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ABSTRACT: Adult nesidioblastosis, a rare form of abnormal islet cell proliferation arising from the pancreatic ductal epithelium, is usually associated with severe hyperinsulinemic hypoglycemia. Overall, seventy-three cases of nesidioblastosis have been described in the English literature since the entity was first described by Laidlaw in 1938, and only a minute fraction of these have occurred in children.
We present the case of a previously healthy 45-year-old woman with new-onset severe hypoglycemia 4 and seizures. The differential diagnosis at presentation included factitious hypoglycemia and insulinoma. Extensive imaging and laboratory examination, including repeated CT and MRI scans, toxicology assays, and insulin-based chemical studies, were either inconclusive or negative. Subsequent testing involved stimulation of the pancreas through cannulation of the pancreatic vascular supply by interventional radiology. This testing revealed marked insulin release to low-level calcium challenge across multiple pancreatic segments. Based on these functional radiological findings, the patient underwent subtotal pancreatectomy. Gross and histologic examination of the resected tissue revealed no evidence of a pancreatic mass. Diffuse islet cell hyperplasia was noted in a pattern consistent with nesidioblastosis. The patient remained normoglycemic in the months following partial pancreatectomy.
Nesidioblastosis, while exceedingly rare in adult populations, should be considered in the differential diagnosis of severe hypoglycemia. This diagnosis cannot be easily made through routine diagnostic laboratory or radiological procedures and likely requires a histological tissue diagnosis.
JOP: Journal of the pancreas 01/2008; 9(4):504-11.
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ABSTRACT: Abstract
Background
To evaluate the effectiveness and safety of the support given to women by a companion of their choice during labor and delivery.
Methods
A total of 212 primiparous women were enrolled in a randomized controlled clinical trial carried out between February 2004 and March 2005. One hundred and five women were allocated to the group in which support was permitted and 107 to the group in which there was no support. Variables regarding patient satisfaction and events related to obstetrical care, neonatal results and breastfeeding were evaluated. Student's t-test or Wilcoxon's test, chi-square or Fisher's exact test, risk ratios, and their respective 95% confidence intervals were used in the statistical analysis.
Results
Overall, the women in the support group were more satisfied with labor (median 88.0 versus 76.0, p < 0.0001) and delivery (median 91.4 versus 77.1, p < 0.0001). During labor, patient satisfaction was associated with the presence of a companion (RR 8.06; 95%CI: 4.84 – 13.43), with care received (RR 1.11; 95%CI: 1.01 – 1.22) and with medical guidance (RR 1.14 95%CI: 1.01 – 1.28). During delivery, satisfaction was associated with having a companion (RR 5.57, 95%CI: 3.70 – 8.38), with care received (RR 1.11 95%CI: 1.01 – 1.22) and with vaginal delivery (RR 1.33 95%CI:1.02 – 1.74). The only factor that was significantly lower in the support group was the occurrence of meconium-stained amniotic fluid (RR 0.51; 95%CI: 0.28 – 0.94). There was no statistically significant difference between the two groups with respect to any of the other variables.
Conclusion
The presence of a companion of the woman's choice had a positive influence on her satisfaction with the birth process and did not interfere with other events and interventions, with neonatal outcome or breastfeeding.
Reproductive Health. 01/2007;
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ABSTRACT: Nasopharyngeal adenocarcinomas (NPACs) are uncommon neoplasms with a diverse histomorphology and clinical behavior. The purpose of our study was to better understand the clinicopathologic characteristics of NPACs and to identify the histologic and immunohistochemical features that distinguish the subtypes of papillary NPACs. We conducted a retrospective review of 44 cases of NPACs accessioned between 1985 and 2000. We obtained follow-up information for all patients. We identified 24 females and 20 males, ranging in age from 9 to 74 years (median, 50 years). There were 28 salivary gland type, 13 conventional low-grade papillary NPACs of surface origin, and 3 metastatic adenocarcinomas, 2 thyroid and 1 lung. We performed immunohistochemical studies in 18 papillary NPACs. Two of the low-grade papillary NPACs simulated thyroid carcinoma; they expressed CK7, CK19, and TTF-1 but were negative for thyroglobulin. Polymorphous low-grade papillary NPACs expressed diffuse reactivity to S-100, whereas low-grade papillary NPACs were negative or focally positive. All patients with low-grade NPACs were alive at 5 to 20 years. In contrast, 18 (64.2%) of the 28 patients with salivary gland-type NPACs had died of the disease or were living with disease at follow-up. Primary NPACs can be classified by their morphology and clinical behavior into 2 groups: surface origin type and salivary gland type. Papillary NPACs can be identified by their histology and selective immunohistochemical expression. Pathologist should distinguish the different types of NPACs because their treatment and prognosis differ.
Annals of Diagnostic Pathology 09/2006; 10(4):215-21. · 0.88 Impact Factor
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ABSTRACT: Erdheim-Chester disease is a very rare xanthogranulomatous, non-Langerhans cell systemic histiocytosis with an unknown etiology and pathogenesis. Histologically, it is characterized by a diffuse infiltration with large, foamy histiocytes, rare Touton-like giant cells, lymphocytic aggregates, and fibrosis. The histiocytes differ from the Langerhans cell group in ontogenesis, immunohistochemistry (positive for CD68 and negative for CD1a and S100 protein), and ultrastructural appearance (lack of Birbeck granules). Although most of the cases have symmetric osteosclerosis of the long bones, an involvement of the axial skeleton has also been described. Extraskeletal lesions are present in more than 50% of the patients and may involve the retroperitoneal space, lungs, kidneys, brain, retro-orbital space, and heart. This study presents the case of a patient with Erdheim-Chester disease with vertebral destruction and, for the first time, to our knowledge, involvement of the liver. The diagnosis is based on radiologic, histologic, immunohistochemical, and ultrastructural findings.
Archives of pathology & laboratory medicine 09/2003; 127(8):e337-9. · 2.58 Impact Factor
