The Ehlers-Danlos syndrome

A case study of a nineteen year old female illustrates the main features of the Ehlers-Danlos syndrome. This is a rare syndrome, the characteristic features of which are hyper elasticity and fragility of the skin, hyperextensibility of joints and fragility of blood vessels. Musculo-skeletal, ocular and internal manifestations are present in some cases. The main feature of this case was severe bleeding from minor trauma. The Kyphoscoliosis was first noticed in childhood but had become worse in her teens and she had evidence of respiratory insufficiency secondary to the marked chest deformity. No treatment is known. Surgical procedures may present a problem as the tissues are friable and difficult to suture. Meticulous techniques and pressure dressing are desirable.peer-reviewe

The value of X-ray chest screening

Paper read at the 1973 Annual Meeting of the Association of Physicians and Surgeons of Malta. Two cases of serious thoracic disease were discovered on routine Chest X-Ray screening in 150 schoolboys: one student being found to be suffering from ganglioneuroma, the other from coarctation of the aorta. Both were symptom free and both required thoracic surgery. The purpose of this paper is to emphasize and illustrate a well-known fact; the value of screening in the community. The example used is Chest X-Ray Screening.peer-reviewe

Intersex : case report and discussion

This case illustrates one type of intersex, the term denoting the condition in patients whose sex characteristics are incompletely differentiated. The case is reported of a child with congenital malformation of the external genitalia who was brought up as a girl but has now, at the age of 25, been found to be a male. The factors determining sex and the various clinical types of intersex are discussed. This individual who has been taught to be female and has been reared as female all through infancy, childhood and youth is, in fact, a male. He is suffering from bifid scrotum, with undescended testicles and hypospadias. One testicle is palpable in the left part of the scrotum. In managing this patient the psychological trauma he was suffering from had first to be dealt with. The patient felt male and wanted to lead a male life. The second step in the management of the patient is to correct as far as possible the congenital abnormalities of the male genitalia.peer-reviewe

An integrated approach to the management of health care in Malta

A successful health care programme depends directly on the team work of a group of people who care about and who care for patients in the management of their sickness with the aim of allaying the symptoms in all' cases and of a complete cure whenever possible. This team is made up of general practitioners, hospital medical officers, consultants, pharmacists, dentists, physiotherapists, occupational therapists, speech therapists, nurses and social workers. According to the front cover of the Pharmaceutical Journal of September 18th, 1982, in the 16th Century the physician and pharmacist worked closely together, but today we rarely see them depicted together. This might be so in other countries, but I am pleased to say, it is not the case in Malta. The pharmacist is an important member of the team looking after the sick. His place as a prominent member of this team has been enhanced by a working programme planned between the Department of Medicine and the Department of Pharmacy of our University under the auspices of the Ministry of Health. This working programme will be discussed later in this paper; at this stage I am going to dwell on the situation in Malta vis-a-vis the traditional relationship between the pharmacist and the physician.peer-reviewe

Albinism : a case report

Albinism means the absence of cutaneous pigments. This is a rare hereditary disorder of the skin found in all races. The basic defect is an inherent deficiency of the enzyme tyrosinase usually found in melanocytes. Albinism can be total or partial. The occurrence of total albinism in Malta, in spite of the smallness of the country, is very rare. A case of total albinism in a Maltese child is here reported. At birth, features of total albinism were present. Her skin is extremely white which can be seen by contrast with the normally pigmented skin of the nurse holding her. Her hair is fine, silky and whitish. Her eyebrows and eyelashes are also white. She has gross photophobia and nystagmus. In sunlight or bright artificial light, her eyes are kept tightly closed. Because of this photophobia, and the harmful effect of sunlight on the unprotected unpigmented skin, the child is never taken out in the sunlight and is kept indoors most of the time.peer-reviewe

