High urinary excretion of N-(pyrrole-2-carboxyl) glycine in type II hyperprolinemia

2008 ◽  
Vol 37 (6) ◽  
pp. 485-489 ◽  
Author(s):  
Moacir Wajner ◽  
Clóvis M. D. Wannmacher ◽  
Paul Purkiss
2008 ◽  
Vol 22 (S1) ◽  
Author(s):  
Souad Belmadani ◽  
S Park ◽  
B J Bivona ◽  
D Seth ◽  
H Kobori ◽  
...  

2011 ◽  
Vol 2 (4) ◽  
pp. 337 ◽  
Author(s):  
Kunal Sah ◽  
Alka Kale ◽  
Ajit Kadam ◽  
Hetul Shah ◽  
Sunira Chandra ◽  
...  

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Minghao Li ◽  
Christina Pamporaki ◽  
Stephanie M. J. Fliedner ◽  
Henri J. L. M. Timmers ◽  
Svenja Nölting ◽  
...  

Abstract Background The presence or future development of metastatic pheochromocytomas or paragangliomas (mPPGLs) can be difficult to diagnose or predict at initial presentation. Since production of catecholamines from mPPGLs is different from non-metastatic tumors (non-mPPGLs), this study aimed to clarify whether presenting catecholamine-related signs and symptoms (cSS) might also differ. Methods The study included 249 patients, 43 with mPPGL and 206 with non-mPPGL. Clinical data at the time of biochemical diagnosis (i.e. at entry into the study) were used to generate a cumulative score of cSS for each patient. Results Patients with mPPGL were significantly younger (43.3 ± 14 vs. 48.9 ± 16.1 years) and included a lower proportion of females (39.5% vs. 60.7%) than patients with non-mPPGLs. Frequencies of signs and symptoms did not differ between the two groups. Patients with mPPGLs had lower (P < 0.001) urinary excretion of epinephrine (3.5 (IQR, 1.9—6.5) µg/day) than those with non-mPPGLs (19.1 (IQR, 4.3—70.2) µg/day). There was no difference in urinary excretion of norepinephrine. In patients with mPPGLs a high cSS score was associated with high urinary excretion of norepinephrine and normetanephrine. In contrast, in patients with non-mPPGLs, a high cSS was associated with high urinary excretion of epinephrine and metanephrine. Conclusion Although presenting signs and symptoms were associated with production of norepinephrine in patients with mPPGLs and of epinephrine in patients with non-mPPGLs, there were no differences in signs and symptoms between the two groups. Therefore, consideration of signs and symptoms does not appear helpful for distinguishing patients with and without mPPGLs.


Author(s):  
S. Similä

Hydroxyproline metabolism was studied in two patients with type II hyperprolinaemia (HP II) using oral loadings of hydroxyproline or hydroxyproline-ornithine. Δ1-pyrroline-3-hydroxy-5-carboxylic acid (3 OH-PC) and Δ1-pyrroline-5-carboxylic acid (PC) were identified in the urine. The urinary excretion of both 3-OH-PC and PC increased in HP II patients but not in healthy controls during oral loading of hydroxyproline and hydroxyproline-ornithine. The plasma level of proline in patients with HP II is very high but the hydroxyproline concentration is normal or only slightly increased. Therefore one can assume that hydroxyproline is converted to pyrrole-2-carboxylic acid, which is excreted in urine as a glycine conjugate. In this study it was demonstrated that the highly elevated plasma level of proline in one of the patients with HP II decreased greatly after hydroxyproline-ornithine load; this change was followed by a 40-fold rise in urinary excretion of proline.


1987 ◽  
Vol 170 (2-3) ◽  
pp. 297-304 ◽  
Author(s):  
Takeyori Saheki ◽  
Keiko Kobayashi ◽  
Ituro Inoue ◽  
Seiji Matuo ◽  
Sumio Hagihara ◽  
...  
Keyword(s):  

Metabolism ◽  
1989 ◽  
Vol 38 (5) ◽  
pp. 419-420 ◽  
Author(s):  
G. Gambaro ◽  
E. Cicerello ◽  
S. Mastrosimone ◽  
T. Lavagnini ◽  
B. Baggio

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