Resumo As doenças renais genéticas raras compreendem mais de 150 desordens. Elas podem ser classificadas segundo achados diagnósticos como (i) distúrbios do crescimento e estrutura, (ii) doenças glomerulares, (iii) tubulares e (iv) metabólicas. Nos últimos anos, houve uma mudança de paradigma nesse campo. Os testes moleculares tornaram-se mais acessíveis, nossa compreensão sobre os mecanismos fisiopatológicos subjacentes a essas doenças evoluiu e novas estratégias terapêuticas foram propostas. Portanto, o papel do nefrologista mudou progressivamente de mero espectador a participante ativo, parte de uma equipe multidisciplinar, no diagnóstico e tratamento desses distúrbios. O presente artigo oferece um panorama geral dos recentes avanços a respeito dos distúrbios renais hereditários raros, discutindo aspectos genéticos, manifestações clínicas e abordagens diagnósticas e terapêuticas de alguns desses distúrbios, mais especificamente a glomeruloesclerose segmentar e focal familiar, complexo da esclerose tuberosa, nefropatia de Fabry e doença relacionada ao MYH9.

Abstract There are more than 150 different rare genetic kidney diseases. They can be classified according to diagnostic findings as (i) disorders of growth and structure, (ii) glomerular diseases, (iii) tubular, and (iv) metabolic diseases. In recent years, there has been a shift of paradigm in this field. Molecular testing has become more accessible, our understanding of the underlying pathophysiologic mechanisms of these diseases has evolved, and new therapeutic strategies have become more available. Therefore, the role of nephrologists has progressively shifted from a mere spectator to an active player, part of a multidisciplinary team in the diagnosis and treatment of these disorders. This article provides an overview of the recent advances in rare hereditary kidney disorders by discussing the genetic aspects, clinical manifestations, diagnostic, and therapeutic approaches of some of these disorders, named familial focal and segmental glomerulosclerosis, tuberous sclerosis complex, Fabry nephropathy, and MYH-9 related disorder.

RESUMO A doença relacionada ao MYH9 é um distúrbio autossômico dominante causado por mutações no gene MYH9 que codifica a cadeia pesada da miosina não muscular IIA no cromossomo 22q12. Ela é caracterizada por macrotrombocitopenia congênita, tendência a sangramento, perda auditiva e catarata. A nefropatia ocorre em aproximadamente 30% dos pacientes. O presente artigo relata o caso de um paciente com doença relacionada ao MYH9 portador de mutação missense de novo no exon 1 do gene MYH9 [c.287C > T; p.Ser(TCG)96(TTG)Leu]. Com a exceção de catarata, o paciente apresentou todas as manifestações fenotípicas da doença. As alterações renais incluíram micro-hematúria, proteinúria nefrótica (até 7,5 g/24h) e perda rápida da função renal. O declínio anual da taxa de filtração glomerular foi de 20 mL/min/1,73 m2 durante cinco anos. Foi receitado bloqueio do sistema renina-angiotensina, a única terapia recomendada para retardar a progressão dessa nefropatia. Embora a doença relacionada ao MYH9 seja uma causa rara de glomerulopatia e doença renal terminal, a conscientização sobre distúrbios genéticos renais raros é essencial para garantir o diagnóstico preciso e o manejo adequado dos pacientes com tal doença órfã.

ABSTRACT MYH9-related disease is an autosomal dominant disorder caused by mutations of the MYH9 gene, which encodes the non-muscle myosin heavy chain IIA on chromosome 22q12. It is characterized by congenital macrothrombocytopenia, bleeding tendency, hearing loss, and cataracts. Nephropathy occurs in approximately 30% of MYH9-related disease in a male patient carrier of a de novo missense mutation in exon 1 of the MYH9 gene [c.287C > T; p.Ser(TCG)96(TTG)Leu]. He presented all phenotypic manifestations of the disease, but cataracts. Renal alterations were microhematuria, nephrotic-range proteinuria (up to 7.5 g/24h), and rapid loss of renal function. The decline per year of the glomerular filtration rate was 20 mL/min/1.73m2 for five years. Blockade of the renin-angiotensin system, the only recommended therapy for slowing the progression of this nephropathy, was prescribed. Although MYH9-related disease is a rare cause of glomerulopathy and end-stage renal disease, awareness of rare genetic kidney disorders is essential to ensure accurate diagnosis and proper management of orphan disease patients.

