Syed Wajid Ali

Magnesium Sulfate in Severe Perinatal Asphyxia: A Randomized, Placebo-Controlled Trial

OBJECTIVE. The goal was to study whether postnatal magnesium sulfate infusion could improve neurologic outcomes at discharge for term neonates with severe perinatal asphyxia. METHODS. Forty term (≥37 weeks of gestation) neonates with severe perinatal asphyxia were studied in a prospective, longitudinal, placebo-controlled trial. Patients were assigned randomly to receive either 3 doses of magnesium sulfate infusion at 250 mg/kg per dose (1 mL/kg per dose) 24 hours apart (treatment group) or 3 doses of normal saline infusion (1 mL/kg per dose) 24 hours apart (placebo group). Both groups also received supportive care according to the unit protocol for perinatal asphyxia. RESULTS. In the treatment group, moderate encephalopathy was present in 35% (7 of 20) of the patients and severe encephalopathy in 65% (13 of 20) of patients at admission. In the placebo group, 40% (8 of 20) of patients had moderate encephalopathy and 60% (12 of 20) of patients had severe encephalopathy. The mean serum magnesium concentration in the treatment group remained at ≥1.2 mmol/L for 72 hours after the first infusion. At discharge, 22% (4 of 18) of infants in the treatment group had neurologic abnormalities, compared with 56% (10 of 18) of infants in the placebo group. Also, neuroimaging (head computed tomography) performed on day 14 yielded abnormal findings for fewer infants in the treatment group than in the placebo group (16% vs 44%). Infants in the treatment group were more likely to be receiving oral feedings (sucking) at discharge than were those in the placebo group (77% vs 37%). Good short-term outcomes at discharge occurred for 77% of the patients in the treatment group, compared with 37% of the patients in the placebo group. CONCLUSION. Postnatal magnesium sulfate treatment improves neurologic outcomes at discharge for term neonates with severe perinatal asphyxia.

Antiphospholipid Syndrome in a Neonate

Abstract:  Antiphospholipid syndrome presenting in the neonatal period is very rare. Although antiphospholipid antibodies from mothers with antiphospholipid syndrome can cross the placenta and put their neonates at risk, the occurrence of thrombotic complications in these neonates is uncommon. We present a 10‐day‐old neonate who developed Klebsiella sepsis with arterial gangrene of the left lower limb. Investigations revealed thrombosis of the left femoral artery with both the mother and the neonate positive for antiphospholipid antibodies. In conclusion, passive transfer of antiphospholipid antibodies from mothers to their offspring can be associated with significant complication in the presence of secondary risk factors.

Acute salt poisoning due to different oral rehydration solution (ORS) packet sizes.

The packing and composition of ORS has undergone a change since its introduction. In India, some companies are manufacturing smaller pouches (4.2 g) to be dissolved in 200 ml of water. Therefore, out of confusion some prescribers routinely advise the patients to dissolve the standard formulation ORS pouch (21 g) in a glass (200 ml) of water. Two cases are discussed. First patient developed salt poisoning due to improper dilution and recovered after rapid correction. In the second patient improper reconstitution led to hypernatremia and death.

Clinical and mutation profile of children with cystic fibrosis in Jammu and Kashmir

ObjectiveTo study the clinical and mutation profiles of children with cystic fibrosis in Jammu and KashmirMethodsOne hundred consecutive patients presenting with one or more phenotypic features suggestive of cystic fibrosis (CF) were screened by quantitative sweat chloride testing. For patients with positive/equivocal test result on two occasions, CFTR gene mutation analysis was done by polymerase chain reaction.ResultsOf the 100 patients, 18 (10 females) were diagnosed to have CF at a median age of 10.5 y (IQR 4.75–15.25 y) while the median age at the onset of symptoms was 12 mo (IQR 4–63 mo) with a delay in diagnosis by 102.4±80.5 months. Clinical features at presentation included failure to thrive (94.4%), chronic cough (78%), recurrent pneumonia (61%), persistent pneumonia (11%), and chronic diarrhea (50%). Positive sweat chloride (>60 meq/L) was seen in 14 (14%) patients and 4 (4%) patients had equivocal (40–60 meq/L) value on two different occasions. Mutational analysis done in 15 patients showed DeltaF508 mutation in 20% (3/15) patients in homozygous form and in 13% (2/15) patients in heterozygous form. Intron 19 mutation 3849+10kb C>T was found in 40% (6/15) in heterozygous form. One (6.6%) patient had DeltaF508 and 3849+10kbC>T mutations in compound heterozygous form. Patients with equivocal sweat chloride and 3849+10kbC>T mutation had delayed onset of pulmonary involvement.Conclusion3849 +10kbC>T mutation appears to be common in children with cystic fibrosis in Jammu and Kashmir followed by DeltaF508, although the data are quite limited. Although presentation is delayed and sweat chloride is in the equivocal range, severe lung involvement may occur in these patients.

Blood Pressure-to-Height Ratio as a Screening Tool for Hypertension in Children

ObjectiveTo test whether blood pressure-to-height ratio (BPHR) can be used to screen for hypertension in children.MethodsData regarding blood pressure and other variables was recorded for 2702 school children between the ages of 10-16 years as a part of a nutritional survey.ResultsThe optimal thresholds for defining hypertension in boys were 0.76 for systolic BPHR and 0.50 for diastolic BPHR; the respective threshold in girls were 0.80 and 0.52.ConclusionBPHR can be used as an effective screening test for diagnosing both hypertension and prehypertension in children aged 10–16 years.

