Life-threatening laryngeal attacks in hereditary angioedema patients

Background: Hereditary angioedema due to C1-inhibitor deficiency (HAE-C1INH) is a rare genetic disease that runs in the family. As a result of the disease, acute swellings of the subcutaneous tissue and mucous membranes of the digestive and respiratory systems, including the larynx, occur. Any attack of the disease involving the throat and larynx is particularly dangerous and requires knowledge of clinical determinants of the disease and its proper management. Materials and methods: The study included adult consecutive HAE-C1INH patients having follow-up visits in our centre. The group was examined with a structured clinical questionnaire, concerning the last 6 months and focusing particularly on laryngeal swelling attacks. Results: 55 subjects (F/M – 35/20, age range – 18–76) were included in the study. Laryngeal attacks occurred in 19 individuals (34.5%): 1–3, 4–6, and ≥7 attacks in 9, 8 and 2 patients, respectively, two of whom required intubation. In comparison to other patients, subjects with laryngeal attacks were characterised by significantly more frequent: (1) facial attacks, (2) severe disease activity, (3) the occurrence of female patients, (4) mental stress as a trigger of attacks. All patients with laryngeal attacks had a rescue medication at home and 15/19 (78%) patients could use it at home. Most of them used plasma-derived C1-inhibitor 17/19 (89.5%) and icatibant, 8/19 (42.1%). Discussion: HAE-C1INH patients with laryngeal attacks require particular attention. Proper training regarding the identification of these patients, adequate management, access to emergency services and emergency drugs are essential to ensure the safety of subjects with this localization of HAE-C1INH attacks

The effect of hemodynamically-guided hypotensive therapy in one-year observation: Randomized, prospective and controlled trial (FINEPATH study)

Background: The use of impedance cardiography (ICG) revealed to provide beneficial blood pressure (BP) lowering effect. However, the follow-up in previous trials was short and brachial BP was the only evaluated hemodynamic variable. Thus, we aimed to estimate the influence of ICG-guided therapy on brachial and central BP, impedance-derived hemodynamic profile and echocardiographic features after 12 months in a randomized, prospective and controlled trial (NCT01996085). Methods: One hundred and forty-four hypertensives were randomly assigned to groups of empiric (GE) and ICG-guided therapy (HD). Office BP, ambulatory BP monitoring, central BP and echocardiography (left ventricular hypertrophy and diastolic function assessment) were performed before and after 12 months of treatment. Results: Blood pressure reduction was higher in HD (office BP: 21.8/14.1 vs. 19.9/11.8 mm Hg; mean 24-h BP: 19.0/10.9 vs. 14.4/9.2 mm Hg). However, the only statistically significant differences were: percentage of patients achieving BP reduction of minimum 20 mm Hg for of­fice diastolic BP (27.3% vs. 12.1%; p = 0.034) and mean 24-h systolic BP (49.1% vs. 27.3%; p = 0.013). More pronounced improvement in the left ventricular diastolic dysfunction (delta E/A 0.34 vs. 0.12, p = 0.017) was the only other beneficial hemodynamic effect. Conclusions: Beneficial BP lowering effect of hemodynamically-guided pharmacotherapy, observed previously in short-term observation, persists over time. Hemodynamic effects of such a treatment approach, especially those of prognostic value (central BP, myocardial hypertrophy), should be evaluated in further studies including patients with resistant hypertension, heart failure, diabetes mellitus and chronic kidney disease

Zagrażające życiu napady krtaniowe u chorych z wrodzonym obrzękiem naczynioruchowym

