Neurocognitive outcome in survivors of childhood acute lymphoblastic leukemia: experience at a tertiary care hospital in Korea.

This study was conducted to investigate long-term neurocognitive outcomes and to determine associated risk factors in a cohort of Korean survivors of childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL were compared with 42 healthy controls on measures of a neurocognitive test battery. We analysed potential risk factors (cranial irradiation, sex, age at diagnosis, elapsed time from diagnosis, and ALL risk group) on neurocognitive outcomes. ALL patients had lower, but non-significant full-scale intelligence quotient (FSIQ, 107.2±12.2 vs. 111.7±10.2), verbal intelligence quotient (VIQ, 107.7±13.6 vs. 112.2±11.4), and performance intelligence quotient (PIQ, 106.3±14.2 vs. 110.1±10.7) scores than healthy controls. However, patients treated with cranial irradiation performed significantly lower on FSIQ (102.2±8.1), VIQ (103.3±11.7), and PIQ (101.4±13.2) compared to non-irradiated patients and healthy controls. ALL patients also had poor attention, concentration, and executive functions. Among ALL survivors, cranial irradiation was a risk factor for poor FSIQ, being male was a risk factor for poor PIQ, and younger age was a risk factor for poor attention. Therefore, the delayed cognitive effects of ALL treatment and its impact on quality of life require continuing monitoring and management.

Articulation error of children with adenoid hypertrophy.

PURPOSE: Adenoid hypertrophy is a physical alteration that may affect speech, and a speech disorder can have other negative effects on a child's life. Airway obstruction leads to constricted oral breathing and causes postural alterations of several oro-facial structures, including the mouth, tongue, and hyoid bone. The postural modifications may affect several aspects of speech production. METHODS: In this study, we compared articulation errors in 19 children with adenoid hypertrophy (subject group) to those of 33 children with functional articulation disorders independent of anatomical problems (control group). RESULTS: The mean age of the subject group was significantly higher (P=0.016). Substitution was more frequent in the subject group (P=0.003; odds ratio [OR], 1.80; 95% confidence interval [CI], 1.23-2.62), while omission was less frequent (P<0.001; OR, 0.43; 95% CI, 0.27-0.67). Articulation errors were significantly less frequent in the palatal affricative in the subject group (P=0.047; OR, 0.25; 95% CI, 0.07-0.92). The number of articulation errors in other consonants was not different between the two groups. Nasalization and aspiration were significantly more frequent in the subject group (P=0.007 and 0.014; OR, 14.77 and 0.014; 95% CI, [1.62-135.04] and NA, respectively). Otherwise, there were no differences between the two groups. CONCLUSION: We identified the characteristics of articulation errors in children with adenoid hypertrophy, but our data did not show the relationship between adenoid hypertrophy and oral motor function that has been observed in previous studies. The association between adenoid hypertrophy and oral motor function remains doubtful.

Aggressive systemic mastocytosis after germ cell tumor of the ovary: C-KIT mutation documentation in both disease states.

We report a case of aggressive systemic mastocytosis in a 3-year-old girl, who had undergone treatment for ovarian germ cell tumor during the previous 8 months. On diagnosis of systemic mastocytosis, she was treated with interferon-alpha and steroids. She showed tolerable side effects of interferon-alpha infusion, but died of multiple organ failure after 2 months of treatment. Point mutations of the C-KIT gene, previously implicated in the genesis of mastocytosis, were discovered not only in the bone marrow and the peripheral blood of the patient, but also in the tissue of the previously diagnosed germ cell tumor as well.

Monokine levels in cancer and infection.

The levels of monocyte intracellular monokines (TNFalpha, MIP, and MIG) in patients with cancer or bacterial infection were studied by multiparameter flow cytometry and comparative fluorescence analysis. TNFalpha, MIP, and MIG levels in peripheral blood of patients with cancer or bacterial infection were higher than in normal controls (p < 0.005). In normal controls, no significant relationships were found among TNFalpha, MIG, MIP levels, monocyte count, and lymphocyte count in peripheral blood. In cancer patients, TNFalpha was strongly related to MIP (r = 0.809, p < 0.001) and MIG (r = 0.773, p < 0.001). Of the 3 monokines, TNFalpha and MIG levels were related to monocyte count, but none showed correlation with lymphocyte count in cancer patients. In patients with bacterial infection, TNFalpha was not significantly related to MIP (r = 0.423, p = 0.051), but it was related to MIG (r = 0.457; p = 0.033). None of the monokines (TNFalpha, MIP, MIG) was related to the monocyte count, but the MIP level was related to the peripheral blood lymphocyte count in patients with bacterial infection (r = 0.559, p = 0.008). These results suggest that circulating monocytes may play an important role in both cancer and bacterial infection through increased production of monokines. Moreover, correlations of the monokine levels with each other and their relationships to the monocyte count differ in patients with cancer and bacterial infection.