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Propranolol is the treatment of choice for infantile hemangioma. We investigated the effects of long-term propranolol use in early infancy on learning and memory later in life in mice. At three weeks of age, mice were randomly divided into six experimental groups. Groups 1 and 2 (controls) received only saline for 21 days. Groups 3 and 4 received propranolol (2.5 mg/kg) for 21 days. Groups 5 and 6 received propranolol (5 mg/kg) for 21 days. Groups 1, 3 and 5 were tested at the end of 21 days of treatment (week 6). However, groups 2, 4 and 6 received a 2-week break and then (week 8) exposed to tests. In the Morris water maze test, propranolol (2.5 and 5 mg/kg) dose-dependently increased the time spent in the target quadrant in mice at weeks 6 and 8. However, propranolol did not affect the swimming speed in both time periods. There were no significant effects of propranolol on the number of errors evaluated during the radial arm maze tests. In conclusion, long-term use of propranolol in early infancy did not disrupt the learning and memory of mice., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Iron deficiency anemia in children is a public health problem. Although oral iron treatment is the first choice, common side effects and compliance problems can cause the treatment to be interrupted. This study retrospectively evaluated children treated with intravenous (IV) iron sucrose or ferric carboxymaltose (FCM) and compared the treatment processes and efficacy. The demographic characteristics and treatment details of the 44 children with iron deficiency anemia were retrospectively evaluated. Iron sucrose was administered to 25 patients and FCM was administered to 19 patients. The IV iron infusion was applied to 64% of the patients because of unresponsiveness to oral treatment, 25% of the patients because of compliance problems, and 11% of the patients because of severe anemia. IV iron therapy increased hemoglobin, mean corpuscular volume, mean corpuscular hemoglobin, red-cell distribution width, and serum ferritin levels and decreased platelet count. The mean number of infusions per patient in the FCM group was lower, and the total treatment time was shorter. In conclusion, IV iron sucrose or FCM can be used in children with nonadherence to oral therapy and severe anemia in addition to specific indications., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Priapism is a clinical condition that rarely presents with leukemia in childhood. Management of priapism treatment can become more complex and difficult when accompanied by acute leukemia. We presented a 16 years old child with t-cell acute lymphoblastic leukemia (ALL) who developed priapism. Due to the failure of conservative methods and intracavernosal drainage, we performed epidural blockade which has limited data reported with successful results in the literature before shunt surgery., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Transcobalamin deficiency (OMIM 275350) is a rare autosomal recessive disease presenting with nonspecific clinical features in early infancy. We report the clinical and laboratory manifestations of 7 children diagnosed with transcobalamin deficiency. All patients were admitted between 2 and 4 months of age with anemia, thrombocytopenia, and hyperhomocysteinemia. The most common complaints at admission were pallor, weakness, and poor feeding. Genetic analysis was performed in 5 patients and it revealed the same homozygous mutation. We initially treated all patients with intramuscular injections of a maximum of 1 mg cyanocobalamin (CN-Cbl) daily and with a final dose of 1 mg per week. Hemoglobin and platelet counts significantly decreased upon decrease or cessation of CN-Cbl therapy. The patients were reevaluated between 2 and 4 years of age and all had delay in speech and walking. In conclusion, 1 mg of intramuscular CN-Cbl every week suffices for hematological improvement but not for normal neurological development in patients who all had relapse due to decrease or cessation of treatment.

Cancer and its treatment are stressful and reduce the quality of life in children. The aim of this study was to investigate the effect of massage therapy on pain and anxiety arising from intrathecal therapy or bone marrow aspiration in children with cancer. We conducted a controlled pretest/posttest quasi-experimental study at a paediatric oncology unit in Turkey. Twenty-five children were enrolled in this study. Their pain and anxiety were determined using a visual analogue scale. When the pretest and posttest pain and anxiety levels of the groups were compared, no statistically significant difference was found (P > 0.05). It was determined that pain and anxiety levels in the experimental group decreased significantly. This study provides preliminary evidence for the effectiveness in children of massage in reducing pain and anxiety arising from intrathecal therapy or bone marrow aspiration., (© 2014 Wiley Publishing Asia Pty Ltd.)

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.

