Ligneous Gingivitis Due to Type I Plasminogen Deficiency in a Hydrocephalic Patient

Çimen, Müge; Özer, Sezin; Özyılmaz, Berk

doi:10.5336/caserep.2018-64148

Cited by 1 publication

(4 citation statements)

References 9 publications

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“… 9 , 10 LC is the most common presentation in the affected patients that are accounting for at least 81%. 1 It is a rare form of chronic pseudomembranous conjunctivitis, that results from the development of fibrin-rich, woody-like pseudomembranous lesions mainly on the tarsal conjunctiva. 11 Typically, it is preceded by conjunctival erythema and chronic tearing, followed by the development of red, yellow-white, or white masses.…”

Section: Discussionmentioning

confidence: 99%

“…Congenital hydrocephalus is uncommon in patients with PLG I deficiency and has been reported particularly in association with the more severe forms, representing 12% of the cases. 1 The clinical course is generally complicated by shunt thrombotic occlusion and subsequent decompensated hydrocephalus. However, the etiology of hydrocephalus nevertheless remains uncertain; it has been attributed, mainly within the fetal period, to a CSF block that may be resulting from extravascular fibrin deposits.…”

Section: Discussionmentioning

confidence: 99%

“…There are two types of congenital PLG deficiency which are type I hypoplasminogenemia and type II dysplasmincogenemia. 1 Type I plasminogen (PLG I) deficiency is a genetic disorder inherited in an autosomal recessive mode that affects 1.6 per 1 million people, 2 with a female to male ratio 1.55:1. 3 K19E mutation is the most common molecular genetic defect worldwide.…”

Section: Introductionmentioning

confidence: 99%

“… 4 The severe form of PLG I deficiency has broad clinical manifestation including ligneous conjunctivitis (LC), ligneous gingivitis, respiratory tract, and female genital tract involvement as well as congenital occlusive hydrocephalus. 1 , 5 The prognosis is variable based on the extent, length, and site of the symptoms. 6 …”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Congenital hydrocephalus and ligneous conjunctivitis in two children with severe type I plasminogen deficiency: A case report and literature review

Jaber,

Abdallah

et al. 2024

SAGE Open Medical Case Reports

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Type I plasminogen (PLG I) deficiency is a genetic disorder inherited in an autosomal recessive mode and carries high mortality and morbidity. This case report discusses two babies, aged 2 and 3 months, who were diagnosed with ligneous conjunctivitis and congenital hydrocephalus. They had progressive macrocephaly, which led to the insertion of a ventriculoperitoneal shunt. However, there was no significant improvement. During the course of the disease, they underwent genetic testing and were diagnosed with PLG I deficiency. One of the babies underwent ventriculocholecystic shunt insertion as part of palliative care and management, since this disease has poor absorption in the peritoneal cavity. Unfortunately, there was no improvement observed, and he died at 18 months. The other baby received intravenous plasma (10 ml/kg) three times a week, plus using several eye drops daily, with moderate improvement. Promising results are expected with the approved plasminogen, human-tvmh, by the Food and Drug Administration. However, access to the newly approved drug in developing countries is challenging, often hindered by cost or supply issues, necessitating the use of alternative treatments.

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