Another reason for difficult pulmonary artery catheterization.
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Aged↗
Biomedical subjects
J F Jimenez
Publications and source records attributed to J F Jimenez.
Arteriovenous malformation of the spermatic cord.
We report a case of an arteriovenous malformation of the spermatic cord, a lesion that to our knowledge has not been described previously.
Arteriovenous Malformations↗
Perinatal nonimmune hydrops: diagnostic ultrasonography and related aspects of management.
Nonimmune hydrops fetalis may become the commonest form of hydrops seen in Western countries during the perinatal period, and it has at least a 50% mortality. This report describes five infants with nonimmune hydrops associated with maternal hydramnios and with congenital fetal lesions or disorders, ie, mediastinal teratoma, pulmonary leiomyosarcoma, Beckwith-Weidemann syndrome with omphalocele, fetal tachycardia, and Down's syndrome. Three of the infants survived the neonatal period and two of these underwent surgery for resection of their tumors early in the neonatal period. The third had an omphalocele repaired at 6 hours of age. The literature is reviewed with respect to the pathophysiology of nonimmune hydrops. Its diagnosis and treatment are discussed, with special emphasis on the role of ultrasound in its early diagnosis and optimal prenatal and postnatal management, and on the morbidity seen in survivors.
Female↗
Fetal teratomas: antenatal diagnosis and clinical management.
Four cases of fetal teratomas arising from the sacrococcygeal and oropharyngeal areas are reported. The diagnosis, method of management, and grade of tumor are compared with previous experience in the literature. Pharyngeal lesions carry a worse prognosis for survival compared to the more common sacrococcygeal type (SCT). Atraumatic delivery and early repair of the lesion contribute to prolonged survival and lack of complications in cases of sacrococcygeal teratomas.
Adult↗
The ultrasonographic diagnosis of typhlitis (neutropenic colitis).
Typhlitis is a necrotizing inflammatory disease of the cecum, usually with secondary infection. It is most often found in acute leukemia patients on chemotherapy but has also been reported in other patients on chemotherapeutic drugs. Diagnostic features of typhlitis have been reported on plain radiographs, barium enema, angiography, CT, and one other reported case with ultrasound. We report three cases of typhlitis with a characteristic echogenic thickening of the mucosa on ultrasound. The sonographic findings in the one previous report were identical to those of our three cases. We believe that the sonographic findings of typhlitis are unique and that ultrasound offers an easy noninvasive method of diagnosing this potentially lethal disease.
Adolescent↗
Isochromosome 12p mosaicism (Pallister mosaic aneuploidy or Pallister-Killian syndrome): report of 11 cases.
We report on 11 cases of isochromosome 12p mosaicism (or Pallister mosaic aneuploidy syndrome) in which the isochromosome is usually absent in cultured lymphocytes but present in fibroblasts. The patients range in age from a 22-week-gestation fetus to a 45-year-old man. They have a distinct pattern of anomalies which enables one to make a diagnosis based on clinical manifestations alone. Craniofacial manifestations include "coarse" face with prominent forehead, sparsity of scalp hair, hypertelorism, epicanthal folds, flat bridge of nose, and highly arched palate. Affected newborn infants are profoundly hypotonic with sparsity of scalp hair especially bitemporally and a prominent forehead. Most have accessory nipples. Birthweight and growth parameters are usually normal; however, some newborn infants are unusually large. In infancy, the facial appearance becomes "coarse," hypotonia persists, and seizures may occur. As adults, growth may be normal, scalp hair is thicker and the mandible becomes prominent. Most have a generalized pigmentary dysplasia which may be evident with a Wood's lamp only. All cases have been sporadic and there is no consistent pattern of advanced parental age.
Abnormalities, Multiple↗
Pulmonary blastoma in childhood.
Pulmonary blastoma (PB) has been considered a very rare primary lung malignant neoplasm. In children, it is one of the rarest cancers reported. To my knowledge, there are only 24 pediatric cases of PB in patients ranging from 2 months to 15 years of age, representing one-fourth of all reported cases in the literature. The association of PB with an underlying abnormal pulmonary morphogenesis, characterized by "cystic lesions," suggests that there is a teratogenic-oncogenic relationship. Review of the literature and the clinical and pathologic features of PB in a 2-1/2-year-old white female are discussed.
