RESUMEN
We present a series of five cases diagnosed and treated as reactive haemophagocytic lymphohistiocytosis (HLH) in three tertiary referral centers of Kolkata, within a time frame of 3 months. The initial presentations were very variable, the most prominent clinical feature being - acute renal failure in the first patient, convulsions in the second, encephalopathy the third, marked cervical lymphadenopathy in the fourth and polyserositis in the fifth. All had a history of prolonged fever preceding admission and hepatosplenomegaly on examination. Investigations revealed multi-organ involvement with pancytopenia;- haemophagocytosis was eventually diagnosed by bone marrow examination. These cases highlight the diagnostic challenge posed by infection associated haemophagocytosis and the need for maintaining a high index of suspicion to promptly diagnose and treat this potentially life threatening condition.
Asunto(s)
Niño , Humanos , India/epidemiología , Linfohistiocitosis Hemofagocítica/complicaciones , Linfohistiocitosis Hemofagocítica/diagnóstico , Linfohistiocitosis Hemofagocítica/epidemiología , Pancitopenia/etiología , Convulsiones/etiología , Sepsis/etiologíaRESUMEN
Thirty children (45 units) in the age group 1 day-12 yrs with primary vesicoureteral reflux were studied prospectively and periodically assessed for renal function, scarring, grade of reflux and somatic growth parameters. Four children (6 units) with grade IV reflux underwent ureteric reimplantation. Complete resolution with medical management was seen in all 12 units of grade I-III reflux and in 5 of the remaining 27 units of grade IV-V reflux over 6 months-6 yrs. This group showed highly significant improvement in height . The weight gain of the above 2 groups was statistically significant compared to those with persistent reflux. Focal defects were seen initially in 62 percent refluxing units. None of the patients showed deterioration in renal function or formation of new scars over the next 1-7 years. Three children on conservative management showed persistent growth retardation with associated breakthrough infection, hypertension, multiple renal scars and poor renal functional volume.