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Osteoporosis International : a Journal... Jan 2024Hypophosphatasia (HPP) is a rare inborn error of metabolism that presents variably in both age of onset and severity. HPP is caused by pathogenic variants in the ALPL... (Review)
Review
Hypophosphatasia (HPP) is a rare inborn error of metabolism that presents variably in both age of onset and severity. HPP is caused by pathogenic variants in the ALPL gene, resulting in low activity of tissue nonspecific alkaline phosphatase (TNSALP). Patients with HPP tend have a similar pattern of elevation of natural substrates that can be used to aid in diagnosis. No formal diagnostic guidelines currently exist for the diagnosis of this condition in children, adolescents, or adults. The International HPP Working Group is a comprised of a multidisciplinary team of experts from Europe and North America who have expertise in the diagnosis and management of patients with HPP. This group reviewed 93 papers through a Medline, Medline In-Process, and Embase search for the terms "HPP" and "hypophosphatasia" between 2005 and 2020 and that explicitly address either the diagnosis of HPP in children, clinical manifestations of HPP in children, or both. Two reviewers independently evaluated each full-text publication for eligibility and studies were included if they were narrative reviews or case series/reports that concerned diagnosis of pediatric HPP or included clinical aspects of patients diagnosed with HPP. This review focused on 15 initial clinical manifestations that were selected by a group of clinical experts.The highest agreement in included literature was for pathogenic or likely pathogenic ALPL variant, elevation of natural substrates, and early loss of primary teeth. The highest prevalence was similar, including these same three parameters and including decreased bone mineral density. Additional parameters had less agreement and were less prevalent. These were organized into three major and six minor criteria, with diagnosis of HPP being made when two major or one major and two minor criteria are present.
Topics: Adult; Child; Humans; Adolescent; Hypophosphatasia; Alkaline Phosphatase; Europe; Prevalence; Mutation
PubMed: 37982855
DOI: 10.1007/s00198-023-06843-2 -
Dental and Medical Problems 2023Endemic fluorosis (skeletal and dental) is a serious public health problem in many parts of the world, especially in India. Age, sex, dietary calcium (Ca), the hormonal...
BACKGROUND
Endemic fluorosis (skeletal and dental) is a serious public health problem in many parts of the world, especially in India. Age, sex, dietary calcium (Ca), the hormonal status, the dose and duration of the fluoride intake, and renal efficiency in handling fluoride all influence fluoride metabolism.
OBJECTIVES
The aim of the study was to evaluate the effect of the fluoride present in drinking water on the serum alkaline phosphatase (ALP) and phosphate levels in pregnant women and newborn infants.
MATERIAL AND METHODS
In the present cross-sectional study, the participants were categorized into 2 groups based on a fluoride concentration in their drinking water: the low/optimum-fluoride group (<1 ppm); and the high-fluoride group (≥1 ppm). Each group was comprised of 90 pregnant women who were recruited from the hospital at the time of admission for delivery. Fluoride was measured in their drinking water, urine, maternal serum, and cord blood. The ALP and phosphate levels were measured in serum using a fully automated analyzer.
RESULTS
The drinking water consumed by the pregnant women contained fluoride, which was significantly positively correlated with the urine and blood serum fluoride levels. There were significant differences in the ALP levels between the 2 groups in both maternal serum and cord blood. The level of phosphate in maternal serum was significantly higher in the high-fluoride group. The results of both simple and multivariate regression analyses revealed that the fluoride content in drinking water was significantly associated with the ALP level in cord blood and the phosphate level in maternal serum.
CONCLUSIONS
The ALP levels were negatively associated with drinking water fluoride concentrations in both maternal serum and cord blood. The phosphate levels in maternal serum were positively associated with drinking water fluoride concentrations.
Topics: Infant, Newborn; Humans; Female; Pregnancy; Fluorides; Drinking Water; Alkaline Phosphatase; Pregnant Women; Cross-Sectional Studies; Phosphates
PubMed: 37555277
DOI: 10.17219/dmp/132692 -
Journal of Stroke and Cerebrovascular... Jan 2024Alkaline phosphatase (ALP) catalyzes the hydrolysis of pyrophosphate and facilitates vascular calcification. We aimed at investigating serum ALP levels in intracerebral...
OBJECTIVES
Alkaline phosphatase (ALP) catalyzes the hydrolysis of pyrophosphate and facilitates vascular calcification. We aimed at investigating serum ALP levels in intracerebral hemorrhage (ICH) patients and ascertaining its relationship to severity and prognosis.
