ADRENOCORTICAL FUNCTION AND SKIN PIGMENTATION IN IDIOPATHIC HEMOCHROMATOSIS

1973 ◽  
Vol 2 (1) ◽  
pp. 19-20
Author(s):  
R. Sinniah
PEDIATRICS ◽  
1952 ◽  
Vol 10 (3) ◽  
pp. 286-292
Author(s):  
HANS W. KUNZ

A case of Fanconi syndrome at an unusually young age presenting anemia, leukopenia, thrombocytopenia, microcephaly, hypogenitalism, skin pigmentation, and anomalies of thumbs, kidneys and heart is described. The patient's younger brother with multiple anomalies of the extremities, hydrocephalus, abnormal ears and genitalia died at the age of 17 months without pigmentation and hematologic symptoms, changes which usually do not occur before the age of 5 years. The literature is reviewed and a follow-up on previously reported cases is given. It seems that in addition to a hypoplasia of the bone marrow an extracorpuscular hemolytic mechanism plays a role in the pathogenesis of the anemia. It is suggested that some manifestations may be due to impaired adrenocortical function.


Author(s):  
Raul I. Garcia ◽  
Evelyn A. Flynn ◽  
George Szabo

Skin pigmentation in mammals involves the interaction of epidermal melanocytes and keratinocytes in the structural and functional unit known as the Epidermal Melanin Unit. Melanocytes(M) synthesize melanin within specialized membrane-bound organelles, the melanosome or pigment granule. These are subsequently transferred by way of M dendrites to keratinocytes(K) by a mechanism still to be clearly defined. Three different, though not necessarily mutually exclusive, mechanisms of melanosome transfer have been proposed: cytophagocytosis by K of M dendrite tips containing melanosomes, direct injection of melanosomes into the K cytoplasm through a cell-to-cell pore or communicating channel formed by localized fusion of M and K cell membranes, release of melanosomes into the extracellular space(ECS) by exocytosis followed by K uptake using conventional phagocytosis. Variability in methods of transfer has been noted both in vivo and in vitro and there is evidence in support of each transfer mechanism. We Have previously studied M-K interactions in vitro using time-lapse cinemicrography and in vivo at the ultrastructural level using lanthanum tracer and freeze-fracture.


JAMA ◽  
1965 ◽  
Vol 194 (6) ◽  
pp. 670-672 ◽  
Author(s):  
A. S. Zelickson
Keyword(s):  

1999 ◽  
Vol Volume 17 (Number 4) ◽  
pp. 0327-0338 ◽  
Author(s):  
Andrew J. Harper ◽  
John E. Buster ◽  
Peter R. Casson

1962 ◽  
Vol 40 (2) ◽  
pp. 254-262 ◽  
Author(s):  
H. H. Bassøe ◽  
R. Emberland ◽  
E. Glück ◽  
K. F. Støa

ABSTRACT The steroid excretion and the plasma corticosteroids were investigated in three patients with necrosis of the brain and of the pituitary gland. The patients were kept alive by artificial ventilation. In two of the patients the neutral 17-ketosteroids and the 17-hydrocorticosteroids fell to extremely low levels. At the same time, the number of eosinophil cells showed a tendency to increase. Corticotrophin administered intravenously twice to the third patient had a stimulating effect on the adrenal cortex. The theoretical and practical significance of these findings is discussed.


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