Resumo A prevalência da nefrolitíase está aumentando em todo o mundo e resulta em ônus significativo para o sistema de saúde. Novos estudos revelam que a formação de cálculos urinários está associada a várias morbidades graves. No entanto, poucos estudos observacionais ou ensaios clínicos randomizados de qualidade demonstraram que intervenções clínicas específicas diminuem a recorrência da nefrolitíase. Portanto, nesta revisão são analisadas as evidências disponíveis da terapia médica expulsiva para cálculos ureterais; avaliam-se os dados da terapêutica não farmacológica, incluindo modificações dietéticas e terapia à base de sucos cítricos; e discute-se a eficácia dos diuréticos tiazídicos no tratamento da hipercalciúria associada à nefrolitíase recorrente.

Abstract The prevalence of kidney stone disease is increasing worldwide with significant health and economic burden. Newer research is finding that stones are associated with several serious morbidities. Yet, few randomized clinical trials or high quality observational studies have assessed whether clinical interventions decrease the recurrence of kidney stones. Therefore, in this review we analyze the available evidence on medical expulsive therapy for ureteral stones; describe the evidence about non-pharmacological stone therapy including dietary modifications and citrus juice-based therapy; and discuss the efficacy of thiazide diuretics for the treatment of hypercalciuria in recurrent nephrolithiasis.

Objective: To measure length of hospital stay (LHS) in patients receiving medication reconciliation. Secondary characteristics included analysis of number of preadmission medications, medications prescribed at admission, number of discrepancies, and pharmacists interventions done and accepted by the attending physician. Methods: A 6 month, randomized, controlled trial conducted at a public teaching hospital in southern Brazil. Patients admitted to general wards were randomized to receive usual care or medication reconciliation, performed within the first 72 hours of hospital admission. Results: The randomization process assigned 68 patients to UC and 65 to MR. LHS was 10±15 days in usual care and 9±16 days in medication reconciliation (p=0.620). The total number of discrepancies was 327 in the medication reconciliation group, comprising 52.6% of unintentional discrepancies. Physicians accepted approximately 75.0% of the interventions. Conclusion: These results highlight weakness at patient transition care levels in a public teaching hospital. LHS, the primary outcome, should be further investigated in larger studies. Medication reconciliation was well accepted by physicians and it is a useful tool to find and correct discrepancies, minimizing the risk of adverse drug events and improving patient safety.

Objective: to evaluate protein intake, nitrogen balance and nutritional status of clinically stable patients with Parkinson's disease (PD). Methods: a cross-sectional study of PD patients Hoehn- Yahr scale stage 1-3 and subjects with no neurologic disease (controls) matched for age and gender. All participants underwent a diet history interview, anthropometric measurements, bioelectrical impedance and food record over three non-consecutive days, including a weekend. A 24-hour urine collection and fasting venous blood sampling were collected from the participants for evaluation of creatinine clearance, creatinine height index and the nitrogen balance. Results: the mean age of PD patients was 58.9 ± 12.8 year compared to 54.7 ± 12.6 year of the controls, P = 0.34. One third of PD group had symptoms of dysphagia and ingested less water and fibers when compared to controls. Calf circumference was small in PD group (35.5 ± 2.8 vs. 38.4 ± 3.5 cm, P = 0.012). Intake of nitrogen was significantly lower and nitrogen balance was negative in PD patients (-1.8 ± 3.9 vs. 1.1 ± 4.2 controls, P = 0.06). The antioxidants folate and vitamin E were consumed in small amounts in both groups, although significantly less in PD patients (P = 0.04 and 0.03, respectively). Discussion: daily intakes of protein of approximately 1.1 g/kg by clinically stable PD patients may not be enough to ensure a neutral calorie-nitrogen balance and muscle tissue conservation. Larger studies are necessary to provide a more comprehensive picture of PD patients' metabolic status.

