Dr. Eugene Kalman Genga

BACKGROUND: Diabetes Mellitus is a common and demanding health related problem that has a wide effect on every day’s life of the patients. It can have a profound effect on quality of life in terms of social and psychological well-being as well as physical ill-health. It is one of the most psychologically demanding of the chronic diseases; with psychosocial factors pertinent to nearly every aspect of the disease and its treatment.
OBJECTIVE: To Assess the perceived Health-related quality of life of diabetic patients not on insulin therapy using the WHOQoL-Bref (World Health Organization Quality of Life – Brief).
STUDY DESIGN: This was a cross-sectional study.
STUDY SITE: The study was conducted on patients attending the Diabetic clinic at Kenyatta National Hospital.
RESULTS: Study recruited 139 patients with type2 diabetes not on insulin therapy. The study population was predominantly female (61%) , majority were 40-60yrs, having had diabetes for less than 5yrs, 75% having more than one complication. Most (75%) of the study participants were poorly controlled with HbA1C mean score of 8.04% .Majority of the study participants( 84%) achieved a good score on the HRQoL scale using the WHOQoL-Bref tool. The determinants of HRQoL in our study were: age of study participants, duration of diabetes, presence of complications and income related factors. Age of the study subjects had significant association only in the social domain of HRQoL with a p-value of 0.037. Level of income had a significant association with overall HRQoL score (p-value of 0.029), psychological domain (p value of 0.023) and in the social domain (p-value of 0.029). Health care financing was significantly associated with psychological domain (p-value 0.006) and environmental domain (p-value 0f 0.04) and overall score (p-value 0.011). There was an association between employment status and HRQoL. Having a job improved the scores in physical domain (p-value of 0.013) and social domain (p value of 0.020). Duration with diabetes had significant association with physical domain where the p value was 0.007. The HRQoL of the study subjects was associated significantly with the number of complications. Indeed the association of complications with the HRQoL involved physical domain (p-value of <0.0001) and psychological domain (p-value of 0.041) which directly impacted on the overall total score (p value of 0.041).
CONCLUSION: The results of this study show that diabetes affected HRQoL of our study participants. There is a need for interventions programs to improve glycemic control and inclusion of HRQoL assessment as part of patients on follow up. Age and duration of disease are not modifiable but complications can be reduced by better health care initiaves. Income-related factors can be modifiable through poverty alleviation and pooled health care financing.

INTRODUCTION: A pheochromocytoma is a rare, catecholamine-secreting tumour that may precipitate life-threatening hypertension. The tumour is malignant in 10% of cases but may be cured completely by surgical removal. Because of excessive catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or cardiac arrhythmias. If the diagnosis of a pheochromocytoma is overlooked, the consequences can be disastrous, even fatal; the diagnosis can be established by measuring catecholamines and metanephrines in plasma (blood) or through a 24-hour urine collection. The most common clinical sign of pheochromocytoma is sustained or paroxysmal hypertension, and the most common symptoms are headache, excessive truncal sweating, and palpitation. In some cases, the clinical symptoms are not clear. Roughly 70% of adrenal incidentalomas are non-functional. A small group of 5–7% of the functional ones (30%) may exist as pheochromocytoma. Ten percent of pheochromocytoma cases are diagnosed incidentally during computed tomography (CT) or magnetic resonance imaging (MRI) screenings for other reasons.
CASE PRESENTATION: 21 year old female patient who was referred to Kenyatta National Hospital with diagnosis of gangrene in a young lady newly diagnosed with diabetes in a known hypertensive for three years. The gangrene was of a duration of two weeks. She was diagnosed with diabetes during work up for the cause of the gangrene. Investigations revealed a 24 hour-urine norepinephrine levels of 5085nmol, Normetanephrines excretion of 45213nmol over 24hoursShe tested negative for HIV, Hepatitis B and C surface antigens, VDRL, ANA, C-Anca and P-Anca. Abdominal ultrasound showed normal sized kidneys with a suprarenal mass (80 *63) mm with ectopic right kidney in pelvis, ECG a sinus tachycardia, Echo cardiogram reported as normal with an LVEF of 54%. Arteriogram had a vaso- occlusive disorder at the digital femoral artery and CT abdomen showing a supra renal mass (8x6x5) cm border of head and body of pancreas displacing the right kidney inferiorly. The patient underwent an amputation of the limb and adrenelectomy. Following the surgery the blood pressure and the glucose has normalised and currently is of medication
CONCLUSION: Diagnosis of hypertension in a young patient should involve looking for secondary causes of the disease. A young hypertensive patient presenting with a triad of headaches, palpitations, and sweating was then investigated for pheochromocytoma. Pheochromocytoma can present and occur as an emergency ranging from pheochromocytoma-related multisystem failure, cardiovascular emergencies, pulmonary emergencies, abdominal emergencies, neurologic emergencies, renal emergencies, and metabolic emergencies. This presentations are associated with a high morbidity and mortality if pheochromocytoma is unsuspected. This presentation was unique because it was none of the expected emergencies but a rapidly evolving asymmetrical gangrene of the right foot.
Key words: pheochromocytoma, gangrene
Abbreviations: ANA- Antinuclear antibody
C-Anca- Cytoplasmic antineutrophil cytoplasmic antibodies
P-Anca- Perinuclear Anti-Neutrophil Cytoplasmic Antibodies
HIV- human immunodeficiency virus

