مجله علوم پزشکی رازی
سال دوم شماره 4 (پیاپی 8، زمستان 1374)

The ataxia - telangiectasia or Louis - Bar syndrome is a rare hereditary disease. The inheritance pattern is autosomal recessive, locus gene has been mapped to the long arm of chromosome 11.
The onset of the disease coincides more or less with the acquisition of walking, which is awkward and unsteady. The characteristic telangiectatic lesions, are mainly subpapillary vascular plexuses of the bulbar conjunctivae and over the ears. In these cases ataxia at 1.5 and 2 years old appeared , but telangiectatic lesions appeared at 4 years.
There are high circulating levels of alpha - fetoprotein this is thought to be due to immaturity of the liver, and serum concentration of IgA and IgG are reduced. In these cases (one brother and sister), serum alpha fetoprotein significantly increased. In the sister, IgA is absent and in the brother level of IgA is decreased.

A 55 - years - old woman was referred to Firoozabadi General Hospital with abdominal pain and vomiting of 2 months duration. Physical examination revealed a pelvic mass located in left lower quadrant, and no any lymph node enlargement was noted.
At laparatomy a large solid mass was removed, with irregular surface and necrotic areas.
Pathology was reported as primary diffuse large cell immunoblastic lymphoma of the ovary.
Diagnosis was also confirmed by immunohistochemical studies. Review of literature from 1990 -95 revealed only two cases with this diagnosis , and in Imam Khomeini Cancer Institute reports there were two such cases during last 20 years.

This prospective study was designed to find the incidence of streptococcal pharyngitis in childhood sore throat and also to compare the clinical diagnosis of disease with the results of throat cultures. Children, 5 to 15 years of age with sore throat who were seen in pediatric out - patients department formed the subjects of this study. The duration of study was one year (October 1992 - October 1993). There were 443 children with sore throat. 100 patients (22.5%) had positive throat culture (group A Beta hemolytic streptococci) . 53 patients (53%) were male and 47% were female. The mean age of the group was 8.8 ± 3 years. Clinical findings in order of frequency were as follow: redness of pharynx 99% , acute onset of symptoms 86% , fever 84% 1 exudate on tonsils 69% , enlargement and tenderness of anterior cervical lymph nodes 30% , and associated gastrointestinal symptoms 22% . Seasonal prevalence of disease were as follow: spring 35%, winter 34% , autumn 28 % and summer 3%. 53% of this patients had 5 clinical symptoms. 5 clinical findings such as sore throat fever acute onset of disease redness of pharynx exudate on tonsils and absence of viral symptoms (cough, coryza, conjunctivitis, hoarseness) had a sensitivity of 57% , specititv of 98% J positive predictive value of 89% , negative predictive value of 89% and efficiency of 89%. This study showed that in the absence of laboratory facilities careful clinical findings can be helpful for reaching accurate diagnosis of streptococcal pharyngitis.

In order to evaluate the incidence and frequency of clinical and laboratory findings in newborns with polycythemia in Tehran a retrospective study was carried out in newborns who were admitted in newborn service in Ali Asghar Children Hospital between years 1988-1995 . During this period 3086 newborns were admitted and 32 patients had central hematocrit above 65% (1.03%) . Of the polycythemic infants, 84.4 % were sympthomatic. Frequent signs and symptoms included: cyanosis 28. 1 %, plethora 28.1%, jitteriness 25%, poor feeding 25%, lethargia 21.8%, respiratory distress 12.5% and hypotonia 12.5% . Other findings included hypoglycemia 43.7% and hyperbilirubinemia 25% . Of the polycythemic infants, 15.6% had no clinical or laboratory abnormality. It was noted that SGA & LGA newborns are more prone to polycythemia. With early diagnosis and appropriate treatment the prognosis is excellent.

