Neuroimaging findings of four patients with Sandhoff disease.

Sandhoff disease is a severe form of GM2 gangliosidosis that is caused by the deficiency of both hexosaminidase A and B. Startle reaction, hypotonia, psychomotor retardation, and blindness are the main clinical features. Presented are computed tomography and magnetic resonance imaging findings of four patients with Sandhoff disease diagnosed by enzymatic analyses. Bilateral homogeneous thalamic hyperdensity was evident on computed tomography. Magnetic resonance imaging scans revealed mild cortical atrophy, a thin corpus callosum, and abnormal signal intensities in the caudate nucleus, globus pallidum, putamen, cerebellum, and brainstem. No correlation was evident between the severity of the central nervous system imaging findings and the clinical pictures. In this article the neuroimaging findings of four patients with Sandhoff disease are discussed.     

Neuroblastoma with spermatic cord metastasis in a child: sonographic findings.

We report a case of neuroblastoma in a patient who had no involvement of the spermatic cord at diagnosis but who developed spermatic cord metastasis 2 months later. The metastasis appeared on sonography as a hypoechoic, highly vascular, fusiform, hard, 14x10x7 mm mass located in the right inguinal canal and extending into the scrotum. The diagnosis of spermatic cord metastasis was confirmed by resection and histopathologic examination. We recommend that the scrotum and spermatic cord be evaluated by high-resolution sonography in children with neuroblastoma, both at the time of diagnosis and during follow-up.     

Butterfly vertebra: a case report.

Butterfly vertebra is a rare congenital anomaly associated with syndromes such as Pfeiffer, Jarcho-Levin, Crousen, Alagille. In the literature, only a few cases of butterfly vertebra have been reported as incidental finding. We described a 37-year-old male who had an L3 butterfly vertebra associated with an L4-L5 disc protrusion. Awareness of this anomaly is important for making correct diagnosis. Although this uncommon anomaly is considered to be usually asymptomatic, we suggest that it might increase the incidence of disc herniation, because the condition may alter the spinal biomechanics.     

Modified oral rehydration therapy in a case with cystic fibrosis

Infants with cystic fibrosis can develop episodes of hyponatremic, hypochloremic dehydration with metabolic alkalosis, and management is difficult. In this paper, we present a nine-month-old case with cystic fibrosis with pseudo-Bartter syndrome, who was rehydrated with two types of modified oral rehydration solution. Intravenous rehydration was impossible due to inaccessibility of route. With this oral rehydration treatment, her purging rate decreased, and serum sodium levels improved and remained stable. In cases with impossible intravenous route, one of these modified rehydration solutions could be given.     

The chondroprotective effects of intraarticular application of statin in osteoarthritis: An experimental study

Background:

Osteoarthritis (OA) is the most frequent chronic joint disease causing pain and disability. Recent reports have shown that statin may have the potential to inhibit osteoarthritis. This study of early stage OA developed in an experimental rabbit model, aimed to evaluate the chondroprotective effects of intraarticularly applied atorvastatin on cartilage tissue macroscopically and histopathologically by examining intracellular and extracellular changes by light and electron microscope.

Materials and Methods:

The experimental knee OA model was created by cutting the anterior cruciate ligament of the 20 mature New Zealand rabbits. The rabbits were randomly allocated into two groups of 10. Study group: The group that received intraarticular statin therapy; Control group: The group that did not receive any intraarticular statin therapy. The control group received an intraarticular administration of saline and the study group atorvastatin from the 1^st week postoperatively, once a week for 3 weeks. The knee joints were removed including the femoral and tibial joint surfaces for light and electron microscopic studies of articular cartilages.

Results:

The mean total points obtained from the evaluation of the lesions that developed in the medial femoral condyle were 11.33 ± 0.667 for the control group and 1.5 ± 0.687 for the study group. The mean total points obtained from the evaluation of the lesions that developed in medial tibial plateau cartilage tissue were 11.56 ± 0.709 for the control group and 1.40 ± 0.618 for the study group. Electron microscopic evaluation revealed healthy cartilage tissue with appropriate chondrocyte and matrix structure in study group and impaired cartilage tissue in control group.