Background/aim: Limited data are available on the effect of melatonin (MLT) on the cytotoxicity of chemotherapeutic drugs in tumor cells. In this study, we aimed to evaluate the effect of MLT on the cytotoxicity of different chemotherapeutic agents in leukemia cells in vitro., Materials and Methods: The experiments were carried out using human leukemia cell lines, Jurkat, MOLT-4, Daudi, HL-60, CMK, and K562, and two patient samples. Leukemia cells were incubated with cytarabine, daunorubicin, and etoposide with or without 10(-5) M and 10(-3) M concentrations of MLT. Cytotoxicity was measured by detecting apoptosis using flow cytometry., Results: Overall, co-incubation with melatonin did not alter the cytotoxicity of chemotherapeutic drugs in cell lines and patient samples except one. In a patient sample with acute myeloid leukemia, etoposide treatment in combination either concentrations of MLT resulted in increased elimination of the leukemia cells., Conclusion: Melatonin does not interfere with the cytotoxic effect of cytarabine, daunorubicin and etoposide in leukemia cells.

Corticosteroids have been widely used in the treatment of idiopathic thrombocytopenic purpura (ITP). We evaluated the late side effects of high-dose methylprednisolone (HDMP) therapy on bone metabolism in children with ITP. Twenty-eight children with acute ITP treated with HDMP (30 mg/kg/d for 3 d then 20 mg/kg/d for 4 d) and 28 controls were enrolled in the study. Bone mineral density (BMD), urinary calcium creatinine ratio, urinary levels of deoxypyridinoline, serum levels of calcium, phosphate, parathyroid hormone, total alkaline phosphatase, and bone-specific alkaline phosphatase were measured in both groups. Magnetic resonance imaging of the femoral head was performed only in study group. The mean levels of serum phosphate, parathyroid hormone, urinary deoxypyridinoline, and calcium creatinine ratio were significantly increased in the study group. There was no significant difference between the 2 groups in terms of serum calcium, total alkaline phosphatase, bone-specific alkaline phosphatase, and BMD values. There was a statistically significant negative correlation between cumulative steroid dose and BMD values in study group (r = -0.379). Osteonecrosis was observed in 3 of 25 patients by magnetic resonance imaging. In conclusion, HDMP therapy, especially in high cumulative doses, increases the bone resorption and may cause osteonecrosis in children with ITP.

We report a case of pleuropulmonary blastoma in a child initially diagnosed with encysted pleural empyema. She was treated with combination chemotherapy including ifosfamide, etoposide, vincristine, adriamycin, actinomycin-D, and cyclophosphamide after surgical excision.

Melatonin has a variety of functions in human physiology and is involved in a number of pathological events including neoplastic processes. The tissue protective actions of melatonin are attributed to its antioxidant activity though, under certain conditions, melatonin might also exert oxidant effects, particularly in cancer cells. This study evaluated the effects of 10(-5) and 10(-3) m concentrations of melatonin on human leukemia cells. Moderate cytotoxic effects of melatonin at 10(-3) m concentrations were observed in CMK, Jurkat and MOLT-4 cells which was associated with significant reactive oxygen species (ROS) generation. Melatonin treatment was not associated with significant cytotoxicity in HL-60 cells, although the generation of ROS was significantly increased. K562 and Daudi cells did not appear to be effected by melatonin treatment. Cellular membrane lipid peroxidation was not influenced by melatonin with the exception of CMK cells. Cell cycle kinetics were not affected in melatonin-treated samples, again with the exception of CMK cells which showed increased apoptosis. Melatonin, therefore, induces the production of ROS that may be associated with cytotoxicity depending on the concentration of melatonin in some leukemia cells and does not appear to stimulate leukemia cell growth. These pro-oxidant actions of melatonin may assist in limiting leukemic cell growth.

Xanthoma disseminatum is a rare, usually self-healing dermatologic disease of unknown etiology. Involvement of other organs and tissues including bone marrow, bone, and brain may be seen rarely in children. However, to date, hepatic involvement has not been reported. We describe a child with xanthoma disseminatum who had hepatic involvement, and discuss his course and treatment with steroid and azathioprine.

