Pertussis and pertussis like illness: Pediatric experience in Oman

Objectives: Aresurgence of pertussis or whooping cough has been observed worldwide despite broad vaccination coverage. Pertussis like illness [PLI] refers to a clinical syndrome compatible with pertussis infection but lacking laboratory confirmation or an epidemiological link to a confirmed case. Our study aimed to estimate the contribution of Bordetella pertussis infection and identifying predictors of its diagnosis in a cohort of children with PLI

Methods: Demographic and clinical information were retrospectively collected from the medical records of children < 13 years old and hospitalized for PLI in two pediatric units in Oman from 1 January 2012 to 31 December 2013. The laboratory data of all cases were reviewed and confirmed cases of pertussis were identified, analyzed, and compared with non-confirmed cases

Results: A total of 131 patients were enrolled in this study. The majority [95.4% [125/131]] were infants. Only 54.1% [71/131] of admitted children with PLI were tested for pertussis. The incidence of pertussis infection among the tested group was 16.9% [12/71] with a 95% confidence interval 8.2?25.6. Severe illness occurred in 56.4% [74/131] of patients, and six were confirmed to have pertussis. Pediatric intensive care unit admission was required for one confirmed case of pertussis and eight cases from the PLI group [three were negative for pertussis, and five were not tested]. Receiver operator characteristic curve analysis revealed that a white blood cell count >/= 23.5 × 10[9]/L had 96.6% specificity and lymphocytes >/= 17 × 10[9]/L had 98.3% specificity

Conclusions: Taking into consideration that the number tested for pertussis was limited, the incidence of pertussis was 16.9% [12 out of 71 patients]. Lymphocytosis can be used as a reliable predictor for the diagnosis of pertussis especially in the absence of specific confirmatory tests or until their results are available

Human parvovirus B19 in children with sickle cell disease; poking the spleen

Parvovirus is a known culprit of transient red cell aplasia [TRCA] in children with sickle cell disease [SCD]. Few reports have previously described the association between the virus and acute splenic sequestration crisis [ASSC] in the same patient. Here, we are shedding light on such a potentially serious combination by reporting two cases of siblings with SCD complicated with concurrent ASSC and TRCA and presenting a review of the relevant literature

Single breath-hold physiotherapy technique effective tool for T2[asterisk] magnetic resonance imaging in young patients with thalassaemia major

Magnetic resonance imaging using T2[asterisk] [MRI T2[asterisk]] is a highly sensitive and non-invasive technique for the detection of tissue iron load. Although the single breath-hold multi-echo T2[asterisk] technique has been available at the Sultan Qaboos University Hospital [SQUH], Muscat, Oman, since 2006, it could not be performed on younger patients due to their inability to hold their breath after expiration. This study was carried out between May 2007 and May 2015 and assessed 50 SQUH thalassaemic patients aged 7-17 years old. Seven of these patients underwent baseline and one-year follow-up MRI T2[asterisk] scans before receiving physiotherapy training. Subsequently, all patients were trained by a physiotherapist to hold their breath for approximately 15-20 seconds at the end of expiration before undergoing baseline and one-year follow-up MRI T2[asterisk] scans. Failure rates for the pre- and post-training groups were 6.0% and 42.8%, respectively. These results indicate that the training of thalassaemic patients in breathhold techniques is beneficial and increases rates of compliance for MRI T2[asterisk] scans

overlooked complication of vancomycin induced acute flaccid paralysis in a child with acute leukemia: a case report

Vancomycin is a glycopeptide antibiotic which is commonly used to treat methicillin-resistant staphylococcal infections. It is commonly used in pediatric oncology wards for children with febrile neutropenia. We report a very rare side effect of vancomycin induced myopathy in a child with acute lymphoblastic leukemia. To the best of our knowledge, this is the first case reported from Oman

Vehicular entrapment and heat stroke in three children: is it a form of child neglect?

