Massive fetomaternal hemorrhage with persistent pulmonary hypertension in a neonate.
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Biomedical subjects
Publications and source records attributed to S K Patole.
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Hyperekplexia (startle disease) is a rare non-epileptic disorder characterised by an exaggerated persistent startle reaction to unexpected auditory, somatosensory and visual stimuli, generalised muscular rigidity, and nocturnal myoclonus. The genetic basis is a mutation usually of the arginine residue 271 leading to neuronal hyperexcitability by impairing glycinergic inhibition. Hyperekplexia is usually familial, most often autosomal dominant with complete penetrance and variable expression. It can present in fetal life as abnormal intrauterine movements, or later at any time from the neonatal period to adulthood. Early manifestations include abnormal responses to unexpected auditory, visual, and somatosensory stimuli such as sustained tonic spasm, exaggerated startle response, and fetal posture with clenched fists and anxious stare. The tonic spasms may mimic generalised tonic seizures, leading to apnoea and death. Consistent generalised flexor spasm in response to tapping of the nasal bridge (without habituation) is the clinical hallmark of hyperekplexia. Electroencephalography may show fast spikes initially during the tonic spasms, followed by slowing of background activity with eventual flattening corresponding to the phase of apnoea bradycardia and cyanosis. Electromyography shows a characteristic almost permanent muscular activity with periods of electrical quietness. Nerve conduction velocity is normal. No specific computed tomography findings have been reported yet. Clonazepam, a gamma aminobutyric acid (GABA) receptor agonist, is the treatment of choice for hypertonia and apnoeic episodes. It, however, may not influence the degree of stiffness significantly. A simple manoeuvre like forced flexion of the head and legs towards the trunk is known to be life saving when prolonged stiffness impedes respiration.
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In a five-year retrospective data analysis, incidence of > stage II necrotising enterocolitis (NEC) was four times higher in aboriginal (18/125) than non-aboriginal (11/306) neonates, all < or = 32 weeks' gestation. Stage III NEC occurred more frequently (10/18 vs 3/11) and related mortality was higher (44.4% vs 0%) in aboriginal than non-aboriginal neonates. Risk factors for NEC-prolonged rupture of membranes (50% vs 9.1%, p = 0.01), prematurity (median [range] gestational age: 25 (24-28.5) vs 30 [27-33]) weeks, p = 0.02), birth weight (< 1 kg 13 [72%] vs 2 [18%], p = 0.007) and intrauterine growth retardation (50% vs 0%, p = 0.01) occurred more frequently in aboriginal neonates. Though feed type and increments per day were similar, aboriginal neonates received higher volume of feeds/kg/day (median [range]: 154 [145-189] vs 106 [103-134] ml, p < 0.05). Condition at delivery and respiratory status before development of NEC were not significantly different.
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Aspiration of uncontaminated amniotic fluid as a cause of significant respiratory distress in neonates is not well documented. We report a term neonate who presented with severe respiratory distress soon after birth. The diagnosis of amniotic fluid aspiration was confirmed at autopsy. Implications of this diagnosis in the management of neonates with respiratory distress are discussed.
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OBJECTIVE: To study neonatal outcomes associated with gastroschisis and exomphalos in a regional neonatal unit. METHODS: A retrospective (1988-97) data analysis to study the effect of the type of defect/surgery, mode/place of delivery and associated anomalies on time to start and reach full feeds, duration of total parental nutrition (TPN) support and total hospital stay. Exact bivariate test procedures were used for data analysis. RESULTS: Twenty-one cases of gastroschisis (17 inborn) and five cases (four inborn) of exomphalos were identified. Of these, 23.8% cases of gastroschisis and 60% of cases of exomphalos had associated gut anomalies. The survival rates for gastroschisis and exomphalos were 91 and 100%, respectively. The median time to start and reach full enteral feeds in outborn neonates was longer than in inborn neonates (9 vs 25 days, respectively, P = 0.01; and 16 vs 49 days, respectively, P = 0.01), as was the duration of TPN support (14 vs 42 days, respectively; P = 0.02). Neonates with gastroschisis had significant delays in starting and reaching full feeds compared with neonates with exomphalos (median 13 vs 4.5 days, respectively, P = 0.03; and 24 vs 8, respectively, P = 0.02) and they required prolonged support with TPN (median 23 vs 6 days, respectively; P= 0.01). Antenatal detection was significantly more frequent in inborn compared with outborn neonates (100 vs 67%, respectively; P = 0.03). The severity of associated gut anomalies and the delivery to surgery interval did not differ significantly to explain the increased morbidity in outborn neonates. Outcome was not significantly different after analysis by type of surgery and mode of delivery. CONCLUSIONS: Increased morbidity in outborn neonates may be related to factors such as temperature, care, hydration status, care of the defect and vascular compromise of prolapsed gut during prolonged transportation.
