Parental self-medication with antibiotics in a Tunisian pediatric center.

INTRODUCTION: Self-medication of antibiotics among children is a very common problem in Tunisia. Its prevalence isn't well established. The aims of this study are to evaluate parents' knowledge concerning antibiotic use, and identify the factors associated with this problem. PATIENTS AND METHODS: We conducted a cross-sectional study over a one year period (between August 2019 and July 2020). Data collection was performed using a questionnaire guided interview. We included parents of children consulting or hospitalized in the pediatric department of the university hospital Taher Sfar in Mahdia. RESULTS: A total of 354 parents were included with an average age of 36.4±9.2 years. The average knowledge score was 2±1.3 points. In fact, 61.6% of the parents had poor knowledge about antibiotics. The frequency of non-prescription antibiotics use among children was 20.6%. Amoxicillin was the most used antibiotic (72.6%). Sore throat, important fever and flu-like symptoms were the main symptoms justifying non-prescription antibiotic use among our pediatric population in 60.3%, 34.2% and 23.3% of cases respectively. The main reason of self-medication was the fact that the same antibiotic was once prescribed to treat the same symptoms (58.9%). The used antibiotic came from an old prescription for the same child in 57.5% of the cases and was recommended by the pharmacist in 39.7% of the cases. After multivariate analysis, the factors associated with parental self-medication with antibiotics were: the advanced parent's age, the ability to name an antibiotic and knowledge's score>2. CONCLUSION: Our study confirmed that parental knowledge about antibiotic use is low. In fact, the government should from one hand, organize antibiotic delivery and prohibit off the counter sells and in the other hand promote the education of the public through different procedures to stop this major health problem.

Combined sclerosis of the spinal cord revealing Biermer's anemia: about a case.

Biermer's anemia is an auto immune disease that can lead to neurological manifestations. Medullary combined sclerosis accounts for only 10% of neurological complications. There are few reports of documented bone marrow involvement by magnetic resonance imaging (MRI). Observation: We report a case of combined sclerosis of the cervical spinal cord assessed by cerebro-medullary MRI in a context of vitamin B12 deficiency in a 36-year-old patient who was hospitalized in the intensive care unit for septic shock with respiratory starting point, requiring the use of mechanical ventilation for 13 days and the discovery in post-extubation of pancytopenia with macrocytic anemia and flasquo-spasmodic tetraparesis prompting an emergency MRI showing combined sclerosis of the spinal cord , with vitamin B12 deficiency and megaloblastic anemia at the myelogram. The patient had benefited of vitamin therapy combined with a physical rehabilitation and the evolution was marked by a recovery of walking and normalization of biology after 2 months. Conclusion:    The advantage of medullary MRI in a severe clinical form with neurological impairment of vitamin B12 deficiency to guide the diagnosis. The treatment is based on vitamin therapy with a good evolution.

[Corticosurrenaloma: an exceptional cause of primary hyperaldosteronism]. / Le corticosurrénalome: une cause exceptionnelle d'hyperaldostéronisme primaire.

Corticosurrenaloma is a rare malignant tumor of the adrenal gland that often secretes corticosteroids, sex steroids and precursors. Aldosterone-producing corticosurrenaloma is very rare, accounting for 1 case/10million inhabitants. We report the case of a 38-year old man presenting with severe arterial hypertension associated with deep hypokalaemia (2.2 mmol/L). Exploration showed primary hyperaldosteronism (aldosterone = 2645 pmol/l, aldosterone/renin ratio = 327 pmol/MUI), with hypersecretion of glucocorticoids. Abdominopelvic CT scan revealed left poorly differentiated and heterogeneous adrenal mass measuring 9cm, infiltrating into the surrounding adipose tissue and the diaphragm, extending into the left renal vein, with regional adenopathy and hepatic nodule measuring 4cm. The patient underwent radical nephrectomy followed by right hepatectomy two months after resulting in remission. A year after the patient developed lung metastases. This study highlights that corticosurrenaloma should be suspected in patients with primary aldosteronism despite its rarity.

Primary Hyperparathyroidism and Hyperthyroidism in a Patient with Myotonic Dystrophy: A Case Report and Review of the Literature.

Various endocrine manifestations are commonly described in myotonic dystrophy (MD), including primary hypogonadism, diabetes mellitus, and thyroid and parathyroid dysfunction. We describe a 46-year-old woman with a family history of MD with her son. She was diagnosed with cardiac arrhythmia and required the implantation of a pacemaker. She was noted to have a bilateral cataract. She complained of muscle weakness, diffuse myalgia, and palpitation. The electromyography (EMG) showed myotonic discharges. Laboratory tests showed high serum calcium 2.83 mmol/L, serum phosphate 1.2 mmol/L, parathormone 362.5 pg/mL, thyroid stimulating hormone TSH 0.02 mIU/L (normal range: 0.34-5.6 mIU/L), FT4 21.17 ng/mL, and negative anti-thyroperoxidase antibodies. Cervical ultrasound revealed a multinodular goiter. The 99mTc-MIBI scintigraphy localized a lower right parathyroid adenoma. The clinical data, the family history of MD, EMG data, and endocrine disturbances were strongly suggestive of MD associated with hyperthyroidism and primary hyperparathyroidism.

