Correlación clínica- para-clínica en un escolar con hipertensión porta de etiología a esclarecer / Clinical-paraclinical in a school with portal hypertension to determine

La hipertensión porta (HTP) es el resultado del incremento de la presión dentro del sistema venoso porta. Se presenta con poca frecuencia en el paciente pediátrico pero es una de las mayores causas de morbilidad y mortalidad en el niño con enfermedad hepática. La mayoría de los pacientes con http presentan un estado hiperdinámico, lo cual aumenta el flujo venoso porta y mantiene la hipertensión. Puede ser secundaria a obstrucción a nivel prehepático, intrahepático o extrahehepático.

Portal hypertension (PH) is the result of increased pressure within the portal venous system. It occurs infrequently in the pediatric patient but it is a major cause of morbidity and mortality in children with liver disease. Most patients with PH have a hyperdynamic state, which increases venous flow and portal hypertension remains. May be secondary to obstruction at prehepatic, intrahepatic or extrahehepatic.

Hepatosplenomegaly in Kenyan schoolchildren: exacerbation by concurrent chronic exposure to malaria and Schistosoma mansoni infection.

OBJECTIVES: Chronic exposure to malaria exacerbates Schistosoma mansoni-associated hepatosplenomegaly in school-aged children. However, residual hepatosplenomegaly after treatment of S. mansoni with concurrent mollusciciding suggests malaria could be an underlying cause of hepatosplenomegaly. We investigated the role of chronic malaria in childhood hepatosplenomegaly in the presence and absence of concurrent S. mansoni infection. METHODS: Cross-sectional study of children in an study area where transmission of S. mansoni, but not malaria, is restricted to the eastern end. Clinical and ultrasound examinations were conducted, and parasitological and serological tests used to determine S. mansoni infection intensities and comparative exposure levels to malaria. RESULTS: Chronic exposure to malaria, as determined by Pfs-IgG3 levels, was associated with hepatosplenomegaly even in the absence of S. mansoni infection. Children infected with S. mansoni mostly had light to moderate infection intensities but greater enlargement of the liver and spleen than children who did not have schistosomiasis, and for the left liver lobe this was S. mansoni infection intensity dependent. CONCLUSIONS: Children chronically exposed to malaria but without S. mansoni infection can have hepatosplenomegaly, which even light S. mansoni infections can exacerbate in an intensity-dependent manner. Thus, concurrent chronic exposure to S. mansoni and Plasmodium falciparum can have an additive or synergistic effect on childhood morbidity.

Evaluation of splenomegaly in the hepatosplenic form of mansonic schistosomiasis.

This study was aimed to evaluate splenomegaly in patients with the hepatosplenic (HS) form of mansonic schistosomiasis (MS), analyzing the size and weight of the spleen and their relationships with patients' gender and age. Between October, 1993 to July, 1998, 78 patients with the HS form of MS had undergone splenectomy as treatment of choice for bleeding due to portal hypertension, at Hospital das Clínicas, Pernambuco, Brazil. By means of abdominal palpation, the excess spleen felt below the left costal edge was measured, and the weight was obtained after splenectomy along with the histopathological analysis. Liver biopsy was performed intraoperatively in order to confirm MS and to rule out other liver diseases. The mean age of the 78 patients were 45 years and 41 of them (53%) were female. The average spleen weight was 912g and the mean spleen size palpable below the left costal edge was 9.1cm. There was a positive relationship between size and weight (p<0.001). Spleen weight and size were larger in males (p=0.007 and p=0.001, respectively). An inverse correlation between age and spleen weight was observed (p<0.001). A classification based upon spleen weight showed 53% of patients presenting a moderate (501-1000g) and 33% a severe (>1001g) splenomegaly. As for the spleen size, the classification showed 64% of patients presenting moderate (4.1-10cm below the left costal edge) and 21% severe (>10cm) splenomegaly. In conclusion, splenomegaly may be considered a key physical finding in patients with HS form of MS, and we found a good correlation between the spleen sizes clinically evaluated with its weight. The majority of cases presents a moderate to severe splenomegaly and spleen size is larger in men and it seems to decrease with aging.

Chronic myeloid leukaemia at the Kenyatta National Hospital, Nairobi.

OBJECTIVE: To determine the clinical and haematological factors associated with treatment and outcome of chronic myeloid leukaemia (CML) at Kenyatta National Hospital. DESIGN: Retrospective survey of patients treated for chronic myeloid leukaemia. SETTING: Kenyatta National hospital, Nairobi, Kenya, between April 1990 and August 2000. SUBJECTS: Patients with chronic myeloid leukaemia. RESULTS: One hundred and four patients, 55 males and 49 females, age range 10-72 years with a median age of 35 years. Treatment with busulphan getting less popular in favour of hydroxyurea. Median follow-up 20 months with none of the clinical and haematological parameters impacting significantly on duration of follow-up. CONCLUSION: CML occurs at a younger age-group in Kenya, and none of the clinical or haematological parameters appears to impact on follow-up duration.

