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1.
J Indian Assoc Pediatr Surg ; 21(4): 169-174, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27695208

RESUMEN

AIMS: To compare intravenous urography (IVU) and magnetic resonance urography (MRU) in the preoperative evaluation of pelvi-ureteric junction obstruction (PUJO) in children. MATERIALS AND METHODS: A total of 35 children up to 10 years of age in whom unilateral or bilateral PUJO were suspected on ultrasonography were enrolled in this prospective study. All children underwent IVU and MRU, and the findings were compared. RESULTS: Of the 70 kidneys evaluated, 14 (20%) were not visualized on IVU because of nonexcretion of contrast, whereas all the 70 (100%) kidneys were visualized on MRU. On IVU, nephrogram was not visualized in 66 (94.2%) of the 70 kidneys, whereas MRU showed prompt and homogeneous nephrogram in 68 (97.1%) of the 70 kidneys. No evidence of PUJO was seen in 31 (44.2%) kidneys on both IVU and MRU. IVU showed PUJO in 26 (37.1%) kidneys, whereas MRU showed it in 38 (54.2%) kidneys. MRU detected two duplex systems that were missed on IVU. A focal renal lesion and two incidental extra renal abnormalities were detected on MRU, which were not visualized on IVU. CONCLUSION: MRU is better than IVU, especially in case of poorly functioning kidneys which are not visualized on IVU. MRU also provides anatomic details of the ureter and vessels with better evaluation of renal parenchyma. It also has an additional advantage of detecting incidental extra renal abnormalities, if present.

2.
Childs Nerv Syst ; 30(5): 867-71, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24326950

RESUMEN

BACKGROUND: Predicting the outcome of congenital hydrocephalus in early infancy and childhood is difficult. Various consequences of hydrocephalus like ophthalmic, audiometric, musculoskeletal, and the developmental abnormalities play a complex role. We analyzed the quality of life of these patients in early life. METHODS: A prospective multispecialty assessment of the patients with congenital hydrocephalus, who had undergone ventriculoperitoneal shunt in the Pediatric Surgery unit of a tertiary care hospital, was carried out and analyzed. RESULTS: There were 24 boys and 6 girls. Eighty-three percent were operated before the age of 3 months. Mean age at follow up was 6 years (3-9 years). Shunt complications were seen in 53% (16) of the patients, out of which 68.75% had shunt blocks. In 70% (21) of the patients, the ventricle to hemisphere ratio (VHR) was between 51 and 70%, 20% (6) had a VHR of more than 70% and only 3 patients had a VHR between 40-50%. Audiological and ophthalmic problems were seen in 20 and 66% of the children, respectively. Musculoskeletal abnormalities were present in 36% of the patients. Developmental profile showed 44% being normal, 30% retarded, and 26% were borderline cases. The schooling had started in 56.6%, out of which only one child went to a special school. CONCLUSIONS: A multitude of problems associated with congenital hydrocephalus should be diligently diagnosed, aggressively followed and intervened with in the early years of life. Preschool management well before the peer interaction should optimize the social integration and improve the quality of life in these patients.


Asunto(s)
Hidrocefalia/complicaciones , Hidrocefalia/psicología , Trastornos Psicomotores/etiología , Calidad de Vida/psicología , Análisis de Varianza , Audiología , Niño , Preescolar , Técnicas de Diagnóstico Oftalmológico , Escolaridad , Femenino , Humanos , Hidrocefalia/cirugía , Estudios Longitudinales , Masculino , Anomalías Musculoesqueléticas/complicaciones , Complicaciones Posoperatorias/diagnóstico , Estudios Prospectivos , Derivación Ventriculoperitoneal/métodos , Agudeza Visual
3.
Paediatr Anaesth ; 23(5): 415-21, 2013 May.
Artículo en Inglés | MEDLINE | ID: mdl-23061785

