Incidence and outcome of severe Hyponatremia in children and young adults. A single institution experience

Our two main objectives are to assess the incidence and the outcome of severe hyponatremia in young hospitalized patients. We retrospectively reviewed the incidence and outcome of severe hyponatremiac [Na <125 mmol/l] in-patients less than 18 years of age, admitted as consecutive admissions during one calender year. Psuedohyponatremia and artifactual hyponatremia were excluded. Patients' demographics, clinical features, laboratory, treatment and outcomes were recorded. Of 3561 admissions of patients less than 18 years of age, 20 developed severe hyponatremia. Nausea, vomiting, irritability, clouded sensorium and seizures were the most common symptoms and signs. Underlying central nervous system disease, pneumonia and malignancy were major co-morbid conditions. The initial volume status was determined as hypervolemia [n=7], hypovolemia [n=7] and euvolemia [n=6]. Iatrogenic [diuretics 5 and hypotonic fluids 7] hyponatremia accounted for 60% of all cases. Mortality was 20%. Patients receiving intravenous hypotonic fluids should be closely monitored for the development of hyponatremia. The common etiology of hyponatremia in our studied cohort of patients is iatrogenic

Use of gene expression profiling to identify novel relapse markers in children with high risk acute lymphoblastic leukemia through an international collaboration

Over the years, there have been steady improvements in the management of acute lymphoblastic leukemia in pediatrics [ALL] worldwide, which resulted in a better outcome of the disease. However, 35% of patients relapse while on current therapies. Examining gene expression profiles [GEP] may be the key to further improvement to outcome and decrease the incidence of relapse. This study was done using a novel and unique combination of sophisticated techniques. These are suppression subtractive hybridization [SSH], cDNA concatenated sequencing [CCS] and reverse transcriptase real-time quantitative polymerase chain reaction [RT-RQ-PCR]. This combination allows for the harness of differentially expressed genes, high-throughput sequencing ability and quantitative examination of the expression levels for the differentially expressed sequences respectively, The study focuses on examining differentially expressed genes and potential GEPs for high risk, relapsed and non-relapsed ALL cases with non-determined translocation [NDCT], by performing an SSH on bone marrow samples. The study also aims at providing training in molecular oncology, and transfer of advanced technical and analytical skills from developed to developing countries. Eight genes were found to be differentially expressed between relapsed versus non-relapsed NDCT ALL patients. Seven genes were over expressed in the non-relapsed patients, namely, HNK-Tryp2, TAX, PLSCR, TOP2-B, SP1-2, COP and CASK. Most of these genes have been expressed during cell activation, apoptosis and cancer transformation. Topoisomerase [DNA] II beta [TOP2B] gene functions as the target for several anticancer agents and a variety of mutations in this gene have been associated with the development of drug resistance. Only one gene, FLT-3, was found to be constantly more expressed in NDCT ALL children who have relapsed. This study, to the best of our knowledge, is the first to demonstrate that over expression of FLT3 in pediatric high risk NDCT ALL, is associated with relapse. FLT3 suppression with a specific tyrosine kinase inhibitor may be tried as a new therapeutic agent in patients with relapsed ALL

smallest miracle baby that survived in Oman

Extreme prematurity is a major cause of perinatal morbidity and mortality. Management of such babies is a big challenge to neonatologists. Here we report the smallest baby ever survived in Oman

Visceral leishmaniasis and haemophagocytic syndrome in an Omani child

The paper reports the case of a previously healthy 4-year-old-girl who presented with pallor, fever and hepatosplenomegaly. Laboratory findings included pancytopenia, hypertriglyceridemia and hyperferritinemia. Initial diagnosis of kala-azar could not be confirmed because of the absence of clinical evidence, negativity of bone marrow aspiration or specific serology for visceral leishmaniasis. Repeated marrow aspiration, performed due lack of clinical response, revealed histiocytes showing haemophagocytosis consistent with haemophagocytic lymphohistocytosis [HLH] and appropriate treatment was started. She continued to have high-grade fever, and a third bone marrow aspiration ultimately revealed presence of Leishmania amastigotes with evidence of active haemophagocytosis. The girl was treated with liposomal amphotericin [AmBisome] for 5 days, following which she recovered rapidly with definitive remission

Hemophagocytic lymphohistiocytosis (HLH) presenting on the 3rd day of life.

Hemophagocytic lymphohistiocytosis (HLH) embraces the frequently indistinguishable conditions, namely familial hemophagocytic lymphohistiocytosis (FHLH), sporadic hemophagocytic lymphohistiocytosis (SHLH) and virus associated hemophagocytic syndrome (VAHS). The disease is very rare and invariably lethal. Evidence suggests that the disease may be due to an inherited defect in immunoregulation that predisposes to an uncontrolled proliferation of activated histiocytes in response to a stimulus such as viral infection. We report here a 3-day-old neonate with confirmed HLH who had a stormy course and a fatal outcome to the disease process, in spite of early chemotherapy. To our knowledge, we believe this is the youngest reported case of HLH from Middle East. No familial or infective cause could be attributed.

Management of chronic idiopathic thrombocytopenic purpura in childhood: a dilemma

Chronic ITP in children is generally a benign condition but its management is fraught with worry and controversy. Numerous reviews and the large number of therapeutic approaches attest to the uncertainty about the most effective and safe treatment modality and highlights the lack of knowledge about its natural history. Few patients develop serious complications or long term sequel. Steroids or IVIg may benefit some patients who suffer form clinical bleeding. Before resorting to splenectomy in severe resistant cases it may be worthy to try a course of pulsed high dose dexamethasone therapy. The decision to treat a child with chronic ITP should be individualized according to the clinical condition rather than the platelet count alone