-
Biomedicines Oct 2023Our objective was to overview the most recent data on primary hyperparathyroidism (PHP) in children and teenagers from a multidisciplinary perspective. Methods:... (Review)
Review
Our objective was to overview the most recent data on primary hyperparathyroidism (PHP) in children and teenagers from a multidisciplinary perspective. Methods: narrative review based on full-length, English-language papers (from PubMed, between January 2020 and July 2023). Results: 48 papers (14 studies of ≥10 subjects/study, and 34 case reports/series of <10 patients/study). Study-sample-based analysis: except for one case-control study, all of the studies were retrospective, representing both multicenter (n = 5) and single-center (n = 7) studies, and cohort sizes varied from small (N = 10 to 19), to medium-sized (N = 23 to 36) and large (N = 63 to 83); in total, the reviewed studies covered 493 individuals with PHP. Case reports/series (n = 34, N = 41): the mean ages studied varied from 10.2 to 14 years in case reports, and the mean age was 17 years in case series. No clear female predominance was identified, unlike that observed in the adult population. Concerning the assessments, there were four major types of endpoints: imaging data collection, such as ultrasound, 99mTc Sestamibi, or dual-phase computed tomography (CT); gene testing/familial syndrome identification; preoperative findings; and exposure to surgical outcome/preoperative drugs, like cinacalcet, over a 2.2-year median (plus two case reports of denosumab used as an off-label calcium-lowering agent). Single-gland cases (representing 85% of sporadic cases and 19% of genetic PHP cases) showed 100% sensitivity for neck ultrasounds, with 98% concordance with 99mTc Sestamibi, as well as a 91% sensitivity for dual-phase CT, with 25% of the lesions being ectopic parathyroids (mostly mediastinal intra-thymic). Case reports included another 9/41 patients with ectopic parathyroid adenomas, 3/41 with parathyroid carcinomas, and 8/41 subjects with brown tumors. Genetic PHP (which has a prevalence of 5-26.9%) mostly involved , followed by , , , and , as well as one case of . Symptomatic PHP: 70-100% of all cases. Asymptomatic PHP: 60% of genetic PHP cases. Renal involvement: 10.5% of a cohort with genetic PHP, 71% of sporadic PHP cases; 50% (in a cohort with a mean age of 16.7), 29% (in a cohort with a mean age of 15.2); 0% (in infancy) to 50-62% (in teenagers). Bone anomalies: 83% of the children in one study and 62% of those in two other studies. Gastrointestinal issues: 40% of one cohort, but the data are heterogeneous. Cure rate through parathyroidectomy: 97-98%. Recurrent PHP: 2% of sporadic PHP cases and 38% of familial PHP cases. Hungry bone syndrome: maximum rate of 34-40%. Case reports identified another 7/41 subjects with the same post-parathyroidectomy condition; a potential connection with ectopic presentation or brown tumors is suggested, but there are limited data. Minimally invasive thoracoscopic approaches for ectopic tumors seemed safe. The current level of statistical evidence on pediatric PHP qualifies our study- and case-sample-based analysis (n = 48, N = 534) as one of the largest of its kind. Awareness of PHP is the key factor to benefit our young patients.
