Generalidades de un paciente afebril con hepatoesplenomegalia en pediatría / Overview of an afebrile patient with hepatosplenomegaly in pediatrics

Introducción: La hepatoesplenomegalia es el agrandamiento simultaneo del hígado y del bazo, aunque es frecuente en edad pediátrica su literatura se centra en causa infecciosa, siendo también importante otras causas sobre todo en el paciente afebril Método: revisión de literatura actual confrontando con artículos de revisiones de temas en búsqueda electrónica en bases de datos de RIMA, MEDLINE, PUB-MED, MEDSCAPE, de 1981 a 2018. Resultados: La hepatomegalia en paciente afebril se puede encontrar en afectación sistémica o enfermedades hereditarias, algunas prevalentes o extremadamente raras, por lo cual se desarrolló esta revisión para agrupar las causas de esta en un paciente pediátrico afebril.

Introduction: Hepatosplenomegaly is the simultaneous enlargement of the liver and spleen, although it is frequent in pediatric age, its literature focuses on an infectious cause, and other causes are important, especially in the afebrile patient. Method: review of current literature comparing articles from subject reviews. in electronic search in RIMA, MEDLINE, PUB-MED, MEDSCAPE databases, from 1981 to 2018. Results: Hepatomegaly in afebrile patient can be found in systemic involvement or hereditary diseases, some prevalent or extremely rare, for which reason developed this review to group the causes of this in an afebrile pediatric patient.

Lysosomal acid lipase deficiency in Brazilian children: a case series / Deficiência de lipase ácida lisossomal em crianças brasileiras: casuística

Abstract Objective: To describe the demographic, clinical, laboratory and molecular characteristics of patients with lysosomal acid lipase deficiency. Methods: A retrospective review of the medical records of children with the disease. Results: Seven children with lysosomal acid lipase deficiency (5 male; 2 female); 6 were mixed race, and 1 was black. The mean ages at the first onset of symptoms and at diagnosis were 5.0 years (4 months to 9 years) and 6.9 years (3-10 years), respectively. Symptom manifestations at onset were: 3 patients had abdominal pain, one had bone/joint pain due to rickets, and 1 had chronic diarrhea and respiratory insufficiency due to interstitial pneumonitis. One was asymptomatic, and clinical suspicion arose due to hepatomegaly. Six patients had hepatomegaly, and none had splenomegaly. Two patients were siblings. Enzymatic assay and molecular analysis confirmed the diagnoses. Genetic analysis revealed a rare pathogenic variant (p.L89P) in three patients, described only once in medical literature and never described in Brazil. None of those patients were related to each other. Lysosomal acid lipase deficiency was previously described as an autosomal recessive disease, but three patients were heterozygous and undoubtedly had the disease (low enzyme activity, suggestive lab findings and clinical symptoms). Conclusion: This case series supports that lysosomal acid lipase deficiency can present with highly heterogeneous signs and symptoms among patients, but it should be considered in children presenting with gastrointestinal symptoms associated with dyslipidemia. We describe a rare variant in three non-related patients that may suggest a Brazilian genotype for lysosomal acid lipase deficiency.

