keyword
https://read.qxmd.com/read/37705405/refeeding-syndrome-in-pediatric-age-an-unknown-disease-a-narrative-review
#21
REVIEW
Antonio Corsello, Chiara Maria Trovato, Valeria Dipasquale, Giulia Bolasco, Flavio Labriola, Frédéric Gottrand, Elvira Verduci, Antonella Diamanti, Claudio Romano
Refeeding syndrome (RS) is characterized by electrolyte imbalances that can occur in malnourished and abruptly refed patients. Typical features of RS are hypophosphatemia, hypokalemia, hypomagnesemia, and thiamine deficiency. It is a potentially life-threatening condition that can affect both adults and children, although there is scarce evidence in the pediatric literature. The sudden increase in food intake causes a shift in the body's metabolism and electrolyte balance, leading to symptoms such as weakness, seizures, and even heart failure...
December 1, 2023: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/37638982/kidney-manifestations-of-pediatric-sj%C3%A3-gren-s-syndrome
#22
JOURNAL ARTICLE
Saverio La Bella, Marina Vivarelli, Armando Di Ludovico, Giulia Di Donato, Francesco Chiarelli, Luciana Breda
Approximately 1% of all patients with Sjögren's syndrome (SS) are children. Unlike the adult form, in which sicca syndrome is the main presentation, in children, the most common clinical finding is recurrent enlargement of the salivary glands. In pediatric SS, extraglandular manifestations represent a significant feature and, among these, kidney manifestations are relevant. Kidney involvement is observed in 5-20.5% of children with SS, most frequently tubulointerstitial nephritis. This injury can lead to serious phenotypes, including distal kidney tubular acidosis with the development of severe hypokalemia, which can lead to ECG abnormalities, weakness, and hypokalemic periodic paralysis...
August 28, 2023: Pediatric Nephrology
https://read.qxmd.com/read/37634509/pediatric-macrocorticotropinoma-do-they-differ-from-microcorticotropinoma
#23
JOURNAL ARTICLE
Chethan Yamichannaiah, Saba Samad Memon, Vijaya Sarathi, Anurag Ranjan Lila, Shilpa Sankhe, Sneha Arya, Manjiri Karlekar, Virendra Ashokrao Patil, Nalini Shah, Tushar Bandgar
INTRODUCTION: Cushing's disease (CD) due to macrocorticotropinoma (MC) in children and adolescents is a rare entity with limited information regarding its characteristics. The objective of the study is to describe the clinical, biochemical, imaging, management, outcome and genetic characteristics of children and adolescents with CD due to MC and compare them with those of microcorticotropinoma (mc). METHODS: This retrospective study was conducted in a single tertiary care center...
August 25, 2023: Neuroendocrinology
https://read.qxmd.com/read/37602070/massive-chronic-hypernatremia-associated-with-failure-to-thrive-in-a-pediatric-patient
#24
Jack A Tellerday, Vincent Calleo
Hypernatremia is a severe, potentially life-threatening condition that can manifest with altered mental status, coma, seizure, and even death. Values above 190 mmol/L are seldom reported in young pediatric patients and often have poor outcomes. We present a case of severe chronic hypernatremia secondary to failure to thrive (FTT) in a toddler, which led to significant pathology including bilateral metabolic strokes. A 21-month-old female was found unresponsive and brought to the hospital. The patient's childhood was complicated by prematurity, poor weight gain, and persistent postprandial emesis...
