Efficacy of a new software in eliminating the angulation errors in digital subtraction radiography.

OBJECTIVES: To evaluate the efficacy of a new software developed for digital subtraction radiography at tolerating the vertical and horizontal angulation changes between the X-ray source and object. METHODS: The right first molar of a preserved dry mandible was imaged with 0 degrees vertical and 0 degrees horizontal tube angulation. Then, the vertical angle was changed to +10 degrees, -10 degrees, +15 degrees and -15 degrees, and the same tooth was radiographed in each condition, accordingly. The same procedure was performed with 5 degrees horizontal angular modifications in both mesial and distal directions. All radiographs were automatically developed and were digitized with 600 dots per inch (dpi) spatial resolution. New software and Emago were used to subtract the images with angulation alterations from the original image, and a total of 20 subtracted images were obtained. Ten experienced specialists evaluated the images and rated the efficacy of the programs. Data were analysed statistically and alpha was accepted as 0.05. RESULTS: The total score of the new software was 190 and that of Emago was 117 for vertical angular alterations, whereas the scores were 51 and 21, respectively, for horizontal angular alterations. The differences between the overall scores of the softwares were statistically significant (P<0.05). Overall evaluation revealed that the new software was rated with higher scores for subtracted images obtained with both vertical and horizontal angular alterations. CONCLUSIONS: Both programs managed to tolerate vertical angulation alterations up to 10 degrees and horizontal angulation changes up to 5 degrees ; however, the new program performed better than Emago.

Clinical efficacy of a new software developed for dental digital subtraction radiography.

OBJECTIVES: The aim of this study was to test and compare the efficacy of software developed recently for digital subtraction radiography (DSR) in vivo. METHODS: An algorithm performing both manual and automated image reconstructions and contrast correction was developed for the manipulation of radiographic images. Pre- and post-operative radiographic images of ten patients were obtained and the automated subtraction analyses were performed using four different softwares (new software, Emago, Photoshop 8.0 and Paintshop Pro 9). Ten experienced dental specialists evaluated the clinical efficacy of each program and scored the softwares by using visual analogue scales (VAS). The results were statistically analysed and alpha was set as 0.05. RESULTS: The newly developed algorithm received higher scores than the others (new software =67.89, Emago = 64.26, Paintshop Pro 9 = 33.41 and Photoshop 8.0 = 27.24, respectively). The clinical efficacies of the new software and Emago were not significantly different (P = 0.720); likewise, Photoshop 8.0 and Paintshop Pro 9 performed comparably (P = 0.295). CONCLUSIONS: Considering this study, the new software and Emago would be suggested for DSR in dental practice.

Primary hemophagocytic lymphohistiocytosis in Turkish children.

Nineteen children with hemophagocytic lymphohistiocytosis (HLH) were studied in the Department of Pediatric Hematology, Hacettepe University. Patients were divided into two groups. Group 1: Thirteen patients were classified as having a genetic etiology (7 familial, 6 presumed familial) on the basis of an affected sibling and consanguinity. There was a history of consanguineous marriage in 13 of the families. Seven of them had a history of a sibling with HLH. Group 2: Six patients were diagnosed with sporadic HLH. The age at presentation for familial patients was 0.7-84 months (mean 21.9 +/- 24.9 months), and for sporadic cases it was 2.5-48 months (mean 22.7 +/- 19.8 months). The clinical and laboratory data of these two groups were similar at diagnosis. Thirteen cases were diagnosed premortem by bone marrow aspiration. Splenic biopsy was performed in 2 patients. Four patients were diagnosed by postmortem examination. Elevated LDH levels were found in all patients tested. No significant differences for clinical and laboratory data were found between the two groups.

A comparative histopathologic and immunohistopathologic evaluation of cremaster muscles from boys with various inguinoscrotal pathologies.

AIM: It has been hypothesized that the cremaster muscle (CM) might play a part in the pathogenesis of various inguinoscrotal pathologies such as undescended testis and hernia. A prospective study was carried out to determine if CM of boys with hydrocele, inguinal hernia, and undescended testis reveal any histopathological and immunohistopathological alterations. METHODS: Samples of CM from 29 patients presenting with inguinal hernia (15), undescended testis (9), and hydrocele (5), and CM from autopsies of boys without inguinal pathology (2), and samples of internal oblique muscles from patients undergoing laparotomy (3) were obtained. The biopsies were frozen in isopentane-cooled liquid nitrogen, cut in 6 micron sections and stored at -80 degrees C until processed. Sections were stained by hematoxylin-eosin, modified Gomori-trichrome, PAS, Oil Red-O, NADH, SDH and COX. All specimens were evaluated for seven parameters including variation in fibre size, presence of central nucleus, endomysial fibrosis, inflammation, presence of basophilic fibres, increase in perimysial connective tissue, and variation in fibre size between fascicules. The specimens were also evaluated for beta-spectrin, laminin alpha-1 chain, laminin alpha-2 chain, 43 kd distroglycan, and fetal myosin by immunofluorescence. The presence of each parameter was compared, individually and in combination, according to the ages and underlying pathologies. RESULTS: None of the internal oblique muscles were positive for any of the seven parameters. Only one of the two CM obtained during autopsy revealed a slight variation in fibre size. However, fibre size variation and increase in perimysial connective tissue were found in all but one CM from a patient with hernia. The presence or absence of parameters did not differ according to age. Comparison of the groups with inguinal hernia and undescended testis for each of the individual parameters did not reveal significant differences. However, the presence of four or more parameters in each CM was more commonly encountered among patients with undescended testis compared to patients with hernia (p < 0.05). The CM of patients with hydrocele suggested similar findings to patients with inguinal hernia. All of the specimens, regardless of origin, revealed normal sarcolemmal and basal laminal stainings, and fetal myosin was expressed in only two specimens which were obtained from an internal oblique muscle and the CM of a patient with an inguinal hernia. CONCLUSION: The CM of patients with inguinal hernia, hydrocele, and undescended testis reveal histopathological alterations. Furthermore, CM from patients with undescended testis reveal more profound alterations. Whether these alterations reflect a primary muscle pathology or a defect in innervation or changes secondary to inguinoscrotal pathology requires further study.

