The kidney in children with tyrosinemia: sonographic, CT and biochemical findings

BACKGROUND: Tyrosinemia relates to a deficiency of fumarylacetoacetate hydrolase and presents early in life with central nervous system and liver abnormalities. Renal function is often impaired. Little is known about the architecture and function of the kidneys. OBJECTIVE: Imaging changes on US and CT are compared to the function of the kidneys in children with tyrosinemia, and followed after liver transplantation. MATERIALS AND METHODS: Renal sonography, CT and renal function tests in 32 children were reviewed. Renal length, volume, echogenicity and nephrocalcinosis were evaluated. Renal function was assessed by glomerular filtration rate, and the presence of aminoaciduria, acidosis and calciuria. Seventeen children had open renal biopsy during time of liver transplantation. Histology was reviewed. Statistical analyses relating renal structure to function were performed, and repeated after transplantation. RESULTS: The kidneys were enlarged (47 %), hyperechogenic (47 %) and showed nephrocalcinosis (16 %). There was delayed excretion of contrast medium at CT in 64 %. Aminoaciduria was present in 82 % of children, hypercalciuria in 67 %, tubular acidosis in 59 %, and low GFR in 48 %. Delayed excretion of contrast was associated with low GFR (P < 0.05). Renal biopsies showed dilated tubules (81 %), interstitial fibrosis (56 %), glomerulosclerosis (56 %) and tubular atrophy (56 %). During a mean observation period of 3 years following liver transplantation, GFR improved in 50 %, tubular acidosis in 50 % and hypercalciuria in 70 %. No change was noted in renal size or sonographic architecture. CONCLUSION: Renal architecture and function are abnormal in the majority of children with tyrosinemia. Liver transplantation improves renal function in about 50 % of patients, but abnormal renal size and architecture persist.     

Thyroid metastasis from rectal carcinoma coexisting with Hashimoto''s thyroiditis: gray-scale and power Doppler sonographic findings

BACKGROUND: Interferon (IFN) alpha and IFN gamma have synergistic in vitro and in vivo effects and have each been used in the treatment of anogenital condylomata acuminata. STUDY GOALS: To compare the efficacy and safety of regional 3 x 10(6) U IFN alpha (Group A), 3 x 10(6) U IFN gamma (Group B), 1.5 x 10(6) U IFN alpha plus 1.5 x 10(6) U IFN gamma (Group C), and 3 x 10(6) U IFN alpha plus 1.5 x 10(6) U IFN gamma (Group D) in the treatment of recalcitrant anogenital condylomata acuminata. STUDY DESIGN: Six-week courses of regional IFNs were administered in a randomized, double-blind, multicenter study. Response was assessed as change in the total area affected by condylomata. RESULTS: The treatments of Groups A, B, and C were similarly effective (complete response in 13.6%, 18.5%, and 16.0%, respectively). Group D had the lowest rate of complete response (3.8%), but this combination was the most effective when partial and complete responses were combined (73.0%). CONCLUSION: Regional IFN is a moderately effective and safe treatment in patients with recalcitrant anogenital condylomata acuminata. Combinations of IFNs alpha and gamma were not superior to IFN monotherapy.     

Malignant splenic lymphoma: sonographic patterns, diagnosis and follow-up

In 1972, Fried described a large Scottish family affected by X linked mental retardation (XLMR), hydrocephalus, and mild facial dysmorphism. The phenotype has considerable similarity to the MASA syndrome, which results from mutations of the L1CAM gene in Xq28, and this family has since been assumed to be an example of this condition. We have reinvestigated the family for linkage to X chromosome markers, and obtained additional clinical information on surviving affected subjects. The phenotype in these patients has evolved into a distinctive syndrome, with severe mental retardation (MR), spastic diplegia, ventricular dilatation, and calcification of the basal ganglia. Linkage to Xq28 markers has been excluded, suggesting that Fried syndrome is not allelic with MASA syndrome. Two point and multipoint linkage analysis indicates that the gene for this condition lies within the interval KAL-DXS989 in Xp22. We propose the designation Fried syndrome to emphasise the disorder's distinctive phenotype.     