July 2023: Curēus
https://read.qxmd.com/read/37597047/evaluation-of-the-efficacy-and-associated-complications-of-regional-citrate-anticoagulation-in-neonates-experience-from-a-fourth-level-neonatal-intensive-care-unit
#25
JOURNAL ARTICLE
Yasemin Ezgi Köstekci, Tanıl Kendirli, Emrah Gün, Hacer Uçmak, Ferhan Demirtaş, Merve Havan, Engin Köse, Emel Okulu, Fatma Tuba Eminoğlu, Ömer Erdeve, Begüm Atasay, Saadet Arsan
UNLABELLED: Continuous kidney replacement therapy (CKRT) use has increased in recent years, but anticoagulation is a challenge for neonates. Regional citrate anticoagulation (RCA) is rarely preferred in neonates because of citrate accumulation (CA) and metabolic complications. We aimed to demonstrate the efficacy and safety of RCA in neonates. We retrospectively analyzed the medical records of 11 neonates treated with RCA-CKRT between 2018 and 2023. The initial dose of RCA was 2.1-3 mmol/l, and then, its dose was increased according to the level of ionized calcium (iCa+2 ) in the circuit and patients...
August 19, 2023: European Journal of Pediatrics
https://read.qxmd.com/read/37526697/arrhythmias-and-laboratory-abnormalities-after-an-electrical-accident-a-single-center-retrospective-study-of-333-cases
#26
JOURNAL ARTICLE
Miroslava Seyfrydova, Richard Rokyta, Daniel Rajdl, Michal Huml
BACKGROUND: Even though electrical injuries are common in the emergency room, guidelines, consensus, and general recommendations for the management of these patients do not exist in Europe. Documented cases of delayed arrhythmias are rare and their connection with electrical injury has not been fully confirmed. We also use cardio-specific markers for the risk stratification of myocardial injury, but there is no significant study referring to their utility in this clinical situation. These reasons led us to retrospectively analyze all cases of electrical injuries over 23 years to determine the prevalence of cardiac arrhythmias (mainly malignant arrhythmias and delayed arrhythmias)...
August 1, 2023: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://read.qxmd.com/read/37417032/psychosis-following-caffeine-consumption-in-a-young-adolescent-review-of-case-and-literature
#27
Qadri Adebayo Adeleye, Chukwuma Melitus Attama, Odunanimen Egbeobauwaye, Onuorji Angela
Caffeine, a popular over-the-counter methylxanthine, is widely consumed for its potent psychoactive properties. Toxicity generally occurs with intentional overdose and is often multisystemic and life-threatening. Consumption by children is rarely planned, and safe doses are potentially toxic in them. A 12-year-old boy whose parents had denied him coffee on several occasions eventually had access to it. The caffeine dose ingested was sub-toxic although he developed severe and life-threatening multisystemic caffeinism...
2023: Annals of African Medicine
https://read.qxmd.com/read/37415038/distal-renal-tubular-acidosis-as-presenting-manifestation-of-wilson-disease-in-a-11-year-old-girl
#28
JOURNAL ARTICLE
Paraselli Saiteja, Sriram Krishnamurthy, Bobbity Deepthi, Sudarsan Krishnasamy, Madhileti Sravani
A 11-year-old girl was referred to the pediatric nephrology services of our hospital for evaluation of vitamin-D-refractory rickets. She was born to second-degree consanguineous parents. On examination, she had wrist widening and bilateral genu varum. She had normal anion gap metabolic acidosis, hypokalemia, and hyperchloremia. The fractional excretion of bicarbonate was 3% and the urine anion gap was positive. She also had hypercalciuria, but no phosphaturia, glucosuria or aminoaciduria. In view of a family history of an elder sister having rigidity with cognitive and speech impairment, an ophthalmic evaluation by slit lamp examination was performed in the index case that revealed bilateral Kayser-Fleischer rings...
July 6, 2023: CEN Case Reports
https://read.qxmd.com/read/37408591/clinical-epidemiology-features-and-risk-factors-for-acute-diarrhea-caused-by-rotavirus-a-in-vietnamese-children
#29
JOURNAL ARTICLE
Dang Van Chuc, Dang Phuong Linh, Dang Viet Linh, Pham Van Linh
INTRODUCTION: Acute diarrhea caused by group A rotavirus (RVA) is a leading cause of morbidity and mortality globally in children less than 5 years old. Acute diarrhea caused by RVA is often manifested by loose/watery stool leading to different degrees of dehydration. The detection of risk factors, diagnosis, and prompt treatment of acute diarrhea caused by RVA is critical. We aimed to describe clinical epidemiological features of acute diarrhea caused by RVA and its associated risk factors...
