Cases reported "Disease Progression"

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1/54. epilepsy surgery in children with pervasive developmental disorder.

    Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.
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2/54. Reduction of end-stage malignant glioma by injection with autologous cytotoxic T lymphocytes.

    Autologous cytotoxic T lymphocytes (CTL) against primary-cultured malignant gliomas were generated from peripheral blood mononuclear cells in vitro in 4 patients. Activities of the CTL were highly specific to the corresponding autologous glioma and were inhibited, in one patient, with antibodies against CD3, CD8 and MHC-class I molecules. When the CTL were injected 3 times into the primary-tumor-resected cavity via an Ommaya tube, reduction of the recurrent tumors with magnetic resonance imaging (MRI)-measured volumes exceeding 45 cm3 was observed in 3 patients. In a patient with glioblastoma multiforme (GBM), the tumor volume (estimated, 130 cm3) was rapidly reduced to 1/3, although re-recurrence of the tumor followed 40 days later. A slight but distinct rapid reduction of the tumor volume was observed in another GBM patient and in an anaplastic astrocytoma patient; essentially no change was observed in a further GBM patient. These results suggest that adoptive immunotherapy with autologous CTL will be clinically effective against end-stage malignant gliomas.
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3/54. Spontaneous disappearance of temporo-frontal arachnoid cyst in a child.

    We report a child with a large temporo-frontal arachnoid cyst which resolved spontaneously. There was no history of a head injury. The patient was a boy aged 1.6 years. Though a large head was pointed out ( 2SD), no therapeutic intervention was made because the relationship of the head circumference and the cyst was not established. No change in cyst size was visualized on the follow-up CTs at the age of 2.5 years and 6 years. At the age of 7 years, the arachnoid cyst had completely disappeared on CT. In order not to overlook a minute change in cyst size, the volumetry of the cyst and the intracranial cavity was performed, using the Photoshop, Macintosh. Both the cyst volume and the volume ratio of the cyst to the intracranial cavity slightly decreased and then increased. It is speculated that the cyst spontaneously ruptured by factors such as extreme breath holding and crying on the presence of the higher intracystic tension which might become a factor to accelerate spontaneous rupture of the cyst. Since a number of paediatric cases of symptomatic arachnoid cysts in need of surgical intervention has been larger than that of adult cases, we can speculate that a large arachnoid cyst might spontaneously resolve more frequently than we had expected. This case demonstrates that the surgical treatment of asymptomatic arachnoid cyst in the middle cranial fossa is not necessarily indicated in children.
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4/54. Tracheal intubation in a child with trismus pseudocamptodactyly (Hecht) syndrome.

    Tracheal intubation of a child with trismus pseudocamptodactyly (Hecht) syndrome is described. This disorder is characterized by progressive trismus and the need for repeated surgeries. Children intubated orally on a prior occasion may require an alternative approach subsequently due to progressive inability to open the mouth. An antegrade fiberoptic-guided nasotracheal technique initially was chosen due to extremely limited mouth opening. After this approach was unsuccessful, a retrograde guidewire-assisted fiberoptic intubation was performed. The manifestations of Hecht syndrome, as well as both techniques for tracheal intubation employed, are reviewed.
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5/54. A five-year-old with a dental abscess: a case study.

    dental caries remain one of the most common disorders of childhood in the United States. Often nurse practitioners (NPs) will see children who are suffering from the complications of a dental carie, such as a dental abscess and/or cellulitis. This article describes the case of a 5-year-old girl who presented at an evening clinic with tooth pain, fever, and facial swelling. Three treatment choices are discussed: (1) 400 mg of amoxicillin (Augmentin), by mouth, with comfort measures, and return to the clinic in the morning; (2) 2 g of ceftriaxone by injection, with comfort measures, and return to the clinic in the morning; (3) or hospitalize via emergency department for intravenous fluids and antibiotics. The treatment that was chosen not only takes into account the disease process, but also the impact of this choice on the family. A model for the progression of dental caries in low-income groups with recommendations for prevention is also presented.
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6/54. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.

    BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.
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7/54. Abdominal, scrotal, and thoracic calcifications owing to healed meconium peritonitis.

    meconium peritonitis is a form of chemical peritonitis resulting from in utero perforation of the gastrointestinal tract, with subsequent leakage of sterile meconium into the peritoneal cavity and the potential spaces connected with it. Involvement of the tunica vaginalis may be the sole presenting clinical manifestation of the gut perforation resolving spontaneously. In such instances, radiologically detectable calcifications in the abdomen, scrotum, and thorax are essential diagnostic points. In this study, a 4-month-old baby with abdominal, scrotal, and thoracic calcifications owing to healed meconium peritonitis is presented.
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8/54. Rare presentation of small bowel leiomyosarcoma with liver metastases.

    Intraabdominal sarcomas are rare tumours usually diagnosed at an advanced stage. These lesions at presentation are bulky and symptoms are often related to pressure effects on adjacent organs. This case report describes a rare presentation of a small bowel leiomyosarcoma whose initial presentation was free haemorrhage into the abdominal cavity and concomitant liver metastases. This case report also demonstrates that, even with such a rare presentation, an aggressive surgical approach is indicated in this type of tumour and helps a patient with advanced disease to live a few disease-free months with a good quality of life.
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9/54. Prolonged remission of recurrent, metastatic placental site trophoblastic tumor after chemotherapy.

    BACKGROUND: Placental site trophoblastic tumor (PSTT) is a form of gestational trophoblastic neoplasm that is frequently resistant to chemotherapy. In most cases disease is confined to the uterus and can be cured by curettage or simple hysterectomy. patients with metastases, however, frequently have progression of disease and die despite aggressive multiagent chemotherapy. CASE: A 31-year-old woman was found on review of uterine curettings to have a PSTT. Imaging studies revealed multiple lung lesions, a liver lesion, and an enlarged irregular uterus. hysterectomy and staging surgery revealed a large tumor in the endometrial cavity and multiple metastases. She was treated with etoposide-methotrexate-dactinomycin and cyclophosphamide-vincristine and had a complete clinical remission. Six months later, however, she had a recurrence. She was then treated with six cycles of etoposide-methotrexate-dactinomycin and etoposide-cisplatin. Three years after completion of the second regimen she is without evidence of disease. CONCLUSION: Treatment with multiagent chemotherapy can produce long-term remission, even in patients with recurrent, metastatic PSTT. Addition of platinum may be helpful in patients who have recurred or progressed after treatment with non-platinum-containing regimens.
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10/54. Does amniotomy influence the prognosis of babies in cases with severe chorioamnionitis? Report of a twin pregnancy with varying outcome.

    We report our experience in a woman with a twin pregnancy. The patient suffered severe escherichia coli chorioamnionitis and the outcomes were different between the two babies after birth. The first baby had only a mild infection, but the second suffered sepsis and subsequent perinatal death. These differences in outcome appeared to be due to amniotomy performed for the first baby after late labor stage I to augment uterus contractions. Removal of infectious amniotic fluid from the amniotic cavity might thus have prevented the spread of the chorioamnionitis. E. coli sometimes causes severe infection during pregnancy and the perinatal period. In this case, a large number of enteropathogenic E. coli (serotype O-6) was cultured from blood, stool, pharyngeal swab, gastric juice and puncture fluid from the thoracic cavity of the second baby. O-6 is classified an enterotoxigenic strain mainly causing diarrhea because of endotoxin released from bacteria. O-6 has not hitherto been reported as a cause of severe infection in chorioamnionitis and perinatal sepsis.
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