Alveolar capillary dysplasia. Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature

We describe an unusual infant with the diagnosis of alveolar capillary dysplasia who had a relatively prolonged life without extracorporeal membrane oxygenation (ECMO). We have used this case as a springboard for a thorough review of the literature. This was a full-term female infant who presented with a picture of persistent pulmonary hypertension of the newborn. She was treated as such, with various ventilatory modes, alkalinizing agents, surfactant therapy, tolazoline, prostacyclin and nitric oxide. Because of the prolonged clinical course the possibility of alveolar capillary dysplasia was raised. The parents refused ECMO. Despite all efforts she progressively deteriorated and died at 22 days of age. Macro- and microscopic examination of the lung at autopsy were diagnostic of alveolar capillary dysplasia. A detailed review of 39 cases published in the literature with comments regarding incidence, etiology, pathophysiology, clinical picture, diagnosis and treatment is presented.     

A clinical and cytological study of vitamin A deficiency in Kiribati.

Vitamin A status was assessed clinically and by conjunctival impression cytology in a selected group under seven years of age in the Republic of Kiribati. A total of 230 children were studied. Of 185 with readable histology, 14.6% were clinically and histologically abnormal; 19.5% were clinically abnormal but histologically normal; and 15.7% were clinically normal and histologically abnormal. The remainder (50.3%) were clinically and histologically normal. There was a significant correlation between clinical disease and histology (P < 0.001). The prevalence of the disease in males was 45.8% and 26.3% in females. The risk of deficiency appeared to increase with advancing age. There was no significant difference between the two villages studied.     

Efficacy of antimicrobial treatment in non-dysenteric persistent diarrhoea in a community setting

Objectives : To determine the efficacy of antimicrobial treatment in non-dysenteric persistent diarrhoea in a community setting. Methods : In this double-blind field trial, 156 children aged 4 36 months with persistent diarrhoea not associated with Giardia lamblia infestation seeking treatment in a community outpatient clinic, were randomized to receive a combination of nalidixic acid and metronidazole, metronidazole alone, or placebo for 7 days. Results : In comparison with placebo, metronidazole treatment did not result in a significant reduction in the mean post-enrolment diarrhoeal duration and stool frequency, increase in the proportion of patients recovered by days 3, 5 and 7 of treatment, and increase in weight gain at days 7 and 14. Comparing the combination of nalidixic acid and metronidazole with metronidazole alone, 17.5% more children treated with the combination recovered by day 3 of treatment ( p = 0.08) and the mean stool frequency ascertained on day 7 for the previous 24 h was 26.8% less in them ( p = 0.05). The weight gains at days 7 and 14 were similar in the two groups. Conclusions : These findings indicate that metronidazole offers no therapeutic benefit in persistent diarrhoea not associated with Giardia lamblia and nalidixic acid has only a modest clinical benefit, which is not substantial enough to warrant its routine use.     

Mental retardation,unusual face,and intrauterine growth retardation: A new recessive syndrome?

We describe two mentally retarded sisters with intrauterine growth retardation and subsequent dwarfism and an unusal and characteristic face together with two other unrelated patients with similar manifestations. These four patients may represent a new autosomal recessive syndrome.     

Platelet Functions in Cardiopulmonary Bypass Surgery

Background

Haemorrhage after Cardio Pulmonary Bypass (CPB) Surgery is a well recognised complication that leads to significant morbidity and mortality. The incidence varies between 5-25% depending upon the clinical situation. Several factors are implicated as causative but none have been precisely proved.

Methods

Our study was an attempt to evaluate the haemostatic defect with particular reference to platelet function abnormalities during cardio pulmonary bypass surgery, in order to reduce the morbidity and mortality associated with post CPB haemorrhage. Flow cytometric evaluation of different platelet glycoproteins like GPIb/IX, GPIIb/IIIa and GMP-140 was done.

Results

The marker expression showed deregulation during surgery which returned to base after bypass was terminated. In contrast, the cases with bleeding showed significant variation. P-Selectin (GMP 140) expression decreased progressively till 3^rd post-operative day showing lack of activation of platelets in cases of severe bleeding.

Conclusion

Longer duration of CPB initiates plasmin generation through heparin, which raises the PAI-1-tPA complex and thereby down regulating the functions of platelets. This suggests a link between duration of CPB, bleeding, platelet dysfunction and fibrinolysis. Hence serial estimations of the levels of GMP-140 and tPA can predict severe bleeding.Key Words: CardioPulmonary Bypass, Platelet dysfunction, flowcytometry, platelet glycoproteins, haemorrhage     

Abnormal copper metabolism in cultured fibroblasts from patients with Wilson's disease

Skin fibroblasts derived from patients with Wilson's disease have, under certain conditions, elevated concentrations of copper. However, the levels of intracellular copper varied from one experiment to another and the reason for this inconsistency has not yet been determined.⁶⁴Cu retention after 24 hours and its release in ‘chase’ experiments was not abnormal, thus distinguishing these fibroblasts from Menkes' syndrome fibroblasts. The data provides evidence that the mutant gene responsible for Wilson's disease is expressed in fibroblasts under certain conditions. This study was supported by a grant from the Australian National Health and Medical Research Council.