Wstęp: Wrodzony obrzęk naczynioruchowy z niedoboru C1-inhibitora (HAE-C1INH) to rzadka choroba genetyczna wystę-pująca rodzinnie. W jej wyniku dochodzi do napadowych obrzęków tkanki podskórnej oraz śluzówek układu pokarmowegoi oddechowego, w tym krtani. Napady obrzęków gardła i krtani są szczególnie niebezpieczne, wymagają znajomości uwarun-kowań klinicznych, a także właściwego postępowania. Materiały i metody: Badaniem objęto dorosłych pacjentów z HAE-C1INH kolejno zgłaszających się na wizyty kontrolne w naszym ośrodku. Grupę przebadano przy pomocy usystematyzowanego kwestionariusza klinicznego, ze szczególnym uwzględnieniem obrzęków krtani. Pytania odnosiły się do ostatnich 6 miesięcy poprzedzających badanie. Wyniki: Do badania włączono 55 dorosłych osób (K/M – 35/20) w wieku 18–76 lat. Napady krtaniowe występowały u 19 z nich (34,5%): 1–3 napady u 9 osób, 4–6 napadów u 8 osób, ≥7 u 2 osób. U 2 badanych konieczne było wykonanie intubacji. W po-równaniu z pacjentami bez napadów krtaniowych, u tych z takimi napadami znamiennie częściej obserwowano: (1) napady obrzęków twarzy, (2) ciężki stopień nasilenia choroby, (3) występowanie chorych płci żeńskiej, (4) stres psychiczny jako czynnik wywołujący napady. Wszyscy pacjenci z obrzękami krtaniowymi posiadali w domu lek do przerywania napadu. Najczęściej stosowali oni dożylny osoczopochodny C1-inhibitor – 17/19 (89,5%) lub podskórny inhibitor receptorów bradykininy (ikaty-bant) – 8/19 (42,1%). U 15/19 (79%) osób lek podawany był samodzielnie przez pacjenta lub osobę bliską. Dyskusja: Pacjenci z napadami krtaniowymi w przebiegu HAE są szczególnie wymagającą grupą chorych. Właściwe prze-szkolenie dotyczące ich identyfikacji i postępowania z nimi, zabezpieczenie w leki ratunkowe oraz szybki dostęp do jednostek pomocy doraźnej są niezbędne dla zapewnienia bezpieczeństwa osobom z tą lokalizacją obrzęków HAE

Hypersensitivity Reactions in Serious Adverse Events Reported for Paracetamol in the EudraVigilance Database, 2007–2018

Paracetamol is a popular and easily available drug which is used world-wide as analgesic, antipyretic agent. Hypersensitivity reactions to this drug involve a wide range of symptoms of various importance for patient management. The EudraVigilance (EV) database serves as a system for monitoring adverse events (AE) due to drug intake. We retrospectively recorded AE reports for “paracetamol” reported from 1 January 2007 to 1 October 2018 which fulfilled the category of “serious” in EV. For further analysis the retrieved AE reports were selected according to the keywords corresponding to hypersensitivity symptoms. We included in the study 4589 AE reports with 9489 particular AEs. 24.2% of all the AE reports concerned children. The most often reported symptoms were “angioedema,” “rash” and “urticaria” (each of them with a frequency of >10% in the AE reports). An important group of AEs were oedema reported as being located in the head, neck or respiratory tract. We recorded 58 AE reports with fatal outcomes, including 9 Stevens-Johnson syndrome/toxic epidermal necrolysis cases (SJS/TEN), 10 anaphylactic reactions, 21 cases of hepatic failure and a further 18 cases which occurred for other reasons. SJS/TEN, acute generalized exanthematous pustulosis and drug reaction with eosinophilia and systemic symptoms were reported 129, 42 and 25 times, respectively. Prodromes and symptoms of potentially life-threating SJS/TEN appeared in 286 of the AE reports. 380 AE reports pointed to a diagnosis of anaphylaxis. To improve patient safety, healthcare professionals, including pharmacists, can identify warning signs of severe hypersensitivity reactions to paracetamol

Summary of recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency

The paper summarizes the recommendations of the Hereditary Angioedema Section of the Polish Society of Allergology regarding the management of this disease in a view on up-to-date data. Its classification, pathomechanism, clinical picture and diagnosis were discussed. The principles of treatment and prevention of edema attacks and disease monitoring were presented. Characteristic features related to the course and management during pregnancy and in the pediatric group of patients were mentioned.W pracy podsumowano, w świetle zaktualizowanych danych, zalecenia Sekcji Wrodzonego Obrzęku Naczynioruchowego Polskiego Towarzystwa Alergologicznego dotyczące postępowania w tym schorzeniu. Omówiono jego klasyfikację, patomechanizm, obraz kliniczny i rozpoznawanie. Przedstawiono zasady leczenia i zapobiegania napadom obrzęku oraz monitorowania choroby. Wymieniono cechy charakterystyczne odnoszące się do przebiegu i postępowania w okresie ciąży i w pediatrycznej grupie chorych