Lethal hydrops fetalis due to congenital dyserythropoietic anemia in a newborn: association of a new skeletal abnormality.

Congenital dyserythropoietic anemias (CDAs) are a group of hereditary refractory anemias characterized by ineffective erythropoiesis, typical morphological abnormalities of erythroblasts, a low or no reticulocyte response, hyperbilirubinemia, and splenomegaly. A massive hydropic newborn born with a very severe anemia (Hb 4.8 g/dL), diffuse edema, hepatosplenomegaly, ascites, pulmonary edema and respiratory distress, and shortness and hallux varus deformity of the great toe of the right foot was diagnosed to have congenital dyserythropoietic anemia on the basis of the hematological (macrocytosis, anisopoikilocytosis, fragmented red cells and erythroblastosis in the peripheral blood, and erythroid hyperplasia with erythroblastosis and erythroblasts with double nuclei and thin chromatin bridges connecting these nuclei in the bone marrow) and serological (negative acidified serum lysis test and no agglutination with anti-i antibodies) findings. In this article the seventh case of neonatal congenital dyserythropoietic anemia presenting with a very severe (lethal) form of hydrops fetalis and a new (hallux varus) deformity of the great toe of the right foot is presented. Congenital dyserythropoietic anemia should be considered in the differential diagnosis of hydrops fetalis presenting with a very severe anemia and a skeletal abnormality of the great toe.

Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts unlinked to the LAMA2, FCMD and MEB loci.

We report a case of congenital muscular dystrophy with secondary merosin deficiency, structural involvement of the central nervous system and mental retardation in an 8-year-old girl from a consanguineous family. She had early-onset hypotonia, generalized muscle wasting, with weakness especially of the neck muscles, joint contractures, mental retardation and high creatine kinase. Muscle biopsy showed dystrophic changes with partial deficiency of the laminin alpha(2) chain. Cranial magnetic resonance imaging revealed multiple small cysts in the cerebellum, without cerebral cortical dysplasia or white matter changes. The laminin alpha(2) chain (6q2), Fukuyama type congenital muscular dystrophy (9q31-q33) and muscle-eye-brain disease (1p32-p34) loci were all excluded by linkage analysis. We suggest that this case represents a new entity in the nosology of congenital muscular dystrophy.

Paraneoplastic cerebellar degeneration and Horner syndrome: association of two uncommon findings in a child with Hodgkin disease.

An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. Hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and Horner syndrome.

Clinical and histopathological study of merosin-deficient and merosin-positive congenital muscular dystrophy.

The clinical features of merosin-positive congenital muscular dystrophy (CMD) and merosin-deficient CMD are well known, with those of merosin-deficient CMD being more severe. Whether the severity of histopathological findings correlates with these clinical features remains unanswered. In this study, the clinical and histopathological findings of 39 merosin-deficient and 37 merosin-positive CMD patients were compared. Merosin-deficient CMD patients were found to be younger, with earlier onset of symptoms, age of diagnosis, and a more severe clinical state (reflecting maximum motor capacity and contractures). On histopathological evaluation, endomysial fibrosis, perimysial fibrosis, and histopathological state (reflecting fibrosis, adiposis, necrosis, and variation in fiber size) were more severe in merosin-deficient CMD. There was a correlation between clinical and histopathological states only in merosin-deficient CMD.

DNA content and proliferative activity in children with neuroblastoma.

Many studies have reported that neuroblastoma patients with aneuploid DNA content and a low cellular proliferative activity have better outcome; other studies have reported contradictory results. Formalin-fixed, paraffin-embedded archival pretreatment specimens of 56 neuroblastomas were studied. Thick sections from paraffin blocks were deparaffinized, and rehydrated. Following enzymatic digestion and filtration, cellular suspensions were analyzed by flow cytometry. Six tumors were aneuploid (13.3%) and 39 samples were diploid (86.7%). S-phase fraction (SPF) ranged from 1 to 78% with a median of 31%. DNA ploidy and proliferative activity results showed no correlation with the prognostic variables. There was no significant difference between the 5-year overall and event-free survival rates of the aneuploid and the diploid neuroblastomas or between the neuroblastomas with a high and low proliferative activity. The results revealed the prognostic significance of neither DNA ploidy nor the cellular proliferative activity in neuroblastoma in contrast to other studies.

