Thalassemia ENDOCRINOPATHIES IN THALASSEMIA PATIENTS

Objective: To determine the common endocrine complications found in children having thalassemia major. Study Design: Cross-sectional study. Place and Duration of Study: Department of Pediatric Medicine, Combined Military Hospital Multan, from May to Nov 2019. Methodology: A total of 160 Children with thalassemia were taken in this study. Once registered, venous blood sample was taken and sent to the laboratory for endocrine profile. SPSS-21 was applied for analysis of collected data. Results: Out of 160 study cases, 100 (62.5%) were boys while 60 (37.5%) were female patients. Mean age of our study cases was 8.58 ± 1.98 years. Mean duration of disease was 5.28 ± 3.29 years. Parental consanguinity was positive in 122 (76.3%) and only 47 (29.4%) were taking chelation therapy. Mean HbA1C level was 6.23 ± 1.18% mg/dl and diabetes was noted in 41 (25.6%) of our study cases. Mean FT4 was 0.98 ± 0.13 ng/dl and hypothyroidism was noted in 24 (15%) of our study cases. Conclusion: Diabetes and hypothyroidism were the common endocrine complications noted in our study among children having thalassemia. All physicians treating such patients should always screen such patients for early diagnosis and timely management in order to reduce burden of related morbidities and enhance quality of life of these patients.

A Systematic Review of Subacute Thyroiditis Related to COVID-19

Background: The novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), which belongs to the family Coronaviridae, is the cause of COVID-19 infection. Its outbreak was declared a pandemic on March 11, 2020. COVID-19 does not involve the respiratory system solely, but other systems were also noted to be affected, including the endocrine, cardiovascular, and gastrointestinal systems. Several case reports and series have been published regarding SAT related to COVID-19 infection, yet management and clinical outcomes of the disease have not been discussed in detail. Methods: This is a systematic review of cases that have been reported to have subacute thyroiditis induced by COVID-19 infection. A systematic search was conducted throughout multiple databases, including PubMed, Google Scholar, and MeSH network. Results: The total number of reported subacute thyroiditis cases attributed to COVID-19 is 24. There was a female predominance (18 females and 6 males) with a female to male ratio of 3:1. Ages ranged from 18 to 69 years (mean = 38.67). Twenty-four symptoms related to thyroiditis were reported, the most common of which being neck pain (95.83%, n=23), palpitations (79.17%, n=19), and fever (66.67%, n=16). The outcome was complete resolution in 70% of cases. Conclusion: The endocrine complications of COVID-19 and their management have been disregarded by most as they are rare. Our knowledge of COVID-19 and its complications is growing rapidly. More favourable outcomes were linked with the use of corticosteroid therapy. Until larger studies can be conducted, the management of SAT caused by COVID-19 remains to be based on each individual case. However, the treatment regimen should include corticosteroid therapy.

Glycated Albumin as Marker for Early Hyperglycemia Detection in Adolescent with β Thalassemia Major

BACKGROUND: Hyperglycemia is one of the most common endocrine complications in children with β thalassemia major. Though the current diagnostic marker either requires fasting, has low reproducibility, or it is not an accurate for thalassemia patients. Glycated albumin (GA) is a quick and easy alternative marker for hyperglycemia detection and monitoring of glycemic control. However to date, no studies have analyzed the role of GA value in detection of hyperglycemia in children with thalassemia major. This study analyzed the value of GA as an alternative screening marker for hyperglycemia detection in children with β thalassemia major.METHODS: A single-blind prospective diagnostic test was conducted in 9 to 18 years old children with β thalassemia major and who were treated at the Dr. Moewardi Regional General Hospital between October 1, 2018 and December 31, 2019. In a single, fasted study visit, height, weight, fasting blood glucose (FPG), GA, oral glucose tolerance test (OGTT) were measured. The area under a receiver operating characteristic curve (AUC) was used to determine the cut-off value at which hyperglycemia prediction (OGTT ≥140 mg/dL) display optimal sensitivity and specificity. RESULTS: Among the 53 children with β thalassemia major, 1 (1.9%) had diabetes mellitus and 4 (7.5%) had prediabetes based on their OGTT values. The median GA value in this study was 10.9% (range: 7.6–12.4%). GA had a low AUC (0.646, p=0.287) for hyperglycemia detection in pediatric patients with β thalassemia major. At a cut-off of 11.45%, GA demonstrated 60% sensitivity and 60.4% specificity.CONCLUSION: GA cannot be used as an alternative marker for hyperglycemia detection in children with β thalassemia major.KEYWORDS: hyperglycemia, diabetes mellitus, prediabetes, β thalassemia major, glycated albumin