Objetivo: evaluar ingestión proteica, balance nitrogenado y estado nutricional de pacientes con enfermedad de Parkinson (EP) clínicamente estables. Métodos: estudio transversal de pacientes con EP en los niveles 1-3 según la escala de Hoehn-Yahr e individuos sin enfermedad neurológica (controles), pareados por edad y género. Todos los participantes fueron sometidos a una entrevista de la historia nutricional, antropometría, impedancia eléctrica y registro alimentario de 3 días consecutivos, incluyendo un fin de semana. Fueron colectados sangre venosa en ayuno y orina de 24 horas para evaluación de la depuración de la creatinina, índice creatinina-altura y balance nitrogenado. Resultados: el promedio de edad en pacientes con EP fue 58,9 ± 12,8 años en comparación con 54,7 ± 12,6 años de los controles, p = 0,345. Un tercio del grupo EP tuvo síntomas de disfagia, con menor ingestión de agua y fibras, comparados a los controles. La circunferencia de la pantorrilla fue menor en grupo EP (35,5 ± 2,8 vs. 38,4 ± 3,5 cm, p = 0,012). La ingestión de nitrógeno fue significativamente menor y el balance de nitrógeno fue negativo en grupo EP (-1,8 ± 3,9 vs. 1,1 ± 4,2 controles, p = 0,064). Los antioxidantes folato y vitamina E fueron consumidos en pequeñas cantidades en ambos grupos, aunque significativamente menor en los pacientes con EP (p = 0,042 y 0,031, respectivamente). Discusión:: la ingestión proteica diaria de aproximadamente 1,1 g/kg en pacientes clínicamente estables con EP puede no ser suficiente para garantizar un balance neutro de calorías-nitrógeno, así como para mantener la masa muscular. Serán necesarios mayores estudios que produzcan una imagen más completa del estado metabólico de los pacientes con Parkinson.

Introdução: A nefrolitíase é uma patologia frequente, com alta taxa de prevalência e recorrência, ocorrendo por processo multifatorial e complexo. Objetivo: Analisar as principais características dietéticas e metabólicas de pacientes com nefrolitíase e compará-los com grupo controle. Métodos: Estudo observacional, transversal, com 31 pacientes com nefrolitíase (NE) e 18 saudáveis. Na ingestão dietética, foram verificados sódio, cálcio, proteína, potássio, vitamina C, oxalato e a ingestão hídrica em ambos os grupos. Na avaliação metabólica, foi analisada excreção urinária de citrato e oxalato. Também foi avaliada presença de hipertensão arterial e Índice de Massa Corporal (IMC). Resultados: Quanto ao grupo NE, verificou-se que 45,2% apresentou alta ingestão de sódio e 100% de oxalato. Foi também observada baixa ingestão de cálcio em 93,5%, potássio em 100% e vitamina C em 94,9%. Com relação à proteína, apenas 12,5% apresentou ingestão normoproteica. Quanto à ingestão hídrica, 12,9% apresentou ingestão menor que 1 litro, 54,8% entre 1 a 2 litros, e 32,3% maior que 2 litros. Foi observada hipertensão arterial sistêmica em 64,5% desses pacientes e excreção adequada de citrato e oxalato em 90,5% deles. Não foi verificada diferença estatística significativa na ingestão alimentar, IMC, e excreção de oxalato entre os grupos. No entanto, o grupo NE apresentou maior excreção de citrato. Conclusão: Verificou-se nos dois grupos elevada prevalência de pacientes com sobrepeso, alta ingestão de oxalato e sódio, além de inadequação nas ingestões de cálcio, potássio e vitamina C. No grupo NE, foi observada alta ingestão proteica e maior excreção de citrato.