METHODS
Serum ALP levels from 148 patients and 148 healthy controls were detected. Glasgow coma scale (GCS) score and hematoma volume at admission were recorded to evaluate hemorrhagic severity. Modified Rankin Scale (mRS) score > 2 at 90 days after onset was judged as a poor prognosis.
RESULTS
Serum ALP levels in patients with ICH were substantially elevated compared with healthy controls, and were significantly related to hematoma volume and GCS score. Serum ALP levels significantly distinguished ICH patients at risk for unfavorable prognosis. Serum ALP levels > 78.5 U/L in ICH patients may indicated a unfavorable prognosis with 69.1 % sensitivity and 83.6 % specificity, and served as an independent predictor for unfavorable prognosis.
CONLUSIONS
Elevated serum ALP levels were intimately connected with increased severity and 90-day unfavorable prognosis in patients with ICH. Serum ALP could be a potential biomarker for severity and prognosis of ICH.
Topics: Humans; Alkaline Phosphatase; Biomarkers; Cerebral Hemorrhage; Hematoma; Prognosis
PubMed: 37952310
DOI: 10.1016/j.jstrokecerebrovasdis.2023.107478 -
Medicina (Kaunas, Lithuania) Jul 2023Alkaline phosphatase activity, mineralized matrix, and osteogenic-related gene expression have been shown to increase in response to bone morphogenetic protein-9...
Alkaline phosphatase activity, mineralized matrix, and osteogenic-related gene expression have been shown to increase in response to bone morphogenetic protein-9 (BMP-9). In this study, spheroids derived from human gingival stem cells were used to determine the effects of BMP-9 on cell survival, osteogenesis, and mineralization. : Human gingival stem cells were used to produce spheroids and then grown to concentrations of 0, 0.1, 1, 10, and 100 ng/mL with BMP-9. On days 1, 3, 5, and 7, morphological examination was carried out. A live/dead assay and Cell Counting Kit-8 was used to assess the vitality of cells. On days 7 and 14, alkaline phosphatase activity assays were carried out using a commercially available kit to examine the osteogenic differentiation of cell spheroids. Alizarin Red Staining was performed on the 7th and 14th days to evaluate mineralization, and and expression levels were evaluated on the 7th and 14th days using real-time polymerase chain reactions. The BMP-9 added at the measured quantities did not appear to alter the shape of the well-formed spheroids produced by stem cells on day 1. In addition, treatment with BMP-9 at doses of 0, 0.1, 1, 10, or 100 ng/mL did not significantly alter cell diameter. Throughout the whole experimental process, viability was maintained. On day 14, the alkaline phosphatase activity in the groups dosed with 0.1, 1, 10, or 100 ng/mL was statistically higher than that in the unloaded control group ( < 0.05). According to qPCR data, the mRNA expression level of with 1 ng/mL dosing was higher on day 7 compared to that of the unloaded control group ( < 0.05). These findings suggest that BMP-9 can be employed to stimulate early osteogenic differentiation in stem cell spheroids.
Topics: Humans; Osteogenesis; Growth Differentiation Factor 2; Core Binding Factor Alpha 1 Subunit; Alkaline Phosphatase; Bone Morphogenetic Protein 2; Cell Differentiation; Stem Cells; Cells, Cultured
PubMed: 37512125
DOI: 10.3390/medicina59071315 -
Journal of Medicine and Life Oct 2023Dental anxiety is a familiar problem among children, which may affect their oral health. This cross-sectional study aimed to evaluate dental anxiety during the first...
Dental anxiety is a familiar problem among children, which may affect their oral health. This cross-sectional study aimed to evaluate dental anxiety during the first dental examination in relation to dental caries and salivary alkaline phosphatase, taking into account the nutritional status of children. Eighty-two children (45 boys and 37 girls), aged six to eight years old (average 6.96), were recruited from public clinics in Baghdad city. Participants were divided into the anxious and the non-anxious groups according to physiological measures (oxygen saturation and heart rate) before the first dental treatment. The weight and height of each child were measured to determine the nutritional status (normal weight, overweight, and obese). Dental caries (prevalence and severity) in children with different nutritional statuses were recorded using dmf, DMF, and the guidelines outlined by Manjie . Saliva was collected to analyze salivary alkaline phosphatase. No statistically significant differences were observed between anxious and non-anxious children in means of dmf and DMF indices (teeth and surfaces) in primary and permanent teeth (p>0.05) in the three nutritional status groups. However, non-anxious children exhibited a higher mean of d1 (initial enamel caries) compared to anxious children, with statistical significance (p<0.05) observed only in normal-weight children. Salivary alkaline phosphatase was not significantly different between the two groups. In conclusion, dental anxiety did not appear to significantly impact caries development in primary teeth or salivary alkaline phosphatase levels in children with varying nutritional statuses. Anxiety reduced the initial enamel caries in normal-weight children.
