Biometric and corneal characteristics in marfan syndrome with ectopia lentis.

PURPOSE: To describe the biometric and corneal characteristics of patients with Marfan Syndrome (MFS) and ectopia lentis. STUDY DESIGN: Observational, descriptive, prospective study. Subjects Individuals with MFS with ectopia lentis (EL). METHODS: Fourty-four eyes of 23 patients underwent Scheimpflug analysis using the Pentacam (Oculus, Wetzlar, Germany), axial length (AL) using the IOL master 700 (Carl Zeiss AG, Oberkochen, Germany), endothelial cell count (ECC) using the CEM-350 (NIDEK, Maihama, Japan) and corneal biomechanics evaluation with the Ocular Response Analyzer: ORA (Reichert Ophthalmic Instruments, Buffalo, New York, USA) and Corvis (Oculus, Wetzlar, Germany). Statistical analysis was performed using IBM SPSS Statistics 25.0. RESULTS: The direction of lens subluxation was most frequently supero-nasal 40.9% (18/44). Mean keratometry (Km) was 40.22±1.76 Diopters (D); mean corneal astigmatism was 1.68±0.83 D; total corneal aberrometric root mean square (RMS) was 2.237±0.795µm; higher-order aberrations (HOAs) RMS were 0.576±0.272µm; mean AL was 25.63±3.65mm; mean ECC was 3315±459cell/mm2; mean CBI was 0.13±0.24, mean TBI was 0.31±0.25, mean posterior elevation was 4.3±4.5µm; mean total corneal densitometry was 16.0±2.14 grayscale units (GSU). CONCLUSION: Increased axial length, flatter and thicker corneas with higher regular astigmatism, normal densitometry, normal corneal biomechanical indices and normal posterior elevation were observed in Marfan patients with EL.

Arcuate nucleus neuropeptide coexpression and connections to gonadotrophin-releasing hormone neurones in the female rhesus macaque.

The underlying hypothalamic neurocircuitry by which metabolism and feeding regulates reproductive function has been well-studied in the rodent; however, recent data have demonstrated significant neuroanatomical differences in the human brain. The present study had three objectives, centred on arcuate nucleus neuropeptides regulating feeding and reproduction: (i) to characterise coexpression patterns in the female nonhuman primate; (ii) to establish whether these neuronal populations make potential contacts with gonadotophin-releasing hormone (GnRH) neurones; and (iii) to determine whether these contacts differ between the low and high GnRH-releasing states of pre-puberty and adulthood, respectively. Female nonhuman primates have several coexpression patterns of hypothalamic neuropeptides that differ from those reported in rodents. Cocaine- and amphetamine-regulated transcript (CART) is not coexpressed with pro-opiomelanocortin but instead with neuropeptide Y (NPY). CART is also expressed in a subpopulation of kisspeptin cells in the nonhuman primate, similar to observations in humans but diverging from findings in rodents. Very few GnRH-expressing neurones received close appositions from double-labelled kisspeptin/CART fibres; however, both single-labelled kisspeptin and CART fibres were in frequent apposition with GnRH neurones, with no differences between prepubertal and adult animals. NPY/agouti-related peptide (AgRP) coexpressing fibres contacted significantly more GnRH neurones in prepubertal animals than adults, consistent with increased NPY and AgRP mRNA observed in prepubertal animals. The findings of the present study detail significant differences in arcuate nucleus neuropeptide coexpression in the monkey compared to the rodent and are consistent with the hypothesis that arcuate nucleus NPY/AgRP neurones play an inhibitory role in controlling GnRH neuronal regulation in the prepubertal primate.