Objective: To present a case of a 6-month-old infant with melanotic neuroectodermal tumor of infancy (MNTI) in the upper arm. Clinical Presentation and Intervention: A 6-month-old female presented with a well-circumscribed lesion of the upper arm at the Children's Hospital Zagreb. A biopsy was performed and microscopy revealed 2 cell populations consisting of small neuroblastic cells and larger melanin-containing epithelial cells. An excisional biopsy performed 1 month later confirmed the initial diagnosis of MNTI, but the tumor had increased in size since the initial biopsy. After complete surgical excision the patient recovered well with no recurrence. Conclusion: The MNTI located in the upper arm was diagnosed on first biopsy and surgically excised completely. The patient recovered without recurrence in a follow-up of 2.5 years.
SUMMARY -Th e aim of the study was to assess diagnostic accuracy (sensitivity and specifi city) of Fenyö-Lindberg and Teicher scores for distinguishing patients that need immediate surgical treatment from the others, in a female population from an urban setting. Th e study prospectively included 130 female patients admitted to the emergency department with abdominal pain indicating acute appendicitis. Th e scores and parameters of validity were calculated and compared to defi nitive diagnosis. For Fenyö-Lindberg score of -17 or less, 84.5% sensitivity, 55.6% specifi city, 87.9% positive predictive value (PPV) and 48.4% negative predictive value (NPV) were recorded. For cut-off value greater or equal to -2, there was 59.2% sensitivity, 77.8% specifi city, 91% PPV and 33.3% NPV. Th e Receiver Operating Characteristic (ROC) curve analysis of Fenyö-Lindberg score showed that the best single cut-off value for discriminating acute appendicitis in the study population was -15. For Teicher score, values greater than -3 yielded 89.3% sensitivity and 22.2% specifi city, 81.4% PPV and 35.3% NPV. In conclusion, Fenyö-Lindberg score could be used as an additional tool to exclude appendicitis and avoid unnecessary appendectomies. Teicher score may help in recognizing patients with appendicitis. None of the two scores can indicate or decline appendectomy in all cases. Scoring systems may be useful for pointing to important clinical signs and symptoms in specifi c subpopulations.
was considered (on admission MELD score 24, King's Wilson index 12; on day 10 MELD score 18, King's Wilson index 8).The decision was made to attempt plasma exchange over three days and introduce penicillamine. The patient improved clinically, tolerated penicillamine well and was released subsequently after 34 days of hospitalization and five plasma exchanges overall. Liver synthetic function completely normalized few months later. Genetic analysis of ATP7B gene (H1069Q mutation) confirmed presence of the H1069Q mutation on one allele.We presented a patient suffering from WD in whom the disease presented acutely with development of liver failure with near-normal values of liver enzymes, mild hyperbilirubinemia, markedly impaired synthetic liver function and Coombs-negative haemolytic anaemia. Acute liver failure in WD may be successfully managed with plasma exchange therapy.
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