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We aimed to investigate the effect of superior hypogastric plexus (SHP) block on pain relief and quality of life of patients with refractory endometriosis. Sixteen patients with refractory endometriosis underwent SHP block. The outcome measures included visual analogue scale (VAS) for chronic pelvic pain, VAS for dysmenorrhoea, and VAS for dyspareunia. The short-form endometriosis health profile (EHP-5) was used to measure quality of life. All the outcome measures were evaluated at weeks 0, 1, 4, 12 and 24. The mean baseline VAS scores significantly improved after the SHP block ( p  < .001 for all). The mean overall EHP-5 score also significantly improved from 54.3 ± 18.2 to 24.6 ± 13.3 ( p  < .001). The positive effects of SHP were not diminished over time. No serious adverse effect was noticed in any of the patients. Preliminary results suggest that SHP block could be used as an effective method in pain control and improvement of quality of life in refractory endometriosis.IMPACT STATEMENT What is already known on this subject? Safety and efficacy of SHP block in the treatment of CPP has been revealed in earlier investigations. However, the efficacy of SHP block for pain management in patients with refractory endometriosis has not been investigated in earlier investigations. What do the results of this study add? SHP block is an effective method for pain control and improvement of quality of life in patients with refractory endometriosis. The positive effects of this treatment did not diminish over 24-weeks follow-up of the study. No serious adverse effect was noticed in any of the patients. What are the implications of these findings for clinical practice and/or further research? Preliminary results suggest that SHP block could be used safely and effectively for controlling pain and improvement of quality of life in patients with refractory endometriosis.

Background: This study assessed the effects of a lactobacillus-based medication on pain intensity scores in women with endometriosis., Materials and Methods: The present randomized pilot placebo-controlled trial was done on eligible women who were surgically and pathologically diagnosed with endometriosis. Thirty-seven participants who had not received hormonal treatment in the last three months, were enrolled and randomized into LactoFem® and placebo groups. Lactobacillus capsules or placebo were administrated orally once a day for 8 weeks. Patients were assessed for pain severity using Visual Analogue Scale (VAS) scores for dysmenorrhea, dyspareunia and chronic pelvic pain at baseline and after 8 and 12 weeks post-intervention., Results: Mean age of participants and mean body mass index (BMI) for the LactoFem® and control groups were comparable. All patients had stage 3 and 4 of the disease based on revised American fertility society (AFS) classification of endometriosis. Mean initial pain scores for dysmenorrhea, dyspareunia and chronic pelvic pain were 6.53 ± 2.88, 4.82 ± 3.76 and 4.19 ± 3.53, respectively in the LactoFem® group and 5.60 ± 2.06, 3.67 ± 2.64 and 2.88 ± 2.80, respectively for the control group; the two groups had comparable scores in this regard. There was more decrease in pain scores for both dysmenorrhea and the overall pain after 8 weeks of treatment in LactoFem® group compared to the control group. The scores for dysmenorrhea were 6.53 ± 2.88 and 5.60 ± 2.06 in the LactoFem® and control groups, respectively, before intervention but, after 8-week treatment, these values were 3.07 ± 2.49 and 4.47 ± 2.13 (P=0.018), respectively. The changes in overall pain score in the LactoFem® and control group during this period were 7.33 ± 7.00 and 4.11 ± 1.68, respectively (P=0.017)., Conclusion: This study showed some beneficial effects of lactobacillus administration on endometriosis-related pain (Registration number: IRCT20150819023684N5)., Competing Interests: The authors declare no conflicts of interest., (Copyright© by Royan Institute. All rights reserved.)

Atopic dermatitis (AD) is an inflammatory skin disease in which both genetic and environmental factors seem to be involved. Several studies investigated the association of certain genetic factors with AD in different ethnic groups, but conflicting data were obtained. This study was performed to check the possible association between single nucleotide polymorphisms (SNPs) of interleukin 4 (IL-4) and the IL-4 receptor α chain (IL-4Rα) and AD in a group of Iranian patients. The allele and genotype frequencies of genes encoding for IL-4 and IL-4Rα were investigated in 89 patients with AD in comparison with 139 healthy controls, using methods based on polymerase chain reaction sequence-specific primers. The most frequent alleles of IL-4 in patients were T at -1098 (P<0.001, odds ratio (OR)=2.35), C at -590 (P<0.001, OR=4.84) and C at -33 (P=0.002, OR=2.08). The most frequent genotypes of IL-4 in patients were TT, CC, and CC at positions -1098 (P<0.001, OR=3.59), -590 (P<0.001, OR=31.25) and -33 (P<0.001, OR=3.46), respectively. We found a significant lower frequency of GT at -1098 GT, TC at -590, and TC at -33 in patients. There were no statistically significant differences in the frequency of alleles and genotypes of IL-4Rα gene at position +1902. A strong positive association was seen between TCC haplotype and AD (68% in patients vs. 23.4% in controls, P<0.001, OR=8.91). We detected a significantly lower frequency of TTC, GCC, and TTT haplotypes (P<0.001, OR=0.02, P<0.001, OR=0.40, P<0.001, OR=0.39, respectively) in patients compared to controls. A significant association between the polymorphisms of the IL-4 gene promoter at positions -1098, -590, and -33 and AD was detected in the Iranian population.

