Doege-Potter Syndrome

N Ahluwalia1, R Attia1, A Green1

  • 1Guy's and St Thomas' NHS Foundation Trust , UK.

Insights

Doege-Potter syndrome, a rare condition causing low blood sugar, is linked to solitary fibrous tumors secreting insulin-like growth factor II. Surgical removal of the tumor effectively cures this paraneoplastic syndrome.

Area of Science:

  • Endocrinology
  • Oncology
  • Pathology

Background:

  • Doege-Potter syndrome is a rare paraneoplastic syndrome characterized by hypoinsulinaemic hypoglycemia.
  • It results from ectopic secretion of insulin-like growth factor II (IGF-II) prohormone, typically from a solitary fibrous tumor.

Observation:

  • A 76-year-old inpatient presented with severe hypoglycemia.
  • The patient was admitted for resection of a recurrent left-sided pleural tumor.

Findings:

  • Investigations confirmed true hypoglycemia and diagnosed Doege-Potter syndrome.
  • The underlying cause was identified as a solitary fibrous tumor secreting IGF-II prohormone.

Implications:

  • Complete surgical resection of the tumor led to a full recovery.
  • Early diagnosis and treatment are crucial to prevent hypoxic cerebral injury or death.

Related Concept Videos

Pleiotropy01:33

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,...
44.1K
Meiosis I01:49

Meiosis I

Meiosis is a carefully orchestrated set of cell divisions, the goal of which—in humans—is to produce haploid sperm or eggs, each containing half the number of chromosomes present in somatic cells elsewhere in the body. Meiosis I is the first such division, and involves several key steps, among them: condensation of replicated chromosomes in diploid cells; the pairing of homologous chromosomes and their exchange of information; and finally, the separation of homologous chromosomes by...
221.9K
Genetic Lingo01:11

Genetic Lingo

Overview
118.1K
Personality Disorders: Schizotypal and Histrionic01:20

Personality Disorders: Schizotypal and Histrionic

Schizotypal personality disorder and histrionic personality disorder are two distinct psychological conditions classified under personality disorders, each characterized by unique behavioral patterns and social difficulties. Both disorders significantly affect interpersonal relationships and emotional well-being, leading to social isolation and frustration.
Schizotypal Personality Disorder: Eccentric Behavior and Social Withdrawal
Schizotypal personality disorder is marked by odd or eccentric...
945
Nondisjunction01:21

Nondisjunction

Nondisjunction is the failure of homologous chromosomes or sister chromatids to separate correctly and move to the opposite poles of the cells. This produces daughter cells with abnormal chromosome numbers.  Nondisjunction is common during anaphase I or anaphase II of meiosis.  Mutations in synaptonemal complex proteins that attach homologous chromosomes increase the chances of nondisjunction in anaphase I of meiosis I. In contrast, mutations in topoisomerases and condensins that hold...
5.8K
Nondisjunction01:29

Nondisjunction

During meiosis, chromosomes occasionally separate improperly. This occurs due to failure of homologous chromosome separation during meiosis I or failed sister chromatid separation during meiosis II. In some species, notably plants, nondisjunction can result in an organism with an entire additional set of chromosomes, which is called polyploidy. In humans, nondisjunction can occur during male or female gametogenesis and the resulting gametes possess one too many or one too few chromosomes.
83.5K