Hereditary angioedema: a decade of human C1-inhibitor concentrate therapy.

BACKGROUND: C1-inhibitor (C1-INH) is a serine protease inhibitor regulating the complement, kinin-kallikrein, coagulation, and fibrinolytic systems. Hereditary angioedema (HAE) is caused by an inherited deficiency of C1-INH characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues. The optional therapy for the acute management of HAE is administration of human C1-INH (hC1-INH) concentrate. However, hC1-INH is not available in many countries, in which case fresh frozen plasma is an alternative. OBJECTIVE: To summarize our experience with hC1-INH concentrate in patients with HAE. METHODS: Clinical and laboratory information on the effectiveness and safety of hC1-INH administered to relieve 468 acute edematous attacks in 61 patients with HAE was analyzed. RESULTS: Severe abdominal or subcutaneous attacks and laryngeal edema were consistently relieved by the administration of 500 U hC1-INH concentrate. Symptoms improved within 15 to 60 minutes of administration. Progression of the attacks was never observed, and there were no recurrent attacks within 72 hours. hC1-INH concentrate requirements did not change after repeated use. hC1-INH concentrate proved effective in the management of 94 attacks in 22 children and 6 attacks in 4 pregnant women. Adverse reactions, viral infections, and antibody formation against the purified protein did not occur. CONCLUSION: The administration of hC1-INH concentrate in HAE is highly effective and safe for the treatment of acute attacks and short-term prophylaxis and in pediatric patients and pregnant women. CLINICAL IMPLICATIONS: Human C1-INH concentrate is effective and safe for the treatment of acute HAE attacks as well as for short-term prophylaxis.

[Ultrasound screening program for neonates and infants in Hungary]. / Ujszülöttek és csecsemók ultrahanggal végzett szúróvizsgálata Magyarországon.

INTRODUCTION: A children's hospital in Budapest (The Madarász Street Children's Hospital) and two children's department of two county hospitals (Toldy Ferenc Hospital, Cegléd and Zala County Hospital, Zalaegerszeg) started a common ultrasound screening programme for children in 1990. OBJECTIVES: This three screening centres agreed which illnesses, pathological states and developmental disorders will be screened. In neonatological departments this screening was carried out usually in the first week of life. In the children's hospital, the majority of measurements was performed between 2 weeks and 3 months of the infants life time. The ratio between this two age group was 43.6 and 56.4%. The authors compared and analysed the results of screenings in neonates and those in infants. Another important objective of the programme was to compare the results of prae- and postnatal screening. METHODS: Examinations were carried out by up-to-date instruments corresponding to the given period. Data for neonates were compared with those for infants and with clinical findings, analysed, stored in computers and yearly evaluated. Screenings were performed by neonatologists, pediatric radiologists or pediatricians with appropriate practice. RESULTS: The three centres examined altogether 51,688 children during their 10-years activity, and found 4758 pathological cases. The majority of pathological cases (3447) was renal and urinary tract disorders, in 1224 cases intracranial occurrences were diagnosed, whereas the remaining cases were mainly tumours or cysts in the liver, spleen or ovarium. CONCLUSION: Numerous pathological changes can be detected by ultrasound screening postnatally, which have great therapeutic significance and are very important for differential diagnostics. By comparing the results for neonates and infants, it can be established that screening in infancy is usually important if no screening was carried out in the neonate age, but control examinations should be performed as well, when it is justified by some physiological or pathological reason. In case of slight deviations, when the first examination cannot provide unambiguous diagnosis, later, repeated examinations can support an accurate diagnosis. Documentation of the results from neonate age and infancy facilitates the correct judgment in later pathological states.