RESUMEN

INTRODUCTION: Aim of sedation during pediatric urodynamic studies (UDS) is a calm and cooperative child while not affecting measurements. We compared the effectiveness of midazolam to low-dose ketamine infusion for sedation and their impact on urodynamics. MATERIALS AND METHODS: ASA-I children undergoing UDS were randomly assigned to group K (ketamine) loading dose (0.25 mg·kg(-1)) followed by infusion of 10-20 µg·kg(-1) ·min(-1) or group M (midazolam) loading dose of (0.02 mg·kg(-1)) followed by 1-2 µg·kg(-1) ·min(-1). The sedation scores and reactivity to catheterization were monitored by Children Hospital of Wisconsin Sedation Scale and Frankl Behavior Rating Scale, respectively. The UDS included two-channel filling cystometry in supine position followed by a free uroflowmetry in sitting position. The UDS was performed and interpreted in accordance with good urodynamic practice guidelines of International Continence Society (2002). RESULTS: A total of 34 children were enrolled. Group K children (n = 17) attained sedation earlier 6.80 (±3.36) min vs. 9.40 (±2.82) min; (P = 0.03) than group M (n = 17) and also recovered earlier 11.60 (±3.13) min vs. 19.67 (±5.49) min (P = 0.01). Reactivity scores during urinary and rectal catheterization were lower in group K (P = 0.03 and 0.01), respectively. Historical UDS data of 21 participants were available for comparison with effect of medication. None of the study drugs affected UDS parameters significantly. CONCLUSIONS: Midazolam or low-dose ketamine provide satisfactory sedation during pediatric UDS without impacting urodynamic values.


Asunto(s)
Anestésicos Disociativos , Hipnóticos y Sedantes , Ketamina , Midazolam , Urodinámica , Anestésicos Disociativos/administración & dosificación , Presión Sanguínea/efectos de los fármacos , Niño , Preescolar , Sedación Consciente , Método Doble Ciego , Femenino , Frecuencia Cardíaca/efectos de los fármacos , Humanos , Hipnóticos y Sedantes/administración & dosificación , Ketamina/administración & dosificación , Masculino , Midazolam/administración & dosificación , Oxígeno/sangre , Enfermedades Urológicas/complicaciones , Enfermedades Urológicas/diagnóstico
4.
AJR Am J Roentgenol ; 196(4): W438-45, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21427309

RESUMEN

OBJECTIVE: The objective of our study was to prospectively assess the role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia (EHBA) in infants younger than 90 days. SUBJECTS AND METHODS: Sonography was performed in 99 infants younger than 90 days with conjugated hyperbilirubinemia (total bilirubin > 3 mg/dL, conjugated bilirubin > 20% of total) after 4 hours of fasting. They were evaluated for the "triangular cord" sign, the presence and morphology of the gallbladder, gallbladder contraction after oral feeding, the presence and diameter of the common bile duct (CBD), liver size and echotexture, spleen size, caliber of the right branch of the hepatic artery, and caliber of the right branch of the portal vein. The final diagnosis of EHBA was made on basis of surgery. The performance of sonography in the diagnosis of EHBA was evaluated. RESULTS: The study group was composed of 68 boys and 31 girls (age range, 13-89 days); of the 99 infants, 30 had EHBA. The triangular cord sign had a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 23.3%, 97.1%, 77.8%, and 74.4%, respectively. The gallbladder was not visualized in seven infants, all of whom had EHBA. The sensitivity, specificity, PPV, and NPV of an abnormal gallbladder were 83.3%%, 82.6%, 67.6%, and 91.9%, respectively, and for noncontraction of the gallbladder were 87%, 72.5%, 51.3%, and 94.3%, respectively. A nonvisualized CBD had a sensitivity, specificity, PPV, and NPV of 93.3%, 47.8%, 43.8%, and 94.3%, respectively. A negative triangular cord sign with normal gallbladder morphology had an NPV of 91.9% for excluding EHBA. CONCLUSION: Comprehensive sonographic evaluation can help in segregating infants at high risk of EHBA from those at low risk.


