Clinical presentation of hemophagocytic lymphohistiocytosis in adults is less typical than in children

OBJECTIVE: Hemophagocytic lymphohistiocytosis in adults is largely underdiagnosed. To improve the rate and accuracy of diagnosis in adults, the clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis were analyzed in and compared between adults and children in a Chinese cohort. METHOD: Data from 50 hemophagocytic lymphohistiocytosis patients, including 34 adults and 16 children who fulfilled the 2004 hemophagocytic lymphohistiocytosis diagnostic criteria, were collected and analyzed. RESULTS: 1. Etiological factors: The proportion of Epstein-Barr virus infection was lower in adults compared with children, whereas fungal infection and natural killer/T cell lymphoma were more frequent in adults (P<0.05). 2. Clinical manifestations and laboratory findings: Over 90% of adults and pediatric patients presented with fever, thrombocytopenia and high serum ferritin levels. However, in adults, the proportions of hepatomegaly, splenomegaly and jaundice were much lower (P<0.01) than in children, and serous cavity effusion was more frequent in adult patients (P<0.05). More children had hemoglobin <90 g/L, total bilirubin >19 mmol/L and lactate dehydrogenase >500 U/L compared with adults (P<0.05). 3. The time interval from the onset of symptoms to clinical diagnosis was significantly shorter in pediatric patients than in adults (P<0.05). CONCLUSIONS: Certain clinical features were different between the two groups. The less characteristic clinical presentation of hemophagocytic lymphohistiocytosis in adults may make the disease more difficult to diagnose. Our findings suggest that hemophagocytic lymphohistiocytosis should be considered when an adult patient presents with the above-mentioned symptoms.

Malignant infantile osteopetrosis.

Osteopetrosis, a rare congenital genetic disease characterized by increased bone density due to impaired bone resorption by osteoclasts. It is classified into three forms: Infantile malignant autosomal recessive (AR) osteopetrosis, intermediate (AR) osteopetrosis and autosomal dominant (AD) osteopetrosis. Incidence of infantile malignant AR is 1/2,00,000 and if untreated has a fatal outcome. The condition is commonly diagnosed in infancy with symptoms of significant hematologic abnormalities with bone marrow failure, hepatosplenomegaly, macrocephaly with frontal bossing and bone fractures. Because of rarity of this type of malignant infantile form of osteopretrosis, we like to report this case of malignant infantile osteopetrosis who presented with bronchopneumonia, anemia with melaena at 2 months 15 days of age.

epidemiological profile of brucellosis in Tabuk Province, Saudi Arabia

All 137 brucellosis cases occurring in Tabuk Province, Saudi Arabia in 1997 were studied retrospectively. Brucella agglutination titre of > or = 1/80, or rising titre plus history of typical signs and symptoms were considered evidence of infection. The incidence rate was 34/100,000, mean age 33.8 +/- 13.9 years [range: 3-72 years] and male:female ratio 1.8:1. There were 63.5% of cases rurally resident, 58.4% kept animals at home or elsewhere, 27.0% worked with animals and/or on farms, and 88.3% reported a history of raw milk ingestion. The most common infecting agents were Brucella melitensis, B. abortus and B. suis. Splenomegaly and hepatomegaly were detected in 25.5% and 22.6% of cases respectively

Experience in the evaluation of children with hepatosplenomegaly at a teaching ambulatory, Sao Paulo, Brazil

Objetivos: descrever os casos de criancas com hepatoesplenomegalia (HE) matriculadas no Ambulatorio Geral (AGER) do Instituto da Crianca, Sao Paulo, identificando as principais causas, evolucao, necessidade de hospitalizacao e / ou encaminhamento a especialistas. Metodologia: estudo retrospectivo dos prontuarios de criancas matriculadas no AGER para investigacao de HE de 01/09/93 a 31/08/96. Resultados: das 89 criancas incluidas (idade: 1 a 148 meses; mediana, 24 meses), 64 (72 por cento) foram encaminhadas de outros servicos para investigacao de HE. As queixas mais frequentes foram: febre - 39 (44 por cento); palidez - 26 (29 por cento); emagrecimento - 21 (24 por cento) e ictericia - 14 (16 por cento). As principais alteracoes ao exame fisico foram: palidez - 47 (53 por cento) e baixa estatura - 17 (19 por cento). Setenta criancas (79 por cento) apresentavam anemia; 35 (39 por cento) tinham infeccoes; 7 (8 por cento) doencas metabolicas e 5 (6 por cento) doencas neoplasicas. Trinta e seis criancas (40 por cento) foram encaminhadas a especialistas...

An epidemic of dengue hemorrhagic fever and dengue shock syndrome in children in Delhi.

