Results of the ACTH stimulation test in hirsute women

OBJECTIVE: To determine the incidence of late-onset congenital adrenal hyperplasia (LOCAH) due to 21-hydroxylase deficiency among hirsute women and to evaluate the results of the ACTH stimulation test with the clinical characteristics. STUDY DESIGN: Prospective, controlled study. One hundred women with hirsutism and 14 normally cycling women without hirsutism were included in this study at the Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, Cerrahpasa School of Medicine, Istanbul University. After basal serum progesterone (P) and 17 hydroxyprogesterone (17OHP) levels were determined, an ACTH stimulation test was performed on cycle day 3-5. The same parameters were checked 30 minutes later. We estimated the 21 hydroxylase activity by calculating the change in 17OHP (17OHP 30-0) and the summed rate of the change in P and 17OHP ([P30-0] + [17OHP30-01/30 minutes). The 95th percentile for these estimates in normal women were calculated, and values above three times the 95th percentile were considered to distinguish women with LOCAH due to 21-hydroxylase deficiency. RESULTS: The 95th percentile for 17OHP 30-0 and (P30-0) + (17OHP30-0)/30 minutes in normal women was 1.6 and 8.9 ng/dL/min, respectively. Regarding 17OHP 30-0 values, three women with hirsutism had levels above three times the 95th percentile of these estimates, and 28 women had estimates of more than the 95th percentile but less than threefold. Seventeen of 28 women had oligomenorrhea, and all had severe hirsutism. The women with severe hirsutism and oligomenorrhea had significantly higher ACTH-stimulated serum 17OHP levels and values for 17OHP 30-0 and (P30-0 + (17OHP30-0)/30 min) than did normally cycling women. CONCLUSION: The incidence of LOCAH due to 21-hydroxylase deficiency and mild 21-hydroxylase deficiency is 3% and 28%, respectively, in women with hirsutism. Clinical characteristics are not helpful in determining 21-hydroxylase deficiency. However, the incidence of 21-hydroxylase deficiency is more common among women with severe hirsutism and oligomenorrhea. The change in serum 17OHP 30-0 seems to be greater than the summed rate of change in serum 17OHP and P in the detection of 21-hydroxylase enzyme deficiency.     

Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome

Introduction

The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, further investigations are needed with larger numbers of patients.

Chondromyxoid fibroma involving the entire metacarpal: a case report

The occurrence of chondromyxoid fibroma in the hand is rare. We report a case of chondromyxoid fibroma involving the whole fourth metacarpal that was treated by curettage and cancellous bone allograft.     

Radiological outcome of proximal femoral varus osteotomy for the treatment of lateral pillar group-C Legg-Calvé-Perthes disease

Results from 26 hips of 24 Legg-Calvé-Perthes disease patients were evaluated retrospectively. There were 22 male and two female patients in the study group. The mean age of the patients was 8.3 years and the mean follow-up was 13.03 years. All the patients were older than 6 years of age and all of them had lateral pillar group C hips. The patients were treated with intertrochanteric uniplanar varus osteotomy. All the patients were mature at the time of the last evaluation. Radiological end results were evaluated according to Stulberg's classification. Six hips healed with spherical congruency (Stulberg class 1 or 2), 19 with aspherical congruency (Stulberg class 3 or 4) and one with aspherical incongruency (Stulberg class 5). Six of 14 hips treated before 9 years of age healed with spherical congruency but all hips treated after 9 years of age healed with Stulberg class 3, 4 or 5 results. Trochanteric overgrowth was evident in 20 hips. The results of this radiological outcome study showed that Herring class C hips treated with proximal femoral varus osteotomy had a poor outcome, especially after 9 years of age in this group of patients.     

