Poorly Controlled Congenital Hypothyroidism due to an Underlying Allgrove Syndrome

Tellingen, Vera van; Finken, Martijn J J; Israëls, Joël; Hendriks, Yvonne; Kamp, Gerdine A; Santen, Hanneke M van

doi:10.1159/000446604

Cited by 4 publications

(2 citation statements)

References 14 publications

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“…Similarly, gastric sleeve surgery and other bariatric surgery can cause malabsorption in some LT4-treated patients ( 49 ). Recently, systemic sclerosis and esophageal achalasia were found to cause severe hypothyroidism ( 50 , 51 ). Researchers hypothesized that tablets did not completely reach the stomach and that an inadequate dissolution phase was the main reason for malabsorption.…”

Section: Impaired Bioavailability and Malabsorption Of Oral Lt4 Tabletsmentioning

confidence: 99%

Levothyroxine: Conventional and Novel Drug Delivery Formulations

Liu

Zhang

et al. 2022

Endocrine Reviews

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Despite the fact that levothyroxine is one of the most prescribed medications in the world, its bioavailability has been reported to be impaired by many factors, including interfering drugs or foods and concomitant diseases, and persistent hypothyroidism with a high dose of levothyroxine is thus elicited. Persistent hypothyroidism can also be induced by noninterchangeability between formulations and poor compliance. To address these issues, some strategies have been developed. Novel formulations (liquid solutions and soft-gel capsules) have been designed to eliminate malabsorption. Some other delivery routes (injections, suppositories, sprays, and sublingual and transdermal administrations) are aimed at circumventing different difficulties in dosing, such as thyroid emergencies and dysphagia. Moreover, nanomaterials have been used to develop delivery systems for the sustained release of levothyroxine to improve patient compliance and reduce costs. Some delivery systems encapsulating nanoparticles show promising release profiles. In this review, we first summarize the medical conditions that interfere with the bioavailability of oral levothyroxine and discuss the underlying mechanisms and treatments. The efficacy of liquid solutions and soft-gel capsules are systematically evaluated. We further summarize the novel delivery routes for levothyroxine and their possible applications. Nanomaterials in the levothyroxine field are then discussed and compared based on their load and release profile. We hope the article provides novel insights into the drug delivery of levothyroxine.

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Section: Impaired Bioavailability and Malabsorption Of Oral Lt4 Tabletsmentioning

confidence: 99%

Levothyroxine: Conventional and Novel Drug Delivery Formulations

Liu

Zhang

et al. 2022

Endocrine Reviews

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“…Congenital hypothyroidism (CH) is the most treatable cause of mental retardation [ 1 ]. Thyroid hormones (THs) are essential in terms of neurogenesis, myelination, neuronal migration, and synaptogenesis.…”

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confidence: 99%

Necroptosis mediated by receptor interacting protein kinase 3 as critical player in experimental congenital hypothyroidism related neuronal damage

Duman¹

2021

North Clin Istanbul

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OBJECTIVE: Congenital hypothyroidism (CH) is literally described as congenital thyroid hormone imperfection. The primary objective of this research was to reveal the possible relation between receptor-acting protein kinase 3 (RIPK3) activity and neuronal damages in rat pups with CH. In addition, we evaluated the favorable impacts of 3.6-dibromo-α-([phenylamino] methyl)-9H-carbazole-9-ethanol (P7C3) reducing RIPK3 activity. METHODS: Adult rats were accordingly assigned into four groups: Group 1, which is called congenital hypothyroid; Group 2, which is called congenital hypothyroid administered P7C3; Group 3, called CH administered P7C3 and L-thyroxine; and Group 4, control group. RIPK3 level in plasma concentration and its expression in tissue was determined in all groups. RESULTS: Increased RIPK3 expressions were detected as high in the CH group when it is compared to the control group. Furthermore; the expressions in neuronal cytoplasm were found similar among Groups II and III. RIPK3 expressions in those two groups were relatively higher than in the control group. Most reacted parts of the brain were especially Purkinje cells in the cerebellum. CONCLUSION: It is concluded that there is excellent parallelism among damaged neurons and high RIPK3 activity in CH pathogenesis. P7C3 compounds may have a safeguarding impact on CH due to decreasing RIPK3 activity.

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Case report of a familial triple: a syndrome and review of the literature

et al. 2020

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Rationale: Triple-A syndrome, or Allgrove syndrome (AS), is a rare autosomal recessive disorder characterized by the alacrimia, achalasia, and adrenal insufficiency triad. Alacrimia usually starts at early infancy, while achalasia and adrenal insufficiency appear later during childhood or adulthood. Some patients may also present with the so-called Double-A syndrome (i.e., alacrimia and achalasia, or alacrimia and adrenal insufficiency); adrenal insufficiency usually represents a life-threatening event due to severe hypoglycemia. Many patients may also present other associated manifestations, such as neurological disorders. We describe, here, 2 sisters of non-consanguineous parents. Patient concerns: An 8-year-old girl was admitted to the Pediatric Care Unit of Parma after an episode characterized by seizure with loss of consciousness and generalized hypertonia lasting a few minutes. Her sister, a 6-year-old girl, presented with recurrent episodes of vomiting and failure to thrive. Diagnoses: Both children were investigated by laboratory tests, esophagogastroduodenoscopy, and imaging. The first patient had the complete triad of AS (alacrimia, achalasia, adrenal insufficiency), while the second one presented only alacrimia and achalasia. Both resulted from a mutation in the achalasia, addisonianism, alacrimia syndrome gene. Interventions: Both patients were treated with oral hydrocortisone for Addison disease, and with artificial tears in the first case. After many pneumatic endoscopic dilations and therapy with nifedipine, both patients underwent surgical Heller myotomy for achalasia. Outcomes: A rapid and favorable recovery to normal diet and with improvement of growth parameters was obtained. These cases are also compared with the literature data, reported in a brief review. Lessons: AS is a rare multisystemic disorder. The longer diagnosis is delayed, the greater extent to which this syndrome may be life-threatening, mainly because of hypoglycemia due to adrenal insufficiency. In AS, the red-flag symptom of alacrimia should instigate investigation for achalasia, Addison disease, and achalasia, addisonianism, alacrimia syndrome gene mutation.

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Poorly Controlled Congenital Hypothyroidism due to an Underlying Allgrove Syndrome

Cited by 4 publications

References 14 publications

Levothyroxine: Conventional and Novel Drug Delivery Formulations

Levothyroxine: Conventional and Novel Drug Delivery Formulations

Necroptosis mediated by receptor interacting protein kinase 3 as critical player in experimental congenital hypothyroidism related neuronal damage

Case report of a familial triple: a syndrome and review of the literature

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