An Atypical Presentation of Rheumatoid Arthritis as an Asymmetrical Arthropathy.

We report a rare entity of distinctly asymmetrical rheumatoid arthritis (RA) in a 71-year-old Chinese lady with a history of cervical radiculopathy secondary to trauma sustained during childhood. The joints on the side of the paresis were spared from severe clinical and radiological manifestations of RA. We review the plausible mechanisms that could explain the link between neurological impairment and rheumatoid joint involvement.

Unravelling the mystery in a case of persistent ACTH-independent Cushing's syndrome.

INTRODUCTION: We present a rare variety of adrenocorticotrophic hormone (ACTH)-independent Cushing's syndrome known as primary pigmented nodular adrenocortical disease (PPNAD). CLINICAL PICTURE: The patient initially underwent unilateral adrenalectomy for what was thought to be a left adrenal adenoma. OUTCOME: Partial resolution of symptoms and demonstrable persistent hypercortisolism after surgery prompted further evaluation with findings leading to the diagnosis of Carney complex. A review of the adrenal histology was consistent with PPNAD. CONCLUSION: This entity of PPNAD, which has rarely been reported in Asians, forms part of the Carney complex. The diagnosis may not be simple and straightforward, as illustrated in this patient.

Clinical Ward Rounds-Challenges and Opportunities.

Hospitalised patients' needs are complex and the ward environment is demanding of time and resources that must be optimised. Clinical ward rounds in hospitalised patients are fundamental to patient care. Ward rounds in recent years have undergone changes which have contributed to reduced professionalism and opportunities to learn as well as increased distrust of patients of the care they receive. Calls for a revival of the traditional ward rounds have been sounded which we must contextualise in modern settings. This commentary calls for a clearer definition of the purpose of ward rounds, outlines the roles and responsibilities of those involved in rounds, defines a 4-step process in the conduct of a ward round, and seeks support from hospitals' management in the facilitation and implementation of these.

Prime time to resuscitate clinical medicine and kill diagnostic greed?

Modern healthcare faces the challenges of rising costs, increasing expectations of patients and changing disease patterns. Physicians practise medicine in an era of easy availability and access to a plethora of modern and sometimes expensive diagnostic aids. The powerful utility of clinical skills cannot be underestimated nor lost. The physician has a powerful platform to encourage the rational use of tests, prevent wasteful overutilisation and ensure that tests do not cause more harm than benefit in physical, emotional or financial terms. Diagnostic skills should not be substituted by diagnostic greed. It is possible to do more for the patient rather than to the patient.

A germline mutation of the thyrotropin receptor gene associated with thyrotoxicosis and mitral valve prolapse in a Chinese family.

Activating mutations of the TSH receptor (TSH-R) have been reported to result in toxic adenomas, multinodular goiters, sporadic neonatal hyperthyroidism, and familial autosomal dominant nonautoimmune hyperthyroidism. To date, all descriptions of such mutations, whether somatic or genomic, have been confined to the Caucasian population. We describe a Chinese family in whom a germline proline to serine substitution in position 639 resulted in familial thyrotoxicosis. This constitutively activating mutation has been previously described in a hyperfunctioning thyroid nodule. The three children in this family developed thyrotoxicosis during childhood; their father was diagnosed as thyrotoxic at the age of 38 yr. Two of the children and the father had mitral valve prolapse (MVP) associated with mitral regurgitation. There was a close temporal relationship between the onset of thyrotoxicosis and the diagnosis of mitral valvular disease in these patients. An increased prevalence of MVP has been reported in Graves' disease and chronic lymphocytic thyroiditis, but the pathophysiological mechanisms linking MVP and autoimmune thyroid disease are still not understood. This is the first report of an association between activating TSH-R mutations and MVP. We postulate that TSH-R activation may increase the clinical expression of MVP in genetically predisposed individuals.

The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis.

A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome.

Long-term treatment outcome in acromegaly.

A number of groups have developed guidelines to indicate whether an individual with acromegaly has been cured by treatment. However, studies to date do not provide a robust definition of biochemical remission of the disorder based on correlation with long-term outcome. Available data suggest that those with a random serum growth hormone (GH) level of <2.5 microg/l, or a glucose-suppressed GH level of <1 microg/l following treatment have mortality figures indistinguishable from the general population. However, the confidence limits for these mortality estimates are quite wide. It remains possible that growth hormone levels lower than 1 microg/l for random samples, or even lower when using ultrasensitive GH assays, may indicate superior outcome, but this remains to be confirmed. There are limited data relating serum insulin-like growth factor-I (IGF-I) levels to outcome, although normalisation of serum IGF-I clearly improves outcome compared with continued elevation of measurements after treatment. Current evidence suggests that a post-treatment random serum GH <2.5 microg/l and a normal serum IGF-I value defines biochemical cure. Available data suggest that achieving similar growth hormone levels after treatment also reduces the prevalence of chronic complications of the disorder, which is subsequently reflected in improved mortality.

