Left Main Coronary Artery Aneurysm.

Aneurysms of the left main coronary artery are exceedingly rare clinical entities, encountered incidentally in approximately 0.1% of patients who undergo routine angiography. The most common cause of coronary artery aneurysms is atherosclerosis. Angiography is the gold standard for diagnosis and treatment. Depending on the severity of the coexisting coronary stenosis, patients with left main coronary artery aneurysms can be effectively managed either surgically or pharmacologically. We herein report a case of left main coronary artery aneurysm in a 72-year-old man with a prior history of hypertension presenting to our hospital because of unstable angina. The electrocardiogram showed ST-segment depression and T-wave inversion in the precordial leads. All the data of blood chemistry were normal. Echocardiography showed akinetic anterior wall, septum, and apex, mild mitral regurgitation and ejection fraction of 45%. Coronary angiography revealed a saccular aneurysm of the left main coronary artery with significant stenosis in the left anterior descending, left circumflex, and right coronary artery. The patient immediately underwent coronary artery bypass grafting and ligation of the aneurysm. At six months' follow-up, he remained asymptomatic.

Successful use of central venous catheters in the management of recurrent malignant pleural effusions: one new option.

BACKGROUND: Malignant pleural effusion (MPE) is a common clinical problem in patients with malignancy. To date, placement of various catheters has been suggested as an effective alternative method for traditional treatment of recurrent MPE. In this study, we report our experience in managing treatment of recurrent MPE by placing a central vein catheter without a radiologic guide. METHODS: Patients with recurrent MPE who underwent triple-lumen central vein catheter insertion (2010-2013) were retrospectively reviewed. Clinical, procedural, complication, and outcome details were analyzed. Patients were carefully selected, and the central catheters were inserted as a palliative measure. We assessed the quality of life of patients using the EORTC QLQ-C30. RESULTS: A total of 84 patients with recurrent MPE were enrolled in this study. Fifty-six males and 28 females with mean age of 57.8 ± 12.4 years old underwent the procedure. There were no preoperative or postoperative complications related to the procedure. The EORTC QLQ-C30 questionnaire showed a significant improvement following catheter placement in symptom scales at 30 days (p = 0.01) and at 60 days (p = 0.002). CONCLUSIONS: Triple-lumen central catheter insertion is a simple, noninvasive option in patients with recurrent MPE that can be performed the patient's bedside. Further research is needed to confirm the results and to assess the impact of central catheter insertion on the quality of life of these patients.

Diabetes mellitus, diabetes insipidus, optic atrophy, and deafness: A case of Wolfram (DIDMOAD) syndrome.

PURPOSE: To report a case of Wolfram syndrome (WS) characterized by diabetes mellitus, diabetes insipidus, progressive optic atrophy, and deafness. CASE REPORT: A 19-year-old female patient, a known case of diabetes mellitus type I from six years before, presented with progressive vision loss since four years earlier. On fundoscopic examination, she had bilateral optic atrophy without diabetic retinopathy. The patient also had diabetes insipidus, neurosensory deafness, and neurogenic bladder. CONCLUSION: WS should be considered a differential diagnosis in patients with diabetes mellitus who present with optic atrophy, and it is necessary to perform a hearing test as well as collecting 24-h urine output.

Papillary carcinoma of the thyroglossal duct cyst: case report.

Thyroglossal duct cysts are the most common form of congenital cysts on the neck. The incidence of thyroid papillary carcinoma in thyroglossal duct cyst is less than 1%. In most cases the diagnosis is made postoperatively. We present a 22-year-old female with thyroid papillary carcinoma arising from thyroglossal duct cyst,identified in pathologic study after sistrunk operation.In our case there was neither invasion to adjacent tissue nor lymph node involvement.The patient then underwent total thyroidectomy and bilateral neck dissection. The patient was treated with radioactive iodide and thyroid suppression therapy was given as adjuvant treatment.The patient has been following for two years without any metastasis.

Eosinophilic ascites and duodenal obstruction in a patient with liver cirrhosis.