Resumo: Objetivo: Descrever as características demográficas, clínicas, laboratoriais e moleculares de pacientes com deficiência de lipase ácida lisossomal. Métodos: Análise retrospectiva dos prontuários médicos de crianças com a deficiência de lipase ácida lisossomal. Resultados: Sete crianças com deficiência de lipase ácida lisossomal (5 M:2F); seis eram pardas e uma negra. As faixas etárias no início dos sintomas e no diagnóstico foram 5 anos (4 meses a 9 anos) e 6,9 anos (3 a 10 anos), respectivamente. As manifestações dos sintomas no início foram as que seguem: três pacientes apresentaram dor abdominal, um apresentou dor nos ossos/articulações devido a raquitismo e um apresentou diarreia crônica e insuficiência respiratória devido à pneumonite intersticial. Os outros não apresentaram sintomas e a suspeita clínica surgiu devido à hepatomegalia. Seis pacientes apresentaram hepatomegalia e um apresentou esplenomegalia. Dois pacientes eram irmãos. O ensaio enzimético e a análise molecular confirmaram os diagnósticos. A análise genética revelou uma variante patogênica rara (p.L89P) em três pacientes, descrita uma única vez na literatura médica e nunca descrita no Brasil. Nenhum desses pacientes tinha parentesco com os outros. A deficiência de lipase ácida lisossomal foi anteriormente descrita como uma doença recessiva autossômica, porém três pacientes eram heterozigotos e, sem dúvida, apresentaram a doença (atividade enzimática baixa, achados laboratoriais sugestivos e sintomas clínicos). Conclusão: Esta casuística afirma que a deficiência de lipase ácida lisossomal pode se manifestar com sinais e sintomas altamente heterogêneos entre os pacientes, porém deve ser considerada em crianças que apresentam sintomas gastrointestinais associados à dislipidemia. Descrevemos uma variante rara em três pacientes não relacionados que pode sugerir um genótipo brasileiro para deficiência de lipase ácida lisossomal.

Hematopoyesis extramedular en paciente con esferocitosis hereditaria asintomática / Extramedullary hematopoiesis in patients with asymptomatic hereditry spherocytosis

En diversas patologías con alteraciones en la producción de los elementos formes de la sangre pueden desarrollarse focos de hematopoyesis extramedular en diferentes sitios. Los más frecuentes son: bazo, hígado, ganglios linfáticos, y más raramente otros órganos como: glándulas adrenales, hillios renales, cartílagos, ligamentos, tejido adiposo, timo, pulmón, mediastino y duramadre de cráneo y columna. Generalmente el proceso es difuso pero pueden formarse grandes tumores de tejido hematopoyético. Las condiciones patológicas de la médula ósea más frecuentemente asociadas a hematopoyesis extramedular son: esferocitosis hereditaria, talasemia, síndromes mieloproliferativos con fibrosis medular, ocupación medular por patologías neoplásicas. presentamos un paciente de 45 años con esferocitosis hereditaria con masas de tejido hematopoyético extramedular paravertebral mediastinal que respondieron favorablemente a la esplenectomía.

Hereditary spherocytosis (HS) is a relatively common inherited hemolytic disorder in northern Europe and in the US. The reported prevalence of HS in Western countries is 1:5000. We describe a patient 45 years old, with hereditary spherocytosis with masses of mediastinal paravertebral extramedullary hematopoietic tissue, with a favorable response to splenectomy. The medical lieterature refers some cases of extramedullary hematopoiesis as a clinical expression of hereditary spherocytosis, mainly as thoracic masses with usually paravertebral localization. HS should be distinguished from other spherocytic hermolytic anemias. Diagnosis is usually made uring infancy or in young adults, but it can be at any moment of their life, until the seventh decade of life. Ocasionally, the diagnosis is first made in old age. The clinical expression of HS is highly variable, ranging from asymptomatic condition to a severe life-threatening hemolytic anemia. Laboratory features include spherocytosis, osmotic fragility, manifestations of hemolytic disease, elevated unconjugated bilirubin and reticulocytosis. The principal diagnostic test, RBC osmotic fragility, measures the surface/volume Ratio of the cells. The treatment of choice of HS in patients with inherited spherocytosis is splenectomy, which corrects hemolytic anemia. According to he literature, cases of failure following splenectomy have been reported.

