Effect of oral contraceptives on urinary porphyrins

Several studies have reported on the appearance of cutaneous porphyria in people treated with estrogen. This study was undertaken to investigate the effects of oral contraceptives (OCs) on levels of porphyrins in urine in nonporphyric women. 30 women aged 21-40 and on OCs were observed as the study group, and 10 women using other contraceptive methods were observed as the control group. Urinary porphyrins were measured by the Remington method. Levels of urinary coproporphyrin and of urinary uroporphyrin were higher in the study group than in the control group. These differences, however, are not statistically significant. To study the effect of duration of OC treatment, women who had taken OCs for 3-6 months and women who had taken OCs for longer than 6 months were observed, and the means of urinary coproporphyrin and uroporphyrin measured. Again, the differences were found to be insignificant, although slightly higher values of urinary coproporphyrin were obtained for women who had been on OCs for a longer period. These results compare favorably with others reported in the published literature. It can be concluded that OC treatment has no significant bearing on porphyrin metabolism.     

Acute appendicitis presenting with a testicular mass: ultrasound appearances

Acute appendicitis presenting with scrotal symptoms is a rare event, occurring when a patent processus vaginalis persists. We present a case where ultrasound demonstrated an inflamed appendix and a scrotal abscess, allowing the correct surgical management in a difficult clinical situation. In a child presenting with scrotal signs and vague lower abdominal symptoms, an ultrasound assessment of the right iliac fossa should always be performed.     

Reasons for rejecting potential donors from a sperm bank program

Purpose: Recruiting donors to a sperm bank program is difficult and slow because of high dropout rates and high rejection rates. The profile of successful and unsuccessful donors was determined at our sperm bank. Methods: A total of 199 men was screened from 1986 to 1994 in the anonymous sperm bank donor program; 174 (87%) men dropped out or did not meet minimum guidelines. The study included 25 accepted donors and 20 rejected men (of 52 rejected donors, only 20 donors who came for two consecutive semen analyses were selected). Sperm quality variables and demographic data were compared between the groups. Results: Accepted donors had significantly better semen quality in motility, velocity, linearity, and ALH than did rejected donors (P < 0.01). More rejected donors than accepted donors were single (P < 0.01). A higher percentage of accepted donors consumed caffeine (P < 0.001), and they were more likely to have college degrees (P < 0.03). Conclusions: These results indicate that loss of interest and poor semen quality were the major reasons for rejection of donors in our anonymous donor sperm bank program.     

Moyamoya disease mimicking a spontaneous internal carotid artery dissection on Doppler ultrasound

Moyamoya disease and spontaneous internal carotid artery dissections are rare conditions, but both tend to affect young adults with potentially devastating consequences. A 43-year-old non-Japanese patient presented with neurological symptoms, which, following carotid Doppler ultrasound and angiography, was labelled as being due to a spontaneous internal carotid artery dissection. Repeat imaging at 3 months showed normalisation of the carotid Doppler findings which coincided with the formation of „moyamoya” vessels on the angiogram. This case report illustrates the changes on carotid ultrasound in early moyamoya disease which may mimic the appearances of an internal carotid artery dissection and demonstrates the change of the spectral Doppler waveform that occurs with the formation of new vessels at the base of the brain. Received: 20 August 1998; Revision received: 23 November 1998; Accepted: 23 December 1998     

Complex regional pain syndrome and dysautonomia in a 14‐year‐old girl responsive to therapeutic plasma exchange

Reflex sympathetic dystrophy, also known as complex regional pain syndrome (CRPS), has recently been shown to be associated with autoantibodies against β2‐adrenergic and muscarinic M2 receptors. In addition to pain and sudomotor/vasomotor symptoms, dysautonomia is also observed in a subset of CRPS patients. Despite its severity, there are few effective therapies for CRPS described to date. We report a case of a 14‐year‐old girl with CRPS of her right leg and dysautonomia (gastroparesis, postural tachycardia) refractory to multiple therapies, successfully treated with therapeutic plasma exchange (TPE) with albumin replacement. The patient, who has serum anti β2‐adrenergic and muscarinic M2 receptor autoantibodies in addition to nicotinic acetylcholine receptor ganglionic autoantibodies, underwent an initial course of five TPEs over a 2‐week period. She demonstrated a clinical response to TPE as manifested by a rapid improvement in her fatigue and gastroparesis, with a gradual yet significant improvement in her leg pain and sudomotor/vasomotor flares. Following the loading procedures, the patient was treated with rituximab. She continues to require periodic TPE to maintain a remission, with additional immunosuppression being considered long term. Although further studies are needed, TPE (in combination with immunosuppression) may be an appropriate therapy for CRPS patients with detectable autoantibodies, as it is for better characterized diseases with autoantibodies against neuronal surface receptors such as myasthenia gravis or Lambert Eaton myasthenic syndrome. J. Clin. Apheresis 31:368–374, 2016. © 2015 Wiley Periodicals, Inc.     

Women with the Alzheimer's risk marker ApoE4 lose Aβ-specific CD4+ T cells 10–20 years before men

Adaptive immunity to self-antigens causes autoimmune disorders, such as multiple sclerosis, psoriasis and type 1 diabetes; paradoxically, T- and B-cell responses to amyloid-β (Aβ) reduce Alzheimer''s disease (AD)-associated pathology and cognitive impairment in mouse models of the disease. The manipulation of adaptive immunity has been a promising therapeutic approach for the treatment of AD, although vaccine and anti-Aβ antibody approaches have proven difficult in patients, thus far. CD4⁺ T cells have a central role in regulating adaptive immune responses to antigens, and Aβ-specific CD4⁺ T cells have been shown to reduce AD pathology in mouse models. As these cells may facilitate endogenous mechanisms that counter AD, an evaluation of their abundance before and during AD could provide important insights. Aβ-CD4see is a new assay developed to quantify Aβ-specific CD4⁺ T cells in human blood, using dendritic cells derived from human pluripotent stem cells. In tests of >50 human subjects Aβ-CD4see showed an age-dependent decline of Aβ-specific CD4⁺ T cells, which occurs earlier in women than men. In aggregate, men showed a 50% decline in these cells by the age of 70 years, but women reached the same level before the age of 60 years. Notably, women who carried the AD risk marker apolipoproteinE-ɛ4 (ApoE4) showed the earliest decline, with a precipitous drop between 45 and 52 years, when menopause typically begins. Aβ-CD4see requires a standard blood draw and provides a minimally invasive approach for assessing changes in Aβ biology that may reveal AD-related changes in physiology by a decade. Furthermore, CD4see probes can be modified to target any peptide, providing a powerful new tool to isolate antigen-specific CD4⁺ T cells from human subjects.