Answer: Loss of fat emulsification will result in the malabsorption of vitamins A, C, D, and K

Vitamins are classified as either fat soluble (vitamins A, D, E and K) or water soluble (vitamins B and C). This difference between the two groups is very important. It determines how each vitamin acts within the body. The fat soluble vitamins are soluble in lipids (fats). These vitamins are usually absorbed in fat globules (called chylomicrons) that travel through the lymphatic system of the small intestines and into the general blood circulation within the body. These fat soluble vitamins, especially vitamins A and E, are then stored in body tissues.
Vitamin C is a water soluble vitamin which makes the statement false.
Primary pancreatic maldigestion of carbohydrate, protein, and fat is caused by decreased activity of amylase, trypsin, and lipase. Pancreatic lipase is an enzyme secreted from the pancreas. As the primary lipase enzyme that hydrolyses dietary fat molecules in the human digestive system, it is one of the main digestive enzymes, converting triglyceride substrates found in ingested oils to monoglycerides and free fatty acids.
The duodenum plays a key role in absorption of vitamins and minerals, and is removed in PD resections. This, in combination with malabsorption and increased metabolic demand, results in increased risk of micronutrient deficiencies. Routine supplementation of fat soluble vitamin and trace elements are recommended following resection.

As a reconstructive option after extensive surgery, pedicled musculocutaneous flaps offer several advantages in the setting of previous radiotherapy. Rotational skin flaps will comprise of irradiated tissue and thus won’t heal well.

Optimal physical assessment of an anorectal abscess may require anaesthesia to alleviate patient discomfort that would otherwise limit the extent of the examination.
Drainage should be carried out promptly to prevent the sepsis worsening. Perianal incisions to allow drainage of pus should be circumanal, thus reducing the likelihood of damage to the sphincters and thereby preventing faecal incontinence.

A small number of foreign body aspirations are incidentally found after chest radiography or bronchoscopic inspection. Patients may be asymptomatic or may be undergoing testing for other diagnoses. If present, physical findings may include stridor, fixed wheeze, localized wheeze, or diminished breath sounds. If obstruction is severe, cyanosis may occur. Signs of consolidation can accompany post obstructive pneumonia.

Bronchoscopy can be used diagnostically and therapeutically. Most aspirated foreign bodies are radiolucent. Radiologic procedures do not have extreme diagnostic accuracy, and aspiration events are not always detected. Rigid bronchoscopy usually requires heavy intravenous sedation or general anaesthesia. The rigid bronchoscope has important advantages over the flexible bronchoscope. The larger diameter of the rigid bronchoscope facilitates the passage of various grasping devices, including a flexible bronchoscope. A better chance of quick, successful extraction and better capabilities of suctioning clotted blood and thick secretions are offered by the rigid bronchoscope. The paediatric flexible bronchoscope lacks a hollow working channel through which instruments may be inserted or blood and secretions may be aspirated.
Unlike the flexible bronchoscope, the patient can be ventilated through the rigid scope; therefore, ventilation of the patient can be maintained. Rigid bronchoscopy is the procedure of choice for removing foreign bodies in children and in most adults. Success rates for extracting foreign bodies are reportedly more than 98%. Large solid and semisolid objects are best managed emergently in the operating room with a rigid bronchoscope and appropriate grasping instruments.

Answer: Intestinal malrotation

During normal abdominal development, the 3 divisions of the GI tract (i.e., foregut, midgut, hindgut) herniate out from the abdominal cavity, where they then undergo a 270º counter clockwise rotation around the superior mesenteric vessels. Following this rotation, the bowels return to the abdominal cavity, with fixation of the duodenojejunal loop to the left of the midline and the cecum in the right lower quadrant.

Intestinal malrotation refers to any variation in this rotation and fixation of the GI tract during development. Interruption of typical intestinal rotation and fixation during foetal development can occur at a wide range of locations; this leads to various acute and chronic presentations of disease. The most common type found in paediatric patients is incomplete rotation predisposing to midgut volvulus, requiring emergent operative intervention.

Acute midgut volvulus

Usually occurs during the first year of life

Sudden onset of bilious emesis

Diffuse abdominal pain out of proportion to physical examination

Acute duodenal obstruction

This anomaly is usually recognized in infants and is due to compression or kinking of the duodenum by peritoneal bands (Ladd bands).

Patients present with forceful vomiting, which may or may not be bile-stained, depending on the location of the obstruction with respect to the entrance of the common bile duct (ampulla of Vater).

Malrotation may occur as an isolated anomaly or in association with other congenital anomalies; 30-62% of children with malrotation have an associated congenital anomaly. All children with diaphragmatic hernia, gastroschisis, and omphalocele have intestinal malrotation by definition. Additionally, malrotation is seen in approximately 17% of patients with duodenal atresia and 33% of patients with jejunoileal atresia.

Iron deficiency anaemia is the most common type of anaemia. It can occur due to deficiency of iron due to decreased intake or due to faulty absorption. An MCV less than 80 will indicated iron deficiency anaemia. On the smear the RBC will be microcytic hypochromic and will also show piokilocytosis. iron profiles tests are important to make a diagnosis. Clinically the patient will be pale and lethargic.