Topics: Child; Male; Female; Humans; Nutritional Status; Alkaline Phosphatase; Dental Caries; Cross-Sectional Studies; Dental Anxiety
PubMed: 38313167
DOI: 10.25122/jml-2023-0085 -
Advanced Science (Weinheim,... Nov 2023Castration-resistant prostate cancer (PCa) causes severe bone metastasis (BM), which significantly increases mortality in men with PCa. Imaging tests and radiometric...
Castration-resistant prostate cancer (PCa) causes severe bone metastasis (BM), which significantly increases mortality in men with PCa. Imaging tests and radiometric scanning require long analysis times, expensive equipment, specialized personnel, and a slow turnaround. New visualization technologies are expected to solve the above problems. Nonetheless, existing visualization techniques barely meet the urgency for precise diagnosis because the human eyes cannot recognize and capture even slight variations in visual information. By using dye differentiated superposition enhancement colorimetric biosensors, an effective method to diagnose prostate cancer bone metastases (PCa-BM) with excellent accuracy for naked-eye quantitative detection of alkaline phosphatase (ALP) is developed. The biomarker ALP specific hydrolytic product ascorbic acid can be detected by rhodamine derivatives (Rd) as gold nanobipyramids (Au NBPs) are deposited and grown. Color-recombining enhancement effects between Rd and Au NBPs significantly improved abundance. The 150 U L threshold between normal and abnormal can be identified by color. And with color enhancement effect and double signal response, the ALP index is visually measured to diagnose PCa-BM and provide handy treatment recommendations. Additionally, the proposed colorimetric sensing strategy can be used to diagnose other diseases.
Topics: Male; Humans; Colorimetry; Prostatic Neoplasms; Bone Neoplasms; Alkaline Phosphatase; Biosensing Techniques
PubMed: 37840414
DOI: 10.1002/advs.202303159 -
Journal of Traditional and... Jan 2024Osteoporosis is a chronic and systemic skeletal disease that is defined by low bone mineral density (BMD) along with an increase in bone fragility and susceptibility to... (Review)
Review
BACKGROUND
Osteoporosis is a chronic and systemic skeletal disease that is defined by low bone mineral density (BMD) along with an increase in bone fragility and susceptibility to fracture. This study aimed to overview clinical evidence on the use of herbal medicine for management of osteoporosis.
METHODS
Electronic databases including Pubmed, Medline, Cochrane library, and Scopus were searched until November 2022 for any clinical studies on the efficacy and/or safety of plant-derived medicines in the management of osteoporosis.
RESULTS
The search yielded 57 results: 19 on single herbs, 16 on multi-component herbal preparations, and 22 on plant-derived secondary metabolites. Risk of fracture, bone alkaline phosphatase, BMD, and specific bone biomarkers are investigated outcomes in these studies. Medicinal plants including and phytochemicals including isoflavones, ginsenoside, Epimedium prenyl flavonoids, tocotrienols are among plant-derived medicines clinically investigated on osteoporosis. It seems that multi-component herbal preparations were more effective than single-component ones; because of the synergistic effects of their constituents. The investigated herbal medicines demonstrated their promising results in osteoporosis via targeting different pathways in bone metabolism, including balancing osteoblasts and osteoclasts, anti-inflammatory, immunomodulatory, antioxidant, and estrogen-like functions.
CONCLUSION
It seems that plant-derived medicines have beneficial effects on bone and may manage osteoporosis by affecting different targets and pathways involved in osteoporosis; However, Future studies are needed to confirm the effectiveness and safety of these preparations.
PubMed: 38223808
DOI: 10.1016/j.jtcme.2023.08.001 -
Hepatology Communications Jan 2024Primary sclerosing cholangitis (PSC) is an immune-mediated, chronic cholestatic liver disease. Currently, liver transplantation is the only established life-saving... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Primary sclerosing cholangitis (PSC) is an immune-mediated, chronic cholestatic liver disease. Currently, liver transplantation is the only established life-saving treatment. Several studies have evaluated the effect of different biologic therapies on PSC with inconclusive findings. We conducted a systematic review and meta-analysis to assess the effects of biologics in PSC and associated inflammatory bowel disease (IBD).
