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IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition which can affect various organs including the pituitary gland. The true annual incidence of this condition remains widely unknown. In addition, it is unclear whether IgG4 antibodies are causative or the end result of a trigger. With no specific biomarkers available, the diagnosis of IgG4-related hypophysitis remains a challenge. Additionally, there is a wide differential diagnosis. We report a case of biopsy-proven IgG4-related hypophysitis in a young man with type 2 diabetes mellitus. Learning points: IgG4-related hypophysitis is part of a spectrum of IgG4-related diseases. Clinical manifestations result from anterior pituitary hormone deficiencies with or without diabetes insipidus, which can be temporary or permanent. A combination of clinical, radiological, serological and histological evidence with careful interpretation is required to make the diagnosis. Tissue biopsy remains the gold standard investigation. Disease monitoring and long-term management of this condition is a challenge as relapses occur frequently.
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Hypogonadotrophic hypogonadism is due to impaired or reduced gonadotrophin secretion from the pituitary gland. In the absence of any anatomical or functional lesions of the pituitary or hypothalamic gland, the hypogonadotrophic hypogonadism is referred to as idiopathic hypogonadotrophic hypogonadism (IHH). We present a case of a young lady born to consanguineous parents who was found to have IHH due to a rare gene mutation. Learning points: The genetic basis of a majority of cases of IHH remains unknown.IHH can have different clinical endocrine manifestations.Patients can present late to the healthcare service because of unawareness and stigmata associated with the clinical features.Family members of affected individuals can be affected to varying degrees.
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UNLABELLED: Gonadotrophin therapy with human chorionic gonadotrophin and recombinant FSH is indicated for use in men with reduced spermatogenesis due to hypogonadotrophic hypogonadism (HH). Patients require regular monitoring for side effects and desired response to treatment. We present a man with HH, azoospermia and a history of previous anabolic steroid usage who had undergone gonadotrophin therapy, had subsequently achieved conception and has now fathered a child. LEARNING POINTS: In total, 15% of couples do not achieve pregnancy within 1 year and seek medical treatment for infertility: male factors contribute to 50% of these.The evaluation of male infertility should include a full history and examination, an endocrine profile and a quality-controlled semen analysis.HH with defective spermatogenesis is an important cause of male infertility in a small percentage of cases.Gonadotrophin therapy requires regular monitoring for side effects and desired response to treatment.Any sustained rise in prostate specific antigen levels should prompt urological assessment for possible prostate biopsy.A multidisciplinary approach is required for gonadotrophin therapy, especially if assisted fertilisation techniques are required once, spermatogenesis is achieved.
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UNLABELLED: Testicular adrenal rest tumours (TARTs) are benign ACTH-dependent tumours that occur in males with congenital adrenal hyperplasia (CAH) and if left untreated can destroy testicular tissue. Corticosteroid suppressive treatment could result in the regression of these testicular tumours. We present a patient with bilateral large TARTs as a consequence of poor compliance to treatment and follow-up for his CAH, who consequently had to have bilateral orchidectomies and prosthesis replacement. LEARNING POINTS: TARTs are frequently seen in males with CAH, and can be misdiagnosed as primary testicular cancer.Patient compliance to treatment and follow-up are necessary to reduce the risk of testicular damage as a result of TARTs in patients with CAH.Boys with CAH should have periodic ultrasonographic screening from before adolescent age for early detection of TARTs.Regular monitoring of renin, 17-hydroxyprogesterone and androgens levels is required to assess corticosteroid suppressive treatment.Patients with CAH should be offered psychological support and information concerning CAH support groups.
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AIMS: Peripheral neuropathy affects more than a third of diabetic patients, of whom a significant minority will have disabling symptoms. The aim of this study was to assess the efficacy of pulsed-dose electrical stimulation (through stocking electrodes) in the treatment of painful diabetic neuropathy. METHODS: Thirty patients with painful diabetic neuropathy were consecutively randomised to wear silver-plated nylon-dacron stocking electrodes for 8 h a night for 6 weeks (pulsed electric current of 50 micro amps delivered by a microcomputer). The control, identical stockings received an insignificant current (5 micro amps). Pre-treatment, weekly and end-of-treatment pain and sleep disturbance scores were recorded. RESULTS: Fourteen patients completed the study (the 16 non-completers withdrew during the first phase). Mean (+/- sd) age: 57.5 +/- 10.5 years; HbA(1c): 8.3 +/- 1.4%; median (IQR) duration of diabetes: 14.5 (7.6-19.3) years; duration of neuropathy: 4 (3-7) years. Active treatment and control produced similar reductions in pain scores [median (IQR): 40.1 (4.7-97.7)% vs. 49.2 (0.2-91.1)%, P = 0.70] and sleep disturbance scores [median (IQR): 31.1 (-4.6 to 85.4)% vs. 42.6 (-16.2 to 91.1)%, P = 0.70]. Non-completers (seven on active treatment, nine on control) withdrew for similar reasons (inconvenience, exacerbation of symptoms, dermatitis). CONCLUSIONS: There is no evidence from these results that this treatment is more effective than control in the treatment of painful diabetic neuropathy.
