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INTRODUCTION: Malignant insulinoma is a rare neuroendocrine tumor responsible for excessive insulin secretion and life-threatening hypoglycemia episodes. Computed tomography (CT) of the abdomen can identify a pancreatic tumor corresponding to insulinoma. Loco-regional metastases define the metastatic cases. The first-line therapeutic approach is surgery, while other medical treatments like diazoxide and everolimus play also a role. These treatments have shown efficacy in regulating blood glucose and, to some extent, controlling tumor progression. CASE PRESENTATION: We present the case of a 48-year-old female who was admitted for severe hypoglycemia episodes. She presented neuroglycopenic symptoms without any other clinical features. High levels of C-peptide and insulin during severe hypoglycemia confirmed the presence of endogenous hyperinsulinism. The CT scan of the abdomen confirmed the existence of an insulinoma along with several hepatic metastases. Surgery was proposed as a first-line approach. However, due to the persistent occurrence of severe hypoglycemia episodes, other treatment options were necessary such as diazoxide and everolimus. Diazoxide caused a significant improvement in the patient's blood glucose levels. Nonetheless, glycemic control was unsustainable, obligating the switch to everolimus, which showed better control of blood glucose levels with challenging management due to the appearance of grade 3 stomatitis as a side effect. The patient died 1 year after the diagnosis due to tumor progression. CONCLUSION: Balancing the benefits of enhanced glycemic control with the difficulties posed by side effect management of everolimus underscores the need to carefully consider both efficacy and potential adverse events.
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Everolimo , Hipoglicemia , Insulinoma , Neoplasias Pancreáticas , Humanos , Feminino , Everolimo/uso terapêutico , Everolimo/efeitos adversos , Insulinoma/secundário , Insulinoma/tratamento farmacológico , Pessoa de Meia-Idade , Neoplasias Pancreáticas/tratamento farmacológico , Hipoglicemia/induzido quimicamente , Antineoplásicos/uso terapêutico , Antineoplásicos/efeitos adversos , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/tratamento farmacológico , Glicemia/efeitos dos fármacos , Glicemia/metabolismo , Evolução Fatal , Diazóxido/uso terapêutico , Resultado do TratamentoRESUMO
Acromegaly is a rare systemic syndrome induced by the overproduction of growth hormone (GH) and insulin-like growth factor type one (IGF1). It is responsible for changes in the skeletal and soft tissue systems and it almost always occurs because of a pituitary adenoma. Amongst the skin complications related to acromegaly, cutis verticis gyrate (CVG) is occasionally found. It is a skin condition characterized by excessive growth of the skin of the scalp, resulting in furrows and folds. Only a few cases of this uncommon association have been reported in the literature. The present clinical case illustrates typical CVG associated with acromegaly. Imaging revealed a pituitary macroadenoma lesion and hormonal evaluation revealed elevated IGF1 and hypopituitarism. The patient underwent a transsphenoidal resection of the pituitary adenoma and the histopathological examination confirmed the diagnosis. The diagnosis of CVG is clinical, so radiologic assessments are generally not necessary. The management of acromegaly associated with CVG depends on controlling the serum levels of GH and IGF1. In some cases, specific injections or surgery can be used to minimize CVG.
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Acromegalia , Adenoma , Fator de Crescimento Insulin-Like I , Neoplasias Hipofisárias , Dermatoses do Couro Cabeludo , Humanos , Acromegalia/complicações , Acromegalia/etiologia , Dermatoses do Couro Cabeludo/complicações , Dermatoses do Couro Cabeludo/patologia , Dermatoses do Couro Cabeludo/etiologia , Adenoma/complicações , Adenoma/cirurgia , Adenoma/patologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/cirurgia , Fator de Crescimento Insulin-Like I/metabolismo , Fator de Crescimento Insulin-Like I/análise , Couro Cabeludo/patologia , Masculino , Pessoa de Meia-Idade , FemininoRESUMO
Ellis-Van Creveld (EvC) syndrome is a rare autosomal recessive chondroectodermal dysplasia including chondrodysplasia, postaxial polydactyly, ectodermal dysplasia, and congenital heart disease in 60% of patients. Additional findings may be observed affecting the pulmonary, renal, gastrointestinal, hematologic, and central nervous systems. We report a case of an 11-year-old Moroccan boy with EVC syndrome and Dandy-Walker malformation. To our knowledge, this association has been previously reported in 3 patients in the literature.
