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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 22-year old woman who presented skin lesions of acanthosis nigricans, hirsutism and secondary amenorrhoea. She had high plasma levels of adrenal androgens and low plasma levels of sex steroid binding protein. Polycystic ovaries were discovered in the course of a laparotomy performed for paraovarian cyst. An oral glucose tolerance test revealed a state of hyperinsulinism with intolerance to carbohydrates, while the body mass index was normal. This insulin resistant state corresponded in vitro to a decrease in the number of erythrocyte insulin receptors without decrease in their affinity for insulin. Following paradoxical improvement during a full-term pregnancy, there was gradual deterioration of diabetes control requiring insulin therapy. This metabolic decompensation was accompanied by major hyperlipaemia followed by acute haemorrhagic pancreatitis. This case illustrates the course of a type A insulin resistance syndrome which was detected at an early stage in front of an hirsutism-acanthosis nigricans association. The underlying pathogenic mechanisms of these pathologies are discussed.
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PMID:[Acanthosis nigricans, hyperandrogenism, insulin resistance and mixed hyperlipemia]. 297 81

We investigated the metabolic effects of omega-6 (safflower oil) and omega-3 (fish oil) fatty acid-enriched diets (65% carbohydrate, 20% fat) in two patients with a syndrome of diabetes mellitus, lipodystrophy, acanthosis nigricans, chylomicronemia, and abdominal pain. 3H-glycerol was used to evaluate triglyceride-rich lipoprotein-triglyceride (TRLP-TG) metabolism, and changes in glucose and insulin dynamics were also studied. On the omega-6 diet, both subjects demonstrated four- to five-times normal rates of TRLP-TG production and glycerol biosynthesis, and striking decrements in the fractional catabolic rate (FCR) for TRLP-TG and TRLP-particles. Both subjects had elevations in nonesterified fatty acid (NEFA) concentrations. In one patient, the omega-3 diet markedly decreased serum triglycerides and newly synthesized triglyceride glycerol production, in association with a fall in NEFA. In both subjects, plasma glycerol reutilization for triglyceride synthesis, normal on the omega-6 diet, was abolished on the omega-3 regimen. Plasma postheparin lipolytic activity was normal on both diets. On the omega-3 diet, xanthomas and hepatomegaly decreased and, in the patient who had no reduction in serum triglycerides, pancreatitis attacks virtually ceased. Mean 24-hour serum glucose levels were higher, and both basal and peak C-peptide responses to a carbohydrate meal were blunted on the omega-3 diet. One patient became ketonuric. We conclude the cause of hypertriglyceridemia in these patients was due to increased lipid synthesis and hypothesize that this is secondary to high plasma concentrations of NEFA. In addition, an omega-3 diet in these subjects inhibited insulin secretion and worsened glucose tolerance.
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PMID:Lipodystrophic diabetes mellitus. Investigations of lipoprotein metabolism and the effects of omega-3 fatty acid administration in two patients. 305 Mar 65

A ten-fold increase in daily insulin requirements during the administration of total parenteral nutrition (TPN) is described in a patient with congenital generalized lipodystrophy, insulin-requiring diabetes mellitus, and acanthosis nigricans during an episode of acute pancreatitis secondary to hypertriglyceridaemia. After a period of 13 days on TPN, insulin requirements increased dramatically to an average of 1428 units per day for a period of 12 days, to achieve a mean blood glucose level of 10.9 mmol l-1. When the patient resumed feeding and the TPN was discontinued, the average daily insulin requirement was 104 units with a mean 24 h blood glucose of 11.8 mmol l-1. Parenteral administration of energy substrates in a rare case of diabetes mellitus and congenital lipodystrophy complicated by acute pancreatitis resulted in a severe insulin insensitive state due to the combination of the hypermetabolism conferred by the pancreatitis plus transient impairments of the glucose disposal mechanism by the energy substrates provided.
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PMID:Insulin requirements in lipodystrophic diabetes. 828 34