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Neuropatías Diabéticas/rehabilitación , Terapia por Estimulación Eléctrica/métodos , Adulto , Estudios Cruzados , Método Doble Ciego , Electrodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Proyectos Piloto , Calidad de VidaRESUMEN
AIMS: Peripheral neuropathy affects 30% of Type 1 diabetic patients. Unfortunately, 10-20% of affected patients have disabling symptoms. The aim of this study was to examine the relationship between blood glucose excursions and pain in patients with symptomatic diabetic neuropathy. METHODS: Twenty Type 1 diabetic patients with peripheral neuropathy (10 painful and 10 painless) wore a continuous glucose monitoring system for 3 days. Symptomatic patients kept a daily pain score diary. The mean amplitude of glycaemic excursions (MAGE) and the M-values (measure of glucose deviations from an arbitrarily selected point) were calculated. RESULTS: Groups were matched for (mean +/- sd) age: 52.0 +/- 11.1 years; duration of diabetes: 24.8 +/- 10.7 years; HbA1c: 9.7 +/- 2.3%; duration of neuropathy: 5.6 +/- 2.6 years; and CGMS performance. The painful group had a greater mean glucose (12.1 +/- 2.9 mmol/l vs. 9.3 +/- 1.9 mmol/l, P = 0.02), a greater M-value (68.4 vs. 31.1, P = 0.02) and more glycaemic excursions (13 vs. 10, P < 0.01), compared with the painless group. However, there was no difference in the MAGE between both groups, and no correlation between the number of glycaemic excursions and the number of painful episodes in the painful group. CONCLUSIONS: Patients with painful neuropathy have greater glucose flux and possibly poorer diabetes control, compared with patients with painless neuropathy.
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Glucemia/análisis , Neuropatías Diabéticas/sangre , Dolor/etiología , Enfermedades del Sistema Nervioso Periférico/sangre , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dolor/sangre , Proyectos PilotoRESUMEN
OBJECTIVE: The aim of this study was to quantify the distribution of peripheral arterial disease in the diabetic and nondiabetic population attending for angiography and to compare severity and outcome between both groups of patients. RESEARCH DESIGN AND METHODS: Randomly selected lower-extremity angiograms were examined according to the Bollinger system. Patient demographics and medical history were recorded and case notes were examined to determine which patients later underwent a revascularization procedure or amputation and which patients had died. RESULTS: A total of 136 arteriograms obtained between 1992 and 1996 were analyzed. The age (mean +/- SD) of the patients was 64.7 +/- 10.8 years. Diabetic patients (43%) and nondiabetic patients were of similar age (63.9 +/- 10.4 vs. 65.3 +/- 11.1 years, P = 0.43), with a similar history of smoking (81.0 vs. 76.9%, P = 0.26), ischemic heart disease (41.4 vs. 37.2%, P = 0.54), and hypercholesterolemia (24.4 vs. 30.8%, P = 0.48). However, there were a greater proportion of hypertensive patients in the diabetic group (63.8 vs. 39.7%, P = 0.006). Diabetic patients had greater severity of arterial disease in the profunda femoris and all arterial segments below the knee (P = 0.02). A greater number of amputations occurred in the diabetic group: diabetic patients were five times more likely to have an amputation (41.4 vs. 11.5%, odds ratio [OR] 5.4, P < 0.0001). Mortality was higher in the diabetic group (51.7 vs. 25.6%, OR 3.1, P = 0.002), and diabetic patients who died were younger at presentation than nondiabetic patients (64.7 +/- 11.4 vs. 71.1 +/- 8.7 years, P = 0.04). CONCLUSIONS: In patients with peripheral arterial disease, diabetic patients have worse arterial disease and a poorer outcome than nondiabetic patients.
