Although total body irradiation (TBI) was considered to be the primary cause of thyroid dysfunction following hematopoietic stem cells transplantation (HSCT), a significant prevalence of subclinical ...hypothyroidism after HSCT with chemotherapy-only conditioning regimens has been observed in several studies. The aim of this study was to assess changes in thyroid stimulating hormone (TSH) levels in children after HSCT, without the use of irradiation at any time in the course of the treatment.
Our cohort consisted of 41 children and adolescents who underwent autologous or allogeneic HSCT and were available for follow-up for at least one year after transplantation. Irradiation was not performed in any of the subjects, neither during pretransplatation therapy, nor during conditioning. The median duration of follow-up was 2.9 years. The indications for HSCT were hematologic malignancy (41.5%), solid malignant tumor (34.1%), and other disorders (24.4%). The thyroid status of all the subjects was assessed prior to HSCT and after follow-up period.
Thyroid dysfunction after HSCT was present in 27 (65.8%) subjects. Subclinical hypothyroidism was the most common abnormality, presenting in 23 (56.1%) patients, primary hypothyroidism was present in one (2.4%) patient, while 3 (7.3%) subjects had low free T4 with normal TSH values. Significantly (p < 0.01) higher elevations in TSH levels were present in the patients who received chemotherapy for the underlying disease prior to HSCT.
Our findings emphasize the need for long-term monitoring of thyroid function following HSCT, regardless of whether or not irradiation was used.
Growth hormone deficiency (GHD) can be isolated or associated with deficiency of other pituitary gland hormones. According to age at diagnosis, causes of GHD are divided into congenital or acquired, ...and according to etiology into recognized and unknown.
We analyzed etiology and prevalence of GHD, demographic data at birth, age, body height (BH) and bone age at diagnosis as well as the frequency of other pituitary hormone deficiencies.
The study involved 164 patients (109 male).The main criterion for the diagnosis of GHD was inadequate response of GH after two stimulation tests.The patients were classified into three groups: idiopathic, congenital and acquired GHD.
Idiopathic GHD was confirmed in 57.9% of patients, congenital in 11.6% and acquired in 30.5%. The mean age at diagnosis of GHD was 10.1 +/- 4.5 years.The patients with congenital GHD had most severe growth retardation (-3.4 +/- 1.4 SDS), while the patients with idiopathic GHD showed most prominent bone delay (-3.6 +/- 2.3 SDS).The prevalence of multiple pituitary hormone deficiency was 56.1%, in the group with congenital GHD 73.7%, acquired GHD 54.0% and idiopathic GHD 53.7%.The frequency of thyrotropin deficiency ranged from 88.2-100%, of adrenocorticotrophin 57.1-68.8% and of gonadotrophins deficiency 57.1-63.0%, while deficiency of antidiuretic hormone was 2.0-25.0%.
Although regular BH measurements enable early recognition of growth retardation, patients' mean age and degree of growth retardation indicate that GHD is still diagnosed relatively late. A high incidence of other pituitary hormone deficiencies requires a detailed investigation of the etiology of disorders and evaluation of all pituitary functions in each child with confirmed GHD.
Mastocytosis is a clonal neoplastic disorder of the mast cells. The clinical signs and symptoms of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly ...aggressive neoplasm with survival of about 6 months. Systemic mastocytosis (SM) is characterized by mastocyte infiltration of one or more organs, with or without skin involvment.
The presented patient presents a highly challenging diagnostic and therapeutic case. A 46-year-old man was referred to our Centre due to the 7-year-long history of hepatosplenomegaly and mild thrombocytopenia. Ultrasound examination showed hepatosplenomegaly (liver 170 mm; spleen 200 mm), platelet count was 90 x 10(9)/L, serum tryptase level was elevated and bone marrow biopsy showed infiltration with mast cells (CD117, CD25 and mast cell tryptase positive). Our patient was diagnosed with aggressive systemic mastocytosis (SM) according to WHO Classification (2008), although the clinical course of the disease was indolent, without complications for more than 7 years. Because of the 'intermediate' course, this patient was referred to as smouldering or intermediate SM and was not treated with cytostatics.
Utilizing the established criteria, indolent SM can be discriminated from the aggressive subvariants of SM in most cases. However, a small group of patients, like our case belongs to the "grey zone". Therapeutic approach to these patients is individual and prognosis is uncertain.
Introduction: Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases caused by a deficiency of enzymes responsible for the steroidogenesis. There are three forms of CAH due ...to 21-hydroxylase deficiency: the classic form with salt loss, the classic virilizing and the non-classic form. The aim of the paper was to analyze the changes in the diagnosis and treatment of children with CAH during previous 15 years. Material and methods: This retrospective cohort study includes patients who were diagnosed with CAH due to 21-hydroxylase deficiency in the period from 2007 to 2021 in endocrinology department of the Institute for Mother and Child Health Care Institute of Serbia "Dr Vukan Cupic". Respondents were divided into two groups - a group whose diagnosis was made in the period between 2007 and 2014 and another group of those whose diagnosis was made in the period between 2015 and 2021. Statistical analysis using Hi-square and Mann Whitney U test was conducted using the software IBM SPSS ver. 22, and p values <0.05 were considered significant. Results: Out of the total of 55 patients included in the study, 46 patients (83.6%) had 46, XX karyotype. The diagnosis was made in all patients on the basis of biochemical analyzes. In the second group the diagnosis was confirmed by genetic analysis in statistically significantly higher number of children (p <0.05). 49 patients (89.1%) received hydrocortisone and 16 patients received fludrocortisone. In patients from the second group a statistically significantly higher frequency of fludrocortisone therapy was noticed in patients with classic CAH. Conclusion: Having in mind the constant advancement in the field, frequent improvements in clinical care of children with CAH are needed.
