A clinical checklist for fragile X syndrome: screening of Thai boys with developmental delay of unknown cause.

The aim of this study was to determine a cost-effective clinical checklist for fragile X syndrome (FXS) screening in a Thai male pediatric population with developmental delay of unknown cause. We studied 179 non-FXS male patients and 27 FXS patients from 18 families (age < or = 15 years). A six-item clinical checklist was used including family history (FH), long and narrow face (F), prominent and large ears (E), attention deficit/hyperactivity (AH), autistic-like behavior (AT) and testicular volume (T). These were scored as 0 if absent, 1 if borderline, and 2 if present. All patients were tested by using PCR and/or southern blot for the FMR1 gene. We used a logistic regression model from a computer program to analyze the data (Stata, version 5.0). We used logistic regression with cluster in the same family (average score) to eliminate bias from the related FXS cases. We found that a five-item checklist, 2FH + F + 0.5E + 2AH + T = total score, was the best model. When we used this clinical checklist with a threshold of total score of 4, 78.7 per cent of the screened cases with total scores < or = 4 could be eliminated as negative cases. In addition, all positive FXS cases had total scores > 4. We propose this five-item model for FXS screening in clinical pediatric practice, particularly from Asian population settings.

The usefulness of serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) for evaluation of children with short stature.

The diagnostic value of serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) was studied in 24 growth hormone deficient (GHD) and 36 normal variant short stature (NVSS) children. The serum IGF-1 and IGFBP-3 concentrations were markedly below the 5th centile for chronological age in all 24 GHD children, but were in the low normal range for age in most of the NVSS children. The concentrations of IGF-1 and IGFBP-3 significantly correlated with peak GH concentration, height age, and bone age. To account for the age- and sex-dependency, IGF-1 and IGFBP-3 levels were transformed to standard deviation score (SDS). Using the -2 SDS as a cut-off level to differentiate between GHD and NVSS, the diagnostic value of IGF, as well as IGFBP-3, showed sensitivity 100 per cent, specificity 66.7 per cent, and accuracy 80 per cent. The combined use of IGF-1 and IGFBP-3 < -2 SDS improved the diagnostic value with sensitivity 100 per cent, specificity 77.8 per cent, and accuracy 86.7 per cent. We concluded that the serum concentrations of IGF-1 and IGFBP-3 could reflect endogenous GH secretion and could be used as a screening evaluation of GH status in short children.

p53 protein expression in cutaneous squamous cell carcinoma.

BACKGROUND: p53 is a nucleoprotein encoded by a tumor suppressor gene. It's mutations are implicated in the genesis of a wide variety of malignant neoplasia including skin cancers. OBJECTIVE: To study the expression of the p53 protein in cutaneous squamous cell carcinoma (SCCs) and evaluate the relationships between this expression and sites, varying degrees of differentiation and amounts of apoptotic cells. METHOD: Sixty-seven tissue samples of SCCs from Songklanagarind Hospital obtained from January 1991 to December 1996 were examined by immunohistochemistry using polyclonal anti p53-CM1. (Novocastra Laboratories, Newcastle, England, dilution 1:700) RESULT: p53 Immunoreactivity was demonstrated in 26.87 per cent of SCCs. This was observed in 15/51 of sun-exposed cases and 3/16 of sun-protected cases (p = 0.401). The more differentiated the tumor, the less p53 staining was observed (p = 0.043). There was no association between p53 positivity and the amounts of apoptotic cells. CONCLUSION: The p53 expression is not related to the sun exposure. It does not represent a commitment to apoptosis. However, it may indicate the differentiation and/or proliferative status of the tumor cells.

Serum insulin-like growth factor-1 (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3) in healthy Thai children and adolescents: relation to height, weight, and body mass index.

The objective of this study was to evaluate the correlation between serum concentrations of insulin-like growth factor-1 (IGF-1), insulin-like growth factor binding protein-3 (IGFBP-3) and growth parameters (height, weight, and body mass index) in 260 healthy children and adolescents aged 5-20 years. The subjects were divided into 2 groups according to the age achieving final height. Group 1 included children with active growth consisting of girls aged under 14 years (N = 80) and boys aged under 16 years (n = 74). Group 2 included adolescents who achieved final height consisting of females aged at and over 14 years (n = 82), and males aged at and over 16 years (n = 24). In group 1, the serum concentrations of IGF-1 and IGFBP-3 were significantly positive correlated with all growth parameters. In group 2, although the correlation was insignificant, the concentrations of IGF-1 and IGFBP-3 seemed to be greater in individuals who were relatively taller and had lean body mass than those who were relatively short and over average body mass.

Serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) in healthy Thai children and adolescents: relation to age, sex, and stage of puberty.

The authors studied the serum concentrations of insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP-3) in 260 healthy children and adolescents (115 males, 145 females) aged 5-20 years. The subjects were divided into 12 groups according to age and sex. The serum IGF-1 and IGFBP-3 concentrations increased with age and peaked at age 13-15 years in males, and 11-13 years in females. After the peak concentration, IGF-1 and IGFBP-3 levels declined significantly in males, but were still high in females. Comparing between sexes, the concentrations of IGF-1 and IGFBP-3 were greater in females than males in all age groups. However, when subjects were divided according to the stage of puberty, the different concentrations between sexes were not significant, except for children within Tanner stage V where concentrations of IGF-1 and IGFBP-3 were significantly greater in females than males. Multiple regression analysis demonstrated the age, sex, and stage of puberty-dependent of IGF-1 concentration, and only the age and sex-dependent of IGFBP-3 concentration.

