Tracheal growth assessment in mongrel puppies (dogs) through multidetector CT.

The aim of this study was to describe the tracheal growth pattern and its zoometric relationship in related medium-sized mongrel puppies through adulthood. Fourteen puppies were studied. CT monitoring was performed monthly, starting in the 1st month of life through the 7th month and subsequently at the 9th and 12th months. Additionally, six zoometric measurements were performed. Dorsoventral (DV) and transverse (TV) diameters and the luminal area from C1 to T2 were obtained. The global tracheal growth pattern revealed an increase up to 13 times its initial size, reaching a plateau phase during the last trimester. The relationship between the DV and the TV internal diameters of the tracheal lumen did not change during growth. As previously reported, the cranial tracheal area was wider, while the caudal part gradually decreased towards T1-T2; this consideration is important since the more distal an endotracheal tube is inserted, the greater the risk that injury may occur. The linear correlation between the zoometric measurements and the tracheal ring areas was positive. This study provides evidence for the evaluation of the morphometry of the canine trachea during physiological growth using helicoidal CT as a non-invasive, accurate tool.

Tyrosinemia type I: clinical and biochemical analysis of patients in Mexico.

INTRODUCTION: Hepatorenal tyrosinemia (HT1) is a treatable, inherited, metabolic disease characterized by progressive liver failure with pronounced coagulopathy. The aim of this study is to describe the clinical, biochemical, and histopathological findings in a group of Mexican HT1 patients and their outcome. MATERIAL AND METHODS: Medical records of HT1 patients diagnosed between 1995 and 2011 were analyzed. The diagnosis of HT1 was confirmed by detection of succinylacetone in urine or blood. RESULTS: Sixteen nonrelated HT1 cases were analyzed. Mean age at clinical onset was 9 months, and the mean age at diagnosis was 16.3 months. Main clinical findings were hepatomegaly, splenomegaly, cirrhosis, liver failure, tubulopathy, nephromegaly, Fanconi syndrome, seizures and failure to thrive. Histopathological findings were cirrhosis, fibrosis and steatosis. The HT1 group had a mortality rate of 78%. Patients who received supportive care or nutritional treatment had a 3-year survival rate of 10%. For those who underwent liver transplantation, the 6-year survival rate was 60%. In most cases pharmacological treatment with nitisinone and special dietary products were not available. The leading causes of death were fulminant liver failure, metastatic hepatocellular carcinoma, and porphyria-like neurologic crisis. Newborn screening programs in combination with the availability of orphan drugs, proper monitoring, genetic counseling, and clinical practice guidelines are needed to enable physicians to identify the disease, delay its progression, and improve patients' quality of life. CONCLUSION: The devastating natural history of HT1 is still observed in Mexican patients because they are not diagnosed and treated during the early stages of the disease.

Higher incidence of thyroid agenesis in Mexican newborns with congenital hypothyroidism associated with birth defects.

BACKGROUND: Congenital hypothyroidism (CH) is the most common endocrine system disorder in newborns. Ectopic thyroid and agenesis are the most frequent thyroid structural malformations. Several reports have shown that CH is associated with birth defects (BD) ranging from congenital heart disease to ocular and gastrointestinal anomalies. AIMS: We investigated how many and what types of BD were associated with CH in Mexican children. STUDY DESIGN: Cross-sectional study conducted in patients with confirmed CH. SETTING: Highly specialized government pediatric center in Mexico City. SUBJECTS: We included 212 patients with permanent CH identified by newborn screening. RESULTS: We found that 24% of patients with CH also had BD, and that there was a higher prevalence of thyroid agenesis in the group of patients with CH associated with BD (CH+BD) versus the isolated CH group (p=0.007). There were more females than males in both groups. The most common BD were congenital heart diseases, especially those of the atrial septum, followed by patent ductus arteriosus, found as a single malformation or as part of a complex congenital heart disease. In this study, we found Hirschsprung disease, Beckwith-Wiedemann syndrome, Pierre Robin sequence, Albright's osteodystrophy, VATER association, and frontonasal dysplasia associated with CH. CONCLUSIONS: In this study population, there was a high prevalence of BD in patients with permanent CH. Thyroid agenesis was the main etiological cause of CH in patients with associated congenital malformations. The high prevalence of CH+BD underlines the need for a comprehensive clinical diagnostic approach of the patients with CH.

Necrólisis tóxica epidérmica / Toxic epidermal necrolysis

Objetivo: Presentar el caso de una paciente con necrólisis tóxica epidérmica producido por fenitoína sódica y hacer una profunda revisión de la literatura para señalar la importancia del diagnóstico y tratamiento tempranos. Sede: The American British Cowdray Medical Center, IAP, México, D.F. La necrólisis tóxica epidérmica o síndrome de Lyell es una enfermedad idiosincrásica que se presenta como una reacción mucocutánea severa, aguda y episódica producida por fármacos y que compromete la vida. Presentación del caso: se trata de una mujer de 41 años de edad, quien 10 semanas previas a su ingreso presentó enfermedad vascular cerebral isquémica en la cual se administró fenitoína sódica. Inició con malestar general, fiebre, posteriormente presentó edema facial, erupción cutánea generalizada que cubría en 90 por ciento de la superficie corporal total, que rápidamente progresó a bulas hemorrágicas friables, con denudación de la epidermis, acompañándose de involucro de mucosas oral, conjuntival y urogenital. Se brindó tratamiento con aislamiento inverso, lavado de las lesiones y tratamiento con nitrato de plata al 0.5 por ciento, antibioticoterapia de amplio espectro y fisioterapia. Egresó tres semanas después con la vía oral reinstalada, sin secuelas y con reepitelización del 90 por ciento de las lesiones. Conclusión: La necrólisis tóxica epidérmica es una enfermedad bulosa adquirida que puede confundirse con muchas otras. Es indispensable realizar un diagnóstico oportuno para brindar tratamiento multidisciplinario, evitando complicaciones, secuelas o la muerte misma.