Primary Hyperparathyroidism in Pediatric Patients: Clear-Cut Differences From Adult Patients

PEDIATRICS ◽  
2005 ◽  
Vol 115 (4) ◽  
pp. 1073-1073 ◽  
Author(s):  
R. W. Chesney
2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.


2021 ◽  
Vol 9 (3) ◽  
pp. 232596712199116
Author(s):  
Nicholas J. Lemme ◽  
Daniel S. Yang ◽  
Brooke Barrow ◽  
Ryan O’Donnell ◽  
Alan H. Daniels ◽  
...  

Background: Anterior cruciate ligament reconstruction (ACLR) in pediatric patients is becoming increasingly common. There is growing yet limited literature on the risk factors for revision in this demographic. Purpose: To (1) determine the rate of pediatric revision ACLR in a nationally representative sample, (2) ascertain the associated patient- and injury-specific risk factors for revision ACLR, and (3) examine the differences in the rate and risks of revision ACLR between pediatric and adult patients. Study Design: Case-control study; Level of evidence, 3. Methods: The PearlDiver patient record database was used to identify adult patients (age ≥20 years) and pediatric patients (age <20 years) who underwent primary ACLR between 2010 and 2015. At 5 years postoperatively, the risk of revision ACLR was compared between the adult and pediatric groups. ACLR to the contralateral side was also compared. Multivariate logistic regression was used to determine the significant risk factors for revision ACLR and the overall reoperation rates in pediatric and adult patients; from these risk factors, an algorithm was developed to predict the risk of revision ACLR in pediatric patients. Results: Included were 2055 pediatric patients, 1778 adult patients aged 20 to 29 years, and 1646 adult patients aged 30 to 39 years who underwent ACLR. At 5 years postoperatively, pediatric patients faced a higher risk of revision surgery when compared with adults (18.0 % vs 9.2% [adults 20-29 years] and 7.1% [adults 30-39 years]; P < .0001), with significantly decreased survivorship of the index ACLR ( P < .0001; log-rank test). Pediatric patients were also at higher risk of undergoing contralateral ACLR as compared with adults (5.8% vs 1.6% [adults 20-29 years] and 1.9% [adults 30-39 years]; P < .0001). Among the pediatric cohort, boys (odds ratio [OR], 0.78; 95% CI, 0.63-0.96; P = .0204) and patients >14 years old (OR, 0.62; 95% CI, 0.45-0.86; P = .0035) had a decreased risk of overall reoperation; patients undergoing concurrent meniscal repair (OR, 1.84; 95% CI, 1.43-2.38; P < .0001) or meniscectomy (OR, 2.20; 95% CI, 1.72-2.82; P < .0001) had an increased risk of revision surgery. According to the risk algorithm, the highest probability for revision ACLR was in girls <15 years old with concomitant meniscal and medial collateral ligament injury (36% risk of revision). Conclusion: As compared with adults, pediatric patients had an increased likelihood of revision ACLR, contralateral ACLR, and meniscal reoperation within 5 years of an index ACLR. Families of pediatric patients—especially female patients, younger patients, and those with concomitant medial collateral ligament and meniscal injuries—should be counseled on such risks.


2019 ◽  
Vol 5 (1) ◽  
pp. 126-135
Author(s):  
Nadia Ayala-Lopez ◽  
Roa Harb

Abstract Background The anion gap is primarily used in the diagnosis of acid-base disorders. We conducted a study to determine the anion gap reference interval in our patient population, investigated the workup of abnormal vs normal anion gaps, and examined the anion gap variation upon repeated testing. Methods A retrospective review was performed on 17137 adult and pediatric patients who presented to Yale-New Haven Hospital outpatient clinics, emergency department, or intensive care units between 2012 and 2017. Results We derived a new reference interval of 7 to 18 mmol/L with a median of 13 mmol/L in healthy adults with no significant differences owing to partitioning by sex or age. Based on the new reference interval, 5%, 23%, and 18% of healthy, emergency department, and intensive care unit adult patients, respectively, were misclassified as having high values with the previous interval of 6 to 16 mmol/L. However, there were no significant differences in the number of tests ordered in patients with anion gaps above and below the upper limit of the previous reference interval. The majority of increased anion gaps that were repeated normalized by 12 h. In a subgroup of healthy adult patients with annual testing, the median percent change in each patient's anion gap from 2015 to 2016 was approximately 13%. Conclusions The anion gap should be used with an appropriate reference interval to avoid misclassification. There may be a moderate degree of individuality that argues for comparing the anion gap with its baseline value in the same patient pending further studies that formally derive its biological variation.


2010 ◽  
Vol 54 (8) ◽  
pp. 3225-3232 ◽  
Author(s):  
Claudia Michael ◽  
Uta Bierbach ◽  
Katrin Frenzel ◽  
Thoralf Lange ◽  
Nadezda Basara ◽  
...  