Asunto(s)
Atresia Biliar/diagnóstico por imagen , Atresia Biliar/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Valor Predictivo de las Pruebas , Estudios Prospectivos , Sensibilidad y Especificidad , Ultrasonografía
5.
Surg Today ; 41(10): 1391-4, 2011 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21922362

RESUMEN

PURPOSE: Gastric perforations generally develop in neonates with esophageal atresia (EA) and a tracheoesophageal fistula (TEF), requiring preoperative mechanical ventilation. To the best of our knowledge, spontaneous gastric perforation in patients who have not been treated with mechanical ventilation has not been described in the literature. There is also no current consensus or treatment protocol available for the management of these patients. METHODS: Over a period of 6 years, six patients with EA and TEF presented with gastric perforation at our center. We studied the clinical presentation, initial resuscitation, surgical management, and outcome of these six patients. RESULTS: Out of the six patients, five were treated with initial flank drain insertion for peritoneal decompression. In all of the patients, a thoracotomy was performed first, followed by a laparotomy for closure of the stomach perforation. Four of the six patients survived and were discharged uneventfully. Two patients died of sepsis. Early feeding was established in all of the patients. CONCLUSIONS: Spontaneous gastric perforation can occur in patients with EA and TEF even without mechanical ventilation. Initial stabilization with peritoneal drain insertion and subsequent thoracotomy for esophageal anastomosis followed by laparotomy for stomach repair, both done in a single sitting, should be the ideal management of such patients.


Asunto(s)
Atresia Esofágica/cirugía , Rotura Gástrica/cirugía , Fístula Traqueoesofágica/cirugía , Drenaje , Atresia Esofágica/complicaciones , Atresia Esofágica/diagnóstico por imagen , Femenino , Gastroenterostomía , Gastrostomía , Humanos , Recién Nacido , Masculino , Observación , Radiografía , Estudios Retrospectivos , Rotura Espontánea/diagnóstico por imagen , Rotura Espontánea/etiología , Rotura Espontánea/cirugía , Rotura Gástrica/diagnóstico por imagen , Rotura Gástrica/etiología , Toracotomía , Fístula Traqueoesofágica/complicaciones , Fístula Traqueoesofágica/congénito , Fístula Traqueoesofágica/diagnóstico por imagen , Resultado del Tratamiento , Técnicas de Cierre de Heridas
6.
Paediatr Anaesth ; 18(7): 613-9, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-18482234

RESUMEN

BACKGROUND: Intrathecal (IT) adjuncts often are used to enhance the duration of spinal bupivacaine. Fentanyl is a spinal analgesic that could be a useful adjunct, and enhances the duration and quality of sensory block in adult surgical and obstetric population. However, no data exist to assess the dose-response characteristics of IT fentanyl when added to bupivacaine in infants. METHODS: Fifty-eight infants undergoing lower abdominal and urologic procedures were randomized into four groups to receive plain 0.5% hyperbaric bupivacaine F0 (<5 kg = 0.5 mg.kg(-1); 5-10 kg = 0.4 mg.kg(-1)). Groups F0.25, F0.5, and F1 groups received bupivacaine added with 0.25, 0.5, and 1 mug.kg(-1) of fentanyl, respectively. Duration of spinal anesthesia (SA) as assessed by the recovery of hip flexion in the postoperative period was the primary variable analyzed. In addition, the duration of analgesia in the postoperative period, rescue postoperative analgesic requirements and hemodynamic changes were recorded. RESULTS: Fifty-six infants were studied. The four groups were similar for age, weight, duration of surgery, onset of sensory, motor block, and the highest level of analgesia attained. The addition of 1 mug.kg(-1) fentanyl (F1) significantly increased the duration of SA (74.27 +/- 6.1 min) compared to the control group (51.21 +/- 5.2 min) (P = 0.001). Postoperative pain-free interval was prolonged (P = 0.004) and significantly less rescue analgesics were required after 1 mug.kg(-1) IT fentanyl (P = 0.032). These parameters did not show any significant difference among groups F0, F0.25, and F0.5. CONCLUSIONS: The addition of 1 mug.kg(-1) IT fentanyl to spinal bupivacaine prolonged the duration of spinal block in infants undergoing lower abdominal and urologic procedures.