2023: International Journal of Pediatrics
https://read.qxmd.com/read/37391630/hypokalemia-hyperkalemia-and-hyponatremia-hypernatremia
#30
JOURNAL ARTICLE
Diane H Brown, Neil J Paloian
Electrolyte disorders are very common in the pediatric population. Derangements in serum sodium and potassium concentrations are among the most frequently seen given the risk factors and comorbidities unique to children. Pediatricians, in both outpatient and inpatient settings, should be comfortable with the evaluation and initial treatment of disturbances in these electrolyte concentrations. However, to evaluate and treat a child with abnormal serum concentrations of sodium or potassium, it is critical to understand the regulatory physiology that governs osmotic homeostasis and potassium regulation in the body...
July 1, 2023: Pediatrics in Review
https://read.qxmd.com/read/37362166/pseudo-bartter-syndrome-in-an-infant-without-obvious-underlying-conditions-a-case-report
#31
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/37351317/sudden-cardiac-arrest-in-a-child-with-gitelman-syndrome-a-case-report-and-literature-review
#32
Jakub Zieg, Terezia Tavačová, Miroslava Balaščáková, Petra Peldová, Filip Fencl, Peter Kubuš
Salt-losing tubulopathies are well-recognised diseases predisposing to metabolic disturbances in affected patients. One of the most severe complications can be life-threatening arrhythmias causing sudden cardiac arrest. We present here the first case of a pediatric patient with Gitelman syndrome associated sudden cardiac arrest without precipitating event. A 10-year-old boy collapsed due to ventricular fibrillation in the Prague tram. Lay cardiopulmonary resuscitation was initiated and external defibrillation restored sinus rhythm within minutes...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/37287843/randomized-controlled-trial-to-evaluate-the-effect-of-prophylactic-amiodarone-versus-dexmedetomidine-on-reducing-the-incidence-of-postoperative-junctional-ectopic-tachycardia-after-pediatric-open-heart-surgery
#33
JOURNAL ARTICLE
Santosh Wadile, Kothandam Sivakumar, Udaya Charan Murmu, Selvakumar Ganesan, Giridhar Gopal Dhandayuthapani, Ravi Agarwal, Ejaz Ahamed Sheriff, Roy Varghese
BACKGROUND: Junctional ectopic tachycardia (JET) is the most common arrhythmia after pediatric open-heart surgeries (OHS), causing high morbidity and mortality. As diagnosis is often missed in patients with minimal hemodynamic instability, its incidence depends on active surveillance. A prospective randomized trial evaluated the efficacy and safety of prophylactic amiodarone and dexmedetomidine to prevent and control postoperative JET. METHODS: Consecutive patients aged under 12 years were randomized into amiodarone, dexmedetomidine (initiated during anesthetic induction) and control groups...
2023: Annals of Pediatric Cardiology
https://read.qxmd.com/read/37199305/diagnosis-of-17-alpha-hydroxylase-deficiency-performed-late-in-life-in-a-patient-with-a-46-xy-karyotype
#34
JOURNAL ARTICLE
Bruno Bouça, Mariana Cascão, Pedro Fiúza, Sara Amaral, Paula Bogalho, José Silva-Nunes
SUMMARY: 17-Alpha-hydroxylase deficiency (17OHD) is a rare autosomal recessive disease, representing 1% of cases of congenital adrenal hyperplasia. A 44-year-old female presented to the emergency department complaining of generalized asthenia and polyarthralgia for about 2 weeks. On examination, she was hypertensive (174/100 mmHg), and laboratory results revealed hypokalemia and hypocortisolism. She had an uncharacteristic morphotype, BMI of 16.7 kg/m2, cutaneous hyperpigmentation, and Tanner stage M1P1, with normal female external genitalia...
