Familial Olması Muhtemel Bazı Özellıkler Gösteren Kolon Ve Özofagusta Juvenil Polyposis

Year 1982, Volume: 35 Issue: 3, 621 - 628, 30.09.1982

Uğur Kandilci

Abstract

Nadir raslanan bir generalize juvenil gastrointestinal polyposis vak'ası takdim edildi ve literatür gözden geçirildi. Özofagusta da juvenil polyposis'i olan bu
14 yaşındaki erkek hastada generalize gastrointestinal juvenil polyposis ile birlikte diabetes insipitus ve Jatent diabetes mellitüs de vardı. Önde gelen klinik semptomları kronik rektal kanama, rektal prolapsus, şiddetli anemi, hipoproteinemi ve gelişme geriliği idi. Ailede üç kişinin sağlıklı ve sıhhatli olduğu anlaşıldı. Sekiz yaşında ölen bir erkek kardeşte sunulan vak'aya benzer klinik bulgular mevcutmuş. Erken infant dönemde ölen diğer iki kardeşde benzer klinik durum yokmuş. Anne (35) juvenil rektal polip'li, baba (40) sağ ve sıhhatli idi. Vak'aya destekleyici tedavi, kan transfüzyonları, tekrar edilen polip çıkarma ve koteterizasyonları kan kaybını kontrol altına alamamış ve abdominoperineal rezeksiyonla birlikte distal kolektomi yapılmıştır. Ancak Klinik iyilesme temin edilememigtir. - \ Vak’a, muhtemelen familial olan özofagus polipleri, diabetes insipitiis ve diabetes mellitiis ile birlikte daha ewvel tarif edilmemis bir juvenil kolon poyposis’idir.

Keywords

hipoproteinemi , prolapsus , abdominoperineal

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«Juvenile polyposis of the colon and oesophagus with familial predisposition»

Abstract

In this paper, a unique case of generalized juvenil gastrointestinal polyposis is presented, and medical literature pertinent to this topic is reviewed. In this 14- year-old male, generalized gastrointestinal juvenile polyposis was associated with multiple juvenile polyposis of oesophagus, diabetes insipidus and latent diabetes
mellitus. Rectal prolapsus, chronic recurrent rectal bleeding, severe anemia, hypoproteinemia and growth retardation were presenting symptoms. Family history revealed three siblings, living and well. One brohter had died at the age of eight with a clinical picture similar to the presented case. Two other children died in theire early infancy with unrelated conditions. Mother (35) had juvenil rectal polyp, father (40) was living and well. Since medical treatment consisting supportive measures, blood transfusions and repeated cotorisations and removal of rectal polyps were unable to control blood loss and its results, distal colectomy with abdomino-perineal resection was performed, without apparent clinical improvement. In this case association of oesophageal polyps, diabetes insipidus and diabetes mellitus with juvenile colonic polyposis, and suggestive familial history were distinguishing features which makes the patient unique in these respects.

Keywords

gastrointestinal , hypoproteinemia , polyposis

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