- Lingual amyloidosis associated to long-term hemodialysis: Two case reports. [Case Reports]
- SCSpec Care Dentist 2018 Sep 14
- The β2-microglobulin amyloidosis is a newly recognized type of systemic amyloidosis, which occurs in patients with chronic renal failure undergoing long-term hemodialysis. The dialysis-related amyloi...
The β2-microglobulin amyloidosis is a newly recognized type of systemic amyloidosis, which occurs in patients with chronic renal failure undergoing long-term hemodialysis. The dialysis-related amyloidosis (DRA) of the tongue is an uncommon and late complication, which occurs in patients undergoing chronic hemodialysis for more than 20 years. The aim of this paper is to report two cases of lingual amyloidosis associated with long-term hemodialysis. Two patients were referred to the Oral Medicine Clinic due to the presence of multiple nodules on the tongue and macroglossia. The patients had history of chronic renal failure and had been dialysed for more 20 years with bio-incompatible cellulose membrane. Biopsies of the lesions were performed and the histopathological features and Congo red stain revealed oral amyloidosis. Both patients remain in clinical follow-up after 12 and 6 months, respectively. Recognition of the amyloidosis tongue lesions can minimize the complications caused by this condition such as obstruction of the upper airways, making it imperative to carry out early treatment. Furthermore, in patients with oral amyloidosis, lesions in areas of trauma may ulcerate and cause pain and dysphagia becoming necessary periodic surgical removal and regular follow-up of these patients.
- GeneReviews® [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- 6q24-related transient neonatal diabetes mellitus (6q24-TNDM) is defined as transient neonatal diabetes mellitus caused by genetic aberrations of the imprinted locus at 6q24. The cardinal features ar...
6q24-related transient neonatal diabetes mellitus (6q24-TNDM) is defined as transient neonatal diabetes mellitus caused by genetic aberrations of the imprinted locus at 6q24. The cardinal features are: severe intrauterine growth retardation, hyperglycemia that begins in the neonatal period in a term infant and resolves by age 18 months, dehydration, and absence of ketoacidosis. Macroglossia and umbilical hernia may be present. 6q24-TNDM associated with a multilocus imprinting disturbance (MLID) can be associated with marked hypotonia, congenital heart disease, deafness, neurologic features including epilepsy, and renal malformations. Diabetes mellitus usually starts within the first week of life and lasts on average three months but can last longer than a year. Although insulin is usually required initially, the need for insulin gradually declines over time. Intermittent episodes of hyperglycemia may occur in childhood, particularly during intercurrent illnesses. Diabetes mellitus may recur in adolescence or later in adulthood. Women who have had 6q24-TNDM are at risk for relapse during pregnancy.
- Beckwith-Wiedemann syndrome: clinical and etiopathogenic aspects of a model genomic imprinting entity. [Journal Article]
- AAArch Argent Pediatr 2018 Oct 01; 116(5):368-373
- The Beckwith-Wiedemann syndrome is the most common genetic entity in overgrowth, with an approximate incidence of 1 in 10 00013 700births. Its broad clinical spectrum includes pre- and postnatal macr...
The Beckwith-Wiedemann syndrome is the most common genetic entity in overgrowth, with an approximate incidence of 1 in 10 00013 700births. Its broad clinical spectrum includes pre- and postnatal macrosomia, macroglossia, pinna abnormalities, abdominal wall defects, visceromegaly, and hyperinsulinemic hypoglycemia. This syndrome predisposes to childhood cancer and is caused by diverse genetic and/or epigenetic disorders that usually affect the regulation of genes imprinted on chromosome 11p15.5. The knowledge of (epi) genotype-phenotype correlations has prompted recommendations to propose different health care strategies, including tumor surveillance protocols based on molecular classification, aimed at standardizing clinical practice. The objective of this article is to describe the current status of the Beckwith-Wiedemann syndrome, a model of genomic imprinting.
- Frequency of Pathologic Changes in the Oral Cavity in Patients Subjected to Long-term Pharmacologic Immunosuppressive Therapy After Kidney, Liver, and Hematopoietic Cell Transplantation. [Journal Article]
- TPTransplant Proc 2018; 50(7):2176-2178
- CONCLUSIONS: Both the patients after organ and hematopoietic stem cells transplantations and those qualified for a transplant should undergo multispecialty treatment, particularly dental treatment, to enable the detection of pathologies at an early stage and commencement of effective therapy. Cooperation between the main doctor and the dentist is crucial in the process of treatment of this group of patients.
- Toothache as the initial symptom of plasma cell myeloma. [Journal Article]
- EJEur J Dent 2018 Jul-Sep; 12(3):443-445
- Patients with plasma cell myeloma may initially present to their dentists or dental surgeons with toothache, loose teeth, or gingival masses. An X-ray of the jaw can reveal osteolyses. In addition, a...