OBJECTIVE: To study clinical profiles and outcome of children of dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS) during 1996 Delhi epidemic. DESIGN: Retrospective study. SETTING: Hospital based study. METHODS: Children hospitalized from September to November 1996 were studied. All patients were diagnosed, managed and monitored according to a standard protocol. RESULTS: One hundred and thirty four children (80 (60%) males and 54 (40%) females) were studied. Sixty (45%) children were less than 6 years of age of which 12 presented during infancy. There were 92 (67%) cases of DHF and 42 (33%) cases of DSS. Common symptoms were fever (93%), abdominal pain (49%) and vomiting (68%). The commonest hemorrhagic manifestation was hematemesis (39%) followed by epistaxis (36%) and skin bleeds (33%). Hepatomegaly was observed in 97 (72%) cases and splenomegaly in 25 (19%). Serology was positive (IgM hemaglutination antibody titres > 1: 160) for dengue type 2 in 31 (80%) of 39 patients in whom sera was tested during the acute phase of illness. Mortality was 6%. Hematocrit > 40% was observed in only 25 (18%) patients and hence the management protocol was based on clinical signs and symptoms and not on hematocrit. CONCLUSIONS: A management protocol of DHF/DSS in which fluid therapy is not based on haematocrit values needs to be formulated.

Health status of Orang Asli (aborigine) community in Pos Piah, Sungai Siput, Perak, Malaysia.

A study of health status of Orang Asli population (based on physical examination findings) was conducted in 4 villages in Pos Piah, Sungai Siput Perak, Malaysia. In all 356 individuals between 4 months-72 years old (178 males and 178 females) participated in this study. Poor general health status, physical and mental handicaps were seen in 7.8%, 0.3% and 0.3% of the population, respectively. About one-fifth of the population had dental caries. Splenomegaly, hepatomegaly and hepatosplenomegaly were among the commonest abnormalities with the occurrence rates of 19.8%, 13.7% and 6.7%, respectively, being detected in the population. About one-fifth of the population showed signs suggestive of protein-energy deficiency; whilst less than 5% showed signs indicative of riboflavin, iodine and iron deficiencies. Vitamin A deficiency was the commonest nutritional deficiency identified in this community with almost 38.4% of them showing signs of the deficiency. The commonest skin infection was scabies.

Está desaparecendo a glomerulopatia da esquistossomose mansônica? / Is glomerulopathy due to schistosomiasis mansoni disappearing?

As formas severas de esquistossomose mansônica, particularmente a hépato-esplênica, se acompanham de glomerulopatia geralmente manifesta por síndrome nefrótica. Nos últimos 10 anos reduziu-se muito o número de casos observados desta glomerulopatia no Hospital Universitário Prof. Edgard Santos, um hospital geral num estado onde esta parasitose é endêmica. O objetivo deste estudo foi verificar se estava havendo, de fato, desaparecimento desta patologia ou se este número decrescente refletia apenas diminuição marcante de casos graves de esquistossomose mansônica acompanhados neste Hospital. Para isso foram comparadas as prevalências de glomerulopatia em hepatosplênicos esquistossomóticos autopsiados no Hospital Universitário Prof. Edgard Santos em duas décadas: de 1960-70, antes da intervenção terapêutica nas áreas endêmicas de esquistossomose com oxamniquine, e de 1980-1990, após a adoção desta intervenção. Houve grande redução no número de doentes com hepatosplenomegalia esquistossomótica autopsiados no Hospital Universitário Prof. Edgard Santos quando comparadas a década antes (140 autópsias) e depois (31 autópsias) do tratamento com oxamniquine. A prevalência de glomerulopatia, entretanto, persistiu a mesma (11,4% comparada com 12,9%, respectivamente). A conclusão é que a redução do achado de glomerulopatia esquistossomótica em nossos dias reflete apenas a redução das formas graves da parasitose após a intervenção terapêutica nas áreas endêmicas.

Hepatosplenic form of S. mansoni infection may be accompanied by a glomerulopathy in 12-15% of cases, manifested in the majority by a nephrotic. This type of renal involvement is becoming a rare occurrence in our University Hospital (Hospital Universitário Prof. Edgard Santos) a typical general hospital in an endemic state for this parasitic disease. To investigate this fact, autopsied cases with patients with hepatosplenic form of schistosomiasis mansoni during two decades in our Hospital-1960-70, (before a therapeutic intervention in endemic areas with oxamniquine) and 1980-1990 (after the intervention) were compared in reference to number of cases and the finding of glomerulonephritis by histological examination. Even though there was a striking decrease in number of patients with advanced forms of this disease (140 as compared to 31 autopsies in these two decades), the prevalence of glomerulonephritis diagnosed was 11.4 (16 cases) in the first and 12.9 (4 cases) in the second. As there was no change in pattern of attendance in this Hospital, the drastic decrease in number of severe forms of this parasitic infection following massive therapy of the endemic population with oxamniquine is the most likely explanation not only for the decrease in number of hepatosplenic cases but, also, and as a consequence, the scarcity of cases of the schistosomal glomerulopathy observed.