Conclusion:

Chondroprotective effect of statin on cartilage tissue was determined in this experimental OA model evaluated macroscopically and by light and electron microscope. There are some evidences to believe that the chondroprotective effect of the statin is that, by protecting the structure of the endoplasmic reticulum and the Golgi complex.     

Treatment of unstable thoracolumbar junction burst fractures with short- or long-segment posterior fixation in magerl type a fractures

The treatment of thoracolumbar fractures remains controversial. A review of the literature showed that short-segment posterior fixation (SSPF) alone led to a high incidence of implant failure and correction loss. The aim of this retrospective study was to compare the outcomes of the SS- and long-segment posterior fixation (LSPF) in unstable thoracolumbar junction burst fractures (T12-L2) in Magerl Type A fractures. The patients were divided into two groups according to the number of instrumented levels. Group I included 32 patients treated by SSPF (four screws: one level above and below the fracture), and Group II included 31 patients treated by LSPF (eight screws: two levels above and below the fracture). Clinical outcomes and radiological parameters (sagittal index, SI; and canal compromise, CC) were compared according to demographic features, localizations, load-sharing classification (LSC) and Magerl subgroups, statistically. The fractures with more than 10 degrees correction loss at sagittal plane were analyzed in each group. The groups were similar with regard to age, gender, LSC, SI, and CC preoperatively. The mean follow-ups were similar for both groups, 36 and 33 months, respectively. In Group II, the correction values of SI, and CC were more significant than in Group I. More than 10 degrees correction loss occurred in six of the 32 fractures in Group I and in two of the 31 patients in Group II. SSPF was found inadequate in patients with high load sharing scores. Although radiological outcomes (SI and CC remodeling) were better in Group II for all fracture types and localizations, the clinical outcomes (according to Denis functional scores) were similar except Magerl type A33 fractures. We recommend that, especially in patients, who need more mobility, with LSC point 7 or less with Magerl Type A31 and A32 fractures (LSC point 6 or less in Magerl Type A3.3) without neurological deficit, SSPF achieves adequate fixation, without implant failure and correction loss. In Magerl Type A33 fractures with LSC point 7 or more (LSC points 8-9 in Magerl Type A31 and A32) without severe neurologic deficit, LSPF is more beneficial.     

Whey Feeding Suppresses the Measurement of Oxidative Stress in Experimental Burn Injury

PURPOSE: Burns cause thermal injury to local tissue and trigger systemic acute inflammatory processes, which may lead to multiple distant organ dysfunction. We investigated the protective effect of dietary whey supplementation on distant organs in a rat model. METHODS: Forty-eight rats were divided into six groups of eight: groups 1 and 2 were the controls, fed a standard diet and a whey-supplemented diet, respectively; groups 3 and 4 were fed a standard diet and subjected to burn injury; and groups 5 and 6 were fed a whey-supplemented diet and subjected to burn injury. We measured the oxidative stress variables, as well as glutathione in the liver and kidney, and histologically examined skin samples obtained 4 h (groups 3 and 5) and 72 h (groups 4 and 6) after burn injury. RESULTS: Glutathione (GSH) levels remained the same in the liver but were slightly elevated in the kidneys after burn injury in the rats fed a standard diet. Whey supplementation caused a significant increase in hepatic GSH levels 4 h after burn injury. Moreover, there was a significant rebound effect in the liver and kidney GSH levels after 72 h and whey supplementation potentiated this effect. Hepatic and renal lipid peroxide levels were also increased 4 h after burn injury in the rats fed a standard diet. Whey supplementation significantly suppressed the burn-induced increase in hepatic and renal lipid peroxide levels. Histological examination revealed that although whey supplementation resulted in decreased subepidermal inflammation, the indicators of wound healing and collagen deposition were not improved. CONCLUSION: Whey pretreatment suppressed hepatic and renal oxidative stress measurements after experimental burn injury.     