May 1, 2023: Endocrinology, Diabetes & Metabolism Case Reports
https://read.qxmd.com/read/37184081/novel-sonic-hedgehog-gene-variant-in-a-patient-with-hyponatremia-microsomia-and-midline-defects-phenotype-description-in-association-with-a-variant-of-unknown-significance-c-755_757del-p-phe252del-and-an-approach-to-salt-wasting-in-shh-related-adrenal-disorders
#35
Marita Antoniadi, Dimitra Irinna Vitoratou, Maria Marinou, Olga Fafoula, Fani Mylona, Danai Palaiologou, Lazaros Leandros, Stavroula Kostaridou
OBJECTIVE: To contribute a novel sonic hedgehog (SHH) gene variant in association with a novel-meagerly described phenotype and discuss SHH signaling pathway pathology. CASE PRESENTATION: We present a 5-year-old boy with excessive hyponatremia and natriuresis, microform holoprosencephaly and microsomia, with morphologically intact hypothalamic-pituitary-adrenal (HPA) axis, and hypoaldosteronism, yet without hyperreninemia, hyperkalemia, dehydration episodes, or glucocorticoid insufficiency...
April 27, 2023: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://read.qxmd.com/read/37150827/moyamoya-disease-presenting-with-tubular-dysfunction-in-a-child-pitfalls-in-diagnosing-an-atypical-hyponatremic-hypertensive-syndrome
#36
JOURNAL ARTICLE
Maria Luisa Conte, Claudio La Scola, Francesca Mencarelli, Beatrice Filippini, Elena Fabbri, Valentina Ragnoni, Elisa Ravaioli, Andrea Pasini, Gianluca Vergine
BACKGROUND: Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in childhood, however it rarely underlies a hyponatremic-hypertensive syndrome (HHS). CASE PRESENTATION: We describe an 18-month-old boy with a recent history of polyuria and polydipsia, who presented an acute febrile gastroenteritis with neurological impairment, severe dehydration, hyponatremia, hypokalemia, kidney tubular dysfunction, and elevated aldosterone and renin even with a normal blood pressure...
May 8, 2023: BMC Pediatrics
https://read.qxmd.com/read/37139251/evaluation-of-early-insulin-glargine-administration-in-the-treatment-of-pediatric-diabetic-ketoacidosis
#37
JOURNAL ARTICLE
Kelli J Welter, Jessica L Marquez, Patricia L Marshik, Michael V Yao, Ellen S Bickel
OBJECTIVE: In the management of diabetic ketoacidosis (DKA), the standard of care is to administer insulin glargine after ketoacidosis has resolved and the patient is transitioning from intravenous (IV) insulin to subcutaneous insulin; however, there is evidence to suggest that earlier administration of insulin glargine may accelerate resolution of ketoacidosis. The objective of this research is to determine the efficacy of early subcutaneous insulin glargine on time to resolution of ketoacidosis in children with moderate to severe DKA...
2023: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://read.qxmd.com/read/37138571/a-mosaic-mutation-in-the-clcnkb-gene-causing-bartter-syndrome-a-case-report
#38
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
#39
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/36967744/response-to-magnesium-sulfate-and-adrenocorticotropic-hormone-combination-therapy-for-infantile-spasms-with-failed-first-line-treatments
#40
JOURNAL ARTICLE
Qiuhong Wang, Wen He, Yangyang Wang, Liying Liu, Mengna Zhang, Xiaoyan Yang, Liping Zou
IMPORTANCE: Infantile spasm (IS) is a kind of refractory epilepsy. The first-line treatments for IS are adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin. OBJECTIVE: This study aimed to evaluate the efficacy of magnesium sulfate and ACTH (MgSO4 +ACTH) combination therapy in patients with IS who failed first-line treatments. METHODS: In this retrospective study, the clinical data of patients with IS who failed first-line treatments were collected in the Chinese PLA General Hospital...
March 2023: Pediatric Investigation
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