Strong correlation by ultrasonography of hepatomegaly and the presence of co-infection in HIV/HCV cirrhotic patients

OBJECTIVES: Progression of hepatic fibrosis is accelerated in patients co-infected with human immunodeficiency virus and hepatitis C virus compared to hepatitis C virus mono-infected patients. This study aimed to compare ultrasound features and selected clinical and biochemical variables between patients with human immunodeficiency virus/hepatitis C virus co-infection (n = 16) versus hepatitis C virus mono-infection (n = 16). METHODS: Each patient underwent abdominal ultrasound, and a specific evaluation was performed in order to detect findings consistent with chronic liver disease. Characterization of spleen size, liver structural pattern, diameter of the portal, spleen, and mesenteric veins was based on classical ultrasound parameters. Propensity score was used for control of selection bias and performed using binary logistic regression to generate a score for each patient. The Fisher and Mann-Whitney tests were used to evaluate categorical variables and continuous variables, respectively. RESULTS: On univariate analysis right hepatic lobe size was larger in human immunodeficiency virus/hepatitis C virus patients (157.06 ± 17.56 mm) compared to hepatitis C virus mono-infected patients (134.94 ± 16.95 mm) (p = 0.0011). The left hepatic lobe was also significantly larger in human immunodeficiency virus/hepatitis C virus patients Cirrhosis (115.88 ±22.69 mm) versus hepatitis C virus mono-infected patients (95.06 ±24.18 mm) (p= 0.0177). Also, there was a strong correlation between hepatomegaly and co-infection (p=0.005). CONCLUSION: Human immunodeficiency virus infection was the primary variable influencing liver enlargement in this population. Hepatomegaly on ultrasound was more common among cirrhotic human immunodeficiency virus/hepatitis C virus co-infected patients than among cirrhotic hepatitis C virus mono-infected patients. This aspect is very important in the management of human immunodeficiency virus/hepatitis C virus co-infected patients, because screening for hepatocellular carcinoma is necessary in this population.

Nódulos hepáticos en adolescente con glucogenosis tipo Ia / Liver nodules in an adolescent with glycogenosis type Ia

La glucogenosis tipo I o Enfermedad de Gierke, es una enfermedad metabólica, hereditaria, por deficiencia de la Glucosa6-Fosfatasa, que ocasiona acumulo anormal de glucógeno en hígado, riñón y mucosa intestinal. Las manifestaciones clínicas: hipoglucemia, hepatomegalia, hiperlactacidemia, hiperlipidemia. Las complicaciones a largo plazo: gota, insuficiencia renal progresiva y adenoma hepático en la segunda o tercera década de la vida. Caso clínico: adolescente masculino de 12 años, en control desde su diagnóstico de Glucogenosis Ia a los 7 meses, con tratamiento nutricional y manejo multidisciplinario. El paciente presenta mal control metabólico con hipoglicemias recurrentes y hospitalizaciones secundarias a transgresión dietética. Examen físico: palidez cutánea mucosa moderada, obesidad troncal, cara de muñeca, abdomen prominente, hepatoesplenomegalia y postura lordótica. En control ecográfico, se reporta: nefromegalia bilateral y hepatoesplenomegalia difusa con lesiones de aspecto nodular, múltiples, intrahepáticas, sugestivas de Adenoma hepático. La biopsia de las lesiones: daño hepatocelular difuso, glucogenosis tipo I. TAC abdominal: hepatomegalia con 4 lesiones nodulares (adenomas) y lesión de ocupación de espacio hipercaptante en segmento hepático IV. En su seguimiento anual, se solicita ecografía abdominal, alfa-fetoproteína y otros marcadores, por tratarse de una lesión premaligna, de presentación precoz. En caso de deterioro clínico, el paciente es candidato a trasplante hepático, en caso de deterioro.