Granulomatous osteomyelitis of the sternum presenting with a parasternal mass: a possible relation to the bacillus Calmette-Guérin vaccine.

Because they are frequently malignant, swellings of the chest wall during infancy are alarming lesions. Sternal osteomyelitis is a rare condition that may present with a chest wall mass locating in the parasternal region. A parasternal mass of granulomatous osteomyelitic origin is an extremely rare cause with only few case being reported to date. Herein, the authors report on 2 infants presenting with parasternal masses of sternal granulomatous osteomyelitic origin and discuss the possible etiologic role of the bacillus Calmette-Guérin vaccine.

Cholesteryl ester storage disease: case report during childhood.

Cholesteryl ester storage disease (CESD) is rare and characterized by accumulation of cholesteryl esters and triglycerides in many tissues due to the deficiency of lysosomal acid lipase. We report a 3(1/2)-year-old child with CESD. The diagnosis was indicated by liver biopsy and confirmed by reduced acid lipase activity in leukocytes.

Myelodysplastic syndrome associated with Griscelli syndrome.

Myelodysplastic syndrome (MDS) in children has been reported to be associated with various constitutional anomalies; however, it has not been described previously in patients with Griscelli syndrome (GS). In this report, we present a case with GS and refractory anemia with an excess of blasts. We believe that careful evaluation of peripheral blood and bone marrow could increase the number of patients who are found to have GS-associated MDS.

Enteritis cystica profunda mimicking acute abdominal picture in a twelve-year-old boy.

Enteritis cystica profunda, an uncommon condition, is characterized by nonneoplastic cystic spaces within the wall of the small bowel. The third case of enteritis cystica profunda in children is presented with special emphasis on diagnosis and treatment. This rare entity may mimic acute abdomen and should be kept in mind in the differential diagnosis. Surgical excision of the involved segment is the appropriate treatment.

Chest wall hamartoma: an alarming chest lesion with a benign course.

Chest wall hamartoma is a very rare mass lesion of the chest wall that is manifest at birth or in early infancy with deformity of the thoracic wall and/or varying grades of respiratory distress. The authors report on an 11-month-old infant presenting with chest wall hamartoma who was treated by en bloc excision of the lesion including the involved ribs. They emphasize the importance of early and complete excision of the lesion even in minimally symptomatic patients to avoid lethal respiratory complications, more severe postoperative orthopedic problems, and malignant transformation.

Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years.

Two hundred and fifty-five previously untreated patients (pts) with rhabdomyosarcoma (RMS) (age range 15 days to 17 years, median 5 years) were evaluated and treated in our institution. Head and neck primaries were seen in 125 patients (49%), abdominopelvic in 73 (29%), trunk and lung in 20 (5%) and extremity lesions in 37 (15%). The histology was: embryonal 137; alveolar 42; botryoid 18; pleomorphic 14. Forty-four patients could not be subclassified. The stage of the patients were as follows: 15 in state I, 74 in stage II, 139 in stage III and 27 in stage IV, according to the IRS grouping system. Patients were treated with a combination of surgery and radiation to doses of 35-55 Gy according to the patient's age and stage. All the patients received chemotherapy according to VAC or pulse-VAC (before 1988) and modified AVAC (after 1988) protocol. Survival curves were calculated by the Kaplan-Meier method. The statistical significance of each variable was tested by the log-rank test. Overall survival was 42 percent at 10 years. Three important predictors for survival time were clinical group (p < 0.001), age (p < 0.001) and primary site (p = 0.005). The best results involved clinical group I-II, age one to five years and orbital and genitourinary primary sites. An important predictor of survival time was also detected between those treated during the first ten years (1972-82) and last 10 years (1982-92), p < 0.005. Of the 96 deaths, 37 were from progressive disease, 24 from infection, 4 during postoperative period (first 7 days), 18 from unknown causes and 13 from other causes.

Enteritis cystica profunda causing ileoileal intussusception in a child.

Enteritis cystica profunda (ECP) is an exceedingly rare lesion of the small intestine. It has identical histopathologic features as colitis cystica profunda, which is mainly a disease of adults and a rare benign lesion of the distal colon and rectum of uncertain cause. Only 12 cases of ECP with only one pediatric case, have been reported in English-language medical literature. Here the authors present the second pediatric case of ECP with the literature review and discussion of the etiology.

Portal vein thrombosis in association with factor V Leiden mutation in a patient with hepatocellular carcinoma.

We report a case of hepatocellular carcinoma associated with portal vein thrombosis. Analysis of whole cellular DNA demonstrated heterozygosity for the factor V Leiden mutation. The patient also had marked protein C deficiency. The presence of the mutation associated with protein C deficiency may increase the risk of thrombosis in this patient with hepatocellular carcinoma.