ORIGINAL RESEARCH ARTICLE | Nov. 10, 2023
To Determine the Incidence of Various Subtypes of VWD with Specified VWF:RCof/ VWF:Ag Ratio’s In Pediatric Patients
Mirza Asif Baig, Anas M. Khan, Ameen D. Bakhsh, Thamer Ali Aljohani, Ahmed al Mutairi, Afrah S. Alharbi, Abrar Aljohani, Ahmed A. Alenezi, Abdul Rahman A, Abdulrahim A, Ayshah M. Mostafa, Ghadeer aljohani, Zaraah sofiyani, Mona altarqyi, Fayza Ahmed
Page no 263-267 |
DOI: 10.36348/sjpm.2023.v08i11.001
Background: VWD is the most common inherited bleeding disorder characterized by defects in the concentration, structure, or function VWF. There are three main types of VWD that differ according to the degree of disease severity and inheritance pattern (Type 1, Type 2A, 2B, 2M, 2N and Type 3). Methodology: This is 05 years study (Feb 2016–Jan 2021) conducted in hematology section. In present study, amongst total inherited bleeding disorders, Incidence of VWD is 27%, Hemophilia (50%), inherited platelet disorders (14%) and remainder are undiagnosed. Amongst VWD, Type 1 VWD is the most common subtype studied, comprising 66% of total cases. Type 2 & 3 VWD comprised 23% and 10% of total cases showing good correlation with the literature and other studies. Discussion: The European cross-sectional study yielded a population-based estimate of 0.05 per 100,000 for type 3 VWD. Estimates by VWD type from Europe and Western Pacific reported higher prevalence estimates for type 1 disease than type 2 or type 3: 2.7–7.2 per 100,000 for type 1 VWD, 0.8–2.5 per 100,000 for type 2, and 0.1–0.3 per 100,000 for type 3. Conclusion: VWD usually presents with mild bleeding symptoms (except in type 3 VWD) the diagnosis is often delayed. Prompt diagnosis and management can help to avoid potentially life-threatening bleeding events and unnecessary exposure to blood products.
CASE REPORT | Nov. 16, 2023
Esophageal Plexiform Fibromyxoma: An Extremely Rare Localization
Taha Yassine Aaboudech, Kaoutar Znati, Ahmed Jahid, Zakiya Bernoussi, Fouad Zouaidia
Page no 268-272 |
DOI: 10.36348/sjpm.2023.v08i11.002
Plexiform fibromyxoma (PFM) is a rare gastrointestinal tumor, primarily found in the stomach. Esophageal PFM is exceptionally rare. We describe a case of a mid-20s woman with respiratory and swallowing difficulties, revealing a 105x65 mm upper thoracic esophageal submucosal tumor during endoscopy. Biopsy lacked histological evidence of gastrointestinal stromal tumors (GISTs). Post-tumor removal histopathology showed a spindle tumor with plexiform architecture and myxoid-vascular stroma. Immunohistochemistry revealed vimentin and alpha-smooth muscle actin expression, while desmin, c-kit, DOG1, and CD34 were absent, confirming PFM. No recurrence or metastasis appeared during a 6-month follow-up. This case underscores the extreme rarity of esophageal PFM, emphasizing the need for precise diagnostic tools to navigate challenging differential diagnosis.
CASE REPORT | Nov. 21, 2023
GOC in Maxillary Anterior Jaw: An Infrequent Case Report
Dr. Gauri Bargoti, Dr. Aviral Verma, Dr. Ipshita Yadav, Dr. Amit Gupta
Page no 273-277 |
DOI: 10.36348/sjpm.2023.v08i11.003
Padayachee and Van Wyk in 1987 first documented the case of Glandular odontogenic cyst (GOC) and regarded this as ‘sialo-odontogenic cyst’ they published two cases that mimicked botryoid odontogenic cyst and the central mucoepidermoid tumor of jaws. Glandular odontogenic cyst (GOC) is counted as infrequent developmental odontogenic cyst of jaw that has the incidence of 0.012 to 1.3% only GOC is very rare with only 111 cases have been documented in the English literate till date. This case report in unique as this patient presented with the painless swelling in maxillary anterior region.