Colorectal cancer is one of the most common types of cancer in Western populations. The liver is the first location of metastatic disease; as the main mechanism of dissemination is through the portal system. In addition, the liver may be the sole site of metastasis in 30 to 40% of patients with advanced disease.

Unfortunately, 20% of these patients will develop metastasis in the lungs and >50% in liver. In 20 to 25% of patients at the time of diagnosis, hepatic metastatic disease can be identified clinically, and 40 to 50% will develop during the first 3 years after the primary tumour is diagnosed.
When metastatic lesions are localized in the liver, which corresponds to 30% of patients, there are several options for localized treatment, such as hepatic partial resection, localized ablative therapy, administration of chemotherapy by infusion of the hepatic artery, systemic chemotherapy, and isolated hepatic fusion for patients with high doses of chemotherapy. Surgical resection is the most effective treatment for hepatic metastasis in colorectal cancer, but only a few patients are candidates for initial surgery. Patients with hepatic metastasis that cannot be surgically resected are managed initially with chemotherapy and later are subject to surgery, and these patients present a similar survival rate to those undergoing surgery initially.
Prior to hepatic resection, patients with hepatic metastatic disease frequently receive neoadjuvant chemotherapy, which can aid in disappearing or hidden radiological lesions.
The 5-year survival rate after hepatic resection is 25-40%.

Aflatoxins are naturally occurring toxins produced by the Aspergillus fungus (most often, A. flavus and A. parasiticus). These organisms are common and their native habitat is soil, decaying vegetation and grains. They can contaminate the grain before harvest or after storage, more likely in high-humidity (at least 7%) or high temperature environment of after stressful conditions like drought. Aflatoxins are mycotoxins and also carcinogenic. They get metabolized in the liver to an epoxide, aflatoxin M1. High exposure can lead to acute necrosis, cirrhosis or liver carcinoma. These substances can cause haemorrhage, acute liver damage, oedema, and alteration in digestion, absorption and/or metabolism of nutrients. Although humans are susceptible to these toxins like all other animals, they have a high tolerance level and hence, rarely develop acute aflatoxicosis. However, children are particularly susceptible to exposure leading to growth impairment and delayed development. Chronic exposure carries a high risk of hepatic cancer, due to intercalation of its metabolite aflatoxin M1 into the DNA and alkylation of the bases because of its epoxide moiety.

CHOP is the acronym for a chemotherapy regimen used in the treatment of non-Hodgkin’s lymphoma, comprising cyclophosphamide, hydroxyrubicin (adriamycin), vincristine and prednisone. This regimen can also be combined with the monoclonal antibody rituximab if the lymphoma is of B cell origin; this combination is called R-CHOP.

Answer: Osteogenesis imperfecta

Osteogenesis imperfecta (OI) is a disorder of bone fragility chiefly caused by mutations in the COL1A1 and COL1A2 genes that encode type I procollagen.

Type II – Perinatal lethal
Some providers who treat large numbers of patients with osteogenesis imperfecta suggest that the diagnosis of Type II OI be made in retrospect for patients who do not survive the perinatal period, and that even patients with very severe forms of OI who nonetheless are long term survivors be classified as Type III. Blue sclera may be present. Patients may have a small nose, micrognathia, or both. All patients have in utero fractures, which may involve the skull, long bones, and/or vertebrae. The ribs are beaded, and the long bones are severely deformed.
Causes of death include extreme fragility of the ribs, pulmonary hypoplasia, and malformations or haemorrhages of the CNS.

Type III – Severe, progressively deforming

Patients may have joint hyperlaxity, muscle weakness, chronic unremitting bone pain, and skull deformities (e.g., posterior flattening) due to bone fragility during infancy.

Deformities of upper limbs may compromise function and mobility. The presence of dentinogenesis imperfecta is independent of the severity of the osteogenesis imperfecta. The sclera have variable hues. In utero fractures are common. Limb shortening and progressive deformities can occur. Patients have a triangular face with frontal and temporal bossing. Malocclusion is common. Basilar invagination is an uncommon but potentially fatal occurrence in osteogenesis imperfecta.
Vertigo is common in patients with severe osteogenesis imperfecta. Hypercalciuria may be present in about 36% of patients with osteogenesis imperfecta, and adults may be at higher risk of renal calculi. Respiratory complications secondary to kyphoscoliosis are common in individuals with severe osteogenesis imperfecta.
Constipation and hernias are also common in people with osteogenesis imperfecta.

Obtain a radiographic skeletal survey after birth.

In mild (type I) osteogenesis imperfecta, images may reveal thinning of the long bones with thin cortices. Several wormian bones may be present. No deformity of long bones is observed.

In extremely severe (type II) osteogenesis imperfecta, the survey may reveal beaded ribs, broad bones, and numerous fractures with deformities of the long bones. Platyspondylia may also be revealed.

In moderate and severe (types III and IV) osteogenesis imperfecta, imaging may reveal cystic metaphysis, or a popcorn appearance of the growth cartilage. Normal or broad bones are revealed early, with thin bones revealed later. Fractures may cause deformities of the long bones. Old rib fractures may be present. Vertebral fractures are common.