METHODS
MEDLINE, Scopus, and Embase were searched up to July 31, 2023, for studies reporting the effects of biologics in patients with PSC-IBD. Effects of biologic therapy on alkaline phosphatase, total bilirubin, ulcerative colitis response score, and adverse events were calculated and expressed as standardized difference of means (SMD), proportions, and 95% CI using a random-effects model.
RESULTS
Six studies, including 411 PSC-IBD patients who received biologics, were included. Biologic treatment was associated with no change in alkaline phosphatase (SMD: 0.1, 95% CI: -0.07 -0.17, p=0.43), but a small and statistically significant increase in total bilirubin (SMD: 0.2, 95% CI: 0.05-0.35, p<0.01). 31.2% (95% CI: 23.8-39.7) of patients with IBD achieved endoscopic response, and there was a significant improvement in ulcerative colitis response score (SMD: -0.6,95% CI: -0.88 to 0.36, p<0.01). Furthermore, 17.6% (95% CI: 13.0-23.5) of patients experienced adverse events severe enough to discontinue therapy, and 29.9% (95% CI: 25.2-34.8) had a loss of response to biologics.
CONCLUSIONS
Treatment of patients with PSC-IBD with biologics (vedolizumab, infliximab, and adalimumab) was not associated with improvement of biochemical markers of cholestasis. Biologics are effective in treating the colitis associated with PSC. Vedolizumab was associated with worsening liver enzymes in contrast to other biologics, a finding that warrants further study.
Topics: Humans; Colitis, Ulcerative; Alkaline Phosphatase; Cholangitis, Sclerosing; Inflammatory Bowel Diseases; Bilirubin; Cholestasis; Biological Products
PubMed: 38206197
DOI: 10.1097/HC9.0000000000000347 -
Pakistan Journal of Medical Sciences 2023To identify the latest trends in the clinical picture and severity of the disease, which will help better understand and manage dengue.
OBJECTIVES
To identify the latest trends in the clinical picture and severity of the disease, which will help better understand and manage dengue.
METHODOLOGY
It was a cross-sectional, hospital-based study performed in the tertiary care hospitals of Punjab from August 21 to December 2022, in which serologically and polymerase chain reaction (PCR) confirmed patients with dengue infection, were enrolled. Demographic and clinical variables were recorded on a pre-tested Performa, processed and presented in frequency and percentages, and graphs were generated. Mean and standard deviation was used to present continuous variables.
RESULTS
Out of a total of 580 patients, 472 were diagnosed with Dengue Fever (DF) and 108 with Dengue Hemorrhagic Fever (DHF). About 79.31% of the patients were male and 20.69% were females. The mean age of patients was 32.5±9 years. Among the clinical features the percentage of high-grade fever, body aches, and vomiting were the highest. The liver function profile showed that serum bilirubin, Serum aspartate transaminase (AST), serum alanine transaminase (ALT,) and alkaline phosphatase (ALP) levels were markedly raised.
CONCLUSION
This study showed that with time the trends in the presentation of dengue are slowly shifting, which will help us better manage the disease burden in the future.
PubMed: 37936734
DOI: 10.12669/pjms.39.6.7383 -
Archives of Pathology & Laboratory... May 2024Interpretation of alkaline phosphatase (ALP) activity is essential for the diagnosis of certain diseases. ALP changes during life and may vary between different...
CONTEXT.—
Interpretation of alkaline phosphatase (ALP) activity is essential for the diagnosis of certain diseases. ALP changes during life and may vary between different populations.
OBJECTIVE.—
To establish reference intervals (RIs) and percentile charts for ALP activity in the Spanish population through a multicentric observational study and to compare the RIs to those defined in other countries.
DESIGN.—
A total of 662 350 ALP measurements from individuals ages 0 to 99 years from 9 Spanish tertiary care centers collected between 2020 and 2022 were analyzed. This study is the largest published in the literature to date.
RESULTS.—
Continuous percentile charts for ALP according to sex and age were established which can be used as RIs. Higher levels are reached during the first weeks of life. In puberty, a differential evolution is observed in both sexes, reaching a peak at 10 to 13 years of age in boys and remaining stable in girls at this age. Significant differences were also observed in adults, higher in men between ages 20 and 49 years and between ages 50 and 79 years in women, as reported in some countries.
CONCLUSIONS.—
ALP activity follows an age- and sex-dependent fluctuation with geographic differences. It is important to have appropriate reference values for each population in order to allow for a correct diagnostic interpretation and early diagnosis of diseases related to ALP abnormalities.
PubMed: 38797525
DOI: 10.5858/arpa.2023-0335-OA