Intrapulmonary lymph nodes in children versus lung metastases.
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Adolescent↗
Biomedical subjects
N Aida
Publications and source records attributed to N Aida.
MR findings of bowlegs in toddlers.
BACKGROUND: Toddlers with severe physiologic tibial bowing are considered to be at risk for the development of Blount's disease. Objective. To correlate MR findings of the knee with the clinical outcome in toddlers with severe physiological tibial bowing. MATERIALS AND METHODS: MR findings were evaluated in 22 affected legs of 14 children with severe tibial bowing (mean age 1.9 years). In 18 affected legs, MR findings were compared with the transition of the tibial metaphyseal-diaphyseal angle (MDA) and tibiofemoral angle (TFA) measured serially between 2 and 3 years of age. RESULTS: MR findings of severe tibial bowing comprised undulation of the posteromedial physis of the tibia (3/22), signal alterations in the medial tibial metaphysis (10/22), T2 prolongation in the posteromedial tibial epiphyseal cartilage (14/22) and signal changes in the medial menisci (18/22). The decrease in the TFA was different in the legs with and without increased signal in the epiphyseal cartilage, and the decrease in the MDA was different in the legs with and without physeal undulation. CONCLUSION: MR imaging findings can predict the retarded resolution of tibial bowing, which may be a risk factor for the development of Blount's disease.
Bone Diseases, Developmental↗
A new NEDD8-ligating system for cullin-4A.
NEDD8 is a ubiquitin (Ub)-like protein. Here we report a novel ubiquitinylation-related pathway for modification by NEDD8. NEDD8 was activated by an E1 (Ub-activating enzyme)-like complex, consisting of APP-BP1 and hUba3 with high respective homologies to the amino- and carboxy-terminal regions of E1 and then linked to hUbc12 (a human homolog of yeast Ub-conjugating enzyme Ubc12p). The major target protein modified by NEDD8 was found to be Hs-cullin-4A (Cul-4A), a member of the family of human cullin/Cdc53 proteins functioning as an essential component of a multifunctional Ub-protein ligase E3 complex that has a critical role in Ub-mediated proteolysis.
Amino Acid Sequence↗
Early carcinoma of the gallbladder accompanied by hemobilia: report of a case.
The majority of cases of hemobilia are of traumatic or vascular origin; however, we report herein the rare case of a 51-year-old man with early carcinoma of the gallbladder accompanied by hemobilia. He had been diagnosed as having gallbladder polyps during an admission to a local hospital for treatment of cardiac failure. Preoperative evaluation in our hospital suggested that the polypoid lesion was an advanced carcinoma of the gallbladder. Laparotomy revealed that the gallbladder contained blood clots, and a cholecystectomy was performed. Histological examination confirmed the main lesion to be IIb-type early carcinoma of the gallbladder and proved that the blood clots had been misinterpreted as polyps or advanced carcinoma before the operation. The diagnostic approaches and characteristics of hemobilia in the gallbladder are discussed with a review of the literature.
Adenocarcinoma↗
A distinct subtype of "metatropic dysplasia variant" characterised by advanced carpal skeletal age and subluxation of the radial heads.
BACKGROUND: "Metatropic dysplasia variants" are a group of bone dysplasias whose skeletal abnormalities are similar to, but milder than, those of classical metatropic dysplasia. The genetic and phenotypic heterogeneity has not been thoroughly elucidated. OBJECTIVE: The objective was to designate a distinct subtype of these metatropic dysplasia variants. MATERIALS AND METHODS: The subjects were four Japanese patients, two sporadic cases and two siblings, who all had identical skeletal changes. The radiological features in these patients were compared with those of previously reported metatropic dysplasia variants. RESULTS: Moderate platyspondyly with pear-shaped and/or anterior-tongued vertebral bodies, halberd pelvis, and dumbbell deformity of the tubular bones were regarded as hallmarks of metatropic dysplasia variants. The peculiar skeletal change in our patients was advanced carpal skeletal age in childhood, unlike most patients reported as metatropic dysplasia variants who manifest delayed carpal ossification. Another hallmark was congenital dislocation of the radial heads. A description of a patient with similar skeletal changes was found in the literature. CONCLUSION: These patients are considered to represent a distinct subgroup of metatropic dysplasia variants. It remains unknown whether the present siblings represent an autosomal recessive trait or an autosomal dominant trait with germinal mosaicism related to increased paternal age.
Abnormalities, Multiple↗
[A case of ureteral polyp prolapsing into bladder in a 3-year-old boy].