The comparison of the efficacy of scoring systems in organophosphate poisoning

The purpose of this study was to evaluate the impact of the Glasgow Coma Scale (GCS), Acute Physiology and Chronic Health Evaluation (APACHE) II and Simplified Acute Physiology Score (SAPS) II scoring systems for organophosphate poisoning (OPP) in an intensive care unit (ICU). The following data were collected on all consecutive patients who were admitted to the ICU between June 1999 and December 2004. Demographic data, GCS, APACHE II and SAPS II scoring systems were recorded. Predicted mortality was calculated using original regression formulas. Standardized mortality ratio (SMR) was computed with 95% confidence intervals (CI). The sensitivity and specificity for each scoring system were evaluated by calculating the Area Under the Receiver Operating Characteristic Curves. The actual mortality in OPP was 21.9%. Predicted mortality by all systems was not significantly different from actual mortality [SMR and 95% CI for GCS: 1.00 (0.65 1.35), APACHE II: 0.87 (0.54-1.03), SAPS II: 1.40 (0.98-1.82)]. The area under the ROC curve for APACHE II is largest, but there is no statistically significant difference when compared with SAPS II and GCS (GCS 0.900 +/- 0.059, APACHE II 0.929 +/- 0.045 and SAPS II 0.891 +/- 0.057). In our ICU group of patients, in predicting the mortality rates in OPP, the three scoring systems, which are GCS, APACHE II and SAPS II, had similar impacts; however, GCS system has superiority over the other systems in being easy to perform, and not requiring complex physiologic parameters and laboratory methods.     

Squamous cell carcinoma arising from a fibroepithelial polyp

Fibroepithelial polyps (FEPs) are among the most common benign skin lesions treated frequently without submitting for histologic examination since malignant degeneration is exceedingly rare. In this case report, we describe an aggressive gross squamous cell carcinoma mass arising from a large pedunculated-type FEP located at the lower limb, which, to our knowledge, has not been reported yet.     

Mechanisms of atrial fibrillation: lessons from studies in patients

Recent clinical studies performed in human subjects have provided important clues that improved our understanding of the mechanisms of atrial fibrillation (AF) and facilitated development of new treatment strategies. When analyzed together, these studies confirm the complexity and multifactorial nature of AF. Because a variety of mechanisms such as focal drivers within the thoracic veins, rotors in the left atrium, multiple reentrant circuits, and autonomic innervation may play a role in the initiation and maintenance of AF alone or in combination, the best strategy to eliminate AF may be the accurate identification of one or more of the mechanisms critical to the genesis of AF and to target the specific mechanism(s).     

Ungewöhnliche Ursache einer selbstinduzierten fokalen Epilepsie

Epilepsies and psychiatric disorders such as psychoses are known to have reciprocal influence. There are problems in patient compliance and pharmacological interactions between the drugs used for treatment. We report an unusual, self-induced, focal, symptomatic epilepsy in a hitherto unrevealed schizophrenic psychosis. The interdisciplinary treatment of three different departments was necessary. The initial symptom of a diffuse frontal headache and the patient's penetration of his own skull in an attempt at self-treatment led to the development of a frontal abscess around a foreign body and the described focal epilepsy.     

Scintigraphic findings in osteoarticular brucellosis

AIMS: To describe the distribution of bone and joint involvement in 197 patients with brucellosis, and to detail a spectrum of findings on bone scintigraphy in 38 patients with brucellar spondylitis. METHODS: One hundred and ninety-seven patients (141 females, 56 males; age range, 5-77 years) with osteoarticular brucellosis were studied. Patients were classified into acute (62%) and chronic (38%) stages of the disease, and into age groups of less than 16 (1%), 16-30 (17%), 31-45 (29%), 46-60 (37%) and over 60 (16%) years. All patients were evaluated with Tc-methylene diphosphonate bone scanning. Quantification of sacroiliac joint uptake was performed to improve the sensitivity for the detection of sacroiliitis. Plain radiography and computed tomography (CT) or magnetic resonance imaging (MRI) were performed, when required, to evaluate the areas of the skeleton that showed abnormal uptake on the bone scan. MRI and single photon emission computed tomography (SPECT) were performed in all patients who had spinal lesions. RESULTS: The sites most commonly affected were the sacroiliac joints (53%) and spine (19%), followed by the shoulders (16%). Osteoarticular involvement was more common in females (72%) than in males (28%), and the acute stage (62%) was observed more than the chronic stage (38%). Bone and joint involvement occurred at any age, but the most common age group was 46-60 years. Eight scintigraphic patterns were identified in spinal involvement. CONCLUSION: Brucellosis may affect the musculoskeletal system at any site. Bone scan is a useful method to detect osteoarticular involvement in cases of relapse and progression. Spine involvement has the widest range of scintigraphic findings. A knowledge of the location and distribution of osteoarticular involvement as revealed on the bone scan of patients with brucellosis may be valuable in patient treatment and management.