Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies

Lovinger, Robert D.; Kaplan, Selna L.; Grumbach, Melvin M.

doi:10.1016/s0022-3476(75)80132-6

Cited by 135 publications

(37 citation statements)

References 43 publications

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“…As for the response of TSH to TRH, it has been generally thought that reduced or absent responses indicate a primary pituitary disorder ) and normal Woolf and Schalch, 1973;Lovinger et al, 1975) or delayed, prolonged and/or exaggerated (Costum et al, 1971;Faglia et al, 1973Strauss et al, 1974 responses indicate hypothalamic disorder. In our patient, basal plasma TSH levels were normal and show ed normal but delayed responses to TRH.…”

Section: Discussionmentioning

confidence: 99%

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A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E.

et al. 1979

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Section: Discussionmentioning

confidence: 99%

“…Low or no response of LH to a single dose of LH-RH does not necessarily indicate primary pituitary disorder, because the pituitary gonadotroph requires adequate amounts of LH-RH for production and secretion of pituitary gonadotropin (Lovinger et al, 1975). Hashimoto et al(1975) of the lesion.…”

Section: Discussionmentioning

confidence: 99%

A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E.

et al. 1979

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“…23 Data have shown that pituitary aplasia and congenital hypopituitarism do not cause severe IUGR. 24,25 On the contrary, fetal insulin significantly stimulates fetal growth. It is a known fact that pancreatic agenesis is associated with severe growth restriction 26 and that fetal hyperinsulinemia leads to fetal mass overgrowth.…”

Section: Fetal Growth and Metabolismmentioning

confidence: 99%

Fetal and Maternal Physiology and Ultrasound Diagnosis

Kadić¹,

Predojević²,

Kurjak³

et al. 2013

Donald School Journal of Ultrasound in Obstetrics and Gynecology

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Fetal developmental potential is determined at the moment of conception by genetic inheritance. However, this development is modulated by environmental factors. It is important to recognize that both, the mother and the fetus, actively participate in the maintenance of the physiological intrauterine environment. Unfortunately, the fetus is not entirely protected from harmful influences of the external factors. By altering the intrauterine environment, these factors can have a long-term effect on fetal health.

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“…Newborns and infants with neonatal hypopituitarism [20], most often due to the absent or attenuated pituitary stalk syndrome with an ectopic posterior pituitary gland (pituitary stalk dysplasia) or to hypothalamic hypopituitarism associated with the septo-optic dysplasia syndrome [22, 23], are usually detected because of a low serum glucose or thyroxine during routine screening, symptomatic hypoglycemia due to cortisol and/or GHD, shock owing to hypocortisolism, cholestatic jaundice, microphallus, optic hypoplasia with pendular nystagmus, blindness or impaired vision, or polyuria. Over the years we have studied over 40 infants and young children with idiopathic neonatal hypopituitarism and 43 patients with the septo-optic dysplasia-hypopituitarism syndrome.…”

Section: Neonatal Hypopituitarismmentioning

confidence: 99%

The Growth Hormone Cascade: Progress and Long-Term Results of Growth Hormone Treatment in Growth Hormone Deficiency

Grumbach¹,

Bin-Abbas²,

Kaplan³

1998

Horm Res Paediatr

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The growth hormone (GH) cascade and the remarkable advances over the past four decades in our knowledge of its components are considered. It is now over 40 years since human pituitary GH (pit-hGH) was purified and the first GH-deficient patient, a 17-year-old male, was successfully treated with pit-hGH. However, the shortage of pit-hGH limited its use and the dose, the biopotency of preparations varied, strict criteria of GH deficiency (GHD) were used for patient selection including peak plasma immunoreactive GH levels after provocative stimuli of <3.5–5 ng/ml, treatment was not infrequently interrupted, the mean age for initiating treatment was often late in childhood (12–13 years) and the growth deficiency severe (height –4 to –6 SDS), and finally pit-hGH therapy was often discontinued when girls attained a height of 5′ and boys 5′5′′. Nonetheless, the effects of pit-hGH were dramatic; the final height SDS increased in isolated GHD to about –2 SDS in boys and –2.5 to –3.0 SDS in girls, and in multiple pituitary hormone deficiencies to between –1 and –2 SDS. Between 1962 and 1985 when the Creutzfeldt-Jakob disease crisis struck, the number of GH-deficient patients treated with pit-hGH increased from about 150 to over 3,000. The advent of biosynthetic GH (rhGH) and its availability to treat large numbers of idiopathic GH-deficient children (the minimum prevalence rate of which in the USA and UK is between 1 in 3,400 and 4,000) dramatically changed this picture in 1985. It is estimated that more than 60,000 patients have been or are now on treatment. With rhGH treatment the attained mean adult height SDS is now about –1.0, and in our experience with the treatment of patients under 4 years of age, final height may exceed the target height. It is now recognized that (a) the replacement dose of rhGH ranges from 0.175 to 0.35 mg/kg/week and should be individualized; (b) dividing this dose into 6 or 7 daily subcutaneous injections is more effective than giving the same total dose in three weekly portions, and (c) final height correlates significantly with pretreatment chronologic age, height SDS and predicted adult height, duration of therapy, birth length, in some studies height SDS and age at start of puberty, weight, and serum GHBP (an indicator of GH receptor mass). Early recognition of GHD is essential for an optimal height outcome. rhGH treatment should not be delayed in children with documented GHD; the greater the height deficit, the lower the probability that target height will be reached. GHD needs to be detected earlier in children with organic hypopituitarism whether due to a developmental defect, neoplasm, radiation, head trauma, or a CNS infection. Early rhGH therapy in neonatal hypopituitarism has resulted in excellent growth responses. As the height prognosis in isolated GHD is not as good (especially in girls) as in GHD associated with gonadotropin deficiency, the use of LHRH agonists to delay puberty or potent aromatase inhibitors to delay skeletal maturation should be considered in selected patients ...

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Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies

Cited by 135 publications

References 43 publications

A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E.

A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E.

Fetal and Maternal Physiology and Ultrasound Diagnosis

The Growth Hormone Cascade: Progress and Long-Term Results of Growth Hormone Treatment in Growth Hormone Deficiency

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