{"id":"https://openalex.org/W7225110211","doi":"https://doi.org/10.3329/medtoday.v38i2.94514","title":"Familial Clustering of Primary Hypertrophic Osteoarthropathy (Pachydermoperiostosis): A Case Series of Five Patients","display_name":"Familial Clustering of Primary Hypertrophic Osteoarthropathy (Pachydermoperiostosis): A Case Series of Five Patients","publication_year":2026,"publication_date":"2026-11-02","ids":{"openalex":"https://openalex.org/W7225110211","doi":"https://doi.org/10.3329/medtoday.v38i2.94514"},"language":"en","primary_location":{"id":"doi:10.3329/medtoday.v38i2.94514","doi":"https://doi.org/10.3329/medtoday.v38i2.94514","is_oa":true,"landing_page_url":"https://doi.org/10.3329/medtoday.v38i2.94514","pdf_url":"https://www.banglajol.info/index.php/MEDTODAY/article/download/94514/60756","source":{"id":"https://openalex.org/S4210195798","display_name":"Medicine Today","issn_l":"1810-1828","issn":["1810-1828","2408-8714"],"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Medicine Today","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"diamond","oa_url":"https://www.banglajol.info/index.php/MEDTODAY/article/download/94514/60756","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5126747901","display_name":"Ananta Kumar Kundu","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Ananta Kumar Kundu","raw_affiliation_strings":["Assistant Registrar, Medicine Unit, Kushtia Medical College Hospital, Kushtia, Bangladesh"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Assistant Registrar, Medicine Unit, Kushtia Medical College Hospital, Kushtia, Bangladesh","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5126747901"],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":{"value":0.8720896,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"38","issue":"2","first_page":"336","last_page":"339"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T14057","display_name":"Hypertrophic osteoarthropathy and related conditions","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T14057","display_name":"Hypertrophic osteoarthropathy and related conditions","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T13328","display_name":"Osteomyelitis and Bone Disorders Research","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12199","display_name":"Vascular Anomalies and Treatments","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/primary-hypertrophic-osteoarthropathy","display_name":"primary hypertrophic osteoarthropathy","score":0.9539999961853027},{"id":"https://openalex.org/keywords/pachydermoperiostosis","display_name":"pachydermoperiostosis","score":1.0},{"id":"https://openalex.org/keywords/familial-aggregation","display_name":"familial aggregation","score":0.9850000143051147},{"id":"https://openalex.org/keywords/hereditary-bone-disorders","display_name":"hereditary bone disease","score":0.6510000228881836},{"id":"https://openalex.org/keywords/clubbing","display_name":"clubbing","score":0.949999988079071},{"id":"https://openalex.org/keywords/periostosis","display_name":"periostosis","score":0.9769999980926514},{"id":"https://openalex.org/keywords/pachydermia","display_name":"pachydermia","score":0.9620000123977661},{"id":"https://openalex.org/keywords/cutis-verticis-gyrata","display_name":"cutis verticis gyrata","score":0.8820000290870667},{"id":"https://openalex.org/keywords/hyperhidrosis","display_name":"hyperhidrosis","score":0.9330000281333923},{"id":"https://openalex.org/keywords/15-hydroxyprostaglandin-dehydrogenase","display_name":"15-hydroxyprostaglandin dehydrogenase","score":0.6930000185966492},{"id":"https://openalex.org/keywords/slco2a1","display_name":"SLCO2A1","score":0.9990000128746033},{"id":"https://openalex.org/keywords/bisphosphonate-therapy","display_name":"bisphosphonate therapy","score":0.8640000224113464},{"id":"https://openalex.org/keywords/cox-2-inhibitors","display_name":"COX-2 inhibitors","score":0.8669999837875366},{"id":"https://openalex.org/keywords/corticosteroids","display_name":"corticosteroids","score":0.8659999966621399},{"id":"https://openalex.org/keywords/symptomatic-treatment","display_name":"symptomatic