Radiological measurement of cochlear dimensions in cochlear hypoplasia and its effect on cochlear implant selection.

OBJECTIVE: To determine the effect of cochlear dimensions on cochlear implant selection in cochlear hypoplasia patients. METHODS: Temporal bone computed tomography images of 36 patients diagnosed with cochlear hypoplasia between 2010 and 2016 were retrospectively reviewed and compared with those of 40 controls without sensorineural hearing loss. RESULTS: Basal turn length and mid-modiolar height were significantly lower in the cochlear hypoplasia patients with subtypes I, II and III than in the control group (p < 0.001). Mid-scalar length was significantly shorter in subtype I-III patients as compared with the control group (p < 0.001). In addition, cochlear canal length (measured along the lateral wall) was significantly shorter in subtype I-IV patients than in the control group (subtypes I-III, p < 0.001; subtype IV, p = 0.002). CONCLUSION: Cochlear hypoplasia should be considered if basal turn length is less than 7.5 mm and mid-modiolar height is less than 3.42 mm. The cochlear implant should be selected according to cochlear hypoplasia subgroup. It is critically important to differentiate subtype II from incomplete partition type I and subtype III from a normal cochlea, to ensure the most appropriate implant electrode selection so as to optimise cochlear implantation outcomes.

A study on modelling cochlear duct mid-scalar length based on high-resolution computed tomography, and its effect on peri-modiolar and mid-scalar implant selection.

OBJECTIVE: To determine cochlear duct mid-scalar length in normal cochleae and its role in selecting the correct peri-modiolar and mid-scalar implant length. METHODS: The study included 40 patients with chronic otitis media who underwent high-resolution computed tomography of the temporal bone. The length and height of the basal turn, mid-modiolar height of the cochlea, mid-scalar and lateral wall length of the cochlear duct, and the 'X' line (the largest distance from mid-point of the round window to the mid-scalar point of the cochlear canal) were measured. RESULTS: Cochlear duct lateral wall length (28.88 mm) was higher than cochlear duct mid-scalar length (20.08 mm) (p < 0.001). The simple linear regression equation for estimating complete cochlear duct length was: cochlear duct length = 0.2 + 2.85 × X line. CONCLUSION: Using the mid-scalar point as the reference point (rather than the lateral wall) for measuring cochlear duct mid-scalar length, when deciding on the length of mid-scalar or peri-modiolar electrode, increases measurement accuracy. Mean cochlear duct mid-scalar length was compatible with peri-modiolar and mid-scalar implant lengths. The measurement method described herein may be useful for pre-operative peri-modiolar or mid-scalar implant selection.

The effect of cochlear implant bed preparation and fixation technique on the revision cochlear implantation rate.

OBJECTIVE: This study aimed to determine the effect of the subperiosteal tight pocket technique versus the bone recess with suture fixation technique on the revision cochlear implantation rate and complications. METHODS: This retrospective study included 1514 patients who underwent cochlear implantation by 2 senior surgeons between October 2002 and January 2016. Revision cases were identified and analysed. RESULTS: In all, 52 patients (3.34 per cent) underwent revision cochlear implantation. The revision rate was 7.18 per cent in the subperiosteal tight pocket group versus 2.37 per cent in the bone recess with suture fixation group (p < 0.001). Device failure was the most common reason for revision surgery in both groups. There was a significant difference in the device failure rate between the bone recess with suture fixation group (2.11 per cent) and subperiosteal tight pocket group (6.88 per cent) (p < 0.001). CONCLUSION: Accurate fixation of the cochlear implant receiver/stimulator is crucial for successful cochlear implantation. As the bone recess with suture fixation technique is associated with a lower revision rate and a similar complication rate as the subperiosteal tight pocket technique, it should be the preferred fixation technique for cochlear implantation.

Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature.

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

Epidemiology and outcome of adult-onset Still's disease in Northwestern Thrace region in Turkey.

OBJECTIVES: Adult-onset Still's disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. It is characterised by fever, arthritis and, a typical salmon-coloured rash, and is accompanied by fever at nights. Currently, there is limited data on the prevalence of AOSD. METHODS: Patients diagnosed with AOSD at the Department of Rheumatology of Trakya University Medical Faculty, between 2003 to 2014 were reviewed retrospectively. Patients' clinical features, laboratory measurements, demographics, treatments, follow-up durations, disease courses, outcomes and complications were evaluated. RESULTS: Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. The most common findings were fever (97.6%), arthralgia (95.2%), arthritis (76.2%), rash (73.8%) and sore throat (40.5%). CONCLUSIONS: In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: Clinical features and epidemiological data.

