Case of autoimmune neutropenia with severe marginal periodontitis

Early onset periodontitis is rarely seen in infants, though often leads to an acute and serious clinical course when encountered in such patients. Autoimmune neutropenia presents systemic and dental symptoms, as depressed resistance to bacterial infection is caused by a disorder that reduces the number of neutrophils. This disease can result in not only gingival inflammation but also destruction of periodontal tissues, such as attachment loss, alveolar bone absorption, and early tooth loss in primary as well as mixed dentition. Here, we report treatment of a child with marginal periodontitis from the age of 3 years–7 years 9 months. No systemic manifestations were noted until 3 years of age, thus the patient had never received a detailed examination or medication related to the disease. Following examinations at our department, we referred the patient to a pediatrician at our university hospital for possible systemic disease, who made a diagnosis of autoimmune neutropenia. Although administration of antibiotics and professional dental care were continued, neutrophil count was not increased and progressive periodontal destruction was observed. Extraction of teeth with poor prognosis was performed and a prosthetic strategy for the missing teeth developed. It is important to recognize that periodontitis along with autoimmune neutropenia can appear in infants, even though the incidence is quite low. Early detection and early treatment of this disease is necessary for delaying progression of periodontitis and optimal occlusal induction of permanent teeth.     

Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

Carnitine palmitoyltransferase deficiency in a patient with severe psychomotor retardation

A 13-year-old girl who had severe brain damage due to unknown prenatal cause presented rhabdomyolysis triggered by a mild viral infection. Her muscle biopsy revealed mild variation in fiber size and type 2 fiber atrophy without excess lipid storage. Biochemical analysis of the biopsied material showed decreased carnitine palmitoyltransferase (CPT) activity (15% of the control). Serum and urinary carnitine levels were normal. Skeletal muscle CT scanning showed multiple low density spots. The patient was diagnosed as having CPT deficiency. She recovered from rhabdomyolysis without renal failure after a month with conservative therapy. CPT deficiency is usually found in young healthy persons. This is the first case report of CPT deficiency which presented severe psychomotor retardation since neonatal period.     

Light exposure required for optimum conversion of light activated resin systems

OBJECTIVE: The aim of this study was to evaluate the relationship between the degree of conversion (DC) of composites and the light intensity using LED-curing units and also to determine the amount of exposure required to achieve optimal curing. METHOD: The light outputs of light-curing units and the depths of cure of composites exposed to these units were determined using the methods outlined in modified ISO standards, ISO/TS10650 and ISO 4049, respectively. The distributions of DC in composites were investigated by IR spectra of microareas obtained at various depths from the irradiated surface of thin specimens cut out from the cured composites. IR spectra were measured using a Fourier transform infrared spectrometer equipped with a microscopic unit. DC was calculated from the changes in the amount of C=C double bonds in the IR spectra. RESULTS: The light intensity at various depths through the cured composite was calculated from the attenuation coefficient of each material, obtained from the linear relationship between the depth of cure and the logarithm of the amount of exposure, which is defined as the product of the irradiance and irradiation time. There was a third or fourth order regression relationship between DC and the logarithm of total light energy at a particular depth. SIGNIFICANCE: The minimum light energy required to produce a saturated DC was about 1000 s mW/cm2.     

ANALYSIS OF BURIED BUMPER SYNDROME AFTER PERCUTANEOUS ENDOSCOPIC GASTROSTOMY DUE TO USE OF A BUTTON‐TYPE KIT

Background: Buried bumper syndrome (BBS) is a rare complication of percutaneous endoscopic gastrostomy (PEG). Along with the widespread use of the button‐type kit, BBS is encountered frequently. Methods: In the present study, we examined causes and treatments for BBS among 1400 patients who had undergone PEG. Results: The causes of BBS after PEG were classified into two categories: early causes consisted of wound infection, inappropriate size of kit and severe lordosis, while late causes were inappropriate exchange of kit, rough management or weight gain. The treatments for BBS could be determined by the degree of wound infection, fistula and burial of the bumper. Conclusion: We prepared a flowchart for replacement, by which BBS can be managed safely and quickly without surgical or endoscopic intervention.     

Testicular cancer with tumor thrombus extending to the inferior vena cava successfully removed using veno-venous bypass: A case report

A 33-year-old man with a left testicular tumor was referred to Shinshu University Hospital for advanced therapy. Radiographic imaging revealed multiple metastases in the retroperitoneal lymph nodes (RPLN) and bilateral lungs, as well as tumor thrombus that extended from the left renal vein to the inferior vena cava (IVC) adjacent to the right atrium. After orchidectomy, a diagnosis of embryonal carcinoma was made with a clinical stage of T1N2M1bS3, which has a poor prognosis, based on the International Germ Cell Cancer Collaborative Group consensus. After eight courses of chemotherapy, the patient's tumor markers normalized and the lung metastases disappeared, but the RPLN and tumor thrombus remained. Retroperitoneal lymph node dissection and thrombectomy were performed using a veno-venous bypass (VVB). The pathological examination of the thrombus revealed a mature teratoma. The patient has been disease-free since surgery.     

Morphological Analysis of the Early Development of the Chick Neural Tube Separated from the Floor Plate and Notochord

When the neural tube of avian embryos is separated from the notochord and floor plate, motoneurons in the spinal cord fail to develop. In order to investigate the factors involved in this phenomenon, cell proliferation activity and cell death were observed following paramedian incision of the neural tube at the level of the segmental plate using colchicine, BrdU, and TUNEL methods. If the notochord and/or floor plate produces a substance(s) that promotes cell division in the basal plate neuroepithelium or that supports the survival of the motoneuron's neuroblasts, mitotic figures should not be present in the neuroepithelium nor should substantial cell death be observed in the ventral aspect of the notochord- and floor plate-deprived neural tube. Surprisingly, however, neither result was observed in the present experiments, with the exception of a considerable amount of homogeneously distributed cell death. Neuroepithelial cells continued to proliferate and gave rise to neuroblasts. Nevertheless, motoneurons failed to develop, and the neural tube was enveloped by only the basement membrane of the alar plate (S. Hirano and H. Tanaka, 1994,Dev. Growth Differ.36, 481–488). These morphological results revealed that the cause of the development of the anterior horn lacking a neural tube in the notochord- and neural tube-eliminated embryos is not the elimination of the source of the surviving factor(s) of the motoneuron's neuroblasts, but rather the elimination of the signals to induce the motoneurons, derived from the notochord and/or floor plate. The larger amount of cell death in the neural tube on the experimental side suggests that a nonspecific survival factor(s), necessary for the survival of a variety of types of neuroblasts, is also produced by the notochord and/or floor plate.