Ganglioneuroma in a child with hereditary spherocytosis

Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. Extramedullary hematopoiesis (EMH) mimicking a mass may develop in the lymph nodes, kidneys, pleura, mediastinum, adrenal gland, and in particular the spleen and liver. Other than EMH, B-cell lymphoma, acute lymphoblastic leukemia, and pancreatic schwannoma cases were reported in patients with HS. We present a 13-year-old female patient with HS and ganglioneuroma in the adrenal gland. This association is probably coincidental; however, with increasing cancer cases in HS and the genetic studies being made, this association will be clarified.     

A rare case of asymptomatic bilateral submandibular gland sialolithiasis: a giant, fistulized calculus on the right and multiple calculi on the left

Sialolithiasis is the most common disease of the submandibular gland; sialoliths account for at least 80% of all salivary duct calculi. We present a rare case of asymptomatic bilateral submandibular gland sialoliths. On the right, the patient had a giant (35 × 35 mm) sialolith that had fistulized into the oral cavity. In the left submandibular gland, he had 30 differently sized sialoliths.     

Sugiura procedure in portal hypertensive children

Bleeding from esophageal varices is an important cause of morbidity and mortality in children with portal hypertension. The treatment protocol is planned according to the etiologic factors underlying the portal hypertension, which may be either intrahepatic or extrahepatic. Although portasystemic venous shunt operations were common previously, they are now regarded as nonphysiologic and are rarely used because of their unexpected results and complications. Today, in many centers, endoscopic procedures have become the first-step treatment modality in bleeding esophageal varices. More complicated surgical procedures, such as devascularization procedures in extrahepatic portal hypertension, and liver transplantation in patients with failing liver, should be performed when conservative measures fail. We followed up 69 patients with portal hypertension with endoscopic sclerotherapy in our department. Here we present a retrospective evaluation of the effect of the Sugiura operation on the prognosis of 12 children (6 with extrahepatic and 6 with intrahepatic portal hypertension) who were not responsive to the sclerotherapy program. No rebleeding was seen in 9 of the 12 (75%) patients after the procedure, and the mortality rate in this series was 1 of 12 (8.3%); this patient died of hepatic failure. Received: November 7, 2000 / Accepted: January 25, 2001     

Prognostic value of adenosine stress cardiovascular magnetic resonance in patients with low-risk chest pain

Background

Approximately 5% of patients with an acute coronary syndrome are discharged from the emergency room with an erroneous diagnosis of non-cardiac chest pain. Highly accurate non-invasive stress imaging is valuable for assessment of low-risk chest pain patients to prevent these errors. Adenosine stress cardiovascular magnetic resonance (AS-CMR) is an imaging modality with increasing application. The goal of this study was to evaluate the negative prognostic value of AS-CMR among low-risk acute chest pain patients.

Methods

We studied 103 patients, mean 56.7 ± 12.3 years of age, with chest pain and no electrocardiographic evidence of ischemia and negative cardiac biomarkers of necrosis, who were admitted to the Cardiac Decision Unit of our institution. All patients underwent AS-CMR. A negative AS-CMR was defined as absence of all the following: regional wall motion abnormalities at rest; perfusion defects during stress (adenosine) and rest; and myocardial scar on late gadolinium enhancement images. The patients were followed for a mean of 277 (range 161-462) days. The primary end point was defined as the combination of cardiac death, nonfatal acute myocardial infarction, re-hospitalization for chest pain, obstructive coronary artery disease (>50% coronary stenosis on invasive angiography) and coronary revascularization.

Results

In 14 patients (13.6%), AS-CMR was positive. The remaining 89 patients (86.4%), who had negative AS-CMR, were discharged. No patient with negative AS-CMR reached the primary end-point during follow-up. The negative predictive value of AS-CMR was 100%.

Conclusion

AS-CMR holds promise as a useful tool to rule out significant coronary artery disease in patients with low-risk chest pain. Patients with negative AS-CMR have an excellent short and mid-term prognosis.     

Melanoma outcomes for Medicare patients: association of stage and survival with detection by a dermatologist vs a nondermatologist

OBJECTIVE: To determine whether a difference in melanoma outcomes exists in the United States between tumors detected by dermatologists vs those detected by nondermatologists. DESIGN: Retrospective analysis of linked data from the Medicare enrollment and claims files from the Centers for Medicare and Medicaid Services and the National Cancer Institute's Surveillance, Epidemiology, and End Results program database from 1991 to 1996. The registries are from 12 US sites. PATIENTS: A study sample comprised of 2020 subjects. MAIN OUTCOME MEASURES: Tumor characteristics (Breslow thickness and histologic ulceration), stage at diagnosis, and survival and mortality rates. RESULTS: Tumor detection by a dermatologist vs nondermatologist was associated with an earlier stage melanoma (stage 0, stage I, and stage II vs stage III and stage IV; chi(2) test, P<.01) and a thinner tumor (Breslow thickness, 0.86 mm vs 1.00 mm; P<.05). At all time points (6 months, 2 years, and 5 years), patients whose melanoma was detected by dermatologists had better survival rates (98%, 87%, and 74%, respectively, for those whose melanoma was detected by dermatologists vs 95%, 79%, and 69%, respectively, for nondermatologists; P<.05). Non-cancer-related mortality was similar for the 2 groups, but the patients whose tumors were detected by dermatologists had lower cancer-related mortality (13% vs 21%; P<.01) and overall mortality (29% vs 37%; P<.01). Multivariate analysis showed that age, sex, stage at diagnosis, and melanoma detection by a dermatologist were all significantly predictive of survival. CONCLUSIONS: Earlier stage melanoma and improved survival are associated with detection by a dermatologist rather than by a nondermatologist. Increasing access to dermatologists, particularly for older patients, may represent one approach to improving melanoma-related health outcomes.     

Stress distributions in internal resorption cavities restored with different materials at different root levels: A finite element analysis study

The aim of this study was to evaluate the stresses within simulated roots with internal resorption cavities at the apical, middle and coronal root levels, after obturation with gutta‐percha and/or MTA utilising finite element analysis (FEA). Mandibular premolar teeth with internal resorption cavities at different root levels were modelled. Models were restored with gutta‐percha and/or MTA. An oblique force of 300 N was applied and stress evaluations were carried out. In the MTA‐filled resorption models, the stresses were distributed more homogeneously than the gutta‐percha filled models, and the stress concentrations were lower in the remaining dentinal tissues. If the whole root is considered, the fully gutta‐percha‐filled models generated the highest stress values. Differences between the fully MTA‐filled models and hybrid techniques were present only in the apical resorption models. Both the MTA and combination of MTA and gutta‐percha can be suggested for use in clinical practice, in cases of internal root resorption cavity obturation.