Serdal Korkmaz

Characteristic features of 20 patients with Sheehan's syndrome

Sheehans syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. The aim of the present study was to determine the clinical characteristics of Sheehans syndrome in 20 patients (mean age 60.15 ± 3.41 years) with typical obstetric history. The mean duration between time of diagnosis and date of the last delivery was 26.82 ± 2.52 years (range 2–40 years). All patients had a history of massive hemorrhage at delivery and physical signs of Sheehans syndrome. Fourteen patients (70.0%) lacked postpartum milk production and did not menstruate following delivery. Baseline and stimulated anterior pituitary hormone levels were measured in all patients. According to the hormonal values, 18 (90.0%) patients had secondary hypothyroidism, 11 (55.0%) had adrenal failure and all of them had hypogonadism, prolactin and growth hormone deficiency. Hyponatremia was present in seven patients (35.0%). Total or partially empty sella was revealed in all patients by magnetic resonance imaging. Diabetes insipidus was not found in any patient. We found that lack of lactation in the postpartum period and early menopause seemed the most important clues for diagnosis of Sheehans syndrome, and inadequate prolactin and gonadotropin responses to stimulation tests were the most sensitive diagnostic signs in patients with severe postpartum hemorrhage.

Is Neutrophil/Lymphocyte Ratio Associated with Subclinical Inflammation and Amyloidosis in Patients with Familial Mediterranean Fever?

Background. The purpose of the present study is to determine the association between neutrophil/lymphocyte ratio and both subclinical inflammation and amyloidosis in familial Mediterranean fever. Methods. Ninety-four patients with familial Mediterranean fever and 60 healthy volunteers were included in the study. Of the patients, 12 had familial Mediterranean fever related amyloidosis. The neutrophil/lymphocyte ratio of the patients was obtained from the hematology laboratory archive. Results. The neutrophil/lymphocyte ratio was significantly higher among persons with familial Mediterranean fever compared to healthy individuals (P < 0.0001). Also, neutrophil/lymphocyte ratio was significantly higher in patients with amyloidosis than in amyloidosis-free patients (P < 0.0001). Since NLR was evaluated in nonamyloid and amyloid stages of the same patient population (type 1 phenotype), we obtained significant statistical differences (1.95 ± 0.30 versus 2.64 ± 0.48, P < 0.05, resp.). With the cutoff value of neutrophil/lymphocyte ratio >2.21 and AUC = 0.734 (P = 0.009), it was a reliable marker in predicting the development of amyloidosis. Conclusion. The neutrophil/lymphocyte ratio, an emerging marker of inflammation, is higher in patients with familial Mediterranean fever in attack-free periods. The neutrophil/lymphocyte ratio may be a useful marker in predicting the development of amyloidosis.

Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura: A retrospective multicenter study

UNLABELLED Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP. Also, TTP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in TTP patients. AIM We aimed to present our experience in 163 patients with TTP treated with TPE during the past 5years from 10 centers of Turkey. PATIENTS AND METHODS One hundered and sixty-three patients with TTP treated with TPE during the past 5years from 10 centers of Turkey were retrospectively evaluated. TPE was carried out 1-1.5times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150×10(9)/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded. RESULTS Fifty-eight percent (95/163) of the patients were females. The median age of the patients was 42years (range; 16-82). The median age of male patients was significantly higher than female (53 vs. 34years; p<0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary TTP comprised of 85.9% (140/163) of the patients and secondary TTP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary TTP. The median number of TPE was 13 (range; 1-80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p<0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary TTP (85% vs. 87%, respectively; p=0.806). There was no advantage of TPE+prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p=0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TTP [15% (21/140) vs. 13% (3/23); p=0.806]. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE+prednisolone [14% (12/86) vs. 3% (2/67), p<0.001]. CONCLUSIONS TPE is an effective treatment for TTP and is associated with high CR rate in both primary and secondary TTP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of TTP and if these two criteria met in a patient, TPE should be performed immediately.

