Hypertrophic pyloric stenosis (HPS) is the most common gastrointestinal obstructive disease in infants which requires surgery. Typical presentation is at 4-6 weeks of life but can present very earlier or congenitally. Here we present 3 cases of HPS which presented in the first day of life with co-existing malrotation, annular pancreas, and duodenal atresia. The exact etiology of HPS is not fully understood, however, genetic and maternal factors, hormonal factors, abnormalities of various components of the pyloric muscle, growth factors, extracellular matrix elements, nerve and ganglion cells synapses, nerve supporting cells, neurotransmitters and interstitial cells, drugs, and feeding have been implicated. In the HPS concomitant with a distal obstruction, mean that these obstructions may role in pathogenesis of HPS or may exacerbate other factors to produce HPS in a very early age or congenitally. The purpose of this manuscript is to present three cases of HPS in the first day of life with other GI anomalies. We hypothesize that the etiology of some cases of HPS may be secondary to distal intestinal obstructions especially when it presents very early in life, or at least to concern a distal obstructions in this very early presentation.
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http://dx.doi.org/10.4103/0189-6725.109397 | DOI Listing |
Indian J Nucl Med
November 2024
Department of Nuclear Medicine, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Hepatopulmonary syndrome (HPS) is a rare pulmonary vascular complication of chronic liver disease characterized by dilatation of pulmonary capillaries leading to vascular shunting and systemic hypoxemia. Diagnosis of HPS requires documentation of intrapulmonary vasodilation (IPVD), the two most common imaging studies performed for the detection of IPVD include transthoracic contrast echocardiography (TTCE) and 99m-Tc-macroaggregated albumin scintigraphy (99mTc-MAA scan). TTCE has high sensitivity and thus, is the preferred initial investigation, while 99mTc-MAA scan is highly specific and plays an adjuvant role in diagnosis.
View Article and Find Full Text PDFJ Clin Neurosci
December 2024
Department of Neurosurgery, Gifu Medical University, 1-1 Yanagido, Gifu City, Gifu, Japan.
Background: Hyperperfusion syndrome (HPS) is one of the most serious complications after carotid artery stenting (CAS). Arterial spin labeling (ASL) is a noninvasive method for assessing cerebral perfusion. This study aimed to evaluate the utility of ASL compared to that of SPECT in evaluating changes in intracranial blood flow during the perioperative period of CAS.
View Article and Find Full Text PDFActas Dermosifiliogr
November 2024
Department of Pathology, Fundación Jiménez Díaz-IIS, Universidad Autónoma de Madrid, Madrid, España; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), España.
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma with indolent behavior, mostly present in women and associated with immunological diseases whose pathogenic background is still poorly understood. SPTCL is associated with lupus erythematosus panniculitis (LEP) and histologically misdiagnosed.
Objectives: The aim of our study was to identify mutations affecting the pathogenesis of both SPTCL and LEP.
S Afr Med J
August 2024
Discipline of Orthopaedic Surgery, Inkosi Albert Luthuli Central Hospital, University of KwaZulu-Natal, Durban, South Africa.
Background: Pathology of the hand causes functional impairment, with downstream effects for patient occupation, and consequently presents a socioeconomic burden. Investigation of the epidemiology of hand pathology in KwaZulu-Natal (KZN) can help reduce the burden of disease. Identifying where the greatest need is can direct patient awareness initiatives, medical training and appropriate allocation of resources.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
November 2024
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea.
Objective: Hepatopulmonary syndrome (HPS) is a rare complication of metabolic associated fatty liver disease (MAFLD) occurring subsequent to hypopituitarism, often developing after resection of hypothalamic or pituitary tumors. The aim of this study is to report an illustrative case of an HPS patient who was successfully treated with growth hormone replacement therapy, without liver transplantation which is conventionally regarded as the only treatment option. Additionally, we conducted a comprehensive review of published case reports of HPS in the pediatric population.
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