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1.
Pediatr Res ; 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38575692

RESUMO

BACKGROUND: Beta-thalassemia major (ß-TM) patients are more likely to experience blood glucose intolerance and to date; the blood markers that could evaluate this are debatable. So, this study aimed to assess the roles of glycated hemoglobin A1c (HbA1c) and fructosamine in evaluating glucose intolerance in children with ß-TM and figuring out role of insulin resistance in these patients. METHODS: One hundred children diagnosed with ß-TM and 100 age and sex-matched controls were enrolled. Fasting plasma glucose (FPG), 2-h post-prandial blood glucose (2-h PG), HbA1c, fructosamine, fasting insulin level (FINS), insulin resistance index (HOMA-IR), and insulin sensitivity index (HOMA-IS) were evaluated. RESULTS: FPG and 2-h PG revealed glucose intolerance in 51 patients (51%), 19 of them had diabetes mellitus. HbA1c, fructosamine, FINS, and HOMA-IR showed a high statistically significant increase in patients compared to controls, (P < 0.001). Results revealed fructosamine was more specific in detecting prediabetes state and more sensitive in identifying diabetes mellitus in our patients when compared to HbA1c. CONCLUSIONS: Despite controversies on HbA1c in children with ß-TM, it is still valuable in glucose intolerance detection. Fructosamine showed more sensitivity and specificity. Furthermore, insulin resistance was prevalent in children with ß-TM highlighting the necessity of regular glycemic state evaluation. IMPACT: Glucose intolerance is a common complication in beta thalassemia patients. Conflicting data was reported about the role of HbA1c and fructosamine in evaluating glucose intolerance in thalassemic patients. Fructosamine does not yet have a threshold that may be used to distinguish between patients who have diabetes mellitus and those who do not. Fructosamine was more specific in detecting blood glucose intolerance compared to HbA1c and was more sensitive for diagnosing diabetes mellitus. Insulin resistance was common in patients with beta-thalassemia and often present before the onset of overt diabetes.

2.
Clin Transl Sci ; 14(1): 373-381, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33048456

RESUMO

Organic anion transporting polypeptide (OATP) 1B1 and OATP1B3 are involved in the disposition of a variety of commonly prescribed drugs. The evaluation of OATP1B1/1B3 inhibition potential by investigational drugs is of interest during clinical drug development due to various adverse events associated with increased exposures of their substrates. Regulatory guidance documents on the in vitro assessment of OATP1B1/1B3 inhibition potential are conservative with up to a third of predictions resulting in false positives. This work investigated the utility of OATP1B1/1B3 endogenous biomarkers, coproporphyrin (CP)-I and CP-III, to assess clinical inhibition of OATP1B1/1B3 and potentially eliminate the need for prospective clinical drug-drug interaction (DDI) studies. Correlations between CP-I exposures and various OATP1B1 static DDI predictions were also evaluated. Glecaprevir/pibrentasvir (GLE/PIB) 300/120 mg fixed-dose combination is known to cause clinical inhibition of OATP1B1/1B3. In a clinical study evaluating the relative bioavailability of various formulations of GLE/PIB regimen, CP-I peak plasma concentration (Cmax ) ratio and 0-16-hour area under the concentration-time curve (AUC0-16 ) ratio relative to baseline increased with increasing GLE exposures, whereas there was a modest correlation between GLE exposure and CP-III Cmax ratio but no correlation with CP-III AUC0-16 ratio. This suggests that CP-I is superior to CP-III as an endogenous biomarker for evaluation of OATP1B1 inhibition. There was a significant correlation between CP-I and GLE Cmax (R2  = 0.65; P < 0.001) across individual subjects. Correlation analysis between GLE OATP1B1 R values and CP-I exposures (Cmax ratio and AUC0-16 ratio) suggests that an R value of > 3 can predict a biologically meaningful inhibition of OATP1B1 when the inhibitor clinical pharmacokinetic parameters are available.


