Search

Objective: The aim of this study is to evaluate clinical pharmacist-led medication review service for hospitalized pediatric patients. Material and Methods: This cross-sectional study was carried out between November 2017-April 2018 in an education and research hospital in Istanbul. Clinical pharmacist-led medication review was conducted by using Pharmaceutical Care Network Europe (PCNE) Classification V8.02 in hospitalized pediatric at general pediatric service. Potential drug-related problems (DRPs) were identified and classified. These DRPs were presented the physicians and the percentage of accepted recommendations by the physician were recorded. Results: Among forty-three patients (21 male and 22 female), the median age of them was calculated as 6 (3-36) months. Of them, 25.58% had 16 DRPs. The most common DRPs were associated with potential drug-drug interactions (n= 9); and dose selection (n= 5), which represented 56.25% and 31.25% of drug-related problems, respectively. It was observed that 63% of these problems were occurred during selection of drugs (at prescription level) according to PCNE classification. Of the clinical pharmacist’s recommendations regarding these DRPs, 87.5% were accepted by the physician. Conclusion: To optimize rational drug use, numerous studies related with clinical pharmacist-led medication review by using PCNE classification were present in adult patients; however, there was no sufficient studies conducted in pediatric patients. Clinical pharmacists have an important role in the classification of DRPs and to provide rational drug use in pediatric patients.

Thalassemia major is the most severe form of thalassemia and occurs with the impaired synthesis of β-globin which causes the accumulation of unpaired alpha globin chain. Patients with beta thalassemia major can only survive with periodically safe blood transfusions leading to the accumulation of iron in the bloods of patients, and this causes several endocrinopathies. Although iron overload in thalassemic patients has been extensively studied, there is little information about the levels of other trace elements. The aim of this study was to investigate the differences of serum concentrations of sodium, magnesium, calcium, manganese, iron, copper, and zinc for patients with major β-thalassemia. Concentration of elements was determined by inductively coupled plasma-mass spectrometry system. The statistical difference between the elemental concentrations of the patient and control groups was found by the Mann-Whitney U test. In addition, the relationship between concentrations of the measured elements for each group was determined by the Spearman correlation test. The results revealed that iron, zinc, magnesium, and manganese serum levels of thalassemic patients were significantly higher than the control group while calcium concentration was statistically lower than the control group. There was no significant difference observed for copper and sodium levels of patients when compared to the healthy control group.

Glucose-6-phosphatase catalytic subunit 3 (G6PC3) deficiency is a recently identified form of congenital neutropenia associated with developmental anomalies. The severity of neutropenia and the clinical spectrum are highly variable. Aside from infectious complications and extrahematologic features, inflammatory bowel disease and autoinflammatory complications are less frequently observed manifestations. However, amyloidosis has never been reported in G6PC3 deficiency. Here, we present a 12-year-old patient with incidentally discovered neutropenia because of the p.E65A (c.194A>C) variant of the G6PC3 gene. He had recurrent aphthae and abdominal pain episodes, and developed nephrotic-range proteinuria, amyloidosis, and end-stage renal failure during follow-up.

Objective: Hereditary spherocytosis (HS) is a non-immune hemolytic anemia occurring with anemia, jaundice, splenomegaly symptoms in which the cell membrane of the erythrocytes is transformed into the shape of spherocytes due to congenital membrane protein defects. In this study, the demographic characteristics, clinical and laboratory findings, as well as complications during the follow up of our patients with HS are presented. Methodology: All patients who were diagnosed with hereditary spherocytosis and followed in our pediatric hematology clinic between 2000 and 2021 years were included in the study. Gender, age consanguinity of the parents, family history of HS and splenectomy, the neonatal phototherapy history were retrospectively recorded from patients’ files. The complaints, physical examination findings, and laboratory findings at the first admission were evaluated. Duration of follow-up, transfusion frequency,splenectomy requirement, and response to splenectomy were also recorded. Results: Sixty-seven patients (41 male, 27 female) were religible for the study. The median age of diagnosis was 3 years (range 18 day-15 years). Consanguineous marriage rate was 29.9% whereas 62.7% of the patients had a family history of HS. Neonatal hyperbilirubinemia was present in 67.1% of the patients. The median follow-up period was 8.5 years.The complaints at admission were jaundice (64.2 %), fatigue (26.9 %) and fainting (7.5 %). Physical examination revealed hepatomegaly and splenomegaly in 65.6% and 77.6% of the patients, respectively. Hemoglobin mean values at the time of the admission was 8.3±2.1 g/dl, ranging between 5.1-15.3 g/dl. The mean MCV value was 83.1±9.7fl, mean value of MCH was 28.8±2.9 pg, mean MCHC value was 34.9±1.6 g/l, mean indirect bilirubin was 3.5 ± 4 mg/dl. There were various degrees of spherocytosis observed in peripheral smear examinations in all patients. Incubated osmotic fragility test confirmed the diagnosis in all cases. During follow-up, 24 patients (35.8%) never needed a transfusion; 10 (14.9%) patients had an increased need for transfusion in infection periods; eight patients (12%) were regularly transfused, other 25 patients were transfused one or two times, not regularly. 29 (43.2%) had a splenectomy, 41% of the patients who had a splenectomy had a simultaneous cholecystectomy because of the bile sludge and gallstones identified in the ultrasound. Laboratory findings of the patients were also evaluated before splenectomy and two months after splenectomy. Hemoglobin and platelet levels increased significantly (p

