Staphylococcal Pyomyositis in a Child with Fanconi Aplastic Anemia

We retrospectively analyzed all 164 cases of pediatric lymphoma diagnosed in northern Israel during the 18-year period from 1973 to 1990. Our findings generally conformed to those of other studies with regard to annual incidence (24.7/million), age at diagnosis (5 to 9 years for Jewish males, later for others), histology (Hodgkin's disease most commonly), and male predominance. The northern Israeli pediatric lymphoma pattern of incidence is similar to the Asian-African-South American pattern and unlike that of Europe or North America; in Israel, lymphoma rather than brain tumor is the second most common childhood malignancy. The nodular sclerosing variant was the most common histology seen in Hodgkin's lymphoma (especially in females), followed by mixed cellularity. Malignancies are generally more common in Jewish children but lymphomas in particular were seen more often in Arabs (28.7/million), while the Ashkenasi (20.81 million) and Sephardi (21.61 million) Jewish populations had similar incidences. The high rate of consanguineous marriages among Arabs and their lower socioeconomic level may explain the high incidence of lymphomas in this group.     

Cyclophosphamide-Based In Vivo T-Cell Depletion for HLA-Haploidentical Transplantation in Fanconi Anemia

Allogeneic hematopoietic cell transplantation (HCT) is the only known cure for patients with Fanconi anemia (FA) who develop aplasia or leukemia. However, transplant regimens typically contain high-dose alkylators, which are poorly tolerated in FA patients. Furthermore, as many patients lack human leukocyte antigen (HLA)-matched family donors, alternative donors are used, which can increase the risk of both graft rejection and graft-versus-host disease (GVHD). To improve on these three concerns, we developed a multi-institutional clinical trial using a fludarabine (FLU)-based conditioning regimen with limited alkylators/low-dose radiation, HLA-haploidentical marrow, followed by reduced-dose cyclophosphamide (CY) to treat three FA patients with aplasia. All three patients engrafted with 100% donor CD3 chimerism at 1 month. One patient died early from disseminated toxoplasmosis infection. Of the two survivors, one had significant pretransplant co-morbidities and inadequate immunosuppression, and developed severe acute GVHD. The other patient had only mild acute and no chronic GVHD. With a follow-up of 2 and 3 years, respectively, both patients are doing well, are transfusion-independent, and maintain full donor chimerism. The patient with severe GVHD has resolving oral GVHD and good quality of life. We conclude that using low-intensity conditioning, HLA-haploidentical marrow, and reduced-dose CY for in vivo T-cell depletion can correct life-threatening aplasia in FA patients.     

Cyclophosphamide-Based In Vivo T-Cell Depletion for HLA-Haploidentical Transplantation in Fanconi Anemia

Allogeneic hematopoietic cell transplantation (HCT) is the only known cure for patients with Fanconi anemia (FA) who develop aplasia or leukemia. However, transplant regimens typically contain high-dose alkylators, which are poorly tolerated in FA patients. Furthermore, as many patients lack human leukocyte antigen (HLA)-matched family donors, alternative donors are used, which can increase the risk of both graft rejection and graft-versus-host disease (GVHD). To improve on these three concerns, we developed a multi-institutional clinical trial using a fludarabine (FLU)-based conditioning regimen with limited alkylators/low-dose radiation, HLA-haploidentical marrow, followed by reduced-dose cyclophosphamide (CY) to treat three FA patients with aplasia. All three patients engrafted with 100% donor CD3 chimerism at 1 month. One patient died early from disseminated toxoplasmosis infection. Of the two survivors, one had significant pretransplant co-morbidities and inadequate immunosuppression, and developed severe acute GVHD. The other patient had only mild acute and no chronic GVHD. With a follow-up of 2 and 3?years, respectively, both patients are doing well, are transfusion-independent, and maintain full donor chimerism. The patient with severe GVHD has resolving oral GVHD and good quality of life. We conclude that using low-intensity conditioning, HLA-haploidentical marrow, and reduced-dose CY for in vivo T-cell depletion can correct life-threatening aplasia in FA patients.     

Prevalence of childhood asthma and allergies in Serbia and Montenegro

Background  

This is the first multi-center epidemiological study on the prevalence of childhood asthma in Serbia and Montenegro. The International Study of Asthma and Allergies in Childhood (ISAAC) phase 3 is a large epidemiological multinational and multicentric study on the prevalence of asthma, allergic rhinitis and eczema in children.     

Prevalence of primary headaches in children from Belgrade, Serbia.

The aim of our study is to estimate the prevalence of migraine and tension-type headaches in 7-12-year-old school children from Belgrade. The sample was drawn from a total of 1259 school children from randomly selected primary schools. The diagnosis was made on the basis of a clinical interview combined with the neurological examination. The prevalence of migraine increases with age from 0.5% at the age of seven, to 6.8% at the age of 12. Female to male ratio inverts with age: males predominate at age 7-9, but females predominate at age 10-12. The estimated prevalence of tension-type headache increases with age from 0.5% in 7-year olds to 2.4% in 12-year olds. Statistically, the onset of migraine occurs significantly earlier than that of tension-type headache. Migraine and tension-type headache are frequent headache types and have different demographic characteristics among children.     

Prevalence and Risk Factors of Anemia among Adolescents in Denizli,Turkey

Objective

The aim of this study was to evaluate the prevalence and risk factors of anemia among adolescents in Denizli where Mediterranean cuisine (fresh fruit and vegetables) is adopted.

