A neonate with a spongy failing heart - What could it be?

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.

Oral domperidone versus placebo for enhancing exclusive breastfeeding among post-lower segment cesarean section mothers - a double-blind randomized controlled trial.

OBJECTIVE: To assess whether oral domperidone compared to placebo increases the rate of exclusive breastfeeding for 6 months among post-lower segment cesarean section (LSCS) mothers. METHODS: This double-blind Randomized Controlled Trial, conducted in a tertiary care teaching hospital in South India, included 366 post-LSCS mothers with delayed initiation of breastfeeding or with subjective feelings of not having enough milk. They were randomized to two groups - Group A: Standard lactation counseling and oral Domperidone and Group B: Standard lactation counseling and a placebo. The primary outcome was an exclusive breastfeeding rate at 6 months. Exclusive breastfeeding rates at 7 days and 3 months and serial weight gain of an infant were assessed in both groups. RESULTS: Exclusive breastfeeding rate at 7 days was statistically significant in the intervention arm. The exclusive breastfeeding rates at 3 months and 6 months were higher in the domperidone arm compared to placebo but not statistically significant. CONCLUSION: Oral Domperidone along with effective breastfeeding counseling showed an increasing trend of exclusive breastfeeding rate at 7 days and at six months. Appropriate breastfeeding counseling and postnatal lactation support are important in enhancing exclusive breastfeeding. TRIAL REGISTRATION: The study was prospectively registered with CTRI - Reg no. CTRI/2020/06/026237.

Cerebral sinus venous thrombosis: a rare complication of varicella infection in a 12-year-old girl.

Varicella infection can present with a variety of neurological manifestations, the most common of which are cerebellitis and encephalitis. A 12-year-old girl presented with headache, altered sensorium, blurring of vision and status epilepticus 2 weeks after she developed varicella lesions. Imaging demonstrated cerebral venous sinus thrombosis involving the left transverse sinus, sigmoid sinus and internal jugular vein with a haemorrhagic infarct in the left parieto-occipital region. Measures were taken to decrease the intracranial pressure, and she was commenced on anti-convulsants (phenytoin) and heparin infusion, following which she improved and was discharged after 2 weeks. Repeat imaging undertaken 3 months later demonstrated a resolving thrombus, and a pro-coagulant work-up at follow-up did not show any underlying pro-thrombotic state. Neurological complications post varicella are rare, with encephalitis and ataxia being the most common. Cerebral sinus venous thrombosis secondary to varicella is very rare with only one case reported in a child.

Comparing the Harmonic Scalpel with Electrocautery in Reducing Postoperative Flap Necrosis and Seroma Formation after Modified Radical Mastectomy in Carcinoma Breast Patients: A Double-Blind Prospective Randomized Control Trail.

Introduction Only a few studies compare the efficacy of the harmonic scalpel and electrocautery in performing mastectomies, and these have mainly compared their intraoperative parameters. But the main concern with electrocautery is the incidence of flap necrosis and seroma formation. Therefore, this study was done to determine if the harmonic scalpel has any advantages over electrocautery in reducing postoperative flap necrosis and seroma formation in patients undergoing a modified radical mastectomy (MRM). Methodology This randomized control trial was carried out over a one-year period in a tertiary care centre in South India. The study patients were randomized into an electrocautery group and a harmonic scalpel group. In the first group, mastectomy including flap and axillary dissection was done using electrocautery. In the second group, a harmonic scalpel was used for dissection. This study compared the efficacy of the harmonic scalpel with electrocautery in terms of postoperative seroma formation and flap necrosis. Various other perioperative parameters like the number of drain days, total drainage volume (in mL), operating time (in minutes), intraoperative blood loss (in mL), and postoperative wound site pain were also studied. During each postoperative visit, the presence of seroma was assessed clinically, and the number of aspirations required for the seroma was also analysed. Results A total of 240 patients were randomized into two groups of 120 patients each. Baseline parameters were comparable across both groups. There were significant differences in the duration of surgery [151.38 mins vs. 112.33 mins; p = 0.001] and intraoperative blood loss [276.25 mL vs.200.13 mL; p = 0.001]. On Postoperative Day (POD) 1, the difference in the mean pain scores [6 vs. 4; p = 0.001] was statistically significant. In addition, the differences in the mean total drainage volume [937.5 mL vs. 470 mL; p = 0.002] and the incidence of seroma during the first follow-up [34.2% vs. 21.7 %; p = 0.030] were statistically significant. The difference in the incidence of flap necrosis on POD 4 [7.5% vs. 4.2%; p = 0.2706] was not statistically significant. None of the patients developed flap necrosis after four days. Conclusion The harmonic scalpel reduces the total drainage volume of seromas, the number of drain days, intraoperative blood loss, duration of surgery, postoperative pain (especially on POD 1 and 5), and incidence of flap necrosis.