48 year old patient admitted to hospital after ambulatory detection of a serum creatinine of 7.56mg/dl.
Examination revealed: blood pressure 163/111mmHg, weight 105kg, height 190cm, body mass index 29kg/m2.
Cognitive impairment.
Corpulent body shape
Facies with prognathism and macroglossia.
Thick lips, prominent upper arches, severe voice
Normal fixation.
Normal eye fund.
The patient reported no changes in body morphology.
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Ultrasonography showed kidneys of dimensions at the lower limit of normality, with bilateral renal parenchymal thickness markedly thinned, practically empty.
Blood tests at admission showed biochemical parameters characteristic of uremia with negative viral serology and normal immunological tests.
Hormonal determinations were also performed:
- Cortisol: 23mcg/dl; TSH: 1.01mcIU/ml (0.27-4.2).
- Renin: 29.7microIU/ml (2.8-39.9).
- Aldosterone: 152,8pg/ml (10-160).
- FSH: 12.4mIU/ml (1.5-12.4), LH: 8.4mIU/ml
Prostate cancer: 553m/ml (86-324), total testosterone: 1.99ng/ml (2.5-8.4).
- GH: 4.24ng/ml (0-1).
- Somatomedin C (IGF1): 670ng/ml (100-358).
- IGF1-BP3: 7.59micro/ml (3.3-6.7).
- ACTH: 39pg/ml (8-46).
Hypertension was controlled with medical treatment.
Chronic hemodialysis was initiated due to deterioration of renal function, whose etiology could not be established.
A first glucose tolerance test was performed.
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At this point, clinicians were aware of the phenotype and hormonal profile (increased GH, IGF1-BP3; IGF-1 and without clear suppression of GH), as well as of the clinical features.
Pituitary magnetic resonance imaging (MRI) could not be performed due to the patient's and family refusal.
Four months later, MRI without paramagnetic contrast was finally performed.
The pituitary gland had a size within normal limits, with a centered pituitary stalk.
A new TS was performed showing a normal basal GH, with paradoxical increase at 30 minutes, but adequate suppression at 2 hours.
At that time, IGF-1 (340 ng/ml) and IGF1-BP3 (6.3 μg/ml) levels had also normalized.
Prolactin was still high and testosterone was normal.
The patient is currently under regular endocrine follow-up and is on hemodialysis.