Assuntos
Benzimidazóis/farmacocinética , Biomarcadores Farmacológicos/sangue , Coproporfirinas/sangue , Transportador 1 de Ânion Orgânico Específico do Fígado/antagonistas & inibidores , Pirrolidinas/farmacocinética , Quinoxalinas/farmacocinética , Sulfonamidas/farmacocinética , Adulto , Área Sob a Curva , Benzimidazóis/administração & dosagem , Disponibilidade Biológica , Biomarcadores Farmacológicos/metabolismo , Coproporfirinas/metabolismo , Estudos Cross-Over , Combinação de Medicamentos , Interações Medicamentosas , Monitoramento de Medicamentos/métodos , Feminino , Voluntários Saudáveis , Humanos , Transportador 1 de Ânion Orgânico Específico do Fígado/metabolismo , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Pirrolidinas/administração & dosagem , Quinoxalinas/administração & dosagem , Sulfonamidas/administração & dosagem , Adulto Jovem
3.
Cureus ; 12(10): e10825, 2020 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-33173632

RESUMO

Multiple sclerosis (MS) is a chronic autoimmune disease that causes demyelination of the central nervous system. No treatment has been shown to be curative; thus, we assume that the tendency for patients with MS to use unconventional therapies, such as complementary and alternative medicine (CAM), might increase. The aim of this study was to explore the pattern of CAM use among patients with MS at a tertiary health care center in Saudi Arabia (SA). This was a questionnaire-based observational cross-sectional study that targeted adult patients diagnosed with MS at King Abdulaziz Medical City in Riyadh, SA, from 2018 to 2019. The sample size included 176 patients, and a consecutive non-probability sampling technique was used to engage them during their appointments. An Arabic questionnaire was used to evaluate patients' use of CAM. The mean age of participants was 34.6 ± 10.9 years, females represented the majority 125 (71%) of participants, and 89% of the participants reported using CAM at least once, with one or more modalities being used. Prayer, Salat, was the most frequent modality (60%) followed by supplication, Dua'a (59%), Ruqia, reciting Holy Quran (52%), and vitamins (44%). Symptomatic improvement was reported by 49 (27.8%) of dietary supplement users and 81 (46%) of non-dietary supplement medicine users. The study found a high prevalence of CAM utilization among Saudi adult patients with MS, which exceeded internationally reported rates. Although some patients described some improvement in their symptoms, further research is needed to evaluate the effectiveness of CAM.

4.
PLoS One ; 14(4): e0214521, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30933991

RESUMO

Staphylococcus aureus is a Gram-positive bacterial pathogen of global concern and a leading cause of bacterial infections worldwide. Asymptomatic carriage of S. aureus on the skin and in the anterior nares is common and recognized as a predisposing factor to invasive infection. Transition of S. aureus from the carriage state to that of invasive infection is often accompanied by a temperature upshift from approximately 33°C to 37°C. Such a temperature shift is known in other pathogens to influence gene expression, often resulting in increased production of factors that promote survival or virulence within the host. One mechanism by which bacteria modulate gene expression in response to temperature is by the regulatory activity of RNA-based thermosensors, cis-acting riboregulators that control translation efficiency. This study was designed to identify and characterize RNA-based thermosensors in S. aureus. Initially predicted by in silico analyses of the S. aureus USA300 genome, reporter-based gene expression analyses and site-specific mutagenesis were performed to demonstrate the presence of a functional thermosensor within the 5' UTR of cidA, a gene implicated in biofilm formation and survival of the pathogen. The nucleic sequence composing the identified thermosensor are sufficient to confer temperature-dependent post-transcriptional regulation, and activity is predictably altered by the introduction of site-specific mutations designed to stabilize or destabilize the structure within the identified thermosensor. The identified regulator is functional in both the native bacterial host S. aureus and in the distally related species Escherichia coli, suggesting that its regulatory activity is independent of host-specific factors. Interestingly, unlike the majority of bacterial RNA-based thermosensors characterized to date, the cidA thermosensor facilitates increased target gene expression at lower temperatures. In addition to the characterization of the first RNA-based thermosensor in the significant pathogen S. aureus, it highlights the diversity of function within this important class of ribo-regulators.


