[Doege-Potter syndrome. about one new case]
1Service de pneumologie, Chu Ibn Sina, Rabat, Maroc. herraklaila@yahoo.fr
Revue De Pneumologie Clinique
|December 31, 2011
Summary
Doege-Potter syndrome, a rare paraneoplastic condition, causes hypoglycemia due to tumors producing insulin growth factor-II (IGF-II). Solitary fibrous tumors of the pleura (TFSP) are the most common cause, with surgery offering a cure.
Area of Science:
- Oncology
- Endocrinology
- Pulmonology
Background:
- Doege-Potter syndrome is a rare paraneoplastic syndrome characterized by hypoglycemia.
- It is typically caused by tumors producing insulin growth factor-II (IGF-II).
- Solitary fibrous tumors of the pleura (TFSP) are the most frequent etiology.
Observation:
- TFSP can be asymptomatic and discovered incidentally.
- Symptoms may include non-specific respiratory issues or hypoglycemia.
- Hypoglycemia is associated with large tumor volume.
Findings:
- Surgical resection of the TFSP leads to the resolution of hypoglycemia.
- This case highlights TFSP as a potential cause of hypoglycemia in patients with pleural tumors.
- The syndrome is linked to tumor-produced IGF-II.
Implications:
- Recognizing TFSP as a cause of hypoglycemia is crucial for accurate diagnosis.
- Early detection and surgical intervention can lead to a definitive cure.
- This underscores the importance of considering paraneoplastic syndromes in unexplained hypoglycemia.
Related Concept Videos
Pleiotropy
Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
Sex-linked Disorders
Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.
Sex Linked Disorders
Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.
Genetic Lingo
Overview
Huntington Disease l: Introduction
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
Cushing Syndrome II: Pathophysiology
Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...