A 3-year-old boy was referred to us with a diagnosis of bladder tumor. On cystoscopy, a yellowish-white pedunculated tumor was found at the right trigone and biopsy revealed an inflammatory change. Computed tomographic (CT) scan showed a soft tissue density from the lower ureter to the bladder. Open surgery confirmed a ureteral polyp originating from the lower ureter prolapsing into the bladder, which mimicked bladder tumor. The need of open surgery in such cases is discussed.
Child, Preschool↗
Large intracranial vessel occlusive vasculopathy after radiation therapy in children: clinical features and usefulness of magnetic resonance imaging.
PURPOSE: To assess the relationship between large intracranial vessel occlusive vasculopathy (vasculopathy) and radiation therapy, and to clarify the clinical efficacy of magnetic resonance (MR) imaging in the diagnosis and screening of the vasculopathy. METHODS AND MATERIALS: We retrospectively evaluated the medical records and serial MR images for 32 pediatric patients, in whom radiation therapy had been given to fields including the circle of Willis and major cerebral arteries. All children had periodically undergone follow-up neurologic assessment and MR imaging examinations at Kanagawa Children's Medical Center for more than one year after radiation therapy (range 1.3-14 years). Patients who had not remained free of tumor progression up to the time of final evaluation were excluded. RESULTS: Vasculopathy developed in 6 of 32 patients 2-13 years after radiation therapy. Three of them presented with transient ischemic attacks (TIA) and the other three showed infarctions without preceding TIA. Steno-occlusive changes of major cerebral arteries were identified by MR imaging in all six patients, but not obtained in the remaining 26 patients. In the patients with TIA, MR imaging demonstrated steno-occlusive changes at the time of TIA, before irreversible infarction. They have been doing well subsequent to encephaloduroarteriosynangiosis. In the three patients who presented infarction without preceding TIA, MR imaging did not demonstrate the vascular change before the onset of infarction, and two of them developed neurologic deficits. The mean exposure dose for the circle of Willis and major cerebral arteries in these six patients was significantly higher than that in the remaining 26 patients without this sequela (61 Gy vs. 50 Gy, p < 0.05). The mean age at radiation therapy of the six patients was lower, but the difference was not significant. CONCLUSION: The incidence of vasculopathy after radiation therapy has a considerable correlation with radiation dose and age at radiation therapy. MR examination is useful for the diagnostic evaluation of vasculopathy, and it is also effective in screening for vasculopathy in patients with TIA, and may be helpful in the prevention of neurologic sequela.
Adolescent↗
Acute gastric outlet obstruction following the administration of prostaglandin: an additional case.
We present a case of neonatal acute gastric outlet obstruction related to prostaglandin-induced gastric foveolar hyperplasia, which developed following infusion of prostaglandin E1 (PGE1) for treatment of hypoplastic left heart syndrome. Abdominal distension occurred after administration of PGE1 in a cumulative dose of 2914 microg/kg. Ultrasonography performed after a cumulative dose of 5074 microg/kg had been administered disclosed a lobulated thickening of the gastric mucosa with a brush-like appearance composed of alternately echogenic and hypoechoic, vertically oriented lines. These ultrasonographic findings corresponded to the histological abnormalities of gastric foveolar hyperplasia with impacted interfoveolar mucin products and dilated mucosal glands. The development of gastric outlet obstruction in our patient, a relatively rare manifestation of prostaglandin-induced foveolar hyperplasia, might have been related to the unusually high cumulative dose of PGE1.
Acute Disease↗
Microscopic intraosseous extension of osteosarcoma: assessment on dynamic contrast-enhanced MRI.
OBJECTIVE: This study investigated whether dynamic MRI could determine microscopic intraosseous invasion in osteosarcoma. PATIENTS: Six patients with osteosarcoma who underwent total resection of the tumor after chemotherapy were studied. DESIGN: The steepest slope value of the time-intensity curve was calculated on the basis of the first-pass method on dynamic MRI performed after chemotherapy. Slope value modified log = [100.(SIb - SIa)/(Tb - Ta)/SIa], where SIa and SIb represent the signal intensity at time points Ta and Tb when the steepest slope started and ended, respectively. The slope value was correlated with the histological findings of the surgical specimens. The findings on conventional MRI were also reviewed. RESULTS AND CONCLUSIONS: In five of six patients microscopic tumor invasion ranging from 1 to 3.5 cm in depth was pathologically proven. The slope value within the microscopic invasion (-0.052 +/- 0.267) was significantly greater than that of tumour-free marrow (-0.609 +/- 0.220) and less than that in areas showing macroscopic tumor invasion (0.595 +/- 0.483) (P < 0.001). Histological examination disclosed the dilatation of small vessels within the macroscopic and microscopic invasion. Findings on conventional MRI varied among patients; microscopic invasion was not detected in one, underestimated in two, and overestimated in two. It is concluded that calculation of the slope value on dynamic MRI can discriminate regions of microscopic invasion from tumor-free marrow in patients with osteosarcoma after chemotherapy.
Adolescent↗
Osteopathia striata, short stature, and characteristic facies: a previously unknown skeletal dysplasia.
UNLABELLED: We report two sporadic cases of a hitherto undescribed skeletal dysplasia with short stature and characteristic facies. The present patients, a 6-year-old girl and a 15-year-old boy, were almost equally affected. Craniofacial anomalies included a sloping forehead, bitemporal bulging, sparse medial eyebrows, a prominent nasal bridge, hypertelorism, proptosis, a beaked nose, hypoplastic alae nasi and a pointed chin. Shallow orbits, short anterior cranial fossae and bitemporal bossing found on skull radiograph corresponded with the facial dysmorphism. Thickening of the dorsum sellae was another hallmark in the skull. Skeletal survey revealed mild osteopenia, interpediculate narrowing of the lumbar spine with short neural arches and, most important, osteopathia striata of the long tubular bones. There was no sclerosis of the craniofacial bones. The clinical and radiological findings in the present patients were overall inconsistent with those of previously known skeletal dysplasias and congenital malformation syndromes, which possess osteopathia striata as a cardinal feature. CONCLUSION: The unique clinical and radiological constellation of our patients constitutes a hitherto unknown bone dysplasia.
Adolescent↗
[Two cases of toluene embryopathy with severe motor and intellectual disabilities syndrome].
We reported two patients with severe motor and intellectual disabilities syndrome, who were born to mothers having inhaled organic solvents during pregnancy. They had microcephaly, cerebral palsy, mental retardation, seizures, growth failure and minor craniofacial anomalies, variable growth deficiency including a small midface, narrow bifrontal diameter, low-set ears, thin upper lips and micrognathia. Patient 1, a male, died at 8 years and 8 months. The autopsy of his brain revealed marked cerebral atrophy and destruction of bilateral temporal lobes with ventricular enlargements. Microscopic examination revealed migration disorders with polymicrogria at the remaining cerebrum and the cerebellum as well as very thin white matter. Much hemosiderin was found around ventricles, suggesting recurrent minimal bleedings which led to more brain atrophy. Patient 2, a 5 months old male infant, had infantile spasms. On CT and MRI, he had bilateral temporal lobe defect, which might be due to the infarction of bilateral middle cerebral arteries at the prenatal period. These clinical findings are similar to those of other embryopathies, caused by alcohol, phenytoin and other agents. Hersh et al. reported five cases of toluene embryopathy in 1985 and 1988, but they did not report such central nervous system abnormalities. The pathogenesis of toluene embryopathy remains to be solved, but our cases suggested the possible teratogenesis of toluene.
Abnormalities, Drug-Induced↗
[A case of idiopathic spinal epidural hematoma with a fructuation of the symptoms].
We reported a 9-year-old girl with idiopathic spinal epidural hematoma. She complained of her back and neck pain only during night for a few days, followed by days with no symptoms (a few days remission of her symptoms). This episode repeated, and she was suspected to have a psychogenic reaction. However, 28 days later after the first symptoms appeared, paralysis of lower extremities, anesthesia and urinary bladder dysfunction (neurogenic bladder) appeared. Spinal MRI showed a spinal epidural hematoma at the C 5-Th 2 level. An emergency operation of laminectomy and evacuation of the hematoma was done, and she has recovered gradually. We diagnosed her as having idiopathic spinal epidural hematoma. Excerbations of symptoms during nights were thought to be due to an elevation of venous pressure by lying, and transient remissions were due to intermittent small hemorrhages.
Child↗
MRI findings of dysplasia epiphysealis hemimelica.
We report a boy with dysplasia epiphysealis hemimelica of the knee, particularly emphasizing MRI findings. When he was 14 months of age, plain radiography exhibiting juxta-articular soft tissue mass with small, punctate, calcified foci did not warrant the definitive diagnosis, while MRI clearly demonstrated osteocartilaginous overgrowth of the femoral epiphysis. The epiphyseal mass mostly showed the same signal intensity as normal cartilage but contained low signal spots corresponding to calcified foci. The cartilaginous cap was depicted as a mottled area of high intensity on a T2-weighted image. When the patient was 4 years of age, this ossified mass was resected surgically and pathologically identified as osteochondroma incorporated into the epiphysis.
Cartilage, Articular↗
Currarino triad with a terminal deletion 7q35-->qter.
We describe a de novo terminal deletion of the long arm of chromosome 7 in a 5 year old girl with the Currarino triad, characterised by congenital anorectal stenosis, a sacral defect, and a presacral mass. Recently, this autosomal dominant trait has been shown to be linked to 7q36, the same region as holoprosencephaly (HPE3). The cytogenetic findings in the present patient with the Currarino triad provided further evidence that a gene(s) for the Currarino triad is located in the 7 q terminal segment.
Abnormalities, Multiple↗
Brain MR in Fukuyama congenital muscular dystrophy.
PURPOSE: To determine the MR characteristics of brain abnormalities in Fukuyama congenital muscular dystrophy (FCMD). METHODS: We reviewed 30 MR examinations of 21 patients with FCMD to assess cerebral and cerebellar cortical dysplasia, white matter changes, and miscellaneous abnormalities. RESULTS: On MR images, all patients had thick and bumpy cortices with shallow sulci corresponding to polymicrogyria, and 12 patients had pachygyric cortices with smooth surfaces, corresponding to type II lissencephaly. Both types of cortical dysplasia had characteristic distributions: the first type involved the frontal lobe in all 21 patients and also the parietotemporal lobe in 6 patients; the second type involved the temporooccipital lobes. Eighteen patients had prolonged T1 and T2 signal in the white matter, which was indistinct in neonates and seen infrequently in adolescents. In four patients, abnormal vessels were seen within the pachygyric cortices. CONCLUSION: MR studies of the brain show findings consistent with the known characteristics of FCMD. The MR detection of the two types of cerebral cortical dysplasia with characteristic distribution and cerebellar abnormalities is helpful in the differential and early diagnosis of FCMD.
Adolescent↗
Cystic lesion of the buttock communicating with the lateral side of the posterior urethra.
We report a case of a congenital cystic lesion of the right buttock without cutaneous or rectal fistula. The cystic lesion communicated with the right lateral side of the posterior urethra via a fistulous tract.
Buttocks↗
Cerebellar MR in Fukuyama congenital muscular dystrophy: polymicrogyria with cystic lesions.
PURPOSE: To determine the MR appearance of cerebellar abnormalities in Fukuyama congenital muscular dystrophy. METHODS: We reviewed brain MR images of 25 patients with Fukuyama congenital muscular dystrophy and examined the autopsy specimens of a 23-month-old girl with the disease to determine the pathologic nature of the MR findings. RESULTS: MR studies revealed two characteristic cerebellar abnormalities: (a) disorganized cerebellar folia (16 cases) that were recognized as unusual distortions of the cortex; and (b) clusters of intraparenchymal cysts (23 cases). The two lesions were located close to each other, and milder lesions tended to affect only the superior semilunar lobule. The autopsy specimen revealed small cerebellar cysts, which consisted of dilated subarachnoid spaces buried beneath the malformed cortex. CONCLUSION: The disorganized folia represent cerebellar polymicrogyria, and the presence of cerebellar cysts is related to the polymicrogyria. These two MR changes are often present in Fukuyama congenital muscular dystrophy and are distinct enough to suggest the radiologic diagnosis.
Adolescent↗
Arteriovenous malformations of the kidneys: diagnosis and follow-up with color Doppler sonography in six patients.
We performed color Doppler sonography in six patients with arteriovenous malformations of the kidneys. The diagnosis was established by angiography in all patients. Color Doppler sonograms were obtained at a large Doppler frequency-shift range (55 cm/sec of maximal average flow velocity at zero Doppler angle) to depict high-velocity blood flow in the malformation. In all patients, the malformations were seen as focal areas of flow, portrayed as a mixing of lighter colors. These were reflected by a rapid flow rate and marked tortuosity of the vessels. The sonograms showed a small peripheral malformation that was indistinct on selective renal angiography. However, flow in normal vessels grouped in the hilum obscured lighter-color flow of a small central malformation. In three patients who had total or partial ablation of the malformations with alcohol, follow-up color Doppler sonograms showed that the focal areas of flow, represented as mixing of lighter colors, disappeared or became smaller. This study shows that color Doppler sonography is a useful noninvasive procedure for diagnosing arteriovenous malformations of the kidney.
Adult↗