Early and late endocrine complications of COVID-19

Endocrine system plays a vital role in controlling human homeostasis. Understanding the possible effects of COVID-19 on endocrine glands is crucial to prevent and manage endocrine disorders before and during hospitalization in COVID-19-infected patients as well as to follow them up properly upon recovery. Many endocrine glands such as pancreas, hypothalamus and pituitary, thyroid, adrenal glands, testes, and ovaries have been found to express angiotensin-converting enzyme 2 receptors, the main binding site of the virus. Since the pandemic outbreak, various publications focus on the aggravation of preexisting endocrine diseases by COVID-19 infection or the adverse prognosis of the disease in endocrine patients. However, data on endocrine disorders both during the phase of the infection (early complications) and upon recovery (late complications) are scarce. The aim of this review is to identify and discuss early and late endocrine complications of COVID-19. The majority of the available data refer to glucose dysregulation and its reciprocal effect on COVID-19 infection with the main interest focusing on the presentation of new onset of diabetes mellitus. Thyroid dysfunction with low triiodothyronine, low thyroid stimulating hormone, or subacute thyroiditis has been reported. Adrenal dysregulation and impaired spermatogenesis in affected men have been also reported. Complications of other endocrine glands are still not clear. Considering the recent onset of COVID-19 infection, the available follow-up data are limited, and therefore, long-term studies are required to evaluate certain effects of COVID-19 on the endocrine glands.

Endocrine complications in thalassemic children at a tertiary childcare hospital of South Punjab, Pakistan.

Objective: To find out the frequency of endocrine complications in thalassemic children at a tertiary care hospital. Study Design: Cross Sectional study. Setting: Department of Pediatric Medicine, “The Children’s Hospital and Institute of Child Health (CHICH), Multan,”. Period: 10-08-2018 to 09-05-2019. Material & Methods: A total of 160 children of both gender and aged 2 – 14 years having thalassemia major were enrolled from thalassemia center, CHICH Multan. Venous blood sample (5 ml) was taken and dispatched to central institutional laboratory for performing specific investigations. Results: Majority of the patients, 101 (63.1 %) were male. Overall, mean age was 6.79+2.10 years. Mean no. of transfusions in our study cases was 3.16+1.14 while 128 (80.0%) had to undergo blood transfusion more than twice per month. Parental consanguinity was positive in 122 (76.2%). Mean Fasting blood sugar level was 117.28+32.43 mg/dl and diabetes was noted in 70 (43.8%). Mean FT4 was 0.99+0.12 ng/dl and hypothyroidism was noted in 32 (20.0%). Endocrine complications were noted in 102 (36.8%) cases. Conclusion: Very high frequency of endocrine complications was seen among children having thalassemia. Endocrine complications were significantly associated with age, residential status, number of blood transfusions per month, disease duration and parental consanguinity.

