ABSTRACT A literature review on the clinical, laboratory, and treatment features of type B insulin resistance syndrome (TBIRS). Data from PubMed, the Virtual Health Library and Cochrane database were selected and analyzed using the REDCap application and R statistical program. From 182 papers, 65 were selected, which assessed 119 clinical cases, 76.5% in females and 42.9% in African-Americans, with an average age of 44 years. A common feature of TBIRS is co-occurrence of autoimmune diseases, such as systemic lupus erythematosus (most frequently reported). Hyperglycemia of difficult control was the mostly reported condition. Tests for anti-insulin receptor antibodies were positive in 44.2% of the cases. Disease management comprised fractional diet, insulin therapy (maximum dose given was 57 600 IU/day), plasmapheresis and immunosuppression with several classes of drugs, mainly glucocorticoids. Remission occurred in 69.7% of cases, in 30.3% of these spontaneously. The mortality rate was 15.38%. There was an inverse relationship between anti-insulin antibodies and remission (p = 0.033); and a positive correlation between combined immunosuppressive therapy and remission (p = 0.002). Relapse occurred in 7.6% of the cases. This rare syndrome has difficult-to-control diabetes, even with high doses of insulin, and it is usually associated with autoimmune diseases. Therapeutic advances using immunomodulatory therapy have led to significant improvements in the rate of remission.

SUMMARY Insulin autoimmune syndrome (IAS, Hirata's disease) is a rare hypoglycemic disorder characterized by spontaneous hypoglycemia associated with extremely high circulating insulin levels and positive anti-insulin antibody results. Thus far, most cases have been reported in Asian countries, notably Japan, with few cases reported in western countries. As a possible cause, it is associated with the use of drugs containing sulfhydryl radicals, such as captopril. This report refers to a 63-year-old female Brazilian patient with a history of postprandial hypoglycemia. After extensive investigation and exclusion of other causes, her hyperinsulinemic hypoglycemia was considered to have likely been induced by captopril. Most cases of IAS are self-limiting. However, dietary management, corticosteroids, plasmapheresis, and rituximab have already been used to treat patients with IAS. In our case, after discontinuation of captopril, an initial decrease in insulin autoantibody levels was observed followed by improvement in episodes of hypoglycemia. Although it is a rare disease, IAS should be considered in the differential diagnosis of endogenous hyperinsulinemic hypoglycemia. Patients with suspected IAS must be screened for autoimmunity-related drugs for insulin. Initial clinical suspicion of IAS can avoid unnecessary costs associated with imaging examinations and/or invasive surgical procedures.

A gravidez afeta tanto o metabolismo materno quanto o fetal e, mesmo em mulheres não diabéticas, apresenta um efeito diabetogênico. Entre as mulheres grávidas, 2% a 14% desenvolvem o diabetes gestacional. A gravidez pode ocorrer também em mulheres já diabéticas, o que pode predispor o feto a muitas alterações na organogênese, restrição de crescimento e a mãe a algumas complicações relacionadas ao diabetes, tais como retinopatia e nefropatia, ou acelerar o curso dessas complicações se já estiverem presentes. Pacientes com diabetes gestacional geralmente iniciam seu tratamento com dieta e mudanças no estilo de vida; porém, quando essas medidas falham em atingir um controle glicêmico adequado, a insulinoterapia deve ser considerada. Pacientes com diabetes tipo 2 em uso de hipoglicemiantes orais são aconselhadas a iniciar o uso de insulina. Pacientes com diabetes tipo 1 preexistente devem iniciar um controle glicêmico estrito. Em função do fato de os análogos basais de insulina estarem sendo utilizados muito frequentemente off-label em pacientes grávidas, faz-se necessário avaliar sua segurança e eficácia nessa condição. O objetivo desta revisão é avaliar o uso de tais análogos, tanto de ação curta como prolongada, durante a gravidez, para possibilitar médicos clínicos, obstetras e endocrinologistas escolher o melhor regime terapêutico para suas pacientes.

Pregnancy affects both maternal and fetal metabolism, and even in non-diabetic women, it exerts a diabetogenic effect. Among pregnant women, 2% to 14% develop gestational diabetes. Pregnancy can also occur in women with preexisting diabetes, which may predispose the fetus to many alterations in organogenesis, restrict growth, and the mother, to some diabetes-related complications, such as retinopathy and nephropathy, or to acceleration of the course of these complications, if they are already present. Women with gestational diabetes generally start their treatment with diet and lifestyle changes; when these changes are not enough for optimal glycemic control, insulin therapy must then be considered. Women with type 2 diabetes using oral hypoglycemic agents are advised to change to insulin therapy. Those with preexisting type 1 diabetes should start intensive glycemic control. As basal insulin analogues have frequently been used off-label in pregnant women, there is a need to evaluate their safety and efficacy. The aim of this review is to report the use of both short- and long-acting insulin analogues during pregnancy and to enable clinicians, obstetricians, and endocrinologists to choose the best insulin treatment for their patients.

