Clinical observation of a child with hypopituitarism


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Abstract

Introduction. Hypopituitarism is a rare endocrine disease characterized by nonspecific clinical signs, asymptomatic progression, which often leads to late diagnosis and untimely initiation of therapy. The aim of the study was to describe the clinical observation of a child with hypopituitarism, to identify methods for diagnosing this disease according to scientific literature. Materials and methods of research. The analysis of medical documentation (the history of the child's development, the medical record of an outpatient patient, extracts from the medical history of a child undergoing inpatient treatment) of a patient with hypopituitarism was carried out. Results. A clinical case of hypopituitarism in a 4-year-old girl with the manifestation of the disease in the form of progressive obesity in the first year of life is described. During physical examination, excessive development of subcutaneous fat in the absence of any other signs of endocrine pathology attracted attention. The child was monitored for a long time for obesity, hypothyroidism, and received hormone replacement therapy. The diagnosis of hypopituitarism was established only at the age of 3 years on the basis of a clinical, laboratory and instrumental examination, with the determination of a hormonal profile, magnetic resonance imaging (MRI) of the brain. Conclusion. Hypopituitarism, due to the presence of isolated forms, is often disguised as other endocrinological diseases, so clinicians need to be more attentive to obese patients, evaluate their hormonal profile and, if necessary, conduct an MRI of the brain.

Full Text

Introduction. Hypopituitarism (hypothalamic-pituitary insufficiency) is an endocrine disease characterized by partial or complete deficiency of the secretion of hormones from the anterior, posterior or both lobes of the pituitary gland, as well as neurohormones of the hypothalamus [1, 2, 3]. The incidence of this disease varies from 1:4000 to 1:10000 newborns; in the neonatal period it is diagnosed in 23% of cases, despite the fact that symptoms of hypopituitarism with subsequent complications appear in 52% of cases postnatally among patients [4, 5]. In hypopituitarism, impaired secretion may be limited to somatotropic hormone with the subsequent development of an isolated form of somatotropic insufficiency, but can also spread to other pituitary hormones: thyroid-stimulating hormone, adrenocorticotropic hormone, follicle-stimulating and luteinizing hormones, prolactin and antidiuretic hormone (vasopressin) with the formation of multiple deficiency [2 , 3]. Unfortunately, due to the asymptomatic progression of the disease or the nonspecificity of the clinical symptoms of hypopituitarism at an early age, the difficulty of interpreting hormonal parameters and the difficulty of identifying signs characteristic of children on the MRI picture often lead to late diagnosis of the disease and untimely initiation of therapy [4, 5, 6].

The purpose of the work was to describe a clinical case of hypopituitarism in a child and to identify methods for diagnosing this disease according to scientific literature.

Materials and methods of research. An analysis of medical documentation (the history of the child’s development, a medical record of an outpatient, extracts from the medical history of a child who underwent inpatient treatment at the Voronezh Children’s Clinical Hospital of VSMU named after N.N. Burdenko) was carried out.

