Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis
Lymphangioleiomyomatosis (LAM) is an orphan cystic lung disease which can occur sporadically or in association with tuberous sclerosis complex (TSC). We report a 26-year-old woman diagnosed with forme fruste of LAM, who presented with bilateral pneumothoraces requiring both surgical and medical p...
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Pusat Perubatan Universiti Kebangsaan Malaysia
2022
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| Online Access: | http://journalarticle.ukm.my/19677/1/21_ms0418_pdf_57898.pdf http://journalarticle.ukm.my/19677/ https://www.medicineandhealthukm.com/toc/17/1 |
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| author | Andrea YLB, Mas Fazlin MJ, Ng, BH Nik Nuratiqah NA, Mohamed Faisal Abdul Hamid, |
| author_facet | Andrea YLB, Mas Fazlin MJ, Ng, BH Nik Nuratiqah NA, Mohamed Faisal Abdul Hamid, |
| author_sort | Andrea YLB, |
| building | Tun Sri Lanang Library |
| collection | Institutional Repository |
| content_provider | Universiti Kebangsaan Malaysia |
| content_source | UKM Journal Article Repository |
| continent | Asia |
| country | Malaysia |
| description | Lymphangioleiomyomatosis (LAM) is an orphan cystic lung disease which can occur
sporadically or in association with tuberous sclerosis complex (TSC). We report
a 26-year-old woman diagnosed with forme fruste of LAM, who presented with
bilateral pneumothoraces requiring both surgical and medical pleurodesis. Clinical
examination and investigations revealed hepatic and renal angiomyolipomas, a left retinal hamartoma, subcortical tubers and subependymal nodules in the brain
as well as diffuse bilateral thin-walled lung cysts of varying sizes bilaterally. The
histopathological examination (HPE) of the lung biopsy confirmed LAM. She was
treated with 12 months of sirolimus with sustained effects on lung function and
cystic lung lesions. Eight months post cessation of sirolimus, she conceived and
delivered twins at 30 weeks. We describe the efficacy and safety of sirolimus and
the successful twin pregnancy in a patient with LAM. This case highlights that
treatment with sirolimus and surgical and medical pleurodesis was successful in
preventing any further worsening of lung function. |
| format | Article |
| id | my-ukm.journal.19677 |
| institution | Universiti Kebangsaan Malaysia |
| language | en |
| publishDate | 2022 |
| publisher | Pusat Perubatan Universiti Kebangsaan Malaysia |
| record_format | eprints |
| spelling | my-ukm.journal.196772022-09-12T03:20:01Z http://journalarticle.ukm.my/19677/ Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis Andrea YLB, Mas Fazlin MJ, Ng, BH Nik Nuratiqah NA, Mohamed Faisal Abdul Hamid, Lymphangioleiomyomatosis (LAM) is an orphan cystic lung disease which can occur sporadically or in association with tuberous sclerosis complex (TSC). We report a 26-year-old woman diagnosed with forme fruste of LAM, who presented with bilateral pneumothoraces requiring both surgical and medical pleurodesis. Clinical examination and investigations revealed hepatic and renal angiomyolipomas, a left retinal hamartoma, subcortical tubers and subependymal nodules in the brain as well as diffuse bilateral thin-walled lung cysts of varying sizes bilaterally. The histopathological examination (HPE) of the lung biopsy confirmed LAM. She was treated with 12 months of sirolimus with sustained effects on lung function and cystic lung lesions. Eight months post cessation of sirolimus, she conceived and delivered twins at 30 weeks. We describe the efficacy and safety of sirolimus and the successful twin pregnancy in a patient with LAM. This case highlights that treatment with sirolimus and surgical and medical pleurodesis was successful in preventing any further worsening of lung function. Pusat Perubatan Universiti Kebangsaan Malaysia 2022-06 Article PeerReviewed application/pdf en http://journalarticle.ukm.my/19677/1/21_ms0418_pdf_57898.pdf Andrea YLB, and Mas Fazlin MJ, and Ng, BH and Nik Nuratiqah NA, and Mohamed Faisal Abdul Hamid, (2022) Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis. Medicine & Health, 17 (1). pp. 282-287. ISSN 2289-5728 https://www.medicineandhealthukm.com/toc/17/1 |
| spellingShingle | Andrea YLB, Mas Fazlin MJ, Ng, BH Nik Nuratiqah NA, Mohamed Faisal Abdul Hamid, Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title | Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title_full | Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title_fullStr | Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title_full_unstemmed | Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title_short | Twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| title_sort | twin pregnancy following sirolimus therapy in lymphangioleiomyomatosis |
| url | http://journalarticle.ukm.my/19677/1/21_ms0418_pdf_57898.pdf http://journalarticle.ukm.my/19677/ https://www.medicineandhealthukm.com/toc/17/1 |
| url_provider | http://journalarticle.ukm.my/ |