Neurodevelopmental disorders in children aged 2-9 years: Population-based burden estimates across five regions in India.

BACKGROUND: Neurodevelopmental disorders (NDDs) compromise the development and attainment of full social and economic potential at individual, family, community, and country levels. Paucity of data on NDDs slows down policy and programmatic action in most developing countries despite perceived high burden. METHODS AND FINDINGS: We assessed 3,964 children (with almost equal number of boys and girls distributed in 2-<6 and 6-9 year age categories) identified from five geographically diverse populations in India using cluster sampling technique (probability proportionate to population size). These were from the North-Central, i.e., Palwal (N = 998; all rural, 16.4% non-Hindu, 25.3% from scheduled caste/tribe [SC-ST] [these are considered underserved communities who are eligible for affirmative action]); North, i.e., Kangra (N = 997; 91.6% rural, 3.7% non-Hindu, 25.3% SC-ST); East, i.e., Dhenkanal (N = 981; 89.8% rural, 1.2% non-Hindu, 38.0% SC-ST); South, i.e., Hyderabad (N = 495; all urban, 25.7% non-Hindu, 27.3% SC-ST) and West, i.e., North Goa (N = 493; 68.0% rural, 11.4% non-Hindu, 18.5% SC-ST). All children were assessed for vision impairment (VI), epilepsy (Epi), neuromotor impairments including cerebral palsy (NMI-CP), hearing impairment (HI), speech and language disorders, autism spectrum disorders (ASDs), and intellectual disability (ID). Furthermore, 6-9-year-old children were also assessed for attention deficit hyperactivity disorder (ADHD) and learning disorders (LDs). We standardized sample characteristics as per Census of India 2011 to arrive at district level and all-sites-pooled estimates. Site-specific prevalence of any of seven NDDs in 2-<6 year olds ranged from 2.9% (95% CI 1.6-5.5) to 18.7% (95% CI 14.7-23.6), and for any of nine NDDs in the 6-9-year-old children, from 6.5% (95% CI 4.6-9.1) to 18.5% (95% CI 15.3-22.3). Two or more NDDs were present in 0.4% (95% CI 0.1-1.7) to 4.3% (95% CI 2.2-8.2) in the younger age category and 0.7% (95% CI 0.2-2.0) to 5.3% (95% CI 3.3-8.2) in the older age category. All-site-pooled estimates for NDDs were 9.2% (95% CI 7.5-11.2) and 13.6% (95% CI 11.3-16.2) in children of 2-<6 and 6-9 year age categories, respectively, without significant difference according to gender, rural/urban residence, or religion; almost one-fifth of these children had more than one NDD. The pooled estimates for prevalence increased by up to three percentage points when these were adjusted for national rates of stunting or low birth weight (LBW). HI, ID, speech and language disorders, Epi, and LDs were the common NDDs across sites. Upon risk modelling, noninstitutional delivery, history of perinatal asphyxia, neonatal illness, postnatal neurological/brain infections, stunting, LBW/prematurity, and older age category (6-9 year) were significantly associated with NDDs. The study sample was underrepresentative of stunting and LBW and had a 15.6% refusal. These factors could be contributing to underestimation of the true NDD burden in our population. CONCLUSIONS: The study identifies NDDs in children aged 2-9 years as a significant public health burden for India. HI was higher than and ASD prevalence comparable to the published global literature. Most risk factors of NDDs were modifiable and amenable to public health interventions.

Immune complex reaction after successful treatment of meningococcal disease: an excellent response to IVIG.

The convalescence phase of severe meningococcal sepsis is complicated by immune complex reactions with arthritis being the commonest. No standard guidelines exist for management of such complications, but non-steroidal anti-inflammatory drugs and steroids have been used with varying success. We report excellent response to intravenous immunoglobulin in a child with immune complex reaction following meningococcal sepsis.

Fractured tracheostomy tube as foreign body bronchus: our experience with three cases.

OBJECTIVE: Tracheostomy tubes are extensively used in paediatric age group for airway issues. Their fracture and lodgement into trachea is an acute emergency requiring urgent intervention. CASES: We report three such paediatric cases having tracheostomy tube fracture and aspiration into trachea with different presentations and treatment outcomes. RESULTS: One patient was successfully managed with bronchoscopy and fractured tube removal. One patient succumbed to asphyxia before any intervention. The third patient was having supratubal tracheal stenosis making things more dangerous, but was managed successfully by tracheoscopy through tracheostomy opening with removal of fractured tube. CONCLUSION: Immediate identification is the key to successful management of this rare but life threatening situation. In the presence of tracheal stenosis above the tracheostomy opening, situation becomes more dangerous with very limited options for management.

Characteristics of pediatric scrub typhus during an outbreak in the North Eastern region of India: peculiarities in clinical presentation, laboratory findings and complications.