Introduction: Nephrolithiasis is a common condition with high prevalence and recurrence, occuring by a complex and multifactorial process. Objective: To analyze the main dietary and metabolic characteristics of patients with nephrolithiasis and compare them with a control group. Methods: A crosssectional study with 31 patients with nephrolithiasis (NE) and 18 healthy. By the dietary intake it were observed sodium, calcium, protein, potassium, vitamin C, oxalate and water intake in both groups. Metabolic assessment were analyzed in urinary excretion of oxalate and citrate. The presence of hypertension and body mass index (BMI) was also evaluated. Results: In the NE group, it was found that 45.2% had a high intake of sodium and 100% a high intake of oxalate. It was also observed a low calcium, potassium and vitamin C intake by 93.5%, 100% and 94.9% respectively. Regarding protein, only 12.5% had normal protein intake. Concerning water intake, 12.9% had an ingestion less than 1 liter, 54.8% between 1 and 2 liters and 32.3% higher than 2 liters. Hypertension was observed in 64.5% of patients and adequate excretion of oxalate and citrate in 90.5% of them. There was no statistically difference in food intake, BMI and oxalate excretion between groups. However, the NE group showed higher urinary citrate. Conclusion: It was found in both groups a high prevalence of overweight patients, a high intake of oxalate and sodium, in addition to inadequate intakes of calcium, potassium and vitamin C. The NE group showed high protein intake and increased excretion of citrate.

OBJETIVO: Caracterizar o perfil dos atendimentos pediátricos por trauma e verificar a chance de ocorrência de trauma em uma rodovia de alto fluxo de veículos. MÉTODOS: Estudo de coorte descritivo dos dados armazenados nos boletins de atendimentos realizados por grupo de resgate pré-hospitalar da Ponte Rio-Niterói, uma via de grande tráfego, no Rio de Janeiro. Estatística descritiva foi aplicada utilizando-se a frequência simples e percentual, verificando-se o risco estimado de "trauma" em crianças < 12 anos, por meio de odds ratio, com intervalo de confiança de 95%. RESULTADOS: Em 1.244 atendimentos, entre março de 2002 e março de 2003, trauma representou 41,31% (n = 514), 76% do sexo masculino dos casos. O atendimento a crianças de até 12 anos correspondeu a 4,18% (n = 52) e metade das vítimas tinha entre 6 e 12 anos (n = 26), sendo 29 (55,76%) do sexo masculino. Entre as 52 vítimas, 71,15% (n = 37) estavam envolvidas em acidente de trânsito (OR: 3,70; IC95%: 1,94-7,13; p < 0,0001), sendo 28 (75,67%) colisões. Os locais mais frequentes de lesões foram extremidades (n = 12; 32,43%), face (n = 10; 27,02%) e cabeça (n = 9; 24,32%). Os procedimentos pré-hospitalares foram realizados em 23 dos 37 pacientes (62,16%) e 44,23% (n = 23/52) necessitaram de remoção hospitalar. Não houve óbito pediátrico durante o período analisado. CONCLUSÕES: Neste estudo as crianças apresentaram maior probabilidade de serem tratadas para traumatismo nessa rodovia de intenso fluxo de veículos. Os locais mais frequentes de lesões foram membros inferiores e o complexo cabeça, face e pescoço.