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Síndrome de Dandy-Walker , Síndrome de Ellis-Van Creveld , Polidactilia , Sistema Nervoso Central , Criança , Humanos , Rim , MasculinoRESUMO
Achondroplasia is a human bone genetic disorder of the growth plate and is the most common form of inherited disproportionate short stature. It is inherited as an autosomal dominant disease with essentially complete penetrance. Of these most have the same point mutation in the gene for fibroblast growth factor receptor 3 (FGFR3) which is a negative regulator of bone growth. The clinical and radiological features of achondroplasia can easily be identified; they include disproportionate short stature with rhizomelic shortening, macrocephaly with frontal bossing, midface hypoplasia, lumbar hyperlordosis, and a trident hand configuration. The majority of achondroplasts have a normal intelligence, but many social and medical complications may compromise a full and productive life. Some of them have serious health consequences related to hydrocephalus, craniocervical junction compression, or upper-airway obstruction. In this article, we discuss a number of treatments from the surgical limb lengthening approach and the Recombinant Growth Hormone (rhGH) treatment, to future treatments, which include the Natriuretic Peptide C-type (CNP). The discussion is a comparative study of the complications and drawbacks of various experiments using numerous strategies.
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Acondroplasia , Acondroplasia/complicações , Acondroplasia/genética , Acondroplasia/terapia , Obstrução das Vias Respiratórias/etiologia , Estatura , Medula Cervical , Extremidades/cirurgia , Feminino , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Hidrocefalia/etiologia , Masculino , Mutação Puntual/genética , Radiografia , Receptor Tipo 3 de Fator de Crescimento de Fibroblastos/genética , Compressão da Medula Espinal/etiologiaRESUMO
BACKGROUND: Diabetic nephropathy (DN) is associated with a high risk of progression to End Stage Renal Disease (ESRD). While obesity has been identified as a factor in the decline of the glomerular filtration rate (GFR) in chronic kidney disease, its role in the progression of DN remains controversial. The objective of this work is to determine GFR decline in relation to BMI in type 2 diabetic (T2D) patients presenting a DN. METHODS: A prospective 5-year study conducted in the Eastern region of Morocco. Three BMI groups were distinguished: normal weight, overweight and obese and within each group progressors (eGFR > 5 ml/min/1.73 m(2)/year) and non progressors (eGFR > 5 ml/min/1.73 m(2)/year). RESULTS: Data on 292 patients were compiled. The progressors represented 25.8%, 23.1% and 32.3% of the normal weight, overweight and obese patient groups respectively (p = 0.29). ESRD was observed in 9.1%, 6.9% and 8.3% (p = 0.21) in normal weight, overweight and obese patients respectively. In multivariate analysis, low-baseline eGFR was identified as important predictor of progression of DN in each BMI group and in the entire cohort independently of BMI. Vascular co-morbidity events occurred in 9.1%, 16.9% and 19.8% (p = 0.04) in normal weight, overweight and obese patients respectively. CONCLUSION: Our results show that the decline of eGFR in the DN of T2D is not directly influenced by BMI and that the major risk factors contributing to this decline remain low-baseline eGFR and increased baseline albuminuria.