Presentation and management of diabetic ketoacidosis in adults in Malta

Aim: The aim of this audit was to assess adherence to local guideline in the management of Diabetic Ketoacidosis (DKA). Method: Patients admitted with DKA between April 2013 and March 2015 were identified and data was retrospectively collected from patients’ confidential files and Isoft®. Data collected included initial parameters recorded and biochemical investigations taken (initial and subsequent assessment of pH, HCO3-, blood glucose, potassium levels and urinary ketones), insulin regime started and intravenous fluid administered. Results: During the established time period 40 cases of DKA were identified in 18 patients. Median age was 33 years with a female preponderance of 60%. Six patients had newly diagnosed diabetes mellitus while 8 patients had more than one admission of DKA. All cases had capillary blood glucose monitoring (BGM) and/or venous random blood (plasma) glucose (RBG) checked and pH and HCO3- recorded on admission. 0.9% sodium chloride was the intravenous fluid started in all cases (as recommended by the guideline) and a median of 6.75L was prescribed during the first 24 hours. The median time spent on intravenous insulin infusion was 42.7 hours while the median time to pH >7.30, HCO3- >15mmol/L and negligible urinary ketones were 6.88, 12.83 and 34.5 hours respectively. Subcutaneous insulin was started at a median time of 48.21 hours from initiation of DKA protocol. Conclusion: This audit showed good adherence to local guideline. The great discrepancy between the time to pH >7.3 and the time to negligible urinary ketones highlights the need to introduce tools to measure systemic ketone production in the management of DKA with an update in the current local clinical practice guideline.peer-reviewe

Women's views of continuity of information provided during and after pregnancy: A qualitative interview study

Straightforward transfer of care from pregnancy to the postpartum period is associated with health benefits and is desired by women worldwide. Underpinning this transfer of care is the sharing of information between healthcare professionals and the provision of consistent information to women. In this qualitative study, two aspects of continuity of information were examined; first the information passed on from midwife to health visitor regarding a woman and her baby before the health visitor meets the woman postnatally and second, the consistency of information received by women from these two healthcare professionals (the main healthcare providers during and after pregnancy in England). To be eligible for the study, women had to have had a baby in England within 12 months prior to the interview. Participants also needed to be able to read and speak English and be over 18 years old. Recruitment of participants was via word of mouth and social media. Twenty-nine mothers were interviewed of whom 19 were first time mothers. The interviews took place in the summer and autumn of 2016 and were transcribed verbatim and analysed using Framework Analysis. Two overarching themes were identified: not feeling listened to and information inconsistencies. Women reported little experience of midwives and health visitors sharing information about their care, forcing women to repeat information. This made women feel not listened to and participants recommended that healthcare professionals share information; prioritising information about labour, mental health, and chronic conditions. Women had mixed experiences regarding receiving information from midwives and health visitors, with examples of both consistent and inconsistent information received. To avoid inconsistent information, joint appointments were recommended. Findings from this study clearly suggest that better communication pathways need to be developed and effectively implemented for midwives and health visitors to improve the care that they provide to women

Rediscovery of Ornithagalum divergens Boreau (Hyacinthaceae) in Malta (Supplement)

Due to a fortuitous omission, the last few references in the article "Rediscovery of Ornithogalum divergens Boreau (Hyacinthaceae) in Malta" (CMN, Volume 4 Part 3, pp 205-206) were not included. The same references are hereby included as a supplement:peer-reviewe

Rediscovery of Ornithogalum divergens Boreau (Hyacinthaceae) in Malta (Central Mediterranean)

Ornithogalum diver gens had not been reported from the Maltese islands since 1927 and was presumed to be extinct here. Recently the species was rediscovered at the Buskett woodland and a new population was recorded at Girgenti.peer-reviewe

A Directly-Written Monolithic Waveguide-Laser Incorporating a DFB Waveguide-Bragg Grating

We report the fabrication and performance of the first C-band directly-written monolithic waveguide-laser. The waveguide-laser device was created in an Erbium and Ytterbium doped phosphate glass host and consisted of an optical waveguide that included a distributed feedback Bragg grating structure. The femtosecond laser direct-write technique was used to create both the waveguide and the waveguide-Bragg grating simultaneously and in a single processing step. The waveguide-laser was optically pumped at approximately 980 nm and lased at 1537nm with a bandwidth of less than 4 pm.Comment: 6 pages, 13 references, 4 figure