A Severe Form of Atypical Hemolytic Uremic Syndrome in a Two-Year-Old Girl: A Case Report.

Hemolytic uremic syndrome (HUS) is a prevalent cause of severe acute kidney injury in children, often leading to chronic renal damage. It is characterized by thrombotic microangiopathy (TMA), which represents a triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The choice of treatment and management strategies depends primarily on the underlying etiology. We present the case of a two-year-old girl diagnosed with rapidly progressive glomerulonephritis accompanied by hypertension necessitating renal replacement therapy. Initial laboratory findings indicated positive antinuclear antibodies, prompting immunosuppression and renal biopsy, revealing TMA with minimal chronicity changes. The treatment involved plasmapheresis and a single dose of injection rituximab, resulting in clinical recovery with an improved glomerular filtration rate. Since the anti-complement factor H antibody result was negative, the genetic etiology of atypical HUS was considered. The patient was discharged with favorable outcomes, including normal urine output and the absence of edema. This case concludes that young children with atypical HUS may present with a severe clinical course necessitating early intervention. The lack of genetic analysis facilities in severe cases should not hinder the timely initiation of plasmapheresis to prevent further injury and progression to chronic kidney disease.

Evaluating the Clinical Risk Index for Babies (CRIB) II Score for Mortality Prediction in Preterm Newborns: A Prospective Observational Study at a Tertiary Care Hospital.

INTRODUCTION: Neonatal mortality is an issue that affects both the developed and developing world. It is very important in the neonatal intensive care unit (NICU) to do the assessment of the severity of neonatal illness, which in turn helps in estimating and preventing mortality in the NICU by improving healthcare control and by rational use of resources. This research was carried out to evaluate how effectively the Clinical Risk Index for Babies (CRIB) II score can predict mortality rates among newborns treated in our NICU.  Methodology: This prospective observational study spanned one year, commencing in October 2021 and concluding in September 2022, within the confines of our NICU. The CRIB II score calculation was performed for included newborns, and the outcomes of the newborns were compared. A receiver operating characteristic (ROC) curve was obtained to ascertain the optimal CRIB II cut-off score for predicting mortality. RESULTS: Within the designated research timeframe, 292 neonates were admitted to the NICU. Forty-four newborns were enrolled in the study. Preterm neonates who died had higher CRIB II scores than those who survived, and their median (IQR) was 6 (1-12) vs. 9.5 (5-14) (p=0.0003). The estimate for the area under the curve was 0.83 (95% CI 0.68-0.92), and the odds ratio of 2.56 suggests neonates with a higher CRIB II score have higher chances of mortality. CONCLUSION: The CRIB II score is very good at predicting mortality in preterm newborns.

Neonatal hypernatremic dehydration in breastfed neonates: a prospective study unmasking the influences of breastfeeding practices and early weight monitoring.

Introduction: Hypernatremic dehydration in neonates is an uncommon but serious reason for re-hospitalization, especially in exclusively breastfed neonates. The aim was to study the incidence, associated maternal and neonatal characteristics and risk factors, and presenting features of neonatal hypernatremic dehydration (NHD). Methods: A prospective study design was employed to enroll full-term newborns admitted with serum sodium concentrations of ≥145 mEq/L from April 2022 to March 2023 at a tertiary care rural hospital. Maternal and neonatal characteristics and breastfeeding practices of these mother-baby pairs were recorded and observed. Healthy control for every mother-baby pair was taken. Ethical clearance and informed consent were obtained from mothers. Result: 34 newborns out of total 672 NICU admissions were admitted due to NHD, with an incidence of 4.7%. Primiparous mothers were 23 (67.6%) in the cases and 10 (29.4%) in the control group (p = 0.0017). Disparity in maternal breastfeeding practices of cases, such as delayed initiation time 2.3 h vs. 1.27 h (p < 0.0001), less frequency of breastfeeding 6.5 times vs. 9.3 times (p < 0.0001), and duration of breastfeeding sessions 23.3 min vs. 32 min (p = 0.0014) respectively in cases and controls were found to be potential contributing factors. 61.7% of mothers had breast issues in the cases and 17.6% in the control group (p = 0.0002) with average LATCH score of 4.29 in cases as compared to 8.08 in controls (p < 0.0001) at time of baby's admission to NICU. The average neonatal age at presentation was six days and average weight loss was 11.4% in cases vs. 2.8% in controls (p < 0.0001). The main presenting features were excessive weight loss 30 (88.2%), lethargy 20 (58.8%), jaundice 18 (52.9%) and fever 14 (41.1%). Conclusion: Neonatal hypernatremic dehydration (NHD) poses a significant clinical challenge, particularly in full-term, exclusively breastfed healthy neonates. We found an incidence of 4.7%. Delayed initiation of breastfeeding, inadequate breastfeeding techniques, and maternal breast-related issues were significant contributors to NHD. Primiparous mothers were found to be at higher risk, emphasizing the need for targeted breastfeeding education and support for primiparous mothers. The study reaffirmed the critical role of frequent and effective duration of breastfeeding and daily weight monitoring for preventing NHD.

Filling the Gaps for Feeding Difficulties in Neonates With Hypoxic-Ischemic Encephalopathy.

Hypoxic-ischemic encephalopathy (HIE) in neonates poses long-term feeding difficulties and abnormalities of swallowing, the sequel of which is growth impairment. Such infants are also at risk of impaired self-feeding in the grown-up stage along with other motor and tone abnormalities leading to malnutrition and multiple aspiration pneumonia episodes. The lack of evidence-based and pragmatic feeding strategies in such neonates is because of varied unrecognized symptoms and lacking validated diagnostic approaches. This article approaches evidence related to the pathophysiologic basis of feeding difficulties in neonates with HIE as well as standardizing measures and techniques to improve the feeding abilities of such babies and, in turn, their long-term development. The present review provides a scaffold for putting importance on this less taken care issue of feeding problems and emphasizes that more objective and evidence-based studies are required to be added to the literature for early interventions and management of this issue so that caregivers and neonatologists are not misguided by crude subjective opinions.