OBJECTIVE: In this study, we evaluated clinical and epidemiologic features of our giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) patients. METHODS: We retrospectively recorded down the general features of patients with GCA and PMR diagnosed at our center within the last 6 years. The incidence rates per 100000 aged > or =50 were calculated. In addition, we reported the frequencies of GCA/PMR in our previous epidemiologic study. RESULTS: Nineteen patients were diagnosed with GCA (10F, 9M) and 53 with isolated PMR (39F, 14M). The annual incidence for GCA in subjects > or =50 years old was 1.13/100000, and for PMR it was 3.15/100000. The incidence of GCA and PMR in females were, respectively, 1.14/100000 and 4.48/100000. In males, the incidences of GCA and PMR were, respectively, calculated as 1.1/100000 and 1.72/100000. In our population-based study, the prevalences of GCA and PMR (> or =50 ages) were estimated as 20/100000. Fourteen (73.7%) GCA patients had symptoms of PMR. Two patients had developed unilateral and one patient bilateral permanent visual loss. Initial ESR was lower than 40 mm/hr in one GCA patient (5.3%) and in 6 PMR patients (11.3%). The median duration of follow-up was 16 months in GCA; and 8 months in PMR patients. One patient with PMR and another patient with GCA had lung cancer. One PMR patient had myelodysplastic syndrome. During follow-up, 4 patients with GCA died. CONCLUSION: We detected a lower frequency of GCA/PMR in our center in northwestern Turkey than in Scandinavian and southern European countries.

Leptin and resistin levels in serum of patients with hematologic malignancies: correlation with clinical characteristics.

AIM: To evaluate leptin and resistin levels in patients with various hematologic malignancies. METHODS: We included 21 patients with lymphoma, 14 with multiple myeloma (MM), 14 with acute leukemia, 13 with chronic lymphocytic leukemia (CLL), and 25 healthy control subjects into our study. The subjects' body mass indexes (BMI) were calculated; hematological and acute phase response parameters, serum lipid were determined; serum leptin and resistin levels were determined by ELISA. RESULTS: Serum leptin level was significantly increased in CLL and MM groups when compared to the control group (p less, similar 0.01). Resistin level was significantly higher in lymphoma patients than in CLL, acute leukemia and control groups (p less, similar 0.01). In the control group, leptin level was negatively correlated with hemoglobin level (r = -0.44, p = 0.047); and in all patients with hematologic malignancies, leptin level was correlated with BMI (r = 0.32, p = 0.02). Leptin in lymphoma subjects correlated with hemoglobin level (r = 0.64, p = 0.005), resistin level correlated with the platelet count in patients with hematologic malignancies (r = 0.26, p = 0.044). In addition, leptin level had negative correlations with international prognostic score (IPS) in Hodgkin lymphoma (r = -0.9, p = 0.002) and with international prognostic index (IPI) in non-Hodgkin lymphoma (r = -0.77, p = 0.03). In CLL patients, leptin level had a correlation with the poor prognostic marker - CD38 level (r = 0.68, p = 0.03). CONCLUSION: We found higher leptin levels in MM and CLL patients, and higher resistin levels in lymphoma patients: this fact demonstrates that changes in adipose tissue and metabolism occur in these disease states.

Secondary amyloidosis causing nephrotic syndrome in a patient with non-Hodgkin's lymphoma: quite a rare diagnosis.

Secondary amyloidosis is usually a complication of chronic inflammation. Amyloidosis cases during the course of non-Hodgkin's lymphoma (NHL) are usually of AL-type, only one NHL patient with secondary amyloidosis has been reported. Our 79-year-old male patient visited us with multiple lymphadenopathies, and he was diagnosed with nodal marginal zone B-cell lymphoma. After four cycles of combined chemotherapy; his urea, creatinine levels started to increase and he developed nephrotic-range proteinuria. His rectal biopsy demonstrated amyloid deposition in submucosal vessel walls. The patient has been under hemodialysis for 10 months and his lymphoma is still in partial remission. We presented this case because it is the second NHL patient who developed secondary amyloidosis during his disease course.

Increased adiponectin level in non-Hodgkin lymphoma and its relationship with interleukin-10. Correlation with clinical features and outcome.