Research results. An analysis of the medical records of a girl aged 4 years at the time of the study was carried out. A child from the first pregnancy, which was threatened with termination, with gestosis in the third trimester. The birth is the first, urgent, independent with a double tight entanglement of the umbilical cord. Body weight at birth – 2850 g, body length – 51 cm. At the age of 2 months, surgical removal of a hemangioma of the scalp was performed. There is no allergic history. In the first year of life, there was a delay in neuropsychic development. From the anamnesis it is known that until the child was one year old, he gained excessive weight - 1 - 1.5 kg monthly. The weight of the child at the age of 6 months was 10 kg, and therefore the girl was first consulted by a pediatric endocrinologist; as a result of the examination (physical examination, anthropometry, general clinical blood test, biochemical blood test, thyroid hormone test), the diagnosis was established: “I degree obesity . Hyperlipidemia. Subclinical hypothyroidism." Diet therapy and hormone replacement therapy with levothyroxine sodium at a dose of 25 mcg were prescribed. At the age of 1.5 years, the patient was referred to a geneticist for consultation to exclude genetic diseases: a diagnosis of familial hyperlipidemia was established. Despite the prescribed therapy, the child experienced progression of obesity: at the age of 2 years, III degree obesity was diagnosed (weight 23.5 kg, Standard Deviation Score (SDS) body mass index +3.6). The child was sent at the age of 2.5 years for an inpatient examination, where, in addition to stage III obesity and hypothyroidism, diabetes insipidus was detected, and treatment with desmopressin at a dose of 50 mcg was prescribed under the control of urine specific gravity. At the age of 2.6 years, the child underwent a magnetic resonance examination of the brain, which revealed a decrease in the signal intensity from the neurohypophysis and a small cystic structure of the pineal gland. During hospitalizations, the levels of adrenocorticotropic hormone and cortisol were assessed; all indicators were within normal limits. During a planned hospitalization at the age of 3 years in the endocrinology department of the Voronezh Children's Clinical Hospital of VSMU named after. N.N. Burdenko was the first to reveal a decrease in the level of cortisol in the blood serum and in the daily amount of urine with a normal level of adrenocorticotropic hormone. X-rays of the hands were also performed to assess bone age, which revealed no abnormalities. As a result of the examination, a diagnosis of “Hypopituitarism” was made. Obesity IV degree, morbid. Insulin resistance. Diabetes insipidus. Secondary hypothyroidism. Threatened by the development of acute adrenal insufficiency. Delayed speech development." At the time of the study, the girl was 4 years old. Height 105 cm, weight 30 kg, SDS body mass index +7.4. The skin is clean, there are no striae. Subcutaneous fat is overdeveloped with a predominant distribution in the abdominal area. The thyroid gland is not palpable. On auscultation of the lungs, breathing is puerile, there are no wheezes. Heart sounds are sonorous and rhythmic. The abdomen is soft and painless on palpation, the liver is not palpable, the spleen is not palpable. Stool and urination are not impaired. The patient underwent clinical, laboratory and instrumental examination: general clinical blood test, general clinical urine test - without pathology; hormonal profile: a decrease in the level of cortisol and thyroxine was noted, other indicators were within the age norm. Due to drug-induced decompensation of hypothyroidism, the dose of levothyroxine sodium was increased to 37.5 mcg per day for the child, and replacement therapy with desmopressin was continued. Discussion: due to the absence of specific manifestations, the diagnosis in this clinical case was established at 3 years. All this time, the child was regularly observed by an endocrinologist with a diagnosis of morbid obesity, secondary hypothyroidism, and diabetes insipidus of central origin.

Conclusion. Hypopituitarism, due to the presence of isolated forms, is often disguised as other endocrinological diseases, so clinicians need to be more attentive to young patients with obesity, conduct a dynamic assessment of physical development, promptly examine the hormonal profile and, if necessary, prescribe magnetic resonance imaging of the brain and pituitary gland Hypopituitarism is an incurable disease, but with early detection and initiation of replacement therapy, it is possible to normalize the functions of peripheral endocrine glands and maintain a high quality of life for the patient.

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About the authors

Michail Borisovich Remezov

Voronezh State Medical University named after N.N.Burdenko

Author for correspondence.
Email: docmikle@mail.ru
ORCID iD: 0000-0003-1598-6768

4th year student of the Faculty of Pediatrics

Russian Federation, 394036, Russia, Voronezh, st. Studencheskaya, 10

Christina Olegovna Ovsyannikova

Voronezh State Medical University named after. N. N. Burdenko

Email: kr.ovs@yandex.ru
ORCID iD: 0009-0009-4372-5021

2nd year resident at the Department of Faculty Pediatrics

Russian Federation, 394036, Russia, Voronezh, st. Studencheskaya, 10

Anna Sergeevna Ivannikova

Voronezh State Medical University named after. N. N. Burdenko

Email: ivannikofff@mail.ru
ORCID iD: 0000-0002-3664-6394

Ph.D., Associate Professor, Department of Faculty and Palliative Pediatrics

Russian Federation, 394036, Russia, Voronezh, st. Studencheskaya, 10

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