Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report

Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Rotor's syndrome) than expected in pure Dubin-Johnson syndrome.     

The Relationship between Epicardial Adipose Tissue and Malnutrition,Inflammation, Atherosclerosis/Calcification Syndrome in ESRD Patients

Summary

Background and objectives

Malnutrition, inflammation, atherosclerosis/calcification (MIAC) and endothelial dysfunction are the most commonly encountered risk factors in the pathogenesis of cardiovascular disease in ESRD patients. Epicardial adipose tissue (EAT) is the true visceral fat depot of the heart. The relationship between CAD and EAT was shown in patients with high risk of coronary artery disease. In this study, we aimed to investigate the relationship between EAT and MIAC syndrome in ESRD patients.

Design, setting, participants, & measurements

Eighty ESRD patients and 27 healthy subjects enrolled in this cross-sectional study. EAT and coronary artery calcification score were measured by a multidetector computed tomography (MDCT) scanner. Patients with serum albumin <3.5 mg/dl were defined as patients with malnutrition; those with serum C-reactive protein level >10 ng/dl (normal range, 0–5 ng/dl) had inflammation; and those with CACS >10 had atheroscleosis/calcification.

Results

Total CACS and EAT measurements were significantly higher in ESRD patients when compared with healthy subjects. There was a statistically significant relationship between EAT and CACS in ESRD patients (r = 0.48). EAT measurements were higher in PD patients than HD patients. Twenty-four of the patients had no component, 31 had one component, 17 had two components, and nine had all of the MIAC components. EAT was found to be significantly increased when the presence of MIAC components increased. EAT was positively correlated with age, body mass index, and presence of MIAC. These parameters were also found as independent predictors of increased EAT.

Conclusions

We found a relationship between EAT and components of MIAC syndrome in ESRD patients.     

Steroid-responsive recurrent limbic encephalitis associated with small cell lung cancer and neuropil antibodies

Paraneoplastic limbic encephalitis (PLE) associated with small cell lung cancer (SCLC) often presents with antibodies to intracellular antigens and a poor outcome even after tumor resection and immunotherapy. We report a PLE patient presenting with generalized seizures, shortterm memory impairment and medial temporal lobe hyperintensity in MRI. Initial screening revealed significantly elevated thyroid antibody levels suggesting Hashimoto's encephalopathy. Following methylprednisolone treatment, her seizures ceased, MRI findings disappeared and memory impairment showed a partial resolution in 5 months. Two months later, she developed further generalized seizures. Chest X-ray showed a mass lesion, which was demonstrated by needle biopsy to be a small cell lung carcinoma (SCLC). The panel of onconeural antibodies including cell-membrane antigens was negative. However, the patient's serum and cerebrospinal fluid IgG, obtained during both exacerbations, immunolabeled cytoplasm and dendrites of Purkinje cells, cerebellar and hippocampal molecular layers, basal ganglia, thalamus, and the surface of cultured hippocampal neurons, in a manner distinct from previously identified neuropil antibodies associated with SCLC. These neuropil antibodies appear to be associated with a favorable response to treatment. Further studies are required for determination of the target antigen.     

Heart rate variability of young men with idiopathic hypogonadotropic hypogonadism

BackgroundThere is little data available regarding the effects of male sex hormones on cardiac autonomic function. The aim of this study is to evaluate the association between hormones of male hypothalamo–pitiutary–gonadal axis and cardiac autonomic function by comparing heart rate variability (HRV) parameters of young male idiopathic hypogonadotropic hypogonadism patients with those of healthy controls.MethodsThe study consisted of 22 male idiopathic hypogonadotropic hypogonadism patients (mean age 20.8 ± 1.2 years) and the same number of age-matched healthy male controls (mean age 21.0 ± 1.5 years). A 24-hour Holter monitoring was performed to assess the time and frequency-domain parameters. The HRV parameters of patients and control groups were compared, and possible associations between levels of tested hormones and HRV parameters were evaluated.ResultsThe standard deviation of all NN intervals (SDNN), standard deviation of the averages of NN intervals in all 5 min segments (SDANN), power in low frequency range (LF, ms²) and power in high frequency range (HF, ms²) values of patients were significantly lower compared to those of controls (147.47 ± 56.16 vs. 193.63 ± 40.89; 138.31 ± 57.64 vs. 190.15 ± 43.94; 397.8 ± 236.7 vs. 491.5 ± 208.4; and 133.6 ± 97.4 vs. 198.5 ± 91.6 respectively; p < 0.05 for all). Significant negative correlations were observed between serum FSH, LH and testosterone levels and most of the HRV parameters.ConclusionsDeficiency in the male hypothalamo–pituitary–gonadal axis seems to adversely affect cardiac autonomic modulation with increased sympathetic and decreased parasympathetic components of HRV.