PubMed: 37893182
DOI: 10.3390/biomedicines11102810 -
Hormones (Athens, Greece) Mar 2024Primary hyperparathyroidism (PHPT), a relatively common disorder characterized by hypercalcemia with raised or inappropriately normal serum parathyroid hormone (PTH)... (Review)
Review
Primary hyperparathyroidism (PHPT), a relatively common disorder characterized by hypercalcemia with raised or inappropriately normal serum parathyroid hormone (PTH) concentrations, may occur as part of a hereditary syndromic disorder or as a non-syndromic disease. The associated syndromic disorders include multiple endocrine neoplasia types 1-5 (MEN1-5) and hyperparathyroidism with jaw tumor (HPT-JT) syndromes, and the non-syndromic forms include familial hypocalciuric hypercalcemia types 1-3 (FHH1-3), familial isolated hyperparathyroidism (FIHP), and neonatal severe hyperparathyroidism (NS-HPT). Such hereditary forms may occur in > 10% of patients with PHPT, and their recognition is important for implementation of gene-specific screening protocols and investigations for other associated tumors. Syndromic PHPT tends to be multifocal and multiglandular with most patients requiring parathyroidectomy with the aim of limiting end-organ damage associated with hypercalcemia, particularly osteoporosis, nephrolithiasis, and renal failure. Some patients with non-syndromic PHPT may have mutations of the MEN1 gene or the calcium-sensing receptor (CASR), whose loss of function mutations usually cause FHH1, a disorder associated with mild hypercalcemia and may follow a benign clinical course. Measurement of the urinary calcium-to-creatinine ratio clearance (UCCR) may help to distinguish patients with FHH from those with PHPT, as the majority of FHH patients have low urinary calcium excretion (UCCR < 0.01). Once genetic testing confirms a hereditary cause of PHPT, further genetic testing can be offered to the patients' relatives and subsequent screening can be carried out in these affected family members, which prevents inappropriate testing in normal individuals.
Topics: Infant, Newborn; Humans; Hyperparathyroidism, Primary; Calcium; Hypercalcemia; Adenoma; Fibroma; Hyperparathyroidism; Jaw Neoplasms
PubMed: 38038882
DOI: 10.1007/s42000-023-00508-9 -
Endocrine Connections May 2024In this review, we discuss the definition, prevalence, and etiology of sporadic multiglandular disease (MGD), with an emphasis on its preoperative and intraoperative... (Review)
Review
In this review, we discuss the definition, prevalence, and etiology of sporadic multiglandular disease (MGD), with an emphasis on its preoperative and intraoperative predictors. Primary hyperparathyroidism (PHPT) is the third-most common endocrine disorder, and multiglandular parathyroid disease (MGD) is a cause of PHPT. Hereditary MGD can be definitively diagnosed with detailed family history and genetic testing, whereas sporadic MGD presents a greater challenge in clinical practice, and parathyroidectomy for MGD is associated with a higher risk of surgical failure than single gland disease (SGD). Therefore, it is crucial to be able to predict the presence of sporadic MGD in a timely manner, either preoperatively or intraoperatively. Various predictive methods cannot accurately identify all cases of sporadic MGD, but they can greatly optimize the management of MGD diagnosis and treatment and optimize the cure rate. Future research will urge us to investigate more integrative predictive models as well as increase our understanding of MGD pathogenesis.
PubMed: 38513354
DOI: 10.1530/EC-23-0492 -
Frontiers in Endocrinology 2023This study aimed to systematically review research on cinacalcet and secondary hyperparathyroidism (SHPT) using machine learning-based statistical analyses. (Meta-Analysis)
Meta-Analysis
INTRODUCTION
This study aimed to systematically review research on cinacalcet and secondary hyperparathyroidism (SHPT) using machine learning-based statistical analyses.
METHODS
Publications indexed in the Web of Science Core Collection database on Cinacalcet and SHPT published between 2000 and 2022 were retrieved. The R package "Bibliometrix," VOSviewer, CiteSpace, meta, and latent Dirichlet allocation (LDA) in Python were used to generate bibliometric and meta-analytical results.
