keyword
https://read.qxmd.com/read/38510581/utility-of-pyloric-length-measurement-for-detecting-severe-metabolic-alkalosis-in-infants-with-hypertrophic-pyloric-stenosis
#1
JOURNAL ARTICLE
Hyun Jin Kim
PURPOSE: Infantile hypertrophic pyloric stenosis (IHPS) is a common gastrointestinal disease in neonates and hypochloremia metabolic alkalosis is a typical laboratory finding in affected patients. This study aimed to analyze the clinical characteristics of infants with IHPS and evaluate the association of clinical and laboratory parameters with ultrasonographic findings. METHODS: Infants diagnosed with IHPS between January 2017 and July 2022 were retrospectively evaluated...
March 2024: Pediatric Gastroenterology, Hepatology & Nutrition
https://read.qxmd.com/read/38229209/citrate-anticoagulation-in-plasma-exchange-followed-by-continuous-renal-replacement-therapy-in-critically-ill-children
#2
JOURNAL ARTICLE
Zhang Xinping, He Jie, Yao Zhenya, Zhu Desheng, Zhou Xiong
OBJECTIVE: To investigate the effectiveness and safety of regional citrate-anticoagulated (RCA) plasma exchange (PE) and whether citrate-related metabolic disorders can be improved by sequential RCA continuous renal replacement therapy (CRRT). METHODS: This retrospective, single-center observational study included 79 critically ill children requiring PE followed by CRRT (June 2018 to June 2021) at the Pediatric Intensive Care Unit of Hunan Children's Hospital, China...
January 16, 2024: International Journal of Artificial Organs
https://read.qxmd.com/read/38165475/a-previously-healthy-3-year-old-female-with-hypertension-proteinuria-and-hypercalciuria
#3
JOURNAL ARTICLE
Tao Liu, Wenhong Wang, Zhufeng Liu, Guanghua Pei, Chunxiang Wang, Ying Jiang, Chuyue Pang
A 3-year-old female patient with no significant medical history presented to her pediatrician with foamy urine. Initial testing revealed moderate proteinuria on qualitative testing, although she was incidentally noted to have severe hypertension (240/200 mmHg). Physical examination of the carotid and femoral areas revealed significant systolic vascular murmurs. Labs showed elevated serum creatinine, hypokalemia, metabolic alkalosis, elevated renin and aldosterone and hypercalciuria. Echocardiography identified ventricular hypertrophy...
January 2, 2024: Pediatric Nephrology
https://read.qxmd.com/read/38069462/long-term-indomethacin-treatment-in-a-chinese-child-with-gitelman-syndrome-case-report-and-literature-review-on-its-efficacy-and-tolerance
#4
JOURNAL ARTICLE
Xiaoyan Peng, Chaoying Chen, Juan Tu, Yuan Lin, Huarong Li, Haiyun Geng
BACKGROUND Gitelman syndrome (GS) is a rare inherited autosomal recessive salt-losing renal tubulopathy. Early-onset GS is difficult to differentiate from Bartter syndrome (BS). It has been reported in some cases that cyclooxygenase (COX) inhibitors, which pharmacologically reduce prostaglandin E2(PGE2) synthesis, are helpful for GS patients, especially in children, but the long-term therapeutic effect has not yet been revealed. CASE REPORT A 4-year-old boy was first brought to our hospital for the chief concern of short stature and growth retardation...
December 9, 2023: American Journal of Case Reports
https://read.qxmd.com/read/38052189/a-pediatric-case-of-reninoma-presenting-with-paraneoplastic-siadh
#5
Sarpreet Singh Sekhon, Khalid Taha, Laura Kim, Robert Humphreys, Trisha J Patel, Alicia R Andrews, Anna F Lee, Fatema S Abdulhussein
INTRODUCTION: A reninoma (juxtaglomerular cell tumour) is a rare cause of secondary hypertension that can present with headaches alongside the triad of severe hypertension, hypokalemia, and metabolic alkalosis. CASE PRESENTATION: We describe a case of a 15-year-old previously healthy girl who presented with headaches and hypertensive urgency who had severe hypokalemia, moderate hyponatremia and elevated aldosterone and renin levels. Abdominal ultrasound and MRI with contrast revealed a unilateral mass localized to the right kidney...