ABSTRACT The aim of this study was to investigate the pharmacokinetics and safety of voriconazole after intravenous (i.v.) administration in immunocompromised children (2 to 11 years old) and adults (20 to 60 years old) who required treatment for the prevention or therapy of systemic fungal infections. Nine pediatric patients were treated with a dose of 7 mg/kg i.v. every 12 h for a period of 10 days. Three children and 12 adults received two loading doses of 6 mg/kg i.v. every 12 h, followed by a maintenance dose of 5 mg/kg (children) or 4 mg/kg (adults) twice a day during the entire study period. Trough voriconazole levels in blood over 10 days of therapy and regular voriconazole levels in blood for up to 12 h postdose on day 3 were examined. Wide intra- and interindividual variations in plasma voriconazole levels were noted in each dose group and were most pronounced in the children receiving the 7-mg/kg dose. Five (56%) of them frequently had trough voriconazole levels in plasma below 1 μg/ml or above 6 μg/ml. The recommended dose of 7 mg/kg i.v. in children provides exposure (area under the concentration-time curve) comparable to that observed in adults receiving 4 mg/kg i.v. The children had significantly higher C max values; other pharmacokinetic parameters were not significantly different from those of adults. Voriconazole exhibits nonlinear pharmacokinetics in the majority of children. Voriconazole therapy was safe and well tolerated in pediatric and adult patients. The European Medicines Agency-approved i.v. dose of 7 mg/kg can be recommended for children aged 2 to <12 years.


2021 ◽  
Author(s):  
Dilara Bal Topcu ◽  
Gökcen Tugcu ◽  
Berrin Er ◽  
Sanem Eryilmaz Polat ◽  
Mina Hizal ◽  
...  

Abstract Background We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Methods Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. Results YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with % FVC (r= -0.800, p = 0.014) and % FEV1 (r= -0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with % FVC (r= -0.697, p = 0.0082) and % FEV1 (r= -0.720, p = 0.006). Conclusions CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology. Furthermore, as YKL-40 levels are correlated with FEV1 and FVC in patients, it may be useful for the monitoring of pulmonary function in CF patient.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2106-2106
Author(s):  
Madiha Iqbal ◽  
Tea Reljic ◽  
Ernesto Ayala ◽  
Hemant S. Murthy ◽  
Ambuj Kumar ◽  
...  

Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy which affects over 300,000 children born each year worldwide. In spite of improvement in supportive care in recent years, there is still a lack of effective treatment options. SCD leads to debilitating and cyclic episodes of erythrocyte sickling with progressive organ injury, contributing to lifetime morbidity and shortened life expectancy. Allogeneic HCT (allo-HCT) is a potentially curative therapy for SCD because engraftment is associated with resolution of the clinical phenotype of the disease and abrogation of its complications. Medical literature on allo-HCT for SCD is largely limited to children. Recent studies have evaluated the efficacy of allo-HCT in the adult population. Here, we conduct a systematic review/meta-analysis to assess the totality of evidence pertaining to the efficacy (or lack thereof) of allo-HCT in children and adults. Materials and methods: We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE and Cochrane library on July 3rd, 2019. We extracted data on clinical outcomes related to benefits (overall [OS] and disease free/event free survival [EFS/DFS]) and harms (non-relapse mortality [NRM] and graft failure [GF]), independently by two authors. Our search strategy identified 1001 references but only 30 studies (n= 1995 patients) were included in this systematic review/meta-analysis. We also performed a sub analysis on clinical outcomes for studies that included only pediatric patients (defined as <18 years) and those in patients ≥18 years of age. Results: Median age for patients enrolled in all the studies was at 10 years. Recurrent veno-occlusive crises represented the most common indication for allo-HCT followed by acute chest syndrome and stroke; nevertheless, most patients had more than one indication. Matched related donors (MRD) were the most common donor source (93%). Bone marrow was the most common source of hematopoietic stem cells (77%). Majority of patients underwent conditioning with myeloablative regimens (77%). Pooled OS rates (n=29 studies, 1681 patients) after allogeneic HCT was 95% (95%CI=93-96%) with low heterogeneity (I2=6.4%) among included studies (Figure 1). Pooled EFS/DFS rates (n=29 studies, 1894 patients) post-allografting was 90% (95%CI=87-93%) with moderate heterogeneity (I2=54%). Pooled NRM rates from 30 studies (1995 patients) was 4% (95%CI=2-6%) with low heterogeneity (I2=29.4%). Pooled GF rates from 28 studies (1851 patients) was 4% (95%CI=2-6%) with moderate heterogeneity (I2=55%). A subset analysis specifically for pediatric patients (n= 11 studies, 1009 patients, median age at 9.7 years) showed a pooled OS rate of 96% (95%CI=94-97%) with low heterogeneity (I2=0%); and for adult patients (n=3 studies, 51 patients, median age at 33.4 years) the pooled OS was 94% (95%CI=80-100%) with moderate heterogeneity (I2=52%). Pooled EFS/DFS for pediatric patients (n= 11 studies, 1009 patients) was at 89 %( 95%CI=84-93%) with moderate heterogeneity (I2=55.1%); and for adult patients (n=2 studies, 30 patients) was at 95% (95%CI=83-100%) with high heterogeneity (I2=96.5%). Pooled NRM from 10 studies with pediatric patients (281 patients) was at 6 % (95%CI=3-10%) with low heterogeneity (I2=0%); and from 3 studies with adult patients (51 patients) was at 1% (95%CI=0-7%) with low heterogeneity (I2=15.1%). Pooled GF from 10 studies with pediatric patients (281 patients) was at 3 % (95%CI=1-7%) with moderate heterogeneity (I2=40%); and from 2 studies with adult patients (30 patients) was at 5% (95%CI=0-17%) with high heterogeneity (I2=95.4%). Conclusions: The results of our systematic review/meta-analysis show excellent OS, EFS/DFS in children and adults undergoing allo-HCT with pooled OS rates exceeding 90%. The main limitation to offering an allo-HCT in SCD remains the availability of a suitable donor as 85% of patients meeting criteria do not have a MRD. We anticipate that with emergence of haploidentical transplantation the number of allo-HCT will increase in the future. GF remains a significant concern in this population and future studies should focus on novel immune suppression strategies to help reduce GF. Disclosures Kharfan-Dabaja: Pharmacyclics: Consultancy; Daiichi Sankyo: Consultancy.


