Search

The Mexican Society of Pediatric Endocrinology presents recommendations for the diagnosis and treatment of precocious puberty (PP), a condition defined as the development of sexual characteristics due to an increase in pituitary gonadotropin secretion before 8 or 9 years of age in girls and boys, respectively.Three systematic reviews were conducted: controlled clinical trials on interventions for PP treatment, diagnostic tests, and observational studies on the long-term effects of PP. The quality evaluation and data extraction from the studies were conducted by two independent reviewers. The Scottish Intercollegiate Guidelines Network and the Oxford Center for Evidence-Based Medicine systems were used for grading the quality of evidence for recommendations on intervention and diagnosis, respectively. Recommendations were submitted to a consensus by a Delphi method and were validated by another 143 certified pediatric endocrinologists through an online questionnaire.The group generated 12 recommendations on the diagnosis of PP, seven on the diagnosis of secondary causes of PP, eight on interventions for inhibition of puberty, five on other interventions for PP treatment, and 14 for the monitoring and follow-up of these patients. The online questionnaires submitted to certified pediatric endocrinologists showed more than 90% of approval for each one of the recommendations.Although a high degree of consensus for the recommendations for diagnosis, treatment, and monitoring of PP among pediatric endocrinologists was achieved, most of these recommendations showed a low level of evidence.La Sociedad Mexicana de Endocrinología Pediátrica presenta recomendaciones para el diagnóstico y el tratamiento de la pubertad precoz (PP), condición definida como el desarrollo de caracteres sexuales por incremento en la secreción hipofisiaria de gonadotropinas antes de los 8 años en las niñas y de los 9 años en los niños.Se realizaron tres revisiones sistemáticas de ensayos clínicos controlados sobre intervenciones para el tratamiento de la PP, pruebas diagnósticas y estudios observacionales sobre efectos a largo plazo de la PP. La evaluación de la calidad de los estudios y la extracción de datos se realizó por pares. La evidencia se graduó con el sistema de la Scottish Intercollegiate Guidelines Network (SIGN) y del Oxford Centre for Evidence-Based Medicine (OCEBM) para las recomendaciones sobre la intervención y el diagnóstico, respectivamente. Las recomendaciones generadas se sometieron a un consenso por el método Delphi y fueron validadas por otros 143 endocrinólogos pediatras certificados mediante un cuestionario en línea.Mediante consenso se generaron 12 recomendaciones para el diagnóstico de PP, siete sobre diagnóstico de causas secundarias de PP, ocho sobre intervenciones para inhibición de la pubertad, cinco sobre otras intervenciones en PP y 14 para la monitorización del tratamiento y el seguimiento de estos pacientes. Se obtuvo más del 90% de aprobación para cada una de las recomendaciones por el grupo de endocrinólogos certificados que respondieron el cuestionario en línea.Si bien se logró un alto grado de consenso para las recomendaciones para el diagnóstico, el tratamiento y la monitorización de la PP entre los endocrinólogos pediatras, el nivel de evidencia para la mayoría de estas recomendaciones resultó bajo.

Hyperhomocysteinemia as a risk factor for hearing impairment, neuronal damage and cognitive impairment in elderly patients is controversial and is limited by the small number of studies. The aim of this work was determine if elderly patients detected with hyperhomocysteinemia have an increased risk of developing abnormalities in the central auditory processes as compared with a group of patients with appropriate homocysteine levels, and to define the behaviour of psychoacoustic tests and long latency potentials (P300) in these patients.This was a cross-sectional, comparative and analytical study. We formed a group of patients with hyperhomocysteinemia and a control group with normal levels of homocysteine. All patients underwent audiometry, tympanometry and a selection of psychoacoustic tests (dichotic digits, low-pass filtered words, speech in noise and masking level difference), auditory evoked brainstem potentials and P300.Patients with hyperhomocysteinemia had higher values in the test of masking level difference than did the control group (P=.049) and more protracted latency in P300 (P=.000).Hyperhomocysteinemia is a factor that alters the central auditory functions. Alterations in psychoacoustic tests and disturbances in electrophysiological tests suggest that the central portion of the auditory pathway is affected in patients with hyperhomocysteinemia.