Jejunogastric invagination: plain radiologic, sonographic and computed tomographic findings

Jejunogastric intussusception is a rare complication after gastric surgery and when its diagnosis is delayed a high mortality rate can be expected. This paper reports on the characteristic findings of this entity at ultrasound and CT examination. Both diagnostic procedures have a high sensitivity. Sonography is the method of first choice because the diagnosis can be made with a high grade of certainty. CT allows the differentiation of the distinct stages of the disease and the views given by CT are often more easily accepted by the surgeons.     

Acute stroke: usefulness of early CT findings before thrombolytic therapy

PURPOSE: Three-dimensional conformal radiotherapy (3D-CRT) has been associated with a reduction in acute and late toxicity among patients treated for localized prostatic cancer. The purpose of this study is to assess the acute and late toxicity of 3D-CRT delivered to patients in the postprostatectomy setting and to analyze which factors predict for durable biochemical control in this group of patients. METHODS AND MATERIALS: Between 1988 and 1994, 42 patients were treated after prostatectomy with three-dimensional conformal radiotherapy. The median time from prostatectomy to radiotherapy was 11 months. Indications for treatment included a rising serum PSA level in 28 patients (65%) and positive surgical margins without a rising PSA level in 14 (35%). Twenty-five patients (60%) had pathologic stage T3 disease, and 32 (74%) had tumor at or close to the surgical margins. The median dose was 64.8 Gy, and the median follow-up time was 2 years. RESULTS: 3D-CRT in the postprostatectomy setting was well tolerated. Three patients (7%) experienced Grade II acute genitourinary toxicity and nine patients (21%) experienced Grade II acute gastrointestinal toxicity during treatment. No patient experienced Grade III or higher acute morbidity. The 2-year actuarial risk for Grade II late genitourinary and gastrointestinal late complications were 5 and 9%, respectively. In patients with existing incontinence, the incidence of worsening stress incontinence 6 months after treatment was 17%, which resolved within 12 months to its preradiotherapy level in four of six cases (66%). The overall 2-year postirradiation PSA relapse-free survival rate was 53%. The 2-year PSA relapse-free survival was 66% for patients with undetectable PSA levels in the immediate postoperative period compared to 26% for those with detectable levels of PSA after surgery (p < 0.006). Furthermore, for patients with preradiotherapy PSA levels of < or = 1.0 ng/ml, the 2-year PSA relapse-free survival was 74% compared to 17% of those with preradiotherapy PSA levels of > 1.0 ng/ml (p < 0.002). The resection margin status, presence of seminal vesicle involvement, Gleason score, and the preprostatectomy PSA level did not impact on PSA relapse-free survival. A Cox proportional hazards regression analysis demonstrated that a preradiotherapy PSA value of > 1 ng/ml (p < 0.002) was the most important covariate predicting for a rising PSA after radiotherapy. CONCLUSIONS: After prostatectomy, three-dimensional conformal radiotherapy is associated with minimal treatment-related morbidity. Patients with postprostatectomy, preradiotherapy PSA levels < or = 1.0 ng/ml, and those patients who had undetectable PSA levels in the immediate postoperative period are more likely to benefit from local adjuvant therapy.     

Intracranial abnormalities in infants treated with extracorporeal membrane oxygenation: update on sonographic and CT findings

The phylogenetic relationship of the asexual mycorrhizal fungus Cenococcum geophilam Fr. among sexual ascomycetes was examined by phylogenetic analysis of nucleotide sequence data from the nuclear small subunit (18S) ribosomal RNA genie region. A specific focus of this study was to test the hypothesis that the genus Elaphomyces is the closest sexual relative of C. geophilum. Thus nucleotide sequence data of five C. geophilum isolates, three Elaphomyces species, and 44 additional genera of ascomycetes were included in the phylogenetic analyses. The percentage of similarity among the 18S rDNA sequences of the C. geophilum isolates examined was 99.8 to 100%, indicating that C. geophilum is monophyletic. Percent similarity of nucleotide sequence among the three Elaphomyces species was also high and ranged from 99.4 to 99.5%. DNA parsimony and distance analysis of the sequence data separated these 2 genera on distant clades when sequence from 44 additional genera of ascomycetes was included. Parsimony and distance analyses positioned C. geophilum as a basal, intermediate lineage between the two Loculoascomycete orders, the Pleosporales and the Dothidiales, and strongly supported Elaphomyces to be of Plectomycete origin. Among the sexual Ascomycetes examined, which included representative taxa from four classes of filamentous Ascomycetes (Plectomycetes, Pyrenomycetes, Discomycetes, and Loculoascomycetes), no close sexual relative to C. geophilum was identified. At least four independent lineages of mycorrhizal fungi were identified among the ascomycetes examined.     

