1/6. Ocular manifestations of Donohue's syndrome.INTRODUCTION: Donohue's syndrome, also known as Leprechaunism, is a rare autosomal recessive disease that manifests at birth with symptoms of endocrine dysfunction. Metabolic characteristics of the disease include postprandial hyperglycemia, fasting hypoglycemia, insulin resistance, hyperinsulinemia, and failure to thrive. The physical features most often associated with this condition include hypertrichosis, pachyderma, acanthosis nigricans, prominent genitalia, and elfin-like facial characteristics of prominent eyes, wide nostrils, thick lips, and large, low-set ears. Not only is this syndrome rare, but it often results in infant and early childhood mortality. The literature regarding ocular manifestations is limited. CASE REPORT: We present a case of a 29-year-old male with Donohue's syndrome and significant ocular findings including a subluxated mature cataract, retinal detachment, high myopia, and optic atrophy. DISCUSSION: These ocular sequelae are discussed with regard to the noted endocrine dysfunction and its effects on tissue development and growth.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/6. blood glucose changes in diabetic children and adolescents engaged in most common sports activities.Circulating insulin levels decrease and substrate glycogenolysis-mediated conversion into glucose increases just a few minutes after normal subjects start exercising, but during sustained physical activity muscles massively utilize blood glucose, thus causing glycogenolysis to increase further until the end of the session. After that, in order to get liver and muscle glycogen stores up to pre-exercise levels again, blood glucose is mostly utilized, thus causing late-onset hypoglycaemia in the absence of any extra carbohydrate supply and rebound hyperglycaemia after a while. This and other patho-physiological mechanisms are dealt with in the present paper, and practical hints are provided to the clinician to cope with children-specific adaptation phenomena to exercise in t1DM.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/6. Treatment of hyperinsulinism after partial pancreatectomy: medical or surgical?A 21 year old male patient with hypoglycemia secondary to hyperinsulinism had no palpable adenoma at surgery, and failed to respond to a partial (75%) pancreatectomy. Subsequently, he was begun on oral diazoxide. Soon after initiation of diazoxide he developed a viral pneumonitis, and because of hyperglycemia, the diazoxide was temporarily discontinued. He has been treated since June of 1975 with diazoxide, 100 mg, three times daily. He is asymptomatic, completely rehabilitated, and physically active at work for 10 hours daily. diazoxide therapy may be an option worth considering even in benign states of hyperinsulinism when surgery has failed to correct the process.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/6. Acute alcohol intoxication in a two-month-old baby.A 2-month-old, well developed, healthy boy, weighing 5.55 kg, was fed 200 ml of bottle-milk containing 65 ml of sake. So-called kanzamashi (sake boiled in the evening and remaining in a bottle overnight,) was mistaken for yuzamashi (water boiled and left to cool), and used to prepare a 15% formula milk. About 10 minutes later, the baby became flushed, began to breath hard, and lose consciousness, and an alcoholic odor was noticed. He was brought to our clinic, where gastric lavage and parenteral fluid therapy were started. On admission, his main physical signs were, whole body had become red, unconsciousness, alcoholic odor, tachycardia and tachypnea, without low body temperature, while his remarked laboratory findings were metabolic acidosis, hyperglycemia, and high A/G ratio. Moreover, a transient proteinuria, alternately followed by a transient glycosuria, appeared within the course. About 10 hours later, he showed an obvious improvement in both physical and laboratory findings. As an explanation of these changes in his condition due to alcohol ingestion, we speculated that a metabolic acidosis with hyperglycemia caused the disturbed reabsorption in his renal tubulus, which revealed alternating proteinuria and glycosuria.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
5/6. Superior mesenteric arteriovenous fistula in vascularized whole organ pancreatic allografts.Despite improved results in vascularized pancreatic transplantation, the incidence of technical complications continues to be high. Vascular complications are an important source of morbidity. Previously undescribed is the occurrence of an arteriovenous fistula (AVF) in the transplanted mesenteric bundle. We have identified this unusual complication in three of 90 consecutive recipients of pancreatic transplant. The first patient presented with severe and protracted endocrine insufficiency, which was effectively reversed by direct surgical ligation of the AVF. The second patient presented with hematuria, which likewise, resolved with correction of the AVF. The third instance was diagnosed immediately after transplant and was successfully corrected by direct surgical ligation. The operative approach with establishment of proximal vascular control differed in each instance. Clinical presentation was variable and diagnosis was suggested by physical examination, duplex ultrasonography and radionuclide perfusion scanning. Arteriography was confirmatory. Mesenteric AVF can be easily identified and corrected at the time of reoperation without compromising allograft function.- - - - - - - - - - ranking = 5.4135550179935keywords = physical examination, physical (Clic here for more details about this article) |
6/6. DHF with complication of acute pancreatitis related hyperglycemia: a case report.DHF is endemic in indonesia, with incidence of 9.72/100,000 population and CFR of 2.5%. Acute pancreatitis is a rare complication in DHF, usually without hyperglycemia. We report here 1 patient of DHF grade II with complication of acute pancreatitis, and hyperglycemia which occured as a result of pancreatitis. A 24 years old female was referred from Santa Jusuf Hospital, with 5 days of fever and hematemesis. On physical examination we found slight fever and hematoma on her left leg. Laboratory examination revealed Hb 13.4 g%, WBC 8,500/mm3, Ht 42%, platelets 22,500/mm3, amylase 317 U/l, lipase 1,198 U/l and blood glucose 397 mg%. CT scan result of pancreas was consistent with acute pancreatitis. diagnosis of dengue infection was made after the finding of positive IgM and IgG for dengue virus. After 18 days clinical symptoms and signs and laboratory results returned to normal.- - - - - - - - - - ranking = 5.4135550179935keywords = physical examination, physical (Clic here for more details about this article) |