It was reported that interleukin-10 (IL-10) level increased in non-Hodgkin lymphoma (NHL) and chronic lymphocytic leukemia (CLL) patients; moreover this was associated with poor prognosis. In addition, it was stated that adiponectin induced the antiinflammatory cytokine IL-10. We evaluated adiponectin and IL-10 levels in NHL and CLL patients. We included newly diagnosed 28 NHL, 23 CLL patients, and 17 healthy subjects. In NHL patients, adiponectin level was higher than in CLL group and controls (p values < 0.05). In CLL group, IL-10 level was lower than in NHL, and control groups (p values < 0.05). Adiponectin level had a positive correlation with IL-10 level in the NHL patients (r = 0.41, p = 0.04). In the NHL group, the median survival of patients with high IL-10 levels was shorter (22 months vs. not reached, p = 0.03). Increased IL-10 levels helped to predict poor outcome in our NHL patients. High adiponectin levels and a relationship between adiponectin/IL-10 in newly diagnosed NHL patients might suggest a role for both in the immunodysregulation in NHL.

An interesting presentation of intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia.

Extramedullary hematopoiesis (EMH) occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. In addition to the more common locations, such as liver, spleen and lymph nodes, a mass of EMH may occasionally occur in the thorax. Intrathoracic EMH is usually asymptomatic. A 69-year-old woman who initially presented with hematuria, dysuria, and left inguinal pain was found to have paravertebral masses in the thorax. Histopathologic examination of a CT-guided needle aspiration biopsy of the masses showed the presence of trilineage hematopoiesis. We present this unusual case, in which EMH was diagnosed by chance in an elderly patient with no symptoms related to thalassemia.

Thrombosis in systemic lupus erythematosus: effect of inherited thrombophilic mutations.

Thrombosis in the venous or arterial system is quite common in systemic lupus erythematosus (SLE). We describe a young female patient whose first presentation was in the form of deep venous thrombosis of the right lower extremity. Her family history for thrombosis was positive and further studies revealed her to have SLE. Genetic studies showed that she had thrombophilic mutations of factor V, prothrombin and methylene tetrahydrofolate reductase genes. Her therapeutic response to anticoagulant therapy was satisfactory. The presence of inherited thrombophilic mutations must be searched for in SLE patients with thrombosis, especially in cases with a positive family history.

Metastatic squamous cell carcinoma of the skin in chronic myeloid leukaemia: complication of hydroxyurea therapy.

Hydroxyurea is a ribonucleotide diphosphate reductase inhibitor used in the treatment of patients with myeloproliferative disorders. Hydroxyurea has some dermatological side-effects. It has recently been recognized that hydroxyurea can induce squamous cell and basal cell carcinomas of skin. We present the case of an elderly man with chronic myeloid leukaemia who was treated with hydroxyurea for 4 years, with good control of his disease. However, in addition to the appearance of various skin lesions and cutaneous squamous cell carcinoma after 3 years of therapy, he was found to have a metastatic squamous cell carcinoma after 4 years. Hydroxyurea was discontinued, and he underwent surgery and radiotherapy. The patient subsequently died of ventricular fibrillation. We present this case to draw attention to the association between hydroxyurea and secondary skin cancers and to emphasize the need for dermatological examination before and during the course of hydroxyurea therapy.

Adrenal carcinoma causing secondary amyloidosis: report of the first case in the literature.

In a 53-year-old male patient with metastatic adrenal carcinoma, treatment with mitotane was instituted but he was lost to follow-up. Two years later, he presented with oedema and nephrotic-range proteinuria. The rectal and renal biopsies revealed an accumulation of secondary amyloid material. The patient died of respiratory failure caused by the progressive pulmonary metastases. This is the first report of a patient with adrenal carcinoma who developed secondary amyloidosis.

Temporary catheter infections in hemodialysis patients: results from a single center in Turkey.

In this study, we aimed to determine the frequencies of catheter exit-site infection (CESI), catheter-related bloodstream infection (CR-BSI) and catheter colonization (CC); causative microorganisms; and resistance patterns in patients with temporary hemodialysis catheters. From March 1999 to March 2000, 67 hemodialysis patients (38 males, 29 females; median age: 52, range: 17-84) were evaluated. The CDC criteria were used to diagnose CESI, CR-BSI and CC. The tips of catheters were cultured by Maki's method. At the same time, two different blood cultures, one from peripheral vein and the other through the catheter lumen were drawn. Swab cultures from the catheter exit sites were also performed. The isolation and identification of bacteria were performed by conventional methods and the susceptibility testing by the Kirby-Bauer method. CESI, CR-BSI and CC were found in, respectively, 20 (29.8%), 16 (23.8%) and 11 (16.4%) patients. The etiologic agents in CR-BSI were as follows: Staphylococcus aureus (5), coagulase-negative staphylococci (2), Enterococcus sp. (1), Escherichia coli (1), Acinetobacter sp. (1) and Proteus sp. (1). Methicillin-resistant coagulase-negative staphylococci and methicillin-resistant S. aureus were found in proportions of 45.5% and 63.6% in CESI and CR-BSI+CC. The only risk factor for the development of CR-BSI and CC was intravenous drug use. In our center, the majority of CESI, CR-BSI and CC were due to staphylococci and there was a high rate of methicillin resistance.

