scholarly journals Evaluation of Endocrine Complications in Beta-Thalassemia Intermedia Patients: A Cross Sectional Multi-Center Study

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2343-2343
Author(s):  
Mehran Karimi ◽  
Tahereh Zarei ◽  
Sezaneh Haghpanah ◽  
Azita Azarkeivan ◽  
Christos Kattamis ◽  
...  

Abstract The diagnosis of beta thalassemia intermedia (BTI) is mainly based on the severity of clinical phenotype. It is associated with a wide range of specific complications including extramedullary hematopoiesis, leg ulcers, gallstones, hypercoagulable state, pulmonary hypertension (PHT), endocrine disorders and osteoporosis. The commonest endocrine complication in beta thalassemia major (TM) patients is hypogonadism followed by hypothyroidism and diabetes but the data on endocrine disorders in BTI patients are scarce. The aim of this study is to determine the prevalence of endocrine complications in a large series of BTI patients. Methods: In this multi-countries cross-sectional study, all BTI patients registered at 12 thalassemic treatment centers in Iran (2 Centers), Italy (2 Centers), Greece, Turkey (2 centers), Oman, Qatar, Jordan, Cyprus and United Kingdom were enrolled during 2017. Non transfusion- dependent beta-thalassemia patients or those who received blood transfusion 3-4 times or less annually, were evaluated. Required information was collected from medical records using a designed questionnaire. Demographic data, clinical characteristics and laboratory data included age, sex, splenectomy, type of treatment (blood transfusion, iron chelation, hydroxyurea), hormonal assays, bone mineral density, calcium -phosphorous metabolism, serum ferritin, liver function tests, fetal and total hemoglobin levels,, platelet and nucleated red blood cell counts, were collected. Results: A total of 721 BTI patients were enrolled in the survey from 9 countries. The most prevalent disease-related complications were osteoporosis (21.6%) and hypogonadism (12.6%) followed by: central hypothyroidism (8.3%), non-insulin-dependent diabetes mellitus (7.8%), primary hypothyroidism (5.5%), insulin-dependent diabetes mellitus (4.2%), hypoparathyroidism (2.2%), growth hormone deficiency (1.1%), adrenal mass (1%) and thyroid cancer (0.5%). Conclusion: This study evaluated the largest cohort of BTI patients with endocrine disorders. Although BTI patients are non-transfusion dependent or only occasionally transfused, iron-overload due to increased intestinal iron absorption and enhanced bone marrow activity cause endocrine disorders and osteoporosis. This study demonstrate that although endocrine complications are less common in patients with BTI compared to data reported in literature in TM patients , a regular monitoring with timely diagnosis and proper management underscoring on osteoporosis and gonadal disorders are crucial to prevent endocrine complications in these patients. Disclosures No relevant conflicts of interest to declare.

2021 ◽  
Vol 7 (2) ◽  
pp. 144-149
Author(s):  
Rizki Fadila ◽  
Alifia Firda Purnomo

Sebagai bagian dari sistem kendali mutu dan kendali biaya dalam pelayanan kesehatan, pemberian layanan kesehatan kepada peserta JKN harus dilaksanakan secara berjenjang sesuai indikasi medis. Fakta menunjukkan bahwa Puskesmas belum menjalankan fungsinya sebagai gatekeeper dengan baik karena tingginya angka rujukan terutama untuk pelayanan non spesialistik. Tujuan penelitian ini adalah untuk mendapatkan gambaran terkait penyebab tingginya angka rasio rujukan non spesialistik di Puskesmas Kabupaten Pasuruan. Penelitian ini merupakan penelitian deskriptif kuantitatif dengan rancang bangun penelitian yaitu cross sectional. Sampel penelitian sebanyak 4 puskesmas yang merupakan puskesmas rawat inap dengan rasio rujukan non spesialistik > 2% di Kabupaten Pasuruan. Hasil penelitian menunjukkan bahwa penyakit yang paling banyak dirujuk adalah insulin-dependent diabetes mellitus. Seluruh puskesmas rawat inap dengan rasio rujukan non spesialistik tinggi tidak memiliki sarana dan prasarana yang lengkap sesuai dengan standar. Selain itu, sebanyak 75% Puskesmas tidak memiliki jenis tenaga kesehatan berdasarkan Permenkes 43 tahun 2019. Dalam hal ini, pemerintah daerah harus selalu berupaya untuk memenuhi kebutuhan sarana, prasarana dan tenaga kesehatan, agar puskesmas dapat memberikan pelayanan kesehatan yang prima.


