A Case of Concurrent Proteus Syndrome and Hemophilia A

Background

Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity.

Case Presentation

A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient''s various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established.

Conclusion

Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases.     

Persistent organic pollutants in muscle and feather of ten avian species from Māzandarān Province of Iran, on the coast of the Caspian Sea

Feather and muscle of 10 avian species (n = 46), were analyzed for polychlorinated biphenyls (PCBs) and organochlorine pesticides (OCPs). Muscle contained significantly higher PCB and OCP than liver and feather. Mean muscle and feather PCB was 408.5 ± 134.5 and 32 ± 4.5 ng/g wet weight. Highly chlorinated PCBs were found in muscle and liver while feather had PCBs with less chlorination. Gulls had highest levels of both pollutants. Gull feather PCB and OCP were strongly correlated with their levels in the liver and muscle tissues (0.6 < r < 0.9, p < 0.01). Analysis of gull feather can be used as a non-invasive method for monitoring organic pollutants.     

The dilemma of ulnar nerve entrapment at wrist in carpal tunnel syndrome

Objective

The commonest compression neuropathy in human being is carpal tunnel syndrome (CTS). The association between CTS and ulnar nerve entrapment is debatable. The objective of this study is to determine the presence of any association between CTS and ulnar entrapment neuropathy at the wrist.

Patients and methods

To test the hypothesis we conducted a case-control study. Ninety-nine healthy volunteers and 181 patients with established diagnosis of CTS enrolled to the study. Distal latencies, peak latencies and action potentials for sensory branches and distal latencies and action potentials for motor branches of both median and ulnar nerves were measured in totally 378 hands. We conducted independent t-test comparing age and sex between control and patient groups and analysis of variance to compare dichotomous and continuous variables between control group and patient subgroups.

Results

Based on our cutoffs, we found that 7.5% of CTS patients had distal latency ≥2.8 ms for ulnar sensory branches, 4.6% had distal latency ≥3.4 ms for ulnar nerve motor branches and 15% had peak latency ≥3.3 ms for ulnar sensory branches. There was not any statistically significant correlation between subgroups of CTS patients and control group.

Conclusion

The authors suggest that there may not be any association between CTS and ulnar nerve compression at the wrist. We suggest that different racial groups and multiple techniques in performing nerve conduction studies and dissimilar cutoff values for the diagnosis of entrapment neuropathies are the major causes of ambiguity in the literature. More relevant studies will have crucial importance for detecting ulnar nerve entrapment at the wrist in CTS patients.     

The Effect of Education and Awareness on the Quality-of-Life in Postmenopausal Women

Objectives:

Women are one of the most important parts of the family and society, and community health is dependent on provision of the needs of this group. Menopause is one of the most critical stages of life among women. One of the aims of health services for all of the people in the 21^st century is improvement of the quality-of-life. In menopausal women, the term quality-of-life incorporates its physical symptoms such as hot flushes, night sweats and vaginal mucosa dryness. We set out this study in order to evaluate the effect of education on the quality-of-life and the improvement of health standards in menopausal women.

Materials and Methods:

Sixty-two women aged 44–55 referring to and academic outpatient clinic in Shiraz were selected by simple random sampling, and allocated in two groups. Data was collected using a modified Hildich questionnaire on quality-of-life in menopause stage. Quality-of-life of the subjects (vasomotor, psychosocial, physical and sexual aspects) were evaluated prior to and 3 months after educational intervention.

Results:

Mean quality-of-life score in study and control groups, prior to education, was 81.7 and 74.8; changing to 75.3 and 75.8, respectively three months after intervention. The study group showed a significant improvement in their quality-of-life (P = 0.001). A significant difference was seen between groups in terms of changing quality-of-life after intervention (P = 0.001).

Conclusions:

Appropriate training to menopausal women improves their quality-of-life and promotes their health.     

Does the abnormal signal-averaged electrocardiogram predict future appropriate therapy in patients with implantable cardioverter-defibrillators?

BACKGROUND: Several studies have documented the prognostic significance of the signal-averaged electrocardiogram (SAECG) both after myocardial infarction and nonischemic cardiomyopathy. However, whether the SAECG can identify patients with implantable cardioverter-defibrillator (ICD) who receive appropriate therapy has not been hitherto completely investigated. METHODS: Between August 2002 and August 2004, 83 consecutive ICD patients who had had SAECGs recorded were enrolled in this study. All patients were followed up in the outpatient ICD clinic, and interrogated electrograms were collected. RESULTS: Over 9.0 +/- 2.8 months of follow-up, 27 (32%) patients had appropriate ICD therapy for ventricular tachycardia or fibrillation; 15 (55.6%) patients had abnormal; and the remaining 12 (44.4%) had normal SAECGs. Of the 56 patients with no appropriate therapy, 27 (48.2%) and 29 (51.8%) patients had abnormal and normal SAECGs, respectively. There were no statistically significant differences between the 2 groups in SAECG findings (P = .41). A Cox regression analysis showed that the left ventricular ejection fraction was the only predictor of appropriate therapy (P = .02). Subgroup analysis of the patients with coronary artery disease and spontaneous monomorphic ventricular tachycardia indicated that left ventricular ejection fraction (P = .03) and abnormal SAECG (P = .02) were predictors of appropriate therapy. CONCLUSIONS: Our data demonstrate that except for the subgroup of patients with coronary artery disease presenting with monomorphic ventricular tachycardia, the SAECG did not predict ventricular tachyarrhythmia recurrence and, hence, appropriate ICD therapy. Thus, SAECG findings should generally not be a factor in decision for ICD implantation.     