OBJECTIVE: To outline a profile of pediatric trauma victims and verify the likelihood of trauma in children on a high traffic roadway. METHODS: A descriptive cohort study of the records of emergency medical service activations on the Rio-Niterói Bridge, a high traffic roadway in Rio de Janeiro, Brazil. Descriptive statistics were expressed as absolute and relative frequencies. The estimated risk of trauma in children aged < 12 years was calculated by means of odds ratios, with a 95% confidence interval. RESULTS: Trauma accounted for 514 of 1,244 activations (41.31%) of the Rio-Niterói Bridge emergency medical service between March 2002 and March 2003. Response to incidents involving children aged < 12 years accounted for 52 of these (4.18%). Half of victims were between the ages of 6 and 12 years (n = 26), and 55.76% were male (n = 29). Of the 52 victims, 37 (71.15%) were involved in motor vehicle accidents (OR: 3.70; 95%CI: 1.94-7.13; p < 0.0001). Of these, 28 were vehicle-vehicle collisions (75.67%). The most common sites of injury were the extremities (n = 12; 32.43%), face (n = 10; 27.02%), and head (n = 9; 24.32%). Pre-hospital procedures were performed on 23 of the 37 patients (62.16%), and 44.23% (n = 23/52) required hospital transportation. There were no deaths during the study period. CONCLUSIONS: In this study, children were at significantly higher odds of being treated for trauma while on a highway with heavy traffic flow. The most common sites of injury in this sample were the lower extremities and the head, face, and neck complex.

OBJETIVO: Analisar a prevalência de hipercalciúria em mulheres na pós-menopausa com osteoporose e avaliar associação de hipercalciúria com parâmetros clínicos e do metabolismo osteomineral. SUJEITOS E MÉTODOS: Foram estudadas 127 mulheres. A calciúria foi determinada em urina de 24 horas. A DMO foi medida em coluna lombar e fêmur por dupla emissão de energia (DXA). RESULTADOS: A média de idade foi de 64 ± 8 anos. De acordo com a excreção urinária de cálcio, as pacientes foram divididas em normo e hipercalciúricas (HC). Das 127 pacientes, 19 (15%) foram classificadas como HC. Houve diferença entre os grupos apenas na idade de início da menopausa (46 ± 6 vs. 50 ± 3 anos HC, p < 0,0005). Não houve associação entre calciúria e idade, IMC, DMO, cálcio, fósforo, PTH e fosfatase alcalina. CONCLUSÃO: A HC é frequente em mulheres na pós-menopausa com osteoporose, e a medida da calciúria deve ser incluída na investigação dessas pacientes.

OBJECTIVE: To determine the prevalence of hypercalciuria (HC) in postmenopausal women with osteoporosis and its relationship with clinical data and bone mineral metabolism. SUBJECTS AND METHODS: Calciuria was measured in 24-hour urine samples of 127 women. BMD was measured in the lumbar spine and femur by dual-energy X-ray absorptiometry (DXA). RESULTS: Mean age (±SD) was 64 (±8) years. According to urinary calcium excretion, patients were divided into normo- and hypercalciuric (HC). Of the 127 patients, 19 (15%) were classified as HC. The only difference between the groups was the age of onset of menopause (46 ± 6 vs. 50 ± 3 years HC, p < 0.0005). No association was found between calciuria and age, BMI, BMD, calcium, phosphorus, PTH, and alkaline phosphatase. CONCLUSION: HC is frequent in postmenopausal women with osteoporosis, and calciuria measurement should be included in the investigation of these patients.

A avaliação da resistência à ação da proteína C ativada (rPCA), causada por mutação no fator V (fator V de Leiden), é fator de risco importante para tromboembolia venosa, cujo papel como geradora de obstruções arteriais in situ é um tema ainda controverso. O caso clínico de um jovem com história de coronariopatia, múltiplas lesões cerebrovasculares e doença arterial periférica é relatado. A investigação diagnóstica apontou a rPCA como possível etiologia.

The assessment of activated protein C resistance (APCR) caused by mutations in factor V (factor V Leiden) is an important risk factor for venous thromboembolism, of which role as the originator of arterial obstructions in situ is still a controversial subject. The clinical case of a young patient with history of coronariopathy, multiple cerebrovascular lesions and peripheral artery disease is reported. The diagnostic investigation showed APCR as the possible etiology.

