Nekroza potkožnog masnog tkiva kod dojenčeta

Subcutaneous fat necrosis of the newborn and infant is a rare disease, with still unknown incidence, which usually occurs in term or post-term newborns that have experienced perinatal stress. It usually occurs within the fi rst six weeks of newborn’s life; however, onset of the disease may be delayed for several months. A 6-week-old female infant was admitted to our department due to failure to thrive, irritability and vomiting. Physical examination in the area of the inner thighs, hips, back and shoulders, revealed the presence of subcutaneous infi ltrations, which were fi rm, slightly livid, and did not seem painful to touch. Laboratory analysis showed hypercalcemia, ultrasonographic review of body fat revealed hyperechogenicity, while abdominal ultrasound revealed nephrocalcinosis. Computerized tomography detected the presence of calcifi cations in the brain. Deep skin biopsy confi rmed the diagnosis of subcutaneous fat necrosis. Treatment included fl uid loading, termination of vitamin D substitution, and low calcium diet. Single doses of calcitonin and pamidronate were administered. After this therapy, calcium levels returned to normal range. Subcutaneous infi ltrates gradually decreased and became softer. In most reported cases, regression of skin lesions is expected after a few months, often without any residue on the skin. Elevated serum calcium may persist long after the withdrawal of cutaneous lesions, which is the reason for continuous monitoring of serum calcium and appropriate treatment in case of hypercalcemia in order to prevent metastatic calcifi cation.Nekroza potkožnog masnog tkiva kod novorođenčeta i dojenčeta je rijetka bolest još uvijek nepoznate incidencije, koja se obično javlja kod novorođenčadi rođene u terminu ili poslije termina izložene perinatalnom stresu. Najčešće nastaje u prvih šest tjedana djetetova života, no moguć je i odgođeni nastup bolesti za nekoliko mjeseci. Žensko dojenče staro 6 tjedana primljeno je na naš odjel zbog slabog napredovanja, razdražljivosti i povraćanja. Fizikalni pregled otkrio je na unutarnjem dijelu bedara, bokovima, leđima i ramenima potkožne infi ltrate koji su bili tvrdi, blago modrosivi i nisu se činili bolnima na dodir. Laboratorijske pretrage pokazale su hiperkalcemiju, dok je ultrazvučni pregled tjelesne masti otkrio hiperehogeničnost, dok je ultrazvuk abdomena pokazao nefrokalcinozu. Kompjutorizirana tomografi ja otkrila je prisutnost kalcifi kacija u mozgu. Duboka kožna biopsija potvrdila je dijagnozu nekroze potkožnog masnog tkiva. Liječenje je provedeno opterećenjem tekućinom, uz prestanak nadomještanja vitamina D i prehranu s niskim sadržajem kalcija. Dijete je dobilo po jednu dozu kalcitonina i pamidronata. Nakon ove terapije razine kalcija vratile su se na normalu. Potkožni infi ltrati postupno su se smanjili i omekšali. U većini opisanih slučajeva regresija kožnih promjena očekuje se kroz nekoliko mjeseci, često bez ikakvih zaostalih tragova na koži. Povišena razina kalcija u serumu može potrajati još dugo nakon što se kožne promjene povuku pa je potrebno stalno praćenje serumskog kalcija i odgovarajuće liječenje u slučaju hiperkalcemije kako bi se spriječila metastatska kalcifi kacija

Persistent wheezing as manifestation of esophageal tubular duplication cyst

Duplications of esophagus are rare congenital anomalies and the second most common duplications of the gastrointestinal tract. This form of bronchopulmonary foregut malformation may appear as a cystic mediastinal mass. On chest radiographs they may be visible as middle or posterior masses. On CT they are well marginated and oppose the esophagus. Usually they are asymptomatic, unless they become infected or cause obstruction. We report a case of thoracic tubular duplication cyst in a 10-month-old boy who presented with persistent wheezing that was unsuccessfully treated in out-patient services

Evaluation of surfactant replacement therapy effects: A new potential role of lung ultrasound