Methods

We accepted hemoglobin values below 12 g/dl for girls and 13 g/dl for boys as the criteria of anemia. We recorded a detailed history including nutritional habits, consumption of animal source foods especially red meat, consumption of fresh fruit and vegetables, presence of parasitic infestation, psychosocial status, school success and any materials consumed except ordinary food.

Findings

We investigated the prevalence of anemia in 1120 children (672 girls and 448 boys), aged 12 to 16 years. We found that the overall prevalence of anemia was 5.6%. 8.3% of the girls and 1.6% of the boys were anemic. We diagnosed iron deficiency anemia in 37(59%) anemic patients and combined iron deficiency and vitamin B12 deficiency anemia in 26 (41%) anemic patients. None of the patients had folic acid deficiency.

Conclusion

Our results suggest that the socioeconomic status of the family, traditional eating habits of the region, the fear of gaining weight and irregular eating habits are of great importance in the development of adolescent anemia in Denizli.     

Carnitine Membrane Transporter Deficiency: A Rare Treatable Cause of Cardiomyopathy and Anemia

Carnitine transporter defect is an autosomal recessive disorder caused by mutations in the SLC22A5 gene that encodes the high-affinity carnitine transporter OCTN2. Affected patients can present with predominant metabolic or cardiac manifestations. Early recognition of this disorder in a context of life-threatening cardiac failure and treatment with carnitine can be lifesaving in this inborn error of fatty acid oxidation. Here we describe a boy with a severe cardiomyopathy and severe anemia who improved with carnitine therapy. Physiopathology of anemia, a probably less recognized symptom of carnitine deficiency, is also discussed.     

Fanconi Syndrome Secondary to Deferasirox in Diamond–Blackfan Anemia: Case Series and Recommendations for Early Diagnosis

Deferasirox is an oral iron chelator used to treat patients with transfusion‐related iron overload. We report, from two institutions, two children with Diamond–Blackfan anemia who developed Fanconi syndrome secondary to deferasirox administration, along with a review of the literature. The current recommendation for the laboratory monitoring of patients receiving deferasirox does not include serum electrolytes or urine analysis. Thus, despite routine clinic visits and bloodwork, these two patients presented with life‐threatening electrolyte abnormalities requiring hospitalization. Hence, we propose the inclusion of serum electrolytes and urine analysis as part of routine monitoring to facilitate the early diagnosis of Fanconi syndrome in the context of high doses of deferasirox therapy.     

Refractory Anemia as a Presenting Feature of Microscopic Polyangitis: A Rare Vasculitis in Children

Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease. Small-vessel vasculitis should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Among these, antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis is a distinct subclass involving anti-neutrophil cytoplasmic antibody (ANCA) as the common pathogenesis. Microscopic polyangiitis is a rare form of such vasculitis in children characterized by pulmonary-renal syndrome with pauci-imune rapidly progressive glomerulonephritis and a poor outcome.     

Squamous Cell Carcinoma of the Tongue in a Child with Fanconi Anemia: A Case Report and Review of the Literature

This report documents a case of squamous cell carcinoma (SCC) of the tongue in a child with Fanconi anemia (FA). FA is an autosomal recessive syndrome defined by chromosomal breakage in response to diepoxybutane or mitomycin C in which many patients present with pancytopenia, hypoplastic bone marrow, hyperpigmentation of the skin, skeletal malformations, small stature, hypogonadism, and chromosomal aberrations. Such patients are prone to the development of hematological malignancies and squamous cell carcinoma, especially of the head and neck. Although FA appears to be genetically heterogeneous, all cases display abnormalities of DNA repair. A gene defective in one of the four subsets of FA patients has been defined. Defects in this gene are thought to play a role in the development of neoplasia in FA patients. However, many other factors may also contribute to the development of malignancies, including immune deficiencies, therapeutic strategies, and bone marrow transplantation. This report reviews the association of FA and SCC and highlights the many factors involved in the development of neoplasia within a single patient, including FA, cyclophosphamide, immunosuppression, X-irradiation, and chronic oral graft-versus-host disease. In addition, the human papillomavirus status, although negative, is documented for the first time in such a case.     

Epilepsy Prevalence in the 0-17 Age Group in Trabzon,Turkey

Objective

Epilepsy is a most common serious neurological disorder and is one of the world''s most prevalent non-communicable diseases. The aim of this study was to determine the prevalence and risk factors of epilepsy in 0–17 year old children in Trabzon, Turkey.

Methods

A cross-sectional epidemiological investigation was performed in two phases, a screening phase and a confirmation of the diagnosis phase. The gold standard was a clinical investigation and neurological examination. The diagnosis of epilepsy followed clinical guidelines proposed by the International League against Epilepsy (ILAE). The chi-square test was used in analysis of the results and P-value <0.05 was calculated.

Findings

The prevalence per 1000 participants of epilepsy was 8.6 (5.9–11.4; ^95%CI). We detected 37 cases (18 males and 19 females) of epilepsy. The male/female ratio was 0.95. This study showed an increased risk for epilepsy with low socioeconomic level, a history of postpartum seizure, meningitis, head trauma, febrile convulsion and family history of epilepsy. More than one seizure type was present in 15 (40.5%) of epileptic children. Generalized tonic-clonic seizures were determined in 24 patients (64.9%) and absence type in 9 (24.3%). It was found that 25.0% of children with epilepsy had never visited the school at the time the study was performed due to the disease and attendant seizures.

Conclusion

The prevalence of epilepsy in Trabzon is low compared to other parts of Turkey and other developing countries.