A terapêutica com drogas antitireoidianas pode ser acompanhada de efeitos colaterais. Propiltiouracil (PTU) pode induzir vasculites anticorpo anti-citoplasma de neutrófilos (ANCA) positivas, na maioria das vezes relacionadas ao subtipo mieloperoxidase (ANCA-MPO). O nosso objetivo é relatar o caso de uma paciente com doença de Graves que desenvolveu auto-imunidade induzida por PTU, com manifestações cutâneas, pulmonares e renais, associadas à positividade do ANCA. O exame anátomo-patológico pulmonar revelou hemorragia difusa e a biópsia renal demonstrou glomeruloesclerose segmentar e focal. Foi tratada com pulsoterapia com corticóides e ciclofosfamida, com boa evolução clínica. Este caso enfatiza a necessidade de detecção e tratamento precoce deste efeito adverso relativamente raro do PTU.

Antithyroid drugs sometimes cause severe complications. Propylthiouracil (PTU) can be associated to ANCA positive vasculitis, most often related to myeloperoxidase subtype (ANCA-MPO). Our objective is to describe a female patient with Graves' disease, who developed PTU induced-autoimmune disease, with cutaneous, pulmonary, and renal lesions, associated with ANCA. Histopathological examination revealed diffuse pulmonary hemorrhage, and focal segmental glomerulosclerosis at the kidney biopsy. She was treated with systemic corticosteroid therapy and cyclophosphamide, with clinical improvement. This case highlights the need for greater awareness of this relatively rare adverse effect of propylthiouracil.

INTRODUCTION: Nephrocalcin (NC) is a glycoprotein produced in the kidney and inhibits calcium oxalate crystal formation. It has been separated into 4 isoforms (A, B, C, and D) and found that (A + B) are more abundant than (C + D) in urine of healthy subjects, but the reverse is seen in human urine of kidney stone patients. To further examine the role of this protein in inhibition of urinary crystallization, nephrocalcin isoforms were purified from 2 genetically pure dog species. MATERIALS AND METHODS: We studied healthy Beagles, known to be non-stone forming dogs, and Mini-Schnauzers, known to be calcium oxalate stone formers. NC was isolated and purified from each group. Urinary biochemistry and calcium oxalate crystal growth inhibition were measured. RESULTS: Specific crystal growth inhibition activity was significantly higher in non-stone forming dogs (9.79 ± 2.25 in Beagles vs. 2.75 ± 1.34 of Mini-Schnauzers, p < 0.005). Dissociation constants toward calcium oxalate monohydrate were 10-fold different, with Beagles' isoforms being 10 times stronger inhibitors compare to those of Mini-Schnauzers'. Isoforms C + D of NC were the main isoforms isolated in stone-forming dogs. CONCLUSION: NC of these two species of dogs differently affects calcium oxalate crystallization and might have a role in determining ulterior urinary stone formation.