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Índice de Massa Corporal , Países em Desenvolvimento , Diabetes Mellitus Tipo 2/complicações , Nefropatias Diabéticas/fisiopatologia , Taxa de Filtração Glomerular , Idoso , Diabetes Mellitus Tipo 2/fisiopatologia , Nefropatias Diabéticas/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Obesidade/complicações , Obesidade/fisiopatologia , Estudos ProspectivosRESUMO
INTRODUCTION AND IMPORTANCE: Lymphocytic hypophysitis is an underestimated disease and the pathogenesis is still poorly elucidated. Post-coronavirus lymphocytic hypophysitis is a new emerging entity. CASE PRESENTATION: A 16-year-old previously healthy girl presented with pituitary tumor syndrome. She suffered from frontal headaches, polyuria-polydipsic syndrome, and impaired visual acuity. She was diagnosed with COVID-19 infection three weeks before. Contrast-enhanced magnetic resonance imaging (MRI) revealed pituitary enlargement with intense homogenous enhancement postgadolinium on T1 weighted images. The diagnosis of lymphocytic hypophysitis was made after ruling out other differential diagnosis. She was started on methylprednisolone. Improvement of clinical symptoms was seen on day 5 with a significant decrease in headache intensity. CLINICAL DISCUSSION: The article summarizes data from cases reported in the literature and our case to highlight coronavirus as a new trigger of lymphocytic hypophysitis. Despite the rarity of this complication, patients with a suspicion of hypophysitis after a recent COVID-19 infection should be carefully evaluated. CONCLUSION: COVID-19 infection can cause lymphocytic hypophysitis. However, it seems premature to conclude on the causal link between COVID-19 and endocrine diseases. Further studies on larger samples are needed to comprehend the pathogenesis of autoimmune endocrinopathies after COVID-19 infection.
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INTRODUCTION AND IMPORTANCE: Tuberculosis of the central nervous system is unusual and accounts for 1 % of all cases of tuberculosis in the world. The pituitary location is even scarcer. CASE PRESENTATION: A 14-year-old girl presented with polyuria-polydipsia syndrome and menstrual irregularity. MRI showed an intrasellar lesion of the pituitary gland. She underwent transsphenoidal surgery for histopathological diagnosis and removal of the lesion. Histological findings were consistent with a tuberculoma. She was put on anti-tuberculosis drugs and is being followed up. CLINICAL DISCUSSION: In endemic areas, pituitary tuberculosis should be considered in the differential diagnosis of pituitary tumors. The histological examination will guide the diagnosis. Sometimes, other complementary examinations such as the tuberculin skin test can be of great help when the histology is not conclusive. Medical treatment can be curative, however, surgery can be necessary for decompression. CONCLUSION: In addition to being the first case of histologically proven primary pituitary tuberculosis in a child reported in Morocco, the present case is unique in the way that the extensive radiological examinations did not reveal any evidence of other systemic or pulmonary tuberculosis.
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Background: Biliary complications are frequent in patients with acromegaly. These complications may be secondary either to acromegaly or to somatostatin analogs (SAs). We aimed in this paper to assess the prevalence of biliary complications in patients with acromegaly at diagnosis and after treatment with SAs. Methods: We conducted an analytical and descriptive retrospective study of 26 patients followed up for acromegaly over 7 years. Biliary complications were screened at diagnosis and follow-up by abdominal ultrasound, biliary magnetic resonance imaging (MRI), and endoscopic ultrasonography (EUS). Data were analyzed using SPSS 21. Results: The mean age of the patients was 49.6 ± 14 years, with a female predominance (53.8%). The evaluation of biliary complications showed vesicular biliary tract lithiasis and/or sludge in seven patients (29%), including two patients at the time of diagnosis of acromegaly and five patients after an average medical treatment duration of 3 years. Six female patients (24%) had dilation of the bile ducts without the presence of obstruction on biliary MRI and EUS and lithiasis/sludge of the common bile duct, tumor or external compression have been excluded. This condition was discovered incidentally at the diagnosis in five patients and during the follow-up in one patient. The preoperative insulin-like growth factor 1 (IGF-1) levels, disease duration, and female sex were significantly correlated with biliary tract dilation occurrence. Dyslipidemia, the preoperative IGF-1 level, and lanreotide treatment duration were significantly correlated with the occurrence of biliary lithiasis (P < 0.05). Conclusion: Biliary stones are a frequent biliary adverse effect in patients with acromegaly undergoing SAs treatment. However, primary bile duct dilation has never been reported in acromegaly to the best of our knowledge. This condition could be considered as a complication or a feature of the disease.
