Renal transplantation for infantile and juvenile cystinosis: Two case report and review of the literature.

Cystinosis is a rare autosomal recessive lysosomal storage disorder characterized by cystine buildup in various tissues, including the kidneys. Renal involvement is the primary manifestation, leading to end-stage renal disease (ESRD) if left untreated. Kidney transplantation (KT) in patients with cystinosis has significantly improved their prognosis for the disease outcome. Detailed reports on preoperative and Long-term postoperative management in these patients remain sparse. This report discusses the outcomes of two young adult patients of Middle Eastern descent with cystinosis who underwent KT. The first patient, diagnosed with infantile nephropathic cystinosis treated by cystine-depleting therapy, was operated by KT at the age of 18. The second patient, diagnosed with juvenile cystinosis, underwent transplantation at the age of 35 after being treated with hemodialysis. Our report describes detailed pre- and postoperative managements, including laboratory results, and pharmacological interventions. Both cases highlighted the varying clinical manifestations and disease severity between infantile and juvenile cystinosis. Pre-transplant conditions included renal dysfunction, growth retardation, secondary hyperparathyroidism, anemia, and extrarenal manifestations. Following KT, both patients experienced regained renal function, resolution of extrarenal complications, and normalization of laboratory parameters. Furthermore, both patients showed excellent postoperative outcomes with no acute rejection or allograft-related complications. KT is the treatment of choice for cystinosis patients with ESRD. Long-term follow-up post-transplantation is crucial to maintain good graft function. Further studies may elucidate optimal pre- and postoperative management protocols for this rare condition.

Rapid clinical recovery of posterior reversible encephalopathy syndrome in two cases of IgA nephropathy disease and nephrotic syndrome type 9 post-renal transplant.

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome often associated with immunosuppressant drug use, renal disease, and eclampsia and characterized by parieto-occipital vasogenic edema that usually resolves within days. Globally and in the settings of renal transplant, literature concerning PRES is very scattered. In this report, we provide two cases, one diagnosed with IgA nephropathy and another with nephrotic syndrome type 9 that developed episodes of tonic-clonic seizure immediately after renal transplant in case 1 and four days post-transplant in case 2. The two patients had a history of hypertension, renal disease, corticosteroid and immunosuppressant drug use. Neuro-imaging showed typical vasogenic edema involving the parieto-occipital areas. Therefore, the clinical-radiological presentation confirmed the diagnosis of PRES in both cases. Also, rapid neurologic recovery was witnessed upon administration of anti-epileptic and anti-hypertensive medications. Non-discontinuation of immunosuppressants, steroids, early symptomatic management, and blood pressure control are favorable factors that might explain the rapid recovery of these two patients.

Kidney transplantation in a patient with aortic bi-iliac endovascular graft case report and literature review.

Arterial hypertension is a leading cause of both vascular diseases and chronic renal failure. With the increasing incidence of patients suffering from hypertension, an increasing number of patients with hypertensive vascular disease are reported, namely aortoiliac atherosclerosis and aneurysms, needing kidney transplantation (KT). Staged or simultaneous surgical repair of aortoiliac lesions with KT have long been described and studied. In this report, we discuss the case of a patient with infra-renal abdominal aortic aneurysm, having an endovascular bifurcated aortic bi-iliac stent (EVBAIS) introduced, who underwent a KT 3 months after his vascular surgery without any post-operative complication. This case, as well as other previous studies supports the fact that the presence of an EVBAIS does not contraindicate KT.