Morphological pituitary abnormalities in patients with congenital Growth Hormone deficiency

Faten Haj Kacem Akid; Abdel Mouhaymen Missaoui; Wafa Belabed; Wajdi Safi; Nadia Charfi; Fatma Mnif; Dhoha Ben Salah; Nabila Mejdoub; Mouna Mnif; Mohamed Abid

doi:10.1530/endoabs.90.EP736

Background and aim: Disorders of the development of the pituitary gland could result in some forms of Congenital Growth hormone deficiency (CGHD). This study aims to assess the morphological abnormalities of the pituitary gland in patients with CGHD.

Patients and Methods: We conducted a retrospective study (1991–2019) at the Endocrinology department of Hedi Chaker University Hospital, Sfax, Tunisia. We involved 87 patients diagnosed with CGHD, whose clinical, biochemical, and imaging peculiarities were collected from medical charts.

Results: The mean age was 14 years, with a male predominance (59%). Seventy-five percent of patients were referred for short stature. We noted a delayed diagnosis in 65% of cases. The median height was 131 cm. Severe growth delay was noticed in 38.6% of cases. The average delay of bone age was 48 months. Eighty percent of CGHD patients combined hypopituitarism. A positive correlation was established between the precocious onset of the pituitary dysfunction and the presence of pituitary hypoplasia (P=0.009), as well as with the severity of growth retardation (P=0.016) and the number of affected axes (P=0.02). The latter correlation was significant when three or more pituitary axes were involved, which also remained significant for pituitary stalk anomalies. Posterior pituitary ectopy was inversely correlated with the age of discovery of hypopituitarism (P=0.001) but not with neonatal distress (P=0.28).

Table 1: Predictive clinical factors of pituitary abnormalities in patients with CGHD
Studied factors	Normal pituitary	Pituitary Hypoplasia	P-value
Age at diagnosis (years)	14	11	0.009
Severe statural retardation	61%	84%	0.016
Number of hypopituitarisms	2.3	3.1	0.02
Severity of GHD	46%	54%	0.16

Table 2: Morphological pituitary abnormalities and the number of hormonal dysfunctions in patients with CGHD
Hormonal dysfunctions	Normal pituitary	Pituitary hypoplasia	Pituitary stalk abnormality
Isolated CGHD	64%	35%	11%
2 axes	68%	31%	18%
3 axes	50%	50%	27%
4 axes	33%	66%	60%
5 axes	11%	88%	66%
P-value	0.037	0.037	0.001

Discussion: The hypoplastic pituitary is the most common morphological abnormality in patients with CGHD since GH-secreting cells represent the largest cell population in the pituitary gland. In our cohort, 49.4% of CGHD cases had pituitary hypoplasia. Similar findings were reported in literature where hypoplasia was noted in 59% to 61% of patients. Pituitary stalk abnormalities expose the affected patient to a 3.28-fold increased risk of progression to combined forms of hypopituitarism.

Endocrine Abstracts

EP736