PURPOSE To report a patient with severe and progressive bilateral deafness who suffered recurrent episodes of diffuse lamellar keratitis (DLK) in both eyes ≥4 months after undergoing uneventful LASIK for myopia. METHODS Four months after LASIK, the patient presented with blurred vision in the left eye. Best spectacle-corrected visual acuity was 0.9. Slit-lamp examination revealed aggregates of white cells at the paracentral and mid-peripheral interface without affecting the visual axis. Diffuse lamellar keratitis stage II was diagnosed and intensive corticosteroid treatment was started. Uncorrected visual acuity (UCVA) in the right eye was 1.2 with no signs of DLK. RESULTS One week after initiating corticosteroid treatment, UCVA improved (1.2) in the left eye. Diffuse lamellar keratitis was diagnosed in the left eye again at 9 months and in the right eye at 15 months after LASIK. The patient was later diagnosed with atypical Cogan’s syndrome. CONCLUSIONS Recurrent and late-onset DLK may be due to other patient systemic conditions predisposing to interstitial keratitis, such as Cogan’s syndrome. [J Refract Surg. 2008;24:548-550.] ABOUT THE AUTHORS From the Department of Cornea and Refractive Surgery, VISSUM, Instituto Oftalmológico de Alicante, Miguel Hernández University School of Medicine, Alicante, Spain (Javaloy, Muñoz, Pérez-Santonja, Vidal, Alió); and Otorhino-Laringology Department, Fundación Hospital de Torrevieja, Torrevieja, Spain (Barrera). The authors have no proprietary interest in the materials presented herein. Correspondence: Jaime Javaloy, MD, PhD, Instituto Oftalmológico de Alicante, Avda Denia, sn. Edificio VISSUM, Alicante, Spain. Tel: 34 965 150 025; E-mail: jjavaloy@coma.es; rdioa@vissum.com Received: June 13, 2007 Accepted: November 7, 2007 Posted online: January 31, 2008

Aims: To ascertain the prevalence of acute trachoma as a supposed endemic disease among infants in the Saharan refugee camps of Tindouf (Algeria) and to evaluate the efficacy of treatment with a single dose of azithromycin. Methods: 527 children aged between 3 and 17 were evaluated in the camp schools in October 2001. All the children were clinically and microbiologically examined, including slit lamp checks of anterior segment and two conjunctival swabs, one for the detection of membrane lipopolysaccharide by quick immunoassay test Clearview Chlamydia MF and the other for the detection of specific DNA by chlamydia plasmid specific polymerase chain reaction (PCR) assay. After examination, a single dose of azithromycin was administered to each child. One month later samples from positive children and 12 random negative children by the first PCR were taken to perform a new PCR assay. Results: Chlamydia trachomatis was suspected in 2.47% of the children, papillary hypertrophy being the most frequently seen clinical sign. Scarring changes secondary to trachoma were detected in 11.7% of the children. Only four cases (0.8%) were positive to the immunoassay test and 12 cases (2.27%) were positive by PCR. After treatment a second PCR was performed on positive children and they were negative of chlamydia DNA amplification. However, one child who was negative and received the treatment was positive in the second PCR assay. Conclusion: Acute trachoma prevalence is much lower than expected among infants in this Saharan population. The possible reasons could be the recent improvements in hygiene and health care. Cicatricial trachoma is more frequent. The Clearview immunoassay test has very low sensitivity in the detection of this disease. A single dose of azithromycin is sufficient to treat chlamydial conjunctivitis. However, a programme of improvement in hygiene and health care is necessary to prevent re-infection.

To analyze the prevalence of choroidal metastases in patients with breast and lung cancer and report their clinical, ophtalmological and angiographic features.88 patients who in 1997 had been diagnosed of breast cancer (60 cases) and lung cancer (28) by the oncology unit in the University Hospital of Elche, underwent a thorough ophtalmological examination in search for choroidal metastases. Eighty six patients were newly diagnosed of cancer; the remaining two patients, in a complete remission status, relapsed during this period.Total prevalence of choroidal metastases in the sample was 4.54% (4 cases). Prevalence in lung cancer was 7.14% (2 cases) compared to 3.33% (2 cases) in breast cancer. Choroidal involvement was found in the following stages: 2 out of 7 patients (28.5%) in stage IV with disseminated breast cancer and 2 out of 9 patients (22.22%) in stage IV with disseminated lung cancer.Approximately one fourth of the patients showing disseminated breast and lung cancer (stage IV) showed metastases in the choroid. It appears from this study, that younger patients suffering from lung cancer are at greater risk of choroidal involvement (Arch Soc Esp Oftalmol 2002; 77: 23-28).