December 5, 2023: Hormone Research in Pædiatrics
https://read.qxmd.com/read/37970141/chloride-reduction-therapy-with-furosemide-short-term-effects-in-children-with-acute-respiratory-failure
#6
JOURNAL ARTICLE
Hisataka Nozawa, Norihiko Tsuboi, Tadashi Oi, Yoshiki Takezawa, Ichiro Osawa, Nao Nishimura, Satoshi Nakagawa
From the perspective of the Stewart approach, it is known that expansion of the sodium chloride ion difference (SCD) induces alkalosis. We investigated the role of SCD expansion by furosemide-induced chloride reduction in pediatric patients with acute respiratory failure. We included patients admitted to our pediatric intensive care unit intubated for acute respiratory failure without underlying diseases, and excluded patients receiving extracorporeal circulation therapy (extracorporeal membrane oxygenation and/or renal replacement therapy)...
December 2023: Journal of Pediatric Intensive Care
https://read.qxmd.com/read/37909881/hereditary-causes-of-hypertension-due-to-increased-sodium-transport
#7
JOURNAL ARTICLE
Jinwei Zhang
PURPOSE OF REVIEW: Hypertension, commonly known as high blood pressure, is a widespread health condition affecting a large number of individuals across the globe. Although lifestyle choices and environmental factors are known to have a significant impact on its development, there is growing recognition of the influence of genetic factors in the pathogenesis of hypertension. This review specifically focuses on the hereditary causes of hypertension that are associated with increased sodium transport through the thiazide-sensitive NaCl cotransporter (NCC) or amiloride-sensitive epithelial sodium channel (ENaC), crucial mechanisms involved in regulating blood pressure in the kidneys...
November 2, 2023: Current Opinion in Pediatrics
https://read.qxmd.com/read/37842418/uncommon-presentation-of-cystic-fibrosis-a-case-report-and-literature-review
#8
Majed Abu Sirhan, Michael Kalinin, Lior Cohen, Evgenia Gurevich
Cystic fibrosis (CF) is a multiorgan disease, caused by autosomal recessive (AR) mutations in the cystic fibrosis transmembrane regulator (CFTR) acting primarily as a chloride channel. CF is most commonly diagnosed in Caucasian populations. Common clinical presentations in pediatric patients include chronic cough, respiratory tract infections such as pneumonia, digestive symptoms, and stunted growth, and malnutrition due to gastrointestinal malabsorption and pancreatic insufficiency. Excessive sweat sodium chloride losses due to dysfunctional sweat glands in CFTR result in volume contraction and secondary hyperaldosteronism leading to renal potassium losses and metabolic alkalosis...
September 2023: Curēus
https://read.qxmd.com/read/37725210/cystic-fibrosis-and-cftr-related-disorder-with-electrolyte-imbalance-at-diagnosis-clinical-features-and-outcome-in-an-italian-cohort
#9
JOURNAL ARTICLE
Vito Terlizzi, Rita Padoan, Giuseppina Leonetti, Pamela Vitullo, Antonella Tosco, Giovanni Taccetti, Cristina Fevola, Francesca Ficili, Angela Pepe, Piercarlo Poli, Laura Claut, Valeria Daccò, Donatello Salvatore
UNLABELLED: There is limited information available on the clinical data, sweat test trends, and outcomes of individuals with cystic fibrosis (CF) who present with an isolated episode of hypoelectrolytemia with metabolic alkalosis (HMA). This study describes a cohort of Italian individuals with HMA as presenting symptom. The study is a retrospective multicenter analysis of individuals who presented with HMA as an initial symptom and was followed at 8 Italian CF Centers, from March 1988 to March 2022...
September 19, 2023: European Journal of Pediatrics
https://read.qxmd.com/read/37587715/a-novel-homozygous-clcnkb-variant-an-early-presentation-of-classic-bartter-syndrome-in-a-neonate
#10
Deniz Yaprak, Hüdaverdi Kara, Erhan Calisici, Belma Saygılı Karagöl, Mustafa Altan
BACKGROUND: Bartter syndrome (BS) is a rare congenital salt-losing renal tubular transport disorder, characterized by salt wasting, polyuria, biochemical abnormalities, and acid-base homeostasis imbalance. The syndrome has five different genetic forms, and novel mutations of CLCNKB gene lead to type 3 BS also known as classic BS. In this case, we report clinical and molecular findings from a newborn baby with BS. CASE: A 10-day-old male infant born at 37 weeks of gestation by cesarean section following a pregnancy complicated with polyhydramnios, and fetal distress to a 30-year-old gravida 3, para 3 mother, with a 2500 g birth weight was brought to the pediatric emergency department due to weight loss and jaundice...
