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).

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.