The medical records of three children who were entrapped inside vehicles are reviewed and their outcome following the incidents were assessed in this report. The children developed heat stroke following the incidents and survived after several days in coma but with severe cognitive functions impairment. Two of the children were left with hyperactivity and attention deficit, while the third had active epilepsy. Vehicular entrapment heat stroke is one of the preventable brain injuries in children. Several children get entrapped in cars or other vehicles yearly and survivors are left with significant brain damage. The usual cause for brain damage is heat stroke the lesson learned was to never leave children unattended in cars. Therefore, it is essential to double check that doors are locked when leaving children unattended near vehicles

Agranulocyosis in beta thalassemia major patients treated with oral iron chelating agent [deferiprone]

Deferiprone is an oral chelating agent that has been recently shown to reduce cardiac siderosis, but is also known to be associated with serious side effects like agranulocytosis which can be fatal. This report is a single centre experience of 5 cases with severe agranulocytosis in amongst 144 patients [3.47%] of thalassemia major on combined chelation therapy with subcutaneous desferrioxamine and oral deferiprone which is much higher than the previous reports

Clinical significance of soluble transferrin receptors in hypochronic microcytic anaemias

The interaction of plasma transferrin with specific membrane transferrin receptors in erythroid precursors is crucial for the process of iron delivery to cells. During this process, soluble truncated monomers of membrane transferrin receptors [sTfRs] are delivered in the serum and their levels appear to be related to the number of erythroid precursors in the bone marrow and to the body iron status. Our aim was to determine the value of sTfRs in the diagnosis of hypochromic micro cytic anaemias. Levels of sTfRs were estimated in the serum of 45 paediatric patients presenting with hypochromic microcytic anaemia. Patients were divided into three groups according to their diagnosis [IDA, A CD and thalassaemia, each group consisting of 15 patients. Ten healthy age and sex matched controls were included in our study. Patients with iron deficiency anaemia [IDA] had significantly higher levels of sTfRs [mean=14.83 +/- 3.38 mg/U than normal controls [mean=4.1 +/- 1.l mg/L] and both patients with thalassaemia [mean=9.27 +/- 4.02 mg/U] and anaemia of chronic illness [ACD] [mean=2.43 +/- l.38 mg/U. sYfRs did not significantly differ in the group of ACD patients from normal controls. Although our thalassaemia patients showed lower sTfRs levels than patients with IDA, their levels were significantly higher than both A CD patients and controls. In thalassaemia patients with Hb F levels of>40%, there was a positive correlation between sTfR levels and the percentage of Hb F

Clinical and epidemiological characteristics of pituitary tumors using a web-based pituitary tumor registry in Oman

From a recently instituted web-based pituitary tumour registry at Sultan Qaboos University Hospital, Oman, this study explores the results of comprehensive clinical evaluation, hormonal levels, radiological evidence of pituitary mass lesion using magnetic resonance [MRI] and the different treatment modalities. All patients who were diagnosed with pituitary mass tumours in our tertiary care endocrinology clinic between January 1998 and February 2006 were registered in the Oman pituitary tumour registry. Two physicians performed hospital chart review and data entry. A total of 160 entries were made into the pituitary tumour registry. The overall mean age of the cohort was 32 +/- 12 years [age range 8-73 years]. The majority of registrations were female [n=114; 71%]. There were 81 patients with non-functioning adenomas [50.6%], 59 with prolactinoma [36.9%] eight with acromegaly [5%], seven with craniopharyngioma [4.4%], four with Cushing's disease [2.5%] and one with sarcoidosis [0.6%]. Sub-group analyses were done only for the subjects with the 3 most prevalent pituitary tumours [non-functioning adenomas, prolactinomas, and acromegaly]. The most prevalent symptoms are amenorrhea-galactorrhea [n=55; 37%], headache [n=31; 21%] and fatigue [n=23; 16%]. The most common treatment modality was medical [n=58; 39%], followed by observation [n=56; 38%], surgery [n=31; 21%] and surgery plus medical [n=3; 2%]. None of the patients in this registry are recorded to have died. To our knowledge, this is the first pituitary tumour registry in the Arabian Gulf countries using a web-based programme. This tumour registry will enable us to characterize clinical and the epidemiological features of pituitary tumours in the Sultanate of Oman

Cerebrospinal fluid involvement in a case of visceral leishmaniasis associated with hemophagocytic lymphohistiocytosis

Hemophagocytic Lymphohistiocytosis [HLH] implies a benign generalized histiocytic proliferate with erythrophagocytosis and it includes familial hemophagocytic lymphohistiocytosis and secondary hemophgocytosis. Spinal fluid changes like mild to moderate pleocytosis [most of the cells are lymphocytes and macrophages] and sometimes hemophagocytosis are seen in primary HLH but are not reported in secondary HLH. Here we report a case of a previously healthy 10 months old male infant who was diagnosed as familial HLH with evidence of CSF hemophagocytosis. He was started on the HLH 2004 treatment protocol with no improvement. A second bone marrow aspiration revealed leshmania donovani antibodies and he was started on anti-leishmania treatment with dramatic response.To the best of our knowledge, this is the first case of secondary HLH with evidence of hemophagocytosis in cerebrospinal fluid