Multifocal diabetic muscle infarction: a rare complication of poorly controlled diabetes mellitus.

Diabetic muscle infarction (DMI) is a rare complication of long-standing poorly controlled diabetes mellitus. We herein describe the case of a 56-year-old man with a 10-year history of poorly controlled type 2 diabetes mellitus with multiple microvascular and macrovascular complications who presented with the sudden onset of left thigh pain and swelling. MRI suggested muscle infarction. A muscle biopsy demonstrated coagulation necrosis in the skeletal muscle with inflammation and infarction in the walls of small blood vessels. Physicians should consider DMI in the differential diagnosis of patients with diabetes who present with painful, swollen muscles without systemic signs of infection.

Pyogenic sacroiliitis and pyomyositis in a patient with systemic lupus erythematous.

Pyogenic sacroiliitis and pyomyositis are uncommon infectious diseases and their diagnoses are often delayed. They are typically seen in children and young adults and are rare in middle-aged people especially in those affected by rheumatic diseases. We present the first case of a Staphylococcus aureus related pyogenic sacroiliitis associated with iliacus and gluteal pyomyositis occurring in a patient with systemic lupus erythematosus. Antibiotic treatment was administered for a total of 6 weeks with a total recovery. Pyogenic sacroiliitis and pyomyositis, although remaining rare events, should be remembered as severe complications in immunosuppressed patients with inflammatory diseases. Early clinical suspicion, imaging diagnosis, and adequate therapy are decisive for the satisfactory outcome.

Congenital adrenal hyperplasia: Treatment and outcomes.

Congenital adrenal hyperplasia (CAH) describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

Cerebral Aneurysms: A Rare Feature of Behçet's Disease-A Case Report and Review of the Literature.

Behçet's disease (BD) is a multisystem vascular inflammatory disease with several clinical manifestations. Intracranial aneurysms are an extremely rare but nevertheless severe complication of BD. We report a case of a 44-year-old man. The diagnosis of BD was made based on the presence of recurrent oral aphthous ulcers and positive human leukocyte antigen (HLA-) B51 in the absence of evidence of other diseases. MRI showed an ancient ischemic right capsulolenticular lesion, subacute white matter hypersignals of the left capsule lenticular region, and multiple arterial aneurysms. The patient underwent two-month systemic high-dose corticosteroids and immunosuppressive therapy associated with severe neurological deficiency upon admission and severe impairment upon discharge. A thorough review of the literature showed 20 case reports of intracranial aneurysms in BD.

Brain magnetic resonance imaging findings in adult patients with congenital adrenal hyperplasia: Increased frequency of white matter impairment and temporal lobe structures dysgenesis.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis. The enzymes most commonly affected are 21-hydroxylase. Past reports suggested brain magnetic resonance imaging (MRI) abnormalities in CAH patients, affecting white matter signal, temporal lobe and amygdala structure and function. AIMS: In the present study, we aimed to investigate the frequency of white matter changes and temporal lobes structures dysgenesis in a population of patients having CAH due to 21-hydroxylase deficiency. MATERIALS AND METHODS: Neurological examination and brain MRI were performed in 26 patients. RESULTS: Neurological examination revealed mental retardation in three patients, tremor in two patients, tendon reflexes asymmetry in one patient, and cerebellar syndrome in one patient. Eleven patients (42.3%) showed MRI abnormalities: Eight of them had white matter hyperintensities, one patient had moderate atrophy in the right temporal, and hippocampal dysgenesis was found in the remaining two patients. CONCLUSIONS: Brain MRI abnormalities in CAH patients include white matter hyperintensities and temporal lobe structures dysgenesis. The mechanisms involved seem related to hormonal imbalances during brain development and exposure to excess exogenous glucocorticoids. Clinical implications of such lesions remain unclear. More extensive studies are required to define better the relationships between brain involvement and different CAH phenotypes and treatment regimens.