Patients with plasma cell myeloma may initially present to their dentists or dental surgeons with toothache, loose teeth, or gingival masses. An X-ray of the jaw can reveal osteolyses. In addition, accumulation of monoclonal light chains in AL-amyloidosis can lead to macroglossia. It is prudent that the dentist or dental surgeon recognizes the underlying disease and refers the patient to the oncologist or hematologist for further workup to prevent the complications of plasma cell myeloma such as renal impairment, fractures, bone pain, infections, hypercalcemia, anemia, or heart failure. Another area where the dentist or dental surgeon is involved with patients suffering from plasma cell myeloma is prevention and therapy of osteonecrosis of the jaw, occurring after administration of bisphosphonates or denosumab for osteolytic bone disease. The case report presented here shows a patient complaining of toothache for whom recognition of a systemic disease by the dentist led to the diagnosis of plasma cell myeloma, highlighting the need for interdisciplinary cooperation. As recent years have seen many changes in the management of patients with plasma cell myeloma, an update for dentists and dental surgeons is warranted.
- Adenoma size and postoperative IGF-1 levels predict surgical outcomes in acromegaly patients: results of the Swiss Pituitary Registry (SwissPit). [Journal Article]
- SMSwiss Med Wkly 2018 Aug 13; 148:w14653
- CONCLUSIONS: This small, observational registry study showed a relevant success rate of initial pituitary surgery in patients with confirmed acromegaly. Initial tumour size and postoperative IGF-1 levels help to risk stratify patients regarding expected outcomes. In the case of disease persistence, a multimodal approach using drug and radiotherapy is mandatory.
- Anesthesia Management of Vitrectomy in a Patient with Sturge-Weber Syndrome. [Journal Article]
- TTanaffos 2018; 17(1):57-61
- Sturge-Weber syndrome (SWS) is a neurocutaneous disorder, characterized by leptomeningeal angiomas involving the oral cavity, trachea, larynx, and face. Herein, we present a case of vitrectomy in a s...
Sturge-Weber syndrome (SWS) is a neurocutaneous disorder, characterized by leptomeningeal angiomas involving the oral cavity, trachea, larynx, and face. Herein, we present a case of vitrectomy in a seven-year-old boy with SWS. The patient showed hemangioma on the left side of his face, as well as mental retardation and epilepsy. Preoperative examination revealed no apparent hemangioma in the oral cavity, pharynx, larynx, or trachea. However, he was predicted to have difficult airway intubation, as the oral cavity was smaller than the normal size. The minimum Mallampati score was 3-4 due to macroglossia. First, we applied awake intubation, but he failed to follow the commands. We proceeded to general anesthesia with propofol and did not use any muscle relaxants to maintain spontaneous breathing. A laryngeal mask airway was inserted to minimize any harm to possible oral angiomas. The patient was hemodynamically stable and extubated without any complications, such as bleeding or respiratory problems.
- Tongue Reduction Surgery and Feeding Difficulties in Infants With Beckwith Wiedemann Syndrome: A Case Series. [Journal Article]
- CPCleft Palate Craniofac J 2018 Aug 15; :1055665618794070
- CONCLUSIONS: Feeding difficulties are common pre-operatively in m-BWS, putting infants at risk of aspiration if left unmanaged. TRS was effective in reducing or eliminating them. This is the first systematic report of infant feeding in m-BWS pre- and post-TRS.
- Magnetic resonance imaging of obstructive sleep apnea in children. [Review]
- PRPediatr Radiol 2018; 48(9):1223-1233
- Sleep-disordered breathing has a spectrum of severity that spans from snoring and partial airway collapse with increased upper airway resistance, to complete upper airway obstruction with obstructive...
Sleep-disordered breathing has a spectrum of severity that spans from snoring and partial airway collapse with increased upper airway resistance, to complete upper airway obstruction with obstructive sleep apnea during sleeping. While snoring occurs in up to 20% of children, obstructive sleep apnea affects approximately 1-5% of children. The obstruction that occurs in obstructive sleep apnea is the result of the airway collapsing during sleep, which causes arousal and impairs restful sleep. Adenotonsillectomy is the first-line treatment of obstructive sleep apnea and is usually effective in otherwise healthy nonsyndromic children. However, there are subgroups in which this surgery is less effective. These subgroups include children with obesity, severe obstructive sleep apnea preoperatively, Down syndrome, craniofacial anomalies and polycystic ovarian disease. Continuous positive airway pressure (CPAP) is the first-line therapy for persistent obstructive sleep apnea despite previous adenotonsillectomy, but it is often poorly tolerated by children. When CPAP is not tolerated or preferred by the family, surgical options beyond adenotonsillectomy are discussed with the parent and child. Dynamic MRI of the airway provides a means to identify and localize the site or sites of obstruction for these children. In this review the authors address clinical indications for imaging, ideal team members to involve in an effective multidisciplinary program, basic anesthesia requirements, MRI protocol techniques and interpretation of the findings on MRI that help guide surgery.
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- Atorvastatin-Associated Macroglossia in a Cardioembolic Stroke Patient. [Journal Article]
- APAnn Pharmacother 2018 Jul 30; :1060028018793111