Cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is a disorder that affects mostly those bones which derived from endochrondal and intramembranous ossification, such as the cranium and the clavicles. CCD is present at a frequency of one in one million individuals. Over 500 cases have been reported. A 13 years old boy reported in the Paediatric Endocrinology outpatient department, Bangladesh Institute of Research and Rehabilitation on Diabetes, Endocrine and Metabolic Disorders (BIRDEM) on 6th April 2006 with complaints of not growing according to his age, a wide gap over the head since birth and delayed shedding of primary teeth and also eruption of secondary teeth. The boy had elongated facies with midfacial hypoplasia, depressed nasal bridge with hypertelorism. His anterior fontanelle was open about 3/1.5 cm. He had short clavicle and his shoulders could be easily apposed. Radiological findings showed anterior fontanellle and sutures were open and widened, all decidual teeth were intact and all secondary teeth were impacted beneath the primary teeth and hypoplastic clavicle. He was diagnosed as a case of Cleidocranial Dysplasia on the basis of history, clinical examination and investigation.

Vaginal bleeding with multicystic ovaries and a pituitary mass in a child with severe hypothyroidism.

A seven year and ten months old girl presented with cyclic vaginal bleeding and a huge abdominopelvic mass. She had clinical features of hypothyroidism. The investigation results were consistent with the diagnosis of primary hypothyroidism with precocious puberty. She also had bilaterally enlarged cystic ovaries on CT scan of abdomen and CT scan of brain showed pituitary macroadenoma. After starting treatment with thyroxine, patient became euthyroid and her general condition improved. Treatment with thyroxine alone halted the cyclic vaginal bleeding, led to rapid resolution of the ovarian cysts and regression of the pituitary mass.

Role of cardiac troponin I in staging and prognosis of congestive heart failure subjects.

Congestive heart failure is the one of the major cardiovascular disorder that is increasing in incidence and cause of death globally. Mortality rate has increased 40%-50% in advanced cardiac failure and 15%-25% in mild to moderate cardiac failure within one year of diagnosis. There is no established biochemical marker for the diagnosis, prognosis and staging of heart failure. Cardiac Troponin I may be a novel useful tool in identifying patients with Heart failure who are at increased risk for progressive ventricular dysfunction and death. Thirty six congestive heart failure cases and thirty six healthy controls were included in this study and serum cardiac troponin I and Ejection fraction were measured. All the study subjects were grouped according to the NYHA class they belong. Cardiac troponin I was significantly higher in CHF cases than the controls. Troponin I also significantly differed among groups. EF of cases was significantly lower than the controls and also differed among groups. A significant negative correlation between cardiac troponin I and progressive decline of ejection fraction was evident in this study. Cardiac troponin I increased progressively with progression of heart failure. Thus, Cardiac troponin I could be used to stratify patients undergoing heart failure in to high and low risk groups for future cardiac events. Cardiac troponin I could also be used as a very important marker for the prognosis of the patients with congestive heart failure.

Maternal and newborn-care practices during pregnancy, childbirth, and the postnatal period: a comparison in three rural districts in Bangladesh.

The aim of this study was to examine the prevalence of maternal and newborn-care practices among women reporting a birth in the previous year in three districts in different divisions of Bangladesh. In 2003, 6,785 women, who had delivered a newborn infant in the previous year, across three districts in Bangladesh, were interviewed. Overall, less than half of the women received any antenatal care, and 11% received a minimum of four check-ups. Only 18% took iron tablets for at least four months during pregnancy. Over 90% of the 6,785 deliveries took place at home, and only 11% were attended either by a doctor or by a nurse. The mothers reported three key hygienic practices in 54% of deliveries: attendants washing their hands with soap and boiling cord-tie and blade for cutting the cord. Forty-four percent of the 6,785 infants were bathed immediately after delivery, and 42% were given colostrum as their first food. The results suggest that maternal and newborn-care remains a cause of concern in rural Bangladesh. Short-term policies to promote healthy behaviour in the home are needed, in addition to the long-term goal of skilled birth attendance.

Spot morning urine protein creatinine ratio and 24 hour urinary total protein excretion rate.