INSPIRATORY MUSCLE STRENGTH IS CORRELATED WITH CARNITINE LEVELS IN TYPE 2 DIABETES

Introduction. Plasma carnitine insufficiency has been known to cause muscle weakness. Carnitine levels and pulmonary functions were lower in patients with diabetes. Patients and Methods. To determine whether pulmonary functions are correlated with carnitine levels in patients with type 2 diabetes. In this study, we evaluated pulmonary functions and carnitine concentrations in 49 patients with type 2 diabetes and 34 healthy controls. Results. Carnitine levels were lower in type 2 diabetes group than control group (52.56 ± 12.38 and 78.96 ± 10.66 hmol/mL, respectively, p < 0.0001). Pulmonary functions were not significantly different between groups. Carnitine levels were not correlated with age, duration of diabetes, fasting blood glucose levels, and glycemic control (HbA1c%) in patients with type 2 diabetes. However, carnitine levels in patient group were correlated with % forced vital capacity (FVC%) (r = 0.35, p = 0.016), % forced expiratory volume in 1 s (FEV1%) (r = 0.318, p= 0.029), FEV1/FVC (r= 0.302, p= 0.039), inspiratory muscle strength (PImax) (r = 0.407, p = 0.023), and PImax% (r = 0.423, p= 0.018). Conclusion. This study suggests that low carnitine levels may be associated with lower PImax and PImax% in type 2 diabetes.

Do neutrophil gelatinase-associated lipocalin and interleukin-18 predict renal dysfunction in patients with familial Mediterranean fever and amyloidosis?

Abstract Background: The aim of this study was to evaluate whether neutrophil gelatinase-associated lipocalin (NGAL) and interleukin-18 (IL-18) predict renal disfunction in patients with familial Mediterranean fever (FMF). Methods: This prospective study consisted of 102 patients with FMF in attack-free period, and 40 matched healthy controls. Of the patients, nine were diagnosed as amyloidosis. The patients were divided into two groups according to eGFR as below 120 mL per minute and above 120 mL per minute. Also, patients were divided into three groups according to the degree of urinary albumin excretion as normoalbuminuric, microalbuminuric, and macroalbuminuric. The serum levels of IL-18 (sIL-18) and NGAL (sNGAL), and urinary levels of IL-18 (uIL-18) and NGAL (uNGAL) were measured by using ELISA kits. Results: The levels of sIL-18, sNGAL, uIL-18, and uNGAL were detected significantly higher in FMF patients, particularly in patients with amyloidosis, when compared to controls. sNGAL, uIL-18, and uNGAL were significantly higher in patients with eGFR < 120 mL per minute than in patients with eGFR ≥ 120 mL per minute. sNGAL, uIL-18, and uNGAL were correlated significantly with urinary albumin excretion, additionally, were inverse correlated with eGFR. The most remarkable findings of this study are of the higher values of sIL-18, sNGAL, uIL-18, and uNGAL in both normoalbuminuric FMF patients and patients with eGFR ≥ 120 mL per minute. Conclusions: The results of this study suggest that sIL-18, uIL-18, sNGAL, and uNGAL are reliable markers of early renal disfunction in FMF patients, and may let us take measures from the early stage of renal involvement.

Is there a link between mean platelet volume and thrombotic events in antiphospholipid syndrome

