Juvenile Nasopharyngeal Angiofibroma

Review of the literature was performed to define the optimal treatment of patients with juvenile nasopharyngeal angiofibroma (JNA). The prognosis for this disease is extremely good if diagnosed well in time and if the tumor has not extended intracranially. Preoperative selective arterial embolization has decreased intraoperative blood loss and facilitated resection of larger tumors. Transnasal endoscopic resection preserves both the anatomy and physiology of the nose, requires less rehabilitation days after surgery, and is highly successful for selected patients. Radiation therapy is generally reserved for larger and/or unressectable tumors but has severe complications. Radiosurgery has several advantages over surgery or classic radiation therapy. However, further experiences and studies are required to confirm the usefulness of radiosurgery on JNA

Subcutaneous fat necrosis with hypercalcemia

Cilt altı yağ nekrozu nadir görülen; gövdede, kollarda, bacaklarda ve yanaklarda ağrılı, eritematöz plak ya da nodüller şeklinde ortaya çıkan yağ dokusunun inflamatuvar iyi huylu hastalığıdır. Cilt altı yağ nekrozu olan yenidoğanlarda sıklıkla asfiktik doğum, mekon- yum aspirasyonu, hipoksi, hipotermi, lokal travma öyküsü mevcuttur. Kendi kendini sınırlayan bir hastalık olmakla beraber ağır hiperkalsemi, hiperlipidemi, geçici trombositopeni gibi komplikasyonlara yol açabilir. Özellikle yaygın cilt tutulumu olan hastalarda hiperkalsemi hayatı tehdit eden bir komplikasyondur. Hiperkalsemiye maruziyet süresi uzarsa metastatik kalsifikasyonlar, nefrokal- sinosis ve böbrek yetmezliği, kalp problemleri ortaya çıkabilir. Bu yazıda bebeklik döneminde hiperkalsemin eşlik ettiği cilt altı yağ nekrozu vakası sunulmuştur.Subcutaneous fat necrosis is a rare and bening inflammatory disease of adipose tissue. It manifests as painful erythematous plaques and nodules in trunk, extremities and cheeks. Neonates with subcutaneous fat necrosis, often had a history of asphyxia ,meconium aspiration, hypoxia, hypothermia and local trauma. Although it is a self-limiting disease, it can lead to complications such as severe hypercalcemia, hyperlipidemia, and transient thrombocytopenia. Especially in patients with extensive skin involvement, hypercalce- mia is a life threatening complication. If exposure to hypercalcemia takes a long time, metastatic calcification, nephrocalcinosis and renal failure, cardiac problems may occur. In this article, a case of subcutaneous fat necrosis accompanied by hypercalcemia in an infant was reported

Assessment of nutritional status: Triceps and subscapular skin-fold thickness in Turkish children and adolescent

Objectives: To determine the mean and percentile values and constitute the percentile curves of the triceps and subscapular skin-fold thickness (SFT) for obtaining a measure to be used in evaluating nutritional status of children and adolescents for age and gender to determine the threat of adiposity in Turkish children. Methodology: This cross-sectional study was carried out between October 2006 and May 2007 with 6917 students selected among those with pre-defined socio-economic criteria and attending primary schools in Van city center. Based on these data, the subjects were distributed to age groups in 6-month intervals, beginning from the age of 7 up to the age of 17. Results: Analysis of mean values of the thickness of triceps and subscapular SFT according to age in boy subjects demonstrated that these values generally increased with age up to thirteen years of age where peak values were observed, and the thickness decreased between 14 and 15 years of age, increasing again thereafter. A comparison of our data with the data reported from other countries revealed lower than median values of triceps and subscapular SFT in our study for both girls and boys regardless of the age group. Conclusions: Subcutaneous fat accumulation is lower in Turkish children compared to those in other countries and no risk of obesity is imminent, at least in the region where this study was carried out

Growth Patterns of Children of Same Geographic Background Reared in Different Environments

Objective: Growth charts are essential tools used to assess children's health status. The aim of the present study was to determine the effect of environmental factors on the growth of children of a common geographic background. We constructed growth charts for children living in the East of Turkey and compared them with those for Turkish children living in other regions or countries

A case of Bartter’s syndrome associated with nephrocalcinosis presenting with tetany

A 10-year-old boy was admitted to hospital with a 3 month history of intermittent spasms of the wrists and ankles, and twitching of the eyelids. He also had polyuria, polydipsia, nocturnal enuresis, fatigue and constipation since he was a toddler. Physical examination revealed normal blood pressure, myokymia on the right eyelid and bilateral carpopedal spasms. Laboratory investigation revealed hypocalcemia, hypokalemia, increased plasma renin and aldosterone , hypercalciuria, metabolic alkalosis, and bilateral medullary nephrocalcinosis. Cranial computed tomography was normal. Based on the clinical and laboratory findings he was diagnosed as having Bartter’s syndrome, which is characterized by hypochloremia, hypokalaemia and metabolic alkalosis associated with potassium renal leakage, with normal blood pressure despite increased plasma renin activity. It is well known that tetany is not uncommon in the neonatal form of Bartter’s syndrome and nephrocalcinosis is usually not present in the classic form. Interestingly, our patient had both the clinical manifestations of the neonatal form and of the classic form of Bartter’s syndrome. In conclusion, we would like to emphasize that both the clinical manifestations of neonatal and classic forms of Bartter’s syndrome (as an overlapping syndrome) might be seen in children and that Bartter’s syndrome should also be considered in children with tetany as in our case. (J Pediatr Neurol 2004; 2 (1): 45-47)

Infantile digital fibromatosis (inclusion body fibromatosis) observed in a baby without finger involvement

A 9-day-old male baby was hospitalized after his birth due to some swells under the skin. The hard consistency nodules observed under the skin all over the body of the patient were of different size, and presented lesions, among which the biggest was 1 × 1 cm. No lesions were observed on the fingers. By superficial ultrasonography, multiple isoechoic hypoechoic lesions were observed among the muscle plan. In thoracolumbar magnetic resonance imaging, multiple massif lesions retaining peripheral contrast (the biggest was 1.7 × 1.4 cm large) had been observed under the skin muscle plans, between the muscles of the extremities. The biopsy was positive for smooth muscle actin, but negative for desmin, S100, and CD34. These findings were diagnosed as infantile digital fibromatosis (IDF) (inclusion body fibromatosis). The case was presented with an objective to illustrate and remind that IDF can be observed in babies without finger involvement

Acute leukemia case presented with hypercalcemia

An 8-year-old girl patient referred to our emergency clinic with articular pain, stomachache and fever complaints. Past history revealed that she was suffering from pain in both knees and ankle joints for 8 days. The joint temperature increased and swelling did not accompany articular pain. Family history was unremarkable. In the physical examination, there was sensitivity in the knees, elbows and ankles during movement. The patient had normal complete blood cell count, and no blast or atypical cells were observed in peripheral smear. Serum electrolytes, liver and kidney function tests were normal except for hypercalcemia. The 25 (OH) vitamin D and 1-25 (OH)2 vitamin D levels were within normal range. In bone marrow aspiration, infiltration of cells with lymphoblastic and homogenous cellular features was observed. With positivity of cCD79, CD19, CD45, the case was considered as preB cell leukemia. Body bone scintigraphy performed for bone metastasis was normal. After the chemotherapy, hydration and furosemid treatment, the calcium level returned to normal. This case emphasized on the fact that, children with hypercalcemia should undergo a detailed examination for malignancies even though no blast or atypical lymphocyte are observed in their peripheral blood smear before steroid treatment is applied and if necessary, bone marrow aspiration should be taken into account.</span