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Introduction: Primary hyperoxaluria type 1 (PH1) is a rare and inherited condition of urolithiasis. The aim of our study was to analyze clinical, paraclinical, and evolutionary aspects of PH1 in adult patients in our Nephrology department. Methods: We conducted a retrospective single-center study between 1990 and 2021. We collected patients followed for PH1 confirmed by genetic study and/or histopathological features of renal biopsy and morphoconstitutional analysis of the calculi. Results: There were 25 patients with a gender ratio of 1.78. The median age at onset of symptoms was 18 years. A delay in diagnosis more than 10 years was noted in 13 cases. The genetic study found the I244T mutation in 17 cases and 33-34 InsC in 4 cases. A kidney biopsy was performed in 5 cases, on a native kidney in 4 cases and on a graft biopsy in one case. The analysis of calculi was done in 10 cases showing type Ic in 2 cases. After a median follow-up of 13 years (1 year-42 years), 14 patients progressed to end-stage chronic renal failure (ESRD). The univariate study demonstrated a remarkable association with progression to ESRD in our population (44% vs. 56%) RR = 13.32 (adjusted ORs (95% CI): 2.82-62.79) (p < 0.01). Conclusion: Progression to ESRD was frequent in our series. Early diagnosis and adequate management can delay such an evolution.
RESUMO
The COVID-19 pandemic has transformed the health landscape by hampering the management of patients with chronic diseases. Providing optimal healthcare has become a critical issue, especially for patients with end-stage renal disease (ESRD) receiving in-center dialysis. Peritoneal Dialysis (PD) has the advantage of being a home-based therapy. Several papers about COVID-19 in the chronic kidney disease (CKD) population have been published, but few studies focused on the PD population, with limited case series. In this paper, we share our strategy for managing PD patients during the pandemic and describe the characteristics of 24 episodes of COVID-19 that occurred in our PD patients. Also, we report the impact of the pandemic on different outcomes and discuss the challenges of renal replacement therapy (RRT) in the time of COVID-19 and the advantages of PD. During the period from December 2019 to September 2021, 127 patients received PD in our center. Among them, we recorded 24 episodes of COVID-19 that occurred in 20 patients, corresponding to an incidence of 8.4 per 1000 patient-months. None of the 20 patients with COVID-19 were vaccinated and there was a significant male gender predominance in the COVID-19 group compared to the non-COVID-19 group. The prevalence of diabetic nephropathy and primary glomerulonephritis were also significantly higher in the COVID-19 group. The revealing symptoms were asthenia, dry cough, and the deterioration of general conditions in 100%, 75%, and 63% of the patients, respectively. A biological inflammatory syndrome was found in 30% of the patients. Chest computed tomography (CT) scan, performed in 5 patients, showed features of COVID pneumonia with an average extent of damage of 55%. The rate of patients starting PD during the study period was comparable to that before the pandemic. Furthermore, we did not find a significant difference between the infected and the non-infected groups regarding the incidence of peritonitis, PD technique failure, and mortality (6.1 [0-1.46] vs 3.9 [0.15-0.64] deaths per 1000 patient-months. COVID-19 does not seem to have influenced the outcomes of our patients treated with PD even before the launch of mass immunization in our country. Thus, PD can be a great option for RRT in the era of the COVID-19 pandemic since many issues could be managed remotely to avoid regular hospital visits and contribute to maintaining social distancing, which is the cornerstone of breaking the chain of transmission of the novel virus.
