Giant malignant pheochromocytoma with palpable rib metastases.

Pheochromocytoma is a rare and usually benign neuroendocrine neoplasm. Only 10% of all these tumors are malignant and there are no definitive histological or cytological criteria of malignancy. Single malignancy criteria are the presence of advanced locoregional disease or metastases. We report a case, with a giant retroperitoneal tumor having multiple metastases including palpable rib metastases, who was diagnosed as a malignant pheochromocytoma. The patient was treated with surgery. The literature was reviewed to evaluate tumor features and current diagnostic and therapeutic approaches for patients with metastatic or potentially malignant pheochromocytoma.

Assessment of bone mineral density in patients with Sheehan's syndrome.

INTRODUCTION: The number of studies concerning bone mineral density (BMD) in patients with Sheehan's syndrome (SS) are scarce. We aimed to investigate the relationship between BMD and deficient hormones in patients with newly diagnosed SS. MATERIAL AND METHODS: Thirty-four patients with SS and age-gender-weight matched 22 controls were included in the study. RESULTS: We found osteoporosis in 61.8%, osteopenia in 32.3%, and normal dual energy X-ray absorptiometry (DEXA) findings in 5.9% patients. In the control group, 68.2% of individuals were osteopenic and 31.8% of them were normal. The number of osteoporotic patients was found to be higher and BMD values were lower in the patient group compared with the control group. There was no relation of DEXA measurements with the period between the last delivery and the initial diagnosis and the estradiol levels. While there was no relation between insulin-like growth factor 1 (IGF-1) and T and Z scores of femur head, the relation between the IGF-1 and L1-L4 T scores was statistically significant. There was a significant relation between the IGF-1 and L1-L4 Z scores. CONCLUSIONS: SS patients should also be assessed in terms of osteoporosis at the time of diagnosis and then proper treatment should be initiated afterwards.

Importance of postoperative stimulated thyroglobulin level at the time of 131I ablation therapy for differentiated thyroid cancer.

BACKGROUND: Serum thyroglobulin detection plays an essential role during the follow-up of thyroid cancer patients treated with total/near total thyroidectomy and radioiodine ablation. The aim of this retrospective study was to evaluate the relationship between stimulated serum thyroglobulin (Tg) level at the time of high dose 131I ablation and risk of recurrence, using a three-level classification in patients with differentiated thyroid cancer (DTC) according to the ATA guidelines. Also we investigated the relationship between postoperative stimulated Tg at the time of ablation and DxWBS results at 8-10 months thereafter. MATERIALS AND METHODS: Patients with radioiodine accumulation were regarded as scan positive (scan +). If there was no relevant pathological radioiodine accumulation or minimal local accumulation in the thyroid bed region, this were regarded as scan negative (scan-) at the time of DxWBS. We classified patients in 3 groups as low, intermediate and high risk group for assessment of risk of recurrence according to the revised ATA guidelines. Also, we divided patients into 3 groups based on the stimulated serum Tg levels at the time of 131I ablation therapy. Groups 1-3 consisted of patients who had Tg levels of ≤ 2 ng/ml, 2-10 ng/ml, and ≥ 10 ng/ml, respectively. RESULTS: A total of 221 consecutive patients were included. In the high risk group according to the ATA guideline, while 45.5% of demonstrated Scan(+) Tg(+), 27.3% of patients demonstrated Scan(-) Tg(-); in the intermediate group, the figures were 2.3% and 90.0% while in the low risk group, they were 0.6% and 96.4%. In 9 of 11 patients with metastases (81.8%), stimulated serum Tg level at the time of radioiodine ablation therapy was over 10, however in 1 patient (9.1%) it was < 2 ng/mL and in one patient it was 2-10 ng/mL (p = 0.005). Aggressive subtypes of DTC were found in 8 of 221 patients and serum Tg levels were ≤ 2 ng/ml in 4 of these 8. CONCLUSIONS: We conclude that TSH-stimulated serum thyroglobulin level at the time of ablation may not determine risk of recurrence. Therefore, DxWBS should be performed at 8-12 months after ablation therapy.

Atrial fibrillation is associated with increased mean platelet volume in patients with type 2 diabetes mellitus.

