Biomedical subjects
Publications and source records attributed to S V Yildirim.
Proboscis lateralis is a rare facial anomaly resulting in incomplete formation of one side of the nose. We report a left-sided proboscis lateralis case of a 9 months old male with left-sided heminasal aplasia, microphthalmi, coloboma iris and retina and an arachnoid cyst in the temporal fossa of the brain. We present the clinical, radiological features of this unusual case.
Behçet's disease is a rare condition in children and characterized by a triad of recurrent aphthous stomatitis, genital ulceration and uveitis. The aetiology is unknown, but systemic vasculitis is the main pathology. The oral lesions resemble Sutton disease, which is another form of recurrent aphthous stomatitis (RAS) that composes approximately 10 per cent of cases and is known as major aphthous stomatitis, can easily be overlooked if the other symptoms are not questioned. We presented a 12-year-old girl with the initial complaint of recurrent aphthous stomatitis. During the follow-up period, we observed the other cardinal signs of the Behçet's disease.
Amiodarone is an effective antiarrhythmic agent that is widely used for tachyarrhythmias, especially ventricular tachycardia and supraventricular tachycardia. It has some mild short-term (e.g., skin rashes, gastrointestinal symptoms, and corneal microdeposits) and long-term side effects (thyroid dysfunction, visual disturbances, pulmonary infiltrates, ataxia, and hepatitis). We present two infants who had hyperglycemia following amiodarone infusion during the early postoperative period.
Supraventricular tachycardia (SVT) is the most common type of arrhythmia observed in children, especially in newborns. Infants with severe SVT must be treated immediately with first-line drugs such as amiodarone. There are some minor and major side effects of amiodarone in this patient group, but no associated electrolyte disorders have been observed. This report describes a newborn whose recurrent SVT attacks during amiodarone treatment were suspected to have been caused by hyperkalemia.
Müllerian agenesis is characterized by the absence of the fallopian tubes, uterus and internal portion of the vagina. Patients have normal female phenotype and genotype, with normal secondary sex characteristics but with amenorrhea. We report a family in which müllerian agenesis was diagnosed in three siblings and their two paternal aunts. This family was ascertained when the proband was evaluated for primary amenorrhea. She had normal secondary sexual development. Her karyotype was 46, XX. Ultrasound examination and magnetic resonance imaging of the pelvis revealed absence of the uterus and vagina. The proband had three sisters and two of them showed similar physical and radiological findings. Two of the proband's paternal aunts had no uterus. Although the pathogenesis of müllerian agenesis is well understood, the etiology and genetics are still unknown. Various forms of inheritance patterns have been suggested by several authors. In conclusion, it would appear that müllerian agenesis is influenced by multifactorial inheritance and polygenic and familial factors.