OBJECTIVE: To identify and report a recent outbreak of scrub typhus cases recorded from October 2009 to January 2010 in the state of Meghalaya, India. METHODS: The case sheets of all the children were retrieved and reviewed retrospectively. Twenty four (24) patients, who were both clinically and serologically confirmed as scrub typhus cases were included in the study. RESULTS: Fever for more than 1 wk duration was the most common manifestation (100%) followed by splenomegaly (45.8%), eschar (41.7%), cough (37.5%), hepatomegaly (33.3%), headache and body ache (25%), pain abdomen (25%), vomiting (20.8%), altered sensorium (16.6%), seizures (12.5%) lymphadenopathy (12.5%), and loose stools (8.3%). Meningoencephalitis was the most common complication (29.2%) followed by pneumonia (16.3%) and subconjunctival hemorrhage (8.3%).Cortical blindness, septic shock, peritonitis, myocarditis with CCF, pancytopenia, acute renal failure, coagulopathy, prolonged oxygen dependency and urinary tract infection (UTI) were found in one of each case. Hyponatremia (66.7%), elevated liver enzymes without significant rise of bilirubin (58.3%), hypoalbuminemia (52.2%) and thrombocytopenia (26%) were the other significant laboratory findings. Patients were treated with chloramphenicol, doxycycline and azithromycin. There was no mortality. CONCLUSIONS: This is the first outbreak report from the north eastern region of India with varied clinical presentations, laboratory investigations and complications. Weil Felix test still remains fruitful for diagnosing this disease in a resource limited set up.

Empyema thoracis: analysis of 150 cases from a tertiary care centre in North East India.

OBJECTIVES: To analyze the clinical characteristics, microbiological profile, management, complications and outcome of cases with empyema thoracis. METHODS: All cases diagnosed as 'empyema thoracis' over a period from January 2006 through June 2010 were identified from the inpatient records and discharge summaries. Of the 160 cases identified, 150 cases were taken up for analysis and the rest 10 cases, of which two had significant predisposing co-morbidity and eight cases diagnosed as tubercular empyema thoracis were excluded from the analysis. RESULTS: Mean age of presentation was 4.74 ± 3.53 years and two thirds of the children were under 5 years with male to female ratio of 1.42:1. Pus culture was positive in 32% (48 cases) with Streptococcus pneumoniae being the commonest organism isolated (31 cases, 64.6%) followed by Staphylococcus aureus (11 cases, 22.9%), Klebsiella pneumoniae (3 cases, 6.3%), Haemophilus influenzae type b (2 cases, 4.2%) and Enterococcus (1 case, 2%). Clustering was seen in the hot and humid months from April to July (63.3%). Fever was the commonest presentation (96.7%) followed by cough (90%), breathing difficulty (66.7%), chest pain (26.7%) and pain abdomen (10.7%). Ampicillin and cloxacillin was used as the first line antibiotic in 57.3% cases. Average duration of intercostal water seal drainage (ICWSD) in situ was 13.5 ± 8.05 days and 59 patients (39.3%) received fibrinolytic therapy. The commonest complications were collapse (18%), thickened pleura (16.7%), pericardial effusion (8%), cardiac tamponade (3.3%) and bronchopleural fistula (3.3%). Surgical procedures involved in this case series were decortication (14 cases, 9.3%), pericardiocentesis (5.3%), pericardiostomy (2.7%) and pericardiectomy (1.6%). Mortality was 3.3%. CONCLUSIONS: This is the first report of empyema thoracis from the north eastern region of India. Streptococcus pneumoniae was found to be the leading cause of empyema thoracis in this case series. Conservative management with ICWSD and antibiotics or early use of fibrinolytic therapy if indicated are effective modalities of treatment.

Unusual presentations of malaria in children: an experience from a tertiary care center in North East India.

OBJECTIVE: To identify cases of malaria with unusual presentations. METHODS: The medical record of all the cases of malaria admitted to PICU and pediatric general ward from Oct 2006 to Sep 2009, were reviewed and cases with unusual presentations were identified. The study design was retrospective descriptive study. RESULTS: Sixteen (10%) out of 162 malaria cases had unusual presentations - three had hemiplegia, two each with viral hepatitis-like presentation, acute abdomen, gastrointestinal bleed, generalized edema and hyperglycemia and one each with ptosis, severe headache and subacute intestinal obstruction-like presentation. Eleven cases had mixed parasitemia and two each with P. vivax and P. falciparum. One case was diagnosed on clinical grounds. CONCLUSION: Malaria is a common disease, but both typical and atypical presentations deserve attention for early diagnosis and management.

Macrophage activation syndrome in malaria.

