Author/Editor		Kržišnik, C; Battelino, T; Žerjav-Tanšek, M; Bratanič, N; Bratina-Uršič, N
Title		30 let zdravljenja hipofizne nanosomije
Translated title		Our 30-year experience in the treatment of pituitary dwarfism in Slovenia
Type		članek
Source		Slov Pediatr
Vol. and No.		Letnik 5, št. Suppl 2
Publication year		1998
Volume		str. 60-2
Language		slo
Abstract		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.
Summary		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.
Descriptors		DWARFISM, PITUITARY SOMATOTROPIN CHILD BODY HEIGHT