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Arteriopatías Oclusivas/fisiopatología , Angiopatías Diabéticas/fisiopatología , Enfermedades Vasculares Periféricas/fisiopatología , Anciano , Amputación Quirúrgica/estadística & datos numéricos , Angiografía , Arteriopatías Oclusivas/epidemiología , Arteriopatías Oclusivas/mortalidad , Arteriopatías Oclusivas/cirugía , Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Angiopatías Diabéticas/epidemiología , Angiopatías Diabéticas/mortalidad , Angiopatías Diabéticas/cirugía , Inglaterra , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Enfermedades Vasculares Periféricas/epidemiología , Enfermedades Vasculares Periféricas/mortalidad , Enfermedades Vasculares Periféricas/cirugía , Sistema de Registros , Fumar , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
AIMS: The outcome of foot ulcers is affected by wound depth, infection, ischaemia and glycaemic control. The aim of this study was to determine the effects of ulcer size, site, patient's age, sex and type and duration of diabetes on the outcome of diabetic foot ulcers. METHODS: Diabetic patients with new foot ulcers presenting during a 12-month period had demographics and ulcer characteristics recorded at presentation. Ulcers were followed-up until an outcome was noted. RESULTS: One hundred and ninety-four patients (77% males) with a mean (+/- SD) age and duration of diabetes of 56.6 +/- 12.6 and 15.4 +/- 9.9 years, respectively, were included in the study. The majority of ulcers were neuropathic (67.0%) and present on the forefoot (77.8%) with a median (interquartile range) area of 1.5 (0.6-4.0) cm2. Amputations were performed for 15% of ulcers; 65% healed; 16% remained unhealed and 4% of patients died. The median (95% confidence interval) time to healing was 10 (8.8-11.6) weeks. Ulcer area at presentation was greater in the amputation group compared to healed ulcers (3.9 vs. 1.2 cm2, P < 0.0001). Ulcer area correlated with healing time (rs = 0.27, P < 0.0001) and predicted healing (P = 0.04). Patient's age, sex, duration/type of diabetes, and ulcer site had no effect on outcome. CONCLUSIONS: Ulcer area, a measure of ulcer size, predicts the outcome of foot ulcers. Its inclusion into a diabetic wound classification system will make that system a better predictor of outcome.
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Pie Diabético/patología , Pie Diabético/terapia , Factores de Edad , Amputación Quirúrgica/estadística & datos numéricos , Intervalos de Confianza , Angiopatías Diabéticas/fisiopatología , Pie Diabético/cirugía , Neuropatías Diabéticas/fisiopatología , Inglaterra , Etnicidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Modelos de Riesgos Proporcionales , Análisis de Regresión , Factores Sexuales , Texas , Factores de Tiempo , Resultado del Tratamiento , Cicatrización de HeridasRESUMEN
OBJECTIVE: In this study the following two ulcer classification systems were applied to new foot ulcers to compare them as predictors of outcome: the Wagner (grade) and the University of Texas (LT) (grade and stage) wound classification systems. RESEARCH DESIGN AND METHODS: Ulcer size, appearance, clinical evidence of infection, ischemia, and neuropathy at presentation were recorded, and patients were followed up until healing or for 6 months. RESULTS: Of 194 patients with new foot ulcers, 67.0% were neuropathic, 26.3% were neuroischemic, 1.0% were ischemic, and 5.7% had no identified underlying factors. Median (interquartile range [IQR]) ulcer size at presentation was 1.5 cm2 (0.6-4.0). Lower-limb amputations were performed for 15% of ulcers, whereas 65% healed [median (IQR) healing time 5 (3-10) weeks] and 16% were not healed at study termination; 4% of patients died. Wagner grade (P < 0.0001), and UT grade (P < 0.0001) and stage (P < 0.001) showed positive trends with increased number of amputations. For UT stage, the risk of amputation increased with infection both alone (odds ratio [OR] = 11.1, P < 0.0001) and in combination with ischemia (OR = 14.7, P < 0.0001), but not significantly with ischemia alone (OR = 4.6, P = 0.09). Healing times were not significantly different for each grade of the Wagner (P = 0.1) or the UT system (P = 0.07), but there was a significant stepwise increase in healing time with each stage of the UT system (P < 0.05), and stage predicted healing (P < 0.05). CONCLUSIONS: Increasing stage, regardless of grade, is associated with increased risk of amputation and prolonged ulcer healing time. The UT system's inclusion of stage makes it a better predictor of outcome.