Endogenous Cushing's syndrome is rare, with an incidence of 0.7-2.4 per a million people a year. Clinical presentation of Cushing syndrome can be pleomorphic, and establishing diagnosis can be difficult. Early recognition and rapid control of hypercortisolaemia are necessary to decrease morbidity and mortality in these patients.We report a series of 6 endogenous Cushing's syndromes of different etiologies (4 Cushing's disease and 2 adrenal Cushing's syndrome) assessed in our endocrine department over a decade (2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018)(2019). In order to highlight the diversity of clinical forms, diagnostic tools and specific management of this condition we labelled each case suggestively: the typical Cushing's disease, the Pseudo Cushing's, the elusive Cushing's disease, the mild autonomous cortisol hypersecretion, Cushing's syndrome in pregnancy and Cushing's disease with thromboembolism. We discussed their particularities which were revelatory for the diagnosis, such as dermatologic, cardiovascular, musculoskeletal, neuropsychiatric, or reproductive signs, reviewing literature for each manifestation. We also discuss the commonalities and differences in laboratory and imagistic findings. Therapeutic approach can also differ with respect to the particular condition of each patient and the multiple choices of therapy will be reviewed.
Background Sweat test (ST) remains gold standard in cystic fibrosis (CF) diagnosis. Alarm symptoms are age-related. Aims Retrospective review of cases subjected to ST. Methods Patients were selected by paediatricians, neonatologists, surgeons, based on suggestive symptoms, personal (PH) and familial history (FH). Inclusion criteria: for 0-1 month age group, patients with PH of atelectasis, meconium ileus, intussusception; 1-12 months, recurrent wheezing (RW), failure to thrive (FTT); 1-5 years, previous group symptoms, plus chronic cough/diarrhoea; >5 years, 1-5 years symptoms, plus recurrent pancreatitis/ sinusitis. For all age, patients with PH of salty taste of sweat (STS), salt wasting syndrome, heat shock (HS), and FH of CF, azoospermia. ST was performed with Nanoduct system. Values <60 mmol/L (equivalent NaCl) were considered normal, between 60-80 equivocal, >80 positive. Normal values patients were eventually retested, those with equivocal at least once, those with positive mandatory twice. Results Were performed 406 ST (344 patients): at 0-1 month 11 tests (45,4% peritonitis), at 1-12 months 175(65,5% RW), at 1-5 years 108(25,9% FTT), at >5 years 50(62,5% repeated pneumonia-RP). 4 tests equivocal, 5 false positive, 6 positive (5 infants: 1 RW, 1 HS, 2 atelectasis, 1 STS; 1 male 4 years old RP). Relating to age, only 1 positive from 141 RW infants (p 0,03) and 1 from 15 RP children; 2 positive from 6 atelectasis infants (p 0,0001), 1 from 2 HS and 1 from 2 STS infants (p 0,0002). Neither pancreatitis, FTT or positive FH. Conclusions Great attention on infants with other symptoms than classical ones: higher statistical significance for STS, HS, atelectasis.
Background Open surgery (OS) repair represents the standard treatment in inguinal hernia (IH) in children, in whole world and still very well credited even though modern surgery provides interventional alternatives. Aim Clinical-evolutive assessment of IH repair by OS in 2011-2013 period. Methods The evaluation criteria of retrospective study were: age, environment provenance (EP), diagnosis at discharge, length of stay (LoS), surgical procedure protocol, cost of hospitalisation (CoH) and complications. Results 64 cases were evaluated: 45 (70,31%) younger than 7 years old, 33 (51,56%) originated from rural environment, 44 right side IH (4 strangulated, 5 scrotal-inguinal, 1 descends into labia) and 20 left side IH (2 scrotal-inguinal, 2 descend into labia), 40 (62,5%) with LoS shorter than 5 days. The average LoS was 4,171 days. The surgery protocols record OS in all cases, 2 cases also requiring hematoma evacuation. Were recorded 8 postsurgical complications (5 hematoma, 3 scrotal oedema). Average CoH per day was 326, 70 RON and average CoH per patient 1324,63 RON.IH were more frequent in patient younger than 7 years old from urban environment (p 0,08). Postsurgical complications were significantly more frequent in patient younger than 7 years old (p 0,05) without being influenced by IH side. There were no significant correlation between age and type of complication. Average CoH per day was negative influenced by complications (p 0,02) and positive by average LoS (p 0,09). Conclusions The OS repair in IH in children still remains a frequently used procedure, implies fewer complications and not expensive costs.
Goal Study of spreading of bronchial asthma (BA) and risk factors in the climate of the region of Georgia in children's populations of Tbilisi and Batumi. Materials and methods Study included 4559 children of Tbilisi and Batumi of 5-16 age, 2193 girls, 2366 boys. Children's population was selected by cluster method. At the first stage identification of BA symptoms was provided based on the questionnaire. At the second stage clinical-allergological study of 389 children with positive answers took place. Obtained data were processed by means of SPSS/v12.5 software. Evaluation of degree of association between probability of disease and certain risk-factor was provided based on tetrachoric table (2x2). Obtained results BA symptoms were analysed, distribution frequency by sex, set of diagnostic criteria was reliably high (p=0.002) among boys. Obtained results showed that in Batumi BA diagnosis was made in 6.4% of cases and on Tbilisi-in 4.6% of cases. Clinical-allergological study showed that among children with BA number of boys exceeded girls (p<0.01). BA factors included: humidity (76.4%), weather (56.9%), cloudy and rainy weather (44.3%), season (24.6%), tobacco smell (29.8%), respiratory infections (34.2%), dust (28.5%), physical load (19.7), medicament sensibilization (11.2%) etc. Conclusion Epidemiological study showed high BA frequency in Batumi (6.4%), compared with Tbilisi children's population (4.6%). Identification of risk factors provides basis for development of prevention programs. Data of BA spreading obtained by this study are dramatically different from official data (0.4%) showing high frequency of hipodiagnostiscs of bronchial asthma in region (p<0.01).
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