Asunto(s)
Abdomen/cirugía , Anestésicos Combinados/farmacología , Anestésicos Locales/farmacología , Bupivacaína/farmacología , Fentanilo/farmacología , Procedimientos Quirúrgicos Urológicos/métodos , Anestesia Raquidea/efectos adversos , Anestesia Raquidea/métodos , Anestésicos Combinados/administración & dosificación , Anestésicos Combinados/efectos adversos , Anestésicos Intravenosos/administración & dosificación , Anestésicos Intravenosos/efectos adversos , Anestésicos Intravenosos/farmacología , Anestésicos Locales/administración & dosificación , Anestésicos Locales/efectos adversos , Bupivacaína/administración & dosificación , Bupivacaína/efectos adversos , Relación Dosis-Respuesta a Droga , Sinergismo Farmacológico , Femenino , Fentanilo/administración & dosificación , Fentanilo/efectos adversos , Hemodinámica/efectos de los fármacos , Humanos , Lactante , Inyecciones Espinales , Masculino , Dolor Postoperatorio/tratamiento farmacológico , Estudios Prospectivos , Respiración/efectos de los fármacos , Factores de Tiempo
7.
Appl Immunohistochem Mol Morphol ; 24(10): 721-728, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26469330

RESUMEN

IgG4-related disease is a recently recognized systemic condition characterized by tumefactive lesions at various sites. Inflammatory pseudotumor (IPT), a tumefactive mass lesion with an unknown etiology, belongs to the spectrum of IgG4-related disease. Inflammatory myofibroblastic tumor (IMT), previously considered under the umbrella of IPT, is now classified as a clonal neoplasm. Previously, both the terms were used interchangeably, because of overlapping morphologic features. This study was carried out to compare the morphologic and the immunohistochemical features of these entities and to study the role of IgG4 in their pathogenesis. Thirty-nine cases comprising of IMT (n=18) and IPT (n=21) were retrieved, and their clinical, morphologic, and immunohistochemical features were studied. IMT was more common in children as compared with IPT. IMT cases showed the proliferation of myofibroblastic cells accompanied by a variable inflammatory infiltrate, whereas IPT cases showed predominantly stromal fibrosis and a lymphoplasmacytic infiltrate with a subset of cases showing a storiform fibrosis and obliterative phlebitis. Anaplastic lymphoma kinase-1 (ALK-1) was positive in 12 of the 18 (66.7%) IMT cases, whereas none of the IPT cases showed ALK-1 immunoreactivity. IPT cases showed significantly increased IgG4+ plasma cells (mean, 127.8/high-power fields vs. 17.8/high-power fields) and a higher IgG4/IgG ratio (mean, 48.2% vs. 10.7%) as compared with IMT. Fluorescence in situ hybridization analysis was positive for ALK rearrangement in 6 of the 9 IMT cases tested. In conclusion, most of the IPT cases can be considered as IgG4 related on the basis of their histopathologic features and immunohistochemistry criteria. However, IMT represents a myofibroblastic neoplasm with ALK-1 overexpression and is clearly not IgG4 related.


Asunto(s)
Granuloma de Células Plasmáticas/patología , Inmunoglobulina G/inmunología , Células Plasmáticas/inmunología , Adolescente , Adulto , Niño , Femenino , Granuloma de Células Plasmáticas/inmunología , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Masculino , Adulto Joven
8.
J Pediatr Urol ; 11(2): 80.e1-6, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25794866