Hereditary folate malabsorption with extensive intracranial calcification

BackgroundAnemia is a common accompaniment of cerebral palsy, mental retardation and neurodegenerative disorders.Clinical CharacteristicsA 4-year-old boy with chronic megaloblastic anemia, global developmental delay, seizures, intracranial calcification and new onset neuro-regression.ObservationA diagnosis of hereditary folate malabsorption was made, and he was put on oral and injectable folinic acid.OutcomeMarked improvement at 6 month follow up.MessageHereditary folate malabsorption should be suspected in any child having megaloblastic anemia and neuro degeneration disorder.

Pulmonary Thromboembolism as the Initial Manifestation in a Child With Antiphospholipid Syndrome in the Emergency Department

Antiphospholipid syndrome is characterized by recurrent arterial or venous thrombosis at any level of the vascular tree and the presence of circulating antiphospholipid antibodies. The syndrome may be idiopathic or secondary to an underlying autoimmune disorder. The disease is uncommon in children, and manifestations are diverse and underreported. We report the case of a 10-year-old boy who presented with features of pulmonary thromboembolism in the emergency department. Subsequently, he proved to have systemic lupus erythematosus with circulating antiphospholipid antibodies. He had no signs of systemic lupus erythematosus at presentation. In conclusion, antiphospholipid syndrome should also be kept as a possibility in children presenting for the first time with pulmonary thromboembolism in the emergency department.

Shoshin beriberi-thiamine responsive pulmonary hypertension in exclusively breastfed infants: A study from northern India

Objective To study the effect of thiamine administration on the resolution of pulmonary hypertension in exclusively breastfed infants. Design Prospective cohort study. Setting Hospital based study of a tertiary care hospital. Patients A total of 29 infants with 17 males (58.6%) and 12 females (41.4%) were included in the study. Intervention In addition to the management of shock, right heart failure and renal failure, patients received intravenous thiamine 100 mg/kg IV followed by 10 mg/day till introduction of supplementary feeds. Main outcomes measures Resolution of shock, metabolic complications and pulmonary hypertension. Results Mean age at presentation was 78.45 ± 30.7 days. All infants were exclusively breastfed. 86.2% of mothers were on customary dietary restrictions. Biventricular failure and tachycardia was commonly present. There were four deaths in our series. Acute metabolic acidosis was a universal feature with a mean pH of 7.21 ± 0.15. Pulmonary hypertension was present in all patients on admission. Intravenous thiamine 100 mg/kg IV stat was given immediately after documenting pulmonary hypertension. Repeat echocardiography showed complete resolution of pulmonary hypertension. Conclusion Many infants present to us with Shoshin beriberi with unusually high pulmonary pressures. These patients respond to thiamine challenge with prompt resolution of metabolic complications and reversal of pulmonary hypertension. We believe this is first of its kind from the region, which is reported.

Cerebrospinal fluid lactate: a differential biomarker for bacterial and viral meningitis in children

Objective To assess the performance of cerebrospinal fluid (CSF) lactate as a biomarker to differentiate bacterial meningitis from viral meningitis in children, and to define an optimal CSF lactate concentration that can be called significant for the differentiation.OBJECTIVE To assess the performance of cerebrospinal fluid (CSF) lactate as a biomarker to differentiate bacterial meningitis from viral meningitis in children, and to define an optimal CSF lactate concentration that can be called significant for the differentiation. METHODS Children with clinical findings compatible with meningitis were studied. CSF lactate and other conventional CSF parameters were recorded. RESULTS At a cut-off value of 3mmol/L, CSF lactate had a sensitivity of 0.90, specificity of 1.0, positive predictive value of 1.0, and negative predictive value of 0.963, with an accuracy of 0.972. The positive and negative likelihood ratios were 23.6 and 0.1, respectively. When comparing between bacterial and viral meningitis, the area under the curve for CSF lactate was 0.979. CONCLUSIONS The authors concluded that CSF lactate has high sensitivity and specificity in differentiating bacterial from viral meningitis. While at a cut-off value of 3mmol/L, CSF lactate has high diagnostic accuracy for bacterial meningitis, mean levels in viral meningitis remain essentially below 2mmol/L.

Prevalence of Depression among Caregivers of Indian Children with Cystic Fibrosis

ObjectiveTo study the prevalence of depression among caregivers of children with cystic fibrosis and its impact on the health and well being of these children.MethodsThis cross-sectional study was conducted in a tertiary care hospital from September 2015 through August 2016. Forty one parents of children receiving treatment at the Cystic fibrosis (CF) clinic were approached to be part of the study. Six families declined the request resulting in 85% recruitment rate. The Centre for Epidemiological Studies Depression Scale (CES-D) was used to assess depression score among caregivers. The CES-D provides clinical cut-off scores of ≥16 that help in identifying persons at risk for depression. CES-D was completed by the parent closely associated with care of the affected child. Main outcome measure was to find the number of caregivers of patients who has score of ≥16 on CES-D scale, and its effect on growth and respiratory exacerbations of the affected child.ResultsA total of 23 fathers and 12 mothers participated in the study. The mean age of male and female caregivers was 30.9 ± 5.4 and 27.8 ± 4.7 y respectively. Eighteen (51.4%) caregivers scored above the clinical cut-off on the CES-D in the index study with mean score of 22.0 ± 4.0. The mean CES-D score among non-depressive caregivers was 7.76 ± 4.2. Significant negative association was found between parental depression and child’s health. Children with high parental CES-D score suffered significantly more respiratory exacerbations (3.83 ± 1.2 episodes) in last six months than parents with low CES-D score (2.18 ± 1.28 episodes) (p value = 0.00). Similarly, stunting was more commonly seen in patients with high caregiver CES-D score (15 vs. 7; P value = 0.01).ConclusionsA very high prevalence of caregiver depression was found in cystic fibrosis, which negatively impacted care and well being of the affected patients. Depression was more common in families with poor economic and education level.