Two cases of macrocephaly and immune deficiency

Macrocephaly describes a head circumference greater than two standard deviations above the mean and is a feature of a number of genetic syndromes. Here we report on two patients with microcephaly, immune deficiency and anemia. In addition, one case had periventricular leukomalacia and the other case had myelinisation delay in periventricular white matter development. These cases may represent a distinct new syndrome.     

Simple preoperative parameters to assess technical difficulty during a radical perineal prostatectomy

Purpose

We aimed to propose a practical selection method predicting the easier radical perineal prostatectomy (RPP) cases before the operation.

Methods

Fifty sequential RPP cases were divided into two groups according to the estimated difficulty of the operation (Group I: Easier, Group II: Difficult) which was assessed by using a RPP difficulty scale, constituted by three parameters (operation time, blood loss, and the judgment of the surgeon) each ranging between 1 and 3 points. As the localization parameters, skin-to-prostatic apex (SPAD) and skin-to-prostatic base (SPBD) distances and distance between bilateral ischial tuberosities (ITD) were measured. During suprapubic ultrasonography, a probe-divergence angle (PDA) and prostate volumes (PV) were recorded. These parameters were compared between the groups.

Results

In Group I (n = 29) and Group II (n = 21), the difficulty scores were 4.37 (3–5) and 6.80 (6–9), respectively. Data of age, clinical stages, and findings of digital rectal examination were not different between groups. While SPBD, SPAD, and ITD values were found similar (p > 0.05), PDA and PV were significantly different. PDA was > 45 degree in 21 cases in Group I (72.4 %) and in 7 cases in Group II (33.3 %) (p = 0.011). The mean of PV was 37.4 (20–60) cc and 49.9 (30–75) cc in Group I and Group II, respectively (p = 0.001).

Conclusions

While planning RPP operations, by selecting the prostate cancer cases with a prostate of low volume and localized deeper in the pelvis during suprapubic ultrasonography, urologists may have a chance to perform this technique more easily during the learning period.     

Protective effects of melatonin and memantine in human retinal pigment epithelium (ARPE-19) cells against 2-ethylpyridine-induced oxidative stress: implications for age-related macular degeneration

Purpose: To investigate the possible protective effects of melatonin and memantine (MMT) against 2-ethylpyridine (2-EP)-induced oxidative stress and mitochondrial dysfunction in human RPE (ARPE-19) cells in vitro.

Materials and methods: The ARPE-19 cells were divided into seven groups. Oxidative stress was triggered by incubating the ARPE-19 cells with 30?μM of 2-EP for 24?h. Then, 200?μM of melatonin was administered over three days and 20?μM of MMT over six hours prior to the experiment. The effects of melatonin and MMT on the intracellular calcium release mechanism, reactive oxygen species production, caspase-3 and caspase-9 activities, as well as vascular endothelial growth factor levels were measured.

Results: Melatonin and MMT were found to significantly decrease apoptosis levels. The intracellular calcium release was regulated by both melatonin and MMT. Further, melatonin and MMT significantly decreased both caspase-3 and caspase-9 activities, as well as pro-caspase and poly(ADP-ribose) polymerase expression, in ARPE-19 cells. Moreover, melatonin significantly increased the protective effect of MMT. The combination of melatonin and MMT significantly decreased 2-EP-induced oxidative toxicity and apoptosis by inhibiting the intracellular reactive oxygen species production and mitochondrial depolarization levels.

Conclusions: These notable findings are the first to demonstrate the synergistic protective effects of melatonin and MMT against 2-EP-induced oxidative stress in ARPE-19 cells.     

Isolated volar surgical approach for the treatment of perilunate and lunate dislocations

Background:

Volar and/or dorsal surgical approaches are used for surgical treatment of perilunate and lunate dislocations. There are no accepted approaches for treatment in the literature. We evaluated the functional results of isolated volar surgical approach for the treatment of perilunate and lunate dislocation injuries.

Materials and Methods:

9 patients (6 male and 3 female patients average age 34.5 ± 3.6 years) diagnosed with perilunate or lunate dislocations between January 2000 and January 2009 were involved in the study. The reduction was performed through isolated volar surgical approach and K-wire fixation, fracture stabilization with volar ligament repair was performed. Range of wrist joint motion, fracture healing, carpal stability, grip strength, return to work were evaluated and also direct radiographs were taken routinely at each control. The scapholunate interval and the scapholunate angle were evaluated radiographically. Evaluations of the clinical results were done using the DASH, VAS and Modified Mayo Wrist Scores.

Results:

The physical rehabilitation was started at 6^th week, after the K-wires were removed. The average followup was 18.2 months (range 12-28 months). At the final followup, the average flexion extension arc was 105.0 ± 9.6° (74.6% of the other side), the average rotation arc was 138.8 ± 7.8° (81.5% of the other side) and the average radioulnar arc was 56.1 ± 9.9° (86.4% of the other side). The grip strength was 0.55 bar; 83.2% that the uninjured arm. According to the Mayo Modified Wrist score, the functional result was excellent in five patients and good in four and the average DASH score was 22.8. The scapholunate interval was 2.1 mm and scapholunate angle was 51°.

Conclusion:

The clinical and radiological results of the isolated volar surgical approach were satisfactory. The dorsal approach was not needed for reduction of dislocations during operations. Our results showed that an isolated volar approach was adequate.     

