renal artery thrombosis and/or adrenal infarct was possible as well. Additionally, although unusual in this population, vasculitis was considered due to the appearance of the nearly symmetric purpuric rash on the infant’s bilateral feet. Congenital adrenal hyperplasia was briefly discussed due to the pattern of electrolyte abnormalities, although this was ultimately attributed to acute renal failure.

What...

Keywords

Neonate Infant Atypical HUS Complement-mediated HUS Complement factor H (CFH) Eculizumab 

Abbreviations

aHUS

Atypical hemolytic uremic syndrome

HUS

Hemolytic uremic syndrome

STEC

Shiga toxin-producing Escherichia coli

MAC

Membrane attack complex

BUN

Blood urea nitrogen

CRP

C-reactive protein

WBC

White blood cells

DIC

Disseminated intravascular coagulation

CFH

Complement factor H

MCP

Membrane cofactor protein

CD46

Cluster of differentiation 46

C5

Complement component 5

ADAMTS13

A disintegrin-like and metalloproteinase with thrombospondin 13

TTP

Thrombotic thrombocytopenic purpura

THBD

Thrombomodulin

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Informed consent

Family provided consent for the patient included in this manuscript.

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Copyright information

© IPNA 2019

Authors and Affiliations

  1. 1.McGaw Medical Center of Northwestern UniversityChicagoUSA
  2. 2.Division of NeonatologyAnn & Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA

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