Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy

Background

Dense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade.

Case-diagnosis/treatment

A 14-year-old girl presented with edema and nephrotic range proteinuria. Blood tests showed hypoalbuminemia, nephrotic range proteinuria, normal renal function, and a low C3 level. Renal biopsy confirmed the diagnosis of crescentic DDD. Complement analysis revealed strong AP activation (low C3), positive C3 nephritic factor (C3NeF), and a decreased complement factor H (CFH) levels with CFH polymorphisms. Therapy with eculizumab was considered after the failure of corticosteroid and plasmapheresis to modulate the ongoing massive proteinuria and persistence of low serum C3 levels. There was a marked clinical and biochemical response following the administration of eculizumab.

Conclusions

Our case emphasizes the efficacy of eculizumab in the management of crescentic DDD in a patient with a normal renal function, in a short follow-up period. Considering previously reported cases, it appears that eculizumab represents a promising new approach which may prevent progression to ESRD in a subset of patients with DDD.     

Sleep quality in patients with systemic sclerosis: relationship between the clinical variables, depressive symptoms, functional status, and the quality of life

The aim of this study is to evaluate the relationship between the sleep quality and the disease-related variables, functional status, quality of life, and depressive symptoms in patients with systemic sclerosis (SSc). Forty-eight patients diagnosed with SSc and 42 healthy control subjects were enrolled in the study. The demographic and clinical characteristics of the patients such as the Raynaud’s phenomenon, SSc subtype, digital ulcers, gastrointestinal and lung involvement, and disease activity were recorded. All patients were assessed using the short form 36 (SF-36) quality of life scale, the health assessment questionnaire and the beck depression inventory. Generalized pain and fatigue were assessed with the Visual Analoge Scale. For the evaluation of the sleep disturbance, the SSc and control groups were assessed with the help of the Pittsburgh Sleep Quality Index (PSQI). The patients with SSc had significantly higher scores in the subjective sleep quality, sleep latency, habitual sleep efficiency, sleep disturbance, daytime dysfunction domains, and in terms of the total PSQI score compared to the healthy control group (p < 0.05). According to the results of spearman’s analysis, there was a significantly higher correlation between the generalised pain, fatigue, depressive symptoms, functional status, and physical score of the SF-36 and the sleep disturbance (p < 0.01). There was also a significantly lower correlation between the menopause status, dyspnoea, gastroesophageal reflux, dysphagia, the mental score of the SF-36, and the sleep disturbance (p < 0.05). The sleep quality is disturbed in patients with SSc. The lower quality of sleep is especially associated with the pain, fatigue, depressive symptoms, and functional status.     

A new classification for septal perforation and effects of treatment methods on quality of life

IntroductionSeptal perforation is a condition characterized by loss of cartilage and/or bony structures along with the mucoperichondrium and mucoperiosteum lining them. The etiology includes a history of nasal surgery or trauma, nose picking, bilateral septal cauterization, overuse of nasal sprays, cocaine abuse, vasculitis, and malignancies.ObjectiveComparison of quality of life in patients with septal perforation after conservative or surgical treatment, and a new approach for the determination of the diameter of the perforation from a different point of view.MethodsThe diameter of septal perforation, total vertical diameter of septum, and horizontal diameter of the perforation were measured in a total of 34 patients. Nineteen of the patients underwent surgical septal perforation repair, and 15 of them received septal button application. The patients were asked to complete the Glasgow Benefit Inventory quality of life questionnaire.ResultsThe septal perforation successfully healed in 18 of 19 patients who underwent surgical treatment. The quality of life scores were statistically significantly higher in the surgical treatment group when compared to the button group (p < 0.05).ConclusionThe septal perforation classification we propose would be beneficial for providing realistic dimensions, treatment methods, and surgical techniques.     

