Unilateral electronegative ERG in a presumed central retinal artery occlusion

A unilateral electronegative electroretinogram (ERG) was seen in a 94-year-old man with presumed central retinal artery occlusion. Goldmann perimetry revealed central scotoma in the right eye and no abnormalities in the left eye. Full-field ERG in the right eye described a reduction of the b-wave with a relative preservation of the a-wave which is characteristic of electronegative ERG. Hence, our case illustrates that ERG testing is essential for the work-up of individuals with suspected retinal vascular disorders

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liège Acromegaly Survey (LAS) Database, a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; P 3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis

Unilateral electronegative ERG in a presumed central retinal artery occlusion

Luiz H Lima1, Wener Cella2,4, Claudia Brue5, Stephen H Tsang2.31Federal University of Sao Paulo (UNIFESP), Sao Paulo, Brazil; 2Department of Ophthalmology, 3Bernard and Shirlee Brown Glaucoma Laboratory, Edward S Harkness Eye Institute and Departments of Opthamology, Pathology and Cell Biology, Columbia University, New York, NY, USA; 4Department of Ophthalmology, University of Brasilia, Brasilia, Brazil; 5Department of Opthamology, University Politecnica delle Marche, Ancona, ItalyAbstract: A unilateral electronegative electroretinogram (ERG) was seen in a 94-year-old man with presumed central retinal artery occlusion. Goldmann perimetry revealed central scotoma in the right eye and no abnormalities in the left eye. Full-field ERG in the right eye described a reduction of the b-wave with a relative preservation of the a-wave which is characteristic of electronegative ERG. Hence, our case illustrates that ERG testing is essential for the work-up of individuals with suspected retinal vascular disorders.Keywords: central retinal artery occlusion, electronegative ERG, inner retina, spectral domain optical coherence tomograph

Pilot Neonatal Screening Program for Central Congenital Hypothyroidism: Evidence of Significant Detection

Background/Aim: Congenital hypothyroidism (CH) is a heterogeneous entity. Neonatal screening programs based on thyrotropin (TSH) determination allow primary CH diagnosis but miss central CH (CCH). CCH causes morbidity, alerts to other pituitary deficiencies, and is more prevalent than previously thought. We aimed at developing a pilot neonatal screening program for CCH detection. Patients and Methods: A prospective 2-year pilot neonatal screening study based on simultaneous dried blood specimen TSH and thyroxine (T4) measurements was implemented in term newborns aged 2-7 days. Those with T4 ≤4.5 μg/dL (-2.3 SDS) and TSH <10 mIU/L were recalled (suspicious of CCH) and underwent clinical and biochemical assessment performed by expert pediatric endocrinologists. Results: A total of 67,719 newborns were screened. Primary CH was confirmed in 24 (1: 2,821). Forty-four newborns with potential CCH were recalled (recall rate 0.07%) at a mean age of 12.6 ± 4.8 days. In this group, permanent CCH was confirmed in 3 (1: 22,573), starting L-T4 treatment at a mean age of 12.3 ± 6.6 days; 14 boys showed T4-binding globulin deficiency (1: 4,837); 24 had transient hypothyroxinemia (21 non-thyroidal illness and 3 healthy); and 3 died before the confirmation stage. According to initial free T4 measurements, CCH patients had moderate hypothyroidism. Conclusions: Adding T4 to TSH measurements enabled the identification of CCH as a prevalent condition and contributed to improving the care of newborns with congenital hypopituitarism and recognizing other thyroidal disorders.Fil: Braslavsky, Debora Giselle. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Centro de Investigaciones Endocrinológicas ; Argentina. Fundación de Endocrinología Infantil; ArgentinaFil: Méndez, María Virginia. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Fundación de Endocrinología Infantil; ArgentinaFil: Pérez Prieto, Laura María. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Fundación de Endocrinología Infantil; ArgentinaFil: Keselman, Ana Claudia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Centro de Investigaciones Endocrinológicas ; ArgentinaFil: Enacan, Rosa. Fundación de Endocrinología Infantil; ArgentinaFil: Gruñeiro Papendieck, Laura. Fundación de Endocrinología Infantil; ArgentinaFil: Jullien, Nicolas. Aix-Marseille Université; Francia. Centre National de la Recherche Scientifique; FranciaFil: Savenau, Alexandru. Aix-Marseille Université; Francia. Hôpital de la Conception; Francia. Centre National de la Recherche Scientifique; FranciaFil: Reynaud, Rachel. Aix-Marseille Université; Francia. Hôpitaux de Marseille; Francia. Centre National de la Recherche Scientifique; FranciaFil: Brue, Thierry. Aix-Marseille Université; Francia. Hôpital de la Conception; Francia. Centre National de la Recherche Scientifique; FranciaFil: Bergadá, Ignacio. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Fundación de Endocrinología Infantil; ArgentinaFil: Chiesa, Ana Elena. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Centro de Investigaciones Endocrinológicas ; Argentina. Fundación de Endocrinología Infantil; Argentin

Acromegaly at diagnosis in 3173 patients from the Liege Acromegaly Survey (LAS) database.

Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liege Acromegaly Survey (LAS) database, a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs. 46.4 years; p3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis