Hypogonadotropic hypogonadism as a presenting feature of late-onset X-linked adrenal hypoplasia congenita.

Mutations in the orphan nuclear receptor DAX-1 cause X-linked adrenal hypoplasia congenita. Affected boys usually present with primary adrenal failure in early infancy or childhood. Impaired sexual development because of hypogonadotropic hypogonadism becomes apparent at the time of puberty. We report adult-onset adrenal hypoplasia congenita in a patient who presented with hypogonadism at 28 yr of age. Although he had no clinical evidence of adrenal dysfunction, compensated primary adrenal failure was diagnosed by biochemical testing. Semen analysis showed azoospermia, and he did not achieve fertility after 8 months of treatment with gonadotropins. A novel Y380D DAX-1 missense mutation, which causes partial loss of function in transient gene expression assays, was found in this patient. This case demonstrates that partial loss-of-function mutations in DAX1 can present with hypogonadotropic hypogonadism and covert adrenal failure in adulthood. Further, an important role for DAX-1 in spermatogenesis in humans is confirmed, supporting findings in the Dax1 (Ahch) knockout mouse.     

Effect of Topical Tetracycline on Seroma Formation in the Lichtenstein Technique: A Prospective Randomized Study

We sought to investigate whether application of topical tetracycline has a limiting effect on seroma formation in patients undergoing hernia repair using a polypropylene mesh. This study was conducted in 96 patients undergoing an elective groin hernia repair. Patients were randomized and divided into 2 groups. After the graft was placed, and before external oblique muscle aponeurosis was closed, 5 mL tetracycline was administered on the graft in the tetracycline group (tetra group, n = 50), and 5 mL isotonic saline was administered in the control group (n = 46) without putting in a drain. Seroma checks via surface ultrasonography were done. Seroma amounts measured on the first day were significantly higher in the tetra group (P = 0.04). There were no significant differences regarding seroma measurements on the seventh day or in the first and second months. Topical tetracycline application has no limiting effect on seroma formation after a groin hernia operation.Key words: Lichtenstein technique, Polypropylene mesh, Topical tetracycline, Seroma, UltrasonographyHernia operations are among the most frequently performed surgical operations. Graft use to repair the defect is commonplace because graft use decreases recurrence rate, and defect closure in large abdominal hernias is easier with a mesh.¹ The most commonly used and least expensive mesh is the polypropylene mesh. This mesh causes a foreign-body reaction where it is placed, which leads to seroma formation.² Repeat aspirations of seromas cause infection, and recurrence is seen on follow-up, especially in large hernias.² While drain use is not recommended in groin hernias, drain use in other abdominal-wall hernias is left to the physician''s discretion. The biggest problem with drain use is transport of the infection by an ascending route.³Tetracycline is an antibiotic, the solution form of which is topically used for pleurodesis in chest surgery.⁴ Some researchers also report successful topical use of its solution in seroma treatment, taking advantage of its sclerosing effect in cases with breast carcinoma.^5,6 Tetracycline has demonstrated increased growth factor–like activity in mesothelial cells exposed to tetracycline leading to fibroblast proliferation.⁷ This study sought to investigate whether tetracycline has a reducing effect on seroma development in cases undergoing elective groin hernia repair using a polypropylene mesh.     

Reactive Hemophagocytic Lymphohistiocytosis After Hepatitis A Infection

Hemophagocytic lymphohistiocytosis (HLH) is a clinical condition which result in cytotoxic Tcell and antigen presenting cell overproduction and also their cytokines. Hepatitis A Virus associated HLH is very rare condition among other viruses. This condition is often difficult to diagnose, so treatment is often delayed. Here we present a case of adolescent boy with secondary virus associated HLH diagnosis with Hepatitis A infection and successfull treatment by short course of intravenous immunoglobulin and steroid.     