Proteinuria helps to establish the diagnosis of most renal diseases and also to predict the outcome of such diseases. Proteinuria is biochemically represented by measuring the protein concentration in timed collection of 24 hour urine. But, 24-hour timed urine collection is time consuming, cumbersome and often unreliable due to collection errors and also results in undue delay on diagnostic process. An alternate approach avoiding arduous and inaccurate timed urine collection can be the measurement of protein creatinine ratio in spot morning urine. This study was aimed to evaluate whether the spot morning urine protein creatinine ratio can be a reliable alternative to 24-hour urinary total protein (UTP) estimation. The study was carried out in the department of Biochemistry, Bangabandhu Sheikh Mujib Medical University, Dhaka on 50 (fifty) non-diabetic Chronic Renal Disease (CRD) patients with an age ranging from 18 -70 years. The study subjects were grouped into mild, moderate and severe CRD on the basis of GFR. Urinary protein and creatinine concentrations were measured in spot morning urine samples and their ratios were calculated. Urinary protein measured in 24-hour timed collected urine samples gave the 24-hour UTP excretion rate. In our study, spot morning urine protein creatinine ratio significantly correlated with 24-hour UTP excretion rate in all CRD patients. Severe CRD patients gave significant positive correlation (p<0.05), whereas mild and moderate CRD patients gave very highly significant positive correlation (p<0.001). Therefore, it may be suggested that protein creatinine ratio in spot morning urine can be accepted as a reliable and alternative to 24-hour UTP excretion rate in non-diabetic chronic renal disease patients. This simple and inexpensive procedure will thus simplify the way of establishing the severity of renal disease along with its prognosis.

Glycemic status in hyperthyroid subjects.

This was an observational case-control study carried out in the Department of Biochemistry, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka jointly with the 'Thyroid and Endocrine Clinic' of the same institution during the period of January 2002 to December 2002. Sixty-five (65) newly diagnosed hyperthyroid patients between 20-60 years of age were studied, where forty-five (45) were Graves' disease and twenty (20) were TMNG (Toxic multinodular goiter) patients. Thyrotoxicosis was diagnosed by history, clinical examination and biochemical investigations- FT4, TSH, and Radioactive iodine uptake (RAIU) test. Thirty (30) age and sex matched healthy subjects were taken as control. The mean age was 33.02+/-9.24 years in Graves' disease and 37.55+/-9.49 years in TMNG. Female predominance observed in both the diseases. Glucose intolerance was found in 72.3% of thyrotoxic patients, which is much higher than European population. Our study showed Diabetes mellitus (DM) in 11% of Graves' disease patients. The incidence of DM in Graves' disease was slightly higher in our population. Incidence of DM in TMNG in our study was much lower (5%) than that of Graves' disease (11%) but the incidence of IGT (Impaired glucose tolerance) in TMNG was more (85%) in relation to Graves' disease (54%). Percentage of RAIU was more marked in Graves' disease than TMNG. There is a significant positive correlation (p<0.05) between plasma glucose and FT4 in Graves' disease. Glucose intolerance is frequently found in Thyrotoxic patients.

Use of Wigglesworth classification for the assessment of perinatal mortality in Bangladesh--a preliminary study.

The Wigglesworth pathophysiological classification was used to analyse perinatal deaths occurring in 5 health centres in Bangladesh. The aims were to assess the feasibility of this classification, to determine the causes of perinatal deaths and thereby to identify the areas in need of intervention. A total of 8058 births were recorded at 5 centres during the period of 11 months from mid-January to mid-December 2001. There were 1069 deaths in the perinatal period. Stillbirths were slightly more frequent (53.5%) than early neonatal deaths (46.5%). Among the stillbirths, fresh stillbirths predominated over normally formed macerated ones at all centers except BIRDEM, where the majority (52.5%) was macerated. The majority (71.6%) of perinatal deaths were in the groups comprising asphyxial conditions (46.8%), conditions associated with immaturity (13.3%), and normally formed macerated stillbirths (NFMSB, 11.5%). In the group, 'other specific conditions' which was responsible for 9.3% of perinatal deaths, all but one case was attributed to sepsis. When the cases were subdivided by birth groups, asphyxia predominated in all but the <1000g group, in whom immaturity was responsible. Conditions associated with immaturity were second highest in number. The majority of the perinatal deaths (83.4%) was in babies less than 2500g. The study has shown that the Wigglesworth classification can be used in different types of health facilities in Bangladesh by doctors, nurses and midwives. The areas which need intervention are antepartum care, obstetric and newborn care practices, and environmental factors responsible for the high prevalence of prematurity and low birth weight.

