pulmonary lymphangioleiomyomatosis
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2022 ◽  
pp. 153758
Author(s):  
Chihiro Inoue ◽  
Yasuhiro Miki ◽  
Ryoko Saito-Koyama ◽  
Kazuma Kobayashi ◽  
Kuniaki Seyama ◽  
...  

2021 ◽  
Vol 24 (9) ◽  
pp. 701-703
Author(s):  
Jalal Heshmatnia ◽  
Maryam Sadat Mirenayat ◽  
Mitrasadat Rezaei ◽  
Felix Bongomin ◽  
Mehrdad Bakhshayeshkaram ◽  
...  

Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease principally affecting women during childbearing years and eventually leading to progressive respiratory failure. Lung transplantation is a viable option for patients with end-stage disease. LAM-related complications remain common, but recurrence of LAM following allograft transplantation is rare. We present a 25-year-old woman who presented with progressive dyspnea five years after bilateral lung transplantation for end-stage LAM. Histological examination of transbronchial lung biopsy sample confirmed recurrent LAM. We changed cyclosporine to sirolimus and she is currently being considered for re-transplantation.


2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Ines Rolim ◽  
Marquetta Makupson ◽  
Aleksandra Lovrenski ◽  
Carol Farver

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Nicola Alesi ◽  
Elie W. Akl ◽  
Damir Khabibullin ◽  
Heng-Jia Liu ◽  
Anna S. Nidhiry ◽  
...  

AbstractTuberous Sclerosis Complex (TSC) is caused by TSC1 or TSC2 mutations, resulting in hyperactivation of the mechanistic target of rapamycin complex 1 (mTORC1). Transcription factor EB (TFEB), a master regulator of lysosome biogenesis, is negatively regulated by mTORC1 through a RAG GTPase-dependent phosphorylation. Here we show that lysosomal biogenesis is increased in TSC-associated renal tumors, pulmonary lymphangioleiomyomatosis, kidneys from Tsc2+/− mice, and TSC1/2-deficient cells via a TFEB-dependent mechanism. Interestingly, in TSC1/2-deficient cells, TFEB is hypo-phosphorylated at mTORC1-dependent sites, indicating that mTORC1 is unable to phosphorylate TFEB in the absence of the TSC1/2 complex. Importantly, overexpression of folliculin (FLCN), a GTPase activating protein for RAGC, increases TFEB phosphorylation at the mTORC1 sites in TSC2-deficient cells. Overexpression of constitutively active RAGC is sufficient to relocalize TFEB to the cytoplasm. These findings establish the TSC proteins as critical regulators of lysosomal biogenesis via TFEB and RAGC and identify TFEB as a driver of the proliferation of TSC2-deficient cells.


2021 ◽  
Vol 11 (6) ◽  
pp. 226-230
Author(s):  
Lamya Al Aamri ◽  
Raafat Dahrouj ◽  
Nasser Al Awaid

Lymphangioleiomyomatosis (LAM) is a rare multisystem disease, predominately affect premenopausal female. LAM could be an inherited disease associated with Tuberous Sclerosis Complex syndrome or sporadic. Most common pulmonary symptoms are dyspnea and pneumothorax. We report 31- year female, presented with right-side pneumothorax chest drainage was inserted. Further investigation revealed multiple cystic lesions in chest computed tomography images suggestive of LAM disease. She underwent video-assisted thoracoscopic surgery (VATs) to obtain a lung biopsy. Histopathology lung tissue confirms pulmonary Lymphangioleiomyomatosis. Key words: Lymphangioleiomyomatosis; Tuberous Sclerosis Complex syndrome; video-assisted thoracoscopic surgery.


Author(s):  
Kusum V Shah ◽  
Sonal Goyal ◽  
Saket Kumar ◽  
Arti D Shah ◽  
Yash Rana

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease, mostly seen in women of reproductive age group. Most common presentation is dyspnoea on exertion, may be associated with haemoptysis and chest pain. LAM is a cystic lung disease, with a poor prognosis and is difficult to treat. In this case reports, we discussed two patients who presented with dyspnoea and chest pain due to pneumothorax and were later diagnosed as case of LAM on clinical and radiological basis. Intercostal Chest tube Drainage (ICD) was inserted for resolution of pneumothorax and subsequently pleurodesis was done to prevent recurrent pneumothorax. In case of recurrent pneumothorax, LAM should be considered as a differential diagnosis. Pleurodesis can be considered to prevent recurrent pneumothorax.


2020 ◽  
Vol 202 (10) ◽  
pp. 1373-1387 ◽  
Author(s):  
Minzhe Guo ◽  
Jane J. Yu ◽  
Anne Karina Perl ◽  
Kathryn A. Wikenheiser-Brokamp ◽  
Matt Riccetti ◽  
...  

2020 ◽  
Vol 2020 (11) ◽  
Author(s):  
Lakshmi Srinivasan ◽  
Saifullah Mohamed ◽  
Akshay Patel ◽  
Khurum Mazhar ◽  
Shilajit Ghosh

Abstract Pulmonary lymphangioleiomyomatosis (LAM) is a rare, well-described pathology and usually is exclusive to females of a reproductive age. We present a 45 year-old lady who presented to the surgeons 1 year after an admission with acute dyspnoea following influenza infection. Initial computed tomography imaging findings demonstrated severe, heterogenous right-sided bullous emphysematous changes, but histopathological analysis of the post-operative specimen favoured a diagnosis of LAM. This case demonstrates the importance of considering LAM as a differential diagnosis for findings of unilateral emphysema or lobar emphysema, in which alpha 1-antitrypsin deficiency has been excluded and in those without a significant smoking history.


Author(s):  
Susmita Reddy Karri ◽  
Amy Hsieh ◽  
John Binder ◽  
Vasant Shenoy

Objective: Hypopituitarism in post-partum women is usually attributed to lymphocytic hypophysitis (LH), but can also be secondary to systemic pathology. We discuss a case of a lady presenting with central hypogonadism and diabetes insipidus, who further developed persistent cough, leading to an unexpected diagnosis of burnt-out Langerhans’ cell histiocytosis (LCH). Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) and open-lung biopsy results are discussed. Results: A 28 year old lady, presented at 10 months postpartum with polydipsia, polyuria and amenorrhoea for 3 months. Her results showed prolactin of 25 (reference,<23.5) μg/L , oestrogen of 24.8 (reference,30–49), follicular stimulating hormone 6 (reference, 2–20) IU/L and luteinizing hormone 6 (reference, 2–70) IU/L. Water deprivation test demonstrated sodium 148 (reference, 135–145) mEq/l, serum osmolality 310 (reference, 275–295) mmol/kg and urine osmolality 107 (reference,50–1450) mmol/kg improving to 142 mEq/L, 295mEq/L and 535 mEq/L respectively, after desmopressin. Gadolinium enhanced pituitary MRI demonstrated a markedly thickened stalk with uniform enhancement. Chest HRCT confirmed bilateral upper zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis (PLAM) or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as PLAM exacerbates with oestrogen therapy and pregnancy which she was able to successfully pursue without disease exacerbation. Conclusion: The patient’s initial presentation was considered as LH, but subsequent cystic changes on HRCT led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.


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