Progeria with unusual ocular manifestations: report of a case with a review of the literature.
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Biomedical subjects
Publications and source records attributed to O N Bhakoo.
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Auditory evoked responses using BERA were studied in 30 newborn babies with plasma bilirubin > or = 15 mgm/dl and repeated after treatment of neonatal jaundice with bilirubin levels of < or = 10 mgm/dl. A few jaundiced babies (16.5%) showed absent BERA response at the initial/subsequent examination. After treatment, 3/30 babies showed absent wave form responses and 2 of these were clinically kernicteric. Jaundiced babies had prolonged wave peak latencies and inter peak latencies. Treated babies showed a tendency towards recovery in their BERA responses which were however not complete. Total plasma bilirubin value at the time of BERA examination and mean maximal bilirubin values had no correlation with the incidence and degree of BERA abnormalities.
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A retrospective study was done to review the clinical experience of septic arthritis and osteomyelitis in the newborns in our centre. Case records of all the neonates born from January 1989 to August 1994 and those admitted to outborn nursery from 1985 to 1993 were reviewed. Diagnosis of septic arthritis/osteomyelitis was made in the presence of relevant clinical signs and supported by positive culture from blood or joint fluid and abnormal X-ray or ultrasound findings. The incidence of septic arthritis and osteomyelitis among inborn babies was 1 in 1500. There were 25 neonates with mean gestational age 34.5 (range 27-40) weeks and mean birth weight 2269 (range 990-4750) gms. Limitation of movement (64%) and local swelling (60%) were commonest presentations. A total of 33 joints were involved in 25 babies. Eight babies (32%) had multiple joint involvement. Hip and knee were the most commonly involved joints (48% each). In 19 babies (76%) joint involvement occurred in association with a generalized septicemic illness while 6 babies (24%) had localised signs and symptoms. Joint aspirate was positive for gram stain or culture in 12 (48%) and 10 babies (40%) had positive blood culture. Klebsiella pneumoniae and Staphylococcus aureus were commonest isolates. Radiological changes were seen in 13 (52%) babies. All were treated with appropriate antibiotics and open surgical drainage was done in 5 (20%) cases. Bone and joint infections are important complications in sick septicemic neonates and need early diagnosis, appropriate management with antibiotics, surgical drainage in selected cases to prevent long term morbidity.
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Persistent pulmonary hypertension of the newborn (PPHN) characterised by right to left shunting with intense cyanosis is difficult to manage, and in the best of centres carries a 40-60 percent mortality. We report our one year's experience of managing six neonates with PPHN. There were 5 males and 1 female with mean birth weight of 2.59 +/- 0.487 kg and gestation period 39 +/- 2.0 wks and 1 minute Apgar score 2.8 +/- 2.1. Four to six babies were born by cesarean section and 3-6 babies had aspiration pneumonia. All babies presented within 12 hours of age (mean 5.08 +/- 5 hrs) with intense cyanosis and respiratory distress. Diagnosis were confirmed in all by (a) hyperoxia test, (b) simultaneous determination of preductal and postductal paO2 (c) contrast echocardiography and (d) hyperoxia-hyperventilation test. Babies were managed with hyperventilation using mean ventilatory rates of 100 +/- 45 per minute, an inspired oxygen concentration of 100%, peak inspiratory pressures 27 +/- 9 cm of H2O, and expiratory pressures 5 +/- 1.6 cms of H2O, and mean air way pressures of 10.4 +/- 2.7 cms H2O. Alkali therapy was used in 3 of the six babies whereas low dose dopamine was infused in all six babies. Inspite of aggressive ventilatory therapy, only 3 out of 6 babies could be salvaged.
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No information is available in the literature about the true incidence of congenital ocular abnormalities and associated systemic abnormalities in an Indian population. Initial results based on the first year of this study are presented. Out of 2,153 children born from January 1978 to December 1978, 2,016 were examined for any congenital ocular or systemic abnormality. The incidence of congenital ocular and systemic abnormalities was 10.5/1000 and 20/1000 live births respectively. Four out of 21 congenital ocular abnormalities were associated with congenital systemic abnormalities. Individual abnormalities are discussed in brief.
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