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.

Sarcoidosis is rare multi-system granulomatous inflammatory disorder. It is reported to present by two distinct presentations in childhood. Sarcoidosis presents in the second decade of life by pulmonary infiltration, lymphadenopathy and hepatosplenomegaly, and rarely before the age of 4 years by typical triad of uveitis, rash and arthritis. We report a child 1 year and 9 months with sarcoidosis who presented by one month fever, hepatosplenomegaly, uveitis, pulmonary infiltration and elevated levels of angiotensin-converting enzyme. Chest computerized tomography revealed multiple enlarged mediastinal and multiple hilar lymphadenopathy. A right inguinal lymph node biopsy showed non-caseating granuloma. Other etiologies were investigated for and ruled out. Hence, sarcoidosis can present in children before 4 years with uveitis, hepatosplenomegaly, pulmonary infiltrates, mediastinal and hilar lymphadenopathy, in the absence of rash and arthritis. Sarcoidosis in children is rare and clinical spectrum can vary considerably. Diagnosis of sarcoidosis should be suspected in any child with uveitis.