Bases da abordagem clínica das esplenomegalias / Basis of the clinical approach of the splenomegaly

O estudo das esplenomegalias pode permear pistas decisivas para a realizaçao do diagnóstico sindrômico e etiológico de grande número de enfermidades, nos domínios da medicina interna. Neste artigo foram revisados os aspectos morfofuncionais essenciais do baço, da fisiopatologia e da clínica das esplenomegalias, tendo sido conferida especial ênfase às etapas propedêuticas do reconhecimento das principais doenças sistêmicas, que, dentre várias peculiaridades, apresentam inequívoca conotaçao diagnóstica com aumento anormal do órgao esplênico.

[Pathological classification of splenomegaly in portal hypertension].

The morphometric analysis was carried out for 150 cases splenomegaly pathological sections (1010 sections) after being stained with histochemical, immunohistochemical, hematoxylin and eosin methods, summarizing that the proliferation of three types of fibers, the number and distribution of T, B lymphocytes and macrophages, the changes of sections stained with HE, the ultrastructure of splenomegaly under electron microscope would be the criteria for partially preserving splenomegaly. The data of morphometry was treated by IBM/PC computer. The research was aimed to establish a pathological indication for preserving partial splenomegaly clinically. The study demonstrated that the pathological classification, based on the fibrosis degree of splenomegaly, may objectively reflect the changes of immunological function of splenomegaly, by which it was also revealed that the immunological function in pathological class II and III was markedly lowered. Because the proportion of class I splenomegaly remained only in 31% of good function, there are less splenomegaly worth preserving.

Enfermedad de Gaucher: presentación de un caso / Gaucher's disease: report of a case

La enfermedad de Gaucher es una enfermedad de tipo autosómica recesiva causada por una deficiencia de la enzima Beta-Glucocerebrosidasa, acumulándose así glucocerebrósido en el sistema retículo endotelial. Su incidencia es muy baja, presentóndose más frecuente en hebreos. Se describen tres formas típicas según su forma de presentación. Se revisa la experiencia con una paciente con Enfermedad de Gaucher a quien se le realizó esplenectomía total por presentar hiperesplenismo

[Etiology of splenomegaly in children in the tropics. 178 cases reviewed at the university hospital center of Abidjan-Cocody (Ivory Coast)]. / Etiologie des splénomégalies de l'enfant en milieu tropical. A propos de 178 cas colligés au CHU d'Abidjan-Cocody (Côte d'Ivoire).

Discovery of an enlarged spleen in a child requires steps to identify the etiology. One hundred and seventy-eight patients seen over a four-year period (1985-1988) at the Cocody Teaching Hospital were reviewed. The incidence of splenic enlargement among pediatric inpatients was 1.6%. Males (n = 106) were more often affected than females (n = 72). Slightly over half the children (54.49%) were 0 to 5 years of age. The main clinical presenting features were fever (90%), anemia (72%), a decline in general health (36.50%), enlargement of the liver (33.50%), jaundice (26.50%), and enlarged lymph nodes (7%). Type II of Hackett's classification accounted for most cases (61.80%), followed by Type III (14%). Main etiologies included malaria (53%), salmonella infections (15%), sickle cell anemia (14%), schistosomiasis (9%), AIDS (3%), and thalassemia (2%). Malignancies (leukemia, lymphoma) were relatively infrequent. More than one etiology was found in 13 cases. The distribution of etiologies by age group was determined and a strategy for investigating children with splenic enlargement in tropical countries was developed.

[Dacie syndrome and other splenomegalies without apparent cause]. / El síndrome de Dacie y otras esplenomegalias sin causa aparente.

Some enlarged spleens do not seem to be related with known pathogenetic mechanisms (passive congestion, functional workload, malignant infiltration and inflammatory or storage disorders). Non-tropical idiopathic splenomegaly (Dacie's syndrome) is a form of hypersplenism of unknown origin that evolves into a non-Hodgkin lymphoma, after a variable interval, in 20% of the patients. Tropical idiopathic splenomegaly (or hyperreactive malarial splenomegaly) develops when a chronic malarial challenge triggers an abnormal immunological response consisting in decreased suppressor T lymphocytes and increased amounts of circulating immunoglobulin M and immunocomplexes, which are cleared by the splenic macrophages. This peculiar response to malaria seems to be linked to particular HLA antigens. Other confusing splenomegalies are seen in Felty's syndrome, in populations subjected to recurrent infections, and in some families. Overlapping findings and diseases suggest chronic antigenic stimulation as a common feature, with diverse responses depending on the host. A small percentage (probably less than 3%) of normal individuals has minimal splenomegaly without any clinical significance.

Clasificación y diagnóstico clínico de las esplenomegalias / Classification and clinical diagnosis of splenomegaly

El hallazgo semiológico y/o ecotomográfico de esplenomegalia constituye un hecho relevante de considerable importancia diagnóstica y/o pronóstica. Puede presentarse en forma aislada en un paciente asintomático, pero lo más frecuente es que se asocie a diversos síntomas o signos, entre los cuales los más significativos y orientadores en lo que a diagnóstico se refiere son: fiebre, adenopatías, hepatomegalia con o sin ictericia, anemia, artritis