Introduction: Diabetic Keto acidosis (DKA) is the most common cause of mortality in children with diabetes mellitus. The aim of this study is to investigate the complications of DKA and its complications in hospitalized patients in Namazi hospital, Shiraz, Iran.
Background. Although thyroid nodules are a common finding in the general population, determining the clinically important nodules is essential. We investigated thyroid nodules or cysts by thyroid ultrasonography (US) in patients with β-thalassemia major (β-TM) and intermedia (β-TI). We also report a β-TI patient who was diagnosed with thyroid cancer six months before our screening. Methods. In this cross-sectional study, 178 patients with β-thalassemias referred to the Thalassemia Clinic in a tertiary hospital affiliated to Shiraz University of Medical Sciences were investigated, from January to June 2016, by US. Results. Thyroid nodules or cysts were detected in 11 patients [total: 6.17 %; 8 patients with β-TM (8.2%) and 3 patients with β-TI (3.7%)]. All nodules were < 1 cm in diameter and were not suspicious of malignancy. All patients, after 1 year of thyroid US follow-up, did not show any significant change in favor of malignancy. Conclusion. Based on our results, the frequency of thyroid nodules was similar to what was reported in the general population. However, a long-term follow-up of these patients is recommended because of the potential carcinogenic effects of iron and hepatitis C infection (HCV). To achieve more precise information, collaborative multicenter studies should be considered.
: Biallelic variants in the pancreas-specific transcription factor 1A (PTF1A) gene are a rare cause of permanent neonatal diabetes. We report a case of neonatal diabetes with unique clinical manifestations. The clinical diagnosis of the affected infant was confirmed by insufficient endocrine and exocrine pancreas activity; however, the pancreas was normal in imaging. Molecular analyses identified a novel homozygous single nucleotide variant (Chr10, g.23508441T > G), affecting a highly conserved nucleotide within a distal enhancer of the PTF1A gene. The literature review showed that most of these patients had IUGR and imaging evidence of pancreatic agenesis or hypoplasia. We suggest that pancreatic imaging and evaluation of exocrine pancreas function can help early confirmation of the diagnosis in patients with permanent neonatal diabetes.
Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population. The Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS) was established to assess cirrhotic children, the course of their disease, and treatment over time. This cohort study aimed to prospectively evaluate the natural course and factors that contributed to complications and death of children with chronic liver disease in the region. SPLCCS was launched in September 2018 after obtaining ethical approval; until August 2022, 370 children with end-stage liver disease were enrolled and followed every six months. Here, the cohort’s features, the included population’s baseline characteristics, and primary outcomes are reported.
Key Clinical Message
Adrenal insufficiency is a rare, important manifestation of secondary antiphospholipid syndrome (APS) in pediatrics. In the presence of hematologic disorders such as thrombosis, we should consider APS.
Abstract
Adrenal insufficiency can rarely occur in the context of vascular disorders and thrombosis in patients with antiphospholipid syndrome. There are few case reports in pediatrics. Here, we present a pediatric case—the first pediatric case report in Iran—and review articles in this age group.
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