Quantitative histology and other perversions.
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Biomedical subjects
Publications and source records attributed to D R Benjamin.
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Congenital posterolateral diaphragmatic hernia (CDH) is widely regarded as an isolated defect, but this view is incomplete. We reviewed our clinical and autopsy experience and the literature from the past 25 years in order to catalogue the frequency and clinical importance of additional malformations in patients with CDH. The study showed two broad categories of patients. In the larger group of infants (65 of 108, or 60%), CDH was the only severe defect, apart from those normally associated with the presence of abdominal viscera in the thorax. Thirty-six of these patients (55%) survived. A striking finding among infants with isolated CDH, not previously highlighted in the literature, was cryptorchidism which was present in 30% of males. By contrast, 43 patients (40%) had one or more severe extradiaphragmatic malformations; only six of these infants (14%) survived. Defects in morphogenesis were widespread, heterogeneous, and seemingly related to numerous pathogenetic mechanisms. Most often abnormalities involved the heart, brain, genitourinary system, craniofacial region, or limbs. The high incidence of multiple anomalies in some patients with CDH should influence our investigations into causes and mechanisms. Patients with CDH should be evaluated carefully for additional defects--their presence has a significant impact on management and worsens prognosis.
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The authors report a case of recurrent pancreatoblastoma in a 3-year-old girl who was successfully treated with radiotherapy. The patient had two local tumor recurrences before radiation treatment; the second recurrence was managed with radiation alone. Computerized tomography scan at the completion of radiotherapy showed dramatic tumor regression. Six weeks after radiotherapy was finished exploratory laparotomy was performed; multiple biopsies showed no evidence of tumor. The patient is currently disease-free 2 years after completion of radiotherapy. This case suggests a role for radiation therapy in the management of children with pancreatoblastoma.
An unusual benign tumor of skeletal muscle origin is described. The tumor was located in the left retroperitoneum of a newborn female. The tumor contained features of both an adult and fetal rhabdomyoma when studied by light and electron microscopy, immunocytochemistry, and histochemistry. This is the only report of this type of neoplasm in the literature.
Infants with congenital diaphragmatic hernia have significantly increased chest circumferences. This implies that intrathoracic volumes are increased as well. Forces produced by the herniated abdominal viscera seem to provide the chief impetus for this change. Other factors may also contribute, for thoracic enlargement is asymmetric and not always ipsilateral to the hernia. The contribution of an enlarged chest to respiratory insufficiency, persistence of the fetal circulation, and hyperinflation is not fully understood, but may have relevance in evaluating new approaches to therapy.
The spectrum of granulomatous inflammation in peripheral lymph nodes in 85 children over the previous decade was systematically investigated. A variety of histopathologic features were examined and correlated with the epidemiologic, clinical, and microbiologic information, in addition to the long-term outcome. Sixty children had head or neck involvement, with atypical mycobacteria accounting for the vast majority of confirmed causes. It was most frequent in cervical and submandibular glands of children aged between 1 and 10 years (35 of 44 patients), peaking between age 2 and 5 years, and was more common in girls (male:female ratio, 1:3). Mycobacterium avium-intracellulare was the predominant pathogen, accounting for 75% of the mycobacteria that could be characterized. Granulomatous inflammation in other sites (axilla and upper extremity, 16 patients; inguinal, nine patients), or older than age 10 years rarely yielded a cause. Such patients had no recurrent or persistent clinical problems following surgical excision. Histopathologic features were extremely variable, and no consistent patterns were discerned that could reliably be related to either cause or time course. The clinical features and bacteriologic investigations were the most useful for establishing a diagnosis.
Two cases of mesenchymal hamartoma of chest wall in infancy are reported. These distinctive and rare lesions arise in the antenatal period, present at birth or in early life as chest wall masses with marked rib deformation, and may produce respiratory compromise through encroachment on the chest cavities. Histologically composed of chondroid and primitive mesenchymal elements with giant-cell formation, endochondral ossification, and maturation to trabecular bone, they exhibit some features of aneurysmal bone cyst. Because of their cellularity and proliferative appearance, they are often misinterpreted as sarcomas; however, they pursue a benign course and can be well managed surgically. Our cases possessed multiple chest wall masses that were clinically apparent at birth and sequentially resected over a period of several months, creating an opportunity to document their hitherto unreported histologic evolution. Their pattern of maturation is in keeping with a hamartomous malformation.
C-reactive protein (CRP) was evaluated in both serum and cerebrospinal fluid in 119 patients to determine if either or both measurements were of clinical value in the diagnosis of bacterial meningitis. CSF C-reactive protein is too insensitive (sensitivity = 66%) to be useful, while serum CRP is too nonspecific for routine application. Serum CRP may have a role if used selectively in those patients with a low-grade CSF pleocytosis and a negative Gram's stain.
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Two patients who presented with bone marrow necrosis and eventually developed acute lymphocytic leukemia are reported, and similar cases in the literature are reviewed. Both patients responded to chemotherapy. Several possible mechanisms are discussed. Bone marrow necrosis appears to be another condition preceding acute lymphocytic leukemia in children.
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Hepatobiliary dysfunction is a well recognized complication in infants and children on long-term total parenteral nutrition. This clinical-pathological study of fifteen patients with this syndrome suggests that cholestasis is the primary pathogenetic mechanism. The cause of the cholestasis is not well understood, but does not appear to be primarily related to the type of intravenous fluids or the occurrence of sepsis. It is suggested that the prolonged fasting results in disruption of the normal gastro-intestinal mechanisms responsible for bile production and flow. This is supported by the pathological findings, the fact that hepatobiliary dysfunction develops late (usually around 2-3 months), the observation that elevated bile acids and direct hyperbilirubinemia occurs prior to any evidence of hepatocellular necrosis and the occurrence of cholelithiasis in some patients.