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Pediatrics Gastro Intestinal System f1ba9e84

Gastro intestinal disturbance in cystic fibrosis are all EXCEPT

A
Spleen infact
B
Biliary cirrhosis
C
Malabsorption
D
Gall stones
High-Yield Explanation
A. i.e. Spleen infact) (282,366 - Ghai 6th) (1803 - Nelson I8th)CYSTIC FIBROSIS* Autosomal recessive, mutation affects the gene's protein product cystic fibrosis trans membrane regulator (CFTR) which acts on chloride channelCLINICAL FEATURES-* Intestinal obstruction may be the initial manifestation in the neonatal period (Meconium ileus)**** Two cardinal symptoms of the disease are chronic diarrhea with massive steatorrhoea and recurrent respiratory tract infections, bronchicetasis* Failure to thrive is also a prominent features* *Rectal prolapse also occurs infrequently* *Biliary cirrhosis of liver is often observed oesophageal varices, Hypersplenism* **Malabsorption, insufficient or low bicarbonate with low ph in the intestine* **Increased secretion of chloride in the sweat > 60 meq/L is diagnostic)*** Pancreatic insufficiency is present in > 85% of CF* With increasing age- azoospermia is observed in almost all patientsComplications of cystic fibrosisRespiratoryGITOthers* Bronchiectasis, bronchitis bronchiolitis, pneumonia* Atelectasis, Hempoptysis* Pneumothorax, Nasal polyp.Sinusitis* Reactive airway disease* Cor pulmonale* Respiratory failure* Allergic broncho pulmonary aspergilosis* Meconium ileus. Meconium plug and peritonitis* Distal intestinal obstruction syndrome* Rectal prolapse. Intussuception volvulus. Fibrosing colonopathy (stricture). Appendicitis pancreatitis,** Cholelithiasis, Inguinal hernia* Vitamin deficiency states (A.K.E.D)* Infertility* DM* Amyloidosis* Dehydration heat exhaustion* Hypertrophic osteoarthropathy- arthritis. Clubbing* Delayed puberty

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