Assuntos
Regiões 5' não Traduzidas , Proteínas de Bactérias/química , Proteínas de Bactérias/genética , RNA Bacteriano/genética , Staphylococcus aureus/genética , Temperatura , Biofilmes , Escherichia coli/metabolismo , Perfilação da Expressão Gênica , Regulação Bacteriana da Expressão Gênica , Genes Reporter , Genoma Bacteriano , Humanos , Mutagênese Sítio-Dirigida , Estrutura Secundária de Proteína , RNA/análise , Processamento Pós-Transcricional do RNA , Infecções Estafilocócicas/microbiologia , Virulência , Fatores de Virulência/genética
5.
Bioresour Technol ; 265: 399-406, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29933187

RESUMO

In this study, the effect of pre-treatments including alkaline, acid and hydrogen peroxide on continuous hydrogen and methane production was investigated. Two different substrates as potatoes and bean wastes were used. Pre-treatment showed positive effect on bio-hydrogen and bio-methane production; higher bio-hydrogen and bio-methane production results using pre-treated samples than the control bioreactors (without pre-treatment), were recorded. In case of potatoes wastes, the hydrogen yield ranged between 126.4 and 252.7 mL-H2/g-TVS using pre-treated samples compared to 58.7 mL-H2/g-TVS observed in the reference test. Pre-treated bean wastes showed hydrogen yield of 93.0-152.1 mL-H2/g-TVS higher than 53.3 mL-H2/g-TVS measured in the control test. In the second stage, average methane yield results of 322.9-507.1 and 284.3-462.6 mL-CH4/g-TVS higher than 198.6 and 124.3 mL-CH4/g-TVS measured for potatoes and bean wastes control bioreactors, respectively. The best results were observed using H2O2 pre-treatment. The energy production efficiency was improved by combining H2 and CH4 bioreactors.


Assuntos
Reatores Biológicos , Fermentação , Hidrogênio , Metano/análise , Anaerobiose , Peróxido de Hidrogênio , Eliminação de Resíduos
6.
Am J Nucl Med Mol Imaging ; 8(2): 86-99, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29755842

RESUMO

Amyloid beta (Aß) plaques are not specific to Alzheimer's disease and occur with aging and neurodegenerative disorders. Soluble brain Aß may be neuroprotective and increases in response to neuroinflammation. Sepsis is associated with neurocognitive compromise. The objective was to determine, in a rat endotoxemia model of sepsis, whether neuroinflammation and soluble Aß production are associated with Aß plaque and hyperphosphorylated tau deposition in the brain. Male Sprague Dawley rats received a single intraperitoneal injection of 10 mg/kg of lipopolysaccharide endotoxin (LPS). Brain and blood levels of IL-1ß, IL-6, and TNFα and cortical microglial density were measured in LPS-injected and control animals. Soluble brain Aß and p-tau were compared and Aß plaques were quantified and characterized. Brain uptake of [18F]flutemetamol was measured by phosphor imaging. LPS endotoxemia resulted in elevations of cytokines in blood and brain. Microglial density was increased in LPS-treated rats relative to controls. LPS resulted in increased soluble Aß and in p-tau levels in whole brain. Progressive increases in morphologically-diffuse Aß plaques occurred throughout the interval of observation (to 7-9 days post LPS). LPS endotoxemia resulted in increased [18F]flutemetamol in the cortex and increased cortex: white matter ratios of activity. In conclusion, LPS endotoxemia causes neuroinflammation, increased soluble Aß and Aß diffuse plaques in the brain. Aß PET tracers may inform this neuropathology. Increased p-tau in the brain of LPS treated animals suggests that downstream consequences of Aß plaque formation may occur. Further mechanistic and neurocognitive studies to understand the causes and consequences of LPS-induced neuropathology are warranted.