RESUMEN

INTRODUCTION: Pediatric ureteropelvic junction obstruction (UPJO) due to an extrinsic crossing vessel (CV) is rare and often remains undiagnosed preoperatively. Vascular hitch procedures are often performed as associated intrinsic obstruction is not expected. We compared data and intravenous urography (IVU) findings of patients with aberrant CV versus those with intrinsic UPJO, all undergoing open dismembered pyeloplasty. PRIMARY OBJECTIVE: Is accurate pre-operative diagnosis of aberrant CV causing extrinsic UPJO possible? SECONDARY OBJECTIVE: To assess differences in the demographic, clinical, radiological, intra-operative features and postoperative improvement after pyeloplasty between patients with a CV and those with only intrinsic UPJO. PATIENTS AND METHODS: Prospective study of all children below 12 years with UPJO presenting to a tertiary referral centre and who underwent open Anderson - Hynes dismembered pyeloplasty between 2003 and 2013 was conducted. Pre-operative investigations included serial ultrasonography, renal dynamic [ethylene di-cysteine (EC)] scan and IVU. These were repeated 3 months after pyeloplasty. Pre-operative IVUs of children with CV were compared with the IVUs of an equal number of similar aged children, randomly selected from the intrinsic obstruction group. RESULTS: Pyeloplasty was performed in 643 children during the study period. Data of 33 children with aberrant CVs (mean age 6.99 years) were compared with the remaining 610 children (mean age 3.27 years) with only intrinsic obstruction. Highly significant associations of those with CV included age above 2 years, female gender, associated anomalies, abdominal pain in those above 2 years and poor preoperative function on IVU. Specific IVU features which were statistically highly significant in favor of presence of CV were small, intrarenal and globular flat bottomed pelvis. (Figure) Calyceal dilatation was also more prominent in the CV group. A funnel shaped, extrarenal pelvis was highly significant in favor of intrinsic obstruction. There was associated intrinsic obstruction in addition to CV obstruction in 8 children. All children symptomatically improved after pyeloplasty and did well on long term follow up. The majority showed improvement or stabilization of function on EC scan. DISCUSSION: With the advent of antenatal ultrasonography, most children with UPJO are detected early. Children with CV tend to present later. This is often detected during surgery. Color Doppler is useful but is operator dependant and not performed routinely. In this study, IVU showed the presence of obstruction and loss of function unlike color Doppler, but also revealed specific diagnostic features not previously reported in literature. This can help in accurate preoperative prediction and avoid endopyelotomy, or a dorsal lumbotomy/retroperitoneal approach. Renal function in CVs is expected to be good as the obstruction is thought to be intermittent. However, we noted delayed contrast uptake on IVU in 60.6% and differential renal function on EC scan below 40% in 17 patients (56.6%). These indicate the effect of the obstruction on the renal parenchyma and the importance of early detection. Higher association with other anomalies and higher incidence in females has also not been emphasized in the literature so far. We noted associated intrinsic obstruction in 24.24% patients which is highly significant. This category of patients is likely to be missed and inappropriately treated if a "vascular hitch procedure" is performed. None of our patients had postoperative complications. CONCLUSIONS: Characteristic features were seen on IVU helping in preoperative diagnosis which can be extrapolated to magnetic resonance urography. There is a higher association of CV in age above 2 years, females, associated congenital anomalies, delayed uptake on IVU and differential renal function below 40% compared to intrinsic obstruction. Associated intrinsic obstruction in 24% with no postoperative complications indicates the superiority of dismembered pyeloplasty over vasculopexy procedures.


Asunto(s)
Hidronefrosis/cirugía , Pelvis Renal/cirugía , Obstrucción Ureteral/cirugía , Malformaciones Vasculares/cirugía , Niño , Preescolar , Estudios de Cohortes , Constricción Patológica , Femenino , Estudios de Seguimiento , Humanos , Hidronefrosis/diagnóstico por imagen , Pelvis Renal/irrigación sanguínea , Pelvis Renal/patología , Cuidados Preoperatorios/métodos , Estudios Prospectivos , Medición de Riesgo , Resultado del Tratamiento , Obstrucción Ureteral/diagnóstico por imagen , Urografía/métodos , Procedimientos Quirúrgicos Urológicos/métodos , Malformaciones Vasculares/diagnóstico por imagen , Procedimientos Quirúrgicos Vasculares/métodos
10.
Eur J Radiol ; 83(4): 710-4, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24447421

RESUMEN

OBJECTIVE: The counterclockwise rotation of the SMV on SMA is a normal and non-specific finding, which results in an incomplete swirl formation on CT scans. However, it has a potential to be misinterpreted as 'midgut volvulus' resulting in serious clinical implications. The study was done to determine the frequency and degree of counterclockwise rotation of the SMV on SMA on CT in normal otherwise asymptomatic pediatric patients undergoing CT scan. METHODS: In this IRB approved study, we retrospectively analyzed abdominal CT scan examinations of 200 consecutive pediatric patients (age range of 11 days to 18 years), which were performed for different clinical indications over a period of 10 months. They were evaluated for the absence or presence and degree of counterclockwise rotation of the mesenteric vessels. RESULTS: Of the 200 patients, 128 (64%) patients showed no clockwise or anticlockwise rotation of mesenteric vessels. Counterclockwise rotation of SMV on SMA was seen in 72 (36%) patients. Further, the degree of rotation of vessels was also calculated, based on the criteria proposed by the authors. CONCLUSIONS: The counterclockwise rotation of SMV on SMA gives an appearance of mesenteric whirlpool in otherwise normal mesenteric vessels and can be misinterpreted as midgut volvulus. It is a normal CT appearance and is due to a variation in branching pattern of mesenteric vessels. Awareness of this normal branching pattern of mesenteric vessels is important to avoid an inadvertent laparotomy.