Postcoital penile drug eruption in a co-trimoxazole-sensitive patient following vaginal use of triple sulfa vaginal cream by his partner

IntroductionThis is a report of a very rare case of proven postcoital penile drug eruption in a patient following vaginal use of triple sulfa vaginal cream by his partner.AimTo report the rare case of cross‐reaction following vaginal use of triple sulfa vaginal cream in partner.MethodsA case of postcoital penile drug eruption in a patient following vaginal use of triple sulfa vaginal cream in his partner is presented including subjective reporting, physical examination, and laboratory evaluations.ResultsWe report a 42‐year‐old man with known sensitivity to trimethoprim/sulfamethoxazole (co‐trimoxazole) who developed a penile drug eruption at the glans after having intercourse with his wife, who was taking sulfathiazole/sulfacetamide/sulfabenzamide (triple sulfa) vaginal cream. The nature of the lesion was confirmed by a rechallenge test.ConclusionTo our knowledge, this is the fourth case of proven postcoital penile drug eruption in a patient following vaginal use of triple sulfa vaginal cream in his partner. Our case illustrates the importance of history taking. In clinical practice of urology, it is not rare to see patients who present with strange penile lesions following coitus. To reach a correct diagnosis, one should obtain a drug history of the sexual partner and allergic history of the patient in such cases. Zargooshi J, Kavoussi H, Rahmanian E, Motaee H, Kohzadi M, and Nourizad S. Postcoital penile drug eruption in a co‐trimoxazole‐sensitive patient following vaginal use of triple sulfa vaginal cream by his partner. J Sex Med 2012;9:758–760.     

Nonischemic priapism following penile tattooing

IntroductionTo our knowledge, here we report the first case of nonischemic priapism following penile tattooing.AimTo report the first case of nonischemic priapism following penile tattooing.MethodsA case with tattooing‐induced priapism is presented including subjective reporting, physical examination, and laboratory/radiologic evaluations.ResultsA 21‐year‐old man, presented with partially rigid penis of 3‐month duration. On examination, the penis was half rigid, with a tattoo on its dorsal surface, and a smaller tattoo on the glans (Figure 1). The patient initially stated that the tattoo had been created years ago, but later admitted that he had it created just before the occurrence of priapism. A traditional tattooist created the tattoo manually, using a handheld needle. Bleeding from deep penile tissue for several days complicated the tattooing.Known etiologies of priapism were investigated and ruled out. Specifically, perineal injury, leukemia, sickle cell trait, thalassemia, urinary tract infection, neurogenic, neoplastic, infectious, toxic, and pharmacological causes were actively investigated and ruled out. There was no history of alcohol consumption or smoking. Aspirated penile blood was bright red. Cavernous blood gas measurements confirmed high oxygen and low carbon dioxide content, diagnostic of arterial priapism.There was no embolization facility in Kermanshah. In fact, there are few experts in superselective embolization in Iran. We referred the patient for superselective embolization. However, he underwent a nonindicated Sacher procedure. Predictably, the procedure was unsuccessful. At present, the patient continues to have priapism. Because of the painless nature of erections, moderately good preservation of erectile function during intercourses, and disappointment with former surgery, the patient declined further therapies, and he lives with his condition.ConclusionsTattooing should be added to the etiologies of nonischemic priapism. Considering this case, we discourage penile tattooing. Zargooshi J, Rahmanian E, Motaee H, and Kohzadi M. Nonischemic priapism following penile tattooing. J Sex Med 2012;9:844–848.     

Brainstem auditory evoked potential (BAEP) in brucellosis

BACKGROUND: Brucellosis is a zoonosis still endemic in many parts of the world including coastal countries of Mediterranean Sea, Middle East regions and Central and South America. Now in this study we surveyed whether patients with systemic brucellosis, BAEP differ from healthy persons or not. This will help us later, when the patients with brucellosis refer to perform BAEP to prevent misdiagnosis. MATERIALS AND METHODS: Fifteen patients with acute systemic brucellosis without neurological involvement and 15 apparently healthy persons underwent a brainstem auditory evoked potentials (BAEP) study. RESULTS: Comparison of pooled data between the systemic brucellosis and healthy groups showed no significant differences in all BAEP parameters. CONCLUSION: In conclusion, BAEP parameters in brucellosis group did not differ from healthy persons. Thus in interpretation of BAEP in patients that referred for another reason and have systemic brucellosis, it is not necessary to consider about the effect of brucellosis on BAEP.