treatment","score":0.6359999775886536}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9557999968528748},{"id":"https://openalex.org/C2778650131","wikidata":"https://www.wikidata.org/wiki/Q935781","display_name":"Hyperhidrosis","level":2,"score":0.6592000126838684},{"id":"https://openalex.org/C2776920502","wikidata":"https://www.wikidata.org/wiki/Q1641153","display_name":"Hypertrophic osteoarthropathy","level":2,"score":0.6535000205039978},{"id":"https://openalex.org/C182276654","wikidata":"https://www.wikidata.org/wiki/Q7577471","display_name":"Spinal osteoarthropathy","level":2,"score":0.526199996471405},{"id":"https://openalex.org/C16005928","wikidata":"https://www.wikidata.org/wiki/Q171171","display_name":"Dermatology","level":1,"score":0.45739999413490295},{"id":"https://openalex.org/C2779252107","wikidata":"https://www.wikidata.org/wiki/Q1004647","display_name":"Bullous pemphigoid","level":3,"score":0.3853999972343445},{"id":"https://openalex.org/C2781083543","wikidata":"https://www.wikidata.org/wiki/Q635339","display_name":"Macrocephaly","level":2,"score":0.3400000035762787},{"id":"https://openalex.org/C187212893","wikidata":"https://www.wikidata.org/wiki/Q123028","display_name":"Pediatrics","level":1,"score":0.33180001378059387},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.33079999685287476},{"id":"https://openalex.org/C64618202","wikidata":"https://www.wikidata.org/wiki/Q3733697","display_name":"Genetic heterogeneity","level":4,"score":0.29269999265670776},{"id":"https://openalex.org/C190892606","wikidata":"https://www.wikidata.org/wiki/Q3512464","display_name":"Radiological weapon","level":2,"score":0.28790000081062317},{"id":"https://openalex.org/C2776139714","wikidata":"https://www.wikidata.org/wiki/Q3480107","display_name":"Bone pain","level":2,"score":0.2842000126838684},{"id":"https://openalex.org/C2778156654","wikidata":"https://www.wikidata.org/wiki/Q666075","display_name":"Genodermatosis","level":3,"score":0.28360000252723694},{"id":"https://openalex.org/C2780673598","wikidata":"https://www.wikidata.org/wiki/Q285223","display_name":"Genetic testing","level":2,"score":0.2827000021934509},{"id":"https://openalex.org/C2776164576","wikidata":"https://www.wikidata.org/wiki/Q62736","display_name":"Osteoarthritis","level":3,"score":0.28209999203681946},{"id":"https://openalex.org/C2778784858","wikidata":"https://www.wikidata.org/wiki/Q1363741","display_name":"Erythroderma","level":2,"score":0.2808000147342682},{"id":"https://openalex.org/C80227256","wikidata":"https://www.wikidata.org/wiki/Q1124169","display_name":"Genetic counseling","level":2,"score":0.27489998936653137},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.2718999981880188},{"id":"https://openalex.org/C2777251235","wikidata":"https://www.wikidata.org/wiki/Q412205","display_name":"Bisphosphonate","level":3,"score":0.26249998807907104},{"id":"https://openalex.org/C2777757330","wikidata":"https://www.wikidata.org/wiki/Q1480377","display_name":"Periostitis","level":2,"score":0.2612000107765198},{"id":"https://openalex.org/C198451711","wikidata":"https://www.wikidata.org/wiki/Q327657","display_name":"Rheumatology","level":2,"score":0.25859999656677246},{"id":"https://openalex.org/C36454342","wikidata":"https://www.wikidata.org/wiki/Q245341","display_name":"Radiography","level":2,"score":0.25209999084472656}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.3329/medtoday.v38i2.94514","doi":"https://doi.org/10.3329/medtoday.v38i2.94514","is_oa":true,"landing_page_url":"https://doi.org/10.3329/medtoday.v38i2.94514","pdf_url":"https://www.banglajol.info/index.php/MEDTODAY/article/download/94514/60756","source":{"id":"https://openalex.org/S4210195798","display_name":"Medicine Today","issn_l":"1810-1828","issn":["1810-1828","2408-8714"],"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Medicine Today","raw_type":"journal-article"}],"best_oa_location":{"id":"doi:10.3329/medtoday.v38i2.94514","doi":"https://doi.org/10.3329/medtoday.v38i2.94514","is_oa":true,"landing_page_url":"https://doi.org/10.3329/medtoday.v38i2.94514","pdf_url":"https://www.banglajol.info/index.php/MEDTODAY/article/download/94514/60756","source":{"id":"https://openalex.org/S4210195798","display_name":"Medicine Today","issn_l":"1810-1828","issn":["1810-1828","2408-8714"],"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Medicine