Evaluating the Effects of a Tissue Selective Estrogen Complex (TSEC) in Women with Newly Diagnosed Ductal Carcinoma In Situ

Annals of Surgical Oncology -     

Total versus subtotal thyroidectomy for the management of benign multinodular goiter in an endemic region

BACKGROUND: Because controversy still continuous to surround use of total thyroidectomy for the management of benign multinodular goiter, the present study aims to prospectively compare the safety and efficacy of total thyroidectomy with subtotal thyroidectomy. METHODS: A total of 200 consecutive patients with benign multinodular goiter were assigned to have either total thyroidectomy (n = 105) or subtotal thyroidectomy (n = 95) based on preoperative evaluation, intraoperative macroscopic findings and nodular dissemination. The patients with no healthy tissue or nodules localized in the dorsal part of the gland, which are usually left during normal subtotal resection, were assigned to the total thyroidectomy group. Demographic details, biochemical findings, indications for operation, operating time, specimen weight, complications and hospital stay were noted. RESULTS: There was no significant difference in the sex, hormonal status or duration of goiter between the two groups (P = 0.74, P = 0.59 and P = 0.59, respectively). The mean operating time was longer (148.52 min +/- 51.10 vs 135.10 min +/- 32.47, P = 0.03), and the mean weight of the specimens was greater (228.40 g +/- 229.91 vs 157.01 g +/- 151.23, P = 0.01) for total rather than subtotal thyroidectomy. Either temporary recurrent laryngeal nerve (RLN) palsy or hypoparathyroidism occurred in 10 (9.3%) or 12 (11.4%) of the patients undergoing total compared with six (6.3%) or nine (9.5%) of the patients undergoing subtotal thyroidectomy (P = 0.40 and P = 0.65, respectively). Either permanent RLN palsy or hypoparathyroidism was observed in one patient undergoing total thyroidectomy (P = 0.34 for each comparison). The mean hospital stay was longer in the total thyroidectomy group (2.24 days +/- 1.18 vs 1.89 days +/- 0.72 for subtotal thyroidectomy, P = 0.01). CONCLUSIONS: The present study shows that total thyroidectomy can be performed without increasing risk of complication, and it is an acceptable alternative for benign multinodular goiter, especially in endemic regions, where patients present with a huge multinodular goiter.     

Effects of intranasal midazolam and rectal diazepam on acute convulsions in children: prospective randomized study

In this study, the effects and side effects of rectal diazepam and intranasal midazolam were compared in the treatment of acute convulsions in children to develop a practical and safe treatment protocol. In the diazepam group, the seizures of 13 (60%) patients terminated in 10 minutes; however, 9 (40%) patients did not respond. In the midazolam group, 20 (87%) patients responded in 10 minutes, but 3 (13%) patients did not respond. Regarding the anticonvulsant effect, midazolam was found to be more effective than diazepam, and the difference was statistically significant (P < .05). The necessity of a second drug for the seizures that did not stop with the first drug was higher in the diazepam group than the midazolam group, and the difference was statistically significant (P < .05). We conclude that as an antiepileptic agent, intranasal midazolam is more effective than rectal diazepam. After administration, we did not observe any serious complications. Further investigations are necessary; however, intranasal administration is easy, so if the nasal drop and spray forms used in some European countries and the United States are available worldwide, it will be very useful for physicians in the emergency room.     

Nonmelanoma skin cancer

Each year, there are as many cases of nonmelanoma skin cancer as all other cancers combined. Although there is relatively low attributable mortality, the morbidity and expense of treatment is significant. Unlike many other malignancies, host and environmental factors relevant to the pathophysiology have been clearly demonstrated. Surgical ablation remains the mainstay of treatment.     

Laparoscopically harvested omental flap for chest wall and intrathoracic reconstruction

Minimally invasive surgery has gained popularity in the last decade and its applications to plastic surgery are expanding. Pedicled omental flaps are used for the reconstruction of chest wall defects following debridement of sternal infections and mediastinitis. The main advantages of using an omental flap are its large size and bulk to fill large 3-dimensional dead spaces, long pedicle, and rich vascular and lymphatic networks. Recently, laparoscopic techniques have been described for harvesting omental flaps. Over the last 5 years in our institution, 9 laparoscopic omental flap harvests were performed. Seven were used in the reconstruction of complicated chest wall defects, sternal infections, mediastinal abscesses, and mediastinitis following cardiac surgery. Two were used to repair intrathoracic viscera. Prior abdominal surgery was not a contraindication to the laparoscopic harvest. In 1 patient, the omental transfer was converted to a free flap due to the detachment of the pedicle, and in 1 patient the omental harvest was converted to open technique due to technical difficulty due to severe abdominal adhesions. None of the patients had major intraabdominal complications postoperatively. One patient had a small transdiaphragmatic hernia treated by laparoscopic techniques. The use of laparoscopy techniques facilitated the harvesting of the omentum, making it ideal in the treatment of complicated patients with multiple comorbidities. With these techniques, pedicled omental flaps will be a reasonable treatment option for chest wall reconstruction.