Platelet abnormalities in diabetes mellitus (DM) and atrial fibrillation (AF) may underline the etiology of a prothrombotic state in these conditions. Increased mean platelet volume (MPV) is a marker of abnormal platelet function and activation. We aimed to investigate the possible association of chronic AF with MPV in patients who have type 2 DM. Patients who had type 2 DM with either chronic (≥6 months) AF or normal sinus rhythm (NSR) were included in the study. A total of 162 patients (aged 38-89 years) were divided into 2 groups according to the presence of either AF or NSR. Group 1 consisted of 81 diabetic patients with AF, and group 2 consisted of 81 diabetic patients with NSR. The two groups were not significantly different in terms of age, and gender, as well as in hypertension, smoking, history of coronary artery disease, previous cerebrovascular accidents, microalbuminuria, retinopathy, duration of DM, body mass index, hemoglobin A1c, total cholesterol, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, and triglyceride (p > 0.05 for all variables). Although no significant difference was present between groups concerning platelet count; for patients with AF, MPV was higher compared with patients with NSR (9.0 ± 0.2 fl vs. 8.4 ± 0.2 fl; p = 0.001). Furthermore, no significant difference was noted between groups regarding routine medications received by patients. In multivariate logistic regression analysis, MPV was the only variable independently related to AF (OR = 2.659; 95% CI, 1.286-5.498; p = 0.008). Consequently, it is concluded that AF is associated with increased MPV in patients with type 2 DM, suggesting the presence of tentatively related processes leading to reciprocal interaction.

Sheehan's syndrome.

Sheehan's syndrome (SS) is characterized by various degrees of hypopituitarism, and develops as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. Increased pituitary volume, small sella size, disseminated intravascular coagulation and autoimmunity are the proposed factors in the pathogenesis of SS. Hormonal insufficiencies, ranging from single pituitary hormone insufficiency to total hypopituitarism, are observed in patients. The first most important issue in the diagnosis is being aware of the syndrome. Lack of lactation and failure of menstrual resumption after delivery that complicated with severe hemorrhage are the most important clues in diagnosing SS. The most frequent endocrine disorders are the deficiencies of growth hormone and prolactin. In patients with typical obstetric history, prolactin response to TRH seems to be the most sensitive screening test in diagnosing SS. Other than typical pituitary deficiency, symptoms such as anemia, pancytopenia, osteoporosis, impairment in cognitive functions and impairment in the quality of life are also present in these patients. Treatment of SS is based on the appropriate replacement of deficient hormones. Growth hormone replacement has been found to have positive effects; however, risk to benefit ratio, side effects and cost of the treatment should be taken into account.

Relationship between homocysteine and non-dipper pattern in patients with type 2 diabetes mellitus.

OBJECTIVE: The rate of reduction of nocturnal blood pressure (NBP) is lesser than normal in patients with type 2 diabetes mellitus (type 2 DM). Hyperhomocysteinemia (HHC) disrupts vascular structure and function, no matter the underlying causes. The risk of development of vascular disease is greater in diabetic patients with hyperhomocysteinemia than in patients with normal homocystein levels. The aim of the study was to investigate whether there are differences of homocystein levels in dipper and non-dippers patients with type 2 DM. SUBJECTS AND METHODS: We compared 50 patien-ts (33 females, 17 males) with type 2 DM and 35 healthy individuals (18 females, 17 males ) in a control group. Ambulatory blood pressure monitoring (ABPM) was performed and homocysteine levels were measured in all patients. RESULTS: We found that the percentage of non-dipper pattern was 72% in patients with type 2 DM and 57% in control group. In diabetic and control individuals, homocystein levels were higher in non-dipper (respectively 13.4 ± 8.1 µmol/L and 11.8 ± 5 µmol/L) than in dipper subjects (respectively, 11.8 ± 5.8 µmol/L and 10.1 ± 4.2 µmol/L), but there was no significant difference between the two groups (respectively, p = 0.545, p = 0.294). CONCLUSION: In both groups, homocystein levels were higher in non-dipper than in dipper participants, but there was no significant difference between the groups. High homocystein levels and the non-dipper pattern increases cardiovascular risk. Therefore, the relationship between nocturnal blood pressure changes and homocystein levels should be investigated in a larger study.