7.
Pediatr Surg Int ; 32(5): 487-91, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26852298

RESUMO

BACKGROUND: Congenital H-type tracheoesophageal fistula (TEF) is very rare and represents <5 % of all congenital tracheoesophageal malformations. This is a national, multicenter review of our experience with isolated H-type TEF outlining clinical presentation, methods of diagnosis, associated anomalies, treatment and outcome PATIENTS AND METHODS: The medical records of all patients with the diagnosis of congenital H-type TEF treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, associated anomalies, method of diagnosis, treatment and outcome. RESULTS: During the study period (January 1998-December 2013), 435 infants and children with the diagnosis of esophageal atresia with or without TEF were treated. Among these, 23 (5.3 %) had isolated TEF. There were 11 males and 12 females. Their age at presentation ranged from 5 days to 3 years and 7 months but the majority (90 %) were diagnosed during their first year of life. Their clinical presentation included: chocking and coughing during feeds in 12 (52.2 %), recurrent chest infection in 16 (69.6 %) and cyanosis in 10 (43.5 %). One presented with abdominal distension also. The diagnosis was made using esophagogram. In 11 (47.8 %), a single study confirmed the diagnosis, 8 (34.8 %) required two studies while 4 (17.4 %) required three studies. Nineteen (82.6 %) had preoperative bronchoscopy and in 13 (56.5 %), a catheter was used to cannulate the fistula. All were operated through a right cervical incision except one who underwent thoracoscopic ligation and division of the fistula. In one, the fistula was only transfixed and tied without being divided. This patient developed a recurrent fistula. Two patients developed postoperative stridor secondary to recurrent laryngeal nerve palsy. In both of them, there was complete recovery. CONCLUSIONS: H-type TEF is very rare and commonly presents with recurrent chest infection, chocking and coughing during feeds and cyanosis. Physicians caring for these patients should be aware of this and a high index of suspicion is of paramount importance to avoid delay in diagnosis with its associated morbidity. A contrast esophagogram is valuable in confirming the diagnosis. The study however may need to be repeated. Preoperative bronchoscopy is valuable to localize and cannulate the fistula for easier access during surgery. Surgical repair is the treatment of choice and this should be performed through a right cervical incision or thoracotomy for low fistulae. Thoracoscopic ligation and division of a low H-type fistula is an alternative and less invasive approach when compared to thoracotomy.


Assuntos
Fístula Traqueoesofágica/epidemiologia , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/cirurgia
8.
J Pediatr Hematol Oncol ; 38(1): 1-4, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24577545

RESUMO

Splenomegaly and hypersplenism are common complications among children with thalassemia necessitating splenectomy. Thirty-six children (27 ß-thalassemia major, 3 Hb H disease, and 6 thalassemia intermediate) had total splenectomy (11 laparoscopic and 13 open splenectomy) or partial splenectomy (12 patients). In the partial splenectomy group, 2 with Hb H required no transfusions. For those with ß-thalassemia major who had partial splenectomy (9 patients), there was a reduction in the number of transfusions from a preoperative mean of 15.2 transfusions per year to a postoperative mean of 8.2 transfusions per year. Subsequently and as a result of increase in the size of splenic remnant, their transfusions increased, but none required total splenectomy. Twenty-four patients had total splenectomy (13 open and 11 laparoscopic splenectomy). Their postsplenectomy transfusions decreased from a preoperative mean of 17.8 transfusions per year to a postoperative mean of 10 transfusions per year. There was no mortality, and none developed postoperative sepsis or thrombotic complications. Total splenectomy is beneficial for children with ß-thalassemia major and hypersplenism by reducing their transfusion requirements. Laparoscopic splenectomy is however more beneficial. Partial splenectomy reduces their transfusion requirements, but only as a temporary measure, and so it is recommended for children younger than 5 years of age.


Assuntos
Esplenectomia/métodos , Talassemia/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Laparoscopia , Masculino , Estudos Retrospectivos
10.
J Pediatr Surg ; 49(7): 1078-82, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24952792

RESUMO

BACKGROUND: Congenital pyloric atresia (CPA) is a very rare anomaly. It is usually seen as an isolated condition with excellent prognosis. Few cases are familial. These are usually associated with other hereditary conditions and have a poor prognosis. This is a review of our experience with 20 patients with CPA outlining aspects of diagnosis, associated anomalies and management. PATIENTS AND METHODS: This is a retrospective analysis of 20 cases seen over a 22 year period (December 1990 to December 2012). Their records reviewed for: age, sex, presentation, prenatal history, associated anomalies, investigations, treatment, operative findings and the outcome. RESULTS: 20 cases (9 Males, 11 Females) were treated. 7 patients were full term and the remaining 13 were prematures. Their mean birth weight was 2.1 kg (1.1 kg to 3.9 kg). Polyhydramnios was seen in 13 patients (65%). Two were brothers and four were members of the same family. Isolated CPA was seen in 7 (35%); 13 had an associated conditions: epidermolysis bullosa (EB) in 8 (40%) and multiple intestinal atresias (MIA) in 5 (25%). Three patients had associated esophageal atresia. All were operated on except two who died early due to unrelenting sepsis. The variety of pyloric atresias encountered were as follows: pyloric diaphragm in 13 including double diaphragms in 2, pyloric atresia with a gap in 4 and pyloric atresia without gap in 3. Ten died postoperatively giving an overall survival of 40%. CONCLUSIONS: CPA is a very rare condition. Isolated CPA carries a good prognosis. Association of CPA with other familial and congenital anomalies like EB and MIA carries a poor prognosis.