Relaçäo entre a patogenicidade do Schistosoma mansoni em camundongos e a susceptibilidade do molusco vetor. III. Mortalidade, pesos corporal e das vísceras / Relationship between the pathogenicity of Schistosoma mansoni in mice and the susceptibility of the vector mollusc. III. Mortality, body weight and viscera weights

Estuda a relaçäo entre o desenvolvimento da hepatomegalia, da esplenomegalia, peso corporal e taxa de mortalidade em camundongos experimentalmente infectados por Schistosoma mansoni com o grau de susceptibilidade de Biomphalaria glabrata e B. tenagophila nas quais se desenvolveram as cercárias infectantes respectivamente, das linhagens BH e SJ. Foram utilizados como hospedeiro definitivo camundongos Swiss, SPF e como hospedeiros intermediários populaçöes de moluscos selecionados geneticamente para o caráter susceptibilidade. Foram observados menores pesos corporais e das vísceras em camundongos infectados com cercárias provenientes de moluscos que apresentaram elevado grau de susceptibilidade. A maior susceptibilidade dos moluscos à infecçäo pelo S. mansoni correspondeu a uma menor sobrevivência dos camundongos infectados. Os resultados fazem crer que a maior adaptaçäo do parasita ao hospedeiro intermediário, traduzidos pelas taxas mais elevadas de susceptibilidade, pode levar a um comportamento diferente deste parasita no hospedeiro definitivo

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

Family occurrence of schistosomal hepatosplenomegaly and maternal effect

As formas clínicas da esquistossomose mansônica, anteriores ao tratamento com oxamniquine, foram estudadas nos membros de 265 famílias, com seis anos ou mais de idade. A probabilidade da ocorrência de dois ou mais casos, na mesma família, da hepatosplenomegalia esquistossomótica (HE) é baixa, no entanto foi obsrevada em 38 famílias; menor probabilidade (P = 0,002) devia ocorrer três ou mais casos, porém dezessete famílias estavam nesta situaçäo. A concentraçäo da forma HE foi alta entre irmäos, comparativamente á observada entre mäes e filhos e pais e filhos. Näo sendo significante a concentraçäo encontrada no casal entre marido e mulher. Estas observaçöes reforçam a evidência do efeito do componente genético da susceptibilidade para a forma HE. Também, quando a mäe era hepatosplênica (HE) a prevalência desta forma nos filhos foi maior; o risco relativo foi cinco vezes superior (efeito materno), comparado ao encontrado quando era o pai hepatosplênico. Quando ambos os genitores eram hepatointestinal os riscos relativos da forma HE, nos filhos, foi semelhante ao da populaçäo geral. Assim, provavelmente fatores pré e pós-natais também estaräo envolvidos na predisposiçäo da forma hepatosplênica

Tuberculosis hepática. Estudio retrospectivo de 30 pacientes con diagnóstico anatómico en el Hospital General Cayetano Heredia. / Hepatic tuberculosis. Retrospective study of 30 patients with anatomic diagnosis at the Hospital General Cayetano Heredia.

Treinta pacientes, 18 adultos y 12 niños, que fueron atendidos en el Hospital General Cayetano Heredia entre abril de 1969 y marzo de 1983, con diagnóstico anatómico de tuberculosis hepática (hecho en 21 por biopsia y en 9 por autopsia), fueron estudiados retrospectivamente desde el punto de vista epidemiológico, clínico bioquímico, bacteriológico e histológico. La tuberculosis hepática es más frecuente en los primeros 5 años de vida. En la mitad de los pacientes hubo un antecedente epidemiológico de tuberculosis. La signología más frecuente en el cuadro clínico son la fiebre y la hepatomegalia. Las alteraciones bioquímicas más frecuentadas son elevación de la fosfatasa alcalina y transaminasas y caída de proteinas; las bilirrubinas se encontraron elevadas en 9 de los 30 pacientes. Se encuentra evidencia radiológica de compromiso pulmonar hasta en el 84% de los pacientes.No existe ningún factor que tenga una clara y significativa asociación con el pronóstico. Se hace resaltar la importancia de la biopsia en el diagnóstico preciso y definitivo.