Asunto(s)
Angiografía/métodos , Anomalías del Sistema Digestivo/diagnóstico por imagen , Vólvulo Intestinal/diagnóstico por imagen , Arterias Mesentéricas/diagnóstico por imagen , Venas Mesentéricas/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Adolescente , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Valores de Referencia , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad
11.
Eur J Radiol ; 82(6): 905-12, 2013 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-23332642

RESUMEN

OBJECTIVE: The purpose of this study was to evaluate the diagnostic performance of intravenous contrast enhanced computed tomographic colonoscopy (IVCTC) in the diagnosis of clinically suspected colorectal polyps in children, using conventional colonoscopy (CC) as the gold standard. METHODS: This was a prospective study conducted between July 2008 and June 2010. 30 pediatric patients with history of rectal bleeding and clinically suspected to have colorectal polyps were enrolled. All of the patients underwent IVCTC followed by CC. 30 IVCTC and 31 CC were performed in 30 patients. The findings of IVCTC were compared with those of CC. Statistical analysis was performed to obtain diagnostic performance values of IVCTC on per polyp (sensitivity and positive predictive value) and per patient (sensitivity, specificity, positive predictive value and negative predictive value) basis. RESULTS: By IVCTC, 63 polyps were detected in 28 patients of which 53 polyps were eligible for inclusion in the statistical analysis. 60 polyps were detected by CC in 28 patients of which 50 polyps were eligible for inclusion in the statistical analysis. The per polyp sensitivity and positive predictive values were 94% and 88.6% respectively. The per patient sensitivity, specificity, positive predictive value, and negative predictive values were 96.4, 50, 96.4, and 50% respectively. Twenty polyps, in 10 patients, were visualized only after intravenous contrast administration of which 5 polyps, in 5 patients, were likely to have been missed in the absence of the intravenous contrast injection as these polyps were submerged in fluid. Four patients would have had a false negative CTC examination if the intravenous contrast had not been injected; while in another patient, the number of polyps would have been underestimated. CONCLUSION: CTC is capable of serving as a safe and efficient non-invasive tool for evaluating children with clinically suspected colorectal polyps. Administration of intravenous contrast improves the sensitivity of polyp detection on CTC.


Asunto(s)
Pólipos del Colon/diagnóstico por imagen , Pólipos del Colon/etiología , Colonografía Tomográfica Computarizada/métodos , Hemorragia Gastrointestinal/complicaciones , Hemorragia Gastrointestinal/diagnóstico por imagen , Yodo/administración & dosificación , Adolescente , Niño , Preescolar , Medios de Contraste/administración & dosificación , Femenino , Humanos , Inyecciones Intravenosas , Masculino , Reproducibilidad de los Resultados , Sensibilidad y Especificidad
12.
Congenit Anom (Kyoto) ; 52(4): 211-5, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23181497

RESUMEN

The present case report describes two patients with a novel combination of VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, limb), neural tube defect and crossed renal ectopia. Though cases of VACTERL associated with crossed renal ectopia have been described, the present case report is the first to describe its combination with neural tube defect. The cases reported here are significant because central nervous system manifestations are scarce in VACTERL syndrome. The role of sonic hedgehog pathway has been proposed in VACTERL association and neural tube defects. Axial Sonic hedgehog signaling has also been implicated in the mediolateral positioning of the renal parenchyma. With this knowledge, the etiopathogenesis of this novel combination is discussed to highlight the role of sonic hedgehog signaling as a point of coherence.