Assuntos
Obstrução da Saída Gástrica/congênito , Obstrução da Saída Gástrica/cirurgia , Piloro/anormalidades , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/cirurgia , Feminino , Obstrução da Saída Gástrica/diagnóstico , Humanos , Recém-Nascido , Masculino , Complicações Pós-Operatórias , Piloro/cirurgia , Estudos Retrospectivos , Sepse/etiologia , Resultado do Tratamento
11.
J Pediatr Surg ; 49(4): 503-7, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24726101

RESUMO

BACKGROUND: Congenital Morgagni's hernia (CMH) is rare and represents less than 5% of all congenital diaphragmatic hernias. This is a national review of our experience with CMH outlining clinical presentation, methods of diagnosis, associated anomalies, treatment, and outcome. PATIENTS AND METHODS: The medical records of all patients with the diagnosis of CMH treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, associated anomalies, diagnosis, operative findings, treatment, and outcome. RESULTS: During a 20-year period (January 1990-December 2010), 53 infants and children with CMH were treated. There were 38 males and 15 females. Their age at diagnosis ranged from 1 month to 9 years (mean 22.2 months). Forty-three (81%) presented with recurrent chest infection. Twenty-two (44.5%) had right CMH, 15 (28.3%) had left-sided hernia and 16 (30.2%) had bilateral hernia. In 7, the diagnosis of bilaterality was made at the time of surgery. Associated anomalies were seen in 38 (71.7%). Twenty-one (39.6%) had congenital heart disease, 8 (15%) had malrotation, and 15 (28.3%) had Down syndrome. All were operated on. Twenty-nine (54.7%) underwent repair via an open approach. The remaining 24 (45.3%) underwent repair using minimal invasive surgery, laparoscopic-assisted hernia repair (19 patients) or totally laparoscopic approach (5 patients). At the time of surgery, the hernia sac content included the colon in 33 (62.3%), part of the left lobe of the liver in 13 (24.5%), the small intestines in 11 (20.75%), the omentum in 5 (9.4%), and the stomach in 4 (7.5%). In 12 (22.6%), the hernia sac was empty. When compared to the open repair, the laparoscopic-assisted approach was associated with a shorter operative time, an earlier commencement of feeds, less requirement for postoperative analgesia, a shorter hospital stay, and better cosmetic appearance. There was no mortality. On follow-up, 2 (7%) of the open surgical group developed recurrence. CONCLUSIONS: CMH is rare and in the pediatric age group commonly presents with recurrent chest infection and has a high incidence of associated anomalies, commonly congenital heart disease and Down syndrome. We advocate a laparoscopic-assisted approach to repair CMH. This is a simple technique that produces a sound repair, and when compared with the open approach it takes less operative time, requires less analgesia, allows earlier commencement of feeds, is associated with a shorter hospital stay, and has a better cosmetic outcome.


Assuntos
Hérnias Diafragmáticas Congênitas/cirurgia , Herniorrafia/métodos , Laparoscopia , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/cirurgia , Criança , Pré-Escolar , Feminino , Seguimentos , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/diagnóstico , Humanos , Lactente , Masculino , Estudos Retrospectivos , Arábia Saudita , Resultado do Tratamento
12.
Surg Laparosc Endosc Percutan Tech ; 24(1): e38-40, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24487175

RESUMO

Segmental omental infarction (SOI) is an unusual and rare cause of abdominal pain in children and is often mistaken for acute appendicitis preoperatively. SOI once diagnosed preoperatively should be treated conservatively. Surgical intervention, however, may be necessary in order to establish definitive diagnosis and treatment. We present 2 children with SOI diagnosed as acute appendicitis preoperatively. Laparoscopy established the diagnosis and treatment. Laparoscopy should be the procedure of choice in children presenting with right-sided abdominal pain that is not well established preoperatively. This is especially so in obese children. Laparoscopy not only establishes the diagnosis of SOI but laparoscopic omentectomy results in resolution of symptoms and faster recovery.