OBJETIVO: Desenvolver um modelo experimental de endo-sutura manual laparoscópica em cólon destinado principalmente ao treinamento de cirurgiões. MÉTODOS: Foram operados 40 cães mestiços, machos, com peso entre 15 e 20 kg, provenientes do laboratório da Faculdade de Veterinária da Universidade Estadual do Ceará. Foram distribuídos aleatoriamente em dois grupos de 20 animais cada: GI-secção e hemostasia do colon com bisturi elétrico e GII-secção do colon com tesoura. Cada grupo foi subdividido em dois subgrupos com 10 animais cada, conforme o acesso abdominal utilizado - A-acesso laparotômico e B-laparoscópico. Sob anestesia geral endovenosa, foi realizada incisão transversal no cólon sigmóide, numa extensão de 50% da circunferência, distando 15 cm da reflexão peritoneal, sendo em seguida suturada em plano único, extra-mucoso, com pontos separados de fio de polidioxanona 000 (PDS®). Os animais foram avaliados quanto à evolução clínica, análise macroscópica, teste de tensão da sutura e estudo histológico qualitativo. Foram sacrificados no 7º dia de pós-operatório. Para analise estatística, foram utilizados os testes Qui-quadrado corrigido de Yates e o teste exato de Fisher. RESULTADOS: Todos os animais cuja colotomia foi realizada com tesoura (GIIA IIB) e 9 operados com bísturi elétrico (GI), sendo 5 (50,0%) operados pelo acesso laparotômico (GIA) e 4 (40,0%) pelo laparoscópico (GIB), apresentaram evolução clínica satisfatória, deambulando e aceitando bem a dieta oral a partir do primeiro dia de pós-operatório. A primeira evacuação ocorreu entre 48 a 72 horas de pós-operatório. Não apresentaram diarréia nem vômitos. Onze animais do grupo GI, sendo 5 (50%) do subgrupo IA e 6 (60,0%) do IB não aceitaram bem a dieta oral, apresentando diarréia (3 a 5 evacuações líquidas por dia) e vômitos (1 a 3 episódios por dia) evoluindo para óbito entre o quarto e o sétimo dia do pós-operatório. Comparando os grupos GI com GII, foi observada diferença significante (p<0,005), sem no entanto observar-se diferença quando comparados os subgrupos IA e IB. A sutura permaneceu íntegra em todos (100,0 %) os animais GII e em 5 GI, demonstrando portanto diferença estatisticamente significante (p<0,005). Desses, 3 (30,0%) eram do subgrupo IA e 2 (20,0%) do IB. Foi evidenciada sutura bloqueada com epíplon em quatro animais, sendo dois (20%) do subgrupo IA e dois (20%) do IB. Houve deiscência da sutura com peritonite em 11 (55%) animais do grupo I (p<0,005), sendo 5 (50%) do subgrupo IA e 6 (60%) do IB (p>0,005). Todos evoluíram para óbito entre o quarto e o sétimo d.p.o. O teste de tensão da sutura foi realizado com a pressão média de 222,1 mmHg e não ocorreu ruptura do cólon em nenhum dos animais do grupo II e em 5 (40,0% ) do Grupo I (p<0,005). Desses, 3 (30%) eram do subgrupo IA, e 2 (20,0%) do IB (P>0,005). Ocorreu ruptura da sutura em 4 ( 20% ) cães do grupo I, sendo 2 do subgrupo IA e 2 (20%) do IB com pressão média de 94,0 mmHg. A análise histológica das amostras obtidas no 7º d.p.o. demonstrou reprodução das fases do processo inflamação-reparação em ambos acessos utilizados. CONCLUSÃO: A endo-sutura manual laparoscópica em cólon de cães pode ser utilizada com segurança pois apresentou os mesmos resultados do acesso laparotômico. Os resultados dependem especialmente do treinamento adequado do cirurgião em técnicas cirúrgicas laparoscópicas.

PURPOSE: Develop an experimental model of laparoscopic hand-sewn suture in colon of dogs to be specially used for surgeons' trainning. METHODS: Forty male dogs were operated on, weight between 15 and 20 kg, from the laboratory of the Veterinary School of the State University of Ceara. They were distributed within two groups with 20 animals each: GI- The colonic wall incision was done with an electrical scapel followed by haemosthasia and GII- The colonic wall incision was performed with a scissors. Each group was shared in two other groups with 10 animals each, according to the abdominal approach used. A- Laparotomic approach and B-Laparoscopic approach. Under intra-venous general anesthesia, a transverse incision was performed envolving 50% of the sigmoid wall, 15cm far from the pelvic peritoneal pouch, followed by an extra-mucosa, one layer and interrupted suture with 000 polidioxanona (PDSâ). The animals were evaluated concerning to the clinical recovery, macroscopic appearance, suture-tension test and histologic study. The animals were sacrified on the 7th postoperative day. Fisher and Qui-square tests were used for statistical analysis. RESULTS: All the animals submitted to colotomy performed by scissor (GIIA, GIIB) and nine animals operated on with an electrical scapel (GI), five (50,0%) by laparotomic approach (GIA) and four (40,0%) laparoscopically (GIB) had a satisfactory clinical recovery, walking and accepting oral diet on the first post-operative day. The first bowel movement occurred between 48 and 72 hours. It didn't occur diarrhea or vomits. Eleven animals from group I, five (50%) from group IA and six (60,0%) from group IB didn't accept oral diet and complained of diarrhea (3 to 5 evacuations each day), 1 to 3 episodes of vomits each day and they die between the fourth and seventh postoperative day. Comparing groups GI with GII, a statistical significant difference was observed (p<0,005). There was no difference between GIA and GIB. Colonic sutures were intact in all the animals from group II and in five from group I with statistical significant difference (p<0,005). Three (30,0%) were from group IA and two (20,0%) from group IB. The colonic suture was envolved by epiplon in four animals, two (20%) from GIA and 2 (20%) from GIB. Suture dehiscence with peritonitis occurred in eleven (55%) animals from GI (p<0,005), five (50%) from GIA and six (60%) from GIB (p>0,005). All the animals died between the fourth and seventh postoperative day. The tension suture test was performed with an average pressure of 222,1 mmHg and there was no colonic suture disruption in any animal from the group II and in five (40,0% ) from the group I (p<0,005). Three (30%) animals were from GIA and two (20,0%) from GIB (P>0,005). Colonic suture rupture occurred in four (20% ) dogs from group I, two from GIA and two (20%) from GIB with an average pressure of 94,0 mmHg. The histological analysis of the surgical specimens removed on the 7th postoperative day demonstrated the same level of the inflamatory process in both approach used. CONCLUSION: The handsewn laparoscopic colonic suture in dogs can be safely performed, showing the same results of the laparotomic approach. The surgical results depend specially on the adequate surgeon's trainning in laparoscopic surgical technique.