2021 ◽  
Vol 10 (17) ◽  
pp. 3936
Author(s):  
Kensuke Yokoyama ◽  
Tomonori Yano ◽  
Atsushi Kanno ◽  
Eriko Ikeda ◽  
Kozue Ando ◽  
...  

Balloon enteroscopy-assisted endoscopic retrograde cholangiography (BEA-ERC) is useful and feasible in adults with pancreatobiliary diseases, but its efficacy and safety have not been established in pediatric patients. We compared the success rate and safety of BEA-ERC between adults and pediatric patients. This single-center retrospective study reviewed 348 patients (pediatric: 57, adult: 291) with surgically altered gastrointestinal anatomies who underwent BEA-ERC for biliary disorders from January 2007 to December 2019. The success rate of reaching the anastomosis or duodenal papilla was significantly lower in pediatric patients than in adult patients (66.7% vs. 88.0%, p < 0.01). The clinical success rate was also significantly lower in pediatric patients (64.9% vs. 80.4%, p = 0.014). The rate of adverse events was significantly higher in pediatric patients than in adults (14.2% vs. 7.7%, p = 0.037). However, if the anastomotic sites were reached in pediatric patients, the treatment was highly successful (97.3%). The time of reaching target site was significantly longer in pediatric patients than in adult patients. This study shows that BEA-ERC in pediatric patients is more difficult than that in adult patients. However, in patients where the balloon enteroscope was advanced to the anastomosis, clinical outcomes comparable to those in adults can be achieved.


2017 ◽  
Vol 22 (5) ◽  
pp. 375-377 ◽  
Author(s):  
Michael Poon ◽  
Brady S. Moffett ◽  
Donald L. Yee

Rifampin has been documented to significantly attenuate the effect of warfarin in adult patients. No data have been presented on the use of rifampin and warfarin in a pediatric patient. We report an extreme case of increased warfarin metabolism in a pediatric patient who was concomitantly receiving rifampin, despite receiving other medications that significantly decrease warfarin metabolism. The inhibitory effect of rifampin on warfarin therapy may be amplified in pediatric patients.


Author(s):  
Sherein G. Elgendy ◽  
Sherine A. Aly ◽  
Rawhia Fathy ◽  
Enas A.E. Deaf ◽  
Naglaa H. Abu Faddan ◽  
...  

Background and Objectives: Clostridium difficile infection (CDI) has become a significant healthcare-associated infection throughout the world and is particularly important in developing countries. This study aimed to investigate clinical characterization and risk factors related to toxigenic C. difficile infection in adult and pediatric patients, antimicrobial susceptibility pattern. Also, to evaluate different diagnostic methods for rapid detection of C. difficile associated diarrhea (CDAD) in Egypt. Materials and Methods: Stool samples were collected from 95 pediatric patients and 37 adult patients suffering from antibiotic associated diarrhea and were subjected to direct toxin immunoassay and culture on cycloserine/cefoxitin/fructose agar. The presence of tcdA and tcdB genes was tested by PCR. Results: Toxigenic C. difficile was isolated from pediatric and adult patients at a rate of 17.89% (17/95) and 27% (10/37) respectively. The sensitivity and specificity of direct PCR from stool are (100%, 100% and 82.4%, 100%) in adult and pediatric samples respectively. The susceptibility of C. difficile to vancomycin and metronidazole were found to be 66.7% and 48.2% respectively. Conclusion: Diabetes mellitus, prior antibiotic treatment, hematological malignancy on chemotherapy, malnutrition, neutropenia and Ryle feeding are risk factors for development of CDAD. Tight restriction of unnecessary antibiotic uses is necessary in our locality. Direct detection of toxin genes in stool by PCR is sensitive and specific method for early detection of C. difficile.


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