BackgroundMulticystic dysplastic kidney (MCDK) is a type of kidney dysplasia consisting of many irregular, varioussized cysts divided by dysplastic renal tissue, which negatively impacts kidney function. MCDK is one of the most common renal congenital disorders seen in antenatal ultrasounds. The typical prognosis of MCDK is complete or partial involution that starts antenatally and continues postnatally. The aim of the study was to shed light on the overall outcome of patients with MCDK.
MethodsWe retrospectively collected data on MCDK patients from 2016 until 2022 at King Abdulaziz Medical City, Ministry of National Guard Health Affairs in Saudi Arabia, Riyadh. The data included the recording of epidemiological data, radiological and laboratory reports, and the presence of urological or non-urologically associated anomalies.
ResultsA total of 57 patients with MCDK were reviewed. Seven of them were excluded due to the diagnosis of bilateral MCDK, which was incompatible with life. Of the remaining 50 patients, the right kidney was affected in 52% of them. Most patients were diagnosed antenatally (98%). The mean duration of follow-up for the study was 48 months. Vesicoureteral reflux (VUR) was detected in 22% of the total sample. Overall, 90% of the patients underwent kidney involution. A small percentage had genitourinary anomalies (20%), while a larger percentage (48%) had extrarenal abnormalities.
ConclusionMulticystic dysplastic kidney disease is relatively common in children. The prognosis is affected by the presence of genitourinary and non-genitourinary anomalies. Patients have an overall good prognosis with conservative management. Antenatal screening, diagnosis, and long-term nephrological follow-up are essential for the optimal management of patients.
A 9-year-old boy with end-stage renal disease who was receiving continuous ambulatory peritoneal dialysis (CAPD) presented with acute visual loss and was found to have papilledema. Neuroimaging and cerebrospinal fluid (CSF) analysis were normal. The lumbar puncture opening pressure was 290 mm of water so the diagnosis of pseudotumor cerebri (PTC) was entertained. Medical treatment was not an option because of renal insufficiency; neither was lumbo-peritoneal shunting, because of the peritoneal dialysis. After a lumbo-pleural shunt was placed, there was marked improvement in symptoms. The lumbo-pleural shunt is a reasonable option for treatment for PTC in patients on CAPD who require a CSF divergence procedure.
PVN is a well-known cause of renal allograft dysfunction and failure. The diagnosis is established by examination of tissue from the renal graft, and confirmed by immunohistochemical or in situ hybridization techniques. Electron microscopy can be utilized as an ancillary modality to identify the viral particles ultrastructurally. The tubular epithelial cells are the primary target of PV cytopathic effect; however, PV-associated glomerular changes have also been described. Immune-type electron-dense deposits in the TBMs have been described in the setting of PVN, and rarely, likewise have glomerular subepithelial hump-like deposits. Diffuse immune-mediated proliferative glomerulonephritis in the setting of PVN has not been reported before. In this report, we describe an 11-yr-old kidney transplant recipient boy who developed immune-mediated glomerulonephritis with light microscopic, immunofluorescence, and ultrastructural features compatible with acute PIGN superimposing chronic PVN, discuss this unusual association and the possible mechanisms of antigen clearance in PVN and present a literature review.
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