Fluid Rate Is Important As Much As Fluid Tonicity: An Experimental Study.

OBJECTIVE: There is no study evaluating the effect on plasma osmolality of both fluid tonicity and high fluid rate at the same time. The aim of this experimental study was to determine the change in the plasma osmolality by different fluid tonicity and rate, and to suggest the safest and the most appropriate fluids based on the plasma osmolality for medical situations requiring fluid therapy with high or maintenance rates. MATERIALS AND METHODS: The rats were randomly divided into seven groups (six rats in each group): [D5] D5 administered at 100 ml/kg/24h; [D5150] D5 administered at 150 ml/kg/24h; [D5(½)100] D5 0.45% NaCl administered at 100 ml/kg/24h; [D5(½)150] D5 0.45% NaCl administered at 150 ml/kg/24h; [D5(1)100] D5 0.9% NaCl administered at 100 ml/kg/24h; [D5(1)150] D5 0.9% NaCl administered at 150 ml/kg/24h; [Control group] non-treated control rats. Intracardiac blood samples were collected from all the groups at the end of 24 h. RESULTS: [D5(1)150] and [D5(½)100] were the group closest to the control group in terms of both sodium (P = .937; P = .699, respectively) and effective osmolality (P = 1, P = .818, respectively). CONCLUSION: Our results showed that 0.9% NaCl and 0.45% NaCl solutions might be the safest and the most appropriate fluids to maintain normal plasma osmolality in medical situations requiring fluid therapy with high or maintenance rates, respectively.

Clinical features and treatment results in preterm infants with intracardiac thrombus.

INTRODUCTION: Thrombus incidence is higher among neonates, especially in preterm infants, due to the associated additional risk factors. MATERIALS AND METHODS: The medical recordings of premature infants who had been diagnosed as having intracardiac thrombus between January 2016 and January 2019 were evaluated retrospectively. We use recombinant tissue plasminogen activator when the thrombus is relatively large compared to left atrium, pedunculated, mobile, or snake shaped. RESULTS: A total of 13 premature patients were diagnosed as having intracardiac thrombus during the 3-year period. All were diagnosed during echocardiographic studies. Low molecular weight heparin was administered in four patients. In three, recombinant tissue plasminogen activator was started with low dose (0.01 mg/kg/h) and increased gradually to 0.06 mg/kg/h. In three, recombinant tissue plasminogen activators were started with standard dose (0.5 mg/kg/h). In one recombinant tissue, plasminogen activator was started with low dose (0.01 mg/kg/h) and increased to standard dose. Two patients died before treatment, three patients died during treatment, follow-up was not available for two patients, and thrombus completely resolved in six patients. DISCUSSION: In preterm babies with risk factors, intracardiac thrombus should be kept in mind during all echocardiographic studies. In our patients, low and standard dose regimens were used, and the treatment results were similar.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.

Homozygous c.130-131 ins A (pW44X) mutation in the HAX1 gene as the most common cause of congenital neutropenia in Turkey: Report from the Turkish Severe Congenital Neutropenia Registry.

BACKGROUND: Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation is the most frequently observed genetic defect in the registries from North America and Western Europe. However, in eastern countries where consanguineous marriages are common, autosomal recessive forms might be more frequent. METHOD: Two hundred and sixteen patients with severe congenital neutropenia from 28 different pediatric centers in Turkey were registered. RESULTS: The most frequently observed mutation was HAX1 mutation (n = 78, 36.1%). A heterozygous ELANE mutation was detected in 29 patients (13.4%) in our cohort. Biallelic mutations of G6PC3 (n = 9, 4.3%), CSF3R (n = 6, 2.9%), and JAGN1 (n = 2, 1%) were also observed. Granulocyte colony-stimulating factor treatment was given to 174 patients (80.6%). Two patients died with infectious complications, and five patients developed myelodysplastic syndrome/acute myeloblastic leukemia. The mean (± mean standard error) follow-up period was 129.7 ± 76.3 months, and overall survival was 96.8% (CI, 94.4-99.1%) at the age of 15 years. In Turkey, severe congenital neutropenia mostly resulted from the p W44X mutation in the HAX1 gene. CONCLUSION: In Turkey, mutation analysis should be started with HAX1, and if this is negative, ELANE and G6PC3 should be checked. Because of the very high percentage of consanguineous marriage, rare mutations should be tested in patients with a negative mutation screen.

Hypophyseal Involvement of Acute Lymphoblastic Leukemia.

We present an unusual case of hypophyseal involvement in a boy with acute lymphoblastic leukemia via magnetic resonance imaging findings. In our case, the acute lymphoblastic leukemia of the pituitary gland was accurately distinguished from a pituitary adenoma by contrast-enhanced dynamic hypophysis magnetic resonance imaging studies.

Assessment of Humoral Immunity to Hepatitis B, Measles, Rubella, and Mumps in Children After Chemotherapy.

BACKGROUND: Cancer survival rates and longevity of patients after therapy have significantly improved during the last few decades. Therefore, lasting protection against infections should be provided. PROCEDURE: A total of 162 children diagnosed with acute lymphoblastic leukemia, acute myelogenous leukemia, solid tumors, non-Hodgkin lymphoma, and Hodgkin lymphoma were enrolled in the study. Antibody levels against hepatitis B virus was assessed both at the time of diagnosis and within 6 months after completion of chemotherapy. However, measles, mumps, and rubella (MMR) antibodies levels were measured just within 6 months after completion of chemotherapy. RESULTS: Anti-HBs antibody titers had decreased below the protective level after treatment in 25 of 96 patients having protective antibody levels for hepatitis B virus before therapy. In 66 patients without pretreatment protective levels of antibody, in spite of the immunization during chemotherapy, only 6 of them were found to be anti-HBS positive after treatment. In 153 patients previously vaccinated with MMR, 19 had protective antibody titers after treatment. MMR seropositivities were negatively correlated to age as expected. CONCLUSIONS: Our data demonstrate that a significant number of children lose preexisting humoral immunity against MMR and hepatitis B after completion of chemotherapy.

Advanced Stage T-Cell Non-Hodgkin lymphoma in an 11-Month-Old Infant and Related Superior Vena Cava Syndrome: Importance of Transthoracic Echocardiography.

Superior vena cava syndrome (SVCS) is rare in infants. Non-Hodgkin lymphoma is the most common cause of SVCS in children. Swelling in the face and neck are the most common clinical symptoms associated with this syndrome. However, these clinical findings are also observed in allergic diseases, which therefore often leads to misdiagnosis. Here, we reported the importance of echocardiography in diagnosing SVCS in an infant with advanced stage non-Hodgkin lymphoma.

Papillon-Lefèvre syndrome: report of three cases in the same family.

Papillon-Lefèvre syndrome is a rare autosomal recessive disorder caused by cathepsin C gene mutation leading to the deficiency of cathepsin C enzymatic activity. The disease is characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition, and increased susceptibility to infections. Pyogenic liver abscess is an increasingly recognized complication. Three cases of Papillon-Lefevre syndrome in the same family are presented here. Two of the three siblings presented with characteristic manifestations of the syndrome. The third case had died previously due to liver abscess prior to a diagnosis of Papillon-Lefèvre syndrome.