RESULTS
A total of 959 articles were included in our bibliometric analysis. In total, 3753 scholars from 54 countries contributed to this field of research. The United States, Japan, and China were found to be among the three most productive countries worldwide. Three Japanese institutions (Showa University, Tokai University, and Kobe University) published the most articles on Cinacalcet and SHPT. Fukagawa, M.; Chertow, G.M.; Goodman W.G. were the three authors who published the most articles in this field. Most articles were published in , , and . Research on Cinacalcet and SHPT has mainly included three topics: 1) comparative effects of various treatments, 2) the safety and efficacy of cinacalcet, and 3) fibroblast growth factor-23 (FGF-23). Integrated treatments, cinacalcet use in pediatric chronic kidney disease, and new therapeutic targets are emerging research hotspots. Through a meta-analysis, we confirmed the effects of Cinacalcet on reducing serum PTH ( = -0.56, 95% = -0.76 to -0.37, = 0.001) and calcium ( = -0.93, 95% = -1.21to -0.64, = 0.001) and improving phosphate ( = 0.17, 95% = -0.33 to -0.01, = 0.033) and calcium-phosphate product levels ( = -0.49, 95% = -0.71 to -0.28, = 0.001); we found no difference in all-cause mortality ( = 0.97, 95% = 0.90 to 1.05, = 0.47), cardiovascular mortality ( = 0.69, 95% = 0.36 to 1.31, = 0.25), and parathyroidectomy ( = 0.36, 95% = 0.09 to 1.35, = 0.13) between the Cinacalcet and non-Cinacalcet users. Moreover, Cinacalcet was associated with an increased risk of nausea ( = 2.29, 95% = 1.73 to 3.05, = 0.001), hypocalcemia ( = 4.05, 95% = 2.33 to 7.04, = 0.001), and vomiting ( = 1.90, 95% = 1.70 to 2.11, = 0.001).
DISCUSSION
The number of publications indexed to Cinacalcet and SHPT has increased rapidly over the past 22 years. Literature distribution, research topics, and emerging trends in publications on Cinacalcet and SHPT were analyzed using a machine learning-based bibliometric review. The findings of this meta-analysis provide valuable insights into the efficacy and safety of cinacalcet for the treatment of SHPT, which will be of interest to both clinical and researchers.
Topics: Child; Humans; Calcimimetic Agents; Calcium; Cinacalcet; Hyperparathyroidism, Secondary; Phosphates; United States; Machine Learning
PubMed: 37538795
DOI: 10.3389/fendo.2023.1146955 -
Gland Surgery Aug 2023Both subtotal parathyroidectomy (SPTX) and total parathyroidectomy with autotransplantation (TPTX + AT) are considered acceptable surgical approaches for renal patients....
BACKGROUND
Both subtotal parathyroidectomy (SPTX) and total parathyroidectomy with autotransplantation (TPTX + AT) are considered acceptable surgical approaches for renal patients. It is common that parathyroid surgery is performed in patients before they undergo kidney transplantation and there is currently no evidence considering the best surgical approach in this subset of patients.
METHODS
Two cohorts were identified of consecutive patients who underwent parathyroidectomy for renal hyperparathyroidism by two surgeons at a single institution over equivalent time periods (SPTX and TPTX + AT). A retrospective chart review was performed to assess these techniques, including outcomes following kidney transplantation.
RESULTS
There were 125 patients analysed, with 56 patients who underwent SPTX and 69 who underwent TPTX + AT. Both cohorts effectively reduced PTH post operatively. There were 22 patients in the SPTX cohort and 26 in the TPTX + AT cohort that subsequently received kidney transplants. There were no cases of recurrent hyperparathyroidism and one of hypoparathyroidism (4.5%) in the SPTX patients post-transplant. There was one case of recurrent hyperparathyroidism (3.8%) and four of persistent hypoparathyroidism (15.4%) in the TPTX + AT patients post-transplant.
CONCLUSIONS
Surgery for renal hyperparathyroidism requires a careful balance of the extent of parathyroid resection to prevent persistent/recurrent disease and avoid permanent hypoparathyroidism. SPTX may be a more appropriate option in kidney transplant candidates in order to minimise the risk of long-term hypoparathyroidism.
PubMed: 37701301
DOI: 10.21037/gs-23-54 -
Journal of Nephrology Sep 2023Severe secondary hyperparathyroidism (SHPT) is associated with mortality in end stage kidney disease (ESKD). Parathyroidectomy (PTX) becomes necessary when medical... (Observational Study)
Observational Study
BACKGROUND
Severe secondary hyperparathyroidism (SHPT) is associated with mortality in end stage kidney disease (ESKD). Parathyroidectomy (PTX) becomes necessary when medical therapy fails, thus highlighting the interest to compare biochemical and clinical outcomes of patients receiving either medical treatment or surgery.