The type I glycogenosis Gierke's Disease is a metabolic disease, hereditary deficiency of glucose-6-phosphatase, which causes abnormal accumulation of glycogen in liver, kidney and intestinal mucosa. Clinical manifestations: hypoglycemia, hepatomegaly, hyperlactataemia, hyperlipidemia. The long-term complications: gout, progressive renal failure and hepatic adenoma in the second or third decade of life. Case report: A male adolescent of 12 years in control after diagnosis of glycogenosis Ia at 7 months, nutritional treatment and multidisciplinary management. The patient has poor metabolic control with recurrent hypoglycemia, and hospitalizations secondary to dietary transgression. Physical exam: moderate mucosal pallor, truncal obesity, doll face, protruding abdomen, hepatosplenomegaly and lordosis. In ultrasound guidance, it is reported: nephromegaly bilateral and diffuse hepatosplenomegaly with nodular lesions, multiple, intrahepatic, suggesting hepatic adenoma. The biopsy of the lesions: diffuse hepatocellular damage, glycogen storage disease type I. Abdominal CT: 4 hepatomegaly with nodular lesions (adenomas) and space-occupying lesion uptake in liver segment IV. In its annual monitoring is required abdominal ultrasound, alpha-fetoprotein and other markers, because it is a premalignant lesion, which occurred early. In case of clinical deterioration, the patient is a candidate for liver transplantation, in case of damage.

Estudio de casos de paracoccidioidomicosis infantil diagnosticados en el área de la Gran Caracas Venezuela / Study paracoccidioidomycosis diagnosed cases of childhood in the area of Greater Caracas Venezuela

La paracoccidioidomicosis pocas veces afecta niños y no es de denuncia obligatoria. Su verdadero impacto en la población infantil es desconocido. El objetivo fue determinar en número de casos de paracoccidioidomicosis infantil diagnosticados en centros de salud de la Gran Caracas, en niños con edades entre 0-15 años. Se evaluaron historias de niños con paracoccidioidomicosis, provenientes de hospitales de la Gran Caracas. Se revisaron 420 historias con diagnóstico de paracoccidioidomicosis, de las cuales 33 (7,9%) fueron niños menores de 15 años, siendo la media de edad 7,97 años. El género masculino predominó (69,7%), la razón masculino-femenino fue 1,5:1 en niños con edades de 0-7 años y 5,5:1 entre 8-15 años. La mayoría procedían del estado Miranda, 11 (35,5%) seguido del Distrito Capital 9 (29,1%). En 26 casos se clasificó la enfermedad, encontrando que 24 (92,3%) desarrollaron la forma aguda. Las principales manifestaciones clínicas precisadas en 25 historias fueron: fiebre 18 (72%) adenomegalias 16 (64%), hepatomegalia 12 (48,0%) y esplenomegalia 11 (44%). De 24 casos que tenían datos de laboratorio se observó que la eosinofilia y la anemia fueron los principales hallazgos. El diagnóstico micológico se realizó por serología en 24 (72,7%) casos, biopsia 11 (45,8%), examen directo 3 (12,5%) y cultivo 1 (4,2%). La paracoccidioidomicosis infantil es una entidad infrecuente, la cual se observa en 7,9% de los casos. Las manifestaciones más frecuentes son fiebre, adenomegalias y hepatoesplenomegalia. El diagnóstico de la paracoccidioidomicosis aguda infantil, siempre debe ser considerado en niños con edades de 0-15 años, en Venezuela.

Paracoccidioidomycosis rarely affects children, and it is not a notifiable disease. Its real impact in the child population is unknown. The aim of the study was to determine the number of paracoccidioidomycosis cases diagnosed in various health centers in Great Caracas, in children between 0-15 years. We reviewed 420 records with diagnosis of paracoccidioidomycosis from Hospital of Caracas. 33 (7,9%) were from children less than 15 years old. Age average 7,97 years. Male gender accounted for 69.7%, with a male: female relation of 1.5:1 in children between 0-7 years and 5,5:1 between 8-15 years. Most of the patients 11 (35,5%) came from Miranda state, followed bu Distrito Capital 9 (29,1%). In 26 patients the disease could be classified, from which 24 (92,3%) developed the acute form. Clinical manifestations, recorded in 25 cases were: fever 18 (72%), enlarged lymph nodes 16 (64%), hepatomegaly 12 (48,0%) and splenomegaly 11 (44%). From 24 cases in which laboratory data was recorded, eosinophilia and anemia were the most relevent findings. Mycological diagnosis was performed by serology in 24 (72,7%) cases, biopsy 11 (45,8%), direct examination 3 (12,5%) and culture 1 (4,2%). Child paracoccidioidomycosis is a rare disease, observed in 7, )% of cases. Most relevant clinical manifestations are fever, enlarged lymph nodes and hepatosplenomegaly. Acute paracoccidioidomycosis must be considered as a possible diagnosis in children between 0-15 years, in endemic countries such as Venezuela.