OBJECTIVE: The objectives of this study are to estimate the prevalence of arterial hypertension (AH) in an adult population with a predominance of families with low education and income levels, in the hinterlands of Pernambuco, Brazil, and to analyze its association with other factors related to cardiovascular diseases (CVD). METHODS: A cross-sectional study in 2008/2009 was conducted with a sample of 198 subjects stratified by age, and representative of the urban adult population of the Canaã district of city of Triunfo, in the hinterlands of Pernambuco, Brazil. RESULTS: One hundred ninety eight individuals with average age of 57.7 years old (31 to 90 years-old), mainly women (65.6%), and with low income and education levels (81.3% with a monthly income of less than one minimum wage) were evaluated. Among these, 127 (64.1%) were identified as having AH, 54 (42.5%) of whom had no prior diagnosis. From those who were previously diagnosed, only 31.3% had good blood pressure control. Higher prevalence was observed in those individuals with lower incomes, higher body mass indexes (BMI), and those with metabolic syndrome (MS). CONCLUSION: These data demonstrated that there was a high prevalence of AH in the urban, low education and income levels adult population of Triunfo, strongly associated with lower income levels, elevated BMI, and the presence of MS; and a high prevalence of bad blood pressure control among the previously diagnosed cases. These results indicate that more effective interventions for early detection and adequate control of this disease and its comorbidities are necessary.

CONTEXTO: A prevalência de diabetes mellitus tem aumentado, em decorrência das mudanças no estilo de vida e do aumento da expectativa de vida da população. . No Brasil, o estudo que avaliou as prevalências de diabetes e intolerância a glicose foi o Estudo Multicêntrico Brasileiro, realizado de 1986 a 1988. OBJETIVO: Determinar a prevalência de diabetes e intolerância a glicose na população urbana, de 30 a 69 anos, do município de Ribeirão Preto (SP), Brasil. TIPO DE ESTUDO: Estudo transversal com inquérito domiciliar em duas etapas. LOCAL: Ribeirão Preto, São Paulo, Brasil. PARTICIPANTES: Amostra randomizada de 1.473 indivíduos. MÉTODOS: O planejamento amostral adotado foi de conglomerados em estágios considerando sexo, faixa etária e renda do chefe dos domicílios Os indivíduos eram rastreados com glicemia capilar de jejum (GCJ). Aqueles com resultado positivo (glicemia capilar em jejum > 100 mg/dl) e a cada sétimo com resultado negativo (< 100 mg/dl) eram submetidos a sobrecarga oral com 75 g de glicose. Os diagnósticos de diabetes e intolerância a glicose foram baseados nos critérios da Organização Mundial da Saúde. RESULTADOS: As prevalências de diabetes e de intolerância a glicose foram de 12,1 e de 7,7%, respectivamente. Homens e mulheres apresentaram freqüências semelhantes de diabetes (12,0 versus 12,1%) e intolerância a glicose (7,9 versus 7,3%). Diferenças entre brancos (11,6%) e não-brancos (13,3%) para diabetes não foram significantes, enquanto a intolerância a glicose foi mais prevalente em brancos . As prevalências de diabetes e intolerância a glicose variaram de 3,3% e 2,6%, no grupo de 30-39 anos, a 21,7% e 11,3% no grupo de 60-69 anos, respectivamente. Indivíduos obesos (índice de massa corpórea > 30 kg/m²) e aqueles com história familiar de diabetes (parentes de primeiro grau) apresentaram prevalência maior de diabetes: 22,6% e 19,7%, respectivamente. CONCLUSÕES: A prevalência de diabetes em Ribeirão Preto se mostrou comparável à observada em países desenvolvidos. Em relação ao Estudo Multicêntrico Brasileiro, verificamos maior prevalência de diabetes, mas prevalência semelhante de intolerância a glicose. Estes achados podem refletir o impacto dos fatores ambientais e de estilo de vida, que têm ocorrido em cidades brasileiras como Ribeirão Preto, principalmente nas taxas crescentes de sedentarismo e obesidade.