Lipatrophic diabetes, also referred to as familial partial lipodystrophy, is a rare disease that is metabolically characterized by hypertriglyceridemia and insulin resistance. Affected patients typically present with regional loss of body fat and muscular hypertrophic appearance. Variable symptoms may comprise pancreatitis and/or eruptive xanthomas due to severe hypertriglyceridemia, acanthosis nigricans, polycystic ovaria, and carpal tunnel syndrome. Mutations within the LMNA gene on chromosome 1q21.2 were recently reported to result in the phenotype of familial partial lipodystrophy. The genetic trait is autosomal dominant. We identified a family with partial lipodystrophy carrying the R482W (Arg(482)Trp) missense mutation within LMNA. Here we present the lipoprotein characteristics in this family in detail. Clinically, the loss of sc fat and muscular hypertrophy especially of the lower extremities started as early as in childhood. Acanthosis and severe hypertriglyceridemia developed later in life, followed by diabetes. The characterization of the lipoprotein subfractions revealed that affected children present with hyperlipidemia. The presence and severity of hyperlipidemia seem to be influenced by age, apolipoprotein E genotype, and the coexistence of diabetes mellitus. In conclusion, dyslipemia is an early and prominent feature in the presented lipodystrophic family carrying the R482W mutation within LMNA.
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PMID:Dyslipemia in familial partial lipodystrophy caused by an R482W mutation in the LMNA gene. 1134 41

Lipodystrophies represent a heterogeneous group of diseases characterized by an abnormal subcutaneous fat distribution, the extent of which can vary from localized, to partial, to generalized lipoatrophy. Whereas partial and generalized lipodystrophies are each associated with metabolic abnormalities, the localized form is not. These metabolic changes include insulin resistance with type 2 diabetes, acanthosis nigricans, dyslipidaemia predominantly consisting of hypertriglyceridaemia (associated with the onset of pancreatitis) and depressed HDL cholesterol, liver steatosis and hypertension. Affected women are often hirsute and this can be associated with the presence of polycystic ovarian syndrome (PCOS). Most of these clinical features are present to some extent in patients with the common metabolic syndrome. As the prevalence of metabolic syndrome far outweighs that of lipodystrophy, the diagnosis of this rare disorder may often be overlooked with the affected patient diagnosed as merely being 'yet' another case of metabolic syndrome. In this article, we draw attention to the importance of recognizing patients with lipodystrophy who present with metabolic abnormalities, as both the diagnostic as well as the therapeutic approach of these patients differ profoundly from patients with the metabolic syndrome.
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PMID:Inherited lipodystrophies and the metabolic syndrome. 1756 81

Type 1 diabetes (T1D) is the most common form of diabetes in children in Western countries. There have been no large studies of childhood diabetes from India. We undertook the MEDI study (Multicenter Survey of Early Onset Diabetes in India) to assess the proportion of various subtypes of diabetes among the young subjects presenting to the endocrinology divisions of seven large teaching hospitals in different regions of India. In addition, we compared the clinical features of T1D and type 2 diabetes (T2D) in Indian subjects. Patients with onset of disease at younger than 20 years of age were included in this study. Six hundred and three subjects (603) were studied of whom 535 subjects (89%) had T1D, 36 (6%) had T2D, 18 (3%) had diabetes related to tropical pancreatitis or other forms of chronic pancreatitis, while other subtypes accounted for the rest. Compared to those with T2D, subjects with T1D were younger, had a lower C-peptide level, higher prevalence of ketosis, lower prevalence of acanthosis nigricans, and lower LDL and triglyceride levels. When compared with that of T2D, a higher proportion of patients with T1D were positive for GAD-65 and IA-2 antibodies, and this difference was statistically significant for GAD-65 antibodies. Overall, this large multicenter study showed that T1D is the commonest form of diabetes in childhood. T2D is the next most common kind, while chronic pancreatitis-related diabetes is uncommon.
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PMID:Type 1 diabetes versus type 2 diabetes with onset in persons younger than 20 years of age. 1912 Mar 3