Endometriosis: clinical, histological and morphometric findings before and after Gn-RH agonist therapy

After bioptical diagnosis of endometriosis, 81 patients were treated with GnRH-agonists buserelin or leuprolide for six months. Biopsies before and after treatment were used to test a semiquantitative score-system, regarding atrophy of glands and stroma cells. Furthermore glandular diameter, circumference and area of nuclei were examined morphometrically using a microscopic semiautomatical measuring system. Morphometrical and histological alterations during therapy were evaluated. Additionally, data suitable for predicting a possible therapeutic success were described. After therapy 40 patients still showed endometriotic implants (partial responder) in contrast to 41 cases without foci (total responder). Therapeutic effect of GnRH-agonists was proved in every respect: clinical complaints decreased markedly during GnRH-agonists therapy. Both buserelin and leuprolide treated groups revealed increase of atrophy and reduction of extension of stroma. Correspondingly morphometrical analysed parameters such as diameter, circumference and area of glands decreased during therapy as well as area of cytoplasm and nuclei. Except the diameter of glands, the leuprolide treated partial responder group (residual foci after GnRH-therapy) revealed a stronger therapeutic effect than the buserelin treated partial responder group. Obviously this effect seems to be produced by the stronger estradiol suppression of leuprolide. Pretherapeutic comparison of measured values pointed out a minor distinct endometriosis in the total responder group. Success or failure of therapy seems to depend more on the pretherapeutic degree of expression of endometriosis. Obviously the kind of applicated GnRH-agonist plays a minor distinct role. Morphometrical data of endometriotic foci appear to be appropriate to predict a possible therapeutic success of GnRH-agonist therapy. But because of many exceptions only a roughly estimated prediction is possible.     

Dilation of the ventriculus terminalis: sonographic findings

A newborn term male infant was found to have the stigmata of VACTERL syndrome, including cervical and thoracic hemivertebra, partial sacral agenesis, 13 pairs of ribs, high imperforate anus with rectovesical fistula, and tracheoesophageal fistula with proximal esophageal atresia. No open spinal dysraphism or skin dimpling was seen. Ultrasonography of the spinal cord was performed to screen for occult anomalies. The examination revealed a lipoma of the filum terminalis and a low-lying conus medullaris at the L3-L4 level with a large central cystic structure in the terminal cord, which was continuous with the central canal (Fig. 1). This was thought to most likely represent a dilated ventriculus terminalis, and the diagnosis was confirmed on a subsequent MR image (Fig. 2). Fluid in the cyst followed cerebrospinal fluid signal in all sequences. We observed no abnormal signal in the adjacent parenchyma or enhancement associated with the cystic mass. A cranial ultrasonogram did not reveal any intracranial anomalies. The patient demonstrated no neurologic deficits in the lower extremities. Bowel and urinary continence was not assessable in this infant.     

Ovarian torsion in prepubertal and pubertal girls: sonographic findings

OBJECTIVE: The purpose of this study is to report the range of gray-scale and color Doppler findings of ovarian torsion in prepubertal and pubertal girls to determine whether there is a difference in appearance between the two age groups. MATERIALS AND METHODS: The study population consisted of 20 patients, 11 who were prepubertal (ages 2 days to 6 years) and nine who were pubertal (ages 12-16 years), who had gray-scale sonograms and surgical confirmation of ovarian torsion. Color Doppler studies were obtained in 14 of 20 patients. All sonograms were reviewed retrospectively by two radiologists with attention to location of the twisted ovary, internal characteristics, and evidence of color Doppler flow. The results were then compared in the prepubertal and pubertal groups. RESULTS: Sonographic findings of torsion in 11 prepubertal patients included complex mass with septations and debris (6/11), cystic mass (1/11), and solid mass with peripheral cysts (4/11). The masses were located in the right lower quadrant (5/11), left lower quadrant (3/11), right adnexa (2/11), and right inguinal canal (1/11). Eight of nine pubertal patients had solid masses, and one had a thick-walled cystic mass. Torsion involved the right ovary in nine patients and the left in 11. Color patterns included central flow (3/14), peripheral flow (6/14), and absence of flow (5/14) and did not correlate with age or gray-scale findings. CONCLUSION: Sonographic findings of ovarian torsion vary with age. Neonates and young children have extrapelvic cystic or complex cystic masses, whereas pubertal girls have predominantly solid masses in an adnexal location. In both groups of patients, color Doppler signal can be documented in many twisted ovaries.     