October 15, 2023: Birth Defects Research
https://read.qxmd.com/read/37362166/pseudo-bartter-syndrome-in-an-infant-without-obvious-underlying-conditions-a-case-report
#11
Junya Toyoda, Masanori Adachi, Ayako Ochi, Yuki Okada, Aiko Honda, Katsumi Mizuno, Kandai Nozu
Pseudo-Bartter syndrome (PBS) develops owing to renal or extrarenal chloride loss, leading to hypokalemic alkalosis. Whereas most adult cases result from diuretic/laxative abuse, many infantile cases occur secondary to cystic fibrosis. Rarely, infantile PBS is caused by renal salt loss with anomalies of the kidney/urinary tract or genetic disorders, such as Dent disease. Here, we report the case of a 10-mo-old girl with a one-month history of decreased formula intake and 5.6% body weight loss. She showed typical laboratory findings as PBS, including hypokalemia (2...
2023: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://read.qxmd.com/read/37157788/does-presenting-facility-and-race-impact-outcomes-and-management-of-pyloric-stenosis
#12
JOURNAL ARTICLE
Cory Nonnemacher, Joshua Glenn, Hannah Moore
Hypertrophic Pyloric Stenosis (HPS) is a common surgical disease in infants. Traditionally, patients present with projectile emesis and severe dehydration with metabolic alkalosis. We looked to assess if patients presenting as a transfer vs directly to our facility as well as race affected patients' initial presentation and outcomes. We performed a retrospective analysis of 131 patients who presented to with a diagnosis of HPS from 2015 to 2021 assessing how transfer status and patient race affected presenting electrolyte levels and length of stay (LOS)...
May 8, 2023: American Surgeon
https://read.qxmd.com/read/37138571/a-mosaic-mutation-in-the-clcnkb-gene-causing-bartter-syndrome-a-case-report
#13
Lan Zhou, Xiaohui Chen, Jiaojiao Xiong, Ling Lei
BACKGROUND: Type III Bartter syndrome (BS) is an autosomal recessive disease caused by mutations in the CLCNKB (chloride voltage-gated channel Kb) gene that encodes CLC-Kb. CLC-Kb is mainly located in the thick ascending limb of Henle's loop and regulates chloride efflux from tubular epithelial cells to the interstitium. Type III BS is characterized by metabolic alkalosis, renal salt wasting, hyperreninemia, and hyperaldosteronism with normal blood pressure. CASE PRESENTATION: We reported the case of a 3-day-old girl whose initial symptom we diagnosed as jaundice, but we accidentally found metabolic alkalosis...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/37063660/bartter-syndrome-type-iii-with-glomerular-dysplasia-and-chronic-kidney-disease-a-case-report
#14
REVIEW
Jingqi Liu, Yudi Zhang, Xiaochuan Wu, Yongzhen Li
BACKGROUND: Bartter syndrome (BS) type III is a rare autosomal recessive genetic disease. Its clinical features are polyuria, hypokalemia, hypochloremia, metabolic alkalosis, and hyperreninaemia. A few BS type III can be complicated with chronic kidney disease. CASE PRESENTATION: We report a 14-year-old boy with Bartter syndrome caused by a c.1792C > T ( p .Q598*) mutation in the CLCNKB gene. He was a no deafness and full-term baby, and he had renal dysplasia and chronic kidney disease (CKD)...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/36729016/juxtaglomerular-cell-tumor-reninoma-as-a-cause-of-arterial-hypertension-in-adolescents-a-case-report
#15
JOURNAL ARTICLE
Luis A Pompozzi, Adriana Iturzaeta, María I Deregibus, Silvina Steinbrun, María Del Valle Centeno
Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.
