Izrail Cat

Blood lactate concentration as prognostic marker in critically ill children

OBJECTIVE To assess the use of lactate as a marker of tissue hypoperfusion and as a prognostic index in critically ill patients. METHODS Prospective, longitudinal, observational study of 75 patients admitted to the pediatric ICU of Hospital de Clínicas of Universidade Federal do Paraná, between November 1998 and May 1999. According to the lactate level on admission, patients were divided into group A (lactate > or = 18 mg/dl) and group B (lactate < 18 mg/dl). In terms of outcome, patients were classified into survivors and nonsurvivors. In group A, the clinical evaluation and the collection of arterial blood samples were performed on admission, at 6, 12, 24, 48 hours, and every 24 hours after that. In group B, they were carried out in the same way, but interrupted 48 hours after admission. RESULTS Groups A and B consisted of 50 and 25 patients, respectively. Group A presented more clinical signs of hypoperfusion (24/50). There was a statistically significant difference regarding the mean lactate levels on admission between those patients who died within 24 hours of admission (95 mg/dl) and those who died 24 hours after admission (28 mg/dl). The lactate level at 24 hours of admission revealed better sensitivity (55.6%) and specificity (97.2%) as a predictor of death. CONCLUSIONS Most patients with lactate levels > or = 18 mg/dl showed clinical signs of hypoperfusion on admission. The normalization or reduction of lactate levels at and after 24 hours of admission was significantly related with higher chances of survival.

Tumores do córtex adrenal na infância

Adrenocortical tumors (ACT) in children are uncommon. However, the incidence of these tumors in Parana, Brazil, is 15 times higher than that worldwide. We describe the clinical, laboratory and treatment characteristics and outcome of 125 patients treated in a single institution in the State of Parana. The median age at diagnosis was 4.3 years, with a female:male ratio of 2.6:1. The most common forms of presentation were isolated virilization (51.2%) and virilization and Cushings syndrome (42%). Nonfunctioning tumors comprised 4.8% of the cases. Two patients (1.6%) had isolated Cushings syndrome and 1 (0.8%) had Conns syndrome. Fifty-six percent presented hypertension. Surgery is the only curative treatment. Our data show that disease stage 1, absence of spillage during surgery and absence of intravenous thrombus were associated with better survival rates.

An Ectodermal Dysplasia Syndrome of Alopecia, Onychodysplasia, Hypohidrosis, Hyperkeratosis, Deafness and other Manifestations

A girl is reported with a hitherto apparently undescribed ectodermal dysplasia syndrome. The main findings include: alopecia, onychodysplasia, hypohidrosis, sensorineural deafness, skin with a tan color and hyperkeratosis (involving also plams and soles), unusual facies (with slight auricle and nose abnormalities), pectus excavatum, severe hyperopia, EEG abnormalities, and retarded bone age. The patient also presents mongoloid palpebral slanting, narrow palpebral fissures, bilateral esotropia, photophobia and dermatoglyphics with extensive ridge dissociation. The etiology is unknown but presumed to be genetic, possibly due to the homozygous state of an autosomal recessive mutation.

Odontotrichomelic Hypohidrotic Dysplasia A Clinical Reappraisal

This paper presents a second clinical appraisal of a dysmorphic syndrome (Odontotrichomelic hypohidrotic dysplasia) presented in two personally examined sib lings and two of their brothers who died in infancy. This syndrome is an ectodermal, dysplasia of the tricho-odonto-onycho-dyshidrotic subgroup, according to Freire-Maia’s classification. The two patients also have other defects, such as thin, dry and shiny skin, protruding lips, enlarged nose, hypoplastic nipples and areolae, severe tetramelic reductions, abnormal auricles and metabolic defect. Cleft lip, EEG abnormalities, and growth retardation have also been verified in the boy; the only additional finding in the girl was an ECG abnormality. This syndrome is presumed to be due to the homozygous state of an autosomal recessive gene.

Tratamento do tumor do córtex adrenal na infância

Adrenocortical tumors (ACT) in children are uncommon. However, the incidence of these tumors in Paraná is 15 times higher than that worldwide. A germline mutation, R337H TP53, present in more than 95% of our patients, is probably the reason for the higher incidence in our state. A hundred twenty-five patients were treated in the period of 1966 to 2003. Surgery is the only curative treatment. In our experience, disease stage I, absence of spillage during surgery and absence of intravenous thrombus are associated with better survival rates. Preliminary data with the combination of etoposide, doxorubicin, cisplatin, and mitotane have shown that in some patients a complete remission is observed both of the tumor and metastasis. Side effects due to these drugs are common and adrenal insufficiency may occur. Glucocorticoid and mineralocorticoid reposition should be done with 2 to 3 times the physiological doses.

Meningencefalite a mucormicose

A case of meningoencephalitis due to mucormycosis in a 8 months-old pacient is reported. The diagnosis was made at necropsy.

Meningencefalite a aspergillus

A case of meningencephalitis due to Aspergillus in a 39 days-old boy is reported. The diagnosis was made by post-morten examination. It seems to be the first case of aspergiloris reported in Brazil.

Meningite eosinofílica e eosinofilia sanguínea de origem parasitária

E relatado o caso de um menino de 12 meses de idade que apresentava febre, rigidez de nuca e hepatoesplenomgalia e que, em dois exames sucessivos, apresentou eosinofilia sanguinea de 72% e 75% e liquorica de 75% e 78%, respectivamente. A biopsia hepatica mostrou um granuloma eosinofilico. Embora a crianca apresentasse grande infestacao por Ascaris lumbricoides, os autores admitem que o quadro clinico apresentado possa ter sido determinado por larvas de Toxocara canis, apesar do parasita nao ter sido encontrado.

A Note on Quantitation of Intermammillary Distance

This note refers to the illusion of widely spaced nipples in some syndromes and adds another example in a pure ectodermal dysplasia on the basis of measurements in the patient and in 50 controls.

Síndrome de Rubinstein-Taybi: relato de dois casos

Two non-inbred cases of Rubinstein-Taybi syndrome is two non-related sibships with a total of 16 sibs are reported. Clinical features are those classicaly reported. One of the patients (case 1) presents left post-axial polydactily and a history of hydramnion. The cariotype is normal in the other patient (case 2). Nothing is added in this paper as regards the etiology of the syndrome.