Central nervous system involvement of leukemia and systemic lymphoma in children: CT and MR findings

The purpose of this paper is to retrospectively evaluate CT and MR findings of central nervous system (CNS) involvement of leukemia and systemic lymphoma in children. Over a 12-year period, sixty-five patients with leukemia and fifteen patients with systemic lymphoma underwent cerebral CT and/or MR imaging. Nine patients (11.3%) were diagnosed as CNS involvement of leukemia and lymphoma. The diagnostic criteria of CNS involvement were as follows; 1) Histological proof was confirmed by surgery, 2) Tumor cells were found in the cerebrospinal fluid examinations, 3) Increase in size of the lesion during observation without specific treatment, and 4) Response to the treatment for leukemia or lymphoma. All of nine patients fulfilled more than two criteria of 1)-4). The CT and MR abnormalities in these patients were correlated with the findings of histology, cerebrospinal fluid cytology, and/or treatment. The age of the patients ranged from 0 to 15 years old. They consisted of 6 boys and 3 girls. The CT examinations were performed before and after contrast administration. MR examinations were performed on a 1.5-T unit, and T1-weighted, T2-weighted, and proton density-weighted images were obtained using spin-echo or fast spin-echo sequences. Tumor masses were present in seven with leukemia (acute lymphoblastic leukemia 4; acute myeloblastic leukemia 1; acute promyelocytic leukemia 1; acute monocytic leukemia 1), and in two with malignant lymphoma. On the CT scan, tumor masses were hyperdense with contrast enhancement. On the MR images, their signals were variable. In all of nine patients, tumor masses were contiguous with a meningeal surface. Postcontrast T1 weighted images were valuable in demonstrating meningeal infiltration. Tumoral hemorrhage was found in two patients. In a patient with tumor at the superior sagittal sinus, venous infarct was observed. CNS leukemic and lymphomatous masses are almost hyperdense on the CT and they are characteristically contiguous with a meningeal surface. MR imaging was valuable in demonstrating meningeal infiltration. Findings of CT and MR imaging, cerebrospinal fluid examinations, and response to the treatment are useful in the differentiation of CNS involvement of leukemia and lymphoma from other lesions such as infectious diseases and leukoencephalopathy.     

Krukenberg tumor findings at color and power Doppler US; correlation with findings at CT, MR imaging, and pathology. Case reports

OBJECTIVE: To describe our experiment with the treatment of GnRH-a in premenarchal girls with idiopathic central precocious puberty (CPP). PATIENTS AND METHODS: Twenty-eight girls, aged 6.5-11 years, with idiopathic central precocious puberty were treated every 28 days with an intramuscular depot gonadotropin releasing hormone agonist (GnRH-a) in an attempt to delay sexual maturation. RESULTS: Eight of the 28 (28.5%) developed vaginal bleeding after GnRH-a administration. Of these, prolonged vaginal bleeding of 11-13 days occurred in four girls, three recurrent episodes occurred in one during the second injection, and in one other girl the 4th episode occurred after 6 months of treatment. CONCLUSION: Uterine bleeding following GnRH-a treatment in premenarchal girls with CPP is common, and may be massive and recurrent, since most episodes resolved spontaneously and necessitated no further treatment, careful advice should be given to the girls and their families prior to treatment initiation, in an attempt to avoid unnecessary anxiety and achieve better compliance.     