Child↗
Cystosarcoma phylloides in the adolescent female.
Between 1979 and 1985, five adolescent females have undergone excision of cystosarcoma phylloides. Mean age at presentation was 15 years with a range of 13 to 18 years. Clinical characteristics unique to this diagnosis included a recent history of rapid growth and the large size of the breast mass (mean diameter 7 cm) at initial presentation. Each patient underwent "cosmetic" excisional biopsy utilizing either a circumareolar or inframammary approach. In four patients, the neoplasm was benign with no postoperative recurrence (mean follow-up 33.8 months). The remaining lesion was malignant (liposarcoma) and subsequent simple mastectomy was performed. There was no evidence of disease at 35 months.
Adolescent↗
Diabetes insipidus in children with brain death.
Central diabetes insipidus (DI) occurs in patients suffering from overwhelming CNS injuries leading to brain death. The purpose of this study was to describe the clinical presentation of DI in children with brain death. The medical records of 34 patients with a diagnosis of brain death were reviewed. Diuresis was present in 76% of patients; however, DI was only present in 38% of patients. On autopsy, the pituitary gland in six patients revealed varying degrees of edema, congestion, hemorrhage, and coagulative necrosis. We concluded that DI is present in many, but not all, patients who experience brain death and therefore, cannot be relied on solely as a marker of brain death.
Adolescent↗
Chromosome abnormalities in familial hemophagocytic lymphohistiocytosis.
Familial hemophagocytic lymphohistiocytosis (FHLH) is an uncommon disorder characterized by multiorgan infiltration with phagocytic histiocytes/macrophages. It may be inherited as an autosomal recessive trait, but specific associated cytogenetic abnormalities have not been documented. The authors describe a 10-week-old white female without prior family history of FHLH, who fulfilled the histologic and clinical criteria for the diagnosis. In addition, cytogenetic abnormalities, including the presence of double minute chromosomes and occasional loss of chromosomes 7 and 12, were documented in unstimulated peripheral blood cells. These karyotypic findings are usually associated with dyserythropoietic and leukemic states, and have not been described previously in the context of FHLH. It may be useful to do chromosome analyses on unstimulated peripheral blood cultures from FHLH patients before treatment to examine the karyotype of proliferating cells, which may represent the infiltrative histiocytes seen in the disorder.
Chromosome Aberrations↗
Liposarcoma arising within a cystosarcoma phyllodes.
A liposarcoma arising within a cystosarcoma phyllodes in a 17-year-old black female was treated by total mastectomy without the need of radiation and/or chemotherapy. The patient is alive and well with no evidence of recurrence or metastasis at 20 months recent follow-up. The clinicopathological features of malignant cystosarcoma phyllodes-liposarcoma are discussed.
Adolescent↗
Spontaneous breast infarction associated with pregnancy presenting as a palpable mass.
Spontaneous infarction of physiologically hyperplastic breast tissue or of an adenoma-fibroadenoma presenting as breast mass during pregnancy or in the early postpartum period is a recognized but rare clinical pathologic entity. Only 24 such cases have been reported in the English language literature to our knowledge. We report four additional cases of breast infarction associated with pregnancy, review the literature, and discuss possible etiologies and pathogenesis of this uncommon event.
Adolescent↗
Lipoblastoma in infancy and childhood.
Lipoblastoma is a rare benign neoplasm of fetal-embryonal fat tissue with a 14% tendency to recur. It occurs almost exclusively in infants and children. To my knowledge, less than 100 cases, including children and adults, have been reported in the English literature. Two retroperitoneal and one case of mesenteric lipoblastoma, all presenting as a large palpable abdominal mass, are described. Our patients, aged 12 years, 7 months, and 11 months, were treated by total surgical excision of the tumors. They are alive and well 5, 4, and 3 years postoperative, respectively, with no evidence of recurrence and no indication for any adjunctive therapy. Pathologic evaluation was decisive for the best treatment and prognosis.