METHODS
We aimed to compare overall survival and biochemical control of hemodialysis patients with severe hyperparathyroidism, treated by surgery or medical therapy followed-up for 36 months. Inclusion criteria were age older than 18 years, renal failure requiring dialysis treatment (hemodialysis or peritoneal dialysis) and ability to sign the consent form. A control group of 418 patients treated in the same centers, who did not undergo parathyroidectomy was selected after matching for age, sex, and dialysis vintage.
RESULTS
From 82 Dialysis units in Italy, we prospectively collected data of 257 prevalent patients who underwent parathyroidectomy (age 58.2 ± 12.8 years; M/F: 44%/56%, dialysis vintage: 15.5 ± 8.4 years) and of 418 control patients who did not undergo parathyroidectomy (age 60.3 ± 14.4 years; M/F 44%/56%; dialysis vintage 11.2 ± 7.6 y). The survival rate was higher in the group that underwent parathyroidectomy (Kaplan-Meier log rank test = 0.002). Univariable analysis (HR 0.556, CI: 0.387-0.800, p = 0.002) and multivariable analysis (HR 0.671, CI:0.465-0.970, p = 0.034), identified parathyroidectomy as a protective factor of overall survival. The prevalence of patients at KDOQI targets for PTH was lower in patients who underwent parathyroidectomy compared to controls (PTX vs non-PTX: PTH < 150 pg/ml: 59% vs 21%, p = 0.001; PTH at target: 18% vs 37% p = 0.001; PTH > 300 pg/ml 23% vs 42% p = 0.001). The control group received more intensive medical treatment with higher prevalence of vitamin D (65% vs 41%, p = 0.0001), calcimimetics (34% vs 14%, p = 0.0001) and phosphate binders (77% vs 66%, p = 0.002).
CONCLUSIONS
Our data suggest that parathyroidectomy is associated with survival rate at 36 months, independently of biochemical control. Lower exposure to high PTH levels could represent an advantage in the long term.
Topics: Adolescent; Aged; Humans; Middle Aged; Hyperparathyroidism, Secondary; Kidney Failure, Chronic; Parathyroid Hormone; Parathyroidectomy; Prospective Studies; Renal Dialysis
PubMed: 37351832
DOI: 10.1007/s40620-023-01658-0 -
Otolaryngologic Clinics of North America Feb 2024The treatment of hyperparathyroidism through parathyroidectomy requires careful and complete preoperative evaluation. There are multiple imaging modalities and methods... (Review)
Review
The treatment of hyperparathyroidism through parathyroidectomy requires careful and complete preoperative evaluation. There are multiple imaging modalities and methods available to clinicians today to aid in identifying a pathological lesion; however, each has limitations that the clinician must understand. A systematic approach to patient evaluation, imaging, and surgical exploration is necessary to ensure accurate diagnosis and maximize the chances of minimally invasive and successful surgical removal.
Topics: Humans; Parathyroidectomy; Minimally Invasive Surgical Procedures; Hyperparathyroidism; Preoperative Care
PubMed: 37634984
DOI: 10.1016/j.otc.2023.07.004 -
Surgery Jan 2024Differences in presenting symptoms of primary hyperparathyroidism and outcomes of parathyroidectomy between sexes have been described, but whether these can be assessed... (Review)
Review
BACKGROUND
Differences in presenting symptoms of primary hyperparathyroidism and outcomes of parathyroidectomy between sexes have been described, but whether these can be assessed by perioperative use of a validated tool, such as the Pasieka Parathyroidectomy Assessment Score, is unknown.
METHOD
All patients with primary hyperparathyroidism were asked to complete symptom assessment at the preoperative and postoperative visits. The assessment included a query for 13 Pasieka Parathyroidectomy Assessment Score parameters evaluated using a visual analog scale as described by Pasieka (summative score 0-1,300), and general quality of life and wellness. A review of a prospectively maintained database of primary hyperparathyroidism patients (January 2016-December 2019) was performed, and those who had a 6-month cure after initial parathyroidectomy were included.