Today","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9940000176429749}],"study_designs":[{"id":"https://openalex.org/study-designs/case-report","display_name":"Case Report"}],"awards":[],"funders":[],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7225110211.pdf","grobid_xml":"https://content.openalex.org/works/W7225110211.grobid-xml"},"referenced_works_count":16,"referenced_works":["https://openalex.org/W1465240559","https://openalex.org/W1527515968","https://openalex.org/W1964950849","https://openalex.org/W2012708618","https://openalex.org/W2028159669","https://openalex.org/W2058919544","https://openalex.org/W2076986495","https://openalex.org/W2087874744","https://openalex.org/W2104341765","https://openalex.org/W2142348665","https://openalex.org/W2307512719","https://openalex.org/W2894213534","https://openalex.org/W2923697016","https://openalex.org/W4323046886","https://openalex.org/W4380682209","https://openalex.org/W4386293567"],"related_works":["https://openalex.org/W1465240559","https://openalex.org/W2168361121","https://openalex.org/W2890898709","https://openalex.org/W7210289073","https://openalex.org/W2468977356","https://openalex.org/W2075602075","https://openalex.org/W3187421894","https://openalex.org/W2021679388","https://openalex.org/W4409295937","https://openalex.org/W2995817659"],"abstract_inverted_index":{"Primary":[0],"hypertrophic":[1,112,183],"osteoarthropathy":[2],"(PHO),":[3],"also":[4],"known":[5],"as":[6],"pachydermoperiostosis":[7],"(PDP),":[8],"is":[9],"a":[10],"rare":[11],"hereditary":[12],"disorder":[13],"characterized":[14],"by":[15],"digital":[16,91],"clubbing,":[17],"periostosis,":[18,97],"pachydermia,":[19,99],"and":[20,26,37,45,70,93,102,119,128,133,153,164,168,185],"variable":[21,57],"systemic":[22],"manifestations1-4.":[23],"Familial":[24],"occurrence":[25],"genetic":[27],"heterogeneity":[28],"have":[29],"been":[30],"described,":[31],"particularly":[32],"involving":[33],"variants":[34],"in":[35,107,142,148,162],"HPGD":[36],"SLCO2A1,":[38],"genes":[39],"related":[40],"to":[41,82],"prostaglandin":[42],"E2":[43],"metabolism":[44],"transport4-7.":[46],"We":[47],"report":[48],"five":[49],"patients":[50,89,158],"from":[51,80],"familial":[52,179],"clusters":[53],"who":[54],"presented":[55],"with":[56,85],"manifestations":[58],"of":[59,96,111,177],"PDP.":[60],"Clinical":[61],"findings,":[62],"radiographic":[63],"features,":[64],"laboratory":[65],"investigations,":[66],"family":[67],"history,":[68],"treatment,":[69],"1-year":[71],"outcomes":[72],"were":[73,122],"reviewed.":[74],"The":[75],"age":[76],"at":[77],"evaluation":[78],"ranged":[79],"18":[81],"70":[83],"years,":[84],"male":[86],"predominance.":[87],"All":[88],"had":[90],"clubbing":[92],"radiological":[94],"evidence":[95],"while":[98],"hyperhidrosis,":[100],"arthralgia,":[101],"cutis":[103],"verticis":[104],"gyrata":[105],"varied":[106],"severity.":[108],"Secondary":[109],"causes":[110],"osteoarthropathy,":[113,184],"including":[114],"pulmonary,":[115],"cardiac,":[116],"malignant,":[117],"infectious,":[118],"endocrine":[120],"causes,":[121],"excluded":[123],"through":[124],"targeted":[125],"clinical":[126],"assessment":[127],"investigations8-12.":[129],"Management":[130],"was":[131],"symptomatic":[132,160],"included":[134],"non-steroidal":[135],"anti-inflammatory":[136],"drugs,":[137],"selective":[138],"COX-2":[139],"inhibitors,":[140],"corticosteroids":[141],"selected":[143],"inflammatory":[144],"episodes,":[145],"bisphosphonate":[146],"therapy":[147],"severe":[149],"bone":[150],"pain,":[151],"physiotherapy,":[152],"supportive":[154,188],"dermatological":[155],"care11,13,19,20.":[156],"Most":[157],"achieved":[159],"improvement":[161],"pain":[163],"function,":[165],"although":[166],"hyperhidrosis":[167],"pachydermia":[169],"persisted.":[170],"This":[171],"case":[172],"series":[173],"emphasizes":[174],"the":[175],"importance":[176],"recognizing":[178],"PDP,":[180],"excluding":[181],"secondary":[182],"providing":[186],"individualized":[187],"treatment.":[189],"Medicine":[190],"Today":[191],"2026,":[192],"Vol.38":[193],"(2):":[194],"336-339":[195]},"counts_by_year":[],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2026-11-03T00:00:00"}