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INTRODUCTION AND IMPORTANCE: Ectopic pituitary neuroendocrine tumor (EPNET) is a very rare entity, seldom with apoplexy evolution. Only three cases of intracranial ectropic pituitary neuroendocrine tumor apoplexy were reported in the literature. CASE PRESENTATION: We report the case of a 45-year-old woman with a history of amenorrhea, and headaches. Neuroimaging showed a very aggressive giant mass within the clivus with the invasion of the sphenoidal sinus and encasement of internal carotid arteries with an empty sella. Endocrinology work-up revealed an exceedingly high level of prolactin surprisingly without galactorrhea. Immunohistochemical analysis after an endonasal biopsy confirmed the diagnosis of prolactinoma. One month after Cabergoline initiation, an apoplexy of the ectopic pituitary neuroendocrine tumor occurred. Conservational management with a decrease in cabergoline dose was performed. DISCUSSION: This article highlights data from various cases reported in the literature in addition to our case to confirm the extreme rarity of apoplexy as a complication of EPNET. CONCLUSION: Pituitary apoplexy in ectopic pituitary neuroendocrine tumor is extremely rare. Therefore, in case of unusual localization of pituitary neuroendocrine tumor, a thorough follow-up is necessary to detect complications and ensure early management.
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Dyggve-Melchiore-Clausen (DMC) syndrome is a are autosomal recessive spondyloepimetaphyseal dysplasia associated with mental retardation resulting from mutations in the Dymeclin (DYM) gene mapped in the 18q12-12.1 chromosomal region. We report a case of a consanguineous Moroccan boy with this disease confirmed by the presence of homozygous mutation at c.1878delA of DYM gene. Our patient additionally has a micropenis. We discuss the clinical severity, difficult management of this syndrome and its association with micropenis never described before in the literature.
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Nanismo/complicações , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Doenças dos Genitais Masculinos/complicações , Deficiência Intelectual/complicações , Osteocondrodisplasias/congênito , Pênis/anormalidades , Criança , Consanguinidade , Nanismo/diagnóstico por imagem , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico por imagem , Doenças dos Genitais Masculinos/diagnóstico por imagem , Mãos/diagnóstico por imagem , Humanos , Úmero/diagnóstico por imagem , Deficiência Intelectual/diagnóstico por imagem , Masculino , Marrocos , Osteocondrodisplasias/complicações , Osteocondrodisplasias/diagnóstico por imagem , Pelve/diagnóstico por imagem , Pênis/diagnóstico por imagem , Radiografia , Coluna Vertebral/diagnóstico por imagemRESUMO
Objectives: Our study aimed to compare the efficacy of transdermal dihydrotestosterone and testosterone enanthate in treating idiopathic micropenis. Patients and methods: It's a comparative randomized study of 49 patients with idiopathic micropenis who are followed up in the Endocrinology-Diabetology and Nutrition Department of Mohammed VI University Hospital Center of Oujda, Morocco. The study was conducted from December 2019 to April 2021. All patients received a clinical examination including measurement of penis size before and after hormonal treatment. The patients were divided into two random groups, each group received a different drug, the first arm was treated with transdermal dihydrotestosterone (27 patients) and the second arm was treated with testosterone enanthate (22 patients). The Trial registration number was researchregistry7745. Results: The majority of the patients were children. The mean age was 9.7 ± 4.4 years. In the first arm, the mean penile size increased from -2.42 SD to -0.7 SD with a gain of 2.37 cm on average. In the second arm, the mean size increased from -2.48 SD to -0.69 SD, with a gain of 1.82 cm on average. The increase in penile size in the first arm was significantly greater than in the second arm (P = .008). No side effects were detected in both arms. Discussion and conclusion: In the present study, we demonstrated the superiority of transdermal DHT compared to injectable exogenous testosterone in the treatment of idiopathic micropenis. According to the age subgroups, there was no significant difference between the 2 treatments in each age group.