BACKGROUND: Scleroderma is a chronic multisystem autoimmune disease of unknown aetiology. Scleroderma is characterized by widespread obliterative vasculopathy of small arteries and is associated with varying degrees of tissue fibrosis and multiple organ involvement. Pulmonary disease is an important component of SSc. It is estimated that 80% of patients with SSc have some evidence of pulmonary disease.Systemic sclerosis has the poorest prognosis amongst rheumatology diseases with the highest case-specific mortality of any of the autoimmune rheumatic diseases as well as causing major morbidity
OBJECTIVE: This article will review pathogenesis, diagnosis and management of pulmonary disease in scleroderma.
DATA SOURCE: Literature review of relevant published literature from both Africa and the rest of the world
DATA SYNTHESIS:The pathogenesis of lung disease in scleroderma involves a variety of pathways, including immunological/inflammatory activation and vascular injury. The primary cytokines responsible for the disease are unknown but it is postulated that it involves a complex interplay between inflammatory, B lymphocyte antibody production, oxidative stress and fibrotic pathways. This leads to the activation of lung fibroblasts by inflammatory and fibrotic mediators. Lung fibroblasts play a central role in the deposition of excess intracellular matrix. This inflammatory response leads to fibrosis and occurs in the setting of vascular derangements.The most common symptoms are dry cough and dyspnea on exertion. The high morbidity and mortality seen in SSc is generally attributed to the two major pulmonary manifestations of the disease: interstitial pulmonary fibrosis, or interstitial lung disease, and pulmonary arterial hypertension.Exertional dyspnea and dry cough are the most common presenting symptoms in patients with SSc who develop pulmonary involvement Algorithm of diagnostic procedures in these patients does not differ considerably from the procedures of any other interstitial lung disease. At the current time, cyclophosphamide remains the best studied therapeutic agent although alternatives are actively being evaluated. The pathogenesis of pulmonary disease in scleroderma is still an enigma and is being actively researched. This will advance our understanding of the disease and ability to care for these patients.
CONCLUSION: Pulmonary complications are common in SSc and are the leading causes of death. Careful evaluation by the clinician is warranted to detect the presence of an ILD and to select patients appropriately for consideration of therapy. It is a major clinical challenge largely due to the enigma of the disease pathology as well as limited therapeutic options available. This is compounded by the perceived lack of evidence for clinical effectiveness of those treatments that are currently in use. Clinical trials are underway and offer hope for novel approaches to this mysterious and often devastating manifestation of scleroderma.

BACKGROUND: Diffuse infiltrative lymphocytosis syndrome (DILS) is characterised by a persistent CD8+ lymphocytosis and lymphocytic infiltration of various organs. The exact prevalence isn’t known but some studies have reported between 0.85 – 3%, and appears to be more common in African population. Patients with DILS tend to have higher CD4cell counts and survive longer than those patients without DILS. Most patients present with bilateral parotid gland enlargement and features of the Sicca syndrome. Common sites of extra glandular involvement are the lungs being the most common site, followed by peripheral neuropathy and liver. With the high incidence of HIV in our population it is likely that DILS is under diagnosed probably due to our ignorance of this disease. Awareness of its various presentations may bring to light undiscovered patients with DILS.
OBJECTIVE: To review pathogenesis, diagnostic approach and current trends in the management of Diffuse interstitial lymphocytic syndrome
DATA SOURCE: Literature review of relevant published literature from both Africa and the rest of the world.
DATA SYNTHESIS:Pathologically, under light microscopy, DILS resembles the focal sialadenitis seen with Sjogren’s syndrome, although it tends to be less destructive of the glandular architecture than in Sjogren’s syndrome. Most of the inflammatory infiltrate is composed of CD8+ lymphocytes unlike Sjogren’s which are CD4+. Lymphoepithelial cysts are frequently observed in the parotid glands of patients with DILS. The variation in CD8 count in the course of HIV disease is less understood. The variation in CD8 lymphocytes is implicated in the pathogenesis of a number of clinical manifestations in HIV diseases including diffuse infiltrative lymphocytic syndrome (DILS) and HIV associated CD8+ lymphocytosis syndrome.Parotid gland enlargement in a patient with HIV infection should prompt clinicians to suspect DILS. In addition, clinicians should be aware that the pulmonary process associated with DILS may mimic clinically and radiographically the pneumonic process caused by Pneumocystis carinii. Other manifestations of DILS to consider include a severe form of peripheral neuropathy; lymphocytic infiltration of the liver, evident as hepatitis; myositis; and lymphocytic interstitial nephritis.Management of DILS is determined by the severity of glandular and extra glandularfeatures.Data on therapeutic trials are lacking although there are isolated reports of good response to antiretroviral and steroid therapy.

CONCLUSION: DILS, a subset of HIV disease manifestation, may present as parotid gland swellings. In general, an HIV patient presenting with DILS has a better prognosis than a patient with HIV alone.With the high incidence of HIV in our population it is likely that DILS is under diagnosed probably due to our ignorance of this disease. Awareness of its various presentations may bring to light undiscovered patients with DILS. Clinicians should watch for the possible transformation into B-cell lymphoma. There is still paucity of data about this disease from pathophysiology to treatment to studies correlating the plasma viral load with CD8 lymphocyte count in patients with HIV disease.