The Dose of Somatostatin Analogues during Pre-Surgical Treatment Is a Key Factor to Achieve Surgical Remission in Acromegaly

Purpose: to determine whether pre-surgical treatment using long-acting somatostatin analogues (SSAs) may improve surgical outcomes in acromegaly. Methods: retrospective study of 48 patients with acromegaly operated by endoscopic transsphenoidal approach and for first time. Surgical remission was evaluated based on the 2010 criteria. Results: most patients, 83.3% (n = 40), harbored macroadenomas and 31.3% (n = 15) invasive pituitary adenomas. In this case, 14 patients were treated with lanreotide LAR and 6 with octreotide LAR, median monthly doses of 97.5 [range 60–120] and 20 [range 20–30] mg, respectively, for at least 3 months preoperatively. Presurgical variables were comparable between pre-treated and untreated patients (p > 0.05). Surgical remission was more frequent in those pre-treated with monthly doses ≥90 mg of lanreotide or ≥30 mg of octreotide than in untreated or pre-treated with lower doses (OR = 4.64, p = 0.025). However, no differences were found between pre-treated and untreated patients when lower doses were included or between those treated for longer than 6 months compared to those untreated or pre-treated for shorter than 6 months. Similarly, no differences were found either in terms of surgical or endocrine complications (OR = 0.65, p = 0.570), independently of the doses and the duration of SSA treatment (p > 0.05). Conclusions: the dose of SSAs is a key factor during pre-surgical treatment, since the beneficial effects in surgical remission were observed with monthly doses equal or higher than 90 mg of lanreotide and 30 mg of octreotide, but not with lower doses.

The Dose of Somatostatin Analogues During Pre-Surgical Treatment is a Key Factor to Achieve Surgical Remission in Acromegaly

Purpose: To determine whether pre-surgical treatment using long-acting somatostatin analogues (SSAs) may improve surgical outcomes in acromegaly. Methods: Retrospective study of 48 patients with acromegaly operated by endoscopic transsphenoidal approach and for first time. Surgical remission was evaluated based on the 2010 criteria. Results: Most patients, 83.3% (n=40), harboured macroadenomas and 31.3% (n=15) invasive pitu-itary adenomas. Fourteen patients were treated with lanreotide LAR and 6 with octreotide LAR, median monthly doses of 97.5 [range 60-120] and 20 [range 20-30] mg, respectively, for at least 3 months preoperatively. Presurgical variables were comparable between pre-treated and un-treatred patients (P>0.05). Surgical remission was more frequent in those pre-treated with monthly doses ≥90 mg of lanre-otide or ≥30 mg of octreotide than in untreated or pre-treated with lower doses (OR=4.64, P=0.025). However, no differences were found between pre-treated and untreated patients when lower doses were included or between those treated for longer than 6 months compared to those untreated or pre-treated for shorter than 6 months. Similarly, no differences were found either in terms of surgical or endocrine complications (OR=0.65, P=0.570)), independently of the doses and the duration of SSA treatment (P>0.05). Conclusions: The dose of SSAs is a key factor during pre-surgical treatment, since the beneficial effects in surgical remission were observed with monthly doses equal or higher than 90 mg of lanreotide and 30 mg of octreotide, but not with lower doses.

An urgent need for improving thalassemia care due to the wide gap in current real-life practice and clinical practice guidelines

Based on Thalassemia International Federation clinical practice guidelines (CPG) for non-transfusion dependent and transfusion dependent thalassemia, several measures should be routinely implemented such as monitoring and surveillance of thalassemia related complications for early detection and proper clinical management. To evaluate the prevalence and the performance of routine surveillance for thalassemia related complications during 2 periods; before and after published CPGs (2012–2014 vs 2015–2017), data from 524 adult thalassemia patients attended at Siriraj hospital were compared among different treating physician groups; thalassemia, private hematology, and internal medicine clinics. Three most common complications were osteopenia/osteoporosis (69.8%), gallstones (67.6%) and abnormal vitamin D level (67.6%). Iron overload has been widely evaluated (93.1%) followed by liver function test (82.3%). However, the rate of evaluation for other complications were significantly reduced and < 25% of patients were evaluated in several complications. Comparing among clinics, the surveillance rate has increased significantly for several endocrine complications only in patients treated at thalassemia clinic but not in others. This study was the first study that evaluated real-world practical management of thalassemia patient in terms of complication surveillance. This different clinical practice has called for an immediate policy change to improve and standardize a care for thalassemia patients in Thailand.