The role of CT brain findings in the early diagnosis of infantile encephalitic beriberi.

BACKGROUND: Thiamine deficiency disease may occur in infants from thiamine-deficient mothers in developing countries, as well as in infants fed solely with soy-based formula. Thiamine deficiency in infants may present with acute neurological manifestations of infantile encephalitic beriberi. OBJECTIVE: To review the role of noncontrast CT brain findings in infantile encephalitic beriberi in early diagnosis. MATERIALS AND METHODS: A retrospective review of noncontrast CT scans of the brain in 21 infants with acute-onset infantile encephalitic beriberi was carried out. RESULTS: On noncontrast-enhanced CT brain, hypodense lesions were seen symmetrically in the putamen in all the babies; symmetric hypodensities were seen in the caudate nuclei in 14/21 (67%), in dorsomedial thalami/hypothalamic/subthalamic area in 4/21 (19%), and in the globi pallidi in 2/21 (9.5%) of the infants. CONCLUSION: Recognition of symmetrical hypodense lesions in the basal ganglia and medial thalami/hypothalamic/subthalamic area on noncontrast CT scan of the brain are important early features to recognize in encephalitic beriberi in at-risk infants. ADVANCES IN KNOWLEDGE: IEBB is a cause of hypodense bilateral basal ganglia and may be identified by this finding in the appropriate clinical settings.

Infantile thiamine deficiency: Redefining the clinical patterns.

OBJECTIVES: Thiamine deficiency (TD) is frequently suspected and treated at our hospital. In our retrospective study, we aimed at finding the clinical and laboratory spectrum of infantile TD presenting to a single center over a period of time. METHODS: The diagnosis was made on criterion standard of response to thiamine challenge. RESULTS: TD was suspected in 189 infants at admission; 43 infants were diagnosed as having TD in three distinct forms and a fourth group with mixed presentation. The first group (n = 30), which was the youngest (mean age = 67 d), was always associated with lactic acidosis. They had history of reflux and suddenly became irritable and developed acidotic breathing. This further worsened into shock (46%) and acute respiratory failure (50%). The second group (n = 5) presented with pulmonary arterial hypertension. They had hoarseness of voice and irritability. Chest radiograph showed prominent pulmonary conus. Their clinical course was complicated by congestive heart failure in three. Echocardiographic response to thiamine was uniformly seen within 3 d in this group. The clinical presentation of infants with Wernicke's encephalopathy (n = 5) who were the oldest of all (mean age = 190 d) was constantly marked by presence of bilateral ptosis and encephalopathy preceded by occurrence of vomiting. Their head ultrasonography showed presence of hyperechoic basal ganglia. CONCLUSIONS: Three clinically distinct forms of TD were recognized. Lactic acidosis was a universal finding in acidotic form. Infants with pulmonary hypertension as primary presentation are typically associated with aphonia. Infants with Wernicke's encephalopathy can be clinically diagnosed by presence of encephalopathy and ophthalmic signs (ptosis).

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 100mg/kg IV followed by 10mg/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 100mg/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.

Epidemiology of bronchial asthma in school children (10-16 years) in Srinagar.

OBJECTIVES: To assess the epidemiological profile of asthma in school going children in Srinagar, Kashmir. STUDY DESIGN: Cross-sectional study. SETTING: Thirty-one schools with proportionate representation from both government and private schools as well as from primary, middle, and high schools. PARTICIPANTS: School children aged 10-16 years with equal representation of sex and all ages. MAIN OUTCOME MEASURE: Prevalence of current and past asthma. METHODS AND RESULTS: After administering a modified pretested questionnaire, peak expiratory flow measurement was carried. Children who had asthma-like symptoms or positive family history of asthma or physician-labeled asthma were subjected to spirometry and bronchodilator reversibility. Out of 806 children, bronchial asthma was seen in 60 (prevalence of 7.4%) which included 34 boys and 26 girls. Majority of asthmatic children (78.3% [n = 47]) had probable asthma; 6.7% (n = 4) had definite asthma; and 15% (n = 9) had physician-diagnosed asthma. Majority of children had intermittent asthma (78.3% [n = 47]). Mild persistent asthma was seen in 12.7% (n = 7) and 10% (n = 6) had moderate persistent asthma. None of the children had severe persistent asthma. The prevalence of current asthma was 3.2% (n = 26). On univariate analysis, the factors found to be statistically significant were family history of asthma (odds ratio [OR] =8.174; confidence interval [CI] =4.403-15.178), seasonal cough (OR = 4.266; CI = 2.336-7.791), allergic rhinitis (OR = 2.877; CI = 1.414-5.852), atopic dermatitis (OR = 6.597; CI = 2.72-16.004), and obesity (OR = 6.074; CI = 2.308-18.034). On multivariate analysis, family history, seasonal cough, allergic rhinitis, atopic dermatitis, and obesity were found to be significant independent risk factors. CONCLUSIONS: Srinagar qualifies as a low prevalence area for bronchial asthma in the age group of 10-16 years. Majority of children had mild intermittent asthma resulting in under diagnosis and wrong treatment.

