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EMI – investigations in psychiatry
Published in Bhaskar Punukollu, Michael Phelan, Anish Unadkat, MRCPsych Part 1 In a Box, 2019
Bhaskar Punukollu, Michael Phelan, Anish Unadkat
3. D, H: Syndrome of Inappropriate Secretion of ADH (SIADH). SIADH may be caused by: oat cell carcinoma of the lung; COAD; pancreatic cancer; Hodgkin’s disease; CNS disorders – cerebral abscess, meningitis, encephalitis; myxoedema; certain drugs – antidepressants, neuroleptics, carbamazepine, diuretics, oral hypoglycaemic agents. Investigations reveal: low serum Na; elevated urine Na; low serum osmolality; high urine osmolality (highly concentrated urine).
Incidence, evolution and risk factors of hypophosphatemia in patients with solid tumors receiving ferric carboxymaltose: a retrospective cohort study
Published in Acta Clinica Belgica, 2023
Alexander Decruyenaere, Koen Kortbeek, Sigurd Delanghe, Sylvie Rottey, Hannelore Denys, Lore Lapeire
Hypophosphatemia due to FCM is mediated by renal phosphate wasting, but the exact mechanism is incompletely understood. FCM is hypothesized to inhibit the proteolytic cleavage of the phosphate-regulating hormone fibroblast growth factor-23 (FGF23), thereby increasing circulating levels of intact FGF23 and triggering a cascade that is sometimes referred to as the ‘6 H-syndrome’ (hypophosphatemia, high FGF23, high urinary phosphate excretion, hypocalcitriolemia, hypocalcemia, and secondary hyperparathyroidism) [13,15]. Risk factors include lower serum phosphorus at baseline, more severe iron deficiency, and preserved kidney function [13,19]. Cancer patients could be even more prone to develop hypophosphatemia after receiving FCM since they already have pre-existing risk factors for hypophosphatemia, such as use of bone-modifying agents (including bisphosphonates and denosumab), administration of chemotherapy affecting the proximal renal tubular function, or malnutrition [20].
Enzyme replacement combinational therapy: effective treatments for mucopolysaccharidoses
Published in Expert Opinion on Biological Therapy, 2021
Azam Safary, Hakimeh Moghaddas-Sani, Mostafa Akbarzadeh-Khiavi, Alireza Khabbazzi, Mohammad A. Rafi, Yadollah Omidi
Recombinant laronidase (IDUA, Aldurazyme®, glycosaminoglycan a-L-iduronohydrolase, EC: 3.2.1.76) produced in CHO cells was the first enzyme approved for ERT of the MPS IH, which has been in clinical use since 2003 [67]. Laronidase is a glycoprotein with a molecular weight of approximately 83 kDa composed of 628 amino acids, which possesses six N-linked oligosaccharide modification sites. The internalization and trafficking of laronidase into the lysosomes are mediated by specific targeting of M6PRs through the M6P residues [68]. Deficiency of α-L-iduronidase hydrolase might lead to a progressive accumulation of GAGs, DS, and HS in all organs and tissues [69]. The H syndrome, as the most severe form of MPS I, typically show cognitive impairment, rapid disease progression, and early mortality during childhood. The H/S and S syndromes are the intermediate forms of MPS I, generally showing milder or no cognitive involvement with minor somatic symptoms, and slower disease progression [32,48]. The IV infusion of laronidase with 0.58 mg/kg per week provides the best results with a near-maximum reduction in GAG levels in urine [11,68]. Preclinical studies in MPS I canine models have introduced a direct enzyme administration via intrathecal (IT) injection. As an alternative successful method, the IT injection has resulted in extremely high enzyme concentrations in the brain, spinal cord, and spinal meninges, a substantial reduction of lysosomal storage, and improved treatment of brain diseases [70–72]. Besides, a phase I clinical trial was performed to treat patients with MPS I (≥8 years old) with symptomatic spinal cord compression due to thickened spinal meninges, by IT administration of laronidase into the lumbar spinal tap. All patients exhibited improvement in symptomatology in neurological examinations [73]. Substantial information on the efficacy and safety of IT-ERT in MPS I patients can be found at the registered trials on ClinicalTrials.gov database (http://clinicaltrials.gov), including NCT02232477 and NCT00638547.