Advances in the surfactant era have resulted in a dramatic rise in the survival of 23-26 week old neonates. For the group as a whole, survival is 50% with a 50% morbidity in survivors. Current literature includes a few 'small baby protocols' suggesting guidelines for management based on institutional experiences. Adopting these with or without any modifications may not be appropriate for other institutions due to the diversity of the patient population involved and the available resources. A review of literature on ELBW neonates reveals an interesting fact that almost all the management strategies and optimal goals in the most critical first few weeks of life involve two numbers--'fifty' and 'eight'! The relevance of these two numbers (or their multiples) in simplifying the management of ELBW neonates is highlighted.
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Bacillus cereus is a Gram-positive aerobic or facultatively anaerobic spore-forming rod, which usually causes food poisoning. Its recognition as a pathogen in neonates has increased over the past two decades. The clinical course of a neonate (gestation 24 weeks) with B. cereus infection refractory to therapy is described. Death occurred after withdrawal of support following persistently positive blood and bone marrow cultures despite therapy with vancomycin, gentamicin, imipenum, clindamycin, ciprofloxacillin, immunoglobulin and granulocyte colony stimulating factor over a period of 49 days. No obvious focus of sepsis was identified. Contamination from the environment into the hospital and clinics occurs because of the ubiquitous presence of B. cereus. Combination therapy with vancomycin and gentamycin is appropriate for meningitis/severe systemic infections related to most bacillus species. The significance of repeated isolation of B. cereus in neonates with compromised host defences is emphasised.
The feeding regimen was standardised for a trial of erythromycin to reduce the time to reach full feeds (150 ml/kg/day) by 30% in neonates of < or = 32 weeks gestation. No significant improvement was noted in the primary outcome (median time: erythromycin 93.5 vs placebo 104 hours, p = 0.60). However, necrotising enterocolitis > or = stage II disappeared and the time to full feeds was reduced by over 50% in all neonates during the 18-month trial, and for more than two years after the trial, when the standardised feeding regimen was adopted as routine policy for feeding neonates of < or = 32 weeks (< 28 weeks: 13 vs 4.8 days, p < 0.05; > 28 weeks: 8 vs 3.9 days, p < 0.05). This was in contrast to an average of six cases of NEC per year with 45% mortality during the previous five years. The benefits of standardised feeding schedules--improved detection/treatment of signs/symptoms of feed intolerance--are emphasised.
Efficacy of oral, prophylactic erythromycin in reducing the time to establish full enteral feeds (150 ml/kg/day) was assessed in neonates < 32 weeks, ready for enteral feeds. Seventy-three consecutive neonates were randomised to receive oral erythromycin ethyl succinate (n = 36) or placebo (n = 37) in a double-blind trial until full enteral feeds or 14 days of therapy were reached. A prospectively designed feeding regimen, including plan of action for signs of feed intolerance, was common for all enrolled neonates. The median gestational age, birth weight and postnatal age at start of feeds were 29 versus 30 weeks (p = 0.40), 1232 versus 1280 g (p = 0.96) and 5 versus 5 days (p = 0.84) for erythromycin and placebo group, respectively. Time to achieve full feeds was not significantly different in the two groups. (median times: erythromycin 93.5 versus placebo 104 hours, p = 0.60). Erythromycin-related side-effects did not occur.
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Hypertension hyponatremia syndrome occurred in a 32-week male neonate following septicemic shock on Day 9. The systolic blood pressure rose from 60 to 85 mmHg as the serum sodium dropped from 136 to 121 mmol/L associated with natriuresis, polyuria, and dehydration. Convulsions occurred at a systolic blood pressure of 102 mmHg. Investigations for hypertension revealed hyper-reninemia without cardio/renovascular or neuroendocrine abnormalities. Salt supplementation and antihypertensive therapy with captopril led to resolution of natriuresis and hyponatremia. Review of literature revealed associated renovascular pathology in all neonatal cases of the syndrome reported so far. Renal ischemia from possible renal microthrombi may have been the triggering event in our case. Decline in renin levels during follow-up favors this hypothesis.
Four cases of intracardiac fungal masses occurred over 2 y amongst 7 cases of systemic candidiasis in a neonatal referral unit. The gestations and birthweights were 25, 23, 24 and 30 wk and 805, 605, 640 and 1395 g, respectively. The pedunculated, solitary right atrial masses were detected 2-17 d after diagnosing candidemia in 3 cases, whereas it was the presenting feature in the 4th. All had indwelling right atrial catheters and received multiple courses of broad-spectrum antibiotics. The masses were removed successfully in two cases fit for surgery. None survived despite antifungal therapy, including liposomal amphotericin B at 6 mg/kg/d. Early introduction of enteral feeds, minimization of prolonged exposure to broad-spectrum antibiotics and judicious use of central catheters may reduce the incidence of systemic candidiasis in high-risk neonates. Surveillance echocardiography and timely surgical intervention may reduce the mortality and/or morbidity related to intracardiac fungal masses.