Asunto(s)
Cardiopatías Congénitas/diagnóstico , Deformidades Congénitas de las Extremidades/diagnóstico , Anomalías Múltiples/diagnóstico , Canal Anal/anomalías , Canal Anal/metabolismo , Esófago/anomalías , Esófago/metabolismo , Cardiopatías Congénitas/genética , Cardiopatías Congénitas/metabolismo , Proteínas Hedgehog/metabolismo , Humanos , Recién Nacido , Riñón/anomalías , Riñón/metabolismo , Deformidades Congénitas de las Extremidades/genética , Deformidades Congénitas de las Extremidades/metabolismo , Imagen por Resonancia Magnética , Masculino , Defectos del Tubo Neural/diagnóstico , Transducción de Señal , Columna Vertebral/anomalías , Columna Vertebral/metabolismo , Tráquea/anomalías , Tráquea/metabolismo
13.
Eur J Gastroenterol Hepatol ; 24(10): 1219-26, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-22850192

RESUMEN

BACKGROUND: This was a prospective observational study carried out to objectively assess the effect of shunt surgery on intestinal morphology and function in patients with extrahepatic portal vein obstruction (EHPVO) and correlate it with growth improvement. PATIENTS AND METHODS: Twenty patients who were operated upon for EHPVO were divided into two groups for the purpose of analysis depending on the outcome of surgery: group A--patients who underwent successful shunt surgery (n=14) and group B--patients who underwent splenectomy with devascularization (n=1) and those with thrombosed shunts (n=5). The patient groups were created on the basis of the type and outcome of the surgery and not prospective stratification. Growth parameters, endoscopy findings, duodenal histology, brush border enzyme activity, urinary D-xylose levels, fecal steatocrit, fecal α-1 antitrypsin, serum growth hormone and insulin-like growth factor-1 levels, and quality-of-life scores were assessed before surgery and at a mean of 24.9 weeks after surgery. RESULTS: There was no significant difference between the preoperative and postoperative duodenal histology. Preoperative brush border lactase activity was significantly lower than normal and did not change significantly after surgery. EHPVO did not affect intestinal absorption or permeability. Shunt surgery resulted in significantly improved z scores for height after surgery as well as quality of life. There was no significant growth hormone resistance. CONCLUSION: Our patients did not have any significant malabsorption or abnormality in small intestinal structure and function when compared with established normal levels. There was no significant change in the above parameters after shunt surgery, although an improvement in growth was observed. Thus, factors other than enteropathy or other lesser known enteral factors seem to be responsible for the growth retardation observed in EHPVO and its subsequent improvement after shunt surgery.


Asunto(s)
Duodeno/enzimología , Hormona de Crecimiento Humana/sangre , Hipertensión Portal/cirugía , Factor I del Crecimiento Similar a la Insulina/análisis , Vena Porta/cirugía , Derivación Portosistémica Quirúrgica/métodos , Esplenectomía/métodos , Adolescente , Niño , Preescolar , Duodeno/patología , Endoscopía Gastrointestinal , Ensayo de Inmunoadsorción Enzimática , Femenino , Crecimiento/fisiología , Humanos , Masculino , Vena Porta/fisiopatología , Estudios Prospectivos , Calidad de Vida , Adulto Joven
14.
J Bronchology Interv Pulmonol ; 18(3): 223-8, 2011 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23208564

RESUMEN

BACKGROUND: Foreign body aspirations (FBA) in the tracheobronchial tree must be suspected in children who present with a witnessed history of choking or respiratory distress of sudden onset and asymmetric breath sounds, even in the absence of pathognomonic radiographic findings. This study reviews our experience with a variety of FBA and outlines the salient differences in the literature. METHODS: One hundred eighty-four consecutive children with a history suggestive of FBA undergoing bronchoscopy over a period of 10 years were reviewed. In all of the cases, rigid bronchoscopy was performed under general anesthesia using a Storz ventilating bronchoscope with the aid of optical forceps. RESULTS: In 166 (90.21%) patients, a foreign body (FB) was discovered, whereas in 18 (9.78%) children, no FB could be found. The highest incidence of FBA (126/166, 75.9%) was found in the age group between 1 and 5 years. FBA of organic origin were more common (77.77%) in the younger patients (<3 y) as compared with the older patients (12.23%, >3 y) (P<0.0001). The radiographs were suggestive in 90% of the children >5 years of age (P<0.0063). Seven patients had a delay in the diagnosis and were being treated for various medical ailments. The incidence of FBA was almost double (64.83%) during the winter months as compared with rest of the year (34.17%). Pen cap aspirations were seen in 7 patients, and 6 of them could be extracted successfully with bronchoscopy. Two patients died. CONCLUSIONS: Bronchoscopy can be a life-saving procedure and is safe even when no FB is found. The parameters of the history of witnessed choking, respiratory distress of sudden onset, and the asymmetric breath sounds are used in the decision making to perform a bronchoscopy. Radiographs are less helpful in younger patients. Nonorganic FBA is more common in older children. There may be seasonal variations and more attention should be given to small children during the times of high incidence.