Assuntos
Dor Abdominal/etiologia , Dor Aguda/etiologia , Infarto/diagnóstico , Omento/irrigação sanguínea , Doenças Peritoneais/complicações , Doenças Peritoneais/diagnóstico , Apendicite/diagnóstico , Criança , Diagnóstico Diferencial , Humanos , Infarto/complicações , Infarto/cirurgia , Masculino , Doenças Peritoneais/cirurgia
13.
J Chemother ; 26(2): 80-5, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24090676

RESUMO

Moxifloxacin has potent bactericidal activity against Streptococcus pneumoniae; a major causative organism of lower respiratory tract infections. This study aims to use the pharmacokinetic/pharmacodynamic indices to predict the therapeutic outcome under different scenarios of moxifloxacin exposure and pneumococcal resistance. STELLA(®) software was used to simulate the pharmacokinetics and pharmacodynamics of moxifloxacin in patients with severe pneumonia and acute exacerbations of chronic bronchitis (AECB). The current dose of moxifloxacin was found to be insufficient for eradication of ciprofloxacin resistant bacteria in ventilated patients with severe bronchopneumonia. This can be attributed to the lower tissue penetration observed in this population. Increasing the dose to 600 mg was able to achieve higher levels of free drug AUC/MIC in both bronchial and plasma compartments. In AECB, moxifloxacin achieved the same AUC/MIC values observed in pneumonia at the different MIC values. This may allow the extrapolation of findings of moxifloxacin studies in pneumonia to the management of patients with AECB.


Assuntos
Fluoroquinolonas/farmacologia , Infecções Respiratórias/tratamento farmacológico , Área Sob a Curva , Broncopneumonia/tratamento farmacológico , Ciprofloxacina/farmacologia , Infecções Comunitárias Adquiridas/tratamento farmacológico , Infecções Comunitárias Adquiridas/metabolismo , Infecções Comunitárias Adquiridas/microbiologia , Farmacorresistência Bacteriana/efeitos dos fármacos , Fluoroquinolonas/farmacocinética , Humanos , Moxifloxacina , Infecções Pneumocócicas/tratamento farmacológico , Infecções Pneumocócicas/metabolismo , Infecções Pneumocócicas/microbiologia , Infecções Respiratórias/metabolismo , Infecções Respiratórias/microbiologia , Streptococcus pneumoniae/efeitos dos fármacos
14.
15.
Pediatr Surg Int ; 29(3): 281-5, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23184265

RESUMO

BACKGROUND: Massive splenic infarction (MSI) is a very rare condition. Few reports of splenic infarction of various etiologies including hematological and non-hematological causes have been published. On the other hand, MSI in patients with sickle cell anemia (SCA) is extremely rare. This report describes our experience with 15 children with SCA and MSI outlining aspects of presentation, diagnosis and management. PATIENTS AND METHODS: The records of all children with MSI were retrospectively reviewed for age at diagnosis, sex, clinical features, precipitating factors, investigations, management and outcome. RESULTS: 15 children (11 M: 4 F) with SCA were treated for MSI. Their mean age was 10.9 years (6-17 years). All presented with severe left upper quadrant abdominal pain. In nine, this was associated with nausea and vomiting. Three were febrile and all had a tender splenomegaly. Their mean hemoglobin was 8.2 g/dl (5.7-11.3 g/dl), mean WBC was 10.97 × 10(3) mm(-3) (3.6 × 10(3)-22.3 × 10(3) mm(-3)) and mean platelet count was 263.3 × 10(3) mm(-3) (40 × 10(3)-660 × 10(3) mm(-3)). In seven, there was a precipitating cause including high altitude in two, acute chest syndrome in two, septicemia in two and severe vasooclusive crisis in one. Abdominal ultrasound and CT scan confirmed the diagnosis of MSI which involved more than half of the spleen in 12 and whole spleen in 3. All were treated with IV fluids, analgesia and blood transfusion where appropriate. Eleven had splenectomy because of persistent abdominal pain, three developed splenic abscess and underwent splenectomy and one settled on conservative treatment. Histology confirmed the diagnosis of splenic infarction in 11 and infarction with abscess in the remaining 3. CONCLUSION: MSI is extremely rare in children with SCA. It can develop spontaneously or precipitated by other factors namely high altitude, acute chest syndrome and severe stress. Most reported cases of splenic infarction are small in size, focal and can be treated conservatively. MSI, on the other hand, may necessitate splenectomy for persistent symptoms or in case of complications, such as abscess formation.