A genomic expression library of Trypanosoma cruzi (T. cruzi) was made using plasmid pcDNA3 as a vector, with which male mice from the Balb/c isogenic line were intramuscullary inoculated. It was used a positive control group that was administered soluble antigens of T. cruzi. Other 2 groups received genomic and plasmid DNA, respectively. One group was not immunized. Weekly blood samples were obtained from all the animals until the fourth week and 2 weeks after reimmunization to study the response of specific antibodies against the microorganism antigens by an indirect immunoenzymatic assay (ELISA). It was observed a significant increase of specific antibodies in the animals reimmunized with 50 micrograms of the library, as well as in the group immunized with soluble antigens of T. cruzi.

In animal models of epilepsy, calcium entry blockers have shown anticonvulsant properties. We studied the antiepileptic effects of nimodipine and two novel dihydropyridines, a calcium antagonist (PCA 50922) and a calcium agonist (PCA 50941), on pentylenetetrazole seizure and maximal electroshock seizure (MES) in mice. Anticonvulsant profile of nimodipine and PCA 50922 was similar to that of clonazepam, but markedly different from that of phenytoin. None of the doses of the PCA 50941 showed anticonvulsant effect., Alter.

A series of 7 cases of chromophobe cell carcinoma with clinicopathological correlation is studied. The immunohistochemistry analysis was carried out evaluating in all cases: low and high cytoqueratines (AE1, AE3), membrane epithelial antigen (EMA), vimentin, carcinoembrionary antigen (CEA), proliferative cell nuclear antigen (PCNA) and the oncoproteins p53 and focal c-erbB2. In all cases a diffuse positivity for EMA, AE3 and focal for AE1 was observed, while vimentin and CEA were negative. The positivity for PCNA was always less than 25% of tumoral cells. The oncoprotein p53 was in all cases negative and only in one case a mild and focal positivity for c-erbB2 was observed.1) The negativity for vimentin and positivity for EMA, AE1 and AE3 allows to differentiate this carcinoma from clear cell carcinoma. This corroborate a distinct histogenesis, thus suggesting their origin in the intercalar cells of distal collector tubules. 2) The low values of PCNA and the negativity for both oncoproteins p53 and c-erbB2 supports the low aggresivity of this tumor and a better prognostic than other types of kidney carcinomas.

Remodeling of the cirrhotic liver was studied retrospectively by mathematical morphologic methods in 75 autopsy cases (40 alcoholic, 17 hepatitis B virus (HBV)-related and 18 cryptogenetic cirrhosis), including 28 hepatocellular carcinomas. The aim was to obtain objective measurements of cirrhotic patterns that could be correlated with liver function evaluated by the Pugh-Child score, establish the relationship among different morphogenetic features and evaluate the implications of an objective classification of cases by numerical taxonomy in terms of their etiology, liver function and malignant transformation. The results indicate that the Pugh-Child score was closely related to the global amount of fibrosis or to the percentage of regenerative nodules0.8 mm in diameter. In contrast, the higher the percentage of lobular-sized regenerative nodules (0.8-1.6 mm), the better the functional score, suggesting that they are probably residual lobules, albeit completely surrounded by fibrous tissue, rather than true regenerative pseudolobules. The four groups of cases obtained by numerical taxonomy (cluster analysis) showed different distributions for alcoholic and HBV-related cirrhosis. The pattern of the latter was practically analogous to that in classically labeled cryptogenetic cirrhosis, suggesting its viral etiology. Taxonomic classification had functional implications. The Pugh-Child score showed a definite relationship with the different clusters obtained. The incidence of malignant transformation gradually decreased from group G1 to G4, with a steeper descent between G2 and G3. These results might contribute to a more dynamic concept of morphologic changes in liver biopsies from patients with cirrhosis.