An analysis of children with brucellosis associated with pancytopenia

Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitted to Yuzuncu Yil University Hospital between 2004 and 2010, were analyzed retrospectively. Over this time period, 187 patients with brucellosis were diagnosed. Twenty-five (13.3%) of 187 patients had pancytopenia during admission to hospital. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titers were 1:320 in 1 patient and 1:1280 in 24 patients. Blood culture was positive for Brucella melitensis in 3 patients (12%). Fever was the most common manifestation, followed by malaise, anorexia, sweating, and weight loss. Fever and splenomegaly were the common signs in most patients. In addition, arthritis was observed in 5 patients, and epistaxis, headache, and abdominal pain were observed in 3 patients. The common bone marrow aspiration findings consisted of increased megakaryocytes and hyperplasia of erythroid series, with a shift to the left of the granulocytic series. Histiocytic hyperplasia was observed in the bone marrow smear of 2 patients. Mild hemophagocytosis was observed in the bone marrow of 3 patients. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 6 weeks after antibiotic treatment of brucellosis. In conclusion, the authors would like to emphasize that brucellosis should be considered in the differential diagnosis of children with pancytopenia.     

Evaluation of obstructive sleep apnea symptoms in pregnant women with chronic disease

Objective: Obstructive sleep apnea syndrome (OSAS) is a disease which is estimated to be undiagnosed to a large extent. Hence, the prevalence of OSAS in pregnant women is unknown. We aimed to evaluate the symptoms of obstructive sleep apnea in pregnant women with chronic diseases.

Methods: In the study, 97 pregnant women with chronic diseases and 160 healthy pregnant women were included. A form questioning socio-demographic characteristics and pregnancy characteristics, Epworth scale and the Berlin questionnaire to evaluate the risk of OSAS were applied to participants.

Results: It has been determined that 10–12.5% of healthy pregnant women, 34–45.4% of pregnants with chronic diseases and 20.6–23.3% of all pregnant women had a high risk of OSAS, the pregnants with chronic disease compared to healthy pregnant women had statistically significant higher risk of OSAS. The risk of OSAS was found to be significantly higher especially in pregnant women with hypertension and diabetes.

Conclusions: OSAS can lead to the adverse consequences in pregnancy, should be questioned for all pregnants especially those with chronic diseases. Pregnant women with OSAS should be monitored more carefully in terms of diabetes and hypertension in antenatal care.     

Effects of lisinopril treatment on the pathophysiology of PCOS and plasminogen activator inhibitor-1 concentrations in rats

Research questionAngiotensin-converting enzyme inhibition results in a significant reduction in plasma concentrations of plasminogen activator inhibitor-1 (PAI-1). What are the effects of lisinopril treatment on PAI-1 concentrations and the morphology and function of the ovaries in the letrozole-induced polycystic ovary syndrome (PCOS) rat model?DesignThis prospective randomized controlled animal study involved female Wistar albino rats. Twelve rats were assigned as controls (group I). In the study group (n = 48), letrozole (an aromatase inhibitor) was administered for PCOS modelling for 9 weeks. After confirming disrupted oestrous cycles, the study group was randomized into two groups: group II (n = 24; letrozole only) and group III (n = 24; letrozole + lisinopril 15 mg/kg per day). After 12 weeks, each group was divided randomly into two. Biochemical, histopathological and immunohistochemical analyses was performed in subgroups designated A, and fertilization rates were studied in subgroups designated B.ResultsLisinopril treatment reduced the weight and area of the ovaries, the number and wall thickness of cystic follicles, and serum concentrations of LH and testosterone, relative to group II (P < 0.001). Circulating PAI-1 concentrations were significantly different among three groups (7.7 ± 0.9 ng/ml, 9.8 ± 0.7 ng/ml and 8.6 ± 0.7 ng/ml for groups IA, IIA and IIIA; P < 0.001). Pregnancy rates were 100%, 0% and 16.7% in groups IB, IIB and IIIB.ConclusionsIn the letrozole-induced rodent PCOS model, lisinopril modifies the action of letrozole, possibly by inhibition of systemic and ovarian production of PAI-1. The use of PAI-1 inhibitors deserves further investigation in understanding the pathogenesis of PCOS.     

Conventional versus epineural sleeve neurorrhaphy technique: functional and histomorphometric analysis

Three methods of nerve repair involving the epineural sleeve technique were compared with conventional nerve repair using the rat sciatic nerve transection model in four groups. In group 1, the sciatic nerve was repaired using the conventional epineural technique by placing four sutures. In groups 2, 3, and 4, the epineural sleeve technique was combined with two sutures, fibrin glue, and two sutures with fibrin glue, respectively. Functional recovery was evaluated using walking track analysis, limb circumference, and the severity of toe contracture. Diameter of the sciatic nerve fibers, total number of myelinated fibers, diameter of the myelin sheath, and the axon-to-fiber diameter ratio were measured at 12 weeks. The results showed better functional recovery as well as a higher number of myelinated fibers in groups using the epineural sleeve technique compared with conventional technique ( < 0.05). The addition of fibrin glue, however, did not make any significant difference. The epineural sleeve technique was found to be superior when compared with conventional nerve repair, providing faster functional recovery and improved nerve regeneration.