There is paucity of literature on the relation of obesity with recurrent and chronic pancreatitis. We recorded the clinical details and the outcome of five patients with recurrent pancreatitis who had components of the metabolic syndrome. Their age ranged from 8 to 20 years. All five patients had acanthosis nigricans. Body mass index (BMI) could not be evaluated as these patients lost weight following episodes of pancreatitis. Three patients had two or more first-degree relatives who had diabetes mellitus. Only one patient had severe necrotizing pancreatitis. Coexisting liver disease was seen in two patients. Elevated serum cholesterol levels and moderately elevated serum triglycerides along with elevated serum amylase levels observed in these patients suggest possibility of a different mechanism from that of hypertriglyceridemia-related pancreatitis. Evaluation of pancreatic steatosis should be considered in patients with pancreatitis in the setting of metabolic syndrome.
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PMID:Pancreatitis in the setting of the metabolic syndrome. 2242 23

Familial partial lipodystrophy (FPL) type 1 is a syndrome characterized by loss of subcutaneous fat in arms and legs and an excess of body fat in face, neck, and torso. This rare syndrome is usually diagnosed when patients present cardiovascular complications or pancreatitis due to the severe metabolic abnormalities. Here we present the case of a 45 year old diabetic female without any pathological family history, a poor glycemic control (HbA1c 11.7%), hypertriglideridemia (3000 mg/dl), a body mass index (BMI) of 38, thin limbs, subcutaneous fat loss in gluteal area and ledge of fat above them, prominent veins in lower extremities, moon face, and acanthosis nigricans; as well as hypertension (150/100 mmHg) and subcutaneous folds measuring less than average were observed. Hypercortisolism was discarded and leptin levels were measured (16.8 mg/ml, VR: BMI > 30: 50 mg/ml). Due to these clinical and biochemical manifestations, and low leptin levels (16.8 mg/ml), Kobberling syndrome was suspected; however, LMNA mutation analysis was negative. Changes in lifestyle and treatment with fenofibrate, biphasic insulin 50/50, and enalapril were initiated showing a a significant metabolic improvement: HbA1c (7.8%) and TG (243 mg/dl). FPL type 1 is a familial disease, although there are spontaneous cases. No specific mutation is responsible for this syndrome. Due to its clinical manifestations, Cushing syndrome must be discarded.
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PMID:[Familial partial lipodystrophy type 1. A rare or underdiagnosed syndrome?]. 2563 99

Pancreatitis is among rare diseases in pediatrics clinics. It is usually presented with a sign of underlying systemic disease. Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare disease characterized by near absence of adipose tissue resulting in apparent muscle hypertrophy from birth or early infancy associated with severe insulin resistance. Common clinical features are hypertriglyceridemia, acanthosis nigricans, hepatomegaly with or without splenomegaly and high stature. Acromegaloid features, cardiomyopathy and mental retardation can also be present. We describe a 7-year-old Turkish boy with these clinical features of BSCL and presented with acute pancreatitis.
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PMID:A very rare cause of acute pancreatitis: Berardinelli-Seip congenital lipodystrophy. 2591 Mar 11

Pancreatic pathology, comprising acute and chronic pancreatitis, autoimmune pancreatitis and pancreatic neoplasms, primarily presents with gastrointestinal symptoms and signs; however, it is well recognized that it can also associate a wide range of extra-digestive features. Among these systemic manifestations, cutaneous involvement plays an important role both as a diagnostic clue for the pancreatic disease itself and serving as a prognostic factor for the severity of the condition. Recognition of these cutaneous signs is, however, far from being satisfactory, all the more as some of them are relatively rare. In the current review, we discuss skin involvement in pancreatic diseases, referring to pancreatic panniculitis, cutaneous hemorrhagic manifestations, skin metastasis, acanthosis nigricans, livedo reticularis, necrolytic migratory erythema and cutaneous fistula. We highlight the clinical characteristics, treatment and prognostic value of these lesions. Better awareness among medical specialties other than dermatology is needed for detection of the skin clues associated with pancreatic pathology.
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PMID:Cutaneous Manifestations in Pancreatic Diseases-A Review. 3280 80


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