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CASE REPORT
Allgrove syndrome (Triple-A Syndrome): A case report from north India
Hamid Ashraf, Nikhil Varshney, M. D Juned, Sheelu Shafiq Siddiqui
January-March 2019, 2(1):13-15
DOI:10.4103/jdep.jdep_11_18  
Triple-A syndrome, also known as Allgrove syndrome, is an uncommon disorder which is inherited as an autosomal recessive disorder. About 100 cases have been described in literature. The three AAA comprises adrenal insufficiency secondary to adrenocorticotropic hormone (ACTH) resistance, achalasia cardia, and alacramia. We are reporting a case of a 10-year-old boy diagnosed as triple-A syndrome with ACTHresistant adrenal insufficiency, achalasia cardia, and alacrimia. He has alacramia since birth, and at the age of 7 years, he was diagnosed to have achalasia cardia. He developed the symptoms of adrenal insufficiency at the age of 9 years. Allgrove syndrome might be underreported in literature as the diagnosis requires high index of suspicion. In our patient, there was a delay of 3 years after the initial diagnosis of achalasia cardia. The diagnosis of Allgrove syndrrome should be considered in every child presenting with alacremia or achalasia cardia.
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ORIGINAL ARTICLES
COVID-19 lockdown and hypoglycemia among patients with diabetes fasting the month of Ramadan 2020
Zahra Abdulkareem Ghareeb, Zainab Al Saffar, Ghadeer Al Ghareeb, Ghalia Jassam Almaalu, Alia Alnaj
April-June 2021, 4(2):64-68
DOI:10.4103/jdep.jdep_1_21  
Background: Patients with diabetes are targeted with diabetes education 8–10 weeks before Ramadan to have a safe fasting experience. In 2020, this timing coincided with COVID-19 lockdown when virtual clinics were implemented to secure patient care continuity. Objectives: We evaluated hypoglycemia episodes among patients with diabetes fasting in Ramadan 2020 during COVID-19 lockdown. Patients and Methods: A cross-sectional study was conducted after the month of Ramadan 2020, using a self-administered online questionnaire and convenient sampling methods. Patients with diabetes aged 14 years and older who practiced fasting in Ramadan were included in the study. Hypoglycemia rate in total and diabetes treatment among observers of Ramadan 2019 and Ramadan 2020 were compared. Predictors of hypoglycemia during fasting Ramadan 2020 were estimated. Results: Out of 367 patients with diabetes aged 14 years and older, 326 (88.8%) participants had the ability to fast Ramadan and were included in the study. Comparing patients' experience in Ramadan 2019 and Ramadan 2020, participants reported lower hypoglycemia episodes during Ramadan 2020 than Ramadan 2019, P = 0.017. In subgroup analysis based on the type of treatment, patients on noninsulin treatment had statistically significant lower hypoglycemia episodes during Ramadan 2020 than Ramadan 2019 (P = 0.006). Patients on insulin treatment had fewer hypoglycemia episodes during Ramadan 2020 than Ramadan 2019, but the difference was not statistically significant (P = 0.405). The probability of hypoglycemia during fasting Ramadan 2020 was higher among younger age groups 14–30 years (odds ratio [OR] 7.24, 95% confidence interval [CI] 1.72–30.39), patients aged 31–45 years (OR 7.18, 95% CI 1.97–26.19), patients with longer duration of diabetes (more than 10 years) (OR 2.30, 95% CI 1.01–5.26), and patients on insulin (OR 14.14, 95% CI 1.72–30.39). Conclusions: COVID-19 pandemic and lockdown did not affect the diabetes-related experience among participants' fasting Ramadan. The hypoglycemia episodes of patients with diabetes in Saudi Arabia were improved during the pandemic era. This could be related to telemedicine services. More studies are required to support its role in improving health conditions beyond our population's pandemic era.
