Successful pregnancy and delivery in a patient with chronic myelogenous leukemia (CML), and management of CML with leukapheresis during pregnancy: a case report and review of the literature

Although infrequently seen, the management of cancer during pregnancy can be difficult for patients, their families and physicians. The concomitant occurrence of pregnancy and chronic myelogenous leukemia is uncommon. We describe the successful management of a 26-year-old woman in the first trimester of her pregnancy with chronic myelogenous leukemia (CML) in chronic phase by using only leukapheresis. She was treated with leukapheresis until her delivery at 36 weeks of gestation. The procedure was without significant adverse effects on the patient or fetus. We applied a total of 15 leukapheresis treatments throughout the pregnancy. The patient gave birth vaginally to a healthy 2800 g boy at 36 weeks of gestation. We conclude that leukapheresis may provide an alternative treatment to chemotherapy, alpha-interferon or imatinib in pregnant patients with CML, particularly with concern over their potential teratogenic and other adverse effects.     

Prevalence of celiac disease in Turkish children

BACKGROUND: Celiac disease has emerged as a public health problem, and the disease prevalence varies among different races and nations. The present study was designed to investigate the prevalence of celiac disease in apparently healthy Turkish schoolchildren and to detect children with silent celiac disease. METHODS: Healthy school children, 6 to 17 years of age, which there are 72,000 living in Erzurum were chosen as the study population. A total of 1,489 children were randomly selected by systematic sampling method. Samples were tested for anti- tissue transglutaminase IgA. Parents of the children who had positive test result were informed about the disease, and a small intestinal biopsy was proposed. A pathologist blinded to the serology results examined all biopsy specimens according to the modified Marsh criteria. RESULTS: A total of 1,263 healthy school children were screened for celiac disease. Of subjects, 687 (54.4%) were boys and 576 (45.6%) were girls. Mean age was 11.9+/-3.4 years (range, 6-17 years). None of the patients had IgA deficiency. Of 1,263 children, 11 had positive anti-tissue transglutaminase IgA. Thus, the total seropositivity was 0.87%. Of seropositive children, 6 (54.6%) were boys and 5 (45.4%) were girls. We calculated the prevalence of celiac disease as 1:115. The prevalence of biopsy proven celiac disease was 1:158. CONCLUSIONS: In this first celiac disease prevalence study in Turkey, we found that celiac disease is highly prevalent in healthy schoolchildren. Children with iron deficiency anemia and malnutrition should be evaluated more carefully with the understanding of the high celiac disease prevalence in Turkey.     

Apoptosis of lymphocytes in peripheral blood of patients with melanoma

In cancer, spontaneous apoptosis of circulating peripheral blood lymphocytes (PBLs) is a general phenomenon. In this study we aimed to determine whether spontaneous apoptosis of circulating PBLs of patients with malignant melanoma occurs. Pathologically proven 45 patients with malignant melanoma and 19 healthy controls were included in this study. Samples were obtained both on first admission before treatment, either adjuvant or metastatic, and follow-up period of patients. Human active caspase-3 immunoassay (R&D Systems, Inc. MN, USA) employs the quantitative sandwich enzyme immunoassay technique. A monoclonal specific for caspase-3 has been used. The spontaneously apoptotic PBLs in melanoma patients were not significantly different from those obtained for normal controls (p=0.25). None of the clinical characteristics were significantly correlated with spontaneous apoptosis (p>0.05). Likewise, we found that apoptosis in PBLs was not a prognostic factor in melanoma patients (p=0.79). In conclusion, we did not observe accelerated apoptosis of PBLs in patients with melanoma. Further studies are necessary to determine the potential prognostic importance of this observation.     