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Introduction Diabetic retinopathy (DR) is a severe complication of diabetes. It remains a major cause of visual impairment and blindness, especially in young people. It is a silent affection that only becomes symptomatic at the onset of complications. Our study aimed to estimate the prevalence of retinopathy in patients with type 1 diabetes mellitus (T1DM) and evaluate the associated risk factors in our population. Materials and methods A descriptive and analytical study, with a cross-sectional study involving 359 patients with type 1 diabetes, was followed up in the Department of Endocrinology, Diabetology, and Nutrition of the University Hospital Center Mohammed VI Oujda, Morocco. Data were collected from medical records and analyzed by binary logistic regression using IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp. Results The average age of our patients was 24.2 ± 11.4 years. The mean duration of diabetes was 11.8±4.4 years. The average glycated hemoglobin (HbA1c) at admission was 10.1 ± 2.4%. DR was found in 30% of patients, including 28.6% with minimal non-proliferative diabetic retinopathy (NPDR), 19.1% with moderate NPDR, 19.1% with severe NPDR, and 33.3% with proliferative DR. Patients with diabetic retinopathy appear to have a longer duration of diabetes (13.05±9.05 vs. 10.6±8.07 years). The longer duration of diabetes, neuropathy, and nephropathy was significantly associated with diabetic retinopathy (p=0.02, p=0.002, and p=0.0001, respectively). Conclusion The frequency of diabetic retinopathy increases with age, poor glycemic control, and the duration of diabetes. Therefore, cooperation between diabetologists and ophthalmologists is essential for making an early diagnosis and providing early treatment.
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INTRODUCTION AND IMPORTANCE: Non-functional pituitary carcinoma is a rare neuroendocrine tumor. It is characterized by the presence of cerebrospinal or distant metastasis of an adenohypophysis tumor without any hypersecretion. Only a few cases of non-functional pituitary carcinomas have been reported in the literature. CASE PRESENTATION: In this paper, we report the case of a 48-year-old female patient who presented with spinal pain and a mass facing the second thoracic vertebrae. Spinal magnetic resonance imaging (MRI) revealed the presence of pituitary and bilateral adrenal incidentalomas. The patient was operated and the histopathological examination of the specimen revealed a non-functional pituitary carcinoma variety "Null Cell". DISCUSSION AND CONCLUSION: There are no clinical, biological, or radiological characteristics that reliably differentiate between non-functional pituitary adenoma and non-functional pituitary carcinoma. Management remains a challenge for clinicians and neurosurgeons. A combination of surgery, chemotherapy, and radiotherapy seems necessary to achieve tumor control.
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Background: Type 1 diabetes mellitus (T1DM) incidence is currently increasing worldwide, and different environmental players along with genetic predisposition, could be involved as powerful triggers of this disease onset. In this study, we aim to shed the light on the relationship between 25OHD deficiency and T1DM. Patients and methods: A case-control study was laid out to compare serum 25OHD level between T1DM patients and controls. A total of 147 T1DM patients aged under 19 years old were recruited from our Endocrinology-Diabetology and Nutrition department between October 2014 and December 2019. A total of 147 controls were randomly enlisted from clinical biochemistry laboratory of our center, and were carefully matched. The levels of 25OHD in the serum were determined in T1DM patients and nondiabetic controls. Results: Average serum 25OHD concentration was established in both groups; reaching 19,29 ±6,13 ng/ml in the control arm and 15,02 ± 6,48 ng/ml in the selected group with T1DM independently of the disease duration. However, the mean serum 25OHD concentration was not significantly different between the two T1DM subgroups according to diabetes duration below or above 5 years, and 25OHD concentration remained lower either in winter or summer months. A negative correlation was noticed between HbA1c and serum 25 OHD concentration in T1DM patients and was statistically significant (p<0,05). Conclusion: Key messages on the importance of vitamin D status, particularly in diabetic children and adolescents, should be spread widely in order to start a suitable vitamin supplementation, and establish guidelines regarding its timing at adequate recommended doses..