1990 ◽  
Vol 11 (8) ◽  
pp. 239-247
Author(s):  
Fredda Ginsberg-Fellner

Insulin-dependent diabetes mellitus (IDDM), now usually called type I diabetes, is the most common endocrine disorder of childhood and adolescence, affecting at least 1 in 400 youngsters. In addition, the prevalence appears to be increasing, particularly as noted in Scandinavia, where registries for this disease are maintained. During the past 10 to 15 years, astounding advances have been made, both in the treatment of this disease and in understanding its etiology. However, perfect treatment, prevention, and cure of this disease and its long-term sequelae, which include cardiovascular disease, nephropathy requiring dialysis and renal transplantation, neuropathy, and retinopathy, are still lacking. During the next two decades, progress in these areas will surely be made, and the prognosis for children and adolescents with IDDM will continue to improve. Until the discovery of insulin in 1921, virtually all children, adolescents, and young adults with IDDM died within 3 years of disease onset, often reduced to mere skeletons as their weight rapidly decreased secondary to low calorie diets. With the advent of insulin administration, mortality rapidly decreased and expectations increased that, as in other endocrine disorders requiring replacement therapy, diabetes would no longer be a problem after insulin was widely available. Indeed, the use of short-acting regular or crystalline insulin four times per day along with dietary restriction in the 1920s more closely parallels recommendations for control of the disease today than did the modes of therapy in vogue from the 1930s through the middle 1970s.


1996 ◽  
Vol 76 (03) ◽  
pp. 328-332 ◽  
Author(s):  
Bernd Jilma ◽  
Peter Fasching ◽  
Christine Ruthner ◽  
Anna Rumplmayr ◽  
Sabine Ruzicka ◽  
...  

SummaryBased on findings that showed increased P-selectin expression on platelets and on choroidal microvessels of patients with insulin dependent diabetes mellitus (IDDM), we hypothesized that also plasma concentrations of circulating (c)P-selectin would be increased in these patients.The aim of this study was to compare the plasma levels of cP-selec-tin between non-smoking patients with IDDM, treated with an intensified insulin therapy, and healthy controls. The study design was prospective, cross-sectional and analyst-blinded. Subjects were matched individually for sex, age and body mass index. Plasma levels of cP-selectin and of von Willebrand antigen (vWF-Ag) were determined by enzyme linked immunoassays.Forty-two pairs were available for intergroup comparison. Median plasma concentrations of cP-selectin in patients with IDDM (285 ng/ml; interquartile range: 233-372) were on average 21% higher than those of controls (236 ng/ml; interquartile range: 175-296; p = 0.004). Also, median plasma levels of vWF-Ag were 10% higher in patients (96 U/dl; interquartile range: 82-127) than controls (87 U/dl; interquartile range: 70-104; p = 0.025). There was no correlation between plasma concentrations of cP-selectin and vWF-Ag levels in either group (p ώ0.05).In conclusion, our results of increased cP-selectin levels are in line with increased P-selectin expression on platelets and on choroidal microvessels found in patients with IDDM. In view of the currently developed small molecule inhibitors of cell adhesion molecules, these independent observations together may provide a sound rationale to select P-selectin as a target for treating or preventing IDDM-associated micro- or macrovascular complications.


Sign in / Sign up

Export Citation Format

Share Document