Introduction. Adrenal insufficiency results from the inadequate adrenocortical conjunction. Adrenal insufficiency can be primary, secondary and tertiary one. The most common cause of adrenal ...suppression is the effect of exogenous therapy with glucocorticoids. Glucocorticoids. Corticosteroids are used in treatment of endocrine and non-endocrine diseases. They are applied as a substitution therapy in the patients with primary and secondary adrenal insufficiency. Due to their immunosuppressive and anti-inflammatory characteristics, they are used to treat a wide range of diseases. They are usually divided according to the length and size of the effect i.e. how they are applied. Adrenal Insufficiency. Glucocorticoid therapy may lead to a number of adverse effects such as a disorder in glucose metabolism, osteoporosis or frequent infections. Adrenal suppression is the most common complication resulting from corticosteroid application. The function of the hypothalamus-pituitaryadrenal axis may be inhibited for months after the treatment cessation. There are several predictors of potential glucocorticoid-induced adrenal suppression. Diagnosing Adrenal Insufficiency. The most frequent symptoms and signs of adrenal insufficiency are fatigue, nausea and vomiting, hyponatremia, hyperpigmentation or hypotension. Algorithm for the diagnosis of adrenal insufficiency must be followed in clinical practice. Reduction in Glucocorticoid Therapy. Reduction or complete cessation of the therapy is indicated when the maximum therapeutic benefit has been achieved or when considerable side effects, such as diabetes mellitus, severe hypertension, osteoporosis i.e. adrenal insufficiency, develop. Conclusion. Numerous synthetic glucocorticoids have been developed to be used in everyday clinical practice and they can be administered systemically or locally. A lot of side effects are associated with chronic administration of glucocorticoids. In order to avoid complications, it is recommended to administer intermediate-acting glucocorticoids every second day. In addition, the patients must be monitored carefully and glucocorticoid therapy should be discontinued gradually to prevent adrenal insufficiency or reactivation of the disease under therapy. Key words: Glucocorticoids; Adrenal Insufficiency; Drug-Related Side Effects and Adverse Reactions; Diagnosis; Signs and Symptoms; Hydrocortisone Sazetak Uvod. Adrenalna insuficijencija posledica je neadekvatne adrenokortikalne sprege. Razlikujemo primarnu, sekundarnu i tercijarnu adrenalnu insuficijenciju. Efekat egzogene terapije glukokortikoidima je najcesci uzrok adrenalne supresije. Glukokortikoidi. Kortikosteroidi se koriste u lecenju endokrinih i neendokrinih oboljenja. Primenjuju se kao supstituciona terapija kod bolesnika sa primarnom ili sekundarnom adrenalnom insuficijencijom. Zbog imunosupresivnih i antiinflamatornih osobina koriste se u sirokom spektru oboljenja. Najcesce ih delimo prema duzini i jacini dejstva, odnosno prema nacinu primene. Adrenalna insuficijencija. Terapija glukokortikoidima moze da dovede do brojnih nezeljenih efekata - poremecaja metabolizma glukoze, osteoporoze ili cestih infekcija. Najcesca komplikacija prilikom upotrebe kortikosteroida je adrenalna supresija. Inhibicija funkcije hipotalamusno-hipofiznonadbubrezne osovine, izazvana upotrebom kortikosteroida, moze trajati mesecima nakon sto je tretman obustavljen. Postoji nekoliko prediktora potencijalne glukokortikoidima indukovane adrenalne supresije. Dijagnostika adrenalne insuficijencije. Nadbubrezna insuficijencija manifestuje se skupom simptoma i znakova, a najcesce su to malaksalost, mucnina i povracanje, hiponatremija, hiperpigmentacija ili hipotenzija. U klinickoj praksi neophodno je pratiti algoritam dijagnostike nadbubrezne insuficijencije. Smanjenje glukokortikoidne terapije. Smanjene ili potpuno ukidanje terapije je indikovano kada je postignuta maksimalna terapeutska korist, ili kada se pojave znacajni sporedni efekti poput dijabetesa melitus, teske hipertenzije, osteoporoze, odnosno nadbubrezne insuficijencije. Zakljucak. Razvijeni su brojni sintetski glukokortikoidi koji se koriste u svakodnevnoj klinickoj praksi koji se mogu ordinirati sistemski ili lokalno. Mnogi sporedni efekti su povezani sa hronicnom administracijom glukokortikoida. Da bi se izbegle komplikacije, preporucuje se terapija na drugi dan primenom glukokortikoida srednje dugog dejstva. Takode, potrebno je pazljivo pracenje pacijenata i postepeno ukidanje glukokortiokoidne terapije kako bi se izbegla nadbubrezna insuficijencija ili reaktivacija bolesti pod terapijom. Kljucne reci: glikokortikoidi; adrenalna insuficijencija; nuspojave i nezeljeni efekti lekova; dijagnoza; znaci i simpotomi; kortizol
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