Thiamine responsive acute life threatening metabolic acidosis in exclusively breast-fed infants.

BACKGROUND: Acute life threatening metabolic acidosis in exclusively breast fed infants due to thiamine deficiency is not described. Kashmir valley, a north Indian state has a population that largely consumes polished rice. METHODS: A six months prospective descriptive study of infants who presented with acute life threatening metabolic acidosis (Blood pH ≤ 7.0) due to thiamine deficiency. RESULTS: Twenty three infants (Eleven male; Twelve female) in the age range of 32 days to 4 months had a pH of ≤7 at admission. Onset of moaning was immediate (2-24 hours). Blood lactate levels were more than 15mmol/L. Blood thiamine levels of six infants in whom it was done ranged from 11-69 nmol/L (control 78-185 nmol/L). All infants were exclusively breast fed. Maternal staple diet consisted of polished rice. All mothers consumed rice after washing it thrice. Twelve lactating mothers were on customary dietary restrictions. Practice of straining rice after cooking was observed in thirteen. The commonest symptoms were irritability (82%) and reflux (56%). Commonest signs were tachycardia (100%) and moaning (73%). At presentation 52% were in cardiogenic shock. Response to thiamine was dramatic with moaning and irritability subsiding in two hours and tachycardia in four hours. Adequate perfusion was achieved in one hour. Eighteen patients seen at six months follow up had normal neurodevelopment. CONCLUSIONS: Thiamine deficiency in an infant can present as sudden onset metabolic acidosis. If treated early, metabolic acidosis due to thiamine deficiency is associated with good immediate and long term prognosis even if pH is less than 7 at presentation.

Clinical Epidemiology of SIRS and Sepsis in Newly Admitted Children.

OBJECTIVE: To assess the clinical and demographic profile of Systemic Inflammatory Response Syndrome (SIRS) and sepsis, among newly admitted children in different age groups in a hospital in North India. METHODS: This prospective study was conducted at a referral care centre in Northern India. All children, age group 0 to <18 y, admitted on days selected for study were screened and those with abnormal temperature and abnormal leukocyte count were included for further assessment. A total of twenty "24 h" periods were randomly chosen during the study period. Patients were assessed according to age specific vital signs and laboratory values to diagnose Systemic Inflammatory Response Syndrome (SIRS) and sepsis and to gain clinical and demographic data. The criteria laid at International consensus conference, 2002, were used to define patients as SIRS, Sepsis, Severe sepsis and Septic shock. RESULTS: During the study period, a total of 865 patients were screened for SIRS. Prevalence of SIRS amongst hospitalised children was 23 % (n = 201). Seventy nine percent (n = 159) of patients had infection associated SIRS and 21 % (42) had non-infective SIRS. Sixty four percent (n = 129) SIRS patients had uncomplicated sepsis, 15 % (n = 30) patients fulfilled criteria for severe sepsis. Out of the latter 30, 19 had septic shock. Organ dysfunction in SIRS was noted in 25 % (n = 51). 37.25 % (n = 19) had multiple organ dysfunction syndrome (MODS). The most common organism isolated was Staphylococcus aureus (n = 9). Focus of infection in majority was pulmonary (44 %). Mean duration of antibiotic therapy and hospital stay in the SIRS group were 6.4 and 6.5 d respectively. In the group without SIRS, mean duration were 2.44 d and 3.07 d respectively The differences were statistically significant. CONCLUSIONS: In conclusion, the proportion of sepsis contributing to SIRS is high in a tertiary care hospital. Therefore rapid recognition of SIRS is essential. Goal directed treatment of sepsis is also important so that high mortality associated with severe sepsis and septic shock are prevented.

Snake bite envenomation in children in Kashmir.

A three year retrospective study was done to study snakebite envenomation among Kashmiri children. Ten children were admitted with snakebite. Snakebite was vasculotoxic in seven and mixed in two. Levantine viper bites were seen in five who had Grade 3 bites with severe local signs, severe coagulopathy and hypotension. Both environmental risk and seasonal incidence was observed.

Klebsiella brain abscess in a neonate.

Klebsiella pneumoniae is rarely associated with neonatal cerebral abscess. A case of Klebsiella brain abscess in a neonate is described. Diagnosis of abscess was confirmed by magnetic resonance imaging and ultrasound-guided aspiration.

Parry-Romberg syndrome associated with unusual intracranial vascular malformations and Phthisis bulbi.

Neurovascular anomalies of Parry-Romberg syndrome have been reported infrequently. We report a case of Parry-Romberg syndrome with hypoplastic left internal carotid, middle cerebral, anterior cerebral, posterior communicating and posterior cerebral artery. The patient presented with partial seizures, hemiparesis and phthisis bulbi.

Neonatal adrenal hemorrhage presenting as late onset neonatal jaundice.

Clinical manifestations of adrenal hemorrhage vary depending on the degree and rate of hemorrhage, as well as the amount of adrenal cortex compromised by hemorrhage. We report here a case of neonatal adrenal hemorrhage that presented with late onset neonatal jaundice. The cause of adrenal hemorrhage was birth asphyxia.