Relationship between homocysteine and non-dipper pattern in patients with type 2 diabetes mellitus / Relação entre a homocisteína e a condição não dipper em pacientes com diabetes melito tipo 2

OBJECTIVE: The rate of reduction of nocturnal blood pressure (NBP) is lesser than normal in patients with type 2 diabetes mellitus (type 2 DM). Hyperhomocysteinemia (HHC) disrupts vascular structure and function, no matter the underlying causes. The risk of development of vascular disease is greater in diabetic patients with hyperhomocysteinemia than in patients with normal homocystein levels. The aim of the study was to investigate whether there are differences of homocystein levels in dipper and non-dippers patients with type 2 DM. SUBJECTS AND METHODS: We compared 50 patien­ts (33 females, 17 males) with type 2 DM and 35 healthy individuals (18 females, 17 males ) in a control group. Ambulatory blood pressure monitoring (ABPM) was performed and homocysteine levels were measured in all patients. RESULTS: We found that the percentage of non-dipper pattern was 72% in patients with type 2 DM and 57% in control group. In diabetic and control individuals, homocystein levels were higher in non-dipper (respectively 13.4 ± 8.1 µmol/L and 11.8 ± 5 µmol/L) than in dipper subjects (respectively, 11.8 ± 5.8 µmol/L and 10.1 ± 4.2 µmol/L), but there was no significant difference between the two groups (respectively, p = 0.545, p = 0.294). CONCLUSION: In both groups, homocystein levels were higher in non-dipper than in dipper participants, but there was no significant difference between the groups. High homocystein levels and the non-dipper pattern increases cardiovascular risk. Therefore, the relationship between nocturnal blood pressure changes and homocystein levels should be investigated in a larger study.

OBJETIVO: A taxa de redução da pressão arterial noturna (PAN) é menor em pacientes com diabetes melito tipo 2 (DM tipo 2). A hiper-homocisteinemia (HHC) perturba a estrutura e a função vascular, independentemente das causas subjacentes. O risco de desenvolvimento de doenças vasculares é maior em pacientes diabéticos com hiper-homocisteinemia do que em pacientes com níveis normais de homocisteína. O objetivo deste estudo foi investigar se há diferenças entre os níveis de homocisteína em pacientes com DM2 em condição dipper ou não dipper. SUJEITOS E MÉTODOS: Comparamos 50 pacientes (33 mulheres, 17 homens) com DM tipo 2 e 35 indivíduos saudáveis (18 mulheres, 17 homens), usados como grupo controle. A monitorização ambulatorial da pressão arterial (MAPA) e os níveis de hemocisteína foram medidos em todos os pacientes. RESULTADOS: Observamos que a porcentagem de não dipper foi de 72% em pacientes com DM tipo 2 e 57% no grupo controle. Em indivíduos diabéticos e controle, os níveis de homocisteína foram mais altos em indivíduos não dipper (respectivamente 13,4 ± 8,1 µmol/L e 11,8 ± 5 µmol/L) do que em indivíduos dipper (respectivamente, 11,8 ± 5,8 µmol/L e 10,1 ± 4,2 µmol/L), mas não houve diferença significativa entre os dois grupos (respectivamente, p = 0,545, p = 0,294). CONCLUSÃO: Em ambos os grupos, os níveis de homocisteína foram mais altos nos indivíduos não dipper do que nos indivíduos dipper, mas não houve diferença significativa entre os grupos. Altos níveis de homocisteína e a condição não dipper aumentam o risco cardiovascular. Portanto, a relação entre as alterações da pressão arterial noturna e os níveis de homocisteína deve ser investigada em um estudo mais amplo.

Allgrove syndrome.

Allgrove syndrome is a rare autosomal recessive disorder. It is also known as the 3A syndrome and characterised by the triad of achalasia, alacrima and adrenal insufficiency. The AAAS gene is encoded on chromosome 12q13. We report the case of a 23-year-old woman who presented at the hospital with adrenal crisis that was triggered by infection of the urinary system and gastrointestinal bleeding. She had a known diagnosis of achalasia for eight years, and ophthalmologic examination revealed alacrima. Based on our findings, the patient was diagnosed with Allgrove syndrome.

Serum angiopoietin-related growth factor (AGF) levels are elevated in gestational diabetes mellitus and associated with insulin resistance.