Traumatic hematomas of the posterior cranial fossa.

The objective of this study was to find out the result of treatment and the factors which can predict the outcome of traumatic hematomas of the posterior cranial fossa. Twenty two patients with traumatic hematomas of the posterior cranial fossa from 1,500 patients with traumatic intracranial hematomas were analyzed. There were fourteen male and eight female patients. The most common etiology was a motor vehicle accident. About 90 per cent of the patients had a direct injury to the occipital region. Ninety per cent of the patients had an occipital skull fracture or diastatic fracture of the lambdoid suture. The overall mortality rate was about 38 per cent. Patients having pure epidural hematoma had zero mortality. By contrast, patients suffering epidural hematoma with associated intracranial hematoma had 20 per cent mortality. Intracerebellar hematoma led to 60 per cent mortality. Glasgow Coma Scale (GCS) before operation was used to predict the patients' outcome. Ninety per cent of the patients who had a GCS between 13 and 15 had a good recovery. By contrast, only 30 per cent of the patients who had a GCS below 9 had a good recovery. Statistical analysis showed that the GCS value of below 9 predicted the poor outcome for the patients.

Cancer incidence in Songkhla, southern Thailand, 1990-1994.

A population-based cancer registry of Songkhla was established by the Cancer Unit of Songklanagarind Hospital under the support of the IARC in 1990. The province is in the southern region of Thailand and has a population of 1.2 million. This study presents the average annual incidence rate of the provincial total and of the district level covering 1990-1994. It is aimed at providing a comprehensive picture of descriptive epidemiology of cancer in the province. Data were collected from all hospitals in the provinces. Analysis was done under the program provided by IARC. There were 3,973 invasive cancer cases in the period. The age-standardized rate for all cancers was 116.7 in males and 88.7 in females. Lung, oral cavity, liver, and esophagus were the main leading sites in males while the cervix and breast were outstanding in females. By comparison, the incidence of most cancers were lower than other registries in Thailand except for two cancer sites. The incidence of male oral cavity and esophagus cancers in males (ASR 10.7 and 8.5 respectively) were considerably higher. Na Mom, Hat Yai, Sadao and Muang were districts having a high incidence of cancer.

Development of cancer registry in Phatthalung Hospital.

Evaluation of feasibility is required for development of a hospital-based cancer registry of Phatthalung Hospital. The completeness of case finding from in-hospital sources was evaluated for cancer cases diagnosed from January 1, 1994 to December 31, 1994. Cancer patients were collected from a file of pathological reports and medical records in Phatthalung Hospital. Items in the registration form included identification, demographic data and the data specific to cancer. During the same period of time, all biopsy cases sent from Phatthalung Hospital were selected from the pathological record of the Department of Pathology, Songklanagarind Hospital and from the database of the cancer registry of Songklanagarind Hospital. Ninety cases with histological verification were identified either from Phatthalung or Songklanagarind sources. Twenty seven cases were male and 63 were female. Fifty records were identified from Phatthalung sources and 40 additional records from Songklanagarind sources. Identification of cancer cases only from Phatthalung sources represented only 56% of all histologically verified cases. The two most common primary sites in males were colon and prostate. The percentages were 18.5 and 11.1 respectively. Lung cancer accounted only for 7.4% of all histologically verified cases. The two most common primary sites in females were cervix uteri and breast which accounted for 31.7% and 23.8% respectively. There was too much failure of case identification by in-hospital sources only. Songklanagarind sources were important for case findings. However, it is important that the medical records and filing system in Phatthalung Hospital should be improved.

Hematoxylin-lac-curcuma polychrome stain for mucin.

A polychrome method for detection of mucin substance in paraffin section is produced by sequential stepwise staining of hematoxylin, crude lac extract (Laccifer lacca), and crude curcuma extract (khamin shan-Curcuma longa). The name LacCur stain is proposed. After a tissue section is deparaffinized and rehydrated, it is stained with Weigert's hematoxylin for 7 minutes. After a quick wash, it is stained for at least 3 hours with lac dye mordanted with aluminum chloride. Washed again and premordanted with ferric chloride for 1 minute, in the last step, it is counterstained with curcuma dye for 5 minutes. With this staining method, the nuclei are stained black, mucin deep red, and organelles and ground substances brownish yellow. The method and outcome colors are comparable to the widely used Mayer's mucicarmine staining method. It costs less than the Mayer's mucicarmine staining method and the procedure is not complicated.

Primary lymphoma of the esophagus: a case report.

A case of primary non-Hodgkin's lymphoma of the esophagus was reported. The macroscopic manifestation from esophagography, computed tomography and esophagoscopy were demonstrated. Pathological findings were demonstrated. The tumour responded well to combined therapy of surgery, radiotherapy and chemotherapy.

Polysplenia syndrome: a case with congenital heart block, infarction of a splenic mass, and a short pancreas.

A case of polysplenia with congenital heart block, infarction of a splenic mass, and a short pancreas was reported. The infant died of severe heart failure at the age of 28 days. Deformity of the SA node was suspected to be the cause of congenital heart block but could not be demonstrated because of some limitations of autopsy procedure. Redundant splenic pedicle might cause infarction of a splenic mass after birth. The author suggests that, in addition to the semi-annular pancreas, the pancreatic anomalies of this syndrome should include the short pancreas.