Macrophage activation syndrome (MAS) is a clinical syndrome caused by an excessive proliferation of T lymphocytes and well-differentiated macrophages; an entity distinct from malignant histiocytosis. Although rheumatologic conditions are the common cause of MAS, a wide range of infections are also seen to cause MAS. We report an adolescent with severe Plasmodium falciparum malaria and MAS. He fulfilled six out of eight criteria required to diagnose hemophagocytic lymphohistiocytosis.

Parvovirus B19-induced multisystem disease simulating systemic vasculitis in a young child.

Parvovirus B19 mostly presents as a transient viral illness or as pure red cell aplasia. However, this virus can sometimes cause an illness that may pose diagnostic difficulties. We describe one such patient who had clinical features simulating a vasculitic disorder secondary to parvovirus B19 infection.

Transfusion associated graft versus host disease.

Transfusion-associated graft-versus-host disease (TA-GVHD) is a dreaded complication in immunocompromized hosts. The diagnosis is often delayed because of lack of awareness and the non-specific clinical features. More than 90% patients succumb to refractory infections. The only effective preventive measure is administration of irradiated blood products, which must be made available in centers managing immunocompromised patients. We report three cases and discuss pathophysiology and preventive strategies in this communication.

Toxic shock syndrome.

The authors present two children who had fever >or=38.9 degree C, diffuse rash, hypotension, deranged renal and hepatic functions, disseminated intravascular coagulation, altered sensorium and inflamed oral mucosa. They responded to fluids, inotropes, antibiotics and intravenous immunoglobulin (2 g/kg). Desquamation particularly of palms and soles and periungal region was noted 1 to 2 weeks after onset of illness. These features were consistent with the diagnosis of staphylococcal toxic shock syndrome (TSS). The cases highlight that TSS is very much with us and can mimic a variety of other diseases. Early recognition, and aggressive antimicrobial supportive and IVIG therapy cover can ensure complete recovery.

Varicella glomerulonephritis mimicking microscopic polyangiitis.

Varicella in childhood is usually a self-limiting illness with few complications. Varicella nephritis is an uncommon entity and seen mostly in immunocompromized individuals. We report a 14-year-old boy with juvenile rheumatoid arthritis who developed varicella nephritis and in whom the renal manifestations preceded the skin lesions by 1 week. This is an extremely unusual occurrence, and only one case has been described before. Such a presentation can mimic the clinical features of microscopic polyangiitis.

Status epilepticus: emergency management.

Status Epilepticus (SE) is a medical emergency and requires prompt and aggressive treatment. Stabilization of airway, breathing and circulation and expeditious termination of seizures are immediate goals. Intravenous benzodiazepines-diazepam, midazolam or lorazepam and phenytoin are the first line drugs recommended for termination of seizures. Diazepam (or midazolam), thiopental and propofol infusion are useful for control of Refractory SE (RSE). Newer drugs are being investigated for use in SE. We prefer diazepam infusion. In children the mortality from SE ranges from 3-10% and the morbidity is twice. Mortality and morbidity are highest with SE associated with CNS infections, which is the most important cause of SE in our country. The outcome depends on the underlying etiology, age, rapidity of SE and adequacy of care. Adherence to a time-framed protocol in the emergency department helps in improving the final outcome.

Hyponatraemia and hypovolemic shock with tuberculous meningitis.

A 12-year-old boy with tuberculous meningitis and hydrocephalous, after undergoing revision of ventriculo-peritoneal shunt had persistent impairment of sensorium and episodes of hyponatremia (serum sodium 104 to 125 mmol/l), accompanied by polyuria, signs of poor peripheral, perfusion hypotension and low CVP, and high urinary sodium excretion (114-60 mmol/l). A diagnosis of cerebral salt wasting syndrome (CSWS) was made and was treated with saline replacement and fludrocortisone (10 microg/kg/day). Within next 3 days the sensorium, signs of shock, urine output and serum and urinary sodium returned to normal. The case illustrates that life-threatening hyponatremia in a child with neurological illness could be caused by CSWS, which must be differentiated from Syndrome of inappropriate antidiuretic hormone secretion (SIADH), as CSWS requires rigorous salt and volume replacement in contrast to fluid restriction in SIADH.

Clinical approach to vasculitides.

The term vasculitis refers to inflammation and necrosis of blood vessels. The vasculitides are best classified according to the size of the involved vessels into large, medium and small vessel vasculitis. As a group the disorders are not uncommon even in children but the diagnosis of a given condition can often get delayed, or ever be entirely missed, because of multi-system involvement and the consequent protean clinical manifestations. Kawasaki Disease and Henoch-Schonlein Purpura are the commonest vasculitides seen in children. Early diagnosis and prompt treatment can go a long way in decreasing the morbidity and mortality associated with these disorders.