|LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 6 | Page : 884-885
Sheehan's syndrome in two generations
Bashir Ahmad Laway1, Mahroosa Ramzan1, Shahnaz Ahmad Mir2
1 Department of Endocrinology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
2 Department of Internal Medicine, Government Medical College, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||24-Oct-2016|
Bashir Ahmad Laway
Department of Endocrinology Sher-i-Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Laway BA, Ramzan M, Mir SA. Sheehan's syndrome in two generations. Indian J Endocr Metab 2016;20:884-5
In developing countries, Sheehan's syndrome (SS) continues to be a common cause of hypopituitarism in women of child-bearing age. SS in two generations has not been reported; we present the clinical course of a mother and daughter, both presenting with SS.
A 55-year-old female, para 4, delivered her last child at home apparently not associated with postpartum hemorrhage (PPH). She had lactation failure and did not menstruate after the last childbirth. Examination at the time of presentation revealed anemia, loss of axillary and pubic hair, breast atrophy, blood pressure of 130/80 mmHg, and delayed relaxation of deep tendon jerks. Hormonal evaluation revealed evidence of lactotroph, corticotroph, somatotroph, and gonadotroph failure (T4 of 2 µg/dl, thyroid-stimulating hormone [TSH] of 1.8 U/L, luteinizing hormone [LH] of 0.55 U/L, follicle-stimulating hormone [FSH] of 1.23 U/L, peak cortisol of <1 µg/dl, peak prolactin of 1.15 ng/ml, and peak GH of 0.1 ng/ml after insulin tolerance test). Magnetic resonance imaging (MRI) of pituitary revealed the evidence of empty sella. With the diagnosis of SS, the patient was given glucocorticoid and thyroxine replacement therapy in appropriate doses and is on follow-up. Six years later in April 2014, her 35-year-old daughter presented to our outpatient services with features of hypopituitarism. History revealed lactation failure and amenorrhea following 4th childbirth though there was no history of PPH. Hormonal evaluation revealed the evidence of thyrotroph, lactotroph, corticotroph, gonadotroph, and somatotroph failure (T4 of 2.30 µg/dl, TSH of 5.7 U/L, LH of 1.83 U/L, FSH of 4.6 U/L, peak cortisol of 2 µg/dl, peak prolactin of 1.7 ng/ml, and peak GH of 0.07 ng/ml after insulin tolerance test). MRI pituitary revealed partial empty sella.
Hypopituitarism occurs in nearly one-third of the patients with severe postpartum hemorrhage, and the extent of pituitary gland involvement is variable. Furthermore, history of PPH may not be available in home-conducted deliveries.,
The presence of SS in mother and daughter is quite interesting as it has not been reported previously. Whether such an association is incidental or has some familial or genetic basis is not clear at present. In adult population with sporadic idiopathic pituitary insufficiency, genetic cause for hypopituitarism is supposed to be rare. Nyström et al. studied 25 patients of idiopathic pituitary insufficiency for genotyping of all coding exons of HESX1, LH X4, PROP 1, POU1 F1, and GH 1 genes. The rate of mutation was higher (50%) in familial cases compared to low frequency of 8% in nonfamilial cases. Based on these observations, it can be concluded that familial cases of adult idiopathic hypopituitarism can be considered for genetic screening, it is not required in adults with nonfamilial idiopathic adult hypopituitarism.
Kajita et al. studied antipituitary antibodies in twenty patients with hypopituitarism and their family members (including two patients of SS). Two types of antibodies such as pituitary cell and pituitary cell surface antibodies were measured with indirect immunofluorescence. Among two patients of SS, one had antibody positive for pituitary cytoplasmic antigen 13 years later and against pituitary cell cytoplasmic antigen 15 years after obstetric insult. Antibody against pituitary cytoplasmic antigen was also positive in her brother and one of two sons who demonstrated subnormal GH response to arginine. The significance of antipituitary antibodies in women with SS is being debated. Although not previously reported, SS in two generations may point to some genetic factor associated with the syndrome, and further studies need to elaborate the possibility.
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Conflicts of interest
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