Assuntos
Anemia Falciforme/complicações , Esplenectomia , Infarto do Baço/cirurgia , Dor Abdominal/etiologia , Abscesso/complicações , Abscesso/cirurgia , Síndrome Torácica Aguda/complicações , Adolescente , Altitude , Analgésicos/uso terapêutico , Transfusão de Sangue , Criança , Feminino , Febre/etiologia , Hidratação , Hemoglobinas/análise , Humanos , Masculino , Náusea/etiologia , Contagem de Plaquetas , Estudos Retrospectivos , Sepse/complicações , Infarto do Baço/diagnóstico , Infarto do Baço/etiologia , Esplenomegalia/etiologia , Doenças Vasculares/complicações , Vômito/etiologia
16.
World J Pediatr ; 9(1): 80-3, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21874612

RESUMO

BACKGROUND: Survivors of childhood malignancies are known to be at an increased risk for developing a variety of secondary cancers. Primary adenocarcinoma of the colon is very rare in children and adenocarcinoma of the colon occurring as a secondary malignancy in children is much rarer. METHODS: A boy with a history of successfully treated embryonal rhabdomyosarcoma developed adenocarcinoma of the colon as a secondary cancer. RESULTS: The boy presented with a solid mass of the left cheek at 3 years of age. The mass was excised and histological examination showed embryonal rhabdomyosarcoma. He was treated with multi-agent chemotherapy and local radiotherapy, which resulted in complete remission. Four years later, he presented with recurrent colicky abdominal pain and bleeding per rectum and was found to have intussusceptions. Colonoscopy revealed a tumor in the transverse colon, which was biopsied and proved to be an adenocarcinoma. The boy underwent excision followed by chemotherapy using an adult colon cancer regimen. He is currently off chemotherapy for 2 years with no evidence of the disease. CONCLUSIONS: We report a rare case of colon cancer after treatment of rhabdomyosarcoma. Colorectal adenocarcinoma must be kept in mind as a secondary neoplasm following treatment for early childhood malignancies although it is extremely rare.


Assuntos
Adenocarcinoma/diagnóstico , Neoplasias do Colo/diagnóstico , Neoplasias Faciais/cirurgia , Segunda Neoplasia Primária/diagnóstico , Rabdomiossarcoma Embrionário/cirurgia , Bochecha , Pré-Escolar , Humanos , Masculino
17.
Pediatr Surg Int ; 28(12): 1229-33, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23076455

RESUMO

Achalasia is rare in the pediatric age group and in most cases it is idiopathic with no family history. Familial achalasia is very rare. This report describes two families with achalasia: in one, six children were affected while in the other a brother and a sister had Allgrove's syndrome (triple-A syndrome consisting of achalasia, adrenal insufficiency, and alacrima). Familial achalasia suggests that it is hereditary and may be transmitted as an autosomal recessive trait. The management of achalasia in children is still controversial. With the recent advances in minimal invasive surgery, laparoscopic Heller's myotomy is the procedure of choice in the management of achalasia in children.


Assuntos
Acalasia Esofágica/genética , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Linhagem
18.
Gastroenterology Res ; 5(4): 162-166, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27785198

RESUMO

The majority of common bile duct stones can be effectively treated by endoscopic sphincterotomy and stone extraction using basket or balloon extractor. Stones more than 2 cm in diameter on the other hand require mechanical, electrohyraulic lithotripsy and sphincterotomy and balloon dilation. Mechanical lithotripsy may not be successful because of the size, consistency and site of the stones. In these cases, laser lithotripsy is the treatment of choice. This however requires direct visualization of the stone which may not be feasible for impacted cystic duct stones. This report describes the successful treatment of difficult cystic duct stones in two patients with Mirizzi's syndrome type I using per oral Spyglass and intraductal holmium: YAG Laser Lithotripter.

