Notes

Apothecary behaviour along with supply of harm reduction companies in Nc: an exploratory research.
Multisystem inflammatory syndrome in Children (MIS-C) is a postinfectious immune mediated hyperinflammatory state seen in children and adolescent below 21 year of age and develop after 4-6 weeks of severe acute respiratory syndrome coronavirus -2 (SARS-CoV-2) infection, however, it is rare in neonates. We report an extremely rare and first of its kind case of MIS-C in a neonate with persistent neutropenia.

A 19-day old boy presented with complaints of fever and loose stools for 1 day and developed rash after admission. learn more Baby was investigated for sepsis and commenced on IV antibiotics empirically. In view of persistent fever, diarrhoea, rash and absence of obvious microbial etiology of inflammation, with elevated inflammatory marker and an epidemiologic link to SARS-CoV-2 infection, the diagnosis of MIS-C-was made. Intravenous immunoglobulin (IVIg) was administered and defervescence occurred within 24 hours. He also developed neutropenia during course of illness which persisted on follow up.

MIS-C in neonates is uncommon and fever with elevated inflammatory markers during COVID-19 pandemic should alert the pediatrician to the possibility of MIS-C. Neutropenia may be associated with MIS-C in neonates and warrants prolonged follow up.
MIS-C in neonates is uncommon and fever with elevated inflammatory markers during COVID-19 pandemic should alert the pediatrician to the possibility of MIS-C. Neutropenia may be associated with MIS-C in neonates and warrants prolonged follow up.
A novel home monitoring program, in which premature infants are cared for at home with a nasogastric tube in place prior to achievement of full oral feeding, was evaluated. The program combines a digital, fully EMR-integrated, virtual daily rounding platform with direct provider video and telephone contact.

A case-control study was performed evaluating infants <  34 weeks' gestation who were followed in our program. A historical control group, was created by matching 2  1 based on gestational age±6 days, retroactively.

15 patients discharged in the program were compared with 30 controls. The home cohort gained an average of 30 g/day compared with the in-hospital group at 27g/day (p = 0.325). The home group required a mean of 5.9±2.9 days to full oral feeding once discharged, not different from the control group at 5.4±3.7 days (p = 0.606). The percentage of oral feeds for the home cohort, however, increased at a rate of 12.2%before discharge compared to rising 57%at home (p <  0.001). The control group spent an additional 8.1±3.9 days in the hospital after reaching criteria. There were no reported adverse events or readmissions.

Premature infants can safely advance oral feeds using a home monitoring program. While at home, infants gained weight similarly to their inpatient controls inpatient, yet gained full oral skills at a significantly faster rate compared to when they were in the hospital.
Premature infants can safely advance oral feeds using a home monitoring program. While at home, infants gained weight similarly to their inpatient controls inpatient, yet gained full oral skills at a significantly faster rate compared to when they were in the hospital.
Hereditary myosin myopathies are muscle disorders caused by mutations in myosin heavy chain genes. The MYH2 gene encodes the fast 2A skeletal muscle isoform, and mutations manifest as joint contractures, muscle weakness, and external ophthalmoplegia. Muscle biopsy shows decreased type 2A fibers, and vacuoles are sometimes present in adults with progressive disease.

This case describes a full term baby boy with hypotonia, dysmorphic features, dysphagia, and aspiration. Whole genome sequencing detected a novel heterozygous variant in the MYH2 gene. Muscle biopsy showed decreased type 2A fibers and vacuoles in myofibers.

Hypotonia and dysphagia are common in infants with a MYH2 myopathy. However, dysmorphic features and vacuoles on biopsy have not previous been described in infants with MYH2 myopathies.

This case reports an unusual phenotype of a rare neonatal-onset congenital myopathy associated with a novel heterozygous variant in MYH2.
This case reports an unusual phenotype of a rare neonatal-onset congenital myopathy associated with a novel heterozygous variant in MYH2.
Increased cardiorespiratory events with bradycardia and oxygen desaturation have been reported in very low birthweight (VLBW) infants following stressors such as immunizations. These events are difficult to quantify and may be mild. Our group developed an automated algorithm to analyze bedside monitor data from NICU patients for events with bradycardia and prolonged oxygen desaturation (BDs) and used this to compare BDs 24 hours before and after potentially stressful interventions.

We included VLBW infants from 2012-2017 with data available around at least one of four interventions two-month immunizations, retinopathy of prematurity (ROP) examinations, ROP therapy, and inguinal hernia surgery. We used a validated algorithm to analyze electrocardiogram heart rate and pulse oximeter saturation data (HR, SpO2) to quantify BD events of HR <  100 beats/minute for≥4 seconds with oxygen desaturation <  80%SpO2 for≥10 seconds. BDs were analyzed 24 hours before and after interventions using Wilcoxon rank-sum tests.

In 354 of 493 (72%) interventions, BD frequency stayed the same or decreased in the 24 hours after the event. An increase of at least five BD's occurred in 17/146 (12%) after immunizations, 85/290 (29%) after ROP examinations, 4/33 (12%) after ROP therapy, and 3/25 (12%) after hernia surgery. Infants with an increase in BDs after interventions had similar demographics compared to those without. More infants with an increase in BDs following immunizations were on CPAP or caffeine than those without.

