Simultaneous three-dimensional correction of percutaneous radial osteotomy with circular external fixator in Madelung's deformity.

Partial deficiency of distal radial and volar cartilage plate growth is the main pathology in Madelung's deformity. Surgical treatment can use a Taylor Spatial Frame (TSF) external fixator, which provides simultaneous multiplanar correction and lengthening after radial osteotomy, sparing Vickers' ligament and the ulna. We sought to evaluate the radiological and functional results of Madelung's deformity correction by TSF, by retrospectively analyzing eight Madelung's deformities in seven patients between August 2011 and May 2015. Mean age was 14.8 years (range, 11-26), and mean follow-up 35.1 months (24-78). We evaluated demographic data and pre- and post-operative radiological and functional results. Mean preoperative visual analog pain scale score improved from 7.7 to 1.6. Mean preoperative DASH score improved from 19.1 (9.5-46.6) to 3.7 (2.3-6.8). Mean preoperative radial length improved from 184 mm (138-209) to 196 mm (142-213). We think that software-based correction using a TSF is a safe solution in Madelung's deformity, with minimal margin of error and risk of postoperative stiffness and infection. LEVEL OF EVIDENCE: IV.

The role of interventional pulmonology for the postoperative bronchopleural fistula.

BACKGROUND: : Postoperative bronchopleural fistula (BPF) remains a serious complication due to its high morbidity and mortality. Although various endoscopic techniques have been defined for the closure of BPF previously, no standard algorithm yet exists. AIMS: To study the effectiveness and safety of various endoscopic procedures in an interventional pulmonology unit. MATERIALS AND METHODS: The medical data of 15 postoperative BPF patients, who were undergone endoscopic intervention were retrospectively investigated. RESULTS: The mean size of the fistulas determined by bronchoscopic evaluation was 7.93 ± 3.26 mm (range 3-15 mm). Applied procedures were as follows: stent implantation (n: 8, 53.3%), stent implantation and polidocanol application (n: 4, 26.7%), only Argon Plasma Coagulation (APC) application (n: 2,13.3%), polidocanol application (n: 1,6.7%). Complete fistula closure was achieved in three of the 15 patients (20%). The procedures were partly successful in five (33.3%) patients and failed to be successful in seven (46.6%) patients. Survival rates in regard to procedural success were determined and a statistically significant difference was found in five-year survival rates (P = 0.027, P < 0.05). CONCLUSION: Our results demonstrated that bronchoscopic procedures can be safely and effectively performed in patients who were not eligible for surgery for various reasons.

Diode laser in the endoscopic treatment of obstructive airway disease: two years experience in Istanbul.

AIM: Laser therapy is a well-known technique which has been safely used for almost thirty years to treat endobronchial obstruction. Nd : YAG and Nd : YAP lasers are commonly used lasers, whose safety and efficacy have been investigated in several large series. Diode laser is a new laser system which has been mainly used in urology, endovascular surgery, ENT, and dermatology. In recent years it has been effectively used in endobronchial treatments. MATERIAL AND METHOD: In this study, 61 patients who underwent endobronchial treatment with a diode laser in the Interventional Pneumology Unit of our hospital were retrospectively evaluated. Fifty-one patients were male. Mean age was 54.72 ± 13.81 years, and a total of 90 laser applications were given. In 39 cases, palliation of a malign obstruction was achieved while cure was achieved in 19 cases with benign obstruction. No major complication other than minor hemorrhage in 4 cases and temporary hypoxia in 2 cases was observed. CONCLUSION: Diode laser can be used as an effective and safe modality for endobronchial treatment.

Results of tetanus vaccination in sarcoidosis.

BACKGROUND AND OBJECTIVE: Cellular immunity abnormalities are associated with sarcoidosis. Normal cellular immunity is required for adequate humoral immunity; therefore, a decreased humoral immune response is possible in patients with sarcoidosis. We evaluated humoral immunity by vaccinating patients with sarcoidosis against tetanus. PATIENTS AND METHOD: We screened 60 patients with sarcoidosis (42 females, average age 39 +/- 11 years) and 40 healthy subjects as a control (23 females, average age 38 +/- 9 years). Of the 51 sarcoidosis patients and 33 controls that did not have sufficient tetanus antibody titers, 48 patients and 31 controls agreed to be vaccinated and were included in the vaccination program. Blood serum samples were collected from the subjects before and after vaccination and evaluated for tetanus toxoid IgG antibodies with an enzyme-linked immunosorbent assay (ELISA). RESULTS: As a result of the vaccination, 24 of the sarcoidosis patients (50%) and 7 of the controls (23%) had insufficient antibody responses (p = 0.019). No relationship was found in sarcoidosis patients between the rate of having sufficient antibody levels and disease duration, activation state, and radiographic staging of the disease. Conversely, mean lymphocyte numbers were significantly lower in patients with insufficient tetanus antibody levels (p = 0.013). CONCLUSION: Tetanus vaccinations in sarcoidosis patients are less effective than in healthy controls, suggesting that patients with sarcoidosis have a hyporesponsive humoral immune system.

