Multiple relapses in extramedullary localization of acute lymphoblastic leukemia.

Extramedullary relapses of acute lymphoblastic leukemia in children and young adults are rare and in most cases are usually related to the central nervous system or testes. We describe a case of a 25-year-old man with an acute lymphoblastic leukemia with multiple extramedullary relapses in the testes, gallbladder, breast, parotid gland and eye at various times (Fig. 5, Ref. 21).

A comparison of the effects of losartan and ramipril on blood pressure, renal volume and progression in polycystic kidney disease: A 5-Year follow-up.

BACKGROUND: The major cause of hereditary renal failure is autosomal dominant polycystic kidney disease (ADPKD). Many factors affect renal progression in these patients. Among these, hypertension and an increase in renal volume are interrelated in terms of their effects on renal progression. We aimed to investigate the effects of losartan and ramipril on renal volume and progression in patients with ADPKD. MATERIALS AND METHODS: Data from 18 hypertensive patients with ADPKD were evaluated. Eleven of the 18 hypertensive patients were on losartan and 7 on ramipril treatment. Demographic parameters, use of antihypertensives and other medications, the course of blood pressure (BP), biochemical parameters, creatinine clearance (CrCL), findings at computed tomography and renal volume were recorded at baseline and at 1 and 5 years. RESULTS: Target BP values were maintained over 5 years. The annual decrease in CrCL was 1.33 mL/min in the losartan group compared with 6.59 mL/min in the ramipril group. There was no significant difference between the groups in terms of annual decrease in CrCL. Annual increase in renal volume was 252.04 cm³ in the losartan group and 167.36 cm³ in the ramipril group. There was no significant difference between the groups in terms of the increase in renal volumes at 1 and 5 years. CONCLUSION: Our study demonstrated that losartan and ramipril provided effective BP control. In addition, the results of our study demonstrated that despite the increase in renal volume, losartan and ramipril may have regressed renal progression via other factors.

Giant mass due to Castleman's disease causing superior vena cava syndrome.

Castleman's disease is an uncommon disorder occurring mostly in patients presenting with localized mediastinal lymphadenopathy.It is usually asymptomatic. With localized disease, surgical excision is curative. Castleman's disease can very rarely present as superior vena cava syndrome. We describe a case of mediastinal Castleman's disease which presented as vena cava superior syndrome; it is the largest mediastinal mass from Castleman's disease reported in the literature.

Iodine status, thyroid function, thyroid volume and thyroid autoimmunity in patients with type 1 diabetes mellitus in an iodine-replete area.

OBJECTIVE: To analyze the prevalence and clinical significance of thyroid autoimmunity, thyroid volume and iodine status in patients with type 1 diabetes mellitus compared with age and sex matched healthy controls, in an iodine-deficiency improved area. METHOD: Fifty-eight patients with type 1 DM, 30 female and 28 male, who attended the pediatric endocrinology clinic of Karadeniz Technical University Hospital were included into the study. They were compared with 58 healthy children matched for sex and age. Routine thyroid function parameters, thyroid autoantibodies (TPOAb, TGAb and TRAb) and urinary iodine excretion were measured and thyroid volume was determined by ultrasonography (US). RESULTS: Twenty-six patients (44.8%) in diabetic patients and 20 subjects (34.5%) in the control group had thyroid autoantibody positivity. TPOAb and TGAb positivity were significantly high in diabetic patients (P=0.01 and P=0.032, respectively). Thyroid US revealed a thyroid volume of 6.6+/-3.5 ml (median 6.4 ml, range 1.117.2 ml) in the diabetic patients compared with 3.7+/-2 ml (median 3.1 ml, range 0.8-8.6 ml) in the control group (P=0.0001). Median urinary iodine levels of both groups were clearly above the threshold level for iodine deficiency, but 26 patients with type 1 DM (44.8%) and 16 controls (27.5%) had urinary iodine excretion below 100 microg/L, and 21 (36.2%) of diabetic patients and two subjects (3.4%) of the control group were consistent with severe iodine deficiency. No significant differences were noted in diabetic patients in terms of age, duration and metabolic control of the disease and thyroid volume when compared according to the autoantibody presence. Additionally, there were no significant differences between the iodine deficient and iodine sufficient diabetic patients in terms of age, sex, duration of disease, HbA1c, thyroid hormones and thyroid volumes. Thyroid autoimmunity was lower in patients with iodine deficiency (38.4% vs. 50%), but not statistically significant. CONCLUSION: We found that type 1 DM patients had larger thyroid volume compared with healthy control groups, and a large portion of them had the markers of autoimmune thyroid disease and iodine deficiency. Surprisingly, we found that a large portion of the healthy children had TRAb positivity. We proposed that TRAb must be considered in community surveys or prevalence studies of autoimmune thyroid disorders in iodine-replete areas. Additionally, prospective longitudinal studies are needed to determine the clinical significance of TRAb positivity in diabetic patients.

