Gungor Karayalcin

Cure of Implantable Venous Port—Associated Bloodstream Infections in Pediatric Hematology-Oncology Patients without Catheter Removal

The efficacy of antibiotic treatment of port-associated bloodstream infection without device removal has not been systematically studied. We analyzed the outcome of 43 consecutive port-associated bloodstream infections in pediatric hematology-oncology patients. Etiologies included Staphylococcus epidermidis (30) and Staphylococcus aureus (6). Antibiotics were given through the port for a median of 11 days. Four ports were removed within 72 hours. In 36 (92%) of the remaining 39 episodes, there was a response to antibiotic therapy (defervescence and negative blood culture). In 78% of episodes in which there was a response (excluding two in which the catheters were removed because of mechanical problems), the infections were cured without port removal. Two of the four relapses were cured with a second course of antibiotics. The cure rate was 92% for S. epidermidis infections and 67% for S. aureus infections. Thus, the majority of port-associated bloodstream infections in pediatric hematology-oncology patients can be cured without device removal.

Vancomycin and Ceftazidime Bioactivities Persist for at Least 2 Weeks in the Lumen in Ports: Simplifying Treatment of Port-Associated Bloodstream Infections by Using the Antibiotic Lock Technique

ABSTRACT The residual antibiotic concentration of vancomycin (2 mg/ml)- or ceftazidime (2 mg/ml)-heparin solutions instilled in ports in pediatric hematology-oncology patients 1 to 34 days earlier was measured. Antibiotic concentrations of ≥100 μg of either antibiotic per ml persisted for at least 21 days. For treatment of lumenal port infections, antibiotic-heparin dwell times of ≥2 weeks may be appropriate.

The molecular basis of severe hemophilia B in a girl

Severe phenotypic expression of recessive X-linked disorders in females is rare, although examples have been reported. Cases have been documented in females who were homozygous for a recessive alle...

Intracerebral presentation of Hodgkin disease mimicking meningioma in a young woman: case presentation with literature review.

Intracranial involvement of the Hodgkin disease (HD) is a rare entity. Until now, 9 cases of initial presentation of the HD as a brain tumor with appropriate morphologic and histochemical confirmation were reported. Of the 9 patients, 6 had isolated primary intracranial HD and 3 patients after further investigation were found to have extracranial involvement. Seven patients had nodular sclerosing histology, 1 had mixed cellularity, and in 1 case histology was not reported. We describe a patient with systemic nodular sclerosing HD, who initially presented with a brain mass mimicking meningioma and was found to have disseminated lymphadenopathy and bone involvement.

Zinc deficiency in children with sickle cell disease.

Zinc status was studied in 47 children with homozygous sickle cell disease (SCD). Decreased hair and plasma zinc was demonstrated in these children and hyperzincuria was found in the older patients. This study indicates that zinc deficiency in patients with SCD is probably due to hyperzincuria.

An adolescent with ovarian osteosarcoma arising in a cystic teratoma.

A 14-year-old girl had an abdominal mass with the characteristics of an ovarian germ cell tumor on computed tomography scan. The mass, arising from the left ovary, was completely resected and found to be osteosarcoma arising from a mature cystic teratoma. A metastatic lesion in the abdomen did not respond to 2 courses of cisplatin, doxorubicin, ifosfamide, and high-dose methotrexate, and was resected. Seven months after completion of chemotherapy, there were simultaneous local recurrence and lung metastases. Previously, 10 cases of ovarian osteosarcoma have been reported in the literature: 5 were primary osteosarcoma of the ovary, 4 were associated with teratomas, and 1 was part of a malignant mixed mesodermal tumor of the ovary. Of the 10, there are only 2 long-term survivors, both of whom were treated with adjuvant chemotherapy following complete resection.

The evaluation of auditory function in homozygous sickle cell disease.

The most significant clinical manifestations of sickle cell disease (SCD) are those associated with CNS involvement. This disease is systemic in character, with the tissues of the CNS being the most sensitive to even temporary anoxia. For this reason, the incidence of peripheral and central auditory dysfunction in patients with SCD may be greater than in the “normal” population. We examined the peripheral auditory function of 54 patients with homozygous sickle cell disease and the central auditory function of 28 of these patients. Results were compared with a control population. Peripheral hearing loss was found in 11% (6) of the SCD population. Central auditory testing indicated that 46% (13) of the sickle cell population had mild central auditory dysfunction not involving Heschls gyrus. This study suggests a higher than usual incidence of peripheral and central auditory impairment in sickle cell disease.

