| Avtor/Urednik | Kržišnik, C; Battelino, T; Žerjav-Tanšek, M; Bratanič, N; Bratina-Uršič, N | |
| Naslov | 30 let zdravljenja hipofizne nanosomije | |
| Prevedeni naslov | Our 30-year experience in the treatment of pituitary dwarfism in Slovenia | |
| Tip | članek | |
| Vir | Slov Pediatr | |
| Vol. in št. | Letnik 5, št. Suppl 2 | |
| Leto izdaje | 1998 | |
| Obseg | str. 60-2 | |
| Jezik | slo | |
| Abstrakt | Congenital and acquired forms of pituitary dwarfism including hereditary growth hormone (GH) deficiency due to different molecular defects, are described. Treatment of first 21 patients (18 boys, 3 girsl) with human GH, 5 withisolated GH deficiency and 16 with multiple pituitary hormone deficiencies is presented. The age at the commencement of GH therapy was 9.9 +- 4.3 years. Replacement therapy for hypothyroidism was started at the age of 7.9 +- 1.2 years, and for hypogonadism at the age of 16.8 +- 1.2 years in boys and 15.8 +- 1.6 years in girls. The therapy with GH in the dose of 2 IU/1 m2 for at least 3 years has improved the height SD score from - 4.16 +- 1.1 at the commercement of therapy to - 1.64 +- 0.98 in adulthood. The final height in males was 16.5 +- 5.9 cm and in females 152.8 +- 1 cm. There were no side effects during GH treatment. In some patients, various psychological problems were registered, in spite of significantly improved final height compared to controls. | |
| Izvleček | Prikazani so prirojeni in pridobljeni vzroki pomanjkanja ali neučinkovitosti rastnega hormona (RH) vključno z dednimi oblikami nanosomije, ki je posledica različnih genskih okvar na molekularnem nivoju. Poleg klinične slike je predstavljeno zdravljenje z RH prvih 21 bolnikov s hpofizno nanosomijo (18 dečkov, 3 deklice) v Sloveniji. Pri petih jebilo diagnosticirano izolirano pomanjkanje RH, pri šestnajstih multipli deficit hipofiznih hormonov. Zdravljenje z RH je bilo začeto v starosti 9,9 +- 4,3 let, nadomestno zdravljenje hipotiroze v starosti 7,9 +- 1,2 let in hipogonadizma 16,8 +- 1,2 let dečkih ter 15,8 +- 1,6 pri deklicah. Zdravljenje z RH v dozi 2 IU/m2 vsaj tri leta je zmanjšalo deficit v višini od - 4,16 +- 1,1 na - 1,64 +- 0,98 SDS. Končna višina pri moških je znašala 165,1 +- 165,1 +- 5,9 cm, pri ženskah 152,8 +- 1 cm. Stranskih učinkov pri zdravljenju ni bilo, posamezniki so imeli psihološke probleme kljub socialno sprejemljivi končni višini. | |
| Deskriptorji | DWARFISM, PITUITARY SOMATOTROPIN CHILD BODY HEIGHT |