THINK PORPHYRIA: CASE REPORT AND REVIEW OF LITERATURE.

Porphyrias are a group of rare disorders caused by enzyme defects in haem biosynthesis pathway. Acute intermittent porphyria is the most common hepatic porphyria. The disorder presents with severe neuropathic abdominal pain that can be accompanied by a wide range of gastrointestinal, psychiatric and neurological symptoms, making the diagnosis clarification very challenging. We report a case of a 27-year-old female patient who presented with acute abdominal pain, vomiting and marked hyponatremia, developed seizures and disorientation, and eventually required intensive care unit treatment to maintain breathing. Her symptoms were initially misinterpreted as a functional gastrointestinal disorder, thus delaying the needed specific treatment. She was diagnosed a week after the initial hospital admission, and her condition improved after receiving treatment with intravenous glucose and haemin. For patients with acute neurovisceral attacks, early clinical recognition is essential. Severe hyponatremia, urine that develops orange colour on exposure to light and gastrointestinal symptom combination with neurologic symptoms are three valuable clues that may lead to the right diagnosis faster. Pathophysiology of hyponatremia in case of acute intermittent porphyria in only partly understood and can be associated with syndrome of inappropriate antidiuretic hormone secretion, gastrointestinal or renal sodium loss.

Breath Sensor Technology for the Use in Mechanical Lung Ventilation Equipment for Monitoring Critically Ill Patients.

BACKGROUND: The need for mechanical lung ventilation is common in critically ill patients, either with COVID-19 infection or due to other causes. Monitoring of patients being ventilated is essential for timely and improved management. We here propose the use of a novel breath volatile organic compound sensor technology to be used in a mechanical lung ventilation machine for this purpose; the technology was evaluated in critically ill COVID-19 patients on mechanical lung ventilation. METHODS: Based on the consistency results of our study data, the breath sensor device with metal oxide gas sensors and environment-controlling sensors was mounted on the ventilation exhaust port of the ventilation machine; this allowed to ensure additional safety since the device was placed outside the contour between the patient and equipment. RESULTS: The sensors allowed stable registration of the signals for up to several weeks for 10 patients in total, depending on the storage amount; a proportion of patients were intubated or received tracheostoma during the evaluation period. Future studies are on the way to correlate sensor readings to other parameters characterizing the severity of the patient condition and outcome. CONCLUSIONS: We suppose that such technology will allow patient monitoring in real-time for timely identification of deterioration, potentially requiring some change of management. The obtained results are preliminary and further studies are needed to examine their clinical significance.