[Total atalectasis of the left lung developing during the third month of treatment in a case of pulmonary tuberculosis]. / Un cas de tuberculose pulmonaire avec atélectasie totale du poumon gauche au troisième mois du traitement.

INTRODUCTION: Pulmonary tuberculosis and lung cancer are still important public health problems and can occur simultaneously. In this article, we present the case of a 38-year-old patient treated for smear positive pulmonary tuberculosis. CASE REPORT: During the third month of treatment, the patient developed respiratory distress and was found to have total atelectasis of the left lung. At rigid bronchoscopy, a lesion obstructing the left main bronchus was removed with a diode laser. Oncological treatment was started following the histological diagnosis of small cell bronchial carcinoma. CONCLUSION: Pulmonary tuberculosis and bronchial carcinoma can occur at the same time and cause diagnostic confusion. The possibility should be considered in situations where both diseases are endemic.

An outstanding non-transplant surgical intervention in progressive familial intrahepatic cholestasis: partial internal biliary diversion.

AIM: Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease with severe cholestasis progressing to cirrhosis and chronic renal failure usually during the first decade. An alternative approach is partial diversion of bile. The aim of this study is to describe four patients with PFIC who underwent partial internal biliary diversion (PIBD). METHODS: Review of three patients, their clinical, laboratory and histologic workups to evaluate the short-term effects of PFIC, a 1-year follow-up. For PIBD, a conduit is performed between the terminolateral side of the gall bladder and distal colon using a segment of jejunum, to divert the biliary flow from the enterohepatic cycle without any external stoma. RESULTS: All four patients were presented with jaundice, pruritus, hepatomegaly, sleep disturbance. They fulfilled the criteria for PFIC. The surgery was uneventful. At follow-up, biochemical parameters improved significantly, growth was regained, relief in pruritus, sleeping pattern was normalized. CONCLUSIONS: Partial internal biliary diversion had a dramatic effect on cholestasis, growth, sleeping and biochemical parameters. It also avoids the disadvantages of a permanent stoma. We believe that it is one of the best surgical procedures ever described for PFIC. Since long-term results of partial external biliary diversion on liver histopathology are successful, we hope that our long-term results will also be similar.

Comparison of the QuantiFERON-TB Gold In-Tube test with the tuberculin skin test for detecting latent tuberculosis infection in hemodialysis patients.

BACKGROUND AND OBJECTIVES: Hemodialysis (HD) patients are at increased risk of reactivation of latent tuberculosis (TB) infection (LTBI). LTBI screening of this population is recommended. The QuantiFERON-TB Gold assay (QFT-G) may be more accurate than the tuberculin skin test (TST) in the detection of LTBI. We prospectively compared the results of QFT-G to TST in HD patients. METHODS: We examined 100 patients and performed TST and QFT-G tests. Data obtained from patients and medical records included medical history (past history of TB, Bacillus Calmette-Guerin [BCG] vaccination, history of contact with previous TB cases), radiography reports (chest x-ray with changes consistent with old TB), and basic laboratory findings. RESULTS: Forty-three of 100 patients (43%) had a positive QFT-G test result and 34 (34%) had a positive TST test result. Overall agreement between the QFT-G and the TST was 65% (concordance [k]=0.26, P=0.01). Discordant test results were seen in 13 TST-positive/QFT-G-negative patients and in 22 TST-negative/QFT-G-positive patients. Before BCG vaccination and radiographic reports (of old TB changes) were associated with discordant test results. On multivariate analysis, a positive QFT-G test was associated with contact with previous TB cases (P=0.026) and radiographic report (P=0.034), whereas a positive TST test also was associated with a history of BCG vaccination (P=0.015). CONCLUSIONS: QFT-G test results were more closely associated with TB risk factors than were positive TST results. Additionally, the QFT-G test was not affected by BCG vaccination. We concluded that QFT-G test is a more useful diagnostic method than TST for detecting LTBI in HD patients.

Celiac disease and glycogenic acanthosis: a new association?

UNLABELLED: A 6-y-old boy and an 8-y-old girl were admitted to our clinic with anaemia and failure to thrive. Laboratory tests revealed iron deficiency anaemia and positive antigliadin antibodies in both of the patients. Slightly raised grey-white plaques were observed on oesophageal mucosa during endoscopical investigation of the patients. While intestinal mucosal samples confirmed diagnosis of celiac disease histologically, histopathological assessment of oesophageal lesions demonstrated glycogenic acanthosis. Since glycogenic acanthosis associated with celiac disease hasn't been reported in the literature previously to our knowledge, case reports of our patients were presented. CONCLUSION: We suggest that glycogenic acanthosis needs to be investigated as a possible new association of celiac disease in greater paediatric series.