Linfosarcoma canino / Canine lymphosarcoma

Un canino criollo hembra de 5 años de edad fue llevado a la clínica veterinaria de la universidad de los llanos, con historia de anorexia y con una lesión en la región frontal de la cara. El análisis hematológico mostró anemia leve, neutropenia acompañada de linfocitosis marcada. En la necropsia se evidenció hepatomegalia y congestión hepática, esplenomegalia, congestión de vasosmesentéricos, la cápsula renal se desprendió con facilidad y la periferia renal presentaba zonas debajo relieve así como zonas pálidas en la corteza. Los resultados histopatológicos mostraron hallazgos compatibles con linfosarcoma que se clasificó como grado IV, con metástasis en hígado riñón y bazo. La distribución de la neoplasia encontrada es compatible con linfoma hepatoesplénico; en el cual se demostró la distribución de células neoplásicas que reflejaron un tropismo vascular y la lesión cutánea en la frente pudo ser resultado primario o secundario del linfosarcoma; la patología fue compatible con microabscesos de Pautrier

A 5 year-old female Creole canine was taken to the veterinary clinic of the Universidad de Los Llanos, with anorexy history and with a lesion in the front region of face. The hemathological analysis showed light anemia, neutropenia with marked lymphocytosis. In the necrosis was evidencedhepatomegaly and hepatic congestion, splenomegaly, congestion of mesenteric vessels, the renal capsule came off with easiness and the renal periphery presented areas of low relief as well as pale areas in peel. The histopathology results showed compatible discoveries with lymphosarcoma that was classified as grade IV, with metastasis in liver kidney and spleen. The neoplasia found is compatible with lymphoma hepatosplenic; in which distribution of neoplasia cells was demonstratedreflecting a vascular tropism and the cutaneous lesion in the forehead could be primary or secondary result of lymphosarcoma; pathology was compatible with Pautrier microabscess.

Acometimento hepático na telangiectasia hereditária hemorrágica (Osler-Weber-Rendu): relato de caso / Liver involvement in hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu): case report

O acometimento hepático nos pacientes com telangiectasia hereditária hemorrágica ou síndrome de Osler-Weber-Rendu é infreqüente e muitas vezes representa um desafio diagnóstico. Os autores apresentam o caso de uma paciente que procurou atendimento médico por aumento de aminotransferases, história prévia de epistaxe, exame físico e endoscopia digestiva alta evidenciando telangiectasia. A tomografia computadorizada de abdome sugeriu neoplasia primária ou metastática do fígado e a arteriografia demonstrou artéria hepática direita ectásica e neovascularização. Para confirmação do diagnóstico e exclusão de neoplasia foi realizada biópsia hepática, a qual evidenciou vasos anômalos e fibrose no espaço porta, espessamento e ectasia de ramos da artéria hepática e dilatação de sinusóides, achados já descritos nesta síndrome (AU)

Hepatic involvement in patients with hereditary hemorrhagic telangiectasia is infrequent and sometimes represents a diagnostic challenge. The authors present a case of a patient that presents with a elevation of aminotransferases, had history of epistaxis and telangiectases diagnosed by physical examination and upper gastrointestinal endoscopy. The computed tomographic scans suggested primary or metastatic liver neoplasia and angiographic studies showed enlarged right hepatic artery and neovascularization. Liver biopsy demonstrated findings of this syndrome like ectatic vascular structures, fibrosis, enlargement of hepatic artery and sinusoidal dilation. This confirmed diagnoses and exclude neoplasia (AU)