Computed tomography evaluation of cavitary necrosis in complicated childhood pneumonia.

The purpose of the present study was to retrospectively investigate the chest radiograph (CR) and CT findings of childhood pneumonia complicated by cavitary necrosis, and to evaluate the role of CT in decision-making for surgical intervention. Chest CT was performed in 51 patients presenting with persistent or progressive pneumonia, respiratory distress and sepsis despite 7-10 days of appropriate antibiotic treatment and closed tube drainage. Chest radiograph and CT findings were retrospectively evaluated in 23 patients (45%) with cavitary necrosis. Chest radiographs showed consolidation in 19 of 23 patients, cavitation in five patients, parapneumonic effusions in 17 patients and air-fluid levels in the pleural space in one patient. The CT scans demonstrated consolidation and cavitary necrosis in all patients. There were parapneumonic effusions in all patients with concomitant loculated collections in six patients. Twenty-two of 23 patients had pleural thickening. In seven patients there were air-fluid levels in the pleural space. In five of these patients, CT scans demonstrated bronchopleural fistulae. On the basis of the CT and clinical findings, 11 patients underwent surgical intervention. Computed tomography is superior to CR for demonstrating cavitary necrosis complicating pneumonia, and other parenchymal and pleural complications. It also has a crucial decision-making role for surgery.

Aneurysmal bone cyst of the sternum: a case report of successful treatment with radiotherapy.

An unusual case of a lytic, expanding lesion of the manubrium with histological diagnosis of aneurysmal bone cyst in a 13-year-old girl is presented. After a recurrence following primary surgery, the patient was treated successfully by external beam radiotherapy. A total dose of 25.2 Gy was delivered using conventional fractionation (1.8 Gy day(-1)) to the whole sternum. She remains recurrence- and symptom-free 46 months after the end of the treatment. This is the sixth patient with primary aneurysmal bone cyst in the sternal region, the first paediatric patient for this location, and the first case of its kind treated exclusively by radiotherapy ever reported in the literature. The histopathological, radiological and clinical findings of the patient are presented, relevant literature is reviewed, and radiotherapeutic management of such lesions is discussed.

Multi-slice computed tomography urography after diuretic injection in children with urinary tract dilatation.

PURPOSE: To evaluate the potential use of multi-slice computed tomography urography (MSCTU) after diuretic injection in children with urinary tract dilatation. MATERIAL AND METHODS: MSCTU was performed in 19 patients (11 boys, 8 girls, mean age 5.4 years) with suspicion of urinary tract obstruction and dilatation. Furosemide, 1 mg/kg, was injected 3 min before contrast material administration and followed by a bolus of 30 ml of physiologic saline solution immediately after application of contrast material. Excretory-phase images were obtained through the abdomen and pelvis beginning 10 min after initiation of the injection of contrast material. Maximum intensity projection (MIP) and volume rendering (VR) images were post-processed to obtain urographic views. RESULTS: MSCTU revealed pathology in 16 of 19 patients, while 3 patients had normal findings. Ureteropelvic obstruction was found in 4 patients, obstructive megaureter in 8. Both ureteropelvic obstruction and obstructive megaureter were disclosed in 1 patient, partial ureteral duplication in 1 patient, and both complete ureteral duplication and ureterocele in 2 patients. In all patients, MIP and VR images could satisfactorily show the pathologies of the urinary tract. The estimated effective average doses of MSCTU were higher than IVU. CONCLUSION: Preliminary results of furosemide-enhanced MSCTU demonstrated consistently dilated urinary tracts, obstruction levels, and underlying pathologies better than US and IVU.

Gallbladder dyskinesia: a cause of chronic abdominal pain in children.

