ABSTRACT
Introduction: In 1977 Birt, Hogg and Dube described Birt-Hogg-Dubé syndrome (BHDS)as an dermatological syndrome.It is an autosomal dominant inherited disease, the gene responsible for the syndrome was cloned in 2002. Only 663 affected families have been reported in literature, To date, 152 unique pathogenic FLCN gene mutations in 616 families have been reported in worldwide;approximately 90% of these mutations were reported in Europe and the United States. It consists of the typical triad - skin lesions, pulmonary cysts, and renal tumors. This syndrome usually goes underdiagnosed since all these different clinical features treated by different specialities. This leads to longdelay in making the correct diagnosis from the first onset of the symptoms. There are many studies which have shown that the prevalence spontaneous pneumothorax ,bullous emphysema, thin-walled cysts are increased in these patients. Case report: A 39 year old male presented with complaints of mild fever for 5 days duration and shortness of breath for 1 day. Fever was continuous in nature, not associated with chillls and rigor. He denied history of diabetes mellitus, hypertension, tuberculosis , bronchial asthma .He was evaluated for covid 19. RT PCR for covid 19 came positive. HRCT chest was done. HRCT showed bilateral peripheral and peribronchovascular non lobar distribution of ground glass opacities with septal thickening in bilateral lung parenchyma. The CT severity score was 14/25. HRCT also showed multiple thin walled cyst in both lungs, largest measuring 22 X 27mm. Few subcentrimetric pre/paratracheal , prevascular and sub carinal lymph nodes was noted.His oxygen saturation was 89% on room air. He was admitted and started on oxygen inhalation via face mask and was managed medically. Relevant blood investigation and bio markers for COVID 19 were done. Reports were total count 3570, differential count N51L35M11E1.8B0.6., Hb 15.4 G/dl, platelet count of 2.14 lakhs/mm3, ESR 16 mm/hr, d dimer 0.77mg/l, LDH 203 U/L, CRP 21.774mg/l, procalcitonin 0.122ng/ml. 3 days later dyspnea aggravated and he was started on non invasive ventillation ( BiPAP). His clinical condition was stable on NIV. On the fifth day of NIV, he started developing subcutaneous surgical emphysema. HRCT chest was done, showed gross left and mild right pneumothorax, moderate pneumomediastinum, multiple focal patchy and confluent ill defined areas of ground glass and reticular densities predominant in basal and peripheral distribution involving bilateral lung parenchyma, diffuse subcutaneous emphysema extending into neck spaces.CT severity had increased to 17/25. He was managed by Intercostal chest tube insertion on left 5th intercostal space in mid axillary line connected to a underwater seal. He improved clinically with subcutaneous emphysema resolving in the next 3 days. He was later discharged and his chest tube was removed on followup. We conclude that Birt-Hogg-Dubé syndrome can be a rare cause of secondary pneumothorax in a patient with multiple skin follicles and having multiple cyst in HRCT.
ABSTRACT
Background: As of 20th November 2021, 29.1% (40.1 crore) of the population has been completely vaccinated while 55.6% (76.6 crore) of the population have received at least one dose of vaccine. Infection despite vaccination (vaccine breakthrough) has been reported, but characteristics of these infection regarding clinico demographic profile, severity of disease and mortality in the fully vaccinated, partially vaccinated and non-vaccinated groups are not well described;during the second Covid-19 wave in India from April to June 2021 when the highly transmissible delta variant predominated.