Treatment results of chronic hepatitis B in children: a retrospective study

In this retrospective study, we aimed to share our experience with different treatment modalities for chronic hepatitis B in a series of children. The study included 126 children (mean: 9.5 +/- 3.8 years). Normalization of alanine aminotransferase (ALT), loss of hepatitis B virus (HBV)-DNA and hepatitis B e antigen (HBeAg), and development of antibody to HBeAg (anti-HBe) altogether at the end of the treatment was considered as end of therapy response (ETR). Seroconversion ongoing one year after the cessation of therapy was considered as sustained response. Of the total children, 90 (71.4%) were treated, whereas the remaining were just followed-up. High-dose interferon (IFN)-alpha (10 MU/m2) alone, standard-dose IFN-alpha (6 MU/m2) plus lamivudine (4 mg/kg/d), high-dose IFN-alpha plus lamivudine, or lamivudine alone was used, IFN-alpha thrice weekly for six months, and lamivudine daily for one year. Of children who had completed their treatment, 34 (37.8%) achieved ETR. Sustained response rate was 36.7%. Response rates were different in the different treatment groups (p: 0.01). The highest response rate was observed in those who received standard-dose IFN-alpha plus lamivudine treatment (61.5%). Of children without treatment, one (2.8%) had anti-HBe seroconversion. Standard-dose IFN-alpha plus lamivudine treatment was found superior to the other treatment modalities. Predictors of ETR were similar to those found in previous studies.     

Dermatological signs in Wilson's disease

Background:  Because no data on skin and mucosal findings of patients with Wilson's disease have been published so far, the aim of the present study was to investigate the prevalence of mucosal and skin findings in childhood Wilson's disease and to determine its specific dermatological findings, if any exist.
Methods:  Thirty-seven 4–17-year-old children with Wilson's disease were included. A complete skin, scalp skin, mucous membrane and nail examination was performed.
Results:  Of the children, 26 (70.3%) had at least one dermatological finding. Twenty-five (67.6%), five (13.5%), nine (24.3%) had at least one skin, mucosal and nail finding, respectively. The most prevalent dermatological diagnosis of the Wilson's disease patients was xerosis (45.7%). The presence of dermatological findings was not related to drug usage, severity of the disease, or malnutrition. The duration of the disease was not different in patients with or without dermatological findings. The frequency of skin findings alone, however, was high in relatively newly diagnosed patients (<2 years).
Conclusion:  Dermatologist should be aware of the various dermatological manifestations of Wilson's disease, because a careful and objective skin, mucosa, nail and hair examination may be indicative of a diagnosis of Wilson's disease, particularly in early cases.     

First Turkish patient with floating harbor syndrome with additional findings: cryptorchidim and microcephaly

We report the first Turkish patient with Floating Harbor Syndrome (FHS). The 12-year old male patient exhibited classical dysmorphic features of FHS, mental retardation, celiac disease and additional undescribed findings: microcephaly and cryptorchidism.     

Individual risk factors for carpal tunnel syndrome: an evaluation of body mass index, wrist index and hand anthropometric measurements

In this study we aimed to identify the role of the body mass index (BMI), wrist index and hand anthropometric measures as risk factors for carpal tunnel syndrome (CTS) in both genders. Based on clinical and electrophysiologic diagnostic criteria, 154 female and 44 male CTS patients, as well as 150 female and 44 male age-matched control subjects, were selected. BMI, wrist index, hand shape index, digit index and hand length/height ratio were compared between the CTS patients and the control subjects for each gender separately. Mean BMI was found to be a significant risk factor for CTS in both genders. The wrist index was found to be higher in female (P<0.001) and in male (P=0.034) CTS groups than in the respective control groups. Logistic regression analysis revealed the wrist index to be an independent risk factor in females, but not in males. Shape and digit indices were significantly higher in female CTS patients than in corresponding control subjects, and regression analysis showed the shape and digit indices to be independent risk factors for CTS. In the male CTS group, the shape and digit indices did not significantly differ from their controls. Differences in the hand length/height ratio were not statistically significant in female and male CTS patients compared to their controls and it was not found to be an independent risk factor for CTS.

Our study confirmed BMI as an independent risk factor for CTS in both genders. Hand and wrist anthropometrics were found to be independent risk factors for CTS in females, but not in males.