The pattern and extent of disorganization of the liver architecture were studied in 25 children undergoing orthotopic liver transplantation for cirrhosis. Image analysis techniques based on mathematical morphology were used to define seven parameters for each case, including fibrosis index (percentage of Sirius-red-stained areas), three categories of regenerative nodules (0.8, 0.8-1.6 and1.6 mm in diameter) and three categories of fibrous septa (0.4, 0.4-1.2 and1.2 mm in width). Fibrosis index ranged from 10.2% to 51.9%. Percentage of small nodules of infralobular size (0.8 mm in diameter) varied from 31.4% to 98.2%. Percentage of large nodules,1.6 mm in diameter, in only four cases was15%. Multivariate clustering analysis classified the cases into three main groups. One of them included only cases of cirrhosis secondary to parenchymal disease or to inborn errors of metabolism. In their pattern, tiny nodules predominated, and the percentage of slender septa,0.4 mm in diameter, was very high, although the overall fibrosis index was relatively low. Patients with biliary cirrhosis were classified into two groups. In one the fibrosis index was low and the size of the nodules variable, with 12% large nodules. In the other the fibrosis index was high, and small nodules,0.8 mm in diameter, predominated. No relationship was found with age at transplantation or previous portoenterostomy. A presumptive explanation for this divergent evolution might be the occurrence of cholangitic episodes. Overall fibrosis that the liver can sustain without failure is apparently limited.(ABSTRACT TRUNCATED AT 250 WORDS)

A 7 year old boy presented with bilateral nodular testicular swelling, adult penis development and scrotal aspect. Laboratory studies revealed a bone age of 13 years, increased testosterone, estradiol, DHEA-S, and 17 ketosteroids levels. The GnRH test revealed puberal findings. Biopsy was consistent with Leydig cell tumor with presence of Reinke crystals. After enucleation of the tumor signs of necrosis and malignancy were shown. Orchiectomy will follow as chemotherapy and radiotherapy have been reported to be without value.

Cryptosporidiosis was observed in 23 undernourished infants of a nutritional center in Temuco. This represents a 34% incidence rate for the period (march to may 1990). Liquid stools (91%), vomiting (39%), resistance to feeding (74%), respiratory symptoms (61%) and weight loss (mean 25 g per day) were the main clinical manifestations. Only 9 patients received specific therapy and all showed clinical improvement and normalization of stool examinations within the study period.

In the present study, 7 patients with secondary amyloidosis and renal involvement who developed rapidly progressive deterioration of renal function are reported. After the investigation of the possibly implicated clinicopathological factors a cause was found for 3 patients. In the remaining 4 patients, the rapid evolution to end stage renal disease was attributable to the rapid progression of amyloid deposits by itself. A correlation between the evolution of renal function, vascular amyloid deposits and interstitial fibrosis is reported.

The authors report nine cases of chronic glomerulonephritis with positive hepatitis B antigen (AgHB) (8 cases) and positive anti-AgHb (1 case). Five patients had membranous glomerulonephritis. The sub-type was ad in 6 cases and ay in 2. In 3 members of two families of patients, AgHB was positive with the same sub-type as the subject studied. In 5 cases, liver biopsy showed signs of chronic hepatitis. According to the authors, a positive AgHB in glomerulonephritis is often associated with clinically latent chronic hepatitis.

Finding a lymphangioma in the upper digestive tract is a rather infrequent event. A case of a 64-year-old man is reported. Diagnosis was made in the course of an endoscopic oesophagogastroduodenal examination and tumor excision with a diathermy snare at the same time. Two years after resection there was no sign of recidivation of the tumor.