HLA-DQ alleles in patients with celiac disease in Turkey.

The prevalence of the HLA-DQA1 and DQB1 alleles in 55 Turkish children with celiac disease and 50 control subjects was investigated by using an allele-specific DNA-based polymerase chain reaction-sequence-specific primer (PCR-SSP) method. The frequency of the DQA1*0501 and DQB1*02 alleles was higher in celiac patients than in controls. The DQA1B1 (*0501; *02) haplotype was present in 46 (83.6%) patients and only in 12 (24%) controls. The remaining 9 celiac patients which were negative for DQA1B1 (*0501;*02) carried the DQA1B1 (*03;*0302) haplotype. We found an excess homozygosity of the DQB1*02 allele and the DQA1B1 (*0501;*02) haplotype in the patients. No statistically significant correlation was found between the homozygosity of this haplotype or the DQB1*02 allele and an earlier onset of the disease.

Carbohydrate malabsorption in acute diarrhea.

A group of 245 well nourished infants with acute diarrhea were screened for carbohydrate malabsorption by evaluating stool pH and reducing substances in the stools. Carbohydrate malabsorption was diagnosed in 28 cases (11%). Clinical features of carbohydrate intolerance were present in only one case. The duration of diarrhea after admission ranged from 1 to 13 days (mean 3.9 days). An oral lactose tolerance test was consistent with lactase deficiency in 32% of all cases. Thin layer chromatography showed many carbohydrates including monosaccharides in the stools, indicating that the defect in intestinal absorption was not specific for lactose.

Oral rehydration therapy: studies on potassium balance.

The clinical response and changes in potassium balance were studied during oral rehydration therapy (ORT) with a solution containing 20 mmol potassium per litre in well-nourished and malnourished infants of 3 to 15 months of age as well as in neonates and young infants less than 2 months old (range 5-60 days). All infants were successfully rehydrated orally. The potassium intake considerably exceeded the potassium output in all groups during the entire rehydration period. The stool and urine potassium output were about the same in the 3 groups of patients. The retention of potassium increased during ORT in all groups at about the same rate. The study thus demonstrates that an oral rehydration solution (ORS) containing 20 mmol potassium per litre provides enough potassium to all patients. An increase in the potassium concentration of the ORT is therefore not recommended.

Salt and water homeostasis during oral rehydration therapy in neonates and young infants with acute diarrhoea. II. Rehydration with a solution containing 90 mmol sodium per litre (ORS90).

The clinical response and changes in water and salt homeostasis were studied during oral rehydration therapy (ORT) with a solution containing 90 mmol sodium per 1 (ORS90) in 9 infants less than 2 months old (range 2-60 days). Two infants were still dehydrated 36 hours after starting ORT and were excluded from the study. Fluid was retained more rapidly and also to a larger extent than in infants of the same age treated with a solution with a sodium concentration of 60 mmol/l (ORS60). The stool sodium output was higher than that found previously in infants of the same age treated with ORS60. We conclude that during ORT the gut plays an active role in the regulation of salt homeostasis. When the sodium intake is high the percentage of sodium remaining unabsorbed is higher than when the intake is low. This mechanism reduces the risk of hypernatremia in young infants treated with ORS90. The study thus demonstrates that ORS90 is effective and also seems to be safe in the treatment of neonates and young infants with dehydration secondary to diarrhoea if fluid intake is kept around 200 ml/kg/day during the rehydration period.

Oral rehydration therapy in neonates and young infants with infectious diarrhoea.

The clinical response and changes in water and salt homeostasis during ORT was studied in 15 infants less than 2 months old (range 2-50 days) with acute diarrhoea. Eight patients were neonates and 7 were 1-2 months old. The oral rehydration solution contained 60 mmol sodium per litre. All patients except one were successfully rehydrated. The fluid retention was significantly higher in neonates and young infants than in infants above 3 months of age treated in the same way. One patient in the group of neonates who had a normal sodium level on admission developed hypernatremia with a sodium level of 162 mmol/l 36 hours after the start of ORT. The urinary sodium excretion was lower in the neonates than in the young infants. The results show that neonates and young infants have a lower capacity than older infants to excrete water and salt and therefore run a great risk of developing fluid and salt retention during ORT. The risk is most pronounced in neonates who, due to immaturity of the renal function, are unable to excrete excess fluid and salt.

A comparative study of size and function of the remnant kidney in patients nephrectomized in childhood for Wilms' tumor and hydronephrosis.