15.
Pathol Int ; 56(2): 84-8, 2006 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-16445820

RESUMEN

A 7-year-old boy presented with midline swelling in the neck. On fine-needle aspiration cytology it was diagnosed as papillary carcinoma of the thyroid. The patient underwent total thyroidectomy. Histopathological examination, immunohistochemistry and electron microscopy revealed the presence of two intermingled components: medullary carcinoma and papillary carcinoma. One of the submandibular lymph nodes had metastasis of both the components. The case was diagnosed as 'mixed medullary and follicular cell carcinoma' with papillary carcinoma pattern and lymph node metastasis. Mixed medullary and follicular cell carcinoma with intermingling of medullary and papillary carcinoma components is a rare tumor. In adults, only eight such cases with lymph node metastasis have been published. To the best of the authors' knowledge no pediatric case has previously been reported in the English-language literature.


Asunto(s)
Carcinoma Medular/patología , Carcinoma Papilar Folicular/patología , Metástasis Linfática/patología , Tumor Mixto Maligno/diagnóstico , Neoplasias de la Tiroides/patología , Biopsia con Aguja Fina , Antígeno Carcinoembrionario/análisis , Carcinoma Medular/química , Carcinoma Medular/diagnóstico , Carcinoma Papilar Folicular/química , Carcinoma Papilar Folicular/diagnóstico , Niño , Humanos , Inmunohistoquímica , Ganglios Linfáticos/patología , Metástasis Linfática/diagnóstico , Masculino , Tumor Mixto Maligno/química , Tumor Mixto Maligno/patología , Tiroglobulina/análisis , Glándula Tiroides/patología , Neoplasias de la Tiroides/química , Neoplasias de la Tiroides/diagnóstico
17.
Ann Card Anaesth ; 7(2): 137-43, 2004 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-17827546

RESUMEN

The present study was designed to compare induction and recovery characteristics of sevoflurane and halothane for rigid bronchoscopy for removal of foreign bodies in tracheobronchial tree in children. Forty four children (age 1-4 years) were allocated randomly to two groups to receive either halothane (group H; n=22) or sevoflurane (group S; n=22) in oxygen. A graded inhalation technique was used with maximum inspiratory concentration of 5% for halothane and 8% for sevoflurane. Time for loss of consciousness and induction time in group H and group S were 2.3+/-0.4 min vs 2.2+/-0.4 min (p>0.05) and 4.6+/-0.7 min vs 4.9+/-0.6 min (p>0.05) respectively. Intubation conditions with rigid bronchoscope were similar in both groups. Fewer children in group H had vocal cord movements as compared to group S on laryngoscopy (3 vs 8, p>0.05). Six children in group H and two children in group S had disturbances of cardiac rhythm (p>0.05). Emergence time was significantly shorter in group S as compared to group H (group H - 29.6+/-10.7 min vs group S- 12.3+/-7.6 min, p<0.05). Modified Aldrete's score of 8 was achieved significantly faster in group S as compared to group H (group H - 33.8+/-9.3 min vs group S- 17.3+/-6.8 min, p<0.05). Adverse events during induction and recovery were comparable between the two groups except for significantly high incidence of excitement in group S. In conclusion, halothane is as suitable as sevoflurane for children undergoing rigid bronchoscopy for airway foreign body retrieval, but sevoflurane has a quicker recovery.

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