Metabolic profile and cardiovascular risk factors in adult patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

BACKGROUND: In congenital adrenal hyperplasia (CAH), long-term glucocorticoid treatment coupled with increased androgens may lead to undesirable metabolic effects. The aim of our report was to determine the prevalence of metabolic abnormalities and cardiovascular risk factors in a population of adult patients with CAH due to 21 hydroxylase deficiency. MATERIALS AND METHODS: Twenty-six patients (11 males and 15 females, mean age ± SD=27.4±8.2 years) were recruited. Anthropometry, body composition, metabolic parameters and cardiovascular risk factors were studied. RESULTS: Obesity (overweight included) was noted in 16 patients (61.5%), with android distribution in all cases. Bioelectrical impedance showed increased body fat mass in 12 patients (46.1%). Lipid profile alterations and carbohydrate metabolism disorders were detected in seven (26.9%) and five (19.2%) patients respectively. Moderate hepatic cytolysis, associated with hepatic steatosis, was found in one patient. Seven patients (27%) had insulin resistance. Ambulatory blood pressure monitoring showed abnormalities in six patients (23%). Increased carotid intima media thickness was found in 14 patients (53.8%). CONCLUSION: Adult CAH patients tend to have altered metabolic parameters and a higher prevalence of cardiovascular risk factors. Lifelong follow-up, lifestyle modifications, and attempts to adjust and reduce the glucocorticoid doses seem important.

Long-term outcome of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

INTRODUCTION: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder affecting adrenal steroid synthesis. In this study, the authors aim to evaluate the impact of CAH due to 21-hydroxylase deficiency on final height (FH), bone health, cardiometabolic risk, fertility, neurocognition and quality of life in a hospital-based sample from Tunisia. METHODS: Twenty-six patients (11 males and 15 females; mean age: 27.4 ± 8.2 years) were recruited. RESULTS: Mean FH was 159.5 ± 9.7 cm. Twenty-one patients (80.7%) had a FH below the target height. Ten patients (38.4%) exhibited bone demineralization. Eight patients (30.7%) had obesity. Lipid profile alterations and carbohydrate metabolism disorders were detected in 10 (38.4%) and 5 (19.2%) patients, respectively. Seven patients (27%) had insulin resistance. Ambulatory blood pressure monitoring showed abnormalities in 6 patients (23%). Increased carotid intima-media thickness was found in 14 patients (53.8%). Inhibin B level was decreased in 4 male patients. Semen analysis showed abnormalities in 4 of 10 patients. Testicular tumors were detected in 6 of 11 patients. Anti-Müllerian hormone level was reduced in 4 female patients. Six patients showed poly-cystic ovary syndrome. Brain magnetic resonance imaging showed abnormalities in 11 patients (42.3%). Quality of life was reduced in 14 of 22 patients (63.6%). Many of the suboptimal outcomes appeared to be related to poor adherence to medication schedules, some to overtreatment. CONCLUSION: CAH patients have a number of issues due to the disease or its treatment. Regular follow-up, early lifestyle interventions, bone health assessment, testicular ultrasound and psychological management are needed.

48XXYY Syndrome in an Adult with Type 2 Diabetes Mellitus, Unilateral Renal Aplasia, and Pigmentary Retinitis.

A 45-year-old male was referred for diabetes mellitus. Clinical examination found a family history of multiple precocious deaths, strong consanguinity, personal history of seizures during childhood, small testicles, small penis, sparse body hair, long arms and legs, dysmorphic features, mental retardation, dysarthria, tremor, and mild gait ataxia. Investigations found pigmentary retinitis, metabolic syndrome, unilateral renal aplasia, and hypergonadotropic hypogonadism, and ruled out mitochondrial cytopathy and leucodystrophy. Karyotype study showed a 48XXYY chromosomal type. Renal aplasia and pigmentary retinitis have not been described in 48XXYY patients. They may be related to the chromosomal sex aneuploidy, or caused by other genetic aberrations in light of the high consanguinity rate in the patient's family.

Isolated neurosarcoidosis revealed by diabetes insipidus, visual loss and diplopia in a child patient: a diagnostic problem.

We report a case of 15-yr-old child that was presented with headache, polyuria, polydipsia, recent ocular motor and abducens nerve palsies and rapid visual loss. He had a long history of progressive symmetric muscular weakness predominant in the lower limb girdle. Water deprivation revealed central diabetes insipidus. Hormonal explorations demonstrated preserved pituitary function with mild hyperprolactinemia at 21.5 ng/ml (N: 2.6 to 13.1 ng/ml). Magnetic resonance imaging showed an extensive isosignal T1 and hyposignal T2 enhanced lesion infiltrating the pituitary gland, optic-chiasmal hypothalamic region, cavernous sinus, cerebrum tent and sphenoid and temporal meningeal spaces. The serum level of angiotensin converting enzyme and cerebrospinal fluid analysis were normal. No other systemic localisation was identified. Muscle biopsy objectified dystrophic changes. Genetic study identified a delT 521 mutation characteristic of Limb-girdle muscular dystrophy type 2C. Corticotherapy rapidly ameliorated the neurological symptoms. This patient was diagnosed as having neurosarcoidosis. Neurosarcoidosis is rarely reported in childhood. We discuss the problems related to diagnosis in such a situation below.