Disaccharidase Levels in Iron Deficient Rats at Birth and during the Nursing and Postweaning Periods: Response to Iron Treatment

Summary: Newborn rats born to iron deficient mothers (IDM) were found to have significantly lower hemoglobin, sucrase, lactase and maltase levels compared to control newborn rats.Rats born to IDM and nursed by IDM, when sacrificed at 21 days of age, had statistically significantly lower hemoglobin, serum iron, sucrase, lactase and maltase levels compared to control rats. Rats born to IDM, but nursed by iron sufficient mothers (ISM) and sacrificed at 21 days of age, had hemoglobin, serum iron and sucrase levels compared to control rats whereas lactase and maltase were not corrected by 21 days of nursing by ISM. Rats born to IDM and nursed by either IDM or ISM for 21 days were given intramuscular iron dextran and placed on iron sufficient diet (ISD) for 7 days. These animals experienced correction of the hemoglobin, serum iron, sucrase and maltase levels compared to control rats, whereas intestinal lactase was not corrected by 7 days of ISD and intramuscular iron.Rats born to ISM, nursed by IDM and sacrificed on day 21 had significantly lower hemoglobin, serum iron and intestinal lactase levels compared to control rats. Rats born to ISM and nursed by IDM were given intramuscular iron dextran on day 21 and placed on an ISD from day 21–28. These animals had a return in hemoglobin, serum iron, sucrase and maltase levels comparable to control rats.Rats born to and nursed by ISM and maintained on an iron deficient diet from day 21–84 had significantly lower hemoglobin, serum iron, sucrase, lactase and maltase levels compared to control rats. Rats born to and nursed by ISM, maintained on iron deficient diet from day 21–84, and then given intramuscular iron dextran on day 84 and maintained on an ISD until day 92, experienced correction of the hemoglobin, serum iron and lactase levels compared to control rats. Intramuscular iron and 7 days of ISD did not correct the sucrase and maltase levels in these rats. Lactose tolerance tests in iron deficient rats showed flat curves compared to controls. After iron treatment, lactose tolerance curves returned to control values.Iron deficiency in rats in utero, during the nursing and postweaning period causes, in addition to anemia, a reduction in jejunal disaccharidase activity because of an alteration in the enzymes of the brush border membrane. Varying degrees of reduction and response of certain disaccharidases to iron treatment are dependent on the time of iron deprivation in relationship to the intra-uterine and postnatal development of the digestive and absorptive functions in the small intestine.Alterations in the levels of disaccharidases demonstrated in this paper represent another aspect of the spectrum of biochemical effects of iron deficiency.Speculation: Iron deficiency has a profound effect on protein synthesis. Alterations in the levels of disaccharidases represent only a fraction of this effect.The temporary reduction in lactase and other disaccharidases in iron deficient-infants might predispose them to malabsorption and diarrhea, which contribute to the lowered serum protein and increased incidence of low weight percentiles observed in iron deficient infants.

Thrombocytopenia Absent Corpus Callosum Syndrome: Third Case of a Distinct Clinical Entity

Thrombocytopenia absent corpus callosum, characterized by refractory thrombocytopenia, agenesis of the corpus callosum, hypoplastic cerebellum, abnormal facies, and developmental delay, represents a relatively newly described clinical entity. An 18-month-old girl with agenesis of the corpus callosum, hypoplasia of the cerebellar vermis, hypotonia, and severe developmental delay presented with thrombocytopenia. She had a distinctive facies with microcephaly, broad nasal root with upturned nose, small upper lip, and micrognathia. A bone marrow aspirate and biopsy showed normal cellularity with dysplastic megakaryocytes. Thrombocytopenia absent corpus callosum is compared with other conditions characterized by congenital non-immune thrombocytopenia.

Lymphomatoid Papulosis and Ki-1 + Anaplastic Large Cell Lymphoma Occurring Concurrently in a Pediatric Patient

Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over the course of time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP, but there are have been no cases reported in patients younger than age 26 years. We describe the first pediatric patient, a 16-year-old girl, who had clinical features of LyP and concurrently was found to have a lesion diagnosed as Ki-1+ anaplastic large cell lymphoma. After treatment with chemotherapy, she has been in continuous remission for 16 months.