Journal of Shandong University (Health Sciences) ›› 2021, Vol. 59 ›› Issue (2): 60-65.doi: 10.6040/j.issn.1671-7554.0.2020.1705

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Clinical characteristics of 9 cases of pituitary stalk interruption syndrome in infants with intractable hypoglycemia as the primary manifestation

MA Xue1, WANG Fengxue2, ZHANG Shule1, LI Guimei1,2   

  1. 1. Department of Pediatrics, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong, China;
    2. Department of Pediatrics, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong, China
  • Published:2021-03-05

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Abstract: Objective To investigate the clinical characteristics of pituitary stalk interruption syndrome(PSIS)in neonates and infants with intractable hypoglycemia as the primary manifestation, so as to improve the diagnosis and treatment level. Methods The clinical data of 9 PSIS patients(21 d-11 months, median age 60 d)were retrospectively analyzed. Neonatal hyperinsulinemic hypoglycemia, febrile convulsion, history of encephalitis, stroke, and history of other neurological diseases and trauma were excluded. The clinical features, pituitary hormone levels, pituitary MRI features and difference of blood glucose levels before and after hormone replacement therapy were recorded. Results There were 4 boy and 5 girl babies, with recurrent hypoglycemia as the primary manifestation. All 9 cases began to show intractable hypoglycemia, growth retardation and poor response to external stimulation in the neonatal period. Neonatal pathological jaundice was observed in 7 cases, recurrent convulsion in 4 cases, polydipsia in 2 cases, elevated glutamous transaminase in 7 cases, and small penis and cryptorchidism in all 4 boy babies. Endocrine examinations showed growth hormone deficiency, thyroid hormone deficiency, adrenocorticotropic hormone or cortisol deficiency. Pituitary MRI indicated PSIS in all 9 cases, including pituitary anterior dysplasia in 6 cases, pituitary anterior disappearance in 3 cases, pituitary posterior disappearance in 2 cases, and ectopic posterior pituitary in 7 cases. All patients were treated with hydrocortisone, levothyroxine and recombinant human growth hormone. Desmopressin was given to 2 cases. Symptoms such as persistent hypoglycemia and convulsion gradually disappeared, and growth rate was improved. Conclusion Neonatal or infant PSIS is characterized by intractable hypoglycemia, which can be improved with hydrocortisone, levothyroxine and recombinant human growth hormone. PSIS can lead to multiple pituitary hormone deficiency in newborns or infants. Pituitary MRI is of great value in the diagnosis of PSIS. With pituitary target gland hormones, early diagnosis and early treatment can be achieved to improve the prognosis.

Key words: Pituitary stalk interruption syndrome, Infants, Hypoglycemia, Hormone replacement

CLC Number: 

  • R725.8
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