Abdominal Neoplasms↗
Monoclonal antibodies for detection of lymphocyte markers: comparison of three commercial kits.
Three commercial monoclonal antibody kits (Histoset, OrthoDiagnostics, Raritan, NJ; T Cell Panel, Becton Dickinson, Mountain View, CA; Histotag Lymphoma Set, Hybritech Inc., San Diego, CA) were compared, using touch preparations, frozen sections, and formalin-fixed paraffin-embedded sections of 14 tonsils. With the first two kits, sensitive results were obtained in touch preparations and frozen sections with strong, crisp, reproducible immunostain. Results were accurate and easily interpreted despite moderate background staining. Distribution and numbers of lymphocytes and subsets were appropriate. The Histotag Lymphoma Set was sensitive for detection of T-cells in touch preparations and frozen sections. Hazy, moderate immunostain was obtained with B-cell and common leukocyte antibodies. Accuracy was questioned because of variable and false-negative results (75% in touch preparations) with all anti-sera, and interfollicular staining for B-lymphocytes. In formalin-fixed sections, positive results of low sensitivity were obtained only with the T Cell Panel. The Histoset and T Cell Panel are recommended, particularly for use with frozen sections.
Antibodies, Monoclonal↗
Primary bronchopulmonary leiomyosarcoma in childhood.
There have been only nine cases of primary bronchopulmonary leiomyosarcoma (PBPL) in children reported in the English language literature, to our knowledge. Herein, we report the tenth case of PBPL in a 24-hour-old newborn infant with associated nonimmunologic hydrops fetalis. We believe this to be the youngest patient ever reported to have PBPL, and the first such tumor, to our knowledge, to be a possible cause of nonimmunologic hydrops fetalis. The patient was alive and well 34 months after a right-sided lower lobectomy, without the need for radiotherapy and/or chemotherapy. We review the clinical pathologic features, treatment, and follow-up of PBPL.
Edema↗
Canine rhinosporidiosis in Arkansas.
Rhinosporidiosis is a fungal infection caused by Rhinosporidium seeberi, a microorganism of complicated, undetermined life cycle. Many species of animals are susceptible to rhinosporidiosis, including human beings. The frequency of occurrence of the disease in domestic animals is unknown. A one-year-old male Collie dog from Northeast Arkansas was found to have rhinosporidiosis presenting as an intranasal polypoid mass. We believe this is the first documented report of rhinosporidiosis from the State of Arkansas.
Animals↗
Spurious elevated platelet counts associated with bacteremia.
Spuriously elevated automated platelet counts secondary to in vivo bacteremia have not been reported previously. Two patients are described with blood cultures positive for Escherichia coli and Klebsiella pneumoniae, respectively, and bacteria present on peripheral blood smear. Those bacteria caused falsely elevated platelet counts to be generated by the Ortho ELT-8. These cases illustrate an unusual artifact and demonstrate that spurious counts can be generated by laser optical blood cell counters.
Adult↗
Acute cytoreduction techniques in the early treatment of hyperleukocytosis associated with childhood hematologic malignancies.
Early and effective cytoreduction for high peripheral white blood cell counts in pediatric patients with acute leukemia may be helpful in preventing complications secondary to hyperviscosity. It also may be a useful adjunct to systemic chemotherapy. As an alternative to automated apheresis for this purpose, manual exchange transfusion is efficacious and does not require hemapheresis instrumentation and disposables and the related special staff. Two patients, a neonate with acute myeloblastic leukemia and a white blood cell count of 422.2 k/microliter as well as a 2 1/2-year-old with an admission diagnosis of acute promyelocytic leukemia and a white blood cell count of 617.4 k/microliter, underwent manual exchange hemotherapy for acute cytoreduction. The procedures were tolerated well, and significant leukocyte removal was achieved, with the respective leukocyte reductions being 81.1 and 68.7%. The techniques available for pediatric cytoreduction are compared, with emphasis on their efficiency and safety and appropriateness for very small children.
Acute Disease↗