Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: Our interdisciplinary rheumatology-pneumology experience, and review of the literature [PDF]
Background: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the lungs, varying from idiopathic interstitial pneumonias to secondary variants, including the ILDs associated to ...
Cerri, Stefania +9 more
core +1 more source
ABSTRACT Cardiac rhabdomyomas are often the presenting sign of tuberous sclerosis complex (TSC). Prior reports have shown that maternal sirolimus treatment can reduce rhabdomyomas. We used maternal sirolimus to reverse hydrops fetalis due to a massive cardiac rhabdomyoma in a twin gestation.
David M. Ritter +6 more
wiley +1 more source
A woman with dyspnea and recurrent pneumothorax: when dyspnea is not asthma
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by cystic lung lesions, lymphatic abnormalities, and angiomyolipomas. It can take a significant amount of time to diagnose LAM due to the vague symptoms of fatigue, progressive dyspnea ...
Anusha Chidharla +3 more
doaj +1 more source
Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex. [PDF]
Background: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA).
Agranovich, Oleg +49 more
core +2 more sources
JAK Inhibitors for Treatment of SAPHO Syndrome: A Systematic Review of 72 Cases
Objective Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome usually involves bones, joints, and skin. Due to a lack of known pathogenesis and clinical trials, there is no standard treatment of SAPHO syndrome. Because none of the current treatments have a high efficacy and the risk of relapse is high, new treatment options for ...
Patrick Fazeli +9 more
wiley +1 more source
Malignant presentation of uterine lymphangioleiomyomatosis
Objective: The main aim of this case report was to present the method of diagnosis, management, and the 12-year-follow-up of a patient diagnosed with primary uterine lymphangioleiomyomatosis (LAM).
Dariusz Szpurek +5 more
doaj +1 more source
Review of the Tuberous Sclerosis Renal Guidelines from the 2012 Consensus Conference: Current Data and Future Study. [PDF]
Renal-related disease is the most common cause of tuberous sclerosis complex (TSC)-related death in adults, and renal angiomyolipomas can lead to complications that include chronic kidney disease (CKD) and hemorrhage.
Anderson, C +11 more
core +3 more sources
The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports [PDF]
J. Shah +5 more
openalex +1 more source
Bilateral Spontaneous Pneumothorax in a Young Gentleman
We present a case of bilateral spontaneous pneumothorax in a 26‐year‐old Chinese male with a history of smoking. Imaging reveals the presence of underlying cystic lung disease and he subsequently underwent bilateral video‐assisted thoracoscopic surgery (VATS) bullectomy, mechanical pleurodesis, and surgical lung biopsy.
Albert Teng +3 more
wiley +1 more source
Lymphangioleiomyomatosis (LAM) is a rare, low-grade malignant condition that typically affects women of childbearing age and primarily involves the lungs. While cases involving males and affecting the gastrointestinal tract are exceedingly uncommon. This
Ying Zi, Yuchen Shi, Rongjie Shi
doaj +1 more source