Most VLBW infants in our cohort had no increase in significant cardiorespiratory events in the 24 hours following potentially stressful interventions.
Most VLBW infants in our cohort had no increase in significant cardiorespiratory events in the 24 hours following potentially stressful interventions.
A congenital lung malformation (CLM) that is diagnosed on prenatal ultrasound exam may subsequently become undetectable on later scans, a "vanishing" CLM.

The purpose of our study is to characterize the prenatal natural history and postnatal outcomes of "vanishing" lesions treated at our institution.

We performed a retrospective chart review of 107 patients diagnosed prenatally with CLM at our institution. Comparisons were made using Kruskal-Wallis or t-test for continuous variables and Fisher's exact test or Chi-Square test for categorical variables. Multivariable analysis using logistic regression was performed.

Of the 104 patients, 59 (56.7%) had lesions that became sonographically undetectable on serial ultrasound scans. Patients with lesions that vanished prenatally tended to need less Neonatal Intensive Care Unit (NICU) admission at birth (persistent CLM 54.8%vs vanished CLM 28.8%), decreased need for supplemental O2 at birth (persistent CLM 31.0%vs vanished CLM 11.9%), and decreased delay in feeds (persistent CLM 26.2%vs vanished CLM 8.5%) compared to those with persistent CLM. After multivariate analysis controlling for maternal steroid administration and sex, admission to NICU maintained a slight statistical significance, with patients in the vanishing CLM group 2.5 times less likely to be admitted to the NICU. None of our patients whose lesions vanished prenatally required mechanical ventilation. Eighty-six patients underwent postnatal computed tomography (CT) chest. Only 2 patients had lesions that regressed on postnatal CT.

Lesions that vanish on prenatal imaging may be associated with improved clinical outcomes. The rate of true regression at our institution was as low as 2.3%.
Lesions that vanish on prenatal imaging may be associated with improved clinical outcomes. The rate of true regression at our institution was as low as 2.3%.
Maintaining and manipulating sequences online is essential for language and memory. In Parkinson's disease (PD), poor performance in sequencing tasks has been associated with basal ganglia dysfunction, especially subthalamic hyperactivity.

This study is aimed to investigate the impact of high-frequency subthalamic nucleus (STN) deep brain stimulation (DBS) on sequence processing in PD.

Twenty-nine patients with PD (17 women) completed a 'before/after' sentence task and a digit ordering task with STN DBS ON and OFF. In the sentence task, patients read a sequence of events expressed in the actual order of occurrence ('after' sentences) or reversed order ('before' sentences) for comprehension. In the digit task, patients recalled a sequence of ordered digits (ordered trials) or reordered and recalled random digits in ascending order (random trials). Volumes of tissue activated (VTAs) were estimated for the motor and associative STN.

Patients were slower with STN DBS ON versus OFF in both tasks, although their motor symptoms were significantly improved under DBS. In the sentence task, patients showed higher ordering-related reaction time costs ('before' >  'after') with DBS ON versus OFF. Moreover, patients with larger left associative VTAs, smaller total motor VTAs, and more daily exposure to dopaminergic drugs tended to show larger reaction time cost increases under DBS. In the digit ordering task, patients with too large or too small right associative VTAs tended to show larger reaction time cost increases under DBS.

Stimulating the STN, especially its associative part, might impair sequence processing in language and memory.
Stimulating the STN, especially its associative part, might impair sequence processing in language and memory.
The concept of motor reserve explains the individual differences in motor deficits despite similar degrees of nigrostriatal dopamine depletion in Parkinson's disease (PD).

To investigate glucocerebrosidase (GBA) variants as potential determinants of motor reserve for exploratory purposes.

A total of 408 patients with drug-naïve PD were enrolled from the Parkinson's Progression Markers Initiative cohort database. All patients underwent SPECT dopamine transporter (DAT) scans and had results for Sanger sequencing of GBA. Parkinsonian motor deficits were assessed using the Movement Disorders Society Unified Parkinson's Disease Rating Scale Part III (MDS-UPDRS-III). We compared MDS-UPDRS-III scores while adjusting for DAT availability in the putamen (i.e., motor reserve) between the PD groups according to the presence of GBA mutations.

Fifty-four (13.2%) patients carried GBA mutations. PD patients with GBA mutations were younger than those without mutations. There were no significant differences in sex, disease duration, years of education, and striatal DAT availability between the PD groups. PD patients with GBA mutations had higher MDS-UPDRS-III scores for the less affected side than those without mutations, despite similar levels of DAT availability in the contralateral putamen. The MDS-UPDRS-III sub-scores of the more affected side did not differ between the two PD groups.

The results of this study demonstrated the detrimental effect of GBA variants on individual capacity to cope with PD-related pathologies, with different impacts depending on the motor laterality.
The results of this study demonstrated the detrimental effect of GBA variants on individual capacity to cope with PD-related pathologies, with different impacts depending on the motor laterality.
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