Alteraçöes hepáticas em pacientes com síndrome de imunodeficiência adquirida: avaliaçäo histopatológica e ultra-estrutural: estudo através biópsias hepáticas / Hepathological alterations in patients carriers of acquired immunodeficiency syndrome: histopathological and ultra-estructural evalution: histopathologiac evaluation and ultrastructural study through liver byopsis

Cerca de dois-terço dos pacientes com SIDA desenvolvem hepatomegalia e anormalidades nos parâmetros bioquímicos que avaliam a funçäo hepática. Aparentemente, a doença hepática näo depende diretamente do vírus da imunodeficiência humana (HIV), mas, pode resultar de processos infecciosos determinados por bactérias, fungos, vírus, protozoários ou da açäo de drogas e do envolvimento por processos neoplásicos. Os objetivos do presente trabalho säo: avaliar a utilidade da biópsia hepática em esclarecer a situaçäo clínica de pacientes com SIDA que apresentam hepatomegalia, elevaçäo sérica de enzimas hepáticas e/ou febre de origem desconhecida; caracterizar as alteraçöes sinusoidais. Foram analisadas 130 biópsias hepáticas de pacientes com SIDA, que apresentavam pelo menos hepatomegalia e febre de origem desconhecida. Em 57 (43,8 porcento) pacientes havia dosagem de aminotrasferases e em 48(36,9 porcento) havia avaliaçäo numérica dos linfócitos T CD4+ e CD8+, no sangue periférico. Procedeu-se à analise do material à microscopia ótica e eletrônica, com técnicas de morfometria e de microanálise química. A biópsia hepática foi considerada diagnóstica em 46(35,4 porcento) dos casos, incluindo-se 22(16,9 porcento) casos de infecçäo microbacteriana, 9(6,9 porcento) casos de infecçäo pelo Histoplasma capsulatum, 4(3,1 porcento) casos de infecçäo pelo Schistosoma mansoni, 1(0,8 porcento) caso de infecçäo pelo Cryptococcus neoformans, 1(0,8 porcento) caso de infecçäo leishmaniótica, 1(0,8 porcento) caso de sarcoma de Kaposi, 1(0,8 porcento) caso de infiltraçäo por linfoma näo Hodgkin e 7(5,4 porcento) casos de inflamação granulomatosa, sem evidências de microorganismos. Inflamaçäo granulomatosa foi a mais freqüente alteraçäo de tipo inflamatório observada, estando presente em 29(22,3 porcento) casos. Näo observamos correlaçäo entre níveis de aminotransferases e a presença de alteraçöes hepáticas que significassem mudança ou introduçäo de terapêutica específica. Näo foram observadas alteraçöes de monta na estrutura de sinusóides hepáticos, através avaliaçäo histopatológica e ultra-estrutural. Com base nos resultados obtidos, podemos concluir que: 1. A biópsia hepática pode fornecer subsídios importantes para o diagnóstico de processos infecciosos e neoplásicos em pacientes com SIDA, que apresentam hepatomegalia e febre prolongada de origem desconhecida. 2. Existe uma maior prevalência em nosso meio, quando comparada com outros países de infecçäo por...

Acute lymphoblastic leukaemia in adults

In this paper, tive have presented a morphological breakup of 30 cases of Acute Lymphoblastic Leukemia [ALL] in adults, according to French-American-British [FAB] criteria. ALL-L1 was the commonest FAB type [60%], followed by L2 [33.3%] and L3 [6.7%], respectively. The commonest clinical features in these patients were pallor [100%],fever [91%], hepatomegaly [75%], lymphadenopathy [63%], splenomegaly [57%] and bleeding manifestations [54%]. Hemoglobin level ranged from 3.2 to 11.0 G/dl, with a mean +/- SD of 6.48 +/- 2.22 G/dl. Total white cell count ranged between 0.75 and 72.4 x 10e9/1, with a mean +/- SD of 13.85 +/- 13.06 x 10e9/1. Platelet count ranged from 5 to 135 x 10e9/1., with a mean +/- SD of 56 +/- 21.5x10e9/1