Vegetarian diet in the treatment of fibromyalgia.

Brain tryptophan is low in fibromyalgia. Intake of protein rich in large neutral amino acids is reported to lower brain tryptophan. This study was undertaken to assess whether any reduction of such proteins by exclusion of animal protein from the diet reduced pain and morbidity in fibromyalgia patients. It was an open, randomized controlled trial. 37 subjects with fibromyalgia were enrolled in the vegetarian diet and 41 in the amitriptyline groups. The outcome was assessed with the help of frequencies of fatigue, insomnia & non-restorative sleep, pain score on a 10-point VAS and tender point count. Fatigue, insomnia and non-restorative sleep were present in 41, 26 and 32 subjects before and in 3, 0 and 0 subjects respectively at six weeks of treatment in the amitriptyline group. The pain score and tender point count were 6.2 +/- 1.9 & 16.1 +/- 2.3 before and 2.3 +/- 1.3 & 6.4 +/- 3.0 after treatment. All these differences were significant (P < 0.001). In the vegetarian diet group, fatigue, insomnia and non-restorative sleep were present in 36, 24 and 27 subjects before and in 34, 29 and 29 subjects at six weeks of treatment. The pain score and tender point count were 5.7 +/- 1.8 and 15.7 +/- 2.4 before and 5.0 +/- 1.8 & 14.7 +/- 3.6 after treatment. All these differences were insignificant except that in the pain score. The decrease in the pain score, though significant, was much smaller than that in the amitriptyline group. So, it may be concluded that vegetarian diet is a poor option in the treatment of fibromyalgia.

TSH assay using dried blood samples: a screening method for congenital hypothyroidism.

Capillary blood samples from three hundred healthy, full-term newborn within 48 and 120 hours of delivery, were collected on filter paper for analysis of thyroid-stimulating hormone (TSH). Blood was collected by pricking the heel of the newborn and spotted and dried on filter paper cards. The samples were analysed by immunoradiometric assay (IRMA) to determine the TSH level. Some fifteen percent of a total 1928 deliveries at two hospitals during a period of one year were included in the study. Male:female ratio was 1.1:1. There was no maternal history of thyroid disease. None of the babies had any clinical feature of hypothyroidism. On analysis, TSH level ranged from 0.6-19.3 microU/ml with a mean (SD) of 7.19 (4.21) microU/ml. TSH assay from dried filter paper blood spot is technically possible and is a cost-effective and reliable method for a screening programme. This is the first time in Bangladesh that this method has been successfully carried out.

Oral health of 6, 9, 12, and 15 year old children of Trinidad and Tobago 1989

The purpose of this study was to conduct an oral health survey of the school age children in Trinidad and Tobago using a population sample of children in the age groups of 6, 9, 12, and 15 years old. This study also served to provide continuing education to three(3)Dental surgeons,ten(10)Nurses, ten(10) Dental surgery assistants in the following areas:(1)Oral Health Survey Design, Planning and Administration.(2)Use of dental indices to evaluate oral health. The overall objective of the survey was to ascertain the present level of oral diseases in the 6-15 year old population, therefore a systematic approach of collection and analysis of data, using specially designed forms, this data will(1)Provide baseline data(indices) of the oral health of specific age groups in Trinidad and Tobago.(2)Serve as a base for initiation of preventive and prophylactic dental health programs.(3)Serve as a baseline for the evaluation of any preventive measure introduced by the Ministry of Health.(4)Serve as a base for planning oral health in the year 2000.(5)Serve as a base for further research