Introduction. Previous studies suggested that effects of the surfactant administration in preterm infants with respiratory distress syndrome cannot be followed by lung ultrasound (L-US). Objective. The aim of the paper is to evaluate the surfactant replacement therapy effects using a new, proposed grading system for L-US findings. Methods. We report the series of 12 preterm infants with clinical and radiographic signs of respiratory distress syndrome, in whom L-US examinations were performed prior to, and within the first 24 hours after surfactant administration. To evaluate the surfactant replacement therapy effects, we proposed a new grading system (1 to 6) for L-US findings at each examined lung area, based on the presence of normal finding, the amount of B-lines and subpleural consolidations. Results. All preterm infants had an improvement of L-US findings from one to four grades observed within the first 24 hours after surfactant administration, which has not been previously reported. The improvement of L-US findings was most commonly observed in anterior lung areas. Conclusion. L-US might enable an early detection of the surfactant replacement therapy effects. Further prospective studies are necessary to define the role of L-US in this field

Sclerosing Pneumocytoma: A Ten-Year Experience at a Western Balkan University Hospital

Background and objective: Sclerosing pneumocytoma is a rare, benign tumor of the lung that represents a diagnostic challenge due to the diversity of pathohistological findings. The aim of this study was to present a 10-year experience with sclerosing pneumocytoma of a large center for the diagnosis and treatment of pulmonary diseases, and to emphasize differential diagnostic dilemmas as a potential source of errors. Material and Methods: This represents a retrospective study of six patients diagnosed and treated with sclerosing pneumocytoma in the 10-year period. The study analyzed various parameters, which are: Sex, age, symptoms, size and localization of the tumor, and its gross and histological features. Results: Sclerosing pneumocytoma was more frequently diagnosed in females (83.34%). The patients ranged in age from 38 to 61. Most of the patients (66.66%) were asymptomatic. Two patients underwent a video-assisted thoracoscopic surgery, two patients had a video-assisted minithoracotomy, and two patients underwent a thoracotomy in order to remove the tumor. The tumor was localized in the left lower lobe, in the right upper lobe, and in the right lower lobe in 50%, 33.34%, and 16.66% of patients, respectively. The tumor size ranged from 1 to 2.5 cm. A pathohistological examination of all six cases reported that all four major histological patterns were found in tissue sections: solid, papillary, sclerosing, and hemorrhagic. In all six cases, an immunohistochemical analysis showed positive expression of TTF-1 and panCK in surface epithelial cells, and TTF-1 positivity and panCK negativity in round stromal cells. Conclusions: Sclerosing pneumocytoma is a strictly histological diagnosis supported by clinical and radiological findings and corresponding immunohistochemical methods. Lung pathologists should always keep this tumor in mind, since its spectrum of differential diagnosis is wide, and therefore it can be an important diagnostic pitfall

A case of severe type of cerebro-costo-mandibular syndrome

Introduction. Cerebro-costo-mandibular syndrome (CCMS) is a rare disorder, with only 75 cases described in the literature to date. CCMS is characterized by association of micrognathia and specific multiple rib defects. It is accompanied by mental deficiency in considerable number of cases. Sometimes, there are associated anomalies and problems, such as spine deformities, brain, heart, kidney or ear anomalies, feeding difficulties, delayed psychomotor development, and growth impairment. Depending on severity of deformities and consecutive respiratory insufficiency, in about 35-50% of CCMS cases, death occurs during the first year of life. These cases are referred to as severe types of CCMS. Case Outline. In this paper we present a female infant with severe type of CCMS. Diagnosis was established in the first day of life, based on micrognathia and findings of posterior rib-gap defects on the chest X-ray, accompanied by dyspnea. Progressive severe respiratory insufficiency caused by chest and air-way deformities and exacerbated by episodes of pneumonia, led to respiratory failure and death at the age of 7.5 months. Conclusion. CCMS should be considered in every infant with micrognathia and rib-gap defects on chest X-ray