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252646

Autosomal Recessive Polycystic Kidney Disease in a Child Complicated by Autoimmune Hemolytic Anemia: A Case Report

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Last updated: 22 Jan 2023

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Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a rare genetic disorder that presents as an isolated polycystic renal disease in childhood, or associated with congenital hepatic fibrosis and/or Caroli disease. The spectrum of complications of ARPKD include end stage kidney disease, systemic hypertension and liver disease. Anemia in ARPKD is commonly due to be reduced erythropoietin or iron deficiency. We present a 2-year old male patient with ARPKD who presented to the emergency room by striking pallor and dark urine. Initial lab work revealed Hemoglobin level 2g/dL.  Blood transfusion was challenging due to difficult typing and frequent mismatch. Other labs showed elevated urea and creatinine, positive direct Coomb's anticoagulant test and positive urine culture.  Imaging was consistent with ARPKD.  The patient was resuscitated and after stabilization, he received pulsed methylprednisolone at 30 mg/day for 5 days followed by prednisone 2mg/kg/day for 4 weeks that was tapered over 2 months with marked improvement. Herein we report autoimmune hemolytic anemia as another and rare cause for anemia associated with ARPKD.

DOI

10.21608/cupsj.2022.126454.1046

Keywords

Autosomal recessive polycystic kidney disease, autoimmune hemolytic anemia, congenital hepatic fibrosis, hepatosplenomegaly

Authors

First Name

Magd

Last Name

Kotb

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

magdkotb@kasralainy.edu.eg

City

-

Orcid

https://orcid.org/00

First Name

Hend

Last Name

Abd El Baky

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

hend.elhossainy@cu.edu.eg

City

Cairo

Orcid

https://orcid.org/my

First Name

Shaimaa

Last Name

Sayed

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

shaimaasayed@kasralainy.edu.eg

City

Cairo

Orcid

0000-0002-9009-165X

First Name

Mohammed

Last Name

Al Komy

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

mohammed_alkomy@hotmail.com

City

-

Orcid

-

First Name

Marwa

Last Name

Onsy

MiddleName

-

Affiliation

Radiology Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

marwaonsy10@gmail.com

City

-

Orcid

-

First Name

Aya

Last Name

Aly

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

aya.s.aly@icloud.com

City

-

Orcid

000000029435543x

First Name

Andrew

Last Name

Tamer

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

andrewnabiltamer1992@gmail.com

City

-

Orcid

0000-0001-8280-6136

First Name

Esraa

Last Name

Mohamed

MiddleName

-

Affiliation

Pediatrics Department, Faculty of Medicine, Cairo University, Cairo, Egypt

Email

essofouad234@gmail.com

City

-

Orcid

0000-0002-7914-7501

Volume

3

Article Issue

1

Related Issue

35726

Issue Date

2023-01-01

Receive Date

2022-03-09

Publish Date

2023-01-01

Page Start

67

Page End

71

Print ISSN

2805-279X

Online ISSN

2682-3985

Article File

CUPSJ_Volume 3_Issue 1_Pages 67-71.pdf

PDF . 1.2MB

Link

https://cupsj.journals.ekb.eg/article_252646.html

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https://cupsj.journals.ekb.eg/service?article_code=252646

Order

10

Type

Case Report

Type Code

1,279

Publication Type

Journal

Publication Title

Pediatric Sciences Journal

Publication Link

https://cupsj.journals.ekb.eg/

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Details

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Article

Created At

22 Jan 2023