Familial Neurofibromatosis-1 and Gilbert Syndrome

Koçer, Uğur; Uysal, Ahmet Çağrı; Sungur, Nezïh; Karaaslan, Önder; Kankaya, Yüksel; Tiftikçioğlu, Yiğit Özer

doi:10.1046/j.1524-4725.2003.29192.x

Cited by 2 publications

(2 citation statements)

References 40 publications

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“…Üzerlerindeki epidermis diğer alanlara göre daha koyu renkli ve fazlaca tüylenme gösterebilir (9). Çok daha büyük olan pleksiform nörofibromlarda tümör üzerindeki derinin birbiri üzerine katlanmasıyla lokalize bir kutis laksa görünü-mü oluşabilir (10). Bazı hastalarda şiddetli kaşıntı ciddi bir sorun oluşturabilir.…”

Section: Discussionunclassified

Plexiform Neurofibroma on Giant Congenital Melanocytic Nevi - Neurofibromatosis Type 1 Case

Basar¹,

Başkan²,

Sarıcaoğlu³

et al. 2011

Turk J Dermatol

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ÖzetPleksiform nörofibromatozis, Schwann hücrelerinden köken alan nadir bir sinir kılıfı tümörüdür. Histolojik olarak benin karakterli olmasına karşın, kozmetik ve fonksiyonel deformitelere yol açabilir. Nörofibromatozis (NF)'in diğer belirtileri ile birlikte veya izole olabilir. Bu lezyonlarda yaklaşık %2-5 oranlarında malin dönüşüm riski vardır. Bu makalede; sağ torakal bölgesinde dev boyutlu konjenital kıllı nevüsü üzerinde, gittikçe büyüme gösteren ve ağrılı, kızıl-kahverengi pigmente pleksiform nörofibroma gelişen, 23 yaşında bayan olgu sunuldu. Bu sunumda pleksiform nörofibromatozisin eşlik edebilecek diğer deri bulguları, deformiteleri ve malin değişim riski literatür bilgileri eşliğinde derlendi. (Turk J Dermatol 2011; 5: 56-60 AbstractPlexiform neurofibromatosis is a rare nerve sheath tumor originating from schwann cells. Although histologically benign, it can cause cosmetic and functional deformity. It may be solitary or can accompany other signs of NF. The rate of malignant transformation in this lesion is 2-5%. In this article, a 23 year old woman with painful, red brown pigmented plexiform neurofibroma over the giant congenital hairy nevus in the right thoracic region was presented. In this presentation, accompanying other skin manifestations of plexiform neurofibromatosis, deformities and rate of malignancy in the literature was reviewed. (Turk J Dermatol 2011; 5: 56-60)

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Section: Discussionunclassified

Plexiform Neurofibroma on Giant Congenital Melanocytic Nevi - Neurofibromatosis Type 1 Case

Basar¹,

Başkan²,

Sarıcaoğlu³

et al. 2011

Turk J Dermatol

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“… 4 , 5 The typical presentation of this syndrome is mild unconjugated hyperbilirubinaemia, and indirect bilirubin levels usually exceed the upper limit of normal after fasting, dehydration, menstruation, intercurrent disease, and overexertion. 6 , 7 Other nonspecific symptoms including epigastric fullness, abdominal pain, fat intolerance, and fatigue have also been reported. 8 , 9 Fusion surgery of spinal deformity with hyperbilirubinemia may induce liver dysfunction or multiple organ failure.…”

Section: Introductionmentioning

confidence: 99%

Scoliosis in a Patient With Gilbert Syndrome

Shen²,

Liang³

2015

Medicine

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Gilbert syndrome (GS) is mainly characterized by intermittent unconjugated hyperbilirubinemia in the absence of hepatocellular disease or hemolysis. Little data are available on operative outcomes in GS patients with spinal deformity surgery.This study has presented a case of GS occurring in the patient with scoliosis.The patient was a 30-year-old female with scoliosis and GS. She was taken a correction form Thoracic 2 to Lumbar 1) levels by using the USS-II spinal system. At 2 years follow-up, the patient was well balanced and pain free. Plain radiographs demonstrated spine solid fusion without correction loss.Although complex scoliosis surgery can be performed safely in these patients with GS, careful perioperative managements including liver function and coagulation function are required.

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Familial Neurofibromatosis-1 and Gilbert Syndrome

Abstract: An interesting diagnosis of NF occurring coincidentally with Gilbert's disease in a family is reported. This clinical study is the first report of Gilbert's disease accompanying familial NF.

Cited by 2 publications

References 40 publications

Plexiform Neurofibroma on Giant Congenital Melanocytic Nevi - Neurofibromatosis Type 1 Case

Plexiform Neurofibroma on Giant Congenital Melanocytic Nevi - Neurofibromatosis Type 1 Case

Scoliosis in a Patient With Gilbert Syndrome

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