Eosinophilic gastroenteritis (EG) is a rare disease characterized by eosinophilic infiltration of portions of the gastrointestinal tract. Eosinophilic ascites is probably the most unusual and rare presentation of EG and is generally associated with the serosal form of EG. Hereby, we report a case of eosinophilic ascites with duodenal obstruction in a patient with liver cirrhosis. A 50-year-old woman was admitted to our hospital because of abdominal pain, nausea, bloating, and constipation. She had a history of laparotomy because of duodenal obstruction 2 years ago. Based on clinical, radiological, endoscopic, and pathological findings, and given the excluding the other causes of peripheral eosinophilia, the diagnosis of eosinophilic gastroenteritis along with liver cirrhosis and spontaneous bacterial peritonitis was established. Based on the findings of the present case, it is highly recommended that, in the patients presented with liver cirrhosis associated with peripheral blood or ascitic fluid eosinophilia, performing gastrointestinal endoscopy and biopsy can probably reveal this rare disorder of EG.

Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.

Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement.

The Relationship between Nocturnal Hypoxemia and Left Ventricular Ejection Fraction in Congestive Heart Failure Patients.

Congestive heart failure (CHF) is a major cause of mortality and morbidity. Among patients with heart failure, sleep disordered breathing (SDB) is a common problem. Current evidence suggests that SDB, particularly central SDB, is more prevalent in patients with CHF than in the general population, but it is underdiagnosed as SDB symptoms that are less prevalent in CHF. The main aims of this study were to determine the relationship between nocturnal hypoxemia and left ventricular ejection fraction in patients with chronic heart failure. By means of echocardiography, 108 patients with left ventricular ejection fraction ≤45% were divided into mild, moderate, and severe CHF. Hypoxemia was recorded overnight in the hospital and was measured by portable pulse oximetry. In the 108 patients with CHF, 44 (40.7%) were severe, 17 (15.7%) moderate, and 47 (43.6%) mild CHF. 95 (88%) of patients with CHF had abnormal patterns of nocturnal hypoxemia suggestive of Cheyne-Stokes respiration. Ejection fraction correlated negatively with dip frequency. There was no correlation between nocturnal hypoxemia with BMI and snoring. This study confirms strong associations between sleep apnea and heart disease in patients with CHF. Overnight oximetry is a useful screening test for Cheyne-Stokes respiration in patients with known heart failure.

The diagnostic value of ultrasound compared with nerve conduction velocity in carpal tunnel syndrome.

OBJECTIVE: Carpal tunnel syndrome (CTS) is the most common form of peripheral entrapment neuropathy. The use of sonography for investigation and diagnosis of musculoskeletal conditions has been rapidly increasing over the past few decades. The purpose of this study was to determine whether sonography can be an alternative method to nerve conduction study (NCS) in the diagnosis of CTS. METHODS: Individuals with electrodiagnostically proven CTS and healthy control subjects were enrolled prospectively. Median nerve cross-sectional area (CSA) and flattening ratio (FR) at three different levels, proximal to tunnel inlet, at tunnel inlet and tunnel outlet, and flexor retinaculum thickness, were measured. Then, comparisons between ultrasonography and NCS were made. RESULTS: We assessed 180 wrists, of which 120 were electrophysiologically confirmed as CTS diseased hands and 60 nondiseased hands in 90 patients (83 women and seven men). The mean median nerve CSA at the tunnel inlet was 13.31 ± 3.23 mm(2) in CTS diseased hands and 8.57 ± 0.82 mm(2) in nondiseased hands. Post hoc comparisons between the diseased and nondiseased hands demonstrated that the CSA at various levels of the median nerve were significantly greater in the CTS diseased hands than the nondiseased hands (P = 0.001). CSA at the tunnel inlet with a threshold of 9.15 mm(2) gave the best diagnostic accuracy with a sensitivity and specificity of 99.2% and 88.3%, respectively. The difference in cross-sectional area of the median nerve in mild, moderate and severe CTS was statistically significant. CONCLUSION: Ultrasonographic measurement of the CSA of the median nerve at the carpal tunnel inlet is useful in diagnosing and grading CTS.