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

Study of central nervous system involvement in children with acute leukemia in relation to different immunophenotypes before and during treatment

Central nervous system [CNS] involvement remains a problem in acute Ieukemias [AL] despite the fact that prophylaxis with intrathecal [IT], systemic high dose chemotherapy and radiotherapy have greatly reduced its incidence. The relationship between the immunophenotypes of AL and development of CNS disease is controversial. The present study included 40 children with newly diagnosed acute leukemia, 36 patients with ALL and 4 patients with AML. They were 23 males and 17 females. Their age ranged from 2-12 years with a mean age of 6.06 +/- 2.59 years. A panel of monoclonal antibodies were used for classifying the Ieukemias into different immunophenotypes. The most common immunophenotype of ALL cases [56%] was early pre-B [CD19, CD22 and CD10]. No CD10 [CALLA] negative cases were detected in our series. Two cases showed cytoplasmic immunoglobulin [Clg] indicating a more mature pre-B immunophenotype. T cell immunophenotype was detected in 14 cases mainly CD2, CD3 and CD7.AML immunophenotype was detected in 4 cases showing CD13 and CD33. Seven cases [17.5%] showed clinical symptoms and signs of CNS involvement at initial diagnosis whereas 8 cases [20%] showed blast cells in CSF. The majority [75%] of CNS disease cases [6 out of 8 cases] had a T cell immunophenotype, while 25% [2 out of 8 cases] were 8 cell lineage. The difference between the 2 groups was significant [P=<0.001]. During follow-up, 5 cases developed CNS relapse. Four of them [80%] were of T cell immunophenotype and only one case [20%] was of B cell lineage [pre 8 cell ALL]. Only one case of CNS relapse did not have CNS disease at initial diagnosis and was of T cell immunophenotype. Four of CNS relapse cases occurred early in the first year of the disease. In view of the poor prognosis of patients with CNS disease and relapse and the higher incidence of these events in T cell ALL children, these patients may need intensive systemic and CNS directed therapy to improve their outcome

Cerebrospinal fluid fibronectin versus thymidine kinase concentrations as predictors of CNS involvement in children with acute lymphoblastic leukemia

Acute Leukemias [AL] are the most common malignancy In childhood [33%] with acute Iymphoblestlc leukemia [ALL] constituting 75% of cases. Thirty years have elapsed since the first description of leukemic inhItration of central nervous system [CNS], however It still remains 8 problem In ALL. Fibronectin is a high molecular weight glycoprotein that Is detectable in the normal CSF at low concentration. Thymidine kinase is a cytoplasmIc scavenger enzyme present In dividing cells which converts thymidine Into thymidine monophosphate. It is usually undetectable In CSF of most normal individuals. We studied 20 newly diagnosed children with ALL [11 males and 9 females], none of them showed any evidence of CNS leukemia at diagnosis. Determination of thymidine kinase and Fibronectin concentrations In CSF of the studied cases was done at diagnosis. The studied children were followed up for an average of 2 years [range 1.6 - 25 years] after the start of Induction treatment. All of them echleved complete remission. During the treatment period 5 patients showed an evidence of CNS Involvement. The duration tiII CNS relapse ranged between 10 months and 12 months. Our results showed significantly higher Ievels of CSF thymidine kinase and fibronectin In patients who showed CNS disease men In those who had either remained on maintenance chemotherapy, completed the treatment or relapsed In other sites [R0. 0001 and P<.05 respectively]. Meningeal leukemia occurred In almost 70% of our patients [5 out of 7 patients] who showed CSF thymidine kinase values above the upper Limit of the normal range [1.4 U/microI.] recorded at diagnosis, while comparable levels were found In only 13% [2 out of 15 patients] without CNS relapse. Determination of levels of these markers [thymidine kinase and fibronectin] may be compiled with the more recent techniques that can Identify leukemic cells In CSF [as sensitive polymerase chain reaction assays] may help In prediction of patients with high risk of CNS relapse. These methods could be potentially useful in identifying sub-groups of ALL patients with no Initial CNS disease who require a more Intensive presymptanatic prophylactic CNS therapy