Objective: Spexin (SPX) is a novel peptide implicated in food intake and satiety. SPX levels are reduced in obese patients. Aim: This study aimed to compare serum SPX levels in obese adolescents versus healthy controls and to assess the associations of metabolic syndrome (metS) antecedents with serum SPX levels. Methods: Eighty consecutive obese adolescents aged 10–18 years and 80 healthy peers were enrolled. Anthropometric measurements, pubertal examinations, and clinical blood pressure measurements were performed. Fasting blood samples were drawn for glucose, insulin, lipids, uric acid, alanine aminotransferase (ALT), and SPX. metS was diagnosed using International Diabetes Federation criteria. Associations of serum SPX with clinical and laboratory variables were assessed. Results: Obese adolescents had lower serum SPX levels than healthy peers (50 pg/mL [25–75% IQR: 25–98 pg/mL] and 67.0 pg/mL [25–75% IQR: 32.5–126.0 pg/mL]; respectively, p = 0.035). Twenty (25%) obese adolescents were diagnosed as having metS. Obese adolescents with metS had lower SPX than those without metS (24.5 pg/mL [25–75% IQR: 15.3–49.5 pg/mL] and 69.0 pg/mL [25–75% IQR: 42.0–142.0 pg/mL]; respectively, p < 0.0001). The frequencies of hyperuricemia, IR, and elevated ALT were similar in obese adolescents with metS and those without metS (p > 0.05 for all). Serum uric acid levels were correlated significantly with serum SPX after correcting for BMI and HOMA-IR (r = −0.41, p < 0.05). A serum SPX level at a cutoff level of 49.5 pg/mL predicted the presence of metS in obese adolescents with 75% sensitivity and 71% specificity. Conclusions: Obese adolescents have reduced SPX levels, and this reduction is more pronounced in those with metS. Further research is needed to verify the utility of SPX as a biomarker in the diagnosis of metS in obese adolescents.

β-thalassemia major is a significant health problem in the world that obliges patient to repeated blood transfusions. Frequent transfusions cause toxic iron overload and the association between serum iron levels and β-thalassemia major have been extensively studied in literature. Nonetheless, data on trace elements is still limited. The aim of this work was to study the relationship between some trace element levels in serum and β-thalassemia major. The quantifications of Al, K, Sn, Sb, Ni, Cr, Cd, Ba, Co, As and Se elements were carried out by Inductively Coupled Plasma - Mass Spectrometry system. Mann Whitney U test is performed in order to test the statistical difference between patient and control groups in terms of their element concentrations. Significant differences were observed for the concentrations of Al, K, Sn and Sb elements and for the correlation between concentrations of K-Sb elements. The study indicates higher levels of Al and Sb, and lower levels of K and Sn elements of patients when compared to control group. These findings reveal the altered profile of serum trace element concentrations and so, further studies are required to evaluate the potential of trace elements as biomarkers and/or to administrate their levels in blood to reduce the related complications.

PubMedID: 30706992 Aim: Since the beginning of the Syrian civil war, more than 3.5 million Syrians have been under temporary protection status in Turkey. Because beta-thalassemia (BT) is a prevalent disorder in the Mediterranean countries, we decided to estimate the prevalence of and make an overview of the demographic, socioeconomic, medical characteristics, and healthcare problems of refugee children with BT. Patients: Eighteen Turkish Pediatric Hematology Oncology Centers (PHOC) with 318 refugee children from 235 families participated in the study. The mean age of the patients was 8.1 ± 4.8 years (0.5–21 years). The mean time after immigration to Turkey was 2.5 ± 1.5 years (range, 0.1–7 years). Seventy-two (22.6%) of them were born and diagnosed with BT in Turkey. On physical examination, 82 patients (26%) were underweight and 121 patients (38%) were stunted. The appearance of a thalassemic face was reported for 207 patients (65.1%). Hepatomegaly and splenomegaly were reported in 217 (68.2%) and 168 (52.8%) patients, respectively. The median ferritin level was 2508 ng/mL (range, 17–21 000 ng/mL) at the first admission, and 2841 ng/mL (range, 26–12 981 ng/mL) at the last visit after two years of follow-up in a PHOC (P > 0.05). The most frequently encountered mutation was IVSI-110 (G>A) (31%). Before immigration, only 177 patients (55.6%) reported the use of chelators; after immigration it increased to 268 (84.3%). Conclusion: Difficulties in communication, finding a competent translator capable in medical terminology, nonregular use of medications, and insensitivity to prenatal diagnosis were preliminary problems. The current extent of migration poses emerging socioeconomic and humanitarian challenges for refugee patients with BT. © 2019 Wiley Periodicals, Inc.

Koker O, Yildirmak ZY, Genc DB, Kilicaslan O. Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases. Turk J Pediatr 2019; 61: 418-423. Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem disorder characterized by single or recurrent episodes of thrombocytopenia, microangiopathic hemolytic anemia and widespread microvascular thrombosis, which causes significant morbidity and mortality unless promptly recognized and treated. The underlying pathogenesis is a defect in von Willebrand factor (vWF) cleaving protease, called `A Disintegrin and Metalloproteinase with Thrombospondin Type 1 Repeats 13 (ADAMTS-13)`. There are 2 forms: congenital TTP (ADAMTS-13 gene mutations) and acquired TTP (autoantibodies and ADAMTS-13 deficiency). We presented two patients who initially presented with thrombotic microangiopathy and were later diagnosed with TTP upon demonstration of the deficiency in ADAMTS-13 activity.