PMID- 2200621
OWN - NLM
STAT- MEDLINE
DCOM- 19900920
LR  - 20190813
IS  - 0300-0664 (Print)
IS  - 0300-0664 (Linking)
VI  - 32
IP  - 6
DP  - 1990 Jun
TI  - Familial acromegaly: studies in three families.
PG  - 719-28
AB  - Acromegaly is usually regarded as a disease which arises sporadically except on 
      those rare occasions in which it occurs as one component of an inherited 
      pluriglandular syndrome. Familial acromegaly occurring in isolation has been 
      reported only rarely. We have studied three families in each of which two 
      first-degree relatives have developed acromegaly without clinical evidence of 
      other endocrinopathies, in order to clarify the relationship of familial 
      acromegaly to multiple endocrine neoplasia, type 1 (MEN 1). No further 
      subclinical cases of acromegaly have been detected in those first-degree 
      relatives of the index cases who were screened. In two of the families, we have 
      found no evidence of abnormal pancreatic or parathyroid function either in the 
      acromegalic index cases or their asymptomatic relatives: a diagnosis of MEN 1 is 
      therefore effectively excluded. In the third family, the presence of multiple 
      subcutaneous lipomata in several members of the family and elevation of PTH 
      levels in two (normocalcaemic) acromegalic index cases, might indicate a highly 
      atypical presentation of MEN 1. None of the six acromegalic index cases had 
      elevations of circulating growth hormone releasing hormone levels. We conclude 
      that isolated familial acromegaly may be more common than has hitherto been 
      realized and that, in these families at least, ectopic production of growth 
      hormone releasing hormone is not responsible.
FAU - McCarthy, M I
AU  - McCarthy MI
AD  - London Hospital Medical College, Department of Chemical Pathology, UK.
FAU - Noonan, K
AU  - Noonan K
FAU - Wass, J A
AU  - Wass JA
FAU - Monson, J P
AU  - Monson JP
LA  - eng
PT  - Journal Article
PL  - England
TA  - Clin Endocrinol (Oxf)
JT  - Clinical endocrinology
JID - 0346653
RN  - 67763-96-6 (Insulin-Like Growth Factor I)
RN  - 9002-62-4 (Prolactin)
RN  - 9002-72-6 (Growth Hormone)
SB  - IM
MH  - Acromegaly/*genetics/physiopathology
MH  - Adult
MH  - Aged
MH  - Female
MH  - Glucose Tolerance Test
MH  - Growth Hormone/blood
MH  - Humans
MH  - Insulin-Like Growth Factor I/analysis
MH  - Islets of Langerhans/metabolism/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Parathyroid Glands/physiopathology
MH  - Pedigree
MH  - Prolactin/blood
EDAT- 1990/06/01 00:00
MHDA- 1990/06/01 00:01
CRDT- 1990/06/01 00:00
PHST- 1990/06/01 00:00 [pubmed]
PHST- 1990/06/01 00:01 [medline]
PHST- 1990/06/01 00:00 [entrez]
AID - 10.1111/j.1365-2265.1990.tb00918.x [doi]
PST - ppublish
SO  - Clin Endocrinol (Oxf). 1990 Jun;32(6):719-28. doi: 
      10.1111/j.1365-2265.1990.tb00918.x.