Five cases of patients aged between 2 years 8 months and 5 years 6 months with subcutaneous nodular granuloma annulare are reported. Histologically the lesions resembled rheumatoid nodules, consisting of acellular central areas surrounded by palisading histiocytes. Complete or partial excisions were done for diagnosis. Initially the granulomata were not associated with any symptoms of systemic illness, but one patient with IgG deficiency developed subsequent polyarthritis. Antistreptolysin O, antinuclear antibodies and latex fixation test for rheumatoid arthritis were negative except for one patient with additional erythema nodosum and elevated antistreptolysin level. In the other patients the laboratory data were uncharacteristic. The clinical course may be characterized by spontaneous regression and frequent recurrence. In asymptomatic patients further treatment is not necessary. Patients with progressive disease or elevated inflammatory activity were treated with nonsteroidal antirheumatics. The development of subsequent rheumatoid disease in primarily asymptomatic patients is unlikely, but we recommend further observation by an experienced pediatrician.
Background: To date, no parameter with satisfactory accuracy exists for the diagnosis of appendicitis. This retrospective study describes the discriminatory value of preoperative hematologic factors associated with complicated and non-complicated pediatric appendicitis. Methods: Clinical and laboratory data were obtained from 294 children diagnosed with appendicitis on admission and treated at our tertiary-level pediatric hospital from 2015 to 2017; they were divided into three groups: control group (n Z 118), histologically proven complicated (n Z 120), and non-complicated (n Z 56) appendicitis. Results: Complicated appendicitis was associated with male preponderance and elevated neutrophil and monocyte levels (all p < 0.001). Non-complicated appendicitis was associated with elevated eosinophil levels (p Z 0.023), and unaltered lymphocyte levels (p Z 0.30). Compared to non-complicated disease, the lymphocyte-to-monocyte ratio (LMR) was decreased in complicated appendicitis (p Z 0.003) but unaltered in the control group (p Z 0.38). In the discrimination analysis, LMR had high accuracy (AUC 0.73 AE 0.05; p < 0.001; odds ratio (OR) (95% confidence interval (CI)) 6.0 (2.4e15.3)) and was the only parameter independently associated with complicated appendicitis on regression analysis (OR (95% CI), 0.544 (0.359e0.825); p Z 0.004).
Conclusion:We identified LMR as a novel potential marker for the differentiation of complicated from non-complicated pediatric appendicitis. This has implications on the treatment approach, either surgical in complicated disease or conservative in non-complicated disease.
Pheochromocytomas are frequently associated with inherited cancer syndromes such as von Hippel-Lindau disease (VHL). Retinal angioma and hemangioblastomas of the central nervous system are hallmarks of VHL, but its clinical variety is remarkably broad. Pheochromocytomas as the sole or first manifestation of VHL are rare but have been observed. In this case report, the authors describe an unusual case of initial collapse, seizures, and hypertensive crisis in a child who later was found to have multiple extraadrenal pheochromocytomas. Molecular diagnostics revealed a novel point mutation in the VHL gene (VHL nt. 406 T-->G). Only 7 months after the first lesions had been removed, a new paraganglioma developed in the contralateral periadrenal region. When encountering pheochromocytomas in children, the clinician should be aware that an associated tumor syndrome might be present, and appropriate molecular screening should be initiated. Molecular genetics aid in the clinical decision-making and clinical management of individual patients with pheochromocytoma.
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