Over a 10-year period, 15 glucose-6-phosphate dehydrogenase (G6PD)-deficient male newborns were admitted to Al-Jahra Hospital with acute haemolysis a few days after applying henna dye over the body, which is a unique Bedouin tribal practice to celebrate the arrival of the first-born boy. Laboratory investigations revealed significant anaemia, reticulocytosis and indirect hyperbilirubinaemia among the index newborns as compared with controls (p < 0.001). The mean (SD) haemoglobin concentration in index patients was 113.4 (13.4) g/l vs 171.2 (11.2) g/l in controls, reticulocytic count 13.8% (4.2%) vs 1.4% (0.74%), and indirect bilirubin 382.8 (58.7) mumol/l vs 63.7 (61.4) mumol/l. G6PD-deficient newborns with haemolysis linked to henna application had delayed age at presentation and a higher reticulocytic count and hyperbilirubinaemia compared with non-henna-induced haemolysis (p < 0.05). Percutaneous henna absorption is well recognized and clinical findings support the harmful effect of henna on G6PD-deficient red blood cells. A local health education programme has been established to prevent the use of henna dye in infancy.
Thoracoplasty was invented for removing cavities between thoracic wall and remnant lung or mediastinum. It was initially used in cases of tuberculosis or unspecific infections, while currently it is used mainly for space problems after lobectomy/pneumonectomy.This article presents an overview of the historical and current techniques of this surgical procedure.Nowadays, thoracoplasty is rarely performed due to the low incidence of diseases for which this method is necessary. Therefore, this method has even been discredited. Furthermore, certain technical aspects of the thoracoplasty are not very well known because of the infrequent application of this procedure.Unfortunately, a look into the literature of thoracoplasty is not always usefull due to the biased views of advocates of different techniques such as Schede's thoracoplasty, Heller's Jalousie-Plastik, Alexander's extramusculoperiosteal thoracoplasty, Bjork's osteoplastic thoracoplasty, etc.Not to forget, there has always been a lack of research on the relevance and on the several techniques of thoracoplasty.The point is precise indication and correct execution of thoracoplasty as a final therapeutic option, which allows a safe and definitive solution of the space problem even in complex cases, without creating serious functional and cosmetic impairment for the patient.The main types of thoracoplasty are described in this article. Although the core principle of this operation remains unchanged, modern techniques are often cosmetically more considerable and less destructive, compared with techniques that were used in the past.
A Bedouin family from Kuwait, with a ‘mosaic’ inbred population, is reported. The family includes 3 citrullinemic sibs and a discordant identical twin pair. Both interfamilial and intrafamilial phenotype variabilities are briefly discussed. The clinical manifestation of the disease in this family is described and the molecular and enzymatic bases of citrullinemia are briefly discussed.
Objective and Importance: Bardet-Biedl syndrome (BBS) is a heterogeneous disorder; clinical dissimilarities do exist among patients. Cases of BBS have been reported with hypertension, diabetes mellitus and renal disease, while others were reported with hepatic disease. Here, we are reporting 3 newly diagnosed unrelated Bedouin children, evaluated for the presence of an endocrine disorder. Clinical Presentation: Our patients were 2 males and 1 female. These patients are obese with a voracious appetite, postaxial polydactyly and mentally retarded. Pigmentary retinopathy was found in the older children and hypogenitalism is apparent in the older male child who is also hypertensive. The clinical, endocrinological and imaging evaluation data are compared with previously published cases. Also, phenotypic variability and genetic heterogeneity of BBS pleiotropic gene are briefly discussed. Conclusion: The results of hormonal studies in our cases showed no endocrinological dysfunction, except for the presence of hypogonadism. Although no specific treatment is available, an early evaluation of BBS cases can be the first step in initiating symptomatic management and preventive measures for the patient and the family.
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