Sphenoid sinus agenesis and sella turcica hypoplasia: very rare cases of two brothers with Hamamy syndrome

AbstractHamamy syndrome (HS) is an autosomal recessive syndrome with a genetic origin that is very rarely observed. The syndrome with craniofacial dysmorphisms, including midface prominence, severe telecanthus, sparse lateral eyebrows, protruding ears, fronto-nasal abnormalities, lacrimal –salivary apparatus agenesis, thin upper vermillion border, myopia, mental retardation, sensorineural hearing impairment, congenital heart anomalies with intraventricular conduction delay, hypochromic microcytic anaemia and skeletal abnormalities of the long bones with recurrent fractures. In this paper, we report a case of two brothers diagnosed with HS at the ages of 25 and 18 years, visited out clinic at different times due to dental reasons. In the radiological examinations, it was observed that both brothers have sphenoid sinuses agenesia, and their sella turcica were smaller than norma l. HS may be observed very rarely, and it should be kept in mind that, in addition to various symptoms, it may also cause sphenoid sinus agenesis and sella turcica hypoplasia as shown for the first time in this case report.
Source: Surgical and Radiologic Anatomy - Category: Anatomy Source Type: research

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Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
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Source: Mutation Research Reviews in Mutation Research - Category: Genetics & Stem Cells Source Type: research
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Source: Anemia - Category: Hematology Tags: Anemia Source Type: research
Authors: Tamai H, Shingaki N, Ida Y, Shimizu R, Maeshima S, Okamura J, Kawashima A, Nakao T, Hara T, Matsutani H, Nishikawa I, Higashi K Abstract BACKGROUND: Although clinical use of sofosbuvir plus ribavirin has been approved for patients infected with genotype 2 hepatitis C virus, patients ≥ 75-years-old have not been included in previous clinical trials. AIM: To evaluate the real-world safety and efficacy of sofosbuvir plus ribavirin for elderly patients (≥ 75-years-old) compared to nonelderly patients, we conducted a post-marketing prospective cohort study. METHODS: We treated 265 patients with ge...
Source: World Journal of Hepatology - Category: Gastroenterology Tags: World J Hepatol Source Type: research
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Source: Evidence-based Complementary and Alternative Medicine - Category: Complementary Medicine Tags: Evid Based Complement Alternat Med Source Type: research
AbstractPurpose of ReviewWith increasing use of prosthetic valves to treat degenerative valvular heart disease (VHD) in an aging population, the incidence and adverse consequences of paravalvular leaks (PVL) are better recognized. The present work aims to provide a cohesive review of the available literature in order to better guide the evaluation and management of PVL.Recent FindingsDespite gains in operator experience and design innovation, significant PVL remains a significant complication that may present with congestive heart failure and/or hemolytic anemia. To date, clear consensus or guidelines on the evaluation and...
Source: Current Cardiology Reports - Category: Cardiology Source Type: research
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Source: Current Cardiology Reports - Category: Cardiology Source Type: research
Background Fat grafting is a common adjunct procedure used to treat temporal fat hollowing in children with craniofacial anomalies. The goal of this study was to assess the surgical and aesthetic outcomes of this procedure. Methods We retrospectively reviewed patients who underwent temporal fat grafting at a single tertiary pediatric craniofacial center. The surgical outcome was assessed based on intraoperativeand 30-day postoperative complication rates. The aesthetic outcome was assessed by 3 independent investigators using a 3-point grading scale for preoperative temporal hollowing severity (1 = mild, 2 = moderate, ...
Source: Annals of Plastic Surgery - Category: Cosmetic Surgery Tags: Head and Neck Surgery Source Type: research
Since its first introduction, autologous fat grafting (AFG) has undergone multiple refinements and has become a common treatment option within plastic surgery. It is a safe, quick, economical, and minimally invasive method for volume augmentation and is finding its own indications in the pediatric craniofacial surgery patients. Because there is currently a paucity of literature regarding the use of AFG in the pediatric population compared with the adults, the authors performed a systematic review of the literature using PubMed, Cochrane Library databases, and the Journal of Plastic and Reconstructive Surgery using the term...
Source: Annals of Plastic Surgery - Category: Cosmetic Surgery Tags: Review Papers Source Type: research
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Source: Annals of Plastic Surgery - Category: Cosmetic Surgery Tags: Letters and Commentary Source Type: research
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