Biopsia hepática percutánea en el paciente adulto / Liver biopsy diagnostic in adult

La biopsia hepática percutánea continúa siendo un método diagnóstico y de evaluación terapéutica ampliamente utilizado en la práctica médica contemporánea. Bien indicada y con un conocimiento adecuado de la técnica, ofrece un margen de seguridad importante, por lo que puede llevarse a cabo en casi todos los pacientes con enfermedad hepática. A lo largo de los años en nuestro hospital se ha acumulado una gran experiencia en este procedimiento por lo que en este trabajo se hace una revisión de las indicaciones y contraindicaciones, además de describirse los tipos de agujas que existen y la técnica que debe emplearse

Pathology of periportal fibrosis involution in human schistosomiasis

Optical and electron microscopical evidences of focal matrix degradation were frequently seen in liver sections of periportal fibrosis caused by schistosomiasis mansoni in man. The material came from 14 wedge hepatic biopsies taken from patients with chronic advanced hepatosplenic disease and undergoing operations for the relief of portal hypertension. Besides the presence of focal areas of rarefaction, fragmentation and dispersion of collagen fibers, the enlarged portal spaces also showed hyperplasia of elastic tissue and disarray of smooth muscle fibers following destruction of portal vein branches. Eggs were scanty in the tissue sections, and matrix degradation probably represented involuting changes related to the progressive diminution of parasite-related aggression, which occurs spontaneously with age or after cure by chemotherapy. The changes indicative of matrix degradation now described are probably the basic morphological counterpart of periportal fibrosis involution currently being documented by ultrasonography in hepatosplenic patients submitted to curative chemotherapy

Nephrotic syndrome associated with hepatointestinal schistosomiasis

Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%), whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%). On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (P < 0.01). In the group with schistosomiasis, 8 patients evidenced mesangial proliferative glomerulonephritis and 5, membranoproliferative glomerulonephritis. In both histological types immunofluorescence showed IgM and C3 granular deposits in the glomeruli. The data in this study suggests that mesangial proliferative and membranoproliferative glomerulonephritis, with glomerular granular IgM and C3 deposits, represent the renal lesions of the schistosomiasis associated nephropathy

Serum laminin in hepatosplenic human schistosomiasis

Serum laminin level was measured in chronic schistosomiasis. A significant increase in the mean serum laminin levels was observed in patients with hepatosplenic (HS) schistosomiasis (2,57 ñ 0,83U/ml), as compared to those in patients with the hepatointestinal (HI) form of the disease (1,38 ñ 0,45-U/ml) and in the control group (1,15 ñ 0,31 U/ml). In the HS patients there was a significant direct relatiom between serum laminin and percutaneous splenic pulp pressure (r = 0,68). These findigs are compatible with an increased production of lamin in hepatosplenic schistosomiasis with may be related to the observed enlarged liver and spleen basement membranes in such disease

retrospective descriptive study of 100 cases of kala-azar in a teaching hospital in Baghdad

This is a descriptive, retrospective study of 100 cases of Kala-azar admitted to Saddam Central Teaching Hospital for Children during 13 months [1 Jan. 1989 - 31 Jan. 1990] to find and discuss the epidemiological and clinical criteria of the disease. All our cases were under the age of 5 years [except one], while the symptoms and signs were: fever [100%], splenomegaly [95%], hepatomegaly [92%], anorexia [70%], respiratory and gastro-intestinal tracts infections [50% and 30% respectively]. There were less than 10% suffering from jaundic, edema or bleeding tendency. There was no sex difference in the cases; and 82% of them were from Al-Anbar and Babylon governorates, in addition to Baghdad city. more cases were found in winter and spring