The management of renal candidiasis in the newborn

Renal candidiasis in the neonate is encountered Infrequently. We report a newborn with ichthyosis, who during the hospital course had five episodes of culture-proven sepsis, probably due to skin lesions. For these infections various antibiotic combinations were used. During the therapy of the last sepsis attack, unilateral hydronephrosis developed secondary to renal candidiasis. Percutaneous nephrostomy with amphotericin B irrigation, coupled with five weeks of intravenous amphotericin B therapy was successful. We believe that with this approach the mortality and morbidity of renal candidiasis could be reduced

Modeling Psychomotor Retardation using iPSCs from MCT8-Deficient Patients Indicates a Prominent Role for the Blood-Brain Barrier

Inactivating mutations in the thyroid hormone (TH) transporter Monocarboxylate transporter 8 (MCT8) cause severe psychomotor retardation in children. Animal models do not reflect the biology of the human disease. Using patient-specific induced pluripotent stem cells (iPSCs), we generated MCT8-deficient neural cells that showed normal TH-dependent neuronal properties and maturation. However, the blood-brain barrier (BBB) controls TH entry into the brain, and reduced TH availability to neural cells could instead underlie the diseased phenotype. To test potential BBB involvement, we generated an iPSC-based BBB model of MCT8 deficiency, and we found that MCT8 was necessary for polarized influx of the active form of TH across the BBB. We also found that a candidate drug did not appreciably cross the mutant BBB. Our results therefore clarify the underlying physiological basis of this disorder, and they suggest that circumventing the diseased BBB to deliver active TH to the brain could be a viable therapeutic strategy