Acromegaly: factors affecting the long term outcome after surgical treatment.

Survival and clinical status in 105 acromegalic patients followed for up to 24 years after surgery is assessed against serum growth hormone (GH) levels and other factors. Primary treatment was at first (90)Yttrium implants or craniotomy, later transsphenoidal operation. GH levels were similar for each method, and the results were combined. Relief of presenting symptoms and tumour clearance related to early post-operative GH levels. Although the level at the last follow-up influenced clinical status at 10 years (P = 0.01), the major contributor was the age at diagnosis (P = 0.0002). Ten year survival was more likely when the final GH level was less than 5mug/l and at this level no tumour recurred. With GH less than 2 mug/l survival rate was higher and clinical status better. With GH between 2 and 5 mug/l radiotherapy may be indicated; levels of Insulin-like Growth Factor I help in this decision. A plan of management is suggested.

Diabetic ketoacidosis--a study of 33 episodes.

Twenty-six patients presenting with 33 episodes of Diabetic Ketoacidosis (DKA) and managed on a protocol oriented system were analysed. Diabetes mellitus was newly diagnosed at presentation in 18% of the 33 episodes. The presenting symptoms were polyuria and polydipsia (58%), nausea and vomiting (52%), change in sensorium (24%), hyperventilation (24%), and abdominal pain (18%). The main clinical findings at admission were dehydration (97%), acidotic respiration (67%), coma and confusion (61%), a clinically detectable source of sepsis (49%), fever (33%) and hypotension (9%). Blood sugar levels at admission ranged between 351 mg/dl and 1200 mg/dl (mean = 633 mg/dl). The mean serum potassium at diagnosis was 5.1 mmol/l and the mean calculated serum osmolality was 320 mOsm/kg. The mean serum osmolality was higher in those with disturbed conscious level. Infections, particularly those of the urogenital tract, were the main precipitating cause for the DKA. Only 12 of the 19 patients with sepsis had fever. Eight of the episodes were attributed to patients' non-compliance with insulin. Four patients died during the 33 hospitalisations, giving a mortality rate of 10%. Death occurred despite glucose control and stabilisation of the ketoacidotic state and was due to uncontrolled septicaemia. The mean duration of hospitalisation was 11 days. The ketoacidosis state was reversed after a mean duration of 9.5 hours, with an average soluble insulin requirement per patient of 52.4 units.

Characteristics of geriatric admissions to two medical departments in Singapore.

A survey was conducted to study certain characteristics of geriatric patients admitted to two medical departments in Singapore. The aim was to create awareness as well as to provide a better understanding of these elderly as more and more doctors would have to attend to them. Our results show that about 27% of admissions were geriatric patients. The typical geriatric patient in this study was about 75 years old (mean age 74.6 years) and was likely to present with breathlessness due to heart disease or pneumonia. The overall duration of stay in hospital was 10 days and only about 6% of the patients had "discharge" problems needing assistance from the Medical Social Worker. About one in five patients died in hospital, usually from pneumonia or ischaemic heart disease.

Simulated bleeding--a forgotten disease in a land of plenty.

The human body is unable to synthesise Vitamin C and a diet deficient in Vitamin C leads to scurvy. Scurvy may mimic other medical conditions, like bleeding diasthesis or deep vein thrombosis, leading to delay in diagnosis and treatment, thus prolonging sufferings of patients. Often, scurvy could have been diagnosed if it is thought of and features of scurvy carefully looked for. Scurvy is easily treated with high dose of oral vitamin C. Recurrences may occur. Education of care providers cannot be overemphasised. We report three local cases of scurvy to highlight the existence of the disease in our modern society.

Carbimazole-induced agranulocytosis--a report of 2 recent cases.

Carbimazole is a useful antithyroid drug with a rare potentially fatal complication of agranulocytosis. We report 2 cases presenting with this problem. One was treated supportively with barrier nursing and broad spectrum antibiotics, and the other needed use of a haemopoietic growth factor, granulocyte colony stimulating factor (G-CSF). As it is indeed possible for thyrotoxic patients who developed agranulocytosis with carbimazole to have the same complication with propylthiouracil, once agranulocytosis had resolved, both patients were treated with radioiodine to maintain euthyroidism. Carbimazole-induced agranulocytosis usually spontaneously resolves within 1 to 2 weeks of stopping the drug. The use of haemopoietic growth factors to stimulate the proliferation and differentiation of progenitor cells, accelerates neutrophil recovery, as in our first case discussed. We recognise that agranulocytosis from carbimazole is a rare, life-threatening complication. Instead of awaiting spontaneous recovery, the use of haemopoietic growth factors certainly seems a justifiable option, with a promise of a reduction in morbidity and mortality.