PURPOSE: Gallbladder dyskinesia (GD) is a well-established disorder in adults, but it is not clearly defined in the paediatric population. Therefore, the aim of this study was to review our experience in a group of children with chronic abdominal pain associated with impaired gallbladder emptying in the absence of cholelithiasis. METHODS: The records of sixteen patients who underwent cholecystectomy with the diagnosis of GD were evaluated retrospectively. Clinical presentation, symptoms, diagnostic studies, and the effect of cholecystectomy in alleviating abdominal complaints were investigated. RESULTS: All patients had symptoms of upper abdominal pain in the absence of other attributable causes associated with low gallbladder ejection fractions (GEF) < 35 %, during cholecystokinin-stimulated hepatobiliary scan (CCK-HBS), and free of gallstones on ultrasound (USG). Abdominal pain and nausea were the most common presenting symptoms. Mean GEF was 15.3 %. All patients underwent cholecystectomy. The histopathological diagnoses of all operated patients were consistent with chronic cholecystitis. Symptoms were completely relieved in all except two patients. CONCLUSION: GD should be considered in the differential diagnosis of recurrent abdominal pain in children. Patients with this condition present with biliary-type pain and investigations show no evidence of gallstones in the gallbladder. Performing a CCK-HBS establishes the diagnosis. Patients with an abnormal GEF (< 35 %) should undergo cholecystectomy. This procedure has been shown to be effective in curing the symptoms in over 80 % of patients. To avoid late diagnosis, CCK-HBS should be employed early in the evaluation of biliary colic with negative sonographic findings.

Mesenteric involvement in neurofibromatosis type 1: CT and MRI findings in two cases.

We report computerized tomographic and magnetic resonance imaging findings of neurofibromatosis type 1 with mesenteric involvement in two patients. The first patient was a 13-year-old female with a panmesenteric plexiform neurofibroma with segmental involvement of the bowel wall; she had a 3-year history of abdominal pain. The second patient was a 60-year-old female who presented with malignant transformation of multiple mesenteric neurofibromas 4 months after primary operation.

Tracheobronchial rupture due to blunt trauma in children: report of two cases.

Tracheobronchial tree injuries occur in a small number of patients after blunt chest trauma, and their occurrence is uncommon in the pediatric trauma population. The authors report two male children, one with a tracheal rupture, and the other with disruption of the main right bronchus. Mediastinal and subcutaneous emphysema resulting in airway obstruction were noted in Case 1 and soft-tissue emphysema, pneumomediastinum and tension pneumothorax were evident in Case 2 at the time of presentation. In the child with bronchial disruption, a major airway injury was suspected early on, because of a massive air leak despite two properly placed chest tubes. The definitive diagnosis was established bronchoscopically, and thoracotomy and primary repair were performed. The child with rupture of the posterior tracheal wall was diagnosed at an early stage by bronchoscopy and he was successfully managed without surgery.

Renal replacement lipomatosis: ultrasonography and computed tomography findings.

Replacement lipomatosis of the kidney is the result of severe atrophy or destruction of the renal parenchyma often caused by calculous disease with secondary marked proliferation of renal sinus, renal hilus, and perirenal fatty tissue. The diagnosis is difficult to establish with conventional radiographic methods. Although ultrasonography may show highly suggestive findings, computed tomography seems to be the most accurate method for demonstrating the distinctive features of replacement lipomatosis. Ultrasonographic and computed tomographic features in three cases of replacement lipomatosis of the kidney are reported.

Does the position of the patient affect the presence and/or degree of reflux in an ileal conduit urinary diversion?

OBJECTIVE: To assess the effect of patient position (supine, sitting or standing) on ileo-ureteric reflux in patients with an ileal conduit urinary diversion, in whom such reflux is normally detected when they are supine during a retrograde loopogram. PATIENTS AND METHODS: The study included 10 patients with an ileal conduit as a primary urinary diversion; a loopogram was obtained with the patient upright or supine and a further film taken with the patient supine but at 45 degrees to the ground. RESULTS: When supine, free ileo-ureteric reflux occurred into both ureterorenal units in eight patients. The remaining two patients, who had previously undergone unilateral nephrectomy, also had reflux into their existing renal units. Of the 18 units, 15 had grade III and three had grade IV reflux. In the upright and 45 degrees position, reflux still occurred in al ureterorenal units. The patient's position did not affect the degree of reflux in 16 units, but in one unit with grade IV reflux and another with grade III reflux, the reflux was one grade less severe. CONCLUSIONS: Ileo-ureteric reflux is common after ileal conduit diversion and may contribute to the likelihood of renal deterioration. The presence and/or degree of reflux is generally not affected by the position of the patient.