Alkaptonuric ochronosis: a clinical study from Ardabil, Iran.

OBJECTIVE: Ochronosis is a term used to describe pigment deposition that occurs in the connective tissues of patients with alkaptonuria, an autosomal recessive disorder that results from a deficiency of homogentisic acid oxidase. Brown or blue-gray discoloration of the skin may be seen on the axillary and inguinal areas, face, palms or soles. In addition, blue-black discoloration can be apparent on skin overlying cartilage in which the pigment is deposited, such as the ears. The sclerae are also typically involved. The cheapest screening test to perform prior to expensive lab tests is the urine oxidation test: having it standing in light for a period of 24 h when suspicion has risen. METHODS: Retrospective analysis of patients with ochronotic arthropathy seen between September 2011 to September 2013 was carried out. RESULTS: Seven patients (four male, three female) with ochronotic arthropathy were seen, their mean age was 46.1 years. All patients had bluish-black pigmentations of the ear cartilage and sclera. Spondylosis was seen in all, whereas peripheral arthritis was present in five patients. Moderate aortic insufficiency and calcification of the aortic valve was detected in one male patient. Urine screening for homogentisic acid was positive in all seven patients. CONCLUSION: Alkaptonuria is a rare autosomal recessive disorder of the metabolism caused by deficiency of homogentisic acid oxidase. It is suggested that more widespread screening should be undertaken in order to assess the true incidence of the disorder.

Chronic constrictive pericarditis.

Constrictive pericarditis (CP) is a rare clinical entity that can pose diagnostic problems. The diagnosis of CP requires a high degree of clinical suspicion. The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curves, which are high and, in end diastole, equal in all chambers. We present a patient with unexplained dyspnea, recurrent right-side pleural effusion, and ascites. Analysis of the ascitic fluid revealed a high protein content and an elevated serum-ascites gradient. Echocardiography, computed tomography, and cardiac catheterization revealed the diagnosis of CP. He underwent complete pericardiectomy and to date has made a good recovery. The diagnosis of CP is often neglected by admitting physicians, who usually attribute the symptoms to another disease process. This case exemplifies the difficulty in diagnosing this condition, as well as the investigation required, and provides a discussion of the benefit and outcomes of prompt treatment.

Seizure, deafness, and renal failure: a case of barakat syndrome.

Barakat syndrome (also known as HDR syndrome) is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness, and renal disease caused by mutation of the GATA3 gene located at chromosome 10p15. The exact prevalence of this disorder is not known but is very rare, with only about a dozen cases reported in the literature. Here, we report a case of 58-year-old man from Ardabil who presented with seizure due to hypocalcemia. Further history revealed bilateral deafness. Audiogram confirmed sensorineural hearing loss of both sides. His laboratory data were consistent with hypoparathyroidism and renal failure. He was diagnosed to have Barakat syndrome based on his clinical and laboratory data. In conclusion, we need to be aware of rare inherited conditions in a patient with abnormal physical and laboratory findings even though their initial presentation was seizure and hypocalcemia.

5-alpha-reductase 2 deficiency in a woman with primary amenorrhea.

Steroid 5-alpha-reductase 2 deficiency is a rare disorder leading to male pseudohermaphroditism, a condition characterized by incomplete differentiation of male genitalia in 46,XY patients. Here, we report a case of a 21-year-old woman from Ardabil who presented with primary amenorrhea, ambiguous genitalia, and lack of breast development. All of the serum hormone profiles were normal except for raised serum total testosterone. Testosterone to DHT ratio (T/DHT) was elevated before (15.72) and further increased after hCG stimulation (32.46). A chromosomal study revealed a 46,XY karyotype. A bilateral gonadectomy, recessive cliteroplasty, urethroplasty, and vaginoplasty were performed and hormonal replacement therapy using estrogen was started. In conclusion, the diagnosis of 5-alpha-reductase 2 deficiency may be suspected in infants with ambiguous genitalia or in adolescents or young adults with the characteristic phenotype and serum hormone profiles.