Type A-insulin Resistance with Lipopexia on Extremities: A Case Report

Naoki Nakashima, Tomoya Miyamura, Tsukasa Yamashita, Teruaki Yamauchi, Fumio Umeda, Yasusada Kawada, Masanori Noda, Hajime Nawata

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

We present the unusual case of a 17-year-old female with insulin-resistant diabetes, acanthosis nigricans, hirsutism, amenorrhea, dental dysplasia and lipopexia on the extremities. She had been diagnosed as having border line diabetes with hyperinsulinemia at age 12 when she was not obese and diabetes mellitus at age 13. On admission, she was obese and had lipopexia only on the extremities. The presence of hyperinsulinemia and poor response to exogenous insulin suggested severe insulin resistance. Insulin binding to transformed B-lymphoblasts derived from her was extremely low compared to the normal control, showing decreased receptor affinity. Her parents and sister exhibited hypersecretion of insulin in response to a 75 g oral glucose tolerance test. Her mother was diabetic, and her father and sister had border line diabetes, whereas her brother had a normal response. These findings support strongly the diagnosis of a type A syndrome with severe insulin resistance associated with lipopexia on the extremities. A genetic defect in the insulin receptor gene may be responsible.

Original languageEnglish
Pages (from-to)347-353
Number of pages7
JournalEndocrinologia Japonica
Volume39
Issue number4
DOIs
Publication statusPublished - Jan 1 1992

Fingerprint

Insulin
Insulin Resistance
Extremities
Siblings
Medical problems
Hyperinsulinism
Acanthosis Nigricans
Hirsutism
Amenorrhea
Glucose Tolerance Test
Fathers
Diabetes Mellitus
Tooth
Parents
Mothers
Glucose
Genes
Defects

All Science Journal Classification (ASJC) codes

  • Endocrinology
  • Engineering(all)

Cite this

Nakashima, N., Miyamura, T., Yamashita, T., Yamauchi, T., Umeda, F., Kawada, Y., ... Nawata, H. (1992). Type A-insulin Resistance with Lipopexia on Extremities: A Case Report. Endocrinologia Japonica, 39(4), 347-353. https://doi.org/10.1507/endocrj1954.39.347

Type A-insulin Resistance with Lipopexia on Extremities : A Case Report. / Nakashima, Naoki; Miyamura, Tomoya; Yamashita, Tsukasa; Yamauchi, Teruaki; Umeda, Fumio; Kawada, Yasusada; Noda, Masanori; Nawata, Hajime.

In: Endocrinologia Japonica, Vol. 39, No. 4, 01.01.1992, p. 347-353.

Research output: Contribution to journalArticle

Nakashima, N, Miyamura, T, Yamashita, T, Yamauchi, T, Umeda, F, Kawada, Y, Noda, M & Nawata, H 1992, 'Type A-insulin Resistance with Lipopexia on Extremities: A Case Report', Endocrinologia Japonica, vol. 39, no. 4, pp. 347-353. https://doi.org/10.1507/endocrj1954.39.347
Nakashima, Naoki ; Miyamura, Tomoya ; Yamashita, Tsukasa ; Yamauchi, Teruaki ; Umeda, Fumio ; Kawada, Yasusada ; Noda, Masanori ; Nawata, Hajime. / Type A-insulin Resistance with Lipopexia on Extremities : A Case Report. In: Endocrinologia Japonica. 1992 ; Vol. 39, No. 4. pp. 347-353.
@article{fd873e84c6bb40f89e6a4df1a174cc5e,
title = "Type A-insulin Resistance with Lipopexia on Extremities: A Case Report",
abstract = "We present the unusual case of a 17-year-old female with insulin-resistant diabetes, acanthosis nigricans, hirsutism, amenorrhea, dental dysplasia and lipopexia on the extremities. She had been diagnosed as having border line diabetes with hyperinsulinemia at age 12 when she was not obese and diabetes mellitus at age 13. On admission, she was obese and had lipopexia only on the extremities. The presence of hyperinsulinemia and poor response to exogenous insulin suggested severe insulin resistance. Insulin binding to transformed B-lymphoblasts derived from her was extremely low compared to the normal control, showing decreased receptor affinity. Her parents and sister exhibited hypersecretion of insulin in response to a 75 g oral glucose tolerance test. Her mother was diabetic, and her father and sister had border line diabetes, whereas her brother had a normal response. These findings support strongly the diagnosis of a type A syndrome with severe insulin resistance associated with lipopexia on the extremities. A genetic defect in the insulin receptor gene may be responsible.",
author = "Naoki Nakashima and Tomoya Miyamura and Tsukasa Yamashita and Teruaki Yamauchi and Fumio Umeda and Yasusada Kawada and Masanori Noda and Hajime Nawata",
year = "1992",
month = "1",
day = "1",
doi = "10.1507/endocrj1954.39.347",
language = "English",
volume = "39",
pages = "347--353",
journal = "Endocrine Journal",
issn = "0918-8959",
publisher = "Japan Endocrine Society",
number = "4",