CONTEXT: Diabetes mellitus prevalence has been increasing worldwide due to factors like lifestyle changes and higher life expectancy. The Brazilian Multicenter Study performed between 1986 and 1988 evaluated the prevalence of diabetes and impaired glucose tolerance. OBJECTIVE: To assess the prevalence of diabetes and impaired glucose tolerance in the urban population aged 30-69 years of the city of Ribeirão Preto, SP, Brazil. TYPE OF STUDY: A two-stage, cross-sectional home survey. SETTING: Ribeirão Preto, São Paulo, Brazil. PARTICIPANTS: A random sample of 1,473 individuals. METHODS: The sample plan was drawn up using a sampling scheme of stage conglomerates according to sex, age and family head income. Subjects were first screened by fasting capillary glycemia (FCG). Those that screened postiive (FCG > 100 mg/dl) and every seventh consecutive person who screened negative (FCG < 100 mg/dl) was submitted to a 75 g oral glucose tolerance test. The diagnosis of diabetes and impaired glucose tolerance were based on World Health Organization criteria. RESULTS: The overall rates of diabetes and impaired glucose tolerance were 12.1 and 7.7%, respectively. Men and women had similar rates of diabetes (12.0 vs. 12.1%) and impaired glucose tolerance (7.9 vs. 7.3%). Differences in the rates for whites (11.6%) and nonwhites (13.3%) for diabetes were not significant, while impaired glucose tolerance was more prevalent among whites. The prevalences of diabetes and impaired glucose tolerance ranged from 3.3% and 2.6% in the 30-39 year age group to 21.7% and 11.3% in the 60-69 year age group, respectively. Obese subjects (BMI > 30 kg/m²) and those with a family history of diabetes (first-degree relatives) presented higher prevalences of diabetes (22.6% and 19.7%, respectively). CONCLUSIONS: The prevalence of diabetes in Ribeirão Preto was found to be comparable to that occurring in developed countries. With respect to the Brazilian Multicenter Study we verified an increased prevalence of diabetes but a similar prevalence of impaired glucose tolerance. These findings may reflect modifications in environmental factors and lifestyle that have been occurring in Brazilian cities like Ribeirão Preto, especially regarding increasing rates of sedentary living and obesity.

CONTEXTO: A associação entre psoríase e hipoparatiroidismo tem sido descrita por vários autores, sendo sugerido que anormalidades na homeostase do cálcio possam estar envolvidas no desenvolvimento ou exacerbação da psoríase. Entretanto, até esse momento, há apenas dois relatos de pseudohipoparatiroidismo em associação com psoríase. OBJETIVO: Descrever pela primeira vez a ocorrência familiar dessa associação. RELATO DE CASO: Apresentamos dois irmãos com psoríase associada ao pseudo-hipoparatiroidismo. O primeiro é um paciente de 24 anos, masculino, branco, com psoríase pustulosa eritrodérmica disseminada, de início 2 meses antes da admissão. Tinha antecedentes de retardo neuropsicomotor, otites de repetição, convulsões e artralgias desde os 11 anos de idade. Apresentava fenótipo característico de Osteodistrofia de Albright - baixa estatura, obesidade, fácies arredondada, fronte ampla, pescoço curto, braquidactilia e sinais clínicos de hipocalcemia (adotava posição de flexão dos membros, tinha fraqueza muscular proximal e sinal de Trosseau positivo), hipocalcemia (cálcio ionizado de 0.69 mmol/l e total de 3,2 mg/dl), hiperfosfatemia (6,6 mg/dl), hipomagnesemia (1,0 mEq/l), hipoalbuminemia (3,1 g/dl), níveis séricos de PTH (molécula intacta) normais (45,1 pg/ml), hipotiroidismo primário (TSH de 12,2 mU/ml, e T4 total de 4,7 mg/dl), hipogonadismo hipergonadotrófico (LH de 116,0 ng/ml, FSH de 13,2 mU/ml e testosterona de 325,0 ng/dl), osteoporose, calcificações difusas em partes moles e no sistema nervoso central. O segundo caso é de uma paciente de 14 anos, feminina, branca, irmã do 1º paciente, com história de psoríase vulgar desde os 5 anos de idade e antecedentes de retardo neuro-psicomotor. Apresentava sinais da Osteodistrofia de Albright (baixa estatura, obesidade, arredondada, pescoço curto, braquidactilia), hipocalcemia (cálcio ionizado de 1,08 mmol/l e total de 6,7 mg/dl) hiperfosfatemia (9,4 mg/dl), níveis séricos de PTH elevados (222,0 pg/ml), osteoporose e hipogonadismo hipergonadotrófico (LH de 7,0 mU/ml, FSH de 9,3 mU/ml e níveis indetectáveis de estradiol).