Necrotizing angiitis or vasculitis exhibits a wide clinical spectrum characterized by many different cutaneous manifestations. Diagnosis must be confirmed by histopathology. We studied in retrospect 25 patients whose conditions had been diagnosed by skin biopsy. Histologic examination revealed infiltration by polynuclear cells and fibrinoid necrosis of the walls of the blood vessels in the skin. The great variety of clinical manifestations and etiologies stands out in a review of the records of these patients. Necrotizing angiitis has been found associated with mixed cryoglobulinemia; administration of drugs, milliary tuberculosis, bacterial meningitis, rickettsiosis, staphylococcal sepsis, pharyngotonsillitis, and rheumatoid arthritis. Necrotizing angiitis is a group of diseases with a great variety of clinical manifestations, ranging from benign to fatal. The various entities described to date have been more like different clinical forms of the same disease that distinct conditions. In cases of necrotizing angiitis caused by basically immunological mechanisms, the walls of the blood vessels may be impaired in varying diffuse degrees. The prognosis of the disease depends on the intensity of the inflammation and its repercussions on the parenchymas of different organs. The kidney is the most susceptible organ in this case. Treatment should be directed toward the avoidance of predisposing and etiologic factors, detection of the immunological reaction, requiring careful and individual attention in every case.

Sixty-six infants aged 8 days to 3 months presented jaundice as a sign of urinary infection during a ten-year period 1968-1977. The main clinical and biochemical aspects are described. "E.coli" grew in 49 urine cultures (74.2%), but other bacteria were also found ("Klebsiella", "Proteus", "Pseudomonas", "A. aerogenes"). Hepatic function tests seem to prove that intrahepatic colostasis is the main mechanism involved, although hemolysis was also found in some cases. The importance of considering urinary infection in the diagnosis of jaundice during infancy is stressed.

The authors report the evolution of 72 patient's visual field operated for one pituitary adenoma. They specify the constant amelioration of this investigation in chromophobic adenomas. The stability in prolactinic adenomas or somatotropic. Allowing the supervision of the visual case, at these patients, the visual field does not seem useful in the diagnosis of the tumors recurrence.

For lupus nephritis (LN) management, it is very important to detect fibrosis at an early stage. Urinary exosomal miRNAs profiling can be used as a potential multi-marker phenotyping tool to identify early fibrosis. We isolated and characterised urinary exosomes and cellular pellets from patients with biopsy-proven LN (n = 45) and healthy controls (n = 20). LN chronicity index (CI) correlated with urinary exosomal miR-21, miR-150, and miR-29c (r = 0.565, 0.840, &minus, 0.559, respectively). This miRNA profile distinguished low CI from moderate-high CI in LN patients with a high sensitivity and specificity (94.4% and 99.8%). Furthermore, this multimarker panel predicted an increased risk of progression to end-stage renal disease (ESRD). Pathway analysis identified VEGFA and SP1 as common target genes for the three miRNAs. Immunohistochemistry in LN renal biopsies revealed a significant increase of COL1A1 and COL4A1 correlated with renal chronicity. SP1 decreased significantly in the high-CI group (p = 0.002). VEGFA levels showed no differences. In vitro experiments suggest that these miRNA combinations promote renal fibrosis by increasing profibrotic molecules through SP1 and Smad3/TGF&beta, pathways. In conclusion, a urinary exosomal multimarker panel composed of miR-21, miR-150, and miR-29c provides a non-invasive method to detect early renal fibrosis and predict disease progression in LN.

At present, Lupus Nephritis (LN) is still awaiting a biomarker to better monitor disease activity, guide clinical treatment, and predict a patient’s long-term outcome. In the last decade, novel biomarkers have been identified to monitor the disease, but none have been incorporated into clinical practice. The transmembrane receptor neuropilin-1 (NRP-1) is highly expressed by mesangial cells and its genetic deletion results in proteinuric disease and glomerulosclerosis. NRP-1 is increased in kidney biopsies of LN. In this work we were interested in determining whether urinary NRP-1 levels could be a biomarker of clinical response in LN. Our results show that patients with active LN have increased levels of urinary NRP-1. When patients were divided according to clinical response, responders displayed higher urinary and tissue NRP-1 levels at the time of renal biopsy. Areas under the receiver operating characteristic curve, comparing baseline creatinine, proteinuria, urinary NRP-1, and VEGFA protein levels, showed NRP-1 to be an independent predictor for clinical response. In addition, in vitro studies suggest that NRP-1could promote renal recovery through endothelial proliferation and migration, mesangial migration and local T cell cytotoxicity. Based on these results, NRP-1 may be used as an early prognostic biomarker in LN., This work was supported by grants from the Spain Government (Instituto de Salud Carlos III). This research also received donations from Catalan Lupus Foundation and A.Bosch Foundation.