VACTERL Association is complex, non-random sporadic association of various birth defects. It includes vertebral, anal anomalies, cardiovascular, tracheoesophageal, renal anomalies, radial anomalies and limb abnormalities.It is first described in 1970s. Full spectrum association is a very rare entity.VACTERL is considered to be present if any three of the above-mentioned abnormalities are present. Along with these features, patients might present with other external anomalies like lung lobation defects, external ear anomalies etc. However, the full spectrum of VACTERL association is very rare diagnosis mainly by clinical approach. Multidisciplinary approach and treatment of surgically correctable anomalies provide a better outcome for this association in new born period. Tracheoesophageal fistula is associated in more than 70 percent of the cases. Limb defects are also present in 70 percent of the cases. Anal atresia is present in 55 percent of the cases. VACTERL Shows phenotypic overlap with various conditions like Goldenhar syndrome, Fanconi anaemia, CHARGE syndrome etc. Here in we are reporting a case of VACTERL with presence of cardiovascular abnormality, Tracheoesophageal fistula and limb abnormalities.
A meconium pseudocyst is formed following meconium peritonitis. At present, antenatal diagnosis and planned management of meconium pseudocyst have reduced the mortality rate significantly. We presented a case of a neonate with abdominal distension and non-passage of meconium who experienced respiratory distress and was taken for exploratory laparotomy at a tertiary care center due to suspected bowel perforation. The neonate was diagnosed with a meconium pseudocyst intraoperatively as maternal ultrasound and ultrasound of the abdomen of the neonate after birth failed to make a definitive diagnosis; even an X-ray abdomen did not reveal pathognomonic egg-shell calcification. An interesting aspect of this case is the mother's complex obstetric history, which compelled us to conjecture whether it was possible to predict the chances of meconium peritonitis and take steps to prevent it. It must be noted that, despite rigorous research, the researchers could not find reliable literature co-relating the obstetric history of the mother with the formation of a meconium pseudocyst in the neonate.
Neonatal hypernatremic dehydration (NHD) is a common complication in breastfed neonates which if not recognized early can lead to life-threatening complications. Only a few cases of NHD leading to peripheral gangrene have been reported in the literature. We report a case of a 14-day-old neonate with complaints of dyspnoea, poor oral intake, and gangrenous changes in the left leg. There was a 28% weight loss since birth due to inadequate feeding. The baby was diagnosed with severe hypernatremic dehydration with Kidney Disease Improving Global Outcomes (KDIGO) stage 4 acute kidney injury requiring peritoneal dialysis. Ultrasonography of the left lower extremity revealed a distal femoral artery thrombus leading to dry gangrene requiring amputation. There were neurological signs like altered sensorium and drug-resistant seizures which were suspicious for intracranial pathology like cerebral venous sinus thrombosis. Prevention and early diagnosis of NHD are essential to prevent the occurrences of such grave complications. It can be easily achieved by improving the vigilance regarding the adequacy of feeds subjectively by the mother if the baby is at home and objectively by physicians in the hospital setting. These simple interventions have the potential to prevent readmissions due to not only simple feeding complications but grave complications as mentioned above as well and save precious lives.
Background: Dengue fever (DF) is an acute viral febrile illness commonly seen in India. If not identified and treated early, it may prove fatal. Objectives:To assess the clinical presentation, laboratory parameters and predictors of severity in DF in children in a metropolitan city in India.Method: This was a cross-sectional observational study of 100 cases of DF conducted at Jagjivan Ram Western Railway Hospital in Mumbai from June 2015 to May 2016. All inclusion and exclusion criteria were defined and patients were enrolled. Data were collected on the preformed questionnaire and analysed statistically on software.Results: DF was commoner in males. It was equally distributed in all parts of the metropolitan city; 58% patients with DF presented without warning sign while 31% presented with warning signs. The clinical features included vomiting, abdominal pain, breathlessness, retroorbital pain, jaundice and hypotension. Fever was seen in almost 100% cases. Thrombocytopenia was the common haematological finding. Raised liver enzymes and serum bilirubin were significantly associated with DF severity. Abnormal coagulation profile was also an important finding; 16% patients had tourniquet test positive.
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