CONTEXT: The association between psoriasis and hypoparathyroidism has been reported by several authors, and it has been suggested that abnormalities in calcium homeostasis may be involved in the development or exacerbation of psoriasis. However, so far there have only been two reports of pseudohypoparathyroidism associated with psoriasis. OBJECTIVE: To describe the familial occurrence of this association for the first time. CASE REPORTS: Two siblings with psoriasis associated with pseudohypoparathyroidism were presented. The first patient was a 24-year-old white male with disseminated erythrodermic pustular psoriasis that began 2 months before admission. He had had a history of mental retardation, recurrent otitis, seizures and arthralgia from the age of 11 years onwards. He presented the characteristic phenotype of Albright osteodystrophy: short stature, obesity, round facies, broad forehead, short neck and brachydactylia. He adopted a position of flexed limbs and showed proximal muscle weakness and a positive Trousseau sign. He had clinical signs of hypocalcemia (0.69 mmol/l ionized calcium and 3.2 mg/dl total calcium), hyperphosphatemia (6.6 mg/dl), hypomagnesemia (1.0 mEq/l), hypoalbuminemia (3.1 g/dl), normal serum intact PTH levels (45.1 pg/ml), primary hypothyroidism (13.2 mU/ml TSH, and 4.7 mg/dl total T4), hypergonadotrophic hypogonadism (116.0 ng/ml LH, 13.2 mU/ml FSH and 325.0 ng/dl testosterone), osteoporosis, and diffuse calcifications in soft tissues and in the central nervous system. The second case was a 14-year-old white girl with a history of psoriasis vulgaris from the age of five years onwards, and antecedents of mental retardation. She presented signs of Albright osteodystrophy (short stature, round facies, obesity, short neck, brachydactylia), hypocalcemia (ionized calcium of 1.08 mmol/l and total calcium of 6.7 mg/dl) hyperphosphatemia (9.4 mg/dl), elevated serum PTH levels (223.0 pg/ml), osteoporosis, and hypergonadotrophic hypogonadism (7.0 mU/ml LH, 9.3 mU/ml FSH and undetectable estradiol levels).

Objetivo: avaliar a relação entre o uso de sulfoniluréias (SF) por gestantes diabéticas seguidas na nossa clínica e as malformações (MF) fetais detectadas. Métodos: estudamos, retrospectivamente, 35 gestantes diabéticas tipo 2, seguidas no Ambulatório de Pré-Natal Patológico do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, USP, no período de 1993-1995, das quais 22 encontravam-se em uso inadvertido de SF no 1º trimestre da gestação (grupo SF). Avaliamos a prevalência de MF e comparamos com as gestantes diabéticas que nesse período encontravam-se em uso de insulina ou apenas dieta (grupo C). Foram também analisadas outras variáveis: tempo de doença, idade, controle metabólico e seguimento pré-natal. Resultados: não houve diferença estatisticamente significativa entre os 2 grupos quanto a faixa etária, tempo de evolução do diabetes, controle metabólico ou precocidade do início do pré-natal, sendo a freqüência de MF semelhante nos 2 grupos (8,3% no grupo C e 13,6% no grupo SF). As malformações encontradas foram: no grupo SF - agenesia renal, hipoplasia pulmonar e gônadas em fita (paciente 1); membros curtos e pododáctilos de implantação anormal (paciente 2); fenda palatina, implantação baixa de orelhas, pescoço alado, prega palmar única e nariz em sela (paciente 3), e no grupo C - micrognatia, orelha displásica, polidactilia, imperfuração anal, hipospadia, comunicação inter-atrial e comunicação interventricular (paciente 4). Conclusões: esses dados não nos permitem atribuir as malformações encontradas no grupo SF ao uso de sulfoniluréias, embora nesse grupo tenham ocorrido alterações não usualmente descritas na embriopatia diabética.

Purpose: to evaluate a possible relationship between fetal malformations (FM) and the use of sulfonylureas (SF) by diabetic pregnant women. Methods: we retrospectively studied 35 type 2 diabetic pregnant women followed at the Pathological Prenatal Care Outpatient Clinic of the University Hospital, Faculty of Medicine of Ribeirão Preto, from 1993 to 1995. Twenty-two of these women had been inadvertently using sulfonylureas during the 1st trimester of gestation (SF group). We determined their prevalence of FM and compared it to that observed for pregnant diabetic women who were only on diet or insulin therapy (group C). We also analyzed other variables such as time of disease, age, metabolic control, and prenatal care. Results: there was no significant difference between groups in terms of age range, duration of diabetes, glycemic control, or early start of prenatal care, with the prevalence of FM being similar for the two groups (8.3% in group C and 13.6% in group SF). The malformations observed in group SF were: renal agenesis, pulmonary hypoplasia and ribbon gonads (patient 1); short limbs and abnormally implanted toes (patient 2); cleft palate, low implanted ears, neck webbing and saddle nose (patient 3), and micrognathia, dysplastic ears, imperforate anus, hypospadia, polydactily, ventricular septal defect and atrial septal defect (patient 4) in group C. Conclusions: these data do not allow us to attribute the malformations detected in group SF to the use of sulfonylureas, although not usually described alterations in diabetic embryopathy occurred in this group.