Pyridoxine dependency is a rare autosomal-recessive disorder causing intractable seizures in neonates and infants. This case report describes an infant with pyridoxine-dependent seizures with microcephaly and discusses a probable pathogenetic mechanism of microcephaly in this condition.

Pseudotumor cerebri is characterized by signs of raised intracranial pressure occurring in the absence of obvious brain pathology. We present an 8-year-old boy with pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The reported patient suggests a possible association between subacute sclerosing panencephalitis and pseudotumor cerebri and that subacute sclerosing panencephalitis should be added to the list of disorders known to be associated with pseudotumor cerebri. Patients with subacute sclerosing panencephalitis might benefit from treatment aimed at increased intracranial pressure.

Aims: Because of the observation of an abundance of leukaemia/lymphoma cell microparticles in the bone marrow aspiration sample of a patient with Burkitt's leukaemia at diagnosis, the occurrence of this phenomenon in leukaemia/lymphoma samples with available immune phenotyping data was investigated retrospectively., Methods: Flow cytometric immune phenotyping and spontaneous apoptosis analysis of the bone marrow mononuclear cell preparation of the index case were performed. Microparticles isolated form the bone marrow sample were also studied for the presence of leukaemia/lymphoma cell microparticles. List mode analysis of 225 cases of acute leukaemia or lymphoma with previously performed immune phenotyping was also carried out., Results: The presence of leukaemia/lymphoma cell microparticles could be detected by flow cytometry and they were found to be different from apoptotic bodies. Leukaemia/lymphoma cell microparticles were released in all cases of mature B cell neoplasms studied, although this phenomenon was rare in precursor B cell disorders and acute myeloid leukaemia., Conclusions: The generation of leukaemia/lymphoma cell microparticles in mature B cell neoplasms appears to be a common phenomenon. The pathogenesis and clinical implications must be investigated.

Acute disseminated encephalomyelitis is an immune-mediated demyelinating disorder usually encountered in children or adolescents and characterized by multifocal neurologic deficits of rapid onset. It is often preceded by various infectious diseases or vaccination, but acute disseminated encephalomyelitis developing after hepatitis A virus infection is rare. We present such a case in a 9-year-old patient who illustrates the importance of considering acute disseminated encephalomyelitis in patients who develop multifocal neurologic signs after hepatitis A virus infection. We suggest that patients with acute disseminated encephalomyelitis be screened for hepatitis A virus because of the occurrence of asymptomatic hepatitis A virus infection.

We studied the attitudes of 87 parents who had the choice to watch their children's single or multiple lumbar punctures (LPs). Anxiety scores were moderate, higher before LP than after, and were unaffected by the number or observation. Parents who observed the LP wished doing likewise in the future.

Tourette's syndrome (TS) is a neuropsychiatric disorder characterized by the presence of involuntary motor and phonic tics. Phonic tics can mimic respiratory system disorders such as asthma, and upper and lower respiratory system infections. We report on twins with chronic persistent cough (CPC): one of whom was followed as an asthmatic for a year and the other was diagnosed with recurrent respiratory tract infection. A careful history and neurologic assessment suggested that TS might be responsible for the first twin's symptoms but that the second one was probably in early TS. All the symptoms of the first patient diagnosed as TS showed a complete improvement with pharmacological treatment in two weeks. Since the history of CPC may in reality be TS, we recommend that TS should be considered in the differential diagnosis of pediatric CPC.

A case with pulmonary atresia/ventricular septal defect associated with port-wine stain and retinal vascular abnormality is reported. Clinical findings were similar to both PHACE syndrome and Sturge-Weber syndrome (SWS). But, the most frequent and well-known features of both syndromes were absent. So, it could not be concluded whether this is a new constellation or an incomplete form of one of the two syndromes. In both situations, presence of a complex congenital heart disease that has not been reported previously makes this case original., (Copyright 2003 Wiley-Liss, Inc.)

Miller Fisher syndrome is characterized by a triad of ataxia, ophthalmoplegia, and reduced or absent tendon reflexes, with minimal if any limb weakness. Anti-GQ1b immunoglobulin G antibodies are present in high titers in most patients. Presented is a two-year-old female with Miller Fisher syndrome whose blood serum was negative for anti-GQ1b immunoglobulin G antibodies.