ABSTRACT
Interstitial lung disease in infants, unlike older children and adults, has diverse etiology, including infective, metabolic, autoimmune,genetic, malignant and idiopathic causes. Clinical recognition of the interstitial pattern of lung involvement is important as the etiology andmanagement is entirely different from that of recurrent or chronic lung parenchymal pathologies. We discuss the clinical and pathologicalfindings of an infant with interstitial pneumonia, who succumbed to hospital-acquired sepsis.
ABSTRACT
Background & objectives: Information on gastrointestinal manifestations and then response after curative parathyroid surgery is scarce in symptomatic primary hyperparathyroidism (PHPT). This study was carried out to analyse gastrointestinal manifestations in patients with PHPT and their associations with biochemical parameters. Methods: This retrospective study included 153 patients with symptomatic primary hyperparathyroidism (PHPT). The signs and symptoms pertaining to gastrointestinal system were analyzed. The difference of symptoms between men and women and difference in biochemical parameters in presence of different symptoms were evaluated. The relationship between serum calcium, phosphate and parathyroid hormone (PTH) levels with presence of gallstone and pancreatitis was also studied. Result: Of the 153 patients, 46 (30%) were men. The mean age was 39.2 ± 13.9 yr. Nearly 80 per cent of PHPT patients had at least one symptom/ sign related to gastrointestinal system. The most common gastrointestinal manifestations were abdominal pain 66 (43%), constipation 55 (36%), and nausea/or vomiting 46 (30%). Nearly one-fourth 34 (22%) of patients had a history of either gallstone disease or cholecystectomy or both. The prevalence of gallstone disease was higher in women (P<0.05). Imaging and biochemical evidence of pancreatitis was found in 27 (18%) patients. Pancreatitis was more common in men compared to women (P<0.05) despite the higher prevalence of gallstones in women. Serum calcium, phosphate or PTH levels were not associated with high risk for gallstone disease, however, serum calcium (P<0.05) was associated with 1.3 times higher risk of developing pancreatitis. In majority of patients, gastrointestinal manifestations resolved within three months of curative parathyroidectomy. Except two patients, none had recurrence of pancreatitis. Interpretation & conclusions: The study revealed that the gastrointestinal symptoms were common in patients with symptomatic PHPT. There was not much gender difference in gastrointestinal symptoms except higher occurrence of gallstones in women and pancreatitis in men. There was no difference in biochemical profile between those who had and did not have gastrointestinal symptoms.
ABSTRACT
Background & objectives Fibrous dysplasia (FD) is a rare metabolic bone disease and information available from India is limited to only anecdotal case reports. We describe the clinical profile and therapeutic outcome of 25 patients with FD observed over a period of 14 yr in a tertiary care centre from north India. Methods In this retrospective study patients (n = 25) with diagnosis of fibrous dysplasia based on either classical radiological features and/or histological evidence on bone biopsy, were analyzed. Associated endocrinopathies if any, were evaluated. The diagnosis of McCune Albright syndrome (MAS) was considered when fibrous dysplasia was accompanied by either café-au-lait macules and/or endocrinopathies. The clinical presentation, biochemical parameters and imaging were analysed. Seven patients received bisphosphonate therapy. The final outcome and side effects were noted. Results Age of the patients ranged from 7 to 48 yr (mean ± SD, 24.2 ± 11.4 yr) with a lag time between onset of symptoms and presentation ranging from 1 to 20 yr (mean ± SD, 6.6 ± 6.2 yr). The mean duration of follow up was 3.5 ± 2.1 yr. Eighteen (72%) patients had polyostotic disease while the remaining had monostotic FD. Eight patients had endocrinopathies: five had acromegaly, one each had gonadotropin independent precocious puberty (GIPP), hyperthyroidism and hypophosphatemic rickets. One child with GIPP later developed hyperthyroidism. McCune Albright syndrome was observed in 10 (40%) patients. A majority of the patients underwent various minor or major surgical procedures and seven patients received bisphosphonates for recurrent pathological fractures. Bone pain was reduced in all bisphosphonate treated patients with a decrease in subsequent fractures. Interpretation & conclusions This series of FD patients from north India shows the varying presentations of this rare disease. Medical treatment with bisphosphonates appears to be potentially rewarding.