Amyloid goitre in familial Mediterranean fever: report on three patients and review of the literature.

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.

Diffuse diabetic glomerulosclerosis in a patient with impaired glucose tolerance: report on a patient who later develops diabetes mellitus.

Diabetic glomerulosclerosis might be seen in diabetics but its presence in patients with impaired glucose tolerance is quite rare. A 31-year-old woman who was admitted to our department was diagnosed with hypertension, nephrotic syndrome and impaired glucose tolerance. Her renal biopsy was compatible with diabetic glomerulosclerosis. She developed overt diabetes mellitus (DM) after one year of impaired glucose tolerance. Hypertension might have accelerated the progression of diabetic nephropathy.

Colchicine neuromyopathy: a report of six cases.

Colchicine has been in use for therapeutic purposes for many years. It can, however, cause subacute onset muscle and peripheral nerve toxicity in patients with chronic renal failure. In this report we describe 6 patients who developed neuromyopathy after the administration of colchicine. All patients presented with proximal muscle weakness, elevated serum creatine kinase (CK) levels, and neuropathy and/or myopathy on electromyography (EMG). The diagnosis of colchicine toxicity was confirmed in all cases by the normalization of CK levels and EMG after discontinuation of the drug. Toxicity developed in 4 renal failure patients on therapeutic doses of the drug, while one patient took a massive dose for suicidal reasons, and the other was on high-dose therapy. Patients using colchicine--especially those with renal failure--should be warned about the side effects of the drug and physicians should be careful in the administration of the drug.

Overview of 321 patients with idiopathic thrombocytopenic purpura. Retrospective analysis of the clinical features and response to therapy.

In this retrospective study, we evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura (ITP) at our center between 1984-2000. We retrospectively examined the medical records of 321 (229 females, 92 males) ITP patients. One hundred and seventy-one (53.3%) patients were lost to follow-up. When evaluating the clinical features, all 321 patients were included; however, when the response to treatment modalities was evaluated only 150 patients followed up regularly were considered. The median age of the patients on initial diagnosis was 34 years (range: 14-78). At initial diagnosis, 235 (73.2%) patients had signs of bleeding. Of patients diagnosed with ITP initially, six later turned out to have systemic lupus erythematosus (SLE) and two myelodysplastic syndrome (MDS). The median follow-up of 150 patients followed up regularly was 30 months (range: 4-396). One hundred and thirty-seven of these subjects had an indication for treatment and 94.2% of them were administered either standard or high-dose steroids as the first-line therapy. Complete remission (CR) was defined as any platelet count >100,000/mm(3) lasting for 3 months or longer without treatment. CR was achieved in 51.9% of the patients given steroids as the initial therapy. During a median follow-up of 33 months, relapse occurred in 58.2% of these patients, and after a median follow-up of 11 months the rest of them were still in remission. Ninety-eight patients followed up regularly were administered second-line therapies. CR was obtained in 44.4% of the patients who used steroids as second-line therapy. Within a median follow-up of 15 months, 20.8% of these patients relapsed. Splenectomy was performed in 76 patients and CR was obtained in 68.4% of the regularly followed up patients. Relapse occurred within a median of 96 months in 15.4% of the patients who had CR. Kaplan-Meier curves showed that the duration of CR obtained by splenectomy was significantly higher than that obtained by steroids (p<0.001). The 10-year disease-free survivals in patients who used steroids and who underwent splenectomy were, respectively, 13% and 58%. In our adult ITP patients, steroids induced nearly similar rates of CR both as first-and second-line therapies. Splenectomy seems to be effective in patients unresponsive to steroids. The duration of CR obtained by splenectomy is significantly longer when compared with the duration of CR obtained by steroid therapy.

Secondary amyloidosis in Castleman's disease: review of the literature and report of a case.

It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.