To obtain more information about the natural history of compensatory renal hypertrophy beginning in childhood we traced those who were nephrectomized in childhood for Wilm's tumor (W) and hydronephrosis (Hn) between 1950 and 1978 at one department of surgery in Stockholm. All W patients had received treatment that suppresses cellular division. None of the patients were in renal failure or treated with antihypertensive drugs. All the patients in the follow-up study (22 W, 15 Hn) had a normal contralateral kidney at nephrectomy. Five healthy adults served as controls. The kidney was enlarged in both Hn (142%) and W (125%), but significantly larger in Hn than in W. Renal compensatory growth in W was retarded during the first two years after nephrectomy. The glomerular filtration rate (GFR) was 92% of control in Hn and 82% of control in W. The GFR did not seem to decline with a longer follow-up time in any of the groups. PAH clearance was the same in Hn and W. Albumin excretion was significantly higher in Hn than in W, but not significantly higher in W than in controls. The highest albumin excretion rates were found among the Hn patients with long follow-up time. The results suggest that the large increases in size and function that follow childhood nephrectomy can be blunted by antimitotic agents.

Oral rehydration therapy in malnourished infants with infectious diarrhoea.

The clinical response and changes in water and salt homeostasis was studied for 36 hours during oral rehydration therapy with a rehydration solution containing 60 mmol sodium/l (ORS60) in 14 malnourished 3- to 15-month-old Turkish infants with acute infectious diarrhoea. All patients were successfully rehydrated with this treatment. Sodium was efficiently absorbed from the gut and water balance was rapidly restored. Because of excess fluid retention following the initial rehydration period about 50% of the patients became oedematous. Urine volume and urinary sodium excretion were found to be much lower than in well-nourished patients of the same age with acute diarrhoea who were treated in the same way. In all of the malnourished infants the serum sodium level remained within the normal range during treatment. The results show that malnourished infants retain much more fluid and sodium than infants who are in a normal nutritional state. Excessive retention of water and salt seem to be due to an inability of the kidneys to control sodium and fluid homeostasis while orally administered sodium and fluid are being absorbed from the gut. The results show that ORT is safe and efficient in the treatment of malnourished infants with acute diarrhoea. But since these infants run a high risk of developing a severe retention of fluid and salt, and consequently may develop circulatory failure due to hypervolaemia during oral rehydration therapy, it is important to carefully monitor the volume of fluid that is given.

Oral rehydration therapy in infectious diarrhoea. Comparison of rehydration solutions with 60 and 90 mmol sodium per litre.

The clinical response and changes in water and salt homeostasis as judged from serum sodium levels, salt and water retention and renal handling of sodium was studied during 36 hours following the start of oral rehydration therapy (ORT) with a solution containing 60 mmol Na/l (ORS60) in 17 well-nourished, moderately dehydrated Turkish infants aged 3 to 15 months who had acute infectious diarrhoea (7 with rotavirus, 3 with enteropathogenic E. coli 0 111: B 84, and one with enteropathogenic E. coli 0 125: B 15, one with salmonella and 5 of unknown etiology. In the successfully treated patients sodium and water balance was normalized within 36 hours. In the cases with hypernatremic dehydration the serum sodium concentration rapidly became normal. The results were compared with those obtained in a previous study of the same type of patients who were rehydrated with a solution containing 90 mmol Na/l (ORS90). Although retention was considered to be satisfactory after ORS60 it was less than after ORS90. The changes in the fractionary urinary sodium excretion and the potassium sodium quotient in the urine indicated a less rapid normalization after ORS60 than after ORS90.

Unsuccessful oral rehydration therapy in an infant with enteropathogenic E. coli diarrhoea. Studies of fluid and electrolyte homeostasis.

A 4-month-old male infant with severe hyponatremic dehydration due to an enteropathogenic E. coli O125: B15-induced diarrhoea had continued very high stool fluid output with a very elevated sodium concentration after hospitalization and the institution of oral rehydration therapy (ORT). Thirty-six hours after start of ORT intravenous therapy was required. The results of studies of fluid and salt homeostasis in this patient have been compared with those obtained in 3 other patients who had acute diarrhoea of the same severity but caused by another strain of enteropathogenic E. coli (O111:B14) and who were successfully treated with ORT. On ORT the patient with treatment failure had a stool volume which was almost 8 times larger and a stool sodium output which was about 5 times higher than in the successfully treated patients. During the 36-hour-period of ORT fluid losses were about the same as the fluid intake. The results as regards urinary fractional sodium excretion and the urinary potassium/sodium quotient indicate that the severe sodium depletion which was present on admission in the unsuccessfully treated patient persisted during ORT. The reason for ORT failure may be that the infectious E. coli strain had bacilli-adherent qualities that cause damage of microvilli.