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Diabetes Mellitus Tipo 1 , Deficiência de Vitamina D , Humanos , Criança , Adolescente , Idoso , Adulto Jovem , Adulto , Vitamina D , Diabetes Mellitus Tipo 1/epidemiologia , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/epidemiologia , Estudos de Casos e Controles , VitaminasRESUMO
INTRODUCTION AND IMPORTANCE: Phaeochromocytomas and paragangliomas are rare neuroendocrine neoplasms that grow outside the adrenal gland and arise from the primitive neural crest cells. The retroperitoneal location is extremely rare with an incidence of 2-8 per million. CASE PRESENTATION: Here we report a case of an 80 years old man presenting with abdominal pain and vomiting associated with hypertensive peaks and weight loss. CT scan showed a retroperitoneal para-aortic tumor invading the inferior vena cava, with significantly elevated urinary catecholamine levels. Histopathological and immunohistochemistry examinations confirmed the diagnosis of paraganglioma. A medical preparation by alpha-blockers was performed. Complete resection of the tumor with the reconstruction of the vena cava was achieved without postoperative complications. After surgery, blood pressure and HbA1c were on the targets and the urinary catecholamine levels were normal. CLINICAL DISCUSSION: The diagnosis of paragangliomas is suspected by clinical symptoms in the case of functional paragangliomas and the confirmation is biological by the plasmatic or urinary catecholamines. Non-functional paragangliomas often represent a diagnostic challenge. In our case, the large size, the location of the tumor, and the invasion of adjacent structures represented a surgical challenge to perform a complete resection. CONCLUSION: In the elderly, this pathology is quite uncommon. Retroperitoneal paraganglioma is a rare location of this type of tumor. Endocrinologists, surgeons, and anesthesiologists should work together to ensure an appropriate diagnosis and treatment of paraganglioma. The gold standard treatment is the complete resection after a medical preparation.
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Introduction: Insulin pump therapy is recommended more and more to achieve and maintain optimal glycaemic control in patients with type 1 diabetes mellitus. The objective of our study was to evaluate the satisfaction of patients using insulin pump therapy and to determine its effectiveness in improving metabolic control in type 1 diabetic patients. Patients-Methods: This is a retrospective, descriptive and analytical study including 20 type 1 diabetic patients treated by insulin pump, between 2017 and 2021. All patients received a clinical evaluation, analysis of capillary blood glucose monitoring and a dosage of HbA1c at the time of the start of insulin pump and during the evolution. Insulin pump satisfaction was assessed using the Diabetes Treatment Satisfaction Questionnaire (DTSQ). Statistical analysis was performed by SPSS version-21. Results: The mean age of the patients was 16,8 ± 8,1 years with a sex ratio (M/F) of 0,42. Thirty per-cent were children. The mean duration of diabetes was 5,8 ± 4,8 years. Seventy-five per-cent of patients practiced functional insulin therapy. The indications for insulin pump treatment were mainly hypoglycaemia and instable diabetes. During follow-up, we found a statistically significant decrease in insulin requirements, improvement in mean HbA1c and maintenance of glycaemic control during follow-up, with a marked reduction in the number of hypoglycaemia events per week. The overall satisfaction score was calculated at 34,6 ± 2,5 out of 36 with a decrease in the score for perception of hyperglycaemia or hypoglycaemia. Conclusion: Insulin pump therapy appears to be reliable and effective when used appropriately, combined with appropriate therapeutic education and glycaemic monitoring to maintain long-term glycaemic control and improved quality of life.
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Diabetes Mellitus Tipo 1 , Hipoglicemia , Criança , Humanos , Recém-Nascido , Diabetes Mellitus Tipo 1/tratamento farmacológico , Hemoglobinas Glicadas , Glicemia/metabolismo , Qualidade de Vida , Estudos Retrospectivos , Automonitorização da Glicemia , Controle Glicêmico , Satisfação do Paciente , Insulina , Insulina Regular Humana/uso terapêutico , Satisfação Pessoal , Hipoglicemiantes/uso terapêuticoRESUMO
Fahr's syndrome is defined by the presence of striato-pallido-dentate calcifications. It is a rare entity with clinical polymorphism, and it occurs in patients with dysparathyroidism, especially those with hypoparathyroidism. It must be distinguished from Fahr's disease (FD), which is defined by the presence of intracerebral calcifications without phosphocalcic metabolism abnormality. In this paper, we report the particulars of five patients diagnosed with Fahr's syndrome revealed by neurological and cognitive disorders, seizures, and abnormal movements associated with tetany crisis. In all cases, brain imaging and biological examinations led to the diagnosis of Fahr's syndrome related to hypoparathyroidism. The evolution was favorable after treatment. Fahr's syndrome is a rare and serious condition for which treatment is simple and effective. Our observations shed light on the necessity of evaluating phosphocalcic metabolism and exploring cerebral calcifications in patients with neurological disorders.