}

TY - JOUR

T1 - Type A-insulin Resistance with Lipopexia on Extremities

T2 - A Case Report

AU - Nakashima, Naoki

AU - Miyamura, Tomoya

AU - Yamashita, Tsukasa

AU - Yamauchi, Teruaki

AU - Umeda, Fumio

AU - Kawada, Yasusada

AU - Noda, Masanori

AU - Nawata, Hajime

PY - 1992/1/1

Y1 - 1992/1/1

N2 - We present the unusual case of a 17-year-old female with insulin-resistant diabetes, acanthosis nigricans, hirsutism, amenorrhea, dental dysplasia and lipopexia on the extremities. She had been diagnosed as having border line diabetes with hyperinsulinemia at age 12 when she was not obese and diabetes mellitus at age 13. On admission, she was obese and had lipopexia only on the extremities. The presence of hyperinsulinemia and poor response to exogenous insulin suggested severe insulin resistance. Insulin binding to transformed B-lymphoblasts derived from her was extremely low compared to the normal control, showing decreased receptor affinity. Her parents and sister exhibited hypersecretion of insulin in response to a 75 g oral glucose tolerance test. Her mother was diabetic, and her father and sister had border line diabetes, whereas her brother had a normal response. These findings support strongly the diagnosis of a type A syndrome with severe insulin resistance associated with lipopexia on the extremities. A genetic defect in the insulin receptor gene may be responsible.

AB - We present the unusual case of a 17-year-old female with insulin-resistant diabetes, acanthosis nigricans, hirsutism, amenorrhea, dental dysplasia and lipopexia on the extremities. She had been diagnosed as having border line diabetes with hyperinsulinemia at age 12 when she was not obese and diabetes mellitus at age 13. On admission, she was obese and had lipopexia only on the extremities. The presence of hyperinsulinemia and poor response to exogenous insulin suggested severe insulin resistance. Insulin binding to transformed B-lymphoblasts derived from her was extremely low compared to the normal control, showing decreased receptor affinity. Her parents and sister exhibited hypersecretion of insulin in response to a 75 g oral glucose tolerance test. Her mother was diabetic, and her father and sister had border line diabetes, whereas her brother had a normal response. These findings support strongly the diagnosis of a type A syndrome with severe insulin resistance associated with lipopexia on the extremities. A genetic defect in the insulin receptor gene may be responsible.

UR - http://www.scopus.com/inward/record.url?scp=0026774783&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026774783&partnerID=8YFLogxK

U2 - 10.1507/endocrj1954.39.347

DO - 10.1507/endocrj1954.39.347

M3 - Article

C2 - 1446650

AN - SCOPUS:0026774783

VL - 39

SP - 347

EP - 353

JO - Endocrine Journal

JF - Endocrine Journal

SN - 0918-8959

IS - 4

ER -