19.
Pediatr Surg Int ; 28(7): 687-91, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22622517

RESUMO

BACKGROUND: Traumatic diaphragmatic hernia (TDH) is very rare in the pediatric age group. Because of its rarity and its coexistence with more serious injuries, the diagnosis is often missed or delayed. PATIENTS AND METHODS: All children with the diagnosis of TDH were retrospectively reviewed for age at diagnosis, sex, mechanism of injury, site of diaphragmatic rupture, associated injuries, injury severity score, investigations, treatment and outcome. RESULTS: Over a period of 15 years (1992-2007), we treated seven children with TDH. All were males except one. Their mean age was 7.4 years (3.75-14 years). The site of TDH was on the left in three, on the right in three and one had bilateral TDH. The mechanism of injury was blunt trauma in six and penetrating injury from a stab in one. Three had right TDH and in all three, the diagnosis of TDH was delayed. All sustained severe injuries as reflected by the ISS score (mean 38.6). The time from presentation to diagnosis was variable ranging from 3 to 240 h (mean 65.7 h). The three patients with left TDH were repaired trans-abdominally while the three with right TDH were repaired via a right thoracotomy. The patient with bilateral TDH required laparotomy and thoracotomy. All our patients survived and the hospital stay was variable ranging from 7 to 24 days (mean 17 days). CONCLUSIONS: TDH is very rare in children and often associated with severe more serious injuries. These two factors contribute to the delay in diagnosis. This is specially so for right TDH. To obviate delay in diagnosis, it is important to have a high index of suspicion as well as careful interpretation of the initial and follow-up radiological investigations including CT-scan of the chest and abdomen. This is important to detect a slowly increasing TDH that may not be apparent initially.


Assuntos
Hérnia Diafragmática Traumática/diagnóstico por imagem , Hérnia Diafragmática Traumática/cirurgia , Ferimentos não Penetrantes/diagnóstico por imagem , Ferimentos não Penetrantes/cirurgia , Ferimentos Penetrantes/diagnóstico por imagem , Ferimentos Penetrantes/cirurgia , Adolescente , Criança , Pré-Escolar , Diafragma/diagnóstico por imagem , Diafragma/cirurgia , Feminino , Humanos , Escala de Gravidade do Ferimento , Tempo de Internação/estatística & dados numéricos , Masculino , Estudos Retrospectivos , Terapêutica , Toracotomia/métodos , Tomografia Computadorizada por Raios X/métodos
20.
Saudi Med J ; 33(2): 172-6, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22327758

RESUMO

OBJECTIVE: To evaluate the safety of Bacillus Calmette-Guerin (BCG) in Saudi infants and outline our management for BCG related lymphadenitis. METHODS: The records of infants who developed BCG related complications were retrospectively reviewed from March 2008 to March 2011 at the Maternity and Children Hospital, Dammam, Saudi Arabia for age, gender, birth weight, presentation, and outcome. All our patients were immunized with the BCG vaccine within 48 hours after birth, and the total number of vaccinated newborns was obtained from the vaccination registry. RESULTS: During a 3-year period (March 2008 to March 2011), 26,000 newborns received BCG and 81 (51 males and 30 females) developed complications. This gives an incidence of 3.12 complications/1000 newborns. Their presentations were: left axillary lymphadenitis (n=62), supraclavicular lymphadenitis (n=9), collection at immunization site (n=6), and one each (left cervical lymphadenitis, bilateral axillary lymphadenitis, left arm abscess, left axillary lymphadenitis and collection at immunization site). Two were immunocompromized and 6 with local collection were aspirated. The arm abscess had drainage. Simple lymphadenitis (n=6) were treated expectantly, while those with suppurative lymphadenitis (n=68) had excision (n=65) or incision and drainage (n=3) without anti-tuberculous treatment. CONCLUSION: Bacillus Calmette-Guerin is safe but is associated with a relatively high incidence of suppurative lymphadenitis. Non-suppurative lymphadenitis can be treated conservatively, while suppurative lymphadenitis should be treated with excision. This is safe, avoids rupture, and shortens the recovery period without anti-tuberculous treatment. Although, the use of BCG vaccine may be associated with side effects, the potential morbidity and mortality from tuberculosis outweighs that from BCG related complications.


Assuntos
Adjuvantes Imunológicos/efeitos adversos , Vacina BCG/efeitos adversos , Linfadenite/induzido quimicamente , Supuração/induzido quimicamente , Tuberculose/prevenção & controle , Feminino , Humanos , Recém-Nascido , Masculino , Estudos Retrospectivos , Arábia Saudita
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