March 2, 2023: Archivos Argentinos de Pediatría
https://read.qxmd.com/read/36709934/-clinical-characteristics-and-genetic-analysis-of-three-children-with-congenital-chlorine-diarrhea
#16
JOURNAL ARTICLE
Hui Yin, Xiaobo Chen, Fuying Song, Hui Wang, Mu Du, Ye Qian, Shuyue Huang
OBJECTIVE: To explore the clinical characteristics and genetic basis for three children with Congenital chlorine diarrhea (CCD). METHODS: Three children with CCD who attended the Affiliated Children's Hospital of Capital Pediatric Institute from June 2014 to August 2020 were selected as the research subjects. Peripheral blood samples of the three children and their parents were collected for genetic testing. And the results were verified by Sanger sequencing. RESULTS: The clinical manifestations of the three children have included recurrent diarrhea, with various degrees of hypochloremia, hypokalemia and refractory metabolic alkalosis...
February 10, 2023: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://read.qxmd.com/read/36688207/bartter-syndrome-perspectives-of-a-pediatric-nephrologist
#17
REVIEW
Naye Choi, Hee Gyung Kang
Bartter syndrome (BS) is one of the most well-known hereditary tubular disorders, characterized by hypokalemic, hypochloremic metabolic alkalosis, and polyuria/polydipsia. This disease usually presents before or during infancy, and adult nephrologists often inherit the patients from pediatric nephrologists since this is a life-long condition. Here, a few case scenarios will be presented to recount how they first got diagnosed and how their clinical courses were during childhood until adulthood, in addition to a brief review of the disease and its treatment...
December 2022: Electrolyte & Blood Pressure: E & BP
https://read.qxmd.com/read/36589503/multimodal-imaging-approach-in-hyponatremic-hypertensive-syndrome-a-rare-case-of-pediatric-unilateral-hypoplasia-of-the-main-renal-artery-combined-itself-with-stenosis-and-review-of-literature
#18
Angela Siervo, Anna Castaldo, Daniela Furlan, Dolores Ferrara, Eugenio Rossi, Domenico Noviello, Massimo Zeccolini, Francesco Esposito
Renal artery stenosis (RAS) accounts for approximately 5%-10% of secondary renovascular hypertension in the pediatric population. It can occur as an isolated entity, or as a hypoplasia combined itself with stenosis. Hypoplasia, or long-segment developmental narrowing, is a rare cause of renovascular hypertension. Hyponatremic hypertensive syndrome (HHS) is a malignant complication of unilateral RAS and/or renal artery hypoplasia. Hyponatremia, hypokalemic hypochloremic metabolic alkalosis, nephrotic range proteinuria, polyuria, polydipsia, and weight loss are the most common findings...
March 2023: Radiology Case Reports
https://read.qxmd.com/read/36553259/diagnostic-accuracy-of-acid-base-status-in-infants-with-hypertrophic-pyloric-stenosis
#19
JOURNAL ARTICLE
Marko Bašković, Dorotea Sinjeri
BACKGROUND: Hypertrophic pyloric stenosis is a condition in newborns in which the hypertrophic pyloric muscle causes gastric obstructive symptoms of progressive vomiting leading to hypochloremic hypokalemic metabolic alkalosis. The main aim of the research was to assess whether, based on the acid-base status, we can distinguish newborns who vomit due to pylorostenosis, compared with newborns who vomit for other unspecific reasons. METHODS: The electronic records of patients in the hospital information system treated under the diagnosis Q40...
November 24, 2022: Children
https://read.qxmd.com/read/36475052/postoperative-apnea-after-pyloromyotomy-for-infantile-hypertrophic-pyloric-stenosis
#20
JOURNAL ARTICLE
Anna Camporesi, Veronica Diotto, Elena Zoia, Simone Rotta, Federica Tarantino, Laura Maria Giuditta Eccher, Valeria Calcaterra, Gloria Pelizzo, Marco Gemma
OBJECTIVE: Infantile hypertrophic pyloric stenosis (IHPS), which causes gastric outlet obstruction and hypochloremic hypokalemic metabolic alkalosis, could pose a risk of postoperative apnea in patients. The aim of this study is to evaluate the incidence of postoperative apnea in babies admitted to a tertiary-level pediatric surgical center in Milano, Italy with diagnosis of IHPS in 2010-2019. The secondary objective is to evaluate the risk factors for postoperative apnea. METHODS: This is a single-center, retrospective, observational cohort study...
2022: World journal of pediatric surgery
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