RESULTS
The study cohort was mostly women (77%, 541/701). The median preoperative Pasieka Parathyroidectomy Assessment Score was higher in women (155, 0-1,190) than in men (80.5, 0-855, P < .001), although there were similar rates of asymptomatic primary hyperparathyroidism (Pasieka Parathyroidectomy Assessment Score = 0, 12.5% vs 7%, P = .042). After curative parathyroidectomy, women reported a substantial reduction in symptomatology, with Pasieka Parathyroidectomy Assessment Score declining by 35% at initial postoperative visit (median, 155 vs 100, P < .001), further decreasing to 48% by 6 months (155 vs 80, P < .001). The Pasieka Parathyroidectomy Assessment Score in men did change but to a much smaller degree at both the initial postoperative visit (80.5 vs 70; P = .036) and at 6 months (80.5 vs 57.5; P = .048).
CONCLUSION
When assessed with the Pasieka Parathyroidectomy Assessment Score, improvement in symptoms was clearly demonstrated for women after curative parathyroidectomy. Whether symptom improvement also occurs in men is less apparent but may be due to disparities in the development and validation of outcomes tools in general.
Topics: Humans; Male; Female; Hyperparathyroidism, Primary; Quality of Life; Sex Characteristics; Prospective Studies; Parathyroidectomy
PubMed: 37980200
DOI: 10.1016/j.surg.2023.07.044 -
Cureus Nov 2023Various minimally invasive techniques exist for surgical parathyroidectomy. The aim of this study was to conduct a meta-analysis comparing two popular minimally invasive... (Review)
Review
Various minimally invasive techniques exist for surgical parathyroidectomy. The aim of this study was to conduct a meta-analysis comparing two popular minimally invasive techniques: minimally invasive video-assisted parathyroidectomy (MIVAP) and open minimally invasive parathyroidectomy (OMIP). An extensive search was conducted of online databases to identify all previous studies that had compared MIVAP and OMIP. The primary outcome measures considered were visual analog scale (VAS) score 24 hours postoperatively, conversion of operation (to open), failure rate and analgesic consumption. The data from these studies was extracted and compiled into a meta-analysis. The literature search yielded 104 studies of which four were included, enrolling 903 patients in this analysis. A significant difference was found regarding rates of conversion to open parathyroidectomy between the two groups, with the OMIP group demonstrating fewer conversions (MD = 3.52, CI = (2.04-6.08), P< 0.00001). No statistically significant differences were found between the two groups when comparing postoperative VAS scores at 24 hours (MD = -1.75, CI = (-9.8-6.3), P = 0.67), consumption of analgesia (OR = 0.49, CI = 0.07-3.54, P = 0.48) or failure rates (OR = 1.81, CI = 0.58-5.72, P = 0.31). OMIP was seen to require less need to convert to open parathyroidectomy with shorter operative times, while similar complication rates and scar lengths to MIVAP. More studies are required to evaluate the superior technique for parathyroidectomy.
PubMed: 38046707
DOI: 10.7759/cureus.48153 -
Revista de La Facultad de Ciencias... Dec 2023Primary hyperparathyroidism (PHPT) and celiac disease (CD) are two distinct medical conditions that can affect bone health. While PHPT leads to excessive calcium levels...
OBJECTIVE
Primary hyperparathyroidism (PHPT) and celiac disease (CD) are two distinct medical conditions that can affect bone health. While PHPT leads to excessive calcium levels and bone abnormalities, CD impairs calcium and vitamin D absorption due to small intestine damage.
CASE REPORT
We present a case of a 49-year-old woman diagnosed with osteoporosis who was found to have both PHPT and CD. The patient underwent a successful minimally invasive parathyroidectomy, which resulted in decreased parathyroid hormone levels.
CONCLUSION
This case highlights the rare coexistence of PHPT and CD and emphasizes the importance of considering secondary causes of osteoporosis in patients with low bone mass. Further studies are needed to explore the underlying mechanisms and potential links between PHPT and CD.
Topics: Female; Humans; Middle Aged; Celiac Disease; Calcium; Hyperparathyroidism, Primary; Osteoporosis; Parathyroidectomy
PubMed: 38150201
DOI: 10.31053/1853.0605.v80.n4.42137