PURPOSE: To study the changes in calcium oxalate crystal morphology induced by different levels of supersaturation (SS) in human urine. MATERIALS AND METHODS: Twenty-four hours urine samples from 5 normal men were collected. Each specimen was centrifuged and filtered. About 200 mL of each sample was dialyzed overnight. Aliquots of 2 mL of urine was then added to a 24-wells tissue culture plate and checked for crystal absence. Calcium oxalate crystals were precipitated from each sample by adding sodium oxalate and calcium chloride in sufficient quantities to induce spontaneous crystallization. Finally, each plate hole was examined with an inverted polarized microscope (X500 magnification). Initial SS of each sample relative to calcium oxalate was calculated using an iterative computer program. RESULTS: Crystal formation was connecte to relative calcium oxalate (CaOx) SS. At SS of 10, small crystals of similar shape were formed, mainly CaOx dihydrate morphology. At SS of 30, there was an enormous increase in the number of crystals, that kept the same size. SS greater than 50 produced larger crystals with different shapes and multiple crystalline aggregates. Urine was able to tolerate, i.e., to avoid crystal formation, until SS ratios of approximately 10. CONCLUSIONS: Relative CaOx SS and the concentration ratio of calcium to oxalate are important determinanting factors of crystal morphology. Non-dialyzable urinary proteins can act as inhibitors and influence the structure of formed crystals. Additional studies from patients with kidney stones are needed in order to establish whether crystal size and habit distribution are different from crystals in normal urine.

O estado de mal epiléptico causa morbidade e mortalidade significativas. Relatamos um caso de mal convulsivo generalizado, associado à aspiração pulmonar maciça, insuficiência respiratória aguda e diabetes insípido central transitório. As convulsões foram rapidamente controladas, porém o paciente necessitou de ventilação mecânica e correção da poliúria com acetato de desmopressina. Durante a hospitalização o nível de consciência normalizou-se, houve remissão espontânea do diabetes insípido e o paciente recebeu alta sem seqüelas neurológicas. São discutidos aspectos clínicos e patogênicos desta condição clínica.

Status epilepticus causes significant morbidity and mortality. A case of generalized status epilepticus followed by massive pulmonary aspiration, acute respiratory failure and transient central diabetes insipidus is presented. Seizures were promptly controlled, but the patient required mechanical ventilation and correction of polyuria with desmopressin acetate. During hospitalization mental status improved, diabetes insipidus spontaneously remitted and he was discharged without neurologic sequelae. The clinical and pathophysiological features of this case are discussed.