  1 1,608 114
Patterns of clinical management of hypothyroidism in adults: An electronic survey of physicians from the Middle East and Africa
Salem A Beshyah, Ibrahim H Sherif, Huda E Mustafa, Hussein F Saadi
April-June 2021, 4(2):75-82
DOI:10.4103/jdep.jdep_14_21  
Background: Hypothyroidism is a common endocrine disorder that is managed by a wide range of physicians. There are no data on the pattern of clinical management of hypothyroidism in the Middle East and Africa (MEA) region. Objectives: We sought to document current practices in the management of primary hypothyroidism in the MEA region and compare these with international recommendations and practices elsewhere. Materials and Methods: A convenience sample of physicians practicing in the MEA in relevant disciplines were invited to take a web-based survey consisting of previously validated multiple-choice questions dealing with investigation and treatment of an index case of overt primary hypothyroidism in general and in three special situations. Results: Out of complete 397 responses, 368 were eligible for inclusion in the analysis. The majority were endocrinologists and internal medicine specialists; 82.2% of them have been in clinical practice for 10 years or more. Overt hypothyroidism would be treated using L-T4 alone by 97.2% of respondents; 1.7% would use a combination of L-T4 and liothyronine (L-T3) therapy. The rate of replacement would be gradual (66.5%), an empiric dose, adjusted to achieve target levels (14.7%); or a calculated full replacement dose (18.5%). A target thyroid-stimulating hormone (TSH) of 2.0–2.9 mU/L was favored in the index case of overt hypothyroidism (by 34.4%) followed by a target of 3.0–3.9 mU/L (by 26.0%) of respondents. However, a target of 4.0–4.9 mU/L was the most commonly selected TSH target for an octogenarian (by 33.5% of respondents). Persistent hypothyroid symptoms despite achieving a target TSH would prompt testing for other causes by 86.9% of respondents, a change to L-T4 plus L-T3 therapy by 5.8%, and an increase in the thyroid hormone dose by 4.6%. Evaluation of persistent symptoms would include measurements of complete blood count (82.4%), complete metabolic panel (68.7%), morning cortisol (65.3%), Vitamin B12 levels (54.5%), and serum T3 levels (27.9%). Subclinical disease with a TSH 7.8 mU/L would be treated without further justification by 9.0% of respondents, or in the presence of positive thyroid peroxidase antibodies (65.3%), hypothyroid symptoms (65.0%), high low-density lipoprotein (51.7%), or a goiter (36.7%). The TSH target for a newly pregnant patient was 2.0–2.4 mU/L for 28.5% of respondents, with 15.8% preferring a TSH target of 1.5–1.9 mU/L. Thyroid hormone levels would be checked every 4 weeks during pregnancy by 62.9% and every 8 weeks by an additional 17.6%. A hypothyroid patient with a TSH of 0.5 mU/L who becomes pregnant would receive an immediate L-T4 dose increase by only 28.5% of respondents. Conclusions: The survey revealed that (1) nearly exclusive preference for L-T4 alone for therapy, (2) use of age-specific TSH targets for replacement therapy, (3) a low threshold for treating mild thyroid failure, (4) complacent and variable attention to TSH targets in the pregnant and prepregnant woman, and (5) a highly variable approach to both the rate and means of restoring euthyroid status for overt disease. Both alignments with and divergence from guidelines were detected. The results should help in directing focused educational activities in the region, providing a baseline for future monitoring of practices.
  1 1,384 85
REVIEW ARTICLE
Ramadan fasting and diabetes (2020): The year in review
Khadija Hafidh, Khawla F Ali, Saira Abbas, Salem A Beshyah
April-June 2021, 4(2):46-58
DOI:10.4103/jdep.jdep_7_21  
The literature on health aspects of Ramadan fasting (RF) is widely spread in many journals, making it not readily accessible to those interested in the subject. We aimed to provide a narrative overview of the global literature production in 1 year (2020) on diabetes and RF. This was a narrative, nonsystematic review of the international literature from a single major medical online database (i.e., PubMed) during the year 2020. The search term “Ramadan fasting AND Diabetes” was used, and the relevant literature was narrated in a concise thematic account. The publications spanned a vast array of topics related to RF, including assessments of safety and efficacy profiles of older and newer diabetes therapies, modes of insulin delivery, evaluating the role of utilizing advanced technology for the treatment, and monitoring of blood glucose during RF. Increased interest was evident in capturing patients' perspectives and healthcare professionals' perceptions, attitudes, and practices during Ramadan. Fasting by high-risk groups was studied. Not surprisingly, some reports covered COVID-19 and Ramadan and the role of telemedicine in ramadan. The current literature review presents this year's research data on the safety of fasting practices, care models, and patients' experiences and perspectives. It emphasizes the need for more comprehensive interventions for high-risk patients, promoting newer antidiabetic medicines, and advanced technology for safer fasting practices.
  1 2,026 146
REVIEW ARTICLES
Nasal glucagon: A new hope for severe hypoglycemia in type 1 diabetes
Salva Fatima Heba, Uzma Parveen, Sara Sana Ayesha Khanum, Maaria Gulnaaz, Maimuna Tabassum, Syeda Batool Safiyya
January-March 2021, 4(1):13-18
DOI:10.4103/jdep.jdep_6_20  
Until now, injectable glucagon was the only available treatment used in the management of severe hypoglycemia wherein glucagon had to be prepared in several steps before administration. This method of delivery of injectable glucagon being cumbersome and unappealing for wide majority of the patients had led to a search for an alternative route of drug delivery. nasal glucagon (NG) now serves an efficient, safe, easy-to-administer, and a favorable substitute to glucagon injections. This ready to use device stand in clear contrast to overcome the limitations associated with the currently available glucagon preparations has emerged a key advancement in the management of severe hypoglycemia in adolescents and children with type 1 diabetes. NG is now being developed and studied in other countries as well to meet the unmet need for an easy and convenient glucagon administration. This review covers the basic information of nasal glucagon, trials on nasal glucagon in children and adults and its potential uses, limitations, and future scope in practice.
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