In order to evaluate the frequency of clinical and laboratory findings of neonatal sepsis in Tehran, a retrospective study was carried out in 100 newborns who were admittedin newborn service of Ali Asghar Children Hospital with diagnosis of neonatal sepsis and eventually had positive blood cultures.
56% of this patients were above 1 week of age (late neonatal sepsis) and 44% were below 1 week (early neonatal sepsis). Among infected newborns, 67% were male and 33% were female. The frequency of clinical findings was: decreased reflexes (74%), poor feeding 44% , lethargy 34%, Jaundice 31%, hypothermia 25%, respiratory distress 24% J vomiting 19%, cyanosis 18% , hyperthermia 18% and irritability 17%.
Other findings included: Leukopenia 22% , increased band cells 28% , thrombocytopenia 36%, prolonged PIT 26% and PT 20% .
With early diagnosis and treatment 82% of the patients recovered completely.

The study was conducted to determine the manifestation of esophageal carcinoma in Iranian patients. 92 cases with esophageal carcinoma that hospitalized in Rasool - E Akram and Rahnamoon General Hospitals were studied retrospectively.
57% of the patients were males and 62% in 50 - 70 years old ages. The common symptoms were: dysphagia (96%), odynophagia (69%) , restrosternal pain (59%) , regurgitation (53%) , nausea and vomiting (47%), coughing (17%) and hematemesis (8%).
The common clinical signs were: cachexia (62%) , lymphadenopathy (16.5%) , hoarseness (9%) and hepatomegaly (7.6%) .
69% of the tumors were detected in distal third of the esophagus and cardia. Squamous cell carcinoma (S.C.C) was the most common pathologic diagnosis.
The post operative staging of the tumors were: stage I (22%) s stage 1/(15%) , stage 11/(49%) and stage IV(15%).
The mortality rate of this patients was 7% .
In conclusion, a significant percent of the patients were detected in early stages and were successfully managed. Many of these patients had no major problem in follow - up.

Platelets as cellular elements are the constituants of blood tissue and their main function is to participate in hemostatic processes.
The glycocalyx which intimately surrounds the platelets contains a number of glycoproteins which are responsible for blood group specificity (ABO), tissue compatibility (human leukocyte antigen = HLA) , and platelet antigenicity. Platelets contain granules which are involved in many sorts of activities like maintaining the integrity of vascular endothelial cells.
This process is mostly mediated by platelet derived growth factor (PDGF) Adhesion of platelets to each other and thrombus formation are mediated by different factors such as platelet activation and release of ADP which facilitate formation of glycoprotein and phospholipoprotein complexes in the membrane of neighboring platelets.
TXA2 released from arachidonic acid originally from membrane otiosphotipides also plays a role in thrombus formation. Thrombus then binds to collagene fibers of subendothelium through VWF or fibronectins and prevents bleeding by forming platelets plugs.
Platelets release factors such as I, V, VII to facilitate coagulation, and for this reason, are called thrombocytes. They also release heparin neutralizing proteins (PF4, TG) that help from clots at the injured sites.
Finally, they participate in clot retraction through contractile proteines, specifically thrombostenin.

Multiple lymphomatous polyposis (M L P) is a rare type of gastrointestinal lymphoma presenting as multiple polyps involving large segments of the bowel. The lesions have been classified by immuno ­histochemical methods as • Mantle Zone Lymphoma' .
According to our knowledge, no case of M L P has been reported in Iran.
The 29 years old male who presented in this paper, is the youngest patient that has been reported in medical literature.

Weight loss resulting from the hypermetabolic response to burn injury is not unusual and is often unavoidable. The loss of retroperitoneal fat has been postulated as a major factor in the cause of the uncommon complication of superior mesenteric artery syndrome. This syndrome is frequently treated nonoperatively with aggressive nutrition support.
Nasojejunal feeding past the point of obstruction should be considered as the primary method of nutrition support. Alternatively, total parenteral nutrition or a combination of enteral and parenteral feeding may be necessary to meet nutritional needs until the duodenal obstruction resolves. This case study described the nutrition management of a burn patient who developed superior mesenteric artery syndrome.