Intussusception in children aged under two years in India: Retrospective surveillance at nineteen tertiary care hospitals.

OBJECTIVE: Intussusception has been linked with rotavirus vaccine (RVV) as a rare adverse reaction. In view of limited background data on intussusception in India and in preparation for RVV introduction, a surveillance network was established to document the epidemiology of intussusception cases in Indian children. METHODS: Intussusception in children 2-23 months were documented at 19 nationally representative sentinel hospitals through a retrospective surveillance for 69 months (July 2010 to March 2016). For each case clinical, hospital course, treatment and outcome data were collected. RESULTS: Among the 1588 intussusception cases, 54.5% were from South India and 66.3% were boys. The median age was 8 months (IQR 6, 12) with 34.6% aged 2-6 months. Seasonal variation with higher cases were documented during March-June period. The most common symptoms and signs were vomiting (63.4%), bloody stool (49.1%), abdominal pain (46.9%) and excessive crying (42.8%). The classical triad (vomiting, abdominal pain, and blood in stools) was observed in 25.6% cases. 96.4% cases were diagnosed by ultrasound with ileocolic location as the commonest (85.3%). Management was done by reduction (50.8%) and surgery (41.1%) and only 1% of the patients' died. 91.1% cases met Brighton criteria level 1 and 3.3% Level 2. Between 2010 and 2015, the case load and case ratio increased across all regions. CONCLUSION: Intussusception cases have occurred in children across all parts of the country, with low case fatality in the settings studied. The progressive rise cases could indicate an increasing awareness and availability of diagnostic facilities.

Serotype-specific differences in the laboratory parameters among hospitalized children with dengue and genetic diversity of dengue viruses circulating in Tamil Nadu, India during 2017.

In 2017, Tamil Nadu, a southern state, had the second highest number of dengue cases from India. In the present study, the serotype-specific differences in the clinical manifestations and laboratory parameters among hospitalized children with dengue were investigated and molecular characterization of the circulating dengue virus (DENV) serotypes during 2017 in Tamil Nadu was performed. Eighty children with dengue-like symptoms consecutively admitted to a tertiary care hospital and positive for DENV NS1 antigen were investigated for DENV serotype utilizing a real-time reverse transcriptase based polymerase chain reaction assay. Complete envelope (E) gene sequencing of the DENV strains was performed. Seventy samples were positive for serotyping (25 DENV-1, 17 DENV-2, six DENV-3, and 22 DENV-4). DENV-4 infections were associated with elevated levels of liver enzymes; Alanine aminotransferase (P = .021) and aspartate aminotransferase (P = .001). However, none of the serotype was associated with any specific clinical features and severe dengue. Asian and American/African genotypes of DENV-1 were cocirculating. The circulating genotype was cosmopolitan for DENV-2 with multiple lineages, genotype III for DENV-3 and genotype I for DENV-4. Unique mutations were present in the 2017 DENV-4 isolates. The present study suggests the association of DENV-4 with elevated liver enzymes in children hospitalized for dengue. Further, the study reports the genetic diversity of DENV circulating in Tamil Nadu during 2017. The study calls for continuous monitoring of the circulating serotypes and genotypes at regional level in India which might result in a region wise database useful in predicting future outbreaks.

A novel nine base deletion mutation in NADH-cytochrome b5 reductase gene in an Indian family with recessive congenital methemoglobinemia-type-II.

Recessive hereditary methemoglobinemia (RCM) associated with severe neurological abnormalities is a very rare disorder caused by NADH- cytochrome b5 reductase (cb5r) deficiency (Type II). We report a case of 11 month old male child who had severe mental retardation, microcephaly and gross global developmental delay with methemoglobin level of 61.1%. The diagnosis of NADH-CYB5R3 deficiency was made by the demonstration of significantly reduced NADH-CYB5R3 activity in the patient and intermediate enzyme activity in both the parents. Mutation analysis of the CYB5R gene revealed a novel nine nucleotide deletion in exon 6 leading to the elimination of 3 amino acid residues (Lys173, Ser174 and Val 175). To confirm that this mutation was not an artifact, we performed PCR-RFLP analysis using the restriction enzyme Drd I. As the normal sequence has a restriction recognition site for Drd I which was eliminated by the deletion, a single band of 603-bp was seen in the presence of the homozygous mutation. Molecular modeling analysis showed a significant effect of these 3 amino acids deletion on the protein structure and stability leading to a severe clinical presentation. A novel homozygous 9 nucleotide deletion (p.K173-p.V175del3) is shown to be segregated with the disease in this family. Knowing the profile of mutations would allow us to offer prenatal diagnosis in families with severe neurological disorders associated with RCM - Type II.

Nephrotic syndrome following a single bee sting in a child.

The occurrence of nephrotic syndrome following a bee sting is rarely reported in the literature. Hypersensitivity is believed to be the precipitating factor for the renal disease. We report a two-year-old boy, who developed generalized edema and decreased urine output, seven days after a bee sting. Physical examination and laboratory findings were consistent with nephrotic syndrome; and corticosteroid treatment induced prompt remission with resolution of clinical symptoms and normalization of laboratory findings. There was no relapse of the disease during a one-year follow up.

Leucocyte adhesion defect-1.

Leucocyte adhesion defect (LAD) is an inherited disorder of phagocytic function. It is characterized by inability of the leucocytes, in particular neutrophilis to migrate from the blood stream towards sites of inflammation. LAD -1 is characterized by the absence of b 2 integrins (CD 11 / CD18) on leucocytes. This disorder is characterized by delayed separation of the umbilical cord, recurrent severe infections, periodontitis, and delayed wound healing. We report an infant where the diagnosis was confirmed by flow cytometry.

Symptomatic hepatic hemangioendothelioma in a newborn.

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.

Radiological evaluation of the urinary tract in children with urinary infection.

OBJECTIVE: This article in to study the association of structural abnormalities of the urinary tract in children with urinary tract infection (UTI) using ultrasound examination. METHODS: 262 children with culture proven urinary tract infection were studied. Antibiotics were given as per sensitivity pattern. All children had an ultrasound of the abdomen done within 3 weeks. A micturating cystourethrogram (MCU) was done in those with abnormalities of the lower urinary tract detected on ultrasound, as well as in those who had recurrence of infection, after a normal ultrasound. IVU and renal isotope scans were done in selected cases. RESULT: All children were followed up until one year after the study period. Fifty-four patients had an underlying urinary tract anomaly; 42 were picked up by ultrasound and 12 by MCU. 22.9% of males and 15.9% of females had anomaly of the urinary tract. Children less than 2 years had the highest incidence of anomalies. CONCLUSION: Pelviureteric junction obstruction with hydronephrosis, vesicoureteric reflux and non-refluxing megaureter are the major anomalies picked up. 20% of children with urinary tract infections have an underlying structural abnormality of the urinary tract, three-fourth of which are picked up on ultrasound. An ultrasound abdomen is recommended in all children after the first UTI. In addition, an MCU is also indicated in all boys below 2 years with UTI, since one-third of anomalies will be missed if only ultrasound is done.