OBJECTIVE: Angiopoietin-related growth factor (AGF) is associated with angiogenesis but it can also affect glucose and energy metabolism. The aim of the study was to determine AGF levels in gestational diabetes mellitus (GDM). MATERIALS AND METHODS: The study included 44 patients with GDM (GDM group) and 33 non-diabetic, healthy women in the third trimester of pregnancy (control group). We analyzed serum levels of AGF and other biochemical and anthropometric markers in all subjects. RESULTS: The study revealed that serum AGF levels were significantly higher in patients with GDM (113.30 +/- 69.92 ng/ml) than in controls (52.30 +/- 35.59 ng/ml), (p-value<0.001). Fasting glucose (117.59 vs. 82.18), homeostasis model of assessment - insulin resistance (HOMA-IR), (2.91 vs. 1.75) diastolic (74.20 vs. 70.00) and mean (89.09 vs. 84.84) blood pressure were found to be significantly higher in the GDM group when compared to the control group (p-value<0.05). There was a significant positive association between AGF and HOMA-IR in the GDM group. CONCLUSIONS: Although gestational diabetes mellitus can be a predictor of serum AGF level, further studies are needed to explain the physiologic roles of AGF in glucose metabolism.

Acute evaluation of pituitary function in patients with Crimean-Congo haemorrhagic fever.

CONTEXT: Crimean-Congo haemorrhagic fever (CCHF) can cause a fatal haemorrhagic syndrome. Pituitary ischaemia/infarction and necrosis are known causes of hypopituitarism, often remaining unrecognized because of subtle clinical manifestations. OBJECTIVE: Our aim was to evaluate the effect of CCHF on pituitary function. SUBJECT AND METHODS: Levels of serum free T3, free T4, TSH, GH, IGF-I, prolactin, cortisol, testosterone (in men) and oestrogen (in women) were studied in 20 patients who had been diagnosed with CCHF. TRH, LH-RH and 1 µg adrenocorticotropin tests were performed in all patients. The hypothalamo-pituitary region was examined by magnetic resonance imaging (MRI) in two patients who were diagnosed with hypocortisolism. RESULTS: We found cortisol insufficiency in 2 (10%) of the 20 with CCHF. However, hypophyseal MRI findings were normal in these two patients. None of the patients had deficiencies of GH, TSH or FSH/LH. CONCLUSION: To our knowledge, this is the first study reporting the effect of CCHF on pituitary function. We found that cortisol insufficiency may occur in patients diagnosed with CCHF; however, studies including a larger number of patients are required to make a definite conclusion on this issue.

Serum cortisol and dehydroepiandrosterone-sulfate levels in schizophrenic patients and their first-degree relatives.

AIMS: Alterations in cortisol and dehydroepiandrosterone sulfate (DHEA-S) levels are thought to play a role in the pathophysiology of neuropsychiatric disorders, including schizophrenia. The aim of this study was to investigate the role of serum cortisol and DHEA-S in the pathophysiology of schizophrenia. METHODS: Sixty schizophrenic patients, 70 healthy first-degree relatives, and 60 healthy volunteers were included. Sociodemographic characteristics, data regarding disease duration and severity, as well as ongoing and previous drug use were recorded. Serum cortisol and DHEA-S levels were measured. RESULTS: Serum cortisol and DHEA-S levels were significantly higher in the schizophrenia group compared with the first-degree relatives and controls (P < 0.05). Serum cortisol levels in the first-degree relatives were significantly higher than in the healthy controls (P < 0.05). There was no significant difference between the first-degree relatives and healthy-controls in terms of DHEA-S levels and between the three groups in terms of serum cortisol/DHEA-S ratios. CONCLUSIONS: Elevated serum cortisol levels in schizophrenic patients might be associated with the role of cortisol in the pathophysiology of schizophrenia. Also, the elevation of serum cortisol levels in first-degree relatives compared to controls suggests that similar pathophysiological processes might have a role in individuals without any disease symptoms, but with a genetic predisposition for schizophrenia. Elevated serum DHEA-S levels might be the result of a compensatory response to elevated cortisol levels. Serum cortisol and DHEA-S levels may be used as a biological marker for the diagnosis of schizophrenia; however, further studies with larger sample sizes are warranted to support this finding.

[Low L-carnitine levels: can it be a cause of nocturnal blood pressure changes in patients with type 2 diabetes mellitus?]. / Düsük L-karnitin düzeyleri: tip 2 diyabetes mellitus olgularinda noktürnal kan basinci degisikliklerinin bir nedeni olabilir mi?

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.

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.

Characteristic features of 20 patients with Sheehan's syndrome.

Sheehan's 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 Sheehan's 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 Sheehan's 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 Sheehan's syndrome, and inadequate prolactin and gonadotropin responses to stimulation tests were the most sensitive diagnostic signs in patients with severe postpartum hemorrhage.