The "nutcracker phenomenon" with orthostatic proteinuria: case reports

The nutcracker phenomenon refers to compression of the left renal vein between the aorta and the superior mesenteric artery. Clinical features are hematuria, abdominal pain, left flank pain, pelvic or scrotal discomfort due to varicocele or ovarian vein syndrome. In this report, 2 patients with orthostatic proteinuria, in whom nutcracker phenomenon was detected as a cause, are presented. One of them had posterior nutcracker with also asymptomatic varicocele that was detected during ultrasonographic examination. Nutcracker phenomenon is a rare but important clinical condition that should be considered in the differential diagnosis of patients with proteinuria and hematuria.     

Intramedullary wire fixation for unstable forearm fractures in children

Displaced fractures of the diaphyseal forearm in children are often treated conservatively, but there is relatively high incidence of redisplacement, malunion and consequent limitation of function. This retrospective study was performed to determine means for minimalising the complications of intramedullary Kirschner (K)-wire fixation used in the treatment of unstable, diaphyseal forearm fractures by pointing out those which most frequently occur with this treatment choice. This treatment method was applied in 48 children with a mean age of 10.3 (range, 5-14) years. A limited open reduction to one or both bones was necessary for insertion of the intramedullary wire in 20 (40%) patients. Although 24 complications, such as pin site infection, loss of forearm rotation, superficial branch of radial nerve palsy, delayed union, nonunion, hardware migration, and K-wire penetration to the opposite cortex, were recorded in 18 patients, 46 patients (96%) had excellent or good, 1 patient (2%) had fair and 1 patient (2%) had poor outcome using the grading scheme adapted by Price. Except for the patient in whom the fracture was not united, the average union time was 6.3 weeks in children less than 10 years and 7.8 weeks in those above 10 years of age. Despite these minor complications, percutaneous intramedullary fixation with K-wires and proper technique is an appropriate, effective and safe operation for unstable diaphyseal fractures of the forearm in children who cannot be treated by closed manipulation.     

Which anesthetic agent alters the hemodynamic status during pediatric catheterization? Comparison of propofol versus ketamine

OBJECTIVE: To compare the effects of propofol and ketamine on systemic and pulmonary circulations in pediatric patients scheduled for elective cardiac catheterization. DESIGN: Prospective, randomized, and blinded. SETTING: University hospital. PARTICIPANTS: Children (n = 41) undergoing cardiac catheterization. INTERVENTIONS: All children were premedicated with oral midazolam 60 minutes before the procedure. Patients were separated into 3 groups according to shunts diagnosed by transthoracic echocardiography before the catheterization procedure: patients without cardiac shunt (Group I, n = 11), left-to-right shunt (Group II, n = 12), and right-to-left shunt (Group III, n = 18). A continuous infusion of propofol (100-200 microg/kg/min) or ketamine (50-75 microg/kg/min) was randomly started in all groups to obtain immobility during the procedure. Hemodynamic data, including systemic venous, pulmonary artery and vein, aortic saturations and pressures, were recorded; Qp/Qs were calculated. The same set of data was recorded before discontinuation of infusions at the end of the procedure. MEASUREMENTS AND MAIN RESULTS: After the propofol administration, in all 3 patient groups propofol infusion was associated with significant decreases in systemic mean arterial pressure. In groups with cardiac shunts (Group II and III), propofol infusion significantly decreased systemic vascular resistance and increased systemic blood flow, whereas pulmonary vascular resistance and pulmonary blood flow did not change significantly. These changes resulted in decreased left-to-right shunting and increased right-to-left shunting; the pulmonary-to-systemic flow ratio decreased significantly. On the other hand, after ketamine infusion, systemic mean arterial pressure increased significantly in all patient groups, but pulmonary mean arterial pressure, systemic vascular resistance, and pulmonary vascular resistance were unchanged. CONCLUSION: In children with cardiac shunting, the principal hemodynamic effect of propofol is a decrease in systemic vascular resistance. In children with intracardiac shunting, this results in an increase in right-to-left shunting and a decrease in the ratio of pulmonary to systemic blood flow, which may lead to arterial desaturation. Ketamine did not produce these changes. The authors suggested that during cardiac catheterization in children, both the anesthesiologists and cardiologists need to know that anesthetic agents can significantly alter the hemodynamic status in children with complex congenital heart defects and affect the results of hemodynamic calculations that are important for decision-making and treatment of these patients.