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Summary: Cushing's disease or pituitary adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome is considered a rare condition. It is caused by hypersecretion of the ACTH by a pituitary adenoma that ultimately induces endogenous hypercortisolism by stimulating the adrenal glands. It is responsible for significant morbidity and mortality. The clinical signs and symptoms of hypercortisolism are usually common and non-specific including obesity, moon face, hypertension, hirsutism and facial plethora. The association between Cushing's disease and calcinosis cutis which is defined as dystrophic calcium deposition in the skin and subcutaneous tissues is extremely rare. To the best of our knowledge, it has never been described previously in humans, probably like a symptom or complication of chronic and severe hypercortisolism. In this paper, we report a case of a 30-year-old female diagnosed with Cushing's disease and presented bilateral leg's calcinosis cutis complicated with ulceration. The evolution was favorable and the complete cicatrization was obtained 12 months following the suppression of systemic glucocorticoid excess. Learning points: Calcinosis cutis is common in autoimmune connective diseases. However, to our knowledge, it has never been reported in humans with Cushing's disease. Given the rarity of this association, the diagnostic approach to calcinosis cutis must exclude the other etiologies. Calcinosis cutis is challenging to treat with no gold standard therapy. In our case, the use of the combination of colchicine and bisphosphonates does not significantly improve the patient's outcomes. In fact, we suppose that without treating the endogenous hypercortisolism, the calcinosis cutis will not resolve.
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Background: Long-term glucocorticoid (GC) use is the most frequent cause of adrenal insufficiency through suppression of the hypothalamic-pituitary-adrenal axis. There are no guidelines for predicting adrenal insufficiency (AI) and minimizing its risk. Methods: This is a prospective observational study carried out in the Endocrinology-Diabetology and Nutrition department between 2014 and 2021. All patients had received GC therapy for longer than 3 weeks before switching to hydrocortisone substitution, for various indications. These patients were admitted to our department for stimulation tests. We assessed the prevalence of AI, predicting factors, screening and management. Results: In our study the GC-induced AI was found in 49% of patients. We found a strong correlation between the basal morning serum cortisol, body mass index and the peak cortisol level after stimulation tests, while no correlation was found between adrenal function and age, sex, indication of GC therapy, duration of corticosteroid therapy, cumulative dose and daily dose. Patients with GC induced AI took a mean of 12 ± 12,18 months to recover. Adrenal function recovery rate was higher in patients tested by short Synacthen than in those tested by Insulin Hypoglycemia. Conclusions: We demonstrated the positive correlation between serum cortisol peak levels after stimulation and body mass index. The study supports that basal cortisol level, the duration of corticoid cessation and the type of stimulation test can predict the response of cortisol to stimulation tests.
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INTRODUCTION: Adrenal myelolipomas are rare non-functioning benign tumors composed of adipose and hematopoietic tissues. Most AMLs are discovered incidentally and represent the second most common adrenal incidentaloma. CASE PRESENTATION: A 58-years-old female patient, obese with a history of diabetes and blood hypertension presented with complaints of pain in the left flank. Abdominopelvic computed tomography showed a giant well-defined mass of the left adrenal gland with fat density suggesting adrenal myelolipoma. The patient underwent open left adrenalectomy. The pathological study confirmed the diagnosis of adrenal myelolipoma. DISCUSSION: Most AMLs are asymptomatic, remain stable in size, or grow slowly. Mass effect symptoms and spontaneous rupture are observed more in larger AMLs. The most common symptoms observed are abdominal discomfort/pain, hypochondrial pain, and flank pain. Most of the AMLs are discovered incidentally and the radiological features are accurate in diagnosing AML in up to 90% of the cases, CT is more sensitive for detection than other imaging modalities. The open surgery approach is the standard treatment of choice for giant AML (>10cm) while the minimally invasive approach has been used in only a few cases. CONCLUSION: The therapeutic management is discussed on a case-by-case basis. Surgical treatment is indicated for larger, symptomatic, or rapidly growing AMLs. Meanwhile smaller and asymptomatic AMLs are managed conservatively.