Abstract The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the production of antiphospholipid antibodies (aPL) that promote vascular thrombosis and pregnancy loss. APS can occur in the absence of underlying or associated disease (primary APS) or in combination with other diseases (secondary APS). Mean platelet volume (MPV) is largely regarded as a useful surrogate marker of platelet activation. We aimed to investigate if there is a relationship between MPV and thrombotic events in APS. The study consisted of 22 patients and 22 healthy controls. Group 1 is defined as all the patients in the first day of thrombotic event. Group 2 is defined as the same patient population three months after the thrombotic event. The erythrocyte sedimentation rate, C-reactive protein, white blood cell count, platelet count, and MPV levels were retrospectively recorded from patient files. Statistical analyses showed that MPV was significantly higher in group 1 than group 2 (p < 0.0001) and healthy controls (p < 0.05). However, there was no difference between group 2 and healthy controls (p = 0.888). WBC, hemoglobin and other platelet indices such as platelet distribution width and platecrit did not differ in groups. In conclusion, MPV was increased at initial thrombotic event of APS, and then it was normalized three months later by therapeutic interventions. To our knowledge, this is the first study demonstrating a correlation between MPV and thrombotic events in APS.

Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis

Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs’ positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease.

Effectiveness of the haemonetics MCS cell separator in the collection of apheresis platelets.

Platelet (PLT) transfusions play an important role in patients with thrombocytopenia or severely impaired platelet function. Platelet concentrates are prepared from whole-blood donations or by plateletpheresis. In recent years, different instruments have been developed to perform plateletpheresis. We evaluated an apheresis instrument, the Haemonetics MCS(®) + with regard to PLT yield, collection efficiency (CE), and collection rate (CR) in a retrospective, randomized study in 526 donors. In this system, we used leukoreduction filters post collection to obtain leukoreduced products. The Haemonetics MCS(®) + cell separator efficiently collected apheresis platelets with median PLT yields of 3.7 × 10(11), mean CE of 66.69 ± 13.73% and mean CR of 0.063 ± 0.013 × 10(11)/min. The median blood volume processed was 3290 (2420-4370) ml, and the median volume of acid citrate dextrose-A (ACD-A) used in collections on the device was 385 (196-517) ml. Also, this device allowed the collection of white blood cell (WBC) reduced plateletpheresis with mean 0.07 ± 0.15 × 10(6) WBC content. No serious donor or recipient reactions occurred.

Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: twenty-four year Turkish experience in cladribine therapy

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty‐six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first‐line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second‐line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28‐month median OS was 91.7% in all patients and 25‐month median OS 96% for patients who were given cladribine as first‐line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population. Copyright

Low L-carnitine levels: Can it be a cause of nocturnal blood pressure changes in patients with type 2 diabetes mellitus?

OBJECTIVE The nocturnal blood pressure (NBP) reduction is less than normal in the patients with type 2 diabetes mellitus (type 2 DM). L-carnitine deficiency may cause changes of NBP by leading to hyperglycemia, insulin resistance and endothelial dysfunction in patients with type-2 DM. The purpose of the study was to investigate whether there is a difference in levels of L-carnitine in dipper and nondippers patients with type 2 DM. METHODS This is a cross-sectional observational cohort study. We compared the 50 (33 females, 17 males) patients with type 2 DM and the 35 healthy persons (18 females, 17 males) as a control group. In all cases, ambulatory blood pressure monitoring (ABPM) was performed and L-carnitine was measured. The independent samples t test, Chi-square test, Mann-Whitney U test and Pearson correlation analysis were used in the statistical evaluation of data. RESULTS We found that the percentage of nondipper pattern was 72% in patients with type 2 DM. L-carnitine levels were lower in patients with type 2 DM (52.77 ± 12.34 µmol/L) than those of control group (79.18 ± 10.59 µmol/L), and these differences were statistically significant (p=0.05). L-carnitine levels were lower in nondipper cases (50.02 ± 16.30 µmol/L) than those of dipper cases (53.83 ± 10.50 µmol/L), but these differences were not statistically significant (p=0.125). CONCLUSION Nondipper pattern is common in patients with type 2 DM. Nondipper pattern is associated with damage of target organ. Therefore, determination of nondipper pattern by ABPM is very important. L-carnitine levels were found to be lower in nondipper patients than in dipper patients with type 2 DM. Although, these differences were statistically nonsignificant, more detailed studies should be performed to determine the frequencies and relationship between nondipper pattern and L-carnitine levels in patients with type-2 DM.