Results 31 to 40 of about 1,234,995 (195)

Idiopathic gastric perforation in an asplenic infant

open access: yesAfrican Journal of Paediatric Surgery, 2012
Introduction: The cause of idiopathic gastric perforations in neonates remains unknown. Perforations of the abdominal oesophagus, stomach and duodenum in infants and children are the rarest type of intestinal perforations.
Alex Olsen, Leigh McGill
doaj   +1 more source

Characterization of the clinical and genetic spectrum of autoimmune polyendocrine syndrome type 1 in Chinese case series

open access: yesOrphanet Journal of Rare Diseases, 2021
Background Autoimmune polyendocrine syndrome type 1 (APS1) is a hereditary disease caused by mutations in the AIRE gene with both endocrine and non-endocrine organ involvement. The existing data from China are limited, and this study aims to describe the
Ya-Bing Wang   +6 more
doaj   +1 more source

Non-cardiac issues in patients with heterotaxy syndrome

open access: yesAnnals of Pediatric Cardiology, 2014
Management of complex congenital heart disease in patients with Heterotaxy syndrome (HS) has steadily improved. However, there is an insufficient appreciation of various non-cardiac issues that might impact the overall status of these patients.
Shyam S Kothari
doaj   +1 more source

Clinical management of the acute complications of sickle cell anemia: 11 years of experience in a tertiary hospital

open access: yesAnales de Pediatría (English Edition), 2022
Introduction: Sickle cell disease is an emerging anemia in Europe leading to high morbidity with severe acute complications requiring hospital admission and chronic consequences.
Patricia Reparaz   +10 more
doaj   +1 more source

Manejo clínico de las complicaciones agudas de la anemia falciforme: 11 años de experiencia en un hospital terciario

open access: yesAnales de Pediatría, 2022
Resumen: Introducción: La drepanocitosis es una anemia emergente en Europa que condiciona una elevada morbilidad con complicaciones agudas y crónicas. El manejo de estos pacientes es complejo y requiere atención interdisciplinar. El objetivo del estudio
Patricia Reparaz   +10 more
doaj   +1 more source

Sickle Cell Disease: Historical Overview and Current Therapies

open access: yesPrenatal Diagnosis, EarlyView.
ABSTRACT Sickle cell disease (SCD) affects millions worldwide, yet the limited treatment options currently available do not always adequately control the disease and carry significant side effects. At present, the only curative treatment is hematopoietic stem cell (HSC) transplantation, a procedure that carries considerable challenges and numerous ...
Oluwaseun O. Babatunde   +4 more
wiley   +1 more source

Risk of invasive pneumococcal disease in patients with asplenia/hyposplenism: A nationwide population-based study in Korea, 2009-2018 [PDF]

open access: yes, 2020
Objectives: We aimed to determine the incidence and relative risk (RR) of invasive pneumococcal disease (IPD) in patients with asplenia/hyposplenism, using a nationwide population-based database.
한민경   +4 more
core   +1 more source

A multi-disciplinary, comprehensive approach to management of children with heterotaxy

open access: yesOrphanet Journal of Rare Diseases, 2022
Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left–right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left–right organizer early in embryogenesis ...
Thomas G. Saba   +13 more
doaj   +1 more source

Cisterna Chyli Dilation Is a Risk Factor for Liver Nodules and Hepatocellular Carcinoma in Fontan‐Associated Liver Disease: Pathophysiological Role of Lymphatic Dysfunction

open access: yesHepatology Research, EarlyView.
ABSTRACT Aim In Fontan‐associated liver disease (FALD), chronic congestion often confounds conventional fibrosis markers, complicating surveillance for hepatocellular carcinoma (HCC). Although lymphatic dysfunction is fundamental to Fontan physiology, its contribution to hepatocarcinogenesis remains unclear.
Koji Imoto   +14 more
wiley   +1 more source

Ablation of Twin Atrioventricular Node‐Mediated Macroreentrant Tachycardia in a Patient With Complex Heterotaxy Syndrome

open access: yesPacing and Clinical Electrophysiology, EarlyView.
ABSTRACT Twin atrioventricular (AV) nodes constitute a rare and highly complex electrophysiological substrate for tachycardia in heterotaxy syndrome (complex cardiac heart disease). We present the case of an 18‐year‐old male with right atrial isomerism, asplenia, univentricular physiology, and a Glenn shunt, who presented with a regular wide‐QRS ...
Rogelio Robledo‐Nolasco   +13 more
wiley   +1 more source

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