Sex Estimation From Sternal Measurements Using Multidetector Computed Tomography

We aimed to show the utility and reliability of sternal morphometric analysis for sex estimation.Sex estimation is a very important step in forensic identification. Skeletal surveys are main methods for sex estimation studies. Morphometric analysis of sternum may provide high accuracy rated data in sex discrimination.In this study, morphometric analysis of sternum was evaluated in 1 mm chest computed tomography scans for sex estimation. Four hundred forty 3 subjects (202 female, 241 male, mean age: 44 ± 8.1 [distribution: 30–60 year old]) were included the study. Manubrium length (ML), mesosternum length (2L), Sternebra 1 (S1W), and Sternebra 3 (S3W) width were measured and also sternal index (SI) was calculated. Differences between genders were evaluated by student t-test. Predictive factors of sex were determined by discrimination analysis and receiver operating characteristic (ROC) analysis.Male sternal measurement values are significantly higher than females (P < 0.001) while SI is significantly low in males (P < 0.001). In discrimination analysis, MSL has high accuracy rate with 80.2% in females and 80.9% in males. MSL also has the best sensitivity (75.9%) and specificity (87.6%) values. Accuracy rates were above 80% in 3 stepwise discrimination analysis for both sexes. Stepwise 1 (ML, MSL, S1W, S3W) has the highest accuracy rate in stepwise discrimination analysis with 86.1% in females and 83.8% in males.Our study showed that morphometric computed tomography analysis of sternum might provide important information for sex estimation.     

Ovarian torsion in puerperium: A case report and review of the literature

INTRODUCTION

Ovarian torsion (OT) is a rare cause of acute abdominal pain that requires prompt recognition and treatment during puerperium. Diagnosis of OT can be challenging due to nonspecific clinical features and uncommon objective findings. The management of OT is often delayed because of diagnostic uncertainty. Early and timely recognition and prompt intervention are crucial to preserve ovarian function and to minimize morbidity.

PRESENTATION OF CASE

We report a 29-year-old postpartum woman who presented to the emergency department (ED) with severe right flank pain, nausea and anorexia initially considered as renal colic. After further investigation, OT caused by large mucinous cyst was diagnosed. Right-sided salpingo-oophorectomy was performed due to hemorrhagic ovary and huge cystic mass causing ischemic OT.

DISCUSSION

OT is often diagnosed based on the clinical presentation, including severe, sharp, sudden onset of unilateral lower abdominal pain and tenderness with a palpable laterouterine pelvic mass and nausea/vomiting. Emergency surgical intervention should be performed if OT is suspected to confirm the diagnosis and uncoil the twist to prevent ovarian damage.

CONCLUSION

In conclusion, emergency physicians should be aware of the possibility of OT in postpartum women. Therefore, early and timely surgical intervention should be undertaken.     

Recommendations for Hepatitis B Immunoglobulin and Antiviral Prophylaxis Against Hepatitis B Recurrence After Liver Transplantation

The combination of hepatitis B immunoglobulin and potent nucleos(t)ide analogs after liver transplantation is considered as the standard of care for prophylaxis against hepatitis B virus recurrence. However, the recommended doses, route of administration, and duration of HBIG administration remain unclear. Moreover, hepatitis B immunoglobulin-free prophylaxis with potent nucleos(t)ide analogs has shown promising disease outcomes in preventing hepatitis B virus recurrence. The current recommendations, produced by the Turkish Association for the Study of the Liver, Acute Liver Failure and Liver Transplantation Special Interest Group, suggest a reduced need for hepatitis B immunoglobulin administration with effective long-term suppression of hepatitis B virus replication using potent nucleos(t)ide analogs after liver transplantation.     

Laparoscopic enucleation of mesenteric cyst: a case report

Intra-abdominal lesions, such as mesenteric cysts, are uncommon disorders. Most are discovered incidentally during routine abdominal examinations. They generally do not show typical clinical findings. Laparoscopic surgery has been used to remove a wide variety of mesenteric cysts. We report a patient with a mesenteric cyst, who was treated by laparoscopic enucleation.     

Infarction of the septomarginal band and tricuspid papillary muscle rupture related to alcohol septal ablation for hypertrophic cardiomyopathy

We presented a 77‐year‐old man with hypertrophic obstructive cardiomyopathy applied with flail tricuspid leaflet and severe tricuspid regurgitation leading to right heart failure 2 months after the failed septal ablation. The ruptured anterior tricuspid papillary muscle resulted from infarction of the base of anterior papillary muscle of the right ventricle (RV) confirmed by magnetic resonance imaging. As the septomarginal band is frequently lit up by intracoronary contrast that particular attention should be paid to the RV papillary muscles. And, if the papillary muscles or the RV free wall is brightened, then the use of that septal artery should be avoided.