0
July 1977, Vol 131, No. 7 >
Article | July 1977

Picture of the Month FREE

Sydney S. Gellis, MD; Murray Feingold, MD; J. P. Farriaux, MD; G. Fontaine, MD
[+] Author Affiliations

Reprint requests to Boston Floating Hospital, 20 Ash St, Boston, MA 02111 (Dr Gellis).


Am J Dis Child. 1977;131(7):807-808. doi:10.1001/archpedi.1977.02120200087020.
Text Size: A A A
Published online
Article
References
Comments

Denoument and Discussion 

Pseudohypoparathyroidism 

Manifestations  Manifestations are shortness of stature, obesity, rounded faces, shortening of one or more metacarpals and metatarsals, and mental retardation. Convulsions may occur, and calcifications are frequently present within the brain and in subcutaneous tissues. Not all end organs (kidney, bone, and intestine) are equally affected, and, therefore, there are several clinical forms of pseudohypoparathyroidism.Biochemical findings of diagnostic importance are hypocalcemia and hyperphosphatemia that, unlike idiopathic hypoparathyroidism, remain uninfluenced by exogenous parathormone injection (Ellsworth-Howard test). The urinary excretion of cyclic adenosine monophosphate (AMP) is also not increased by parathormone injections. Serum parathormone levels are increased because of lack of the feedback regulatory influence by cyclic AMP.It appears that pseudohypoparathyroidism and psuedo-psuedohypoparathyroidism are closely related. The latter is clinically similar to pseudohypoparathyroidism, but biochemical findings are different. Occurrences of normocalcemic and hypocalcemic patients within one family help support the hypothesis that both entities are similar.

REFERENCES

1 +
Costello JM, Dent CE:  Hypohyperparathyroidism . Arch Dis Child 38:397, 1963;.
Link to Article
2 +
Chase LR, Melson GL, Aurback GD:  Pseudohypoparathyroidism: Defective excretion of 3′,5′-AMP in response to parathyroid hormone . J Clin Invest 48:1832, 1969;.
Link to Article
3 +
Drezner MK, Neelon FA, Haussler M, et al:  1,25-dihydroxycholecalciferol deficiency: The probable cause of hypocalcemia and metabolic bone disease in pseudohypoparathyroidism . J Clin Endocrinol Metab 42:621, 1976;.
Link to Article
4 +
Farriaux JP, Vainsel M, Six R, et al:  Le pseudo-hypoparathyroidisme: Resultats complementaires a propos de 4 cas familiaux . Arch Fr Pediatr 33:445, 1976;.
5 +
Frame B, Hanson CA, Brost HM, et al:  Renal resistance to parathyroid hormone with osteitis fibrosa: "Pseudohypohyperparathyroidism." Am J Med 52:311, 1972;.
Link to Article
6 +
Parkin JM:  Spontaneous remission in a patient with pseudohypoparathyroidism . Arch Dis Child 49:821, 1974;.
Link to Article

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Costello JM, Dent CE:  Hypohyperparathyroidism . Arch Dis Child 38:397, 1963;.
Link to Article
2 +
Chase LR, Melson GL, Aurback GD:  Pseudohypoparathyroidism: Defective excretion of 3′,5′-AMP in response to parathyroid hormone . J Clin Invest 48:1832, 1969;.
Link to Article
3 +
Drezner MK, Neelon FA, Haussler M, et al:  1,25-dihydroxycholecalciferol deficiency: The probable cause of hypocalcemia and metabolic bone disease in pseudohypoparathyroidism . J Clin Endocrinol Metab 42:621, 1976;.
Link to Article
4 +
Farriaux JP, Vainsel M, Six R, et al:  Le pseudo-hypoparathyroidisme: Resultats complementaires a propos de 4 cas familiaux . Arch Fr Pediatr 33:445, 1976;.
5 +
Frame B, Hanson CA, Brost HM, et al:  Renal resistance to parathyroid hormone with osteitis fibrosa: "Pseudohypohyperparathyroidism." Am J Med 52:311, 1972;.
Link to Article
6 +
Parkin JM:  Spontaneous remission in a patient with pseudohypoparathyroidism . Arch Dis Child 49:821, 1974;.
Link to Article

Correspondence

Submit a Letter
CME
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Clear Answers | Save For Later
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2013 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems