Cutis verticis gyrata in a child with Turner syndrome.
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Female↗
Biomedical subjects
K Taniguchi
Publications and source records attributed to K Taniguchi.
Congenital plaquelike glomus tumor in a child.
An 8-year-old girl was seen in our outpatient clinic with a history of an angiomatous plaque on the chest since birth. She had complained of pain at the site of the lesion for 1 month. Histopathologic examination and immunohistochemical analysis led to the diagnosis of glomangioma, a rare congenital plaquelike glomus tumor. We review the literature and discuss the clinical presentation and evolution of this rare entity.
Actins↗
Successful immunization following cord blood transplantation in a child with Diamond-Blackfan anemia.
Cord blood transplantation (CBT) has been increasingly used to treat patients with hematological diseases, but active immunizations for patients have not been described. Patients certainly need immunizations following CBT, since transplanted cord blood is naive. The authors previously reported successful hematopoietic reconstitution following cord blood transplantation from an HLA-matched sibling in a transfusion-dependent child with Diamond-Blackfan anemia. No graft-versus-host disease, either acute or chronic, has been observed so far. Here, the authors report that immunological recovery of the patient has been rapid shortly after CBT and immunization has been done successfully. Vaccines (diphtheria, pertussis, tetanus, rubella, measles, and BCG) were administered during 22-34 months post-transplant. Seroconversion to these vaccines was excellent without significant adverse effects. These results indicate that both toxoid and live vaccines have been safely administered in the patient who underwent related cord blood transplantation.
Antibody Formation↗
A new method of assessing left ventricular function under assisted circulation using a conductance catheter.
A new method of assessing left ventricular (LV) systolic function without terminating assisted circulation (AC) was investigated using a LV volume catheter (conductance catheter) in canine cardiopulmonary bypass (CPB) (Group 1, n = 7) and left heart bypass (LHB) (Group 2, n = 5) models. The hearts were subjected to either 20 min of global ischemia (IS), with a subsequent 80 min of reperfusion under CPB, or regional IS under LHB. Instantaneous LV pressure-volume data acquisitions were repeated during transient (15 sec) acute volume loading without terminating bypass. The relationship between the stroke work (SW), determined as the area of the pressure-volume loop, and the end-diastolic volume (EDV), were highly linear at every study point (mean: r = 0.956-0.986 in Group 1, r = 0.974-0.987 in Group 2). The slopes of SW and EDV (preload recruitable stroke work: PRSW) significantly decreased after IS, in both Group 1 and Group 2. In conclusion, measurement of PRSW without terminating bypass seemed to be useful in evaluating LV systolic function in patients under AC.
Animals↗
Normothermia has beneficial effects in cardiopulmonary bypass attenuating inflammatory reactions.
"Post perfusion syndrome" after cardiopulmonary bypass (CPB) has been known to be evoked by inflammatory reactions. To elucidate the effect of perfusate temperature during CPB on subsequent inflammatory reactions, serum levels of interleukin (IL)-6, IL-8, tumor necrosis factor-alpha, IL-1 beta, and polymorphonuclear (PMN) elastase were measured before and after (0, 12 hours, and 24 hours after) CPB in adult patients undergoing cardiac operations. Patients were divided into two groups: the normothermic CPB group (NOR, 34 degrees C perfusate temperature, n = 8) and hypothermic CPB group (HYP, 28 degrees C perfusate temperature, n = 8). In both groups, IL-6, IL-8, and PMN elastase levels were elevated during CPB, while the increase of tumor necrosis factor-alpha and IL-1 beta was not detected. The increase in IL-8 and PMN elastase levels at 12 hours after CPB was significantly less in NOR than in HYP (IL-8; NOR versus HYP, 15.1 +/- 12.9 versus 62.5 +/- 30.6 pg/ml, p < 0.05, PMN elastase; 394 +/- 200 versus 1085 +/- 523 micrograms/L, p < 0.05) while neither group showed any difference in IL-8 and PMN elastase at 24 hours after CPB. There was no difference in IL-6 between the two groups, and no significant increase in tumor necrosis factor-alpha and IL-1 beta levels were detected in either group after CPB. These results demonstrate that although CPB activated inflammatory reactions detected by IL-8 and PMN elastase activity, post operative persistence of these reactions were attenuated by warm CPB.
Adult↗
Gastric intramucosal pH during lower body circulatory arrest under open distal anastomosis with selective cerebral perfusion in aortic arch repair.
Selective cerebral perfusion (SCP) and open distal anastomosis (OD) with hypothermia has been used as a popular means for circulatory assistance in aortic arch surgery. Although SCP has become accepted for brain protection, the influence of OD accompanying circulatory arrest on lower body ischemia is not known. We studied gastric tonometry (gastric intramucosal pH [pHi]) to estimate splanchnic ischemia during OD, and its relationship to postoperative organ function. In five patients (pts) (range, 65-78 years; mean, 71 years; group OD) who underwent arch replacement using SCP and OD with moderate hypothermia (25 degrees C) during the period from March to August of 1999, pHi was measured precardiopulmonary bypass (pre-CPB), 30 min of CPB (CPB30), 10 min after OD (OD10), at end of CPB, and post-CPB. Eight pts (range, 52-78 years; mean; 66 years) who underwent standard CPB (33 degrees C) during the same period (coronary artery bypass surgery in six and valve surgery in two) served as controls (group C). In group OD, pHi was significantly decreased at OD10 (7.35 +/- 0.03 at CPB30 vs. 7.23 +/- 0.07 at OD10, p < 0.05) but recovered by the end of CPB (7.32 +/- 0.02). Creatinine clearance on the first postoperative day (1POD) was significantly (p < 0.05) lower in group OD (82 +/- 40 ml/min) than in group C (126 +/- 25 ml/min), although there was no significant difference in preoperative values between the two groups. The pHi at OD10 did not correlate with the duration of OD (range, 30-47 min; mean, 38 min), whereas pHi at OD10 significantly correlated with BUN (r = -0.973, p = 0.0054), Cr(r = -0.977, p = 0.0043), and CCr (r = 0.908, p = 0.0328) on 1POD. One patient in group OD developed paraplegia and renal failure postoperatively. His pHi at OD10 was severely decreased to 7.11. These results suggest that intraoperative monitoring of pHi may be useful for the evaluation of visceral organ ischemia during OD in arch replacement and may contribute to improved technique for circulatory assistance in aortic surgery.
Aged↗
Usefulness of the low profile "True 8" intra-aortic balloon pumping catheter for preventing limb lschemia.
The most important limitation in the use of an intra-aortic balloon pumping (IABP) is the risk of vascular complications. Recently, an IABP catheter with an 8.0 French shaft and low profile that may decrease the risk of vascular morbidity has been developed. We evaluated the in vitro balloon performance and the prevention of limb ischemia in clinical use. An 8.0 French IABP catheter was compared with a standard 9.5 French catheter. Inflation time (IT), deflation time (DT), and changes in volume (V) generated by the balloon were measured during the pumping cycle in an experimental model. The inflation velocity (V/IT) and deflation velocity (V/DT) were calculated as parameters of balloon performance. At 120 bpm the V/IT and V/DT were 0.34 ml/msec and 0.28 ml/msec with the 8.0 French, and 0.33 ml/msec and 0.24 ml/msec with the 9.5 French catheter. Twelve patients with coronary artery disease, ranging in age from 41 to 87 years (mean, 66 years), who underwent IABP support, were divided into group 1 (8.0 French, n = 4) and group 2 (9.5 French, n = 8). Ankle-arm pressure index (API), lactate extraction ratio (LER) in the limb with IABP insertion, and cardiac index (CI) were measured at 1, 12, and 24 hours postoperatively. There were no major vascular complications and no counterpulsation related morbidity. There was no significant difference between the two groups with regard to age, duration of IABP support, and incidence of peripheral vascular disease and diabetes. The percentage of women patients was significantly higher in group 1 (100% vs. 25%), whereas body surface area was significantly smaller (1.45 +/- 0.14 vs. 1.68 +/- 0.12 m2). The API in group 1 were slightly higher than those in group 2 throughout the observed period (not significant). The LER and Cl showed no significant differences between the two groups. These results suggest that the 8.0 French IABP catheter with a low profile has an acceptable in vitro performance, and its clinical application may be effective in preventing limb ischemia in a high-risk subset of patients such as women and smaller patients.
Adult↗
Focal fibrocartilaginous dysplasia: consideration of healing process.
We report two patients with unilateral tibia vara due to focal fibrocartilaginous dysplasia of the medial aspect of the proximal tibia. It appears that tibia vara, caused by a primary self-limited lesion at the proximal medial metaphysis, is corrected by the response of the proximal tibial physis. This finding would indicate that an infantile growth plate of the proximal tibia has a rather large potential to correct an angular deformity at the adjacent metaphysis.
Biopsy↗
Retinal detachment in spondyloepiphyseal dysplasia congenita.
Retinal detachment is one of the most serious complications of spondyloepiphyseal dysplasia congenita (SEDC). The ocular complication of 12 patients with SEDC was studied. Three patients developed bilateral retinal detachments; five of the six detachments occurred during the period of rapid growth spurt. Our results suggest that patients with SEDC have an increased risk for development of retinal detachment during the period of rapid growth spurt.
Adolescent↗
Pathologic dislocation of the hip in von Recklinghausen's disease: a report of two cases.
We report two cases of von Recklinghausen's disease in which hip dislocations occurred gradually without trauma. In one case, the right hip joint dislocated at the age of 8 years, and in another case, the left hip dislocated at 2 years and the right hip at 3 years. Each case was accompanied by bone changes around the affected hips, vertebral deformity, and spinal tumor. One case had hemihypertrophy of the involved lower extremity. Both structural changes around the hip joint and paralysis may have resulted in the hip dislocation.
Bone Neoplasms↗
A practical classification system for multiple cartilaginous exostosis in children.
Forty-one patients with multiple cartilaginous exostosis (MCE) were classified into three groups: Group I, no involvement of the distal forearm (n = 8); Group II, involvement of the distal forearm without shortening of the radius or ulna (n = 11); Group III, involvement of the distal forearm with shortening of the radius or ulna (n = 22). Groups were compared with regard to number of lesions, regional morbidity rate, age of onset, height, and presence of valgus deformity of the ankle. From these results, children in Group I were mildly affected. Further, those in Group III were severely affected, and all children in Group III had lesions of the innominate bone or proximal femur, where secondary chondrosarcoma occurs most frequently. This classification should prove useful in estimating severity and identifying cases at high risk for malignant transformation. MCE seemed to be an aberrant enchondral ossification and its mode of development to be reflected in the growth potential of each bone. Dislocation of the radial head occurred in three cases and was the initial symptom in two of these. Dislocation of the radial head may occur earlier than previously thought, hindering its prevention.
Adolescent↗
Aortic valve replacement for aortic stenosis with a small mechanical prosthetic valve.
BACKGROUND: Although aortic valve replacement (AVR) is the only effective treatment for patients with aortic stenosis (AS), it is recognized that the use of small prosthetic valves due to a small aortic root often affects postoperative course after AVR. The aim of this study was to determine whether the use of small prosthetic valves was a risk factor of AVR for AS. METHODS: We compared various perioperative factors and operative outcomes between patients with a small mechanical prosthetic valve (small group) and patients with a large mechanical prosthetic valve (large group). RESULTS: Early mortality was 0% in each group and the 5-year mortality was 25% in the small group and 10% in the large group. There were no significant differences in perioperative factors between the two groups. The small group patients were significantly older and smaller compared to the large group patients. The valve size was significantly correlated with age and BSA. CONCLUSIONS: The use of small mechanical prostheses was not a risk factor of AVR for AS when it was proportionate to the BSA even for elderly patients. AVR using a small mechanical prosthetic valve may be performed with good results in the short- and long-term.
Age Factors↗
[Epidermal nevus syndrome - a case report]
OBJECTIVES: To present the case of a patient with an epidermal nevus since birth and its relationship to neurologic signs and symptoms, emphasizing the importance of cutaneous manifestations as early markers of syndromes involving the Central Nervous System. METHODS: Clinical, radiological and histopathological data were analyzed. RESULTS: We report the case of a boy with an erythematous lesion on the neck and cheek since birth. At 9 months the lesion was velvety and slightly brown in color, with associated hemihypertrophy of the face. After 3 months he was hospitalized for having seizures. Computerized tomography of the brain disclosed hemimegalencephaly ipsilateral to the cutaneous lesions. DISCUSSION: The cutaneous findings can be an indicator of neurologic disease since both tissues have the same embryological origin: the neural crest. Thus, the Pediatrician must recognize these cutaneous signs which appear early in life and characterize some of the Neurocutaneous Syndromes so that a proper diagnosis and follow-up can be made.
Journal Article↗
Immunohistochemical study of MT-MMP tissue status in gastric carcinoma and correlation with survival analyzed by univariate and multivariate analysis.
Matrix metalloproteinase (MMP) expression is associated with advanced-stage cancer and contributes to tumor progression, invasion, and metastasis. Membrane type matrix metalloproteinase (MT-MMP) has a potential transmembrane domain at the C terminus and activates pro-MMP-2, which is mainly produced from interstitial fibroblasts. Its expression on the membrane of invasive tumor cells results in the pericellular space degradation at cell-matrix contact sites and renders cancer cells more invasive at the migration front. To elucidate the relationship between MT-MMP expression and metastasis and prognosis in gastric cancer patients, MT-MMP expression was analyzed immunohistochemically in 127 primary tumors and results were correlated with several prognostic parameters and patient's survival. MT-MMP immunoreactivity was stained on the cell membrane of cancer cells and fibroblasts in the invasion front. MT-MMP was detected in 72 tumors (57%) (MT-MMP-positive). MT-MMP expression was closely associated with macroscopically invasive type, nodal involvement, lymphatic invasion, vessel invasion, and peritoneal dissemination. Patients with MT-MMP-positive tumor had a significantly worse prognosis than those with MT-MMP-negative tumor (p<0.001). Multivariate analysis showed MT-MMP overexpression as an independent prognostic factor in gastric cancer patients. Immunohistochemical analysis for MT-MMP may be an indicator of metastatic potential or the prognosis of gastric cancer patients.
Adult↗
IgE deposition in brain microvessels and on parasitized erythrocytes from cerebral malaria patients.
Postmortem brain tissues of 21 cerebral malaria cases were obtained in Myanmar and Vietnam. The tissues were examined by light microscopy and by an immunohistochemical method. Brain microvessels (capillaries and venules) were examined for the presence of immunoglobulins IgE and IgG, Plasmodium falciparum antigen, and parasitized erythrocytes (PRBC). Deposition of IgE, IgG, and P. falciparum antigen was observed in the microvessels from all specimens examined. Sequestered PRBC in the microvessels were positive for IgG in all 21 cases and for IgE in six cases. In the latter cases, the percentage of microvessels with sequestered PRBC was > 50%, with the frequency of IgE-positive cells ranging from 42% to 52%. In contrast, in five cases that were only weakly positive for IgE, the percentage of microvessels with sequestered PRBC was remarkably low (< 1%). These data indicate that the degree of deposition of IgE in microvessels and on PRBC from cerebral malaria patients correlated with that of PRBC sequestration. As IgE-containing immune complexes are known to induce local overproduction of tumor necrosis factor-alpha (TNF-alpha), a major pathogenic factor in cerebral malaria, IgE may contribute to the pathogenesis of this severe disease.
Adolescent↗
Magnetic resonance imaging findings and treatment outcome in late-onset tibia vara.
Several retrospective studies have shown the usefulness of magnetic resonance imaging (MRI) in the evaluation of tibia vara. With respect to the late-onset type, however, there have been no English-language reports on MRI findings. In this article, the authors report on the MRI features of three patients with late-onset tibia vara, the results of treatment that was chosen on the basis of the findings, and the histologic characteristics of the disease. Fat-suppressed MRI revealed partial closure of the tibial physis in one patient and near-closure in another patient. They underwent osteotomy with resection of the bone bridge and have shown no evidence of recurrence. In the third patient, only irregularity of the physis was found on MRI, and she was treated conservatively. Her varus deformity subsequently improved, although it persisted. Histologic examination revealed disorganization and misalignment of the physeal zone and transverse alignment of the trabeculae. The patient with a bony bridge also had discrete ossification centers in the physeal zone.
Bone Diseases, Developmental↗
Serial changes in myocardial beta-adrenergic receptor after experimental brain death in dogs.
Early donor heart failure is one of the serious problems in heart transplantation. This heart failure may be related to myocardial change during brain death before implantation of the heart. Actually, a gradual increment in catecholamine requirement to maintain hemodynamics is not infrequently seen in patients with brain death. This may presumably be related to alterations in the myocardial beta-adrenergic receptor system. In this experimental study, we investigated the changes in myocardial beta-adrenergic receptors after brain death induced by intracranial hypertension in dogs. To maintain the mean arterial pressure higher than 60 mm Hg after brain death, massive crystalloid infusion (group A) or 0.4 to 1.3 micrograms/kg/min of adrenaline infusion (group B) was applied. Blood adrenaline levels and myocardial beta-adrenergic receptor (receptor number and affinity with [125I]iodocyanopindolol binding) were measured at 3 hours (group A-3H or group B-3H, n = 5, respectively) and 6 hours (group A-6H or group B-6H, n = 5, respectively) after brain death. Blood adrenaline levels reduced in group A-6H and elevated in group B-3H and group B-6H. Beta-adrenergic receptor affinity was not different among each group. Beta-adrenergic receptor number decreased in group B-6H. Myocardial beta-adrenergic receptor density decreased in the adrenaline group during 6 hours of hemodynamic maintenance after brain death, whereas myocardial beta-adrenergic receptors did not change in the crystalloid group. Our data suggest that the large doses of catecholamines used to maintain hemodynamics in donors with brain death may depress myocardial beta-adrenergic receptors.
Animals↗
Idiopathic odontoma formation following avulsion of immature permanent incisors: two case reports.
From the findings of Case 1 and those cases reviewed in the literature it would appear that overfilling a canal with gutta percha prevents continued root formation after reimplantation of the tooth. The canal should be underfilled, therefore, if gutta percha is used as the obturating medium. Consequently, the authors recommend that calcium hydroxide be used as the root canal filling material of choice after reimplanting immature permanent teeth subsequent to traumatic avulsion. Since, however, calcium hydroxide paste tends to be resorbed, periodic refilling of the canal with the paste is required. Case 2 emphasizes the importance of periodic postoperative radiographic evaluation for several years after traumatic avulsion of immature permanent teeth.
Calcium Hydroxide↗