Diagnosis of tuberculous peritonitis.

The diagnosis of tuberculous peritonitis may be difficult and elusive. The patient may present with non-specific symptoms of fever, general ill-health or vague abdominal pains. There may be no pulmonary symptoms and the chest X-ray may be normal. The CT scan of the abdomen is sometimes helpful in suggesting the diagnosis. We have found that laparoscopic examination of the abdominal contents and the peritoneum is an effective way to obtain a conclusive diagnosis.

High prevalence of psychiatric morbidity in a medical intensive care unit.

This study seeks to determine the prevalence of psychiatric morbidity within a medical intensive care unit, examine its correlation with the various physiological parameters and delineate any clinical predictors for psychiatric morbidity. Seventy-seven patients who gave informed consent were administered the General Health Questionnaire (GHQ), Acute Physiological And Chronic Health Evaluation II (APACHE II) and thyroid function tests were performed. A high prevalence of psychiatric morbidity was found (36.4%). However, no statistically significant association was found between psychiatric morbidity and gender, age, APACHE II scores and thyroid function indices. Nevertheless, it is hoped that the index of suspicion for psychiatric morbidity can be raised in order to optimise the clinical management of patients within this setting.

Malaria: an audit of 56 cases admitted to a hospital.

A 2-year prospective audit on the profile and outcome of malaria cases admitted to a general hospital was performed. Fifty-six cases were seen from January 1991 to December 1992, 52 of which were due to monoinfections with Plasmodium vivax. The main presenting complaints were fever, chills, sweats, myalgia, dry cough and headache. A significant percentage had anaemia (64.3%), thrombocytopaenia (57.1%), hyponatraemia (42.9%), and liver dysfunction (44.7%). Diagnosis rests on the demonstration of parasites in stained peripheral blood smears. None of the patients developed major complications. A high index of suspicion of malaria must be maintained in the medical evaluation of all patients and in particular, of returning travellers.

Hypertension in the young adult--come feel the pulse.

Hypertension occurring in teenagers and young adults is uncommon. Though the most common form is still essential hypertension, secondary causes are more commonly found here than in older adults. Renal, cardiovascular and endocrine diseases constitute most of these causes. Coarctation of the aorta is the most common cardiovascular cause of hypertension, and its importance lies in the fact that it is correctable, and that its persistence often leads to dangerous complications and early death. The cardinal sign of differential pulse and blood pressures between the upper and lower limbs can be detected clinically. Hence, the importance of a detailed physical examination in all young hypertensives, including palpation of all the pulses, cannot be overemphasized. We present 2 hypertensive young men who were found to have isolated coarctation of the aorta. The lesion in the first patient was located postductally just distal to the left subclavian artery. This area has been found to be the most common site of coarctation. The second patient had an unusual mid-thoracic coarctation. The clinical and radiological features as well as complications are highlighted. In young hypertensive patients, a high index of suspicion may enable the physician to make a timely diagnosis and hence avert the potentially disastrous complications that may arise in undetected cases.

Examining the therapeutic options in hyperthyroidism--a personal perspective.

After confirmation of the diagnosis of hyperthyroidism, an aetiological clue should be ascertained. Any therapy should be directed toward the primary aetiology and should weigh the risk-benefit ratio carefully. Transient thyrotoxic states like thyroiditis may be treated with beta blockers, provided no contraindications exist. Beta blockers may also be used to treat the sympathetic symptoms and the response is usually rapid. The general trend is to use antithyroid drugs as first-line therapy, although relapses are the commonest problems with antithyroid drugs. Thionamides are the most common drugs used to treat the thyrotoxic state. Thionamides used in high doses have immunomodulatory effects and these effects can be utilised to reduce the risk of relapses in Graves' thyrotoxicosis. Iodides are usually reserved in the preoperative preparations of patients. Surgery, unlike medical therapy or radioiodine, can be offered as a therapeutic alternative only once. It may be offered where medical therapy has failed, where patients develop antithyroid drug allergies, and where the goitre causes obstructive symptoms or is of cosmetic concern. Prior to surgical therapy, adequate preparation should be obligatory. It is preferable to achieve clinical and biochemical euthyroidism prior to surgery. The use of radioiodine in therapeutics has become more acceptable and established. Although safe, it has the major drawback of cumulative hypothyroidism. There is still debate on the use of radioiodine in children. In 1989, the incidence of peritonitis was one episode every 24 patient-month which was significantly lower than the general population on CAPD of one episode every 17 patient-month.(ABSTRACT TRUNCATED AT 250 WORDS)