Pulmonary complications after bone marrow transplantation: high-resolution CT and pathologic findings

The explosion of new knowledge about the complex mechanisms mediating high blood pressure is providing new targets for drug therapy of hypertension and other cardiovascular disorders. This article reviews the current status of several new approaches in the management of hypertension, including vasopressin antagonists, natriuretic peptide clearance inhibitors, endothelin antagonists, renin inhibitors, angiotensin receptor antagonists, and selective T-type calcium ion channel antagonists.     

CT analysis of the anterior mediastinal fat: before and after lobectomy

CT analysis was performed to evaluate change in the anterior mediastinal fat (AMF) after lobectomy and differences in AMF in the lobectomized region. In 67 carcinoma patients who underwent lobectomy, the area and volume of AMF were measured on CT before and after surgery. Mediastinal deviation after lobectomy was also correlated to the altered fat tissue. The postoperative AMF distribution was distinctly changed in the left upper lobectomy (LUL) group, showing a marked increase from the infra-aortic arch to the carina level, with converse decreases in upper and lower slices. No significant post-operative change was noted in total AMF volume. There was a close correlation between changes in AMF area and mediastinal deviation after lobectomy, and postoperative mediastinal deviation was also greater the in the LUL group than in any other group. In conclusion, postoperative change in the AMF area was distinctly different in the LUL group. Redistribution of displaced AMF is considered to be the main cause of seemingly increased fat tissue.     

Doppler sonographic diagnosis of severe portal vein pulsatility in constrictive pericarditis: flow normalization after pericardiectomy

This case report describes the noninvasive assessment of hepatic and portal vein hemodynamics in a patient with constrictive pericarditis before and after pericardiectomy. Doppler sonography of the hepatic veins demonstrated a typical W-shaped pattern with pronounced late diastolic flow reversal that disappeared after surgery. Preoperatively, we observed severe pulsatility of the portal vein with flow reversal in systole; after pericardiectomy, portal venous flow was normal. We concluded that the high right atrial pressure in this patient might have led to increased hepatic venous outflow resistance, with subsequent trans-sinusoidal shunting between the hepatic artery and portal vein causing severe portal vein pulsatility. After pericardiectomy and a decrease in right atrial pressure, portal vein flow normalized.     

Morphologic changes of the liver following chemotherapy for metastatic breast carcinoma: CT findings

Detergent-free reaction center (RC) proteins from the photosynthetic bacterium Rhodopseudomonas viridis were obtained using Bio-Beads SM-2. With these RCs, the amount of detergent molecules associated with the protein was measured by determining the detergent concentration at which re-solubilization occurred as a function of the RC concentration. For N,N-dimethyl dodecylamine-N-oxide (LDAO), Triton X-100 and beta-octylglucoside 260 +/- 30,105 +/- 10 and 360 +/- 100 detergent molecules were necessary to dissolve the protein, respectively. With this technique we have studied the effect of the amphiphilic molecule 1,2,3-heptanetriol, which is essential in the crystallization process of these RCs. Addition of 5% 1,2,3-heptanetriol reduces the value for LDAO to 120 +/- 20 LDAO/RC, supporting the notion that crystallization of the RCs is promoted by increasing the number of protein-protein contacts.     

Congenital thymic cyst: prenatal sonographic and postnatal magnetic resonance findings

The link between human Papillomavirus (HPV) and cervical carcinomas has become increasingly convincing recently, and the detection of infection with certain HPV types may become an important element in screening for cervical pre-cancer. We applied the polymerase chain reaction (PCR) technique to detect and type HPV-DNA in biopsies from the uterine cervix with a histological diagnosis of CIN and condylomatosis. Forty-eight consecutive cervical biopsies were analysed for HPV-DNA by PCR, using two different sets of consensus-primers. Typing was performed with HPV type-specific primers. Histological reevaluation revealed that 42 biopsies (87%) had condylomatous features. HPV was demonstrated in 46/48 (95.8%). HPV type 6/11 was found in seven biopsies, while HPV 16 was present in 23 samples, including two cases of double-infection. HPV type 18 was not found at all, and in 19 cases the HPV type present could not be determined. Apparently, HPV-DNA was absent in two cases. The results indicate that HPV-DNA screening is a valuable supplement to histopathological screening for cervical neoplasia. The value of different primer pairs is discussed.