# Multiorgan Failure and Sepsis in an ICU Patient with Prolidase Enzyme Deficiency—The Specificity of Treatment and Care: A Case Report

## TL;DR

A 26-year-old woman with a rare enzyme deficiency faced multiorgan failure and sepsis, highlighting the need for better treatment strategies.

## Contribution

This case report emphasizes the challenges in managing prolidase deficiency through an ICU patient's complex treatment journey.

## Key findings

- The patient developed sepsis and multiorgan failure despite comprehensive ICU interventions.
- Treatment included wound care, amputation, and organ support but ultimately failed to save the patient.
- The case underscores the lack of effective therapies for advanced prolidase deficiency.

## Abstract

Background and Objectives: Prolidase deficiency (PD) is a rare, life-threatening, genetically determined disease with an incidence of 1–2 cases per 1 million births. The disease inhibits collagen synthesis, which leads to organ and systems failure, including hepato- and splenomegaly, immune disorders, chronic ulcerative wounds, respiratory infections, and pulmonary fibrosis. The complexity of the problems associated with this disease necessitates a comprehensive approach and the involvement of an interdisciplinary team. The objective was to present the treatment and care plan, as well as complications of PD, in a young woman following admission to an intensive care unit (ICU). Materials and Methods: A retrospective observational single-case study. Results: A 26-year-old woman with PD was hospitalized in the ICU for acute respiratory failure. The presence of difficult-to-heal extensive leg ulcers and the patient’s immunocompromised condition resulted in the development of sepsis with multiple organ failure (respiratory and circulatory, liver and kidney failure). Complex specialized treatment consisting of wound preparation, limb amputation, the minimization of neuropathic pain, mechanical ventilation, renal replacement therapy, circulatory stabilization, and the prevention of complications of the disease and of therapy were applied. On the 83rd day of hospitalization, the patient expired. Conclusions: Despite the use of complex treatment and care, due to the advanced nature of the disease and the lack of therapies with proven efficacy, treatment was unsuccessful. There is a need for evidence-based research to develop effective treatment guidelines for PD.