Atopic dermatitis (AD) is a relapsing chronic pruritic inflammatory disease of skin for which no monogenic cause has been identified so far. Meanwhile, the filaggrin (FLG) gene is considered as the most important gene associated with predisposition to the disease. One hundred and six patients with AD and 105 healthy individuals were enrolled in this study. Real time polymerase chain reaction was performed to determine frequencies of alleles and genotype in six variants of the FLG gene. The frequencies of allele A in variants of rs3126065, rs2786680, and rs1933063 as well as allele C in variant rs3814300 were 100%. There was no significant difference between allele frequencies in variants rs2485518 and rs3814299. The only genotypes in variants of rs3814299 and rs2485518 were GG and CC, respectively, with no significant difference between the patients and controls. This study demonstrated that there was no significant association between polymorphisms of FLG gene variants and AD.

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal-recessive disease caused by mutations in the NTRK1 gene. The disease is characterized by insensitivity to pain and absence of thermal perception. Herein a 6-year-old boy is presented with a large ulcer on the sole of his right foot and a thick, hyperkeratotic appearance of his palms and soles; there was also a medical history of hyperthermia, anhidrosis, recurrent bone fractures, osteomyelitis, injuries, mental retardation, dry and exfoliative skin, insensitivity to pain, and lack of thermal sensation. Genetic studies revealed a homozygote mutation in the NTRK1 gene. Although the patient initially presented with palmoplantar keratoderma, genetic studies confirmed the diagnosis of CIPA., (© 2013 Wiley Periodicals, Inc.)

Stevens-Johnson syndrome is an uncommon inflammatory skin disorder in which immune mechanisms, cytotoxic reactions, and delayed hypersensitivity seem to be involved. Herein, an unusual case with strange complaint of"bus allergy" is presented, suffering from severe generalized itching and skin lesions, followed by ulcers in his mouth and genitalia. The diagnosis of Stevens-Johnson syndrome was made and appropriate treatment was advised. However, detailed medical history revealed a completely different cause of his allergic reactions, as he had occasionally used dimenhydrinate because of motion sickness in his history. Therefore, adverse drug reaction to dimenhydrinate was considered as the main underlying cause of the disease. In conclusion, thorough medical history should be taken to make a definitive diagnosis and identify the underlying disease, since accurate diagnosis and appropriate treatment can prevent further complications.

Purpose: This study was performed in 24 members of a family with spontaneous pneumothorax to test clinical suspicion of Birt-Hogg-Dubé syndrome (BHDS)., Methods: Computed tomography scan was performed for confirmation of pneumothorax, while genetic tests were done using real-time quantitative polymerase chain reaction., Results: Genetic studies showed a deletion of exon 1 in the FLCN gene in the index case as well as nine other individuals, including two with clinical phenotypes of pneumothorax and seven who are symptom-free to date., Conclusions: Proper imaging and taking accurate family history could be the keys to test clinical suspicion in some syndromes, including BHDS., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Tumor necrosis factor-alpha (TNF-α) could be considered as potential biomarkers in atopic dermatitis (AD), while its level could be influenced by cytokine single gene polymorphisms (SNP). This study was performed in 89 pediatric patients with AD and 137 controls to assess polymorphisms of the TNF-α gene at positions -308 and -238, using the polymerase chain reaction and the sequence-specific primers method. The highest positive allelic association that made the patients susceptible to AD was seen for TNF-α -238/G (p<0.001) and TNF-α -308/G (p = 0.003). The GG genotypes at TNF-α -238 and TNF-α -308, were both significantly higher in the patients with AD, compared to the controls (p<0.01). The GG haplotype at TNF-α (-308,-238) was seen in 92.7% of the patients, which was significantly higher than the controls (p<0.001), while a negative haplotypic association with AD was seen for TNF-α (-308, -238) AG and GA (p<0.01). This study showed that the AG genotype of TNF-α -308, associated with a high production of cytokines, was significantly decreased in patients with AD, while the low-producing GG genotype, which could lead to low production of TNF-α, was over-expressed in the atopic patients.

X-linked agammaglobulinemia (XLA), also known as Bruton's tyrosine kinase (BTK) deficiency, is a primary antibody deficiency, characterized by low number of B cells, agammaglobulinemia and increased susceptibility to a variety of infections. Herein, we report a case of XLA with confirmed BTK mutation that developed neurological deficits. While we could not detect any responsible microorganism in spite of comprehensive workup, brain magnetic resonance imaging revealed moderate brain atrophy. The diagnosis of progressive encephalitis was made for this patient. Patients with XLA have a higher chance of encephalitis compared with other primary antibody deficiencies. Given the violent nature of encephalitis, it is a concern among XLA patients.