Mean age of patients was 5.253 [+ or -] 4.80 years for SRNS
(range from 2 to 17) and 7.7037 [+ or -] 2.829 for SSNS (age range from 2 to 15 years), while mean age at diagnosis was 5.420 [+ or -] 4.114 years for SRNS
and 4.666 [+ or -] 2.650 years for SSNS.
Cyclophosphamide, although widely used in the past to treat SRNS
, is now thought to have little therapeutic efficacy in the treatment of this condition.
It has also not escaped of our notice that 5 out of 7 from the NPHS2 or WT1 disease-causing mutations were detected in a small subgroup of children with SRNS
(n = 7) manifesting in the first year of life.
To provide a basis for evidence-based decision making regarding the use of agency-employed SRNs
, more research exploring the relationship between SRNs
and independent patient outcomes in larger datasets is warranted.
In light of the "promptness and extent of SRNS
' response to the event" the DOE has decided to grant partial mitigation of the base civil penalty of $225,000.
Reasons for the ICU stays were respiratory distress due to pleural effusion in a child with FRNS/SDNS, respiratory distress due to bacterial pneumonia in a child with FRNS/SDNS, hyponatremia in a child with FRNS/SDNS, and seizures from hypertension in a child with SRNS
. Twelve of the 87 (14%) patients experienced serious complications in the first 3 years of disease: two patients with SSNS, seven patients with FRNS/SDNS, and 3 patients with SRNS
Mutations in NPHS2 gene, located at 1q25-31, are the most common cause of SRNS
in childhood and were first described by Boute et al.
There were 49 SSNS patients in relapse stage, 64 SSNS patients in remission stage, and 15 SRNS
Recent studies have demonstrated